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Sample records for bronchiectasis

  1. Bronchiectasis

    Science.gov (United States)

    ... disability. Possible Complications Complications of bronchiectasis may include: Cor pulmonale Coughing up blood Low oxygen levels (in severe ... PA: Elsevier Saunders; 2016:chap 90. Read More Cor pulmonale Cystic fibrosis Foreign object - inhaled or swallowed Pneumonia - ...

  2. Bronchiectasis: still a problem

    Institute of Scientific and Technical Information of China (English)

    Wang Zengli

    2014-01-01

    Objective The purpose of this descriptive review was to delineate the current knowledge of bronchiectasis in terms of prevalence,burden of disease,pathophysiology,and management.Data sources The National Library of Medicines MEDLINE and PubMed database (2005-2013) were used to conduct a search using the keyword term "bronchiectasis".The references for articles being considered for inclusion were searched from additional sources such as conference proceedings.Study selection Criteria for inclusion of articles included data outlining epidemiology,pathogenesis,diagnosis,and evidence-based guidelines for management of bronchiectasis.In assessing the quality of the articles,factors such as size of the population studied,clinical setting of the study,and whether or not the studies were prospective or retrospective were taken into consideration.Review articles were also included in our data collection.Results Despite many advances in modem medicine,bronchiectasis still remains a significant public health problem in developed countries and the developing world.It carries a significant burden worldwide in terms of morbidity and mortality,as well as financially to the affected population.In addition,bronchiectasis may associate with chronic airflow obstruction,regardless of smoking status.Conclusions Bronchiectasis is a debilitating illness responsible for significant morbidity with a poor health-related quality of life.The condition has a substantial socioeconomic cost because both primary and secondary healthcare resources are frequently used and periods of sick leave are common.

  3. Bronchiectasis: Phenotyping a Complex Disease.

    Science.gov (United States)

    Chalmers, James D

    2017-03-15

    Bronchiectasis is a long-neglected disease currently experiencing a surge in interest. It is a highly complex condition with numerous aetiologies, co-morbidities and a heterogeneous disease presentation and clinical course. The past few years have seen major advances in our understanding of the disease, primarily through large real-life cohort studies. The main outcomes of interest in bronchiectasis are symptoms, exacerbations, treatment response, disease progression and death. We are now more able to identify clearly the radiological, clinical, microbiological and inflammatory contributors to these outcomes. Over the past couple of years, multidimensional scoring systems such as the Bronchiectasis Severity Index have been introduced to predict disease severity and mortality. Although there are currently no licensed therapies for bronchiectasis, an increasing number of clinical trials are planned or ongoing. While this emerging evidence is awaited, bronchiectasis guidelines will continue to be informed largely by real-life evidence from observational studies and patient registries. Key developments in the bronchiectasis field include the establishment of international disease registries and characterisation of disease phenotypes using cluster analysis and biological data.

  4. Macrolide maintenance treatment for bronchiectasis

    NARCIS (Netherlands)

    Altenburg, Josje

    2017-01-01

    Bronchiectasis, pathological widening of the small and medium sized bronchi, may result from various disorders with one common trait; a faltering airway defence system. This allows for persistent bacterial infection and an augmented airway inflammatory response. Patients’ suffering is often consider

  5. Global impact of bronchiectasis and cystic fibrosis

    Directory of Open Access Journals (Sweden)

    Margarida Redondo

    2016-09-01

    To understand variation in the aetiology, microbiology and burden of bronchiectasis and cystic fibrosis across different global healthcare systems.; Bronchiectasis is the term used to refer to dilatation of the bronchi that is usually permanent and is associated with a clinical syndrome of cough, sputum production and recurrent respiratory infections. It can be caused by a range of inherited and acquired disorders, or may be idiopathic in nature. The most well recognised inherited disorder in Western countries is cystic fibrosis (CF, an autosomal recessive condition that leads to progressive bronchiectasis, bacterial infection and premature mortality. Both bronchiectasis due to CF and bronchiectasis due to other conditions are placing an increasing burden on healthcare systems internationally. Treatments for CF are becoming more effective leading to more adult patients with complex healthcare needs. Bronchiectasis not due to CF is becoming increasingly recognised, particularly in the elderly population. Recognition is important and can lead to identification of the underlying cause, appropriate treatment and improved quality of life. The disease is highly diverse in its presentation, requiring all respiratory physicians to have knowledge of the different “bronchiectasis syndromes”. The most common aetiologies and presenting syndromes vary depending on geography, with nontuberculous mycobacterial disease predominating in some parts of North America, post-infectious and idiopathic disease predominating in Western Europe, and post-tuberculosis bronchiectasis dominating in South Asia and Eastern Europe. Ongoing global collaborative studies will greatly advance our understanding of the international impact of bronchiectasis and CF.

  6. Pharmacologic agents for mucus clearance in bronchiectasis.

    Science.gov (United States)

    Nair, Girish B; Ilowite, Jonathan S

    2012-06-01

    There are no approved pharmacologic agents to enhance mucus clearance in non-cystic fibrosis (CF) bronchiectasis. Evidence supports the use of hyperosmolar agents in CF, and studies with inhaled mannitol and hypertonic saline are ongoing in bronchiectasis. N-acetylcysteine may act more as an antioxidant than a mucolytic in other lung diseases. Dornase α is beneficial to patients with CF, but is not useful in patients with non-CF bronchiectasis. Mucokinetic agents such as β-agonists have the potential to improve mucociliary clearance in normals and many disease states, but have not been adequately studied in patients with bronchiectasis.

  7. Chest pain and exacerbations of bronchiectasis

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    King PT

    2012-12-01

    Full Text Available Paul T King,1,2 Stephen R Holdsworth,2 Michael Farmer,1 Nicholas J Freezer,1 Peter W Holmes11Department of Respiratory and Sleep Medicine, 2Monash University Department of Medicine, Monash Medical Centre, Melbourne, Victoria, AustraliaBackground: Bronchiectasis is a common disease and a major cause of respiratory morbidity. Chest pain has been described as occurring in the context of bronchiectasis but has not been well characterized. This study was performed to describe the characteristics of chest pain in adult bronchiectasis and to define the relationship of this pain to exacerbations.Subjects and methods: We performed a prospective study of 178 patients who were followed-up for 8 years. Subjects were reviewed on a yearly basis and assessed for the presence of chest pain. Subjects who had chest pain at the time of clinical review by the investigators were included in this study. Forty-four patients (25% described respiratory chest pain at the time of assessment; in the majority of cases 39/44 (89%, this occurred with an exacerbation and two distinct types of chest pain could be described: pleuritic (n = 4 and non-pleuritic (n = 37, with two subjects describing both forms. The non-pleuritic chest pain occurred most commonly over both lower lobes and was mild to moderate in severity. The pain subsided as patients recovered. Conclusion: Non-pleuritic chest pain occurs in subjects with bronchiectasis generally in association with exacerbations.Keywords: sputum, collapse, bronchitis, airway obstruction

  8. Resolution of extensive severe bronchiectasis in an infant.

    Science.gov (United States)

    Crowley, Suzanne; Matthews, Iren

    2010-07-01

    Bronchiectasis is, by definition, an irreversible condition. Following recent reports of reversible bronchiectasis in children, it has been suggested that the definition be broadened to include pre-bronchiectasis and transitional reversible states. We describe the case of a young infant who had extensive, severe bronchiectasis of unknown etiology that resolved following prolonged treatment with antibiotics and a tapering course of oral steroids. We suggest that the prolonged treatment may have played a role, perhaps by eradicating infection and thus enabling regeneration of bronchial anatomy.

  9. Diffuse bronchiectasis as the primary manifestation of endobronchial sarcoidosis

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    Paul D. Hiles

    2017-01-01

    Full Text Available Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa. Endobronchial biopsies and lymphocyte subset analysis of bronchoalveolar lavage fluid were consistent with a diagnosis of sarcoidosis. We believe endobronchial sarcoidosis should be included in the differential diagnosis of patients presenting with bronchiectasis.

  10. Antiglomerular basement membrane antibody-crescentic glomerulonephritis complicating chronic bronchiectasis.

    Science.gov (United States)

    Enríquez, R; Cabezuelo, J B; Sirvent, A E; Andrada, E; Amorós, F; Orti, C

    2001-04-01

    A 68-year-old woman with chronic bronchiectasis presented with haematuria and severe oligoanuric renal failure with no other serious systemic manifestation. Antiglomerular basement membrane (anti-GBM) antibodies and anti-myeloperoxidase antibodies were positive. Renal biopsy revealed anti-GBM crescentic glomerulonephritis. A conservative approach was followed and the patient is stable on chronic haemodialysis 6 months later. To the authors' knowledge, there has only been one previous report of anti-GBM disease complicating bronchiectasis.

  11. Thoracoscopic management for bronchiectasis with non-tuberculous mycobacterial infection

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Background Non-tuberculous mycobacteria (NTM) have emerged as important opportunistic pathogens of the human being in recent years. Patients with pre-existing bronchiectasis are susceptible to NTM. However, information about its occurrence among bronchiectatic patients in Shenzhen, China is lacking and its impact on the course of bronchiectasis following surgical intervention is unknown. This preliminary study aimed to investigate the prevalence of NTM in bronchiectasis that required surgery in our center, evaluate the role of intraoperative routine screening for NTM, and summarize our initial experience in thoracoscopic management for bronchiectatic patients with NTM. Methods A retrospective analysis of clinical, microbiological data of our bronchiectatic patients with NTM over 5 years was made and 40 patients with bronchiectasis were studied to determine the role of intraoperative routine screening for NTM. Results The prevalence of NTM in this population of patients with bronchiectasis in our center was 6.7% (7/105). The diagnostic yield of the 40 intraoperative specimens was 7.5% (3/40). Of the 7 patients with bronchiectasis and NTM, 3 patients developed postoperative wound infections. All were cured with chemotherapy for 8-12 months along with vigorous surgical debridement. Another patient had a slow growth of mycobacteria involving double lungs and the right thoracic cavity and recovered after chemotherapy for nearly 14 months and tube drainage. The affected tissue was completely resected in the remaining 3 patients with no operative mortality and postoperative morbidity, and routine intraoperative screening for NTM was initiated in these patients. Conclusions NTM is not uncommon in bronchiectatic patients which deserves surgeons' utmost attention. Routine intraoperative screening for NTM identified otherwise unsuspected patients has shown favorable outcomes. Thoracoscopic management for bronchiectasis with NTM is technically feasible although its role

  12. Factors Affecting the Outcome of Bronchiectasis in Pediatric Patients

    Directory of Open Access Journals (Sweden)

    Nemat Bilan

    2014-12-01

    Full Text Available Introduction: Bronchiectasis is a common problem in children and early diagnosis can lead to early treatment and prevent of its complications. This study was aimed to evaluate factors effective on outcome of bronchiectasis in children. Methods: In an analytical cross-sectional study, 347 children with bronchiectasis Underwent the study. the patients were diagnosed based on chronic suppurative cough and CT scan findings. . Results: Disease etiology was asthma in 55.6%, Gastroesophagial reflux (GERD in 7.8%, Cystic fibrosis (CF in 4.8%, other causes in 11.2% and idiopathic in 20.6%. All cases complained of chronic cough. The most common sign was daily sputum production (79.1% and common symptoms were ral/crackle in 47.1% and wheezing in 25.4%. Mean treatment period was 32.82±11.56 months. At the end of follow-up, complete improvement occurred in 35.6%, partial improvement in 40.9% and no improvement in 23.5%. Conclusion: In children with chronic cough and crackle in physical examination, consideration of bronchiectasis could be helpful in early diagnosis and complementary evaluations and treatment initiation. Treating the underlying disease could prevent the occurrence and increase the response to treatment of bronchiectasis.

  13. Current management of bronchiectasis: review and 3 case studies.

    Science.gov (United States)

    Silverman, Enid; Ebright, Linda; Kwiatkowski, Marianne; Cullina, Joanne

    2003-01-01

    Bronchiectasis is the abnormal, irreversible dilatation of diseased bronchi. Permanently dilated airways, usually in the medium-sized bronchi, are inflamed and often obstructed with thick, purulent secretions. Known causative factors include postinfection bronchial damage, postinhalation injury, hypersensitivity reactions, and congenital airway obstructive disorders. Typical symptoms include sputum overproduction, fever, pleurisy, dyspnea, and chronic cough. Diagnosis involves radiographic studies and pulmonary function testing. Treatment includes oral, aerosolized, or intravenous antibiotic therapy according to the severity of the exacerbation, and mucus clearance by means of bronchial hygiene assistive devices, chest physiotherapy, postural drainage, and high-frequency chest compression. We present a review of bronchiectasis and offer 3 case studies illustrating current management of different presentations, including use of aerosolized antibiotics for patients infected with Pseudomonas aeruginosa. Although an adjunctive program of pulmonary rehabilitation may be useful for patients with bronchiectasis, no confirming studies have been performed to date, and additional research in this area is warranted.

  14. KARTAGENER SYNDROME: A TRIAD OF CHRONIC SINUSITIS, BRONCHIECTASIS AND SITUSINVERSUS

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    Arvinder

    2013-02-01

    Full Text Available ABSTRACT: Kartagener syndrome is an autosomal recessive disor der with a triad of bronchiectasis, recurrent episodes of sinusitis and situs inversus in early life, with reduced fertility in the later part of life. It is a rare ge netic disorder of primary ciliary dyskinesia (PCD with an estimated incidence of 1: 20,000 to 30,000. Here we present an 11 year old male child who presented with bronchiectasis, recurrent episodes of sinusitis and situs inversus which fits into the triad of Kartagener syndrome. Early diagno sis and treatment of this syndrome is important to prevent the deterioration of the lung f unction and the morbidity caused due to severe intractable upper and lower respiratory tract infections

  15. Evaluation of Humoral Immune Function in Patients with Bronchiectasis

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    Parviz Tabatabaie

    2008-06-01

    Our study along with several other studies confirmed that all patients with bronchiectasis should undergo thorough immunological evaluation in order to identify the presence of the underlying immunologic defect. This evaluation should include serum immunoglobulins, IgG subclasses concentrations and also determination of serum antibodies against pneumococcal antigens. Early diagnosis and appropriate treatment will prevent the subsequent complications and improve quality of life of affected individuals.

  16. Completely thoracoscopic lobectomy for the surgical management of bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    ZHOU Zu-li; ZHAO Hui; LI Yun; LI Jian-feng; JIANG Guan-chao; WANG Jun

    2013-01-01

    Background The feasibility of completing a Iobectomy by completely video-assisted thoracoscopic surgery (cVATS) in the management of bronchiectasis is unclear.By retrospectively comparing the outcomes from the Iobectomies that used thoracotomy vs.cVATS,we determined the appropriateness of the minimally invasive cVATS approach in the management of bronchiectasis.Methods Between June 2001 and October 2010,60 patients with bronchiectasis underwent surgery,of which 56 Iobectomies were performed.All Iobectomies were carried out by either thoracotomy or cVATS approach.Pulmonary vessels and bronchi were manipulated by ligation or stapler in the thoracotomy group,while they were dissected by endo-cutters in the cVATS group.Results There were 21 patients in the thoracotomy group and 35 patients in the cVATS group.Two cVATS patients (5.7%) converted.The difference in operation time,chest tube duration,lengths of hospitalization,and morbidity were not significantly different between the two groups (P >0.05).The blood loss was less in the cVATS group (P=0.015).A total of 52.4% and 62.9% of patients were postoperatively asymptomatic in the thoracotomy and cVATS groups respectively,and symptomatic improvement was obtained in 38.1% patients by thoracotomy vs.31.4% patients by cVATS.Conclusion cVATS Iobectomy is safe and effective for the management of bronchiectasis,especially for the patients with localized lesions.

  17. Prevalence of nontuberculous mycobacteria in patients with bronchiectasis: a meta-analysis

    OpenAIRE

    Chu,Haiqing; Zhao, Lan; Xiao, Heping; Zhang, Zhemin; Zhang, Jinbo; Gui, Tao; Gong, Sugang; Xu, Liyun; Sun, Xiwen

    2014-01-01

    Introduction Nontuberculous mycobacteria (NTM) have emerged as critical opportunistic pathogens of lung diseases recently. Patients with preexisting bronchiectasis are susceptible to NTM. Nevertheless, patients with preexisting bronchiectasis are susceptible to NTM but the prevalence of NTM pulmonary infection in different species and geographical areas is still not fully understood. Material and methods The relevant data of the prevalence of NTM in patients with bronchiectasis were retrieved...

  18. Influence of Pseudomonas aeruginosa on exacerbation in patients with bronchiectasis

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    Kiran Chawla

    2015-01-01

    Full Text Available Background: A majority of the studies done on the western population have shown that Pseudomonas aeruginosa causes many severe infections in patients with bronchiectasis as compared to other pathogens. There is scarcity of similar data from the Asian population. Materials and Methods: A prospective study was undertaken to identify the various pathogens isolated from the respiratory samples of 117 patients with bronchiectasis from south India and to compare the clinicomicrobiological profile of infections caused by P. aeruginosa and other respiratory pathogens. Results: The respiratory pathogens were isolated from 63 (53.8% patients. P. aeruginosa was the most common isolate (46.0% followed by Klebsiella pneumoniae (14.3% and other pathogenic bacteria. Patients included in the P. aeruginosa group had a higher number of exacerbations (p: 0.008, greater number of hospital admissions (p: 0.007, a prolonged hospital stay (p: 0.03, and poor lung function, compared to the patients infected with the non-Pseudomonas group. Conclusion: It is necessary to investigate the etiology of respiratory tract infections among bronchiectasis patients followed by the prompt management of cases diagnosed with P. aeruginosa infections, so as to lower the morbidity and have a better prognosis.

  19. Could a defective epithelial sodium channel lead to bronchiectasis

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    Hubert Dominique

    2008-05-01

    Full Text Available Abstract Background Bronchiectasis is defined as a permanent dilation of the airways arising from chronic bronchial inflammation/infection. In 50% of cases, no etiology can be identified. Recently, the role of the epithelial sodium channel ENaC has been pointed out in the pathophysiology of cystic fibrosis, a disease due to mutations in the CFTR gene and causing bronchiectasis in the airways. Moreover, it was found that transgenic mice overexpressing ENaCβ present cystic fibrosis-like lung disease symptoms. Our aim was to evaluate if a defective ENaC protein could be involved in the development of bronchiectasis. Methods We extensively analysed ENaCβ and γ genes in 55 patients with idiopathic bronchiectasis and without two mutations in the coding regions of CFTR. Thirty-eight patients presented functional abnormalities suggesting impaired sodium transport (abnormal sweat chloride concentration or nasal potential difference measurement, and 17 had no such evidence. Results Sequencing of the exons and flanking introns of the ENaCβ and γ gene identified five different amino-acid changes (p.Ser82Cys, p.Pro369Thr, p.Asn288Ser in ENaCβ ; and p.Gly183Ser, p.Glu197Lys in ENaCγ in heterozygous state in 8 patients. The p.Ser82Cys amino-acid change was found in 3 unrelated patients who were also heterozygous for a CFTR mutation or variant (1 p.F508del, 1 IVS8-5T, and 1 IVS8-5T:1716G>A (p.E528E. The other mutations were found in patients without CFTR mutation, the p.Glu197Lys mutation in 2 patients and the other variants in single patients. Among the 8 patients bearing an ENaC mutation, 5 had functional abnormalities suggesting impaired sodium transport. Conclusion Our results suggest that several variants in ENaCβ and γ genes might be deleterious for ENaC function and lead to bronchiectasis, especially in patients who are trans-heterozygotes for ENaCβ/CFTR mutations or variants.

  20. The evaluation of radiological and clinical findings of bronchiectasis

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    Yoo, Jung Keun; Kang, Sung Ihn; Kim, Kil Jung; Ko, Seung Sook; Kim, Young Sook; Kim, Young Chul [Chosun University College of Medicine, Kwangju (Korea, Republic of)

    1985-10-15

    Bronchiectasis means a permanent abnormal dilatation off one or more large bronchi owing to destruction of the elastic and muscular components of the bronchial wall. Radiological study is the most important and mandatory procedure. Especially bronchography is essential for the definitive diagnosis of bronchiectasis and for the precise delineation of the type and extent of the disease. The radiological and clinical findings of 48 cases of bronchiectasis diagnosed by bronchography and treated at Chosun University Hospital during the 5 years from January 1980 to December 1984 were analyzed retrospectively. The results were as follows; 1. Among the 48 cases, 34 cases (70.8%) were male and 14 cases (29.2%) were female. Peak incidence was in second decade. 2. Chronic cough productive sputum and hemoptysis are main symptoms and others are chest pain, dyspnea and recurrent bouts of pneumonia. The most common physical sign is persistent moist rales over the involved area in 23 cases (47.9%). Others are no sign in 17 cases (35.4%), wheezing in 11 cases (22.9%) and digit clubbing in 3 cases (6.3%). 3. The presumed causes were composed of not known in 30 cases (62.5%)> and complications of measles in 7 cases (14.6%), pertussis in 5 cases (10.4%) and pneumonia in 4 cases (8.3%). Two cases were Kartagener's syndrome and unilateral hyperlucent lung. 4. Plain chest common radiological findings was accentuation of lung marking in 36 cases (85.7%), the others are include in order of frequency; pneumonic infiltration, linear radiolucencies, cystic radiolucencies, decreased affected lung volume, air-fluid, level and pleural thickening. 5. Bilateral bronchiectasis was demonstrated in 11 cases (22.9%) and the disease was much more often involved left lung than right. The most commonly involved lobe is left lower lobe, and the most common site of involvement was the posterior basal segment of the lower lobe. The type of bronchiectasis is cylindrical in 22 cases (45.8%), varicose in

  1. The EMBARC European Bronchiectasis Registry: protocol for an international observational study

    Science.gov (United States)

    Aliberti, Stefano; Polverino, Eva; Vendrell, Montserrat; Crichton, Megan; Loebinger, Michael; Dimakou, Katerina; Clifton, Ian; van der Eerden, Menno; Rohde, Gernot; Murris-Espin, Marlene; Masefield, Sarah; Gerada, Eleanor; Shteinberg, Michal; Ringshausen, Felix; Haworth, Charles; Boersma, Wim; Rademacher, Jessica; Hill, Adam T.; Aksamit, Timothy; O'Donnell, Anne; Morgan, Lucy; Milenkovic, Branislava; Tramma, Leandro; Neves, Joao; Menendez, Rosario; Paggiaro, Perluigi; Botnaru, Victor; Skrgat, Sabina; Wilson, Robert; Goeminne, Pieter; De Soyza, Anthony; Welte, Tobias; Torres, Antoni; Elborn, J. Stuart; Blasi, Francesco

    2016-01-01

    Bronchiectasis is one of the most neglected diseases in respiratory medicine. There are no approved therapies and few large-scale, representative epidemiological studies. The EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) registry is a prospective, pan-European observational study of patients with bronchiectasis. The inclusion criterion is a primary clinical diagnosis of bronchiectasis consisting of: 1) a clinical history consistent with bronchiectasis; and 2) computed tomography demonstrating bronchiectasis. Core exclusion criteria are: 1) bronchiectasis due to known cystic fibrosis; 2) age <18 years; and 3) patients who are unable or unwilling to provide informed consent. The study aims to enrol 1000 patients by April 2016 across at least 20 European countries, and 10 000 patients by March 2020. Patients will undergo a comprehensive baseline assessment and will be followed up annually for up to 5 years with the goal of providing high-quality longitudinal data on outcomes, treatment patterns and quality of life. Data from the registry will be available in the form of annual reports. and will be disseminated in conference presentations and peer-reviewed publications. The European Bronchiectasis Registry aims to make a major contribution to understanding the natural history of the disease, as well as guiding evidence-based decision making and facilitating large randomised controlled trials. PMID:27730179

  2. The EMBARC European Bronchiectasis Registry: protocol for an international observational study

    Directory of Open Access Journals (Sweden)

    James D. Chalmers

    2016-01-01

    Full Text Available Bronchiectasis is one of the most neglected diseases in respiratory medicine. There are no approved therapies and few large-scale, representative epidemiological studies. The EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration registry is a prospective, pan-European observational study of patients with bronchiectasis. The inclusion criterion is a primary clinical diagnosis of bronchiectasis consisting of: 1 a clinical history consistent with bronchiectasis; and 2 computed tomography demonstrating bronchiectasis. Core exclusion criteria are: 1 bronchiectasis due to known cystic fibrosis; 2 age <18 years; and 3 patients who are unable or unwilling to provide informed consent. The study aims to enrol 1000 patients by April 2016 across at least 20 European countries, and 10 000 patients by March 2020. Patients will undergo a comprehensive baseline assessment and will be followed up annually for up to 5 years with the goal of providing high-quality longitudinal data on outcomes, treatment patterns and quality of life. Data from the registry will be available in the form of annual reports. and will be disseminated in conference presentations and peer-reviewed publications. The European Bronchiectasis Registry aims to make a major contribution to understanding the natural history of the disease, as well as guiding evidence-based decision making and facilitating large randomised controlled trials.

  3. Using Cystic Fibrosis Therapies for Non-Cystic Fibrosis Bronchiectasis.

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    ElMaraachli, Wael; Conrad, Douglas J; Wang, Angela C C

    2016-03-01

    Non-cystic fibrosis bronchiectasis (NCFB) is an increasingly prevalent disease that places a significant burden on patients and health systems globally. Although many of the therapies used to treat NCFB were originally developed as cystic fibrosis (CF) therapies, not all of them have been demonstrated to be efficacious in NCFB and some may even be harmful. This article explores the evidence for which therapeutic strategies used to treat CF have been translated into the care of NCFB. The conclusion is that therapies for adult NCFB cannot be simply extrapolated from CF clinical trials, and in some instances, doing so may actually result in harm.

  4. Antibiotic therapy for stable non-CF bronchiectasis in adults

    DEFF Research Database (Denmark)

    Fjaellegaard, Katrine; Sin, Melda Dönmez; Browatzki, Andrea

    2016-01-01

    shown to improve QoL and exacerbation rate, whereas findings regarding sputum production, lung function and admissions have been conflicting. Evidence-based treatment algorithms for antibiotic treatment of stable non-CF BE will have to await large-scale, long-term controlled studies.......To provide an update on efficacy and safety of antibiotic treatments for stable non-cystic fibrosis (CF) bronchiectasis (BE). Systematic review based on the Preferred Reporting Items for Systematic reviews and Meta-Analyses guidelines was done. Twenty-six studies (1.898 patients) fulfilled......, exacerbations and QoL, whereas studies on aztreonam revealed no significant clinical improvements in the outcomes of interest, including exacerbation rate. Adverse events, including bronchospasm, have been reported in association with tobramycin and aztreonam. Several antibiotic treatment regimens have been...

  5. Left Pulmonary Agenesis with Right Lung Bronchiectasis in an Adult.

    Science.gov (United States)

    Kumar, Prabhat; Tansir, Ghazal; Sasmal, Gargi; Dixit, Juhi; Sahoo, Ratnakar

    2016-09-01

    Pulmonary agenesis is a rare congenital anomaly characterized by the absence of pulmonary parenchyma and vasculature. Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral agenesis is often associated with other congenital cardiovascular, genitourinary and gastrointestinal malformations. Right lung agenesis is more frequently associated with congenital anomalies and has poor prognosis as compared to left lung agenesis. Diagnosis is often made in childhood but can be delayed, if the clinician is not aware about this entity. Chest radiograph in unilateral lung agenesis shows opaque hemithorax and these patients are often confused with other common causes of opaque hemithorax like collapse, pleural effusion and diaphragmatic hernia. We report a case of left lung agenesis with right lung bronchiectasis in a middle-aged adult who was treated for tuberculous pleural effusion and was referred to our institute for persistent symptoms despite treatment.

  6. Left Pulmonary Agenesis with Right Lung Bronchiectasis in an Adult

    Science.gov (United States)

    Tansir, Ghazal; Sasmal, Gargi; Dixit, Juhi; Sahoo, Ratnakar

    2016-01-01

    Pulmonary agenesis is a rare congenital anomaly characterized by the absence of pulmonary parenchyma and vasculature. Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral agenesis is often associated with other congenital cardiovascular, genitourinary and gastrointestinal malformations. Right lung agenesis is more frequently associated with congenital anomalies and has poor prognosis as compared to left lung agenesis. Diagnosis is often made in childhood but can be delayed, if the clinician is not aware about this entity. Chest radiograph in unilateral lung agenesis shows opaque hemithorax and these patients are often confused with other common causes of opaque hemithorax like collapse, pleural effusion and diaphragmatic hernia. We report a case of left lung agenesis with right lung bronchiectasis in a middle-aged adult who was treated for tuberculous pleural effusion and was referred to our institute for persistent symptoms despite treatment. PMID:27790501

  7. Severe bronchiectasis in a dog: tomographic evaluation; Severa bronquiectasia em um cao: avaliacao tomografica

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    Babicsak, Viviam Rocco; Campos, Lidice Araujo; Belotta, Alexandra Frey; Oliveira, Hugo Salvador de; Inamassu, Leticia Rocha; Charlier, Murilo Gomes de Soutello; Mamprim, Maria Jaqueline; Vulcano, Luiz Carlos, E-mail: viviam.babicsak@gmail.com [Universidade Estadual Paulista Julio de Mesquita Filho (FMVZ/UNESP), Botucatu, SP (Brazil). Faculdade de Medicina Veterinaria e Zootecnia. Dept. de Reproducao Animal e Radiologia Veterinaria

    2012-07-01

    Bronchiectasis is a bronchial dilatation caused by changes in the bronchial wall usually due to infectious processes. Computed tomography is an important imaging modality for the evaluation of this alteration due to its high sensitivity. In this study we describe the case report of a 6-year-old female mixed breed dog with severe and generalized bronchiectasis on computed tomography. Due to the severe extent of bronchiectasis, the method of measurement of the adjacent pulmonary artery and its relationship to the bronchus did not have to be performed. An indirect sign of bronchiectasis identified in the patient was the bronchial wall thickening, possibly due to the infiltration of inflammatory components, edema and even by peri-bronchial abscesses. (author)

  8. Radiologic and clinical bronchiectasis associated with autosomal dominant polycystic kidney disease.

    Directory of Open Access Journals (Sweden)

    Teng Moua

    Full Text Available BACKGROUND: Polycystin 1 and 2, the protein abnormalities associated with autosomal dominant polycystic kidney disease (ADPKD, are also found in airway cilia and smooth muscle cells. There is evidence of increased radiologic bronchiectasis associated with ADPKD, though the clinical and functional implications of this association are unknown. We hypothesized an increased prevalence of both radiologic and clinical bronchiectasis is associated with APDKD as compared to non-ADPKD chronic kidney disease (CKD controls. MATERIALS AND METHODS: A retrospective case-control study was performed at our institution involving consecutive ADPKD and non-ADPKD chronic kidney disease (CKD patients seen over a 13 year period with both chest CT and PFT. CTs were independently reviewed by two blinded thoracic radiologists. Manually collected clinical data included symptoms, smoker status, transplant history, and PFT findings. RESULTS: Ninety-two ADPKD and 95 non-ADPKD CKD control patients were compared. Increased prevalence of radiologic bronchiectasis, predominantly mild lower lobe disease, was found in ADPKD patients compared to CKD control (19 vs. 9%, P = 0.032, OR 2.49 (CI 1.1-5.8. After adjustment for covariates, ADPKD was associated with increased risk of radiologic bronchiectasis (OR 2.78 (CI 1.16-7.12. Symptomatic bronchiectasis occurred in approximately a third of ADPKD patients with radiologic disease. Smoking was associated with increased radiologic bronchiectasis in ADPKD patients (OR 3.59, CI 1.23-12.1. CONCLUSIONS: Radiological bronchiectasis is increased in patients with ADPKD particularly those with smoking history as compared to non-ADPKD CKD controls. A third of such patients have symptomatic disease. Bronchiectasis should be considered in the differential in ADPKD patients with respiratory symptoms and smoking history.

  9. Bacterial colonisation in patients with bronchiectasis: microbiological pattern and risk factors

    Science.gov (United States)

    Angrill, J; Agusti, C; de Celis, R; Rano, A; Gonzalez, J; Sole, T; Xaubet, A; Rodriguez-Roisin, R; Torres, A

    2002-01-01

    Background: A study was undertaken to investigate the incidence, diagnostic yield of non-invasive and bronchoscopic techniques, and risk factors of airway colonisation in patients with bronchiectasis in a stable clinical situation. Methods: A 2 year prospective study of 77 patients with bronchiectasis in a stable clinical condition was performed in an 800 bed tertiary university hospital. The interventions used were pharyngeal swabs, sputum cultures and quantitative protected specimen brush (PSB) bacterial cultures (cut off point ≥102 cfu/ml) and bronchoalveolar lavage (BAL) (cut off point ≥103 cfu/ml). Results: The incidence of bronchial colonisation with potential pathogenic microorganisms (PPMs) was 64%. The most frequent PPMs isolated were Haemophilus influenzae (55%) and Pseudomonas spp (26%). Resistance to antibiotics was found in 30% of the isolated pathogens. When the sample was appropriate, the operative characteristics of the sputum cultures were similar to those obtained with the PSB taken as a gold standard. Risk factors associated with bronchial colonisation by PPMs in the multivariate analysis were: (1) diagnosis of bronchiectasis before the age of 14 years (odds ratio (OR)=3.92, 95% CI 1.29 to 11.95), (2) forced expiratory volume in 1 second (FEV1) <80% predicted (OR=3.91, 95% CI 1.30 to 11.78), and (3) presence of varicose or cystic bronchiectasis (OR=4.80, 95% CI 1.11 to 21.46). Conclusions: Clinically stable patients with bronchiectasis have a high prevalence of bronchial colonisation by PPMs. Sputum culture is a good alternative to bronchoscopic procedures for evaluation of this colonisation. Early diagnosis of bronchiectasis, presence of varicose-cystic bronchiectasis, and FEV1 <80% predicted appear to be risk factors for bronchial colonisation with PPMs. PMID:11809984

  10. The immune response and its therapeutic modulation in bronchiectasis.

    Science.gov (United States)

    Daheshia, Massoud; Prahl, James D; Carmichael, Jacob J; Parrish, John S; Seda, Gilbert

    2012-01-01

    Bronchiectasis (BC) is a chronic pulmonary disease with tremendous morbidity and significant mortality. As pathogen infection has been advocated as a triggering insult in the development of BC, a central role for the immune response in this process seems obvious. Inflammatory cells are present in both the airways as well as the lung parenchyma, and multiple mediators of immune cells including proteases and cytokines or their humoral products are increased locally or in the periphery. Interestingly, a defect in the immune system or suppression of immune response during conditions such as immunodeficiency may well predispose one to the devastating effects of BC. Thus, the outcome of an active immune response as detrimental or protective in the pathogenesis of BC may be dependent on the state of the patient's immunity, the severity of infection, and the magnitude of immune response. Here we reassess the function of the innate and acquired immunity in BC, the major sites of immune response, and the nature of the bioactive mediators. Furthermore, the potential link(s) between an ongoing immune response and structural alterations accompanying the disease and the success of therapies that can modulate the nature and extent of immune response in BC are elaborated upon.

  11. The development of bronchiectasis on chest computed tomography in children with cystic fibrosis: can pre-stages be identified?

    Energy Technology Data Exchange (ETDEWEB)

    Tepper, Leonie A. [Sophia Children' s Hospital, Department of Pediatric Pulmonology, Erasmus MC, Rotterdam (Netherlands); Sophia Children' s Hospital, Department of Radiology, Erasmus MC, Rotterdam (Netherlands); Caudri, Daan [Sophia Children' s Hospital, Department of Pediatric Pulmonology, Erasmus MC, Rotterdam (Netherlands); Perez Rovira, Adria [Sophia Children' s Hospital, Department of Pediatric Pulmonology, Erasmus MC, Rotterdam (Netherlands); Erasmus MC, Biomedical Imaging Group Rotterdam, Departments of Radiology and Medical Informatics, Rotterdam (Netherlands); Tiddens, Harm A.W.M. [Sophia Children' s Hospital, Department of Pediatric Pulmonology, Erasmus MC, Rotterdam (Netherlands); Sophia Children' s Hospital, Department of Radiology, Erasmus MC, Rotterdam (Netherlands); Sophia Children' s Hospital, Department of Pediatric Pulmonology and Radiology, Erasmus Medical Center, Rotterdam (Netherlands); Bruijne, Marleen de [Erasmus MC, Biomedical Imaging Group Rotterdam, Departments of Radiology and Medical Informatics, Rotterdam (Netherlands); University of Copenhagen, Department of Computer Science, Copenhagen (Denmark)

    2016-12-15

    Bronchiectasis is an important component of cystic fibrosis (CF) lung disease but little is known about its development. We aimed to study the development of bronchiectasis and identify determinants for rapid progression of bronchiectasis on chest CT. Forty-three patients with CF with at least four consecutive biennial volumetric CTs were included. Areas with bronchiectasis on the most recent CT were marked as regions of interest (ROIs). These ROIs were generated on all preceding CTs using deformable image registration. Observers indicated whether: bronchiectasis, mucus plugging, airway wall thickening, atelectasis/consolidation or normal airways were present in the ROIs. We identified 362 ROIs on the most recent CT. In 187 (51.7 %) ROIs bronchiectasis was present on all preceding CTs, while 175 ROIs showed development of bronchiectasis. In 139/175 (79.4 %) no pre-stages of bronchiectasis were identified. In 36/175 (20.6 %) bronchiectatic airways the following pre-stages were identified: mucus plugging (17.7 %), airway wall thickening (1.7 %) or atelectasis/consolidation (1.1 %). Pancreatic insufficiency was more prevalent in the rapid progressors compared to the slow progressors (p = 0.05). Most bronchiectatic airways developed within 2 years without visible pre-stages, underlining the treacherous nature of CF lung disease. Mucus plugging was the most frequent pre-stage. (orig.)

  12. Reproducibility of step tests in patients with bronchiectasis

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    Anderson A. Camargo

    2013-06-01

    Full Text Available BACKGROUND: The step test has been used to assess exercise capacity in patients with chronic respiratory disease; however, its use has not been described with regard to patients with bronchiectasis (BCT. OBJECTIVE: This study assessed the reliability of the Chester step test (CST and the modified incremental step test (MIST and also correlated these tests with pulmonary function, heart rate (HR, and distance walked during the 6-min walk test (6-MWT. METHOD: On separate days, 17 patients randomly underwent two CSTs, two MISTs, and two 6-MWTs. Number of steps (NOSs, HR, and perceived exertion were recorded immediately before and after these tests. RESULTS: NOSs were similar across CSTs (124±65 and 125±67 and MISTs (158±83 and 156±76. Differences were not found across the CSTs and MISTs with regard to HR (138±25 bpm and 136±27 bpm, SpO2 (91±5% and 91±3%, perceived exertion (dyspnea=4 [3-5] and 4 [2-4.5] and fatigue (4 [2-6] and 4 [3-5]. The CST was significantly briefer than the MIST (6.0±2.2 min and 8.6±3.0 min and had fewer associated NOS (125±67 and 158±83. NOSs were correlated with FEV1, the 6-MWD, and HR for both tests. CONCLUSIONS: The CST and MIST are reliable in patients with BCT. Patients tolerated the MIST more than the CST. Better lung function and 6-MWT scores predicted the greater NOSs and greater peak HR.

  13. ASSOCIATION OF BRONCHIECTASIS IN MODERATE TO SEVERE COPD PATIENTS ATTENDING KATURI MEDICAL COLLEGE HOSPITAL, GUNTUR FROM 2011 - 2013

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    Ramakrishna

    2015-03-01

    Full Text Available 50 patients of COPD having moderate to severe COPD were analysed with clinical, chest x - ray, CT scan, serum c reactive protein and albumin levels and microbiological study by sputum microscopy for culture and sensitivity and gram stain. Mean age of the patients was 63±7.87 years. Out of 50 Patients 45 were men ( 90% and the remaining were women ( 10%. Bronchiectasis was present in 30 patients ( 60%. H. influenza was the commonest organism isolated from sputum. Patients with bronchiectasis had significantly more exacerbations ( p=0.0001, severe airway obstruction ( p=0.037, higher crp levels ( p=0.0001 and low album In levels ( p=0.007. Nine patients ( 30% died in bronchiectasis group and only one patient ( 3.33% died in patients without bronchiectasis. Our study showed an elevated prevalence of bronchiectasis in patients with moderate to severe COPD and was associated with severe airway

  14. Spatial and temporal variability of bronchiectasis cases in Silesian voivodeship in 2006–2010

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    Ewa Niewiadomska

    2016-08-01

    Full Text Available Objectives: Reports on an increasing number of hospitalizations in other European countries and the lack of epidemiological data on the prevalence of bronchiectasis in Poland constituted motivation for the authors to investigate temporal changes of the registered incidence and hospitalization due to bronchiectasis in Silesian voivodeship, and to evaluate spatial variability of the disease in the study region. Material and Methods: The study is a descriptive epidemiological project. Temporal and spatial variability of coefficients describing numbers of newly diagnosed cases and first time hospitalizations due to bronchiectasis (code J47 according to International Statistical Classification of Diseases and Related Health Problems, 10th revision (ICD-10 were evaluated based on the registered data available from the National Health Found (2006–2010 and the data from MZ/Szp-11 reports (2000–2011. The data concerned adults aged ≥ 19 years, inhabitants of Silesian voivodeship. Maps of incidence or hospitalization rates due to bronchiectasis were constructed by the use of a geographical information system ArcGIS. Results: The obtained results show a stable trend of reported new diseases, whereas the number of first time hospitalizations is increasing. Values of the standardized incidence were 19.9–25.1/100 000 inhabitants, and values of the standardized first-time hospitalization were 1.2–2.9/100 000 inhabitants. The reported rates of bronchiectasis indicate significant spatial differences in epidemiological situation in the study region. Conclusions: The findings showed territorial variability of the incidence and hospitalization of bronchiectasis recorded in Silesian voivodeship. The observed variability might result from regional differences in the availability of specialized medical services.

  15. Sleep disturbances and health-related quality of life in adults with steady-state bronchiectasis.

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    Yonghua Gao

    Full Text Available Sleep disturbances are common in patients with chronic lung diseases, but little is known about the prevalence in patients with bronchiectasis. A cross sectional study was conducted to investigate the prevalence and determinants associated with sleep disturbances, and the correlation between sleep disturbances and quality of life (QoL in adults with steady-state bronchiectasis.One hundred and forty-four bronchiectasis patients and eighty healthy subjects were enrolled. Sleep disturbances, daytime sleepiness, and QoL were measured by utilizing the Pittsburgh Sleep Quality Index (PSQI, Epworth Sleepiness Scale (ESS and St. George Respiratory Questionnaire (SGRQ, respectively. Demographic, clinical indices, radiology, spirometry, bacteriology, anxiety and depression were also assessed.Adults with steady-state bronchiectasis had a higher prevalence of sleep disturbances (PSQI>5 (57% vs. 29%, P<0.001, but not daytime sleepiness (ESS≥10 (32% vs. 30%, P = 0.76, compared with healthy subjects. In the multivariate model, determinants associated with sleep disturbances in bronchiectasis patients included depression (OR, 10.09; 95% CI, 3.46-29.37; P<0.001, nocturnal cough (OR, 1.89; 95% CI, 1.13-3.18; P = 0.016, aging (OR, 1.04; 95% CI, 1.01-1.07; P = 0.009 and increased 24-hour sputum volume (OR, 2.01; 95% CI, 1.22-3.33; P = 0.006. Patients with sleep disturbances had more significantly impaired QoL affecting all domains than those without. Only 6.2% of patients reported using a sleep medication at least weekly.In adults with steady-state bronchiectasis, sleep disturbances are more common than in healthy subjects and are related to poorer QoL. Determinants associated with sleep disturbances include depression, aging, nighttime cough and increased sputum volume. Assessment and intervention of sleep disturbances are warranted and may improve QoL.

  16. Eradication of Burkholderia cepacia Using Inhaled Aztreonam Lysine in Two Patients with Bronchiectasis

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    A. Iglesias

    2014-01-01

    Full Text Available There are not many articles about the chronic bronchial infection/colonization in patients with underlying lung disease other than cystic fibrosis (CF, especially with non-CF bronchiectasis (NCFBQ. The prevalence of B. cepacia complex is not well known in NCFBQ. The vast majority of published clinical data on Burkholderia infection in individuals with CF is comprised of uncontrolled, anecdotal, and/or single center experiences, and no consensus has emerged regarding treatment. We present two cases diagnosed with bronchiectasis (BQ of different etiology, with early pulmonary infection by B. cepacia complex, which was eradicated with inhaled aztreonam lysine.

  17. Raising awareness of bronchiectasis in primary care: overview of diagnosis and management strategies in adults.

    Science.gov (United States)

    Chalmers, James D; Sethi, Sanjay

    2017-12-01

    Bronchiectasis is a chronic lung disease characterised by recurrent infection, inflammation, persistent cough and sputum production. The disease is increasing in prevalence, requiring a greater awareness of the disease across primary and secondary care. Mild and moderate cases of bronchiectasis in adults can often be managed by primary care clinicians. Initial assessments and long-term treatment plans that include both pharmacological and non-pharmacological treatments, however, should be undertaken in collaboration with a secondary care team that includes physiotherapists and specialists in respiratory medicine. Bronchiectasis is often identified in patients with other lung diseases, such as chronic obstructive pulmonary disease, asthma, and in a lesser but not insignificant number of patients with other inflammatory diseases, such as rheumatoid arthritis and inflammatory bowel disease. Overall goals of therapy are to prevent exacerbations, improve symptoms, improve quality of life and preserve lung function. Prompt treatment of exacerbations with antibiotic therapy is important to limit the impact of exacerbations on quality of life and lung function decline. Patient education and cooperation with health-care providers to implement treatment plans are key to successful disease management. It is important for the primary care provider to work with secondary care providers to develop an individualised treatment plan to optimise care with the goal to delay disease progression. Here, we review the diagnosis and treatment of bronchiectasis with a focus on practical considerations that will be useful to primary care.

  18. Prevalence and risk factors of asymptomatic bronchiectasis in patients with rheumatoid arthritis at a tertiary care center in Saudi Arabia

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    Suzan Mansour Attar

    2015-01-01

    Full Text Available Introduction and Objectives: Bronchiectasis is a pulmonary manifestation that often occurs in individuals with rheumatoid arthritis (RA. Nevertheless, the prevalence of bronchiectasis in RA patients and predictors of its development/progression remain ill-defined. Our objective was to investigate the prevalence of bronchiectasis in a group of RA patients and examine possible clinical or biochemical risk factors that might contribute to its development. Methods: This was an observational study analyzing 100 RA patients with no pulmonary symptoms selected from King Abdulaziz University Hospital in the Western region of Saudi Arabia from October 2013 to 2014. Demographic, clinical and laboratory information were collected for all patients. Diagnosis was based on the 2010 American College of Rheumatology (ACR/European League Against Rheumatism (EULAR classification system, and disease activity was assessed using the 28-Joint Disease Activity Score Index with C-reactive protein; high-resolution computed tomography chest scans were performed. The prevalence of bronchiectasis was recorded and its association with different risk factors was examined using standard statistical methods. Results: All 100 patients fulfilled the ACR and EULAR classification criteria for RA diagnosis. Their mean age was 51.05 ± 13.5 years, disease duration was 6.19 ± 6.4 years and disease activity index was 4 ± 1.3 (moderate activity. A total of 35 (35% patients developed bronchiectasis. Notably, we observed significant positive associations of bronchiectasis with age, disease duration and male gender (P < 0.001, P = 0.006, P = 0.028, respectively. Conclusions: Asymptomatic bronchiectasis represents a common complication in moderately active RA patients within the Western Region of Saudi Arabia. Furthermore, several predictors of bronchiectasis development were identified, which can contribute to effective risk stratification in RA patients. Further prospective studies are

  19. Radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography

    Science.gov (United States)

    de Brito, Milene Carneiro Barbosa; Ota, Maurício Kenji; Leitão Filho, Fernando Sergio Studart; Meirelles, Gustavo de Souza Portes

    2017-01-01

    Objective To evaluate radiologist agreement on the quantification of bronchiectasis by high-resolution computed tomography (HRCT). Materials and Methods The HRCT scans of 43 patients with bronchiectasis were analyzed by two radiologists, who used a scoring system to grade the findings. Kappa (κ) values and overall agreement were calculated. Results For the measurement and appearance of bronchiectasis, the interobserver agreement was moderate (κ = 0.45 and κ = 0.43, respectively), as was the intraobserver agreement (κ = 0.54 and κ = 0.47, respectively). Agreement on the presence of mucous plugging was fair, for central distribution (overall interobserver agreement of 68.3% and κ = 0.39 for intraobserver agreement) and for peripheral distribution (κ = 0.34 and κ = 0.35 for interobserver and intraobserver agreement, respectively). The agreement was also fair for peribronchial thickening (κ = 0.21 and κ = 0.30 for interobserver and intraobserver agreement, respectively). There was fair interobserver and intraobserver agreement on the detection of opacities (κ = 0.39 and 71.9%, respectively), ground-glass attenuation (64.3% and κ = 0.24, respectively), and cysts/bullae (κ = 0.47 and κ = 0.44, respectively). Qualitative analysis of the HRCT findings of bronchiectasis and the resulting individual patient scores showed that there was an excellent correlation between the observers (intraclass correlation coefficient of 0.85 and 0.81 for interobserver and intraobserver agreement, respectively). Conclusion In the interpretation of HRCT findings of bronchiectasis, radiologist agreement appears to be fair. In our final analysis of the findings using the proposed score, we observed excellent interobserver and intraobserver agreement. PMID:28298729

  20. Impacts of Co-Existing Chronic Rhinosinusitis on Disease Severity and Risks of Exacerbations in Chinese Adults with Bronchiectasis.

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    Wei-jie Guan

    Full Text Available Mounting evidence supports the notion of "one airway, one disease."To determine whether chronic rhinosinusitis (CRS poses adverse impacts on Chinese adults with bronchiectasis.We enrolled 148 consecutive adults with clinically stable bronchiectasis. CRS diagnosed based on the 2012 EP3OS criteria. We systematically evaluated the bronchiectasis etiology, radiology, lung function, sputum bacteriology, airway inflammatory biomarkers, Bronchiectasis Severity Index, cough sensitivity and healthcare resource utilization. All patients were prospectively followed-up for 1 year to examine the frequency of bronchiectasis exacerbations (BEs.Forty-seven patients (31.8% were diagnosed as having CRS. Bronchiectasis etiologies did not vary statistically between CRS and no-CRS group. There was a trend towards non-statistically higher Bronchiectasis Severity Index [6.4±3.4 vs. 5.0(6.0, P = 0.19], a higher proportion of patients with BEs needing hospitalization before enrollment (48.9% vs. 29.7%, P = 0.13, poorer FVC [78.2±19.8% vs. 82.2(16.8%, P = 0.54] and FEV1 [68.2±24.8% vs. 74.8(21.2%, P = 0.29], a higher prevalence of Pseudomonas aeruginosa isolated (36.2% vs. 26.7%, P = 0.27 or colonized in sputum (36.2% vs. 21.8%, P = 0.12 and greater capsaicin cough sensitivity [C2: 3.9(123.0 μmol/L vs. 11.7(123.0 μmol/L, P = 0.81; C5: 62.5(996.0 μmol/L vs. 250.0(973.0 μmol/L, P = 0.32]. Patients with CRS had significantly greater risks of experiencing BEs during follow-up (P = 0.02 for negative binominal regression test.Chinese adults with bronchiectasis appear to have a lower prevalence of CRS than that in western countries. There was a trend towards greater adverse impacts on bronchiectasis in patients with CRS. Studies with greater sample sizes might help to resolve this issue. In future clinical practice, physicians should be vigilant to the screening of concomitant CRS in bronchiectasis so as to better improve patient's healthcare. Our findings may be of

  1. Adult non-cystic fibrosis bronchiectasis is characterised by airway luminal Th17 pathway activation.

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    Alice C-H Chen

    Full Text Available Non-cystic fibrosis (CF bronchiectasis is characterised by chronic airway infection and neutrophilic inflammation, which we hypothesised would be associated with Th17 pathway activation.Th17 pathway cytokines were quantified in bronchoalveolar lavage fluid (BALF, and gene expression of IL-17A, IL-1β, IL-8 and IL-23 determined from endobronchial biopsies (EBx in 41 stable bronchiectasis subjects and 20 healthy controls. Relationships between IL-17A levels and infection status, important clinical measures and subsequent Pseudomonas aeruginosa infection were determined.BALF levels of all Th17 cytokines (median (IQR pg/mL were significantly higher in bronchiectasis than control subjects, including IL-17A (1.73 (1.19, 3.23 vs. 0.27 (0.24, 0.35, 95% CI 1.05 to 2.21, p<0.0001 and IL-23 (9.48 (4.79, 15.75 vs. 0.70 (0.43, 1.79, 95% CI 4.68 to 11.21, p<0.0001. However, BALF IL-17A levels were not associated with clinical measures or airway microbiology, nor predictive of subsequent P. aeruginosa infection. Furthermore, gene expression of IL-17A in bronchiectasis EBx did not differ from control. In contrast, gene expression (relative to medians of controls in bronchiectasis EBx was significantly higher than control for IL1β (4.12 (1.24, 8.05 vs 1 (0.13, 2.95, 95% CI 0.05 to 4.07, p = 0.04 and IL-8 (3.75 (1.64, 11.27 vs 1 (0.54, 3.89, 95% CI 0.32 to 4.87, p = 0.02 and BALF IL-8 and IL-1α levels showed significant relationships with clinical measures and airway microbiology. P. aeruginosa infection was associated with increased levels of IL-8 while Haemophilus influenzae was associated with increased IL-1α.Established adult non-CF bronchiectasis is characterised by luminal Th17 pathway activation, however this pathway may be relatively less important than activation of non-antigen-specific innate neutrophilic immunity.

  2. Azithromycin for Indigenous children with bronchiectasis: study protocol for a multi-centre randomized controlled trial

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    Valery Patricia C

    2012-08-01

    Full Text Available Abstract Background The prevalence of chronic suppurative lung disease (CSLD and bronchiectasis unrelated to cystic fibrosis (CF among Indigenous children in Australia, New Zealand and Alaska is very high. Antibiotics are a major component of treatment and are used both on a short or long-term basis. One aim of long-term or maintenance antibiotics is to reduce the frequency of acute pulmonary exacerbations and symptoms. However, there are few studies investigating the efficacy of long-term antibiotic use for CSLD and non-CF bronchiectasis among children. This study tests the hypothesis that azithromycin administered once a week as maintenance antibiotic treatment will reduce the rate of pulmonary exacerbations in Indigenous children with bronchiectasis. Methods/design We are conducting a multicentre, randomised, double-blind, placebo controlled clinical trial in Australia and New Zealand. Inclusion criteria are: Aboriginal, Torres Strait Islander, Maori or Pacific Island children aged 1 to 8 years, diagnosed with bronchiectasis (or probable bronchiectasis with no underlying disease identified (such as CF or primary immunodeficiency, and having had at least one episode of pulmonary exacerbation in the last 12 months. After informed consent, children are randomised to receive either azithromycin (30 mg/kg once a week or placebo (once a week for 12–24 months from study entry. Primary outcomes are the rate of pulmonary exacerbations and time to pulmonary exacerbation determined by review of patient medical records. Secondary outcomes include length and severity of pulmonary exacerbation episodes, changes in growth, school loss, respiratory symptoms, forced expiratory volume in 1-second (FEV1; for children ≥6 years, and sputum characteristics. Safety endpoints include serious adverse events. Antibiotic resistance in respiratory bacterial pathogens colonising the nasopharynx is monitored. Data derived from medical records and clinical

  3. Left Circumflex Coronary Artery Fistula Connected to the Right Bronchial Artery Associated with Bronchiectasis: Multidetector CT and Coronary Angiography Findings

    Energy Technology Data Exchange (ETDEWEB)

    Nam, Kyung Jin; Choo, Ki Seok [Dept. of Radiology, Medical Research Institute, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan (Korea, Republic of)

    2013-04-15

    Coronary to bronchial artery fistula is a rare vascular anomaly secondary to enlargement of pre-existing vascular anastomosis between the coronary and bronchial arteries. This occurs when there is a constant disturbance of the pressure equilibrium involving either coronary or broncho-pulmonary disorder. Localized bronchiectasis is the most common related condition in patients with a coronary to bronchial artery fistula. Herein, we report on a case of a large left circumflex coronary artery to right bronchial artery fistula associated with bronchiectasis.

  4. High Prevalence of Stress Urinary Incontinence in Adult Patients with Bronchiectasis

    LENUS (Irish Health Repository)

    Duignan, N

    2016-07-01

    Stress urinary incontinence (SUI) is frequently under-reported in patients with chronic lung disease and may have negative psychosocial consequences. We conducted a prospective study to determine the prevalence, severity and treatment outcomes of SUI in female bronchiectasis patients referred for airway clearance techniques. Nineteen out of 40 (48%) patients reported SUI symptoms. Of these, 14 (74%) reported a reduced quality of life secondary to SUI. Following personalised intervention, symptom improvement was observed in 13\\/19 (68%). Five out of 19 (26%) required specialist referral for further continence care. No associations with lung disease severity and SUI were noted. SUI is common in adult female bronchiectasis patients and should be routinely screened for to improve patients’ overall quality of life.

  5. Automatic airway-artery analysis on lung CT to quantify airway wall thickening and bronchiectasis

    DEFF Research Database (Denmark)

    Perez-Rovira, Adria; Kuo, Wieying; Petersen, Jens;

    2016-01-01

    area percentage (WAP), wall thickness ratio (WTR), and airway diameters. Results: The method was thoroughly evaluated using 8000 manual annotations of airway-artery pairs from 24 full-inspiration pediatric CT scans (12 diseased and 12 controls). Limits of agreement between the automatically...... and manually measured diameters were comparable to interobserver limits of agreement. Differences in automatically obtained WAR, AAR, WAP, and WTR between bronchiectatic subjects and controls were similar as when manual annotations were used: WAR and outer AAR were significantly higher in the bronchiectatic......Purpose: Bronchiectasis and airway wall thickening are commonly assessed in computed tomography (CT) by comparing the airway size with the size of the accompanying artery. Thus, in order to automate the quantification of bronchiectasis and wall thickening following a similar principle...

  6. Culture and PCR detection of Haemophilus influenzae and Haemophilus haemolyticus in Australian Indigenous children with bronchiectasis.

    Science.gov (United States)

    Hare, K M; Binks, M J; Grimwood, K; Chang, A B; Leach, A J; Smith-Vaughan, H

    2012-07-01

    A PCR for protein D (hpd#3) was used to differentiate nontypeable Haemophilus influenzae (NTHI) from Haemophilus haemolyticus. While 90% of nasopharyngeal specimens and 100% of lower-airway specimens from 84 Indigenous Australian children with bronchiectasis had phenotypic NTHI isolates confirmed as H. influenzae, only 39% of oropharyngeal specimens with phenotypic NTHI had H. influenzae. The nasopharynx is therefore the preferred site for NTHI colonization studies, and NTHI is confirmed as an important lower-airway pathogen.

  7. A case of bronchiectasis needing lung isolation for cerebello pontine angle tumor excision: Anesthetic challenges

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    C Srinivasan

    2016-01-01

    Full Text Available The main goals of neuroanesthesia are the maintenance of adequate cerebral perfusion pressure, avoidance of hypercarbia, hypoxemia, and to provide better brain relaxation. Providing anesthesia for a patient with bronchiectasis needing lung isolation for craniotomy can be challenging. A 56-year-old male patient, case of right lung bronchiectasis with a right cerebello pontine angle tumor underwent excision in the left lateral position. Since he had severe bronchiectasis of the right lung, we had isolated the right lung using right-sided double lumen tube to avoid spillage. Intraoperative split lung test was performed to assess the right lung contribution on carbon dioxide (CO 2 elimination and found that there was a significant contribution from the right lung. Hence, both lungs were ventilated to control CO 2 . The importance of lung isolation to prevent spillage and avoidance of one lung ventilation to control the arterial CO 2 are highlighted in this case report. By providing a balanced anesthetic keeping both, the neurosurgical and thoracic concerns are important for better postoperative outcome.

  8. A decade of non-cystic fibrosis bronchiectasis 1996-2006.

    LENUS (Irish Health Repository)

    Zaid, A A

    2010-03-01

    This study aimed to determine the aetiology, clinical presentation, co-morbidity, severity and the lobar distribution of non cystic fibrosis bronchiectasis (NCFB). We performed a retrospective review of clinical, radiological, immunological and microbiological data from 92 non-CF patients with a High resolution thoracic CT (HRCT) diagnosis of bronchiectasis in the three Dublin Children\\'s referral Hospitals for the period 1996-2006. Of 92 patients (50 female), the median age at diagnosis was 6.4 years. The aetiology of bronchiectasis was as follows; idiopathic 29 (32%), post-pneumonia 16 (17%), immune deficiency 15 (16%), recurrent aspiration 15 (16%), primary ciliary dyskinesia 8 (9%), chronic aspiration with immune deficiency 5 (5%), post foreign body inhalation 2 (2%), tracheomalacia 1 (1%) and Obliterative bronchiolitis 1 (1%). Bronchial asthma and gastroesophageal reflux disease (GORD) were concurrently present in 18 (20%) and 10 (11%) respectively. Left lower lobe was commonly involved followed next by the right middle lobe. The common isolates were Haemophilus influenza (50), Streptococcus pneumoniae (34) and Staphylococcus aureus (14), Moraxella catarrhalis (9) and Pseudomonas auerginosa (8). Surgical interventions were performed in 23 (25%) of patients, lobectomy 11 (12%), pneumectomy 2 (2%), laryngeal cleft repair 4 (5%), rigid bronchoscopy for foreign body removal 2 (2%), Nissan\\'s fundoplication 2 (2%), tracheoesophageal fistula repair 2 (2%). We conclude NCFB is under-recognised in Irish children and diagnosis is often delayed and Bronchial Asthma and GORD are common co morbidity. A high index of suspicion and early HRCT can expedite the diagnosis.

  9. Primary care summary of the British Thoracic Society Guideline on the management of non-cystic fibrosis bronchiectasis.

    Science.gov (United States)

    Hill, Adam T; Pasteur, Mark; Cornford, Charles; Welham, Sally; Bilton, Diana

    2011-06-01

    The British Thoracic Society (BTS) has recently published a guideline for the management of non-cystic fibrosis (non-CF) bronchiectasis in children and adults. This paper summarises the key recommendations applicable to the primary care setting. The key points are: • Think of the diagnosis of bronchiectasis in adults and children who present with a chronic productive cough or unexplained haemoptysis, and in children with asthma which responds poorly to treatment; • High resolution computed tomography (HRCT) scanning is needed to confirm the diagnosis • Sputum culture should be obtained at the start of an exacerbation prior to initiating treatment with antibiotics; Treatment should be started whilst awaiting the sputum result and should be continued for 14 days; • Patients with bronchiectasis have significant morbidity. Management in primary care is aimed at improving morbidity, and includes; patient education, treatment and monitoring, as well as appropriate referral to secondary care including assessment for long term antibiotics.

  10. Analysis of CFTR Gene Variants in Idiopathic Bronchiectasis in Serbian Children

    Science.gov (United States)

    Milosevic, Katarina; Divac Rankov, Aleksandra; Ljujic, Mila; Nestorovic, Branimir; Radojkovic, Dragica

    2013-01-01

    This study has investigated a potential role of common Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene variants in the etiology of noncystic fibrosis bronchiectasis in Serbian children. The study has encompassed 48 patients (19 male and 29 female, aged between 5 and 18 years, median age 10.6±3.3), diagnosed with idiopathic bronchiectasis based on high-resolution computed tomography of thorax and pathologic examination of lobectomy materials. The CFTR gene analysis was performed on genomic DNA extracted from peripheral blood samples of patients by polymerase chain reaction (PCR)-Mediated Site-Directed Mutagenesis method, Denaturing Gradient Gel Electrophoresis method, and DNA sequencing. Mutation c.1521_1523delCTT (F508del) was detected with an allelic frequency of 1.0%, and c.224G>A (R75Q) variant. Carriers of c.1210-12T[5] (IVS8-5T) allele were significantly more common than in the general population (10.4% vs. 5.0%, P=0.0302). The frequency of homozygotes for Met 470 allele was higher in patients than in the general population (33% vs. 20%), while heterozygotes for p.Met470Val were less frequent (31% vs. 50%), and this difference was statistically significant (P=0.0222). The results obtained in this study indicate involvement of 2 common CFTR variants, c.1210-12T[5] and c.1408A, in idiopathic bronchiectasis in children, but this observation should be further confirmed by more extensive analysis of the CFTR gene in a larger group of patients. PMID:23781395

  11. Automated quantification of bronchiectasis, airway wall thickening and lumen tapering in chest CT

    DEFF Research Database (Denmark)

    Perez-Rovira, Adria; Kuo, Wieying; Petersen, Jens

    were obtained using a fully automatic, in-house developed, segmentation method. Subsequently, for each detected airway branch, the Airway-Artery Ratio (AAR, ratio between airway outer wall and accompanying artery radius, a bronchiectasis measurement), Wall-Artery Ratio (WAR, ratio between airway wall...... thickness and accompanying artery radius), and inter-branch Lumen-Ratio (LR, ratio between a branch's lumen and its parent branch lumen radius, a tapering measurement) were computed. Because CF-related structural abnormalities only affect a portion of branches, the 75th percentile was used as summarising...

  12. A 46-year-old man with tracheomegaly, tracheal diverticulosis, and bronchiectasis: Mounier-Kuhn syndrome

    Directory of Open Access Journals (Sweden)

    Ashish K Jaiswal

    2012-01-01

    Full Text Available Lower respiratory tract infection is one of the common causes of morbidity in India which is occasionally undiagnosed. In this regard tracheobronchomegaly is one of those conditions which masquerade as chronic bronchitis and bronchiectasis and are usually undiagnosed. It is a well-defined clinical and radiologic entity characterized by marked dilatation of the trachea and the central bronchi and is frequently associated with recurrent lower respiratory tract infection. Tracheobronchomegaly has been described by a variety of names, including Mounier-Kuhn syndrome, tracheal diverticulosis, tracheobronchiectasis, tracheocele, tracheomalacia, and tracheobronchopathia malacia.

  13. Pulmonary Opacities and Bronchiectasis Avid on 68Ga-PSMA PET

    DEFF Research Database (Denmark)

    Bouchelouche, Kirsten; Vendelbo, Mikkel Holm

    2017-01-01

    Prostate-specific membrane antigen (PSMA) is highly expressed in prostate cancer, and the expression increases with tumor aggressiveness, metastatic disease, and recurrence. Despite its name, PSMA is also expressed in neovasculature of other tumors including lung cancer. Here, we demonstrate a case...... of increased PSMA expression on Ga-PSMA PET/CT in benign lung opacities and bronchiectasis in a prostate cancer patient. Thus, increased PSMA activity in the lungs may be due to both benign and malignant diseases and warrants further evaluation....

  14. Neutrophilic Bronchial Inflammation Correlates with Clinical and Functional Findings in Patients with Noncystic Fibrosis Bronchiectasis

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    Federico L. Dente

    2015-01-01

    Full Text Available Background. Neutrophilic bronchial inflammation is a main feature of bronchiectasis, but not much is known about its relationship with other disease features. Aim. To compare airway inflammatory markers with clinical and functional findings in subjects with stable noncystic fibrosis bronchiectasis (NCFB. Methods. 152 NFCB patients (62.6 years; females: 57.2% underwent clinical and functional cross-sectional evaluation, including microbiologic and inflammatory cell profile in sputum, and exhaled breath condensate malondialdehyde (EBC-MDA. NFCB severity was assessed using BSI and FACED criteria. Results. Sputum neutrophil percentages inversely correlated with FEV1 (P<0.0001; rho = −0.428, weakly with Leicester Cough Questionnaire score (P=0.068; rho = −0.58, and directly with duration of the disease (P=0.004; rho = 0.3 and BSI severity score (P=0.005; rho = 0.37, but not with FACED. Sputum neutrophilia was higher in colonized subjects, P. aeruginosa colonized subjects showing greater sputum neutrophilia and lower FEV1. Patients with ≥3 exacerbations in the last year showed a significantly greater EBC-MDA than the remaining patients. Conclusions. Sputum neutrophilic inflammation and biomarkers of oxidative stress in EBC can be considered good biomarkers of disease severity in NCFB patients, as confirmed by pulmonary function, disease duration, bacterial colonization, BSI score, and exacerbation rate.

  15. Mucociliary clearance techniques for treating non-cystic fibrosis bronchiectasis: Is there evidence?

    Science.gov (United States)

    Snijders, D; Fernandez Dominguez, B; Calgaro, S; Bertozzi, I; Escribano Montaner, A; Perilongo, G; Barbato, A

    2015-06-01

    Non-cystic fibrosis bronchiectasis (nCFb) is an acquired condition of variable etiology. An impaired mucociliary clearance seems to be one of the mechanisms behind nCFb, and treatment involves antibiotics, mucoactive agents, and airway clearance techniques (ACTs). Traditional ACTs have four components: postural drainage, percussion, vibration of the chest wall, and coughing. Reviewing the international medical literature on the use of ACTs for patients with nCFb from 1989 to the present day, we retrieved 93 articles, of which 35 met our selection criteria for this analysis. We reviewed active cycle of breathing techniques (ACBT), forced expiration techniques (FET), autogenic drainage, postural drainage, oscillating positive expiratory pressure (OPep), high frequency chest wall oscillation (HFCWO), and exercise or pulmonary rehabilitation. Overall, ACTs appear to be safe for individuals (adults and children) with stable bronchiectasis; where there may be improvements in sputum expectoration, selected measures of lung function, and health-related quality of life. Unfortunately, there is a lack of RCTs in nCFb patients, especially in children. Moreover, none of the studies describes long-term effects of ACTs. It should be noted that a single intervention might not reflect the longer-term outcome and there is no evidence to recommend or contest any type of ACTs in nCFb management. Multicenter RCTs are necessary to evaluate the different techniques of ACTs especially in children with nCFb.

  16. Distribution of Major Pathogens from Sputum and Bronchoalveolar Lavage Fluid in Patients with Noncystic Fibrosis Bronchiectasis: A Systematic Review

    Institute of Scientific and Technical Information of China (English)

    Xia-Yi Miao; Xiao-Bin Ji; Hai-Wen Lu; Jia-Wei Yang; Jin-Fu Xu

    2015-01-01

    Objective:Noncystic fibrosis (non-CF) bronchiectasis remains as a common health problem in Asia.Pathogens' distribution in airways of patients with non-CF bronchiectasis is important for doctors to make right decision.Data Sources:We performed this systematic review on the English language literatures from 1966 to July 2014,using various search terms included "pathogens" or "bacteria" or "microbiology" and "bronchiectasis" or "non-cystic fibrosis bronchiectasis" or "non-CF bronchiectasis" or "NCFB."Study Selection:We included studies of patients with the confirmed non-CF bronchiectasis for which culture methods were required to sputum or bronchoalveolar lavage fluid (BALF).Weighted mean isolation rates for Haemophilus influenzae,Pseudomonas aeruginosa,Streptococcus pneumoniae,Stapylococcus aureus,Moxarella catarrhails were compared according to different methodology.Results:The total mean bacterial culture positive rates were 63%.For studies using sputum samples,the mean positive culture rates were 74%.For studies using BALF alone or BALF and sputum,it was 48%.The distributions of main bacterial strains were 29% for H.influenzae,28% for P.aeruginosa,1 1% for S.pneumoniae,12% for S.aureus,and 8% for M.catarrhails with methodology of sputum.Meanwhile,the bacterial distributions were 37% for H.influenzae,8% for P.aeruginosa,14% for S.pneumoniae,5% for S.aureus,and 10% for M.catarrhails with methodology of BALF alone or BALF and sputum.Analysis of the effect of different methodology on the isolation rates revealed some statistically significant differences.Conclusions:H.influenzae accounted for the highest percentage in different methodology.Our results suggested that the total positive culture rates and the proportion of P aeruginosa from sputum and BALF specimens had significant differences,which can be used in further appropriate recommendations for the treatment ofnon-CF bronchiectasis.

  17. Antibiotics for bronchiectasis exacerbations in children: rationale and study protocol for a randomised placebo-controlled trial

    Directory of Open Access Journals (Sweden)

    Chang Anne B

    2012-08-01

    Full Text Available Abstract Background Despite bronchiectasis being increasingly recognised as an important cause of chronic respiratory morbidity in both indigenous and non-indigenous settings globally, high quality evidence to inform management is scarce. It is assumed that antibiotics are efficacious for all bronchiectasis exacerbations, but not all practitioners agree. Inadequately treated exacerbations may risk lung function deterioration. Our study tests the hypothesis that both oral azithromycin and amoxicillin-clavulanic acid are superior to placebo at improving resolution rates of respiratory exacerbations by day 14 in children with bronchiectasis unrelated to cystic fibrosis. Methods We are conducting a bronchiectasis exacerbation study (BEST, which is a multicentre, randomised, double-blind, double-dummy, placebo-controlled, parallel group trial, in five centres (Brisbane, Perth, Darwin, Melbourne, Auckland. In the component of BEST presented here, 189 children fulfilling inclusion criteria are randomised (allocation-concealed to receive amoxicillin-clavulanic acid (22.5 mg/kg twice daily with placebo-azithromycin; azithromycin (5 mg/kg daily with placebo-amoxicillin-clavulanic acid; or placebo-azithromycin with placebo-amoxicillin-clavulanic acid for 14 days. Clinical data and a paediatric cough-specific quality of life score are obtained at baseline, at the start and resolution of exacerbations, and at day 14. In most children, blood and deep nasal swabs are also collected at the same time points. The primary outcome is the proportion of children whose exacerbations have resolved at day 14. The main secondary outcome is the paediatric cough-specific quality of life score. Other outcomes are time to next exacerbation; requirement for hospitalisation; duration of exacerbation; and spirometry data. Descriptive viral and bacteriological data from nasal samples and blood markers will also be reported. Discussion Effective, evidence-based management

  18. The association between combined non-cystic fibrosis bronchiectasis and lung cancer in patients with chronic obstructive lung disease

    Directory of Open Access Journals (Sweden)

    Kim YW

    2015-05-01

    Full Text Available Yeon Wook Kim,1 Kwang-Nam Jin,2 Eun Young Heo,3 Sung Soo Park,3 Hee Soon Chung,3 Deog Kyeom Kim31Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea; 2Department of Radiology, Seoul National University College of Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Republic of Korea; 3Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul, Republic of KoreaBackground: Whereas the epidemiological association between lung cancer and chronic obstructive pulmonary disease (COPD, a chronic inflammatory respiratory disease, is well known, limited studies have examined the association between lung cancer and non-cystic fibrosis bronchiectasis, a representative chronic airway inflammatory disease. This study evaluated the association between bronchiectasis and lung cancer in patients with COPD.Methods: A matched case–control study was conducted in a referral hospital in South Korea. Among COPD patients with moderate to very severe airflow limitation (forced expiratory volume in one second/forced vital capacity <0.7 and forced expiratory volume in one second ≤70% [% predicted] who underwent chest computed tomography (CT between January 1, 2010 and May 30, 2013, patients with lung cancer and controls matched for age, sex, and smoking history were selected. The risk of lung cancer was assessed according to the presence of underlying bronchiectasis confirmed by chest CT.Results: The study enrolled 99 cases and 198 controls. Combined bronchiectasis on chest CT was inversely associated with the risk of lung cancer compared with controls (odds ratio [OR] 0.25, 95% confidence interval [CI] 0.12–0.52, P<0.001. Significant associations were found in

  19. Association of body mass index with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis

    Energy Technology Data Exchange (ETDEWEB)

    Qi, Q.; Li, T. [Department of Respirology, Qilu Hospital of Shandong University, Jinan, Shandong Province (China); Li, J.C. [Neurosurgical Intensive Care Unit, the First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong Province (China); Li, Y. [Department of Respirology, Qilu Hospital of Shandong University, Jinan, Shandong Province (China)

    2015-07-10

    The objective of this observational, multicenter study was to evaluate the association of body mass index (BMI) with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis. A total of 339 patients (197 females, 142 males) diagnosed with non-cystic fibrosis bronchiectasis by high-resolution computed tomography were classified into four groups: underweight (BMI<18.5 kg/m{sup 2}), normal weight (18.5≤BMI<25.0 kg/m{sup 2}), overweight (25.0≤BMI<30.0 kg/m{sup 2}), and obese (BMI≥30.0 kg/m{sup 2}). Clinical variables expressing disease severity were recorded, and acute exacerbations, hospitalizations, and survival rates were estimated during the follow-up period. The mean BMI was 21.90 kg/m{sup 2}. The underweight group comprised 28.61% of all patients. BMI was negatively correlated with acute exacerbations, C-reactive protein, erythrocyte sedimentation rate, radiographic extent of bronchiectasis, and chronic colonization by P. aeruginosa and positively correlated with pulmonary function indices. BMI was a significant predictor of hospitalization risk independent of relevant covariates. The 1-, 2-, 3-, and 4-year cumulative survival rates were 94%, 86%, 81%, and 73%, respectively. Survival rates decreased with decreasing BMI (χ{sup 2}=35.16, P<0.001). The arterial carbon dioxide partial pressure, inspiratory capacity, age, BMI, and predicted percentage of forced expiratory volume in 1 s independently predicted survival in the Cox proportional hazard model. In conclusion, an underweight status was highly prevalent among patients with non-cystic fibrosis bronchiectasis. Patients with a lower BMI were prone to developing more acute exacerbations, worse pulmonary function, amplified systemic inflammation, and chronic colonization by P. aeruginosa. BMI was a major determinant of hospitalization and death risks. BMI should be considered in the routine assessment of patients with non-cystic fibrosis bronchiectasis.

  20. Association of body mass index with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis

    Directory of Open Access Journals (Sweden)

    Q. Qi

    2015-08-01

    Full Text Available The objective of this observational, multicenter study was to evaluate the association of body mass index (BMI with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis. A total of 339 patients (197 females, 142 males diagnosed with non-cystic fibrosis bronchiectasis by high-resolution computed tomography were classified into four groups: underweight (BMI<18.5 kg/m2, normal weight (18.5≤BMI<25.0 kg/m2, overweight (25.0≤BMI<30.0 kg/m2, and obese (BMI≥30.0 kg/m2. Clinical variables expressing disease severity were recorded, and acute exacerbations, hospitalizations, and survival rates were estimated during the follow-up period. The mean BMI was 21.90 kg/m2. The underweight group comprised 28.61% of all patients. BMI was negatively correlated with acute exacerbations, C-reactive protein, erythrocyte sedimentation rate, radiographic extent of bronchiectasis, and chronic colonization by P. aeruginosa and positively correlated with pulmonary function indices. BMI was a significant predictor of hospitalization risk independent of relevant covariates. The 1-, 2-, 3-, and 4-year cumulative survival rates were 94%, 86%, 81%, and 73%, respectively. Survival rates decreased with decreasing BMI (χ2=35.16, P<0.001. The arterial carbon dioxide partial pressure, inspiratory capacity, age, BMI, and predicted percentage of forced expiratory volume in 1 s independently predicted survival in the Cox proportional hazard model. In conclusion, an underweight status was highly prevalent among patients with non-cystic fibrosis bronchiectasis. Patients with a lower BMI were prone to developing more acute exacerbations, worse pulmonary function, amplified systemic inflammation, and chronic colonization by P. aeruginosa. BMI was a major determinant of hospitalization and death risks. BMI should be considered in the routine assessment of patients with non-cystic fibrosis bronchiectasis.

  1. Predicting high risk of exacerbations in bronchiectasis: the E-FACED score

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    Martinez-Garcia MA

    2017-01-01

    Full Text Available Martinez-Garcia MA,1,2 Athanazio RA,3 Girón R,4 Máiz-Carro L,5 de la Rosa D,6 Olveira C,7 de Gracia J,2,8 Vendrell M,9 Prados-Sánchez C,10 Gramblicka G,11 Corso Pereira M,12 Lundgren FL,13 Fernandes De Figueiredo M,14 Arancibia F,15 Rached SZ3 1Pulmonary Service, Polytechnic and University La Fe Hospital, Valencia, Spain; 2CIBERes, CIBER de Enfermedades Respiratorias. Madrid. Spain; 3Pulmonary Division, Heart Institute (Incor, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo; 4Pneumology Service, Hospital La Princesa, 5Pneumology Service, Hospital Ramón y Cajal, Madrid, 6Pneumology Unit, Hospital Plató, Barcelona, 7Pneumology, Málaga Regional University Hospital, Instituto de Biomedicina de Málaga (IBIMA, Málaga University, Spain; 8Pneumology Service, Hospital Vall d’Hebron, Barcelona, 9Bronchiectasis Group IDIBGI, Dr. Trueta University Hospital. UdG. Ciberes CB06/06/0030, 10Unidad de Fibrosis Quística y Bronquiectasias. Hospital Universitario La Paz. Madrid. Spain; 11Pneumology Service, Hospital del Tórax Dr A Cetrángolo, Buenos Aires, Argentina; 12Pneumology Service, Universidade Estadual de Campinas UNICAMP, Sao Paulo, 13Pneumology Service, Hospital Octávio de Freitas, Recife, 14Pneumology Service, Hospital de Messejana, Fortaleza, Brazil; 15Pneumology Service, Instituto Nacional del Tórax, Santiago de Chile, Chile Background: Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients.Objective: Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality.Methods: The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three

  2. Prevalence, age distribution and aetiology of bronchiectasis: a retrospective study on 144 symptomatic patients.

    Science.gov (United States)

    Scala, R; Aronne, D; Palumbo, U; Montella, L; Giacobbe, R; Martucci, P; Del Prato, B

    2000-04-01

    The incidence of bronchiectasis (BCT) has probably decreased in developed countries in recent years, but reliable statistical data on its occurrence are still lacking. The aim of the present study was to retrospectively evaluate the prevalence, age distribution and aetiology of BCT, diagnosed in a selected series of symptomatic patients of a Western country by using bronchography. The authors analysed the main known predisposing and associated conditions (PACs), and the occurrence and age distribution of BCT in 144 consecutive patients who underwent bronchological examination (fibreoptic bronchoscopy and bronchography) in the years 1987-1994 because of recurrent purulent bronchitis and/or haemoptysis. The overall prevalence of BCT was 34% (49/144); its age distribution was: 17.2% (0-10 yrs), 43.7% (11-20 yrs), 38% (21-30 yrs), 37.5% (31-40 yrs), 33.3% (41-50 yrs), 40% (51-60 yrs), and 20% (61-70 yrs). Thirty-one PACs were found in 29/144 patients of the whole study group. The prevalence of BCT was significantly higher in the subgroup of 29 patients with PACs than in the subgroup of 115 patients without PACs (75.9% versus 23.5%; p dermatomiositis (one), and toxin inhalation (one). The authors conclude that bronchiectasis still occurs in a large percentage of symptomatic patients of a developed country in the post-antimicrobial era, especially in the second to sixth decades, as well as in the presence of predisposing and associated conditions; its aetiology remains unknown in more than half of cases.

  3. Effects of long-term bronchodilators in bronchiectasis patients with airflow limitation based on bronchodilator response at baseline

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    Jeong HJ

    2016-11-01

    Full Text Available Ho Jung Jeong,1,* Hyun Lee,1,* Keumhee C Carriere,2,3 Jung Hoon Kim,1 Jin-Hyung Han,1 Beomsu Shin,1 Byeong-Ho Jeong,1 Won-Jung Koh,1 O Jung Kwon,1 Hye Yun Park1 1Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University of Medicine, Seoul, South Korea; 2Department of Mathematical and Statistical Sciences, University of Alberta, Edmonton, AB, Canada; 3Biostatistics and Clinical Epidemiology Center, Samsung Medical Center, Seoul, South Korea *These authors contributed equally to this work Purpose: The association between positive bronchodilator response (BDR at baseline and the effect of long-term bronchodilator therapy has not been well elucidated in patients with bronchiectasis. The aims of our study were to explore the association between positive BDR at baseline and lung-function improvement following long-term (3–12 months bronchodilator therapy in bronchiectasis patients with airflow limitation. Materials and methods: The medical records of 166 patients with clinically stable bronchiectasis who underwent baseline pre- and postbronchodilator spirometry and repeated spirometry after 3–12 months of bronchodilator therapy were retrospectively reviewed. For analysis, patients were divided into two groups, responders and poor responders, based on achievement of at least 12% and 200 mL in forced expiratory volume in 1 second (FEV1 following bronchodilator therapy from baseline FEV1. Results: A total of 57 patients (34.3% were responders. These patients were more likely to have positive BDR at baseline than poor responders (38.6% [22 of 57] vs 18.3% [20 of 109], P=0.004. This association persisted after adjustment for other confounding factors (adjusted odds ratio 2.298, P=0.034. However, we found FEV1 improved significantly following long-term bronchodilator therapy, even in patients without positive BDR at baseline (change in FEV1 130 mL, interquartile range -10 to 250 mL; P<0

  4. Focal Bronchiectasis Causing Abnormal Pulmonary Radioiodine Uptake in a Patient with Well-Differentiated Papillary Thyroid Carcinoma

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    Ash Gargya

    2012-01-01

    Full Text Available Background. False-positive pulmonary radioactive iodine uptake in the followup of differentiated thyroid carcinoma has been reported in patients with certain respiratory conditions. Patient Findings. We describe a case of well-differentiated papillary thyroid carcinoma treated by total thyroidectomy and radioiodine ablation therapy. Postablation radioiodine whole body scan and subsequent diagnostic radioiodine whole body scans have shown persistent uptake in the left hemithorax despite an undetectable stimulated serum thyroglobulin in the absence of interfering thyroglobulin antibodies. Contrast-enhanced chest computed tomography has confirmed that the abnormal pulmonary radioiodine uptake correlates with focal bronchiectasis. Summary. Bronchiectasis can cause abnormal chest radioactive iodine uptake in the followup of differentiated thyroid carcinoma. Conclusions. Recognition of potential false-positive chest radioactive iodine uptake, simulating pulmonary metastases, is needed to avoid unnecessary exposure to further radiation from repeated therapeutic doses of radioactive iodine.

  5. The Likelihood of Preventing Respiratory Exacerbations in Children and Adolescents with either Chronic Suppurative Lung Disease or Bronchiectasis

    Science.gov (United States)

    O’Grady, Kerry-Ann F; Grimwood, Keith

    2017-01-01

    Chronic suppurative lung disease (CSLD) and bronchiectasis in children and adolescents are important causes of respiratory morbidity and reduced quality of life (QoL), also leading to subsequent premature death during adulthood. Acute respiratory exacerbations in pediatric CSLD and bronchiectasis are important markers of disease control clinically, given that they impact upon QoL and increase health-care-associated costs and can adversely affect future lung functioning. Preventing exacerbations in this population is, therefore, likely to have significant individual, familial, societal, and health-sector benefits. In this review, we focus on therapeutic interventions, such as drugs (antibiotics, mucolytics, hyperosmolar agents, bronchodilators, corticosteroids, non-steroidal anti-inflammatory agents), vaccines and physiotherapy, and care-planning, such as post-hospitalization management and health promotion strategies, including exercise, diet, and reducing exposure to environmental toxicants. The review identified a conspicuous lack of moderate or high-quality evidence for preventing respiratory exacerbations in children and adolescents with CSLD or bronchiectasis. Given the short- and long-term impact of exacerbations upon individuals, their families, and society as a whole, large studies addressing interventions at the primary and tertiary prevention phases are required. This research must include children and adolescents in both developing and developed countries and address long-term health outcomes. PMID:28393062

  6. Predicting high risk of exacerbations in bronchiectasis: the E-FACED score

    Science.gov (United States)

    Martinez-Garcia, MA; Athanazio, RA; Girón, R; Máiz-Carro, L; de la Rosa, D; Olveira, C; de Gracia, J; Vendrell, M; Prados-Sánchez, C; Gramblicka, G; Corso Pereira, M; Lundgren, FL; Fernandes De Figueiredo, M; Arancibia, F; Rached, SZ

    2017-01-01

    Background Although the FACED score has demonstrated a great prognostic capacity in bronchiectasis, it does not include the number or severity of exacerbations as a separate variable, which is important in the natural history of these patients. Objective Construction and external validation of a new index, the E-FACED, to evaluate the predictive capacity of exacerbations and mortality. Methods The new score was constructed on the basis of the complete cohort for the construction of the original FACED score, while the external validation was undertaken with six cohorts from three countries (Brazil, Argentina, and Chile). The main outcome was the number of annual exacerbations/hospitalizations, with all-cause and respiratory-related deaths as the secondary outcomes. A statistical evaluation comprised the relative weight and ideal cut-off point for the number or severity of the exacerbations and was incorporated into the FACED score (E-FACED). The results obtained after the application of FACED and E-FACED were compared in both the cohorts. Results A total of 1,470 patients with bronchiectasis (819 from the construction cohorts and 651 from the external validation cohorts) were followed up for 5 years after diagnosis. The best cut-off point was at least two exacerbations in the previous year (two additional points), meaning that the E-FACED has nine points of growing severity. E-FACED presented an excellent prognostic capacity for exacerbations (areas under the receiver operating characteristic curve: 0.82 for at least two exacerbations in 1 year and 0.87 for at least one hospitalization in 1 year) that was statistically better than that of the FACED score (0.72 and 0.78, P<0.05, respectively). The predictive capacities for all-cause and respiratory mortality were 0.87 and 0.86, respectively, with both being similar to those of the FACED. Conclusion E-FACED score significantly increases the FACED capacity to predict future yearly exacerbations while maintaining the

  7. 102 Bronchiectasis: Localization and Characteristics, Identified by Using a High Resolution CT Scan in Adults With Common Variable Immunodeficiency

    Science.gov (United States)

    Mendoza, Laura; Segura Mendez, Nora Hilda; Flores, Francisco; Campos Romero, Freya Helena; Guillén, Nelva

    2012-01-01

    Background The common variable Immunodeficiency (CVID) is the second cause of primary immunodeficiency. The bronchiectasis are the most frequent structural pulmonary alterations in CVID, which have been principally described in pediatric population, finding the presence of the same ones in 50% of the cases, nevertheless exists inssuficient information about the location and characteristics as type and distribution of the bronchiectasis; in adult population, less information exists still on this matter. High resolution CT scan is valuable for detection of bronchiectasis and may alter treatment of these patients. Objective The purpose of this study was to determine the presence of bronchiectasis, their characteristics and most frequent location using a high resolution CT scan in adults with diagnosis of CVID. Methods This was a cohort study whit 15 adult subjects whit the diagnosis of CVID, who underwent a chest high-resolution computed tomography scan, previous signature of a letter of informed consent and with the approval of the committee of ethics and investigation (F-2011-3601-21). Results We studied all the subjects (n = 15) whit CVID, finding the presence of bronquiectasias in 73% of the subjects with CVID, 82% was a women and 8% males. The most frequent location was in the left lung in 46% of the cases and 45 bilateral %, with only 9% of location in right lung. These were more frequent in the lower lobe in 42, 17% in top lobe and l6% diffuse, the rest of them were brought like diffuse, bibasal or parahiliar. In one patient we found the presence of a left apical cavitation and only one was brought by presence of pulmonary diffuse fibrosis. Conclusions There was realized a search of bronchiectasis and their characteristics in subjects with CVID disorders, the incidence of bronchiectasis is higher in our poblation (82%) than in the rest (50% described in other pubiclations). The most affected lung was the left in the lower lobe. The most frequent type of

  8. Pulmonary Nocardiosis Induced by Long-Term Use of Steroids on a Bronchiectasis Backgroung

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    Levent Özdemir

    2015-03-01

    Full Text Available A 45-year-old male was assessed with the complaints of cough, sputum, fever, shortness of breath, tingling and burning in both legs. In his history, he had frequent hospitalizations and steroid use. Respiratory system examination revealed inspiratory and expiratory crackles on bilateral lower-middle zones and expiration rhoncus. Laboratory examination revealed leukocytosis and elevated CRP levels; serology for HIV was reported negative. Computed tomography showed diffuse interstitial pneumonia. Electromyography (EMG showed lower extremity muscle activation compatible with myopathy. Acid-fast bacilli was negative (three consecutive negative microscopy results and no reproduction was detected in the culture. Nocardia spp. growth was detected in sputum culture. According to the antibiogram results, he was treated with doxycycline, trimethoprim and sulfamethoxazole for 6 months. At the end of treatment, the lesions were found to be regressed on computed tomography. In conclusion, considering the high frequency of bronchiectasis in our country, the fact that nocardia may develop due to inappropriate long-term steroid use during an attack should be kept in mind.

  9. The reliability of lung crackle characteristics in cystic fibrosis and bronchiectasis patients in a clinical setting.

    Science.gov (United States)

    Marques, Alda; Bruton, Anne; Barney, Anna

    2009-09-01

    Lung sounds provide useful information for assessing and monitoring respiratory patients, but standard auscultation is subjective. Computer aided lung sound analysis (CALSA) enables the quantification and characterisation of added lung sounds (e.g. crackles). At present, little is known about the reliability of these sound characteristics. Therefore, the aim of this study was to explore the reliability of crackle initial deflection width (IDW) and two-cycle deflection (2CD) in a clinical population. Fifty-four subjects (37 bronchiectasis, 17 cystic fibrosis) were recruited from out-patient clinics. Three repeated lung sound recordings were taken at seven anatomical sites with a digital stethoscope connected to a laptop computer. The intra-subject reliability of crackle IDW and 2CD was found to be 'good' to 'excellent', estimated by the analysis of variance, intraclass correlation coefficient (IDW 0.76;0.85, 2CD 0.83;0.94), Bland and Altman 95% limits of agreement (IDW -0.50;0.47 ms, 2CD -2.12;1.87 ms) and smallest real difference (IDW 0.30;0.66 ms, 2CD 1.57;2.42 ms). Crackle 2CD was found to be more reliable than IDW. It is concluded that crackle IDW and 2CD characterized by CALSA have good test-retest reliability. This technique requires further evaluation since CALSA has potential to diagnose or monitor respiratory conditions, and provide an objective physiological measure for respiratory interventions.

  10. Nasopharyngeal carriage and macrolide resistance in Indigenous children with bronchiectasis randomized to long-term azithromycin or placebo.

    Science.gov (United States)

    Hare, K M; Grimwood, K; Chang, A B; Chatfield, M D; Valery, P C; Leach, A J; Smith-Vaughan, H C; Morris, P S; Byrnes, C A; Torzillo, P J; Cheng, A C

    2015-11-01

    Although long-term azithromycin decreases exacerbation frequency in bronchiectasis, increased macrolide resistance is concerning. We investigated macrolide resistance determinants in a secondary analysis of a multicenter randomized controlled trial. Indigenous Australian children living in remote regions and urban New Zealand Māori and Pacific Islander children with bronchiectasis were randomized to weekly azithromycin (30 mg/kg) or placebo for up to 24 months and followed post-intervention for up to 12 months. Nurses administered and recorded medications given and collected nasopharyngeal swabs 3-6 monthly for culture and antimicrobial susceptibility testing. Nasopharyngeal carriage of Haemophilus influenzae and Moraxella catarrhalis was significantly lower in azithromycin compared to placebo groups, while macrolide-resistant Streptococcus pneumoniae and Staphylococcus aureus carriage was significantly higher. Australian children, compared to New Zealand children, had higher carriage overall, significantly higher carriage of macrolide-resistant bacteria at baseline (16/38 versus 2/40 children) and during the intervention (69/152 versus 22/239 swabs), and lower mean adherence to study medication (63 % versus 92 %). Adherence ≥70 % (versus resistant pathogens (OR 0.34, 95 % CI 0.14-0.81). Post-intervention (median 6 months), macrolide resistance in S. pneumoniae declined significantly in the azithromycin group, from 79 % (11/14) to 7 % (1/14) of positive swabs, but S. aureus strains remained 100 % macrolide resistant. Azithromycin treatment, the Australian remote setting, and adherence resistance in children with bronchiectasis. Adherence to treatment may limit macrolide resistance by suppressing carriage.

  11. Longitudinal nasopharyngeal carriage and antibiotic resistance of respiratory bacteria in indigenous Australian and Alaska native children with bronchiectasis.

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    Kim M Hare

    Full Text Available BACKGROUND: Indigenous children in Australia and Alaska have very high rates of chronic suppurative lung disease (CSLD/bronchiectasis. Antibiotics, including frequent or long-term azithromycin in Australia and short-term beta-lactam therapy in both countries, are often prescribed to treat these patients. In the Bronchiectasis Observational Study we examined over several years the nasopharyngeal carriage and antibiotic resistance of respiratory bacteria in these two PCV7-vaccinated populations. METHODS: Indigenous children aged 0.5-8.9 years with CSLD/bronchiectasis from remote Australia (n = 79 and Alaska (n = 41 were enrolled in a prospective cohort study during 2004-8. At scheduled study visits until 2010 antibiotic use in the preceding 2-weeks was recorded and nasopharyngeal swabs collected for culture and antimicrobial susceptibility testing. Analysis of respiratory bacterial carriage and antibiotic resistance was by baseline and final swabs, and total swabs by year. RESULTS: Streptococcus pneumoniae carriage changed little over time. In contrast, carriage of Haemophilus influenzae declined and Staphylococcus aureus increased (from 0% in 2005-6 to 23% in 2010 in Alaskan children; these changes were associated with increasing age. Moraxella catarrhalis carriage declined significantly in Australian, but not Alaskan, children (from 64% in 2004-6 to 11% in 2010. While beta-lactam antibiotic use was similar in the two cohorts, Australian children received more azithromycin. Macrolide resistance was significantly higher in Australian compared to Alaskan children, while H. influenzae beta-lactam resistance was higher in Alaskan children. Azithromycin use coincided significantly with reduced carriage of S. pneumoniae, H. influenzae and M. catarrhalis, but increased carriage of S. aureus and macrolide-resistant strains of S. pneumoniae and S. aureus (proportion of carriers and all swabs, in a 'cumulative dose-response' relationship

  12. Airway malacia in chronic obstructive pulmonary disease: prevalence, morphology and relationship with emphysema, bronchiectasis and bronchial wall thickening

    Energy Technology Data Exchange (ETDEWEB)

    Sverzellati, Nicola; Rastelli, Andrea; Schembri, Valentina; Filippo, Massimo de [University of Parma, Department of Clinical Sciences, Section of Radiology, Parma (Italy); Chetta, Alfredo [University of Parma, Department of Clinical Sciences, Section of Respiratory Diseases, Parma (Italy); Fasano, Luca; Pacilli, Angela Maria [Policlinico Sant' Orsola-Malpighi, Unita Operativa di Fisiopatologia Respiratoria, Bologna (Italy); Di Scioscio, Valerio; Bartalena, Tommaso; Zompatori, Maurizio [University of Bologna, Department of Radiology, Cardiothoracic Institute, Policlinico S.Orsola-Malpighi, Bologna (Italy)

    2009-07-15

    The aim of this study was to determine the prevalence of airway malacia and its relationship with ancillary morphologic features in patients with chronic obstructive pulmonary disease (COPD). A retrospective review was performed of a consecutive series of patients with COPD who were imaged with inspiratory and dynamic expiratory multidetector computed tomography (MDCT). Airway malacia was defined as {>=}50% expiratory reduction of the airway lumen. Both distribution and morphology of airway malacia were assessed. The extent of emphysema, extent of bronchiectasis and severity of bronchial wall thickness were quantified. The final study cohort was comprised of 71 patients. Airway malacia was seen in 38 of 71 patients (53%), and such proportion was roughly maintained in each stage of COPD severity. Almost all tracheomalacia cases (23/25, 92%) were characterised by an expiratory anterior bowing of the posterior membranous wall. Both emphysema and bronchiectasis extent did not differ between patients with and without airway malacia (p > 0.05). Bronchial wall thickness severity was significantly higher in patients with airway malacia and correlated with the degree of maximal bronchial collapse (p < 0.05). In conclusion, we demonstrated a strong association between airway malacia and COPD, disclosing a significant relationship with bronchial wall thickening. (orig.)

  13. Poor survival in rheumatoid arthritis associated with bronchiectasis: a family-based cohort study.

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    Xavier Puéchal

    Full Text Available BACKGROUND: Diffuse bronchiectasis (DB may occur in rheumatoid arthritis (RA. CFTR (cystic fibrosis transmembrane conductance regulator mutations predispose RA patients to DB, but the prognosis of RA-associated DB (RA-DB is unclear. METHODS: We report long-term mortality data from a nationwide family-based association study of patients with RA only, DB only or RA-DB. We assessed mortality as a function of clinical characteristics and CF/CFTR-RD (CFTR-related disorders mutations in 137 subjects from 24 kindreds. Potential risk factors were investigated by Cox proportional-hazard analysis with shared Gaussian random effects to account for within-family correlations. RESULTS: During a median follow-up of 11 years after inclusion, 18 patients died, mostly from cardiorespiratory causes. Survival was significantly lower for RA-DB patients than for unaffected relatives and for patients with RA or DB only. RA patients with DB had also a poorer prognosis in terms of survival after RA diagnosis (HR, 8.6; 95% CI, 1.5-48.2; P = 0.014 and from birth (HR, 9.6; 95% CI, 1.1-81.7; P = 0.039. Early onset of DB (HR, 15.4; 95% CI, 2.1-113.2; P = 0.007 and CF/CFTR-RD mutation (HR, 7.2; 95% CI, 1.4-37.1; P = 0.018 were associated with poorer survival in patients with RA-DB. Thus, CF/CFTR-RD mutations in RA patients with early-onset DB defined a subgroup of high-risk patients with higher mortality rates (log-rank test P = 1.28×10(-5. CONCLUSION: DB is associated with poorer survival in patients with RA. Early-onset DB and CFTR mutations are two markers that identify RA patients at a high risk of death, for whom future therapeutic interventions should be designed and evaluated.

  14. An Analysis of Nursing Treatment of Patients with Bronchiectasis%支气管扩张患者的护理分析

    Institute of Scientific and Technical Information of China (English)

    姜庆珠

    2014-01-01

    Objective The clinical nursing treatment of patients with bronchiectasis to be investigated. Methods Analyzing the clinical data selected from 85 cases of patients with bronchiectasis. Results With being treated and cared physically and psychologically, 60 patients with bronchiectasis in hospital have made an improvement in the health state. Conclusion Bronchiectasis is one of the most common respiratory diseases prevalent among the teenagers. The patients have better be given specified nursing measures based on their own health state when get treated in order to recover and go into rehab soon.%目的探讨支气管扩张患者的临床护理。方法总结分析85例支气管扩张患者的临床护理资料。结果通过对支气管扩张患者的生活护理、心理护理,收治入院的60例支气管扩张患者均有好转。结论支气管扩张一种多发于青少年呼吸系统的常见疾病。  患者在治疗的同时要根据个体情况采取针对性的护理,以促进患者早日康复。

  15. 儿童支气管扩张的病因及早期诊治策略%Etiology of childhood bronchiectasis: strategy of early diagnosis and management

    Institute of Scientific and Technical Information of China (English)

    李昌崇; 苏苗赏

    2011-01-01

    @@ 囊性纤维化(Cystic fibrosis,CF)是一种常染色体隐性遗传性疾病,它是引起白种人儿童支气管扩张(bronchiectasis)的主要原因.而非囊性纤维化支气管扩张(non-cystic fibrosis bronchiectasis)常见于发展中国家,以前认为它主要见于慢性化脓性炎症,表现为支气管不可逆的扩张伴有管壁增厚及破坏.随着高分辨率CT(HRCT)技术的发展,结合近年来大量的研究结果显示,非囊性纤维化支气管扩张在发达国家的诊断率也不断增多.

  16. Identification of a novel SERPINA-1 mutation causing alpha-1 antitrypsin deficiency in a patient with severe bronchiectasis and pulmonary embolism

    Directory of Open Access Journals (Sweden)

    Milger K

    2015-05-01

    Full Text Available Katrin Milger,1 Lesca Miriam Holdt,2 Daniel Teupser,2 Rudolf Maria Huber,1 Jürgen Behr,1 Nikolaus Kneidinger1 1Department of Internal Medicine V, University of Munich, Comprehensive Pneumology Center, Member of the German Center for Lung Research, 2Institute of Laboratory Medicine, University of Munich, Munich, Germany Abstract: Deficiency in the serine protease inhibitor, alpha-1 antitrypsin (AAT, is known to cause emphysema and liver disease. Other manifestations, including airway disease or skin disorders, have also been described. A 44-year-old woman presented to our emergency department with dyspnea and respiratory insufficiency. She had never smoked, and had been diagnosed with COPD 9 years earlier. Three months previously, she had suffered a pulmonary embolism. Chest computed tomography scan revealed severe cystic bronchiectasis with destruction of the lung parenchyma. The sweat test was normal and there was no evidence of the cystic fibrosis transmembrane conductance regulator (CFTR mutation. Capillary zone electrophoresis showed a decrease of alpha-1 globin band and AAT levels were below the quantification limit (<25 mg/dL. No S or Z mutation was identified, but sequencing analysis found a homozygous cytosine and adenine (CA insertion in exon 2 of the SERPINA-1 gene, probably leading to a dysfunctional protein (PI Null/Null. This mutation has not been previously identified. The atypical presentation of the patient, with severe cystic bronchiectasis, highlights AAT deficiency as a differential diagnosis in bronchiectasis. Further, awareness should be raised regarding a possible increased risk of thromboembolism associated with AAT deficiency. Keywords: alpha-1 antitrypsin deficiency, bronchiectasis, SERPINA-1 mutation, pulmonary embolism

  17. Deve-se extrapolar o tratamento de bronquiectasias em pacientes com fibrose cística para aqueles com bronquiectasias de outras etiologias? Should the bronchiectasis treatment given to cystic fibrosis patients be extrapolated to those with bronchiectasis from other causes?

    Directory of Open Access Journals (Sweden)

    Rodrigo Abensur Athanazio

    2010-08-01

    Full Text Available OBJETIVO: Conhecer o perfil de pacientes adultos com bronquiectasias, comparando portadores de fibrose cística (FC com aqueles com bronquiectasias de outra etiologia, a fim de determinar se é racional extrapolar terapêuticas instituídas em fibrocísticos para aqueles com bronquiectasias de outras etiologias. MÉTODOS: Análise retrospectiva dos prontuários de 87 pacientes adultos com diagnóstico de bronquiectasia em acompanhamento em nosso serviço. Pacientes com doença secundária a infecção por tuberculose corrente ou no passado foram excluídos. Foram avaliados dados clínicos, funcionais e terapêuticos dos pacientes. RESULTADOS: Dos 87 pacientes com bronquiectasias, 38 (43,7% tinham diagnóstico confirmado de FC através de dosagem de sódio e cloro no suor ou análise genética, enquanto 49 (56,3% apresentavam a doença por outra etiologia, 34 (39,0% desses com bronquiectasia idiopática. Os pacientes com FC apresentavam média de idade ao diagnóstico mais baixa (14,2 vs. 24,2 anos; p OBJECTIVE: To profile the characteristics of adult patients with bronchiectasis, drawing comparisons between cystic fibrosis (CF patients and those with bronchiectasis from other causes in order to determine whether it is rational to extrapolate the bronchiectasis treatment given to CF patients to those with bronchiectasis from other causes. METHODS: A retrospective analysis of the medical charts of 87 patients diagnosed with bronchiectasis and under follow-up treatment at our outpatient clinic. Patients who had tuberculosis (current or previous were excluded. We evaluated the clinical, functional, and treatment data of the patients. RESULTS: Of the 87 patients with bronchiectasis, 38 (43.7% had been diagnosed with CF, through determination of sweat sodium and chloride concentrations or through genetic analysis, whereas the disease was due to another etiology in 49 (56.3%, of whom 34 (39.0% had been diagnosed with idiopathic bronchiectasis. The mean

  18. Effect of Low-Dose, Long-Term Roxithromycin on Airway Inflammation and Remodeling of Stable Noncystic Fibrosis Bronchiectasis

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    Jifeng Liu

    2014-01-01

    Full Text Available Background. Noncystic fibrosis bronchiectasis (NCFB is characterized by airway expansion and recurrent acute exacerbations. Macrolide has been shown to exhibit anti-inflammatory effects in some chronic airway diseases. Objective. To assess the efficacy of roxithromycin on airway inflammation and remodeling in patients with NCFB under steady state. Methods. The study involved an open-label design in 52 eligible Chinese patients with NCFB, who were assigned to control (receiving no treatment and roxithromycin (receiving 150 mg/day for 6 months groups. At baseline and 6 months, the inflammatory markers such as interleukin- (IL-8, neutrophil elastase (NE, matrix metalloproteinase- (MMP9, hyaluronidase (HA, and type IV collagen in sputum were measured, along with the detection of dilated bronchus by throat computed tomography scan, and assessed the exacerbation. Results. Forty-three patients completed the study. The neutrophil in the sputum was decreased in roxithromycin group compared with control (P<0.05. IL-8, NE, MMP-9, HA, and type IV collagen in sputum were also decreased in roxithromycin group compared with the control group (all P<0.01. Airway thickness of dilated bronchus and exacerbation were reduced in roxithromycin group compared with the control (all P<0.05. Conclusions. Roxithromycin can reduce airway inflammation and airway thickness of dilated bronchus in patients with NCFB.

  19. A Metagenomic approach to characterize temperate bacteriophage populations from cystic fibrosis and non-cystic fibrosis bronchiectasis patients

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    Mohammad Adnan Tariq

    2015-02-01

    Full Text Available Pseudomonas aeruginosa (Pa, normally a soil commensal, is an important opportunistic pathogen in Cystic Fibrosis (CF and non-Cystic Fibrosis Bronchiectasis (nCFBR. Persistent infection correlates with accelerated decline in lung function and early mortality. The horizontal transfer of DNA by temperate bacteriophages can add gene function and selective advantages to their bacterial host within the constrained environment of the lower lung. In this study, we chemically induce temperate bacteriophages from clonal cultures of Pa and identify their mixed viral communities employing metagenomic approaches. We compared 92 temperate phage metagenomes stratified from these clinical backgrounds (47 CF and 45 nCFBR Pa isolates using MG-RAST and GeneWise2. KEGG analysis shows the complexity of temperate phage accessory gene carriage increases with duration and severity of the disease. Furthermore we identify the presence of Ig-like motifs within phage structural genes linked to bacterial adhesion and carbohydrate binding including Big_2, He_Pig and Fn3. This study provides the first clinical support to the proposed bacteriophage adherence to mucus (BAM model and the evolution of phages interacting at these mucosal surfaces over time.

  20. Longitudinal assessment of sputum microbiome by sequencing of the 16S rRNA gene in non-cystic fibrosis bronchiectasis patients

    Science.gov (United States)

    Turek, Elena M.; Hennessy, Catherine; Mirza, Ghazala K.; James, Phillip L.; Coleman, Meg; Jones, Andrew; Wilson, Robert; Bilton, Diana

    2017-01-01

    Background Bronchiectasis is accompanied by chronic bronchial infection that may drive disease progression. However, the evidence base for antibiotic therapy is limited. DNA based methods offer better identification and quantification of microbial constituents of sputum than standard clinical culture and may help inform patient management strategies. Our study objective was to determine the longitudinal variability of the non-cystic fibrosis (CF) bronchiectasis microbiome in sputum with respect to clinical variables. Eighty-five patients with non-CF bronchiectasis and daily sputum production were recruited from outpatient clinics and followed for six months. Monthly sputum samples and clinical measurements were taken, together with additional samples during exacerbations. 16S rRNA gene sequencing of the sputum microbiota was successful for 381 samples from 76 patients and analysed in conjunction with clinical data. Results Microbial communities were highly individual in composition and stability, usually with limited diversity and often containing multiple pathogens. When compared to DNA sequencing, microbial culture had restricted sensitivity in identifying common pathogens such as Pseudomonas aeruginosa, Haemophilus influenzae, Moraxella catarrhalis. With some exceptions, community characteristics showed poor correlations with clinical features including underlying disease, antibiotic use and exacerbations, with the subject showing the strongest association with community structure. When present, the pathogens mucoid Pseudomonas aeruginosa and Haemophilus influenzae may also shape the structure of the rest of the microbial community. Conclusions The use of microbial community analysis of sputum added to information from microbial culture. A simple model of exacerbations driven by bacterial overgrowth was not supported, suggesting a need for revision of principles for antibiotic therapy. In individual patients, the management of chronic bronchial infection may be

  1. Bronquiectasias associadas à síndrome de Sjögren Bronchiectasis associated to Sjögren syndrome: case report

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    SIMONE APARECIDA CÂMARA TECCHIO

    2000-08-01

    Full Text Available Diversas formas de comprometimento pulmonar têm sido descritas na síndrome de Sjögren, incluindo raros casos em que bronquiectasias foram o achado predominante. Relata-se o caso de uma paciente com história de infecções respiratórias de repetição e dispnéia progressiva de longa evolução, cuja tomografia computadorizada de alta resolução evidenciava inúmeras bronquiectasias. A revisão de uma biópsia pulmonar realizada no início dos sintomas mostrava alterações bronquiolares de natureza inflamatória e fibrótica. Apesar da presença de sintomas de síndrome seca há anos, o diagnóstico de SS foi feito apenas tardiamente. São discutidos os possíveis mecanismos patogenéticos que possam ter levado ao desenvolvimento das bronquiectasias e a necessidade de um apurado senso clínico para o diagnóstico precoce de tais condições.Different kinds of pulmonary impairment have been described in Sjögren syndrome, including rare cases of bronchiectasis. The authors report a female patient with a history of episodes of respiratory infections and progressive breathlessness whose high resolution computerized tomography revealed bronchiectasis. A former open lung biopsy showed bronchiolar inflammatory and fibrotic changes. The diagnosis of Sjögren syndrome was made only late in the evolution, although sicca syndrome symptoms had been present for years. The authors discuss the potential pathogenic mechanisms involved in the development of the bronchiectasis and the need for a high degree of clinical medical skill for the early diagnosis of such conditions.

  2. Treatment advances of non-cystic fibrosis bronchiectasis%非囊性纤维化支气管扩张治疗进展

    Institute of Scientific and Technical Information of China (English)

    马姣; 李银鹏; 刘政

    2014-01-01

    非囊性纤维化支气管扩张是慢性气道破坏性疾病,其可造成严重的社会经济负担.近年来,随着诊疗技术尤其是高分辨率CT的应用,支气管扩张症的早期诊断率随之提高,但治疗效果仍不尽如人意.非囊性纤维化支气管扩张的病理生理基础为持续存在的病原微生物的定植、感染、气道损伤的恶性循环.在治疗方面尤其是高效、不良反应小的吸入性抗生素的地位越来越凸显.本文就非囊性纤维化支气管扩张症的病理、病因以及治疗策略的进展作一综述.%Non-cystic fibrosis bronchiectasis is a chronic airway destructive disease.The associated social and economic cost is heavy.Because high-resolution computed tomography scanning is more commonly used nowadays,bronchiectasis is diagnosed at earlier stage,but the treatment result is unsatisfied.Pathologically,a vicious cycle of infection and inflammation exists in the permanently damaged airways.Lately inhaled prolonged antibiotic is becoming especially prominent due to its high efficacy and small adverse effects.This article reviews the pathology,aetiology and management strategies of non-cystic fibrosis bronchiectasis.

  3. Research on nursing for patients with bronchiectasis associated with hemoptysis%支气管扩张咳血患者的护理研究

    Institute of Scientific and Technical Information of China (English)

    项文侠

    2015-01-01

    Objective:To investigate the nursing method and effect for patients with bronchiectasis associated with hemoptysis .Methods:46 cases of bronchiectasis associated with hemoptysis admitted from July 2011 to July 2013 were randomly divided into the nursing group and the con-trol group, 23 cases in each group.The patients in the two groups received the same clinical treatment , but the patients in the nursing group were given comprehensive and standardized nursing.The clinical effect of the two groups was observed and compared.Results:The effective rate of treat-ment in nursing group was higher than that in the control group, with the difference statistically significant ( P <0.05).Conclusion:For patients with bronchiectasis associated with hemoptysis, tne nursing care for clinical monitoring, health education, psychological counseling, life coaching, and diet and exercise is of importance for the improvement of the effective rate of treatment , which can be widely applied.%目的:探讨支气管扩张咳血患者的护理方法和效果。方法:将2011年7月至2013年7月收治的46例支气管扩张咳血患者随机分为护理组和对照组,每组23例。对两组患者进行相同的临床治疗,对护理组患者实施全面、规范化的护理,观察并对比临床效果。结果:护理组患者的治疗有效率高于对照组,差异具有统计学意义( P <0.05)。结论:对支气管扩张咳血患者进行临床监护、健康教育、心理指导、生活指导、饮食运动等方面的护理,对提高治疗有效率具有重要作用,值得推广。

  4. How Is Bronchiectasis Treated?

    Science.gov (United States)

    ... inflatable therapy vest that uses high-frequency air waves to force mucus toward your upper airways so you can cough it up. A small handheld device that you breathe out through. It causes vibrations that dislodge the mucus. A mask that creates ...

  5. Isolated right pulmonary artery agenesis with agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply from celiac axis with normal venous drainage.

    Science.gov (United States)

    Chaudhry, A; Rathore, M; Banavaliker, J N

    2014-01-01

    Isolated unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly. When detected in infancy, the condition is commonly associated with cardiovascular defects which are more frequently associated with left pulmonary artery agenesis. Patients with isolated right pulmonary artery agenesis survive into adulthood with minimal or no symptoms and are diagnosed incidentally on the chest radiographs. We report a case of a 19-year-old female patient who presented to us with recurrent haemoptysis. She was symptomatic since the age of four years. We report the rare occurrence of UAPA on right side, agenesis of right upper lobe and bronchiectasis of right lower lobe with anomalous arterial supply of right lung from coeliac axis in this patient.

  6. 非囊性纤维化性支气管扩张症的免疫调节机制%Immunoregulatory mechanism of non-cystic fibrosis bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    孙雪峰; 高金明

    2014-01-01

    Bronchiectasis is one of chronic inflammatory bronchial diseases,with the pathological presentation of one or more bronchial permanent dilation,Non-cystic fibrosis bronchiectasis is usually associated with recurrent infections,immunodeficiency or autoimmune diseases,and a quite portion is specific.The vicious cycle of bacterial colonization,airway inflammation,and airway structural damage results in bronchiectasis.Airway inflammation is the key of the cycle,therefore,the studies of immunoregulatory mechanism of airway inflammation are important,which can guide the research and development of drugs in bronchiectasis.This review focuses on molecular and cellular immunoregulatory mechanism associated with the pathogenesis of bronchiectasis,and discusses the mechanism of bacterial chronic colonization.Drug research and development associated with immunoregulation in bronchiectasis is also discussed.%支气管扩张症是慢性炎症性支气管疾病的一种,病理上表现为一个或多个支气管的永久性扩张.非囊性纤维化性支气管扩张症的发病往往与反复感染、免疫缺陷或自身免疫性疾病相关,也有相当一部分呈特发性.细菌定植、气道炎症和气道结构损伤这样一个恶性循环导致了支气管扩张症的发生,其中气道炎症承前启后,是整个恶性循环的关键,因此研究气道炎症的免疫调节机制对理解其发病机理具有重要意义,并可指导支气管扩张症的药物研发.本文着重讨论了与支气管扩张症的发病密切相关的细胞与分子免疫调节机理,并阐述了细菌慢性定植的机理,还结合免疫调节讨论了支气管扩张症的新药研发.

  7. Emergency embolization therapy for massive hemoptysis due to bronchiectasis%支气管扩张大咯血的急诊介入栓塞治疗

    Institute of Scientific and Technical Information of China (English)

    徐伟; 史中兴; 徐春阳; 王宏辉

    2014-01-01

    Objective To identify various arteriographic manifestations of massive hemoptysis due to bronchiectasis and the efficacy of emergency embolization .Methods Emergency arteriography was performed for 90 patients of bronchietasis with massive hemoptysis .Emergency embolization was conducted using embolic agents as soon as bleeding arteries were i‐dentified .The arteriographic manifestations and effect of the treatment for massive hemoptysis were retrospectively ana‐lyzed .Results 135 bleeding arteries were found and be embolized succefully in 90 patients .A 12‐month follow‐up re‐vealed that complete hemostasis was achieved in 84 patients within 24 hours ,6 hemoptysis stopped completely in 3 days of embolotherapy .8 patients had recurrent hemoptysis ,among whom 6 hemoptysis stopped completely after lobectomies ,2 patients died of the complications of TB/aspergillus coinfection .The overall effective rate of the emergency embolization was 91 .11% (82/90) .No serious complications occurred after embolotherapy .Conclusion Emergency arterial emboliza‐tion for hemoptysis of bronchiectasis is a safe and effective treatment for massive hemoptysis due to bronchiectasis .The key to successful treatment is reasonable choice and use of embolic agents and thorough and complete embolization of the bleeding arteries .%目的:探讨90例支气管扩张大咯血患者的动脉造影表现及急诊介入栓塞治疗的疗效。方法对90例支气管扩张大咯血的患者行急诊动脉造影检查,明确出血部位后,选用不同的栓塞剂栓塞出血动脉,对动脉造影的表现及治疗结果进行回顾性总结及评价。结果90例患者共找到135支出血动脉,全部成功栓塞治疗。栓塞术后随访12个月,8例患者复发咯血,6例采用肺叶切除手术后出血停止。2例患者死于结核菌合并曲霉菌感染所致的并发症。栓塞治疗的总有效率91.11%(82/90)。栓塞术后无严重并发症出现。结

  8. Recent advance in non-cystic fibrosis bronchiectasis%非囊性纤维化支气管扩张症的最新进展

    Institute of Scientific and Technical Information of China (English)

    张永祥; 侯志云; 尹凤先; 赵莹

    2012-01-01

    支气管扩张症是气道多种病原菌清除不良与反复感染引起的慢性炎症与支气管壁破坏,导致气道永久性扩张.非囊性纤维化支气管扩张症(non-cystic fibrosis bronchiectasis,NCFB)是一种临床低估的疾病,诊断时要注意其基础病因,但大多数病因不清楚.治疗要强调个体化并注意随访,临床评价常用的工具是莱斯特咳嗽问卷与痰液颜色.要对细菌定植进行定期评价.NCFB的治疗研究很少,长期应用抗生素可改善临床症状,但不降低急性加重发生率,也不改善肺功能.有严重感染或出血危险性的1或2叶严重损害的患者可考虑手术治疗.本文重点为NCFB,指出了其处理及肺移植治疗,进一步研究其病理生理学机制与探索新的治疗方法是非常必要的.%Bronchiectasis is permanently dilated airways caused by chronic bronchial inflammation secondary to inappropriate clearance of various micro-organisms and recurrent infections in the airways,as well as destruction of the bronchial walls.Non-cystic fibrosis bronchiectasis (NCFB) is an underestimated disease.At diagnosis,one should search for the underlying disease process.However,in a substantial number of patients no cause is found.The patients need individualised therapy and follow-up by monitoring of their symptoms.Useful tools are the Leicester cough questionnaire and the sputum colour chart.Screening patients for bacterial colonisation on a regular basis seems to be important,as many patients become colonised by pathogenic microorganisms.Treatment for NCFB lacks evidence.Prolongeduse antibiotics improve clinical response rates,but may not reduce exacerbation rates or relieve lung function.Surgery is often considered for people with extreme damage to one or two lobes of the lung who are at risk for severe infection or bleeding.In this review,the authors will focus on NCFB,pointing out in management and lung transplantation.Further research into the underlying

  9. Bronquiectasias: aspectos diagnósticos e terapêuticos Estudo de 170 pacientes Bronchiectasis: diagnostic and therapeutic features A study of 170 patients

    Directory of Open Access Journals (Sweden)

    José da Silva Moreira

    2003-10-01

    Full Text Available INTRODUÇÃO: Bronquiectasias são freqüentemente encontradas na prática médica no Brasil, levando a significativa morbidez e comprometimento da qualidade de vida de seus portadores. OBJETIVOS: Analisar aspectos diagnósticos e terapêuticos em uma série de pacientes com bronquiectasias atendidos em um serviço de doenças pulmonares. MÉTODO: Sinais, sintomas, achados radiográficos e microbiológicos, e resultados terapêuticos foram estudados em 170 pacientes portadores de bronquiectasias hospitalizados no período de 1978 a 2001 - 62,4% do sexo feminino, 37,6% do masculino, com idade média de 37 anos, variando entre 12 e 88 anos. RESULTADOS: Antecedente de pneumonia na infância foi detectado em 52,5% dos pacientes, de tratamento tisiológico em 19,8%; 8,8% tinham asma brônquica, e dois tinham síndrome de Kartagener. Os sintomas mais comuns foram tosse (100%, expectoração (96% e estertores pulmonares (66%. As lesões eram unilaterais em 46,5% dos casos. Pneumococo, H. influenzae ou flora mista estiveram presentes em 85% das amostras de escarro examinadas. Os 170 pacientes receberam inicialmente tratamento clínico à base de antibióticos e fisioterapia respiratória; 88 deles (52% mais jovens, com lesões menores e boa reserva funcional foram submetidos à cirurgia de ressecção pulmonar (82 unilaterais e seis bilaterais. Ocorreram dois óbitos hospitalares entre os pacientes que receberam tratamento exclusivamente clínico. Os pacientes tratados cirurgicamente tiveram acentuada melhora dos sintomas, raramente necessitando ser reinternados. CONCLUSÕES: Os prolongados sintomas broncopulmonares foram permanentemente aliviados na maioria dos pacientes com bronquiectasias que puderam ir à cirurgia de ressecção pulmonar, diferentemente dos que seguiram com o tratamento clínico.BACKGROUND: Bronchiectasis is a frequently found disease in medical practice in Brazil leading to significant morbidity and decrease in quality of life of

  10. Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers - Manes Kartagener and Bjorn Afzelius

    Energy Technology Data Exchange (ETDEWEB)

    Berdon, Walter E. [Department of Radiology, Children' s Hospital of New York, 3959 Broadway, CHN 3-325, NY 10032, New York (United States); Willi, Ulrich [Department of Radiology, University of Zurich Children' s Hospital, Zurich (Switzerland)

    2004-01-01

    The relationship of Kartagener's syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartagener's triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome. (orig.)

  11. Situs inversus, bronchiectasis, and sinusitis and its relation to immotile cilia: history of the diseases and their discoverers-Manes Kartagener and Bjorn Afzelius.

    Science.gov (United States)

    Berdon, Walter E; Willi, Ulrich

    2004-01-01

    The relationship of Kartagener's syndrome to immobile cilia syndrome is a fascinating merging of clinical observations and basic science in Zurich, Stockholm, and Toronto. In 1933, Manes Kartagener, a Zurich pulmonary physician, reported four patients with the triad of sinusitis, bronchiectasis, and situs inversus. In the following decades, he reviewed reports of hundreds of cases, but the fact that the male patients with the condition never had offspring eluded his notice. In the 1970s, Bjorn Afzelius, a Ph.D. ultrastructuralist from Stockholm, reported cilia immotility in infertile males, some of the cases occurring in families. Half of the cases had Kartagener's triad. The observation of Afzelius was soon applied to children by Jennifer Sturgess, a Ph.D. ultrastructuralist, and her medical colleagues in Toronto. With over 500 MEDLINE references since 1966 on Kartagener's and over 1,000 references on immotile cilia, the causes of the pulmonary infections have become clearer as the patients demonstrate impaired clearance of mucus with resultant sinus and bronchial disease. The cause of the situs inversus remains elusive to this day. It is appropriate to call the condition Kartagener-Afzelius syndrome.

  12. Bronquiectasia e fisioterapia desobstrutiva: ênfase em drenagem postural e percussão Bronchiectasis and clearence physiotherapy: emphasis in postural drainage and percussion

    Directory of Open Access Journals (Sweden)

    Neuseli Marino Lamari

    2006-06-01

    Full Text Available Bronquiectasia consiste em dilatação anormal, permanente e irreversível de brônquios e bronquíolos, com infecções recorrentes, inflamações, hipersecreção e redução da limpeza mucociliar. Acomete predominantemente o sexo feminino, entre 28 e 48 anos de idade e afeta com maior freqüência os lobos inferiores bilateralmente. Manifestações clínicas da doença são a tosse crônica, febre e expectoração volumosa, purulenta, com odor fétido. Etiologia é inespecífica e representada pelo estádio final de diversos processos patológicos. Pode ser classificada em cilíndrica, varicosa e sacular, e ainda, em localizada e multissegmentar. Drenagem postural e percussão são técnicas desobstrutivas usuais na prática clínica diária, no entanto, há escassez de estudos comparativos enfatizando-as com amostras populacionais e recursos metodológicos. Tomando por base as considerações, teve-se como objetivo verificar a eficácia da drenagem postural e da percussão na higiene brônquica de pacientes bronquiectásicos, bem como seus efeitos e associação com outras técnicas apontadas pela literatura atual. Os principais achados comprovaram que a drenagem postural e a percussão são efetivas na mobilização da secreção pulmonar, uma vez que aumentam a velocidade do muco transportado, melhoram a função pulmonar e as trocas gasosas. A efetividade requer ajuda de um profissional, o que pode dificultar a prática clínica diária. Por esta razão, fisioterapeutas têm selecionado técnicas que propiciem independência ao paciente.Bronchiectasis consists of abnormal, permanent and irreversible dilation of bronchi and bronchia, with recurrent infections, inflammation, hypersecretion and reduction of mucus clearance. It predominantly affects women of between 28 and 48 years old and more frequently affects the inferior lobes. Clinical manifestations are chronic cough, fever and voluminous expectoration, with a fetid odor. The etiology

  13. 云南白药联合酚妥拉明治疗支气管扩张咯血的临床分析%Clinical Analysis of Yunnan Baiyao Combined with Phentolamine in Treatment of Bronchiectasis Hemoptysis

    Institute of Scientific and Technical Information of China (English)

    杨桂玲

    2014-01-01

    目的:探讨云南白药联合酚妥拉明治疗支气管扩张咯血的临床疗效。方法90例支气管扩张咯血患者,分成观察组和对照组。对照组给予酚妥拉明治疗。观察组给予云南白药联合酚妥拉明治疗。结果观察组总有效率97.78%高于对照组82.22%,差异有统计学意义(P<0.05)。结论云南白药联合酚妥拉明治疗支气管扩张咯血患者疗效佳,建议临床推广应用。%Objective To discuss the clinical effect of Yunnan Baiyao combined with phentolamine in treatment of bronchiectasis hemoptysis. Method 90 cases of bronchiectasis hemoptysis patients, divided into the observation group and the control group. The control group was treated with phentolamine treatment. The observation group was given Yunnan Baiyao combined with phentolamine treatment. Results The observation group the total effective rate was 97.78%higher than 82.22%in the control group, the difference was statistically signiifcant (P<0.05). Conclusion Yunnan Baiyao combined with phentolamine in treating bronchiectasis hemoptysis of patients with good effect, recommended for clinical application.

  14. Clinical observation of pituitrin and phentolamine in the treatment of patients with bronchiectasis hemoptysis%脑垂体后叶素联合酚妥拉明治疗支气管扩张咯血临床观察

    Institute of Scientific and Technical Information of China (English)

    刘芸芸; 刘志刚

    2013-01-01

    目的 观察脑垂体后叶素联合酚妥拉明治疗支气管扩张咯血临床治疗效果.方法 对本院诊治的52例支气管扩张咯血的患者临床治疗进行分析.结果 52例支气管扩张咯血的患者入院后,经过予以脑垂体后叶素联合酚妥拉明止血、抗感染、对症支持等治疗,病情完全缓解43例,占82.7%,明显好转6例,占11.5%,治疗无效2例,占3.8%,死亡1例,占1.9%.结论 脑垂体后叶素联合酚妥拉明治疗支气管扩张咯血,治愈率高,死亡率低.不良反应少.%Objective To observe the curative effect of pituitrin and phentolamine in the treatment of patients with bronchiectasis hemoptysis. Methods The cliniral treatment of 52 patients with bronchiectasis hemoptysis was analyzed. Results 43 out of 52 patients with bionchiectasis hemoptysis were cured while after the treatment of pituitrin and phentolamine, and 6 cases were better improved. 2 cases were ineffective and 1 case dead reported. Conclusion The use of pituitrin combined with phentolamine has a high curative rate, low mortality and little side-effect in the treatment of bronchiectasis hemoptysis.

  15. Bronquiectasias en adultos: Características clínicas Experiencia de 5 años 1998-2003 RETROSPECTIVE EVALUATION OF BRONCHIECTASIS CHARACTERISTICS IN ADULTS AT A CHILEAN GENERAL HOSPITAL: A 5 YEARS EXPERIENCE (1998-2003

    Directory of Open Access Journals (Sweden)

    Jaime Cereceda P.

    2005-09-01

    Full Text Available Para conocer las características actuales de las bronquiectasias en adultos en nuestro medio se revisan los 18 casos de esta patología comprobados por TAC helicoidal de tórax en el Hospital Regional de Concepción entre 1998-2003. La edad promedio de los pacientes fue de 44 ± 13,9 años; 10 hombres y 8 mujeres. Las etiologías más frecuentes de bronquiectasias fueron tuberculosis pulmonar (44,4% y neumonía aguda (38,8%. Los síntomas más relevantes fueron tos crónica (88,8%, expectoración persistente (77,7% y hemoptisis (44,8%. La espirometría reveló alteración ventilatoria obstructiva tanto en fumadores (VEF1/CVF = 58% como en no fumadores (VEF1/CVF = 68%. La bacteriología de expectoración detectó Hemophilus influenzae (16,6%, Neisseria catharralis (15%, Pseudomona aeruginosa (13%, Streptococcus pneumoniae (15,4% y Candida albicans (4%. La TAC de tórax demostró lesiones bilobares (72%, multilobares (16,6% y unilobares (11,1%. La realidad de las bronquiectasias en nuestro medio es que no difiere significativamente de lo descrito en la literaturaIn order to know the actual characteristics of bronchiectasis in an adult population, we reviewed 18 cases with this pathology confirmed by spiral CT at a general hospital (Hospital Regional de Concepción, Chile, between 1998-2003. Ten patients were males and 8 females, their mean age was 44 ± 13.9 years old. Most common etiologies of bronchiectasis were pulmonary tuberculosis (44.4% and acute pneumonia (38.8%; main sypmtoms were chronic cough (88.8%, persistent sputum (77.7% and hemoptysis (44.4%. Spirometry detected airway obstruction in smokers (FEV1/FVC = 58% and in non smokers (FEV1/FVC = 68%. Microbiological sputum exams showed Hemophilus influenzae (16.6%, Neisseria catharralis (15%, Pseudomona aeruginosa (13%, Streptococcus pneumoniae (15.4% and Candida albicans (4%. Spiral CT showed the following distribution of bronchiectasis: unilobular lesion 11.1%, bilobar 72% and

  16. Observing Curative Effect of Pituitrin and Phentolamine in Treatment of Bronchiectasis with Hemoptysis%垂体后叶素联合酚妥拉明治疗支气管扩张伴咯血的疗效观察

    Institute of Scientific and Technical Information of China (English)

    梁文波

    2013-01-01

    Objective: To explore the clinical efficacy of pituitrin and phentolamine in treatment of bronchiectasis with hemoptysis. Methods: 52 patients of bronchiectasis with hemoptysis were randomly divided into study group and control group, on the basis of conventional therapy, study group used pituitrin and phentolamine, while the control group only used pituitrin, the curative effect and adverse reaction of two groups was observed. Results: the total effective rate was 96.15% in study group, higher than 76.92% in the control group (P<0.05); The incidence of adverse reaction in study group was 7.69%, lower than that of 30.77% in the control group (P<0.05). Conclusion: Pituitrin and phentolamine is a better method for treatment of bronchiectasis with hemoptysis, and wor-thy clinical promotion.%  目的:探讨垂体后叶素联合酚妥拉明治疗支气管扩张症伴咯血的临床疗效.方法:将52例支气管扩张伴咯血患者随机分为研究组和对照组,在常规治疗的基础上,研究组使用垂体后叶素联合酚妥拉明治疗,对照组则单纯使用垂体后叶素治疗,观察两组患者的疗效和不良反应发生情况.结果:研究组的总有效率为96.15%,高于对照组的76.92%(P<0.05);研究组不良反应发生率为7.69%,低于对照组的30.77%(P<0.05).结论:垂体后叶素联合酚妥拉明是治疗支气管扩张伴咯血的一种较好的治疗方法,值得临床推广使用.

  17. 垂体后叶素联合酚妥拉明治疗支气管扩张咯血临床观察%Pituitrin Combined with Phentolamine for Hemoptysis Caused by Bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    熊焕文; 徐劲松

    2014-01-01

    目的:探讨垂体后叶素联合酚妥拉明治疗支气管扩张咯血的疗效。方法将56例支气管扩张咯血患者按随机数字表法分为2组,治疗组(28例)联合应用垂体后叶素与酚妥拉明,对照组(28例)单纯应用垂体后叶素,比较2组的疗效及不良反应发生情况。结果治疗组总有效率为96.4%,对照组总有效率为82.0%,2组比较差异有统计学意义(P<0.05),不良反应发生率治疗组为7.1%,对照组为46.4%,2组比较差异有统计学意义(P<0.05)。结论垂体后叶素联合酚妥拉明治疗支气管扩张咯血的疗效显著,不良反应少。%Objective To investigate the efficacy of pituitrin combined with phentolamine in the treatment of hemoptysis caused by bronchiectasis.Methods Fifty-six patients with hemoptysis caused by bronchiectasis were randomly treated with pituitrin alone(control group,n=28)or in combination with phentolamine(treatment group,n=28).The efficacy and adverse reactions were compared between the two groups.Results Compared with control group,the combined treat-ment with pituitrin and phentolamine induced an increase in the total effective rate(96.4% vs 82.0%,P<0.05)and a decrease in the incidence of adverse reactions(7.1% vs 46.4%,P<0.05).Conclusion The combination of pituitrin and phentolamine is an effective treatment with few side effects in patients with hemoptysis caused by bronchiectasis.

  18. 支气管哮喘合并支气管扩张患者临床特点及治疗方法分析%The analysis of clinical characteristics and treatment methods about patients with bronchial asthma complicated with bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    王勇; 何权瀛

    2016-01-01

    Objective To explore clinical characteristics and treatment methods of patients with bronchial asthma complicated with bronchiectasis.Methods 62 patients with asthma complicated with bronchiectasis were selected.Medical history,symptoms,signs,auxiliary examination and treatment methods were extracted from medical records of patients to carry on a summary analysis.Results Clinical characteristics of patients with bronchial asthma complicate with bronchiectasis were as follows:The frequency of personal allergic and family history of asthma was high.Clinical symptoms always showed the overlap symptoms of asthma and bronchiectasis and were atypical.Most patients had obviously seasonal regularity and attack frequency and hospitalization rate were high.The control of asthma symptoms was difficult.Bronchiectasis regions were not obviously regular on chest HRCT.The positive rate of bronchial provocation test was significantly higher than that of the bronchial dilation test.Positive rate of bacterial culture of sputum was not high.All patients were adopted comprehensive treatment measures of anti-asthma and anti-bronchiectasis and achieved good therapeutic effect.Conclusion Bronchial asthma complicated with bronchiectasis is a special clinical type,which can be differentiate from pure bronchiectasis or pure asthma by the clinical features,bronchial provocation tests,bronchial dilation test and chest HRCT. Comprehensive treatment measures of anti-asthma and anti-bronchiectasis are recommended.%目的:探讨支气管哮喘(简称哮喘)合并支气管扩张(简称支扩)的临床特点及治疗方法。方法选取62例哮喘合并支扩的住院患者,从病历中收集患者的病史、症状、体征、辅助检查、治疗方法等资料进行总结分析。结果(1)哮喘合并支扩患者临床特点为:①多有个人过敏性疾病史和哮喘家族史,症状多表现为哮喘和支扩症状的叠加,体征不典型,有明

  19. The analysis of bacteriology and antibiotic susceptibility in 493 patients with bronchiectasis complicated with acute infection%493例支气管扩张急性感染的细菌学特点及药敏分析

    Institute of Scientific and Technical Information of China (English)

    宋杰; 刘保清; 王金祥; 胥振阳; 李晓辉

    2012-01-01

    目的 分析支气管扩张症患者急性感染的细菌学特点及药敏情况.方法 对本院呼吸科收治的493例支气管扩张急性感染患者痰标本的细菌培养及药敏试验进行分析.结果 493例痰标本分离出细菌200株,其中革兰阴性杆菌178株,占89.0%,革兰阳性球菌22株,占11.0%.根据五年统计结果,总数排在前6位的分别是铜绿假单胞菌(21.5%)、洛菲不动杆菌(15.5%)、肺炎克雷伯菌(8.5%),肠杆菌属(包括阴沟肠杆菌、产气肠杆菌、中间型肠杆菌及聚团肠杆菌)(8%)、鲍曼溶血不动杆菌(5.5%)和金黄色葡萄球菌(5%).结论 支气管扩张急性感染患者的细菌分布以革兰氏阴性菌为主,铜绿假单胞菌排在首位,提示合理选用抗菌药物对减少耐药菌的产生有重要作用.%Objective To investigate the bacteriology and antibiotic susceptibility in patients with bronchiectasis complicated with acute infection. Methods Data of sputum culture and antibiotic susceptibility of 493 patients with bronchiectasis complicated with acute infection from Jul 2006 to Aug 2011 were retrospectively analyzed. Results In the test of sputum culture, 200 strains of bacteria were ob-tained. Among them, there were 178 strains of gram negative bacilli ( 89. 0% ), and 22 strains of gram positive coccus ( 11. 0% ) . According to the statistical results of five years, the bacteria ranked the top six were Pseudomonas aeruginosa ( 21. 5% ), Acinetobacter lwoffii (11. 5% ), Klebsiella pneumonia( 8. 5% ), Enterobacter ( Enterobacter cloacae, Enterobacter aerogenes, Enterobacter intermedi-us and Enterobacter agglomerans) ( 8% ). Acinetobacter baumannii ( 5. 5% ), Staphylococcus aureus rosenbach( 5% ). Conclusions The most common pathogenic bacteria that induce acute infection in patients with bronchiectasis are Gram-negative bacteria, especially pseudomonas aeruginosa. It is suggested that reasonable use of antibiotics to reduce drug resistance is very important.

  20. Clinical value of Broncho-Vaxom combined with postural drainage in the treatment of bronchiectasis%泛复舒联合体位引流治疗支气管扩张的临床研究

    Institute of Scientific and Technical Information of China (English)

    谢衬梨; 郭庆玲; 蔡杰; 梁秋亭

    2015-01-01

    目的 探讨泛福舒联合体位引流对支气管扩张患者治疗的临床疗效.方法 选择支气管扩张患者78例,经过急性期治疗病情得到控制后,以自愿原则随机双盲分为两组,对照组单纯体位引流,治疗组口服泛福舒的同时体位引流,观察两组患者3个月后免疫指标变化和6个月内临床症状即每日咳嗽咳痰量及呼吸道再次感染次数.结果 治疗组病例咳嗽咳痰的症状减轻显著高于对照组(P=0.046),呼吸道再次感染次数显著下降(P=3.43× 10-12);治疗组治疗后CD4+、CD4+/CD8+、NK细胞活性值均较治疗前明显提高(P<0.0 1),CD8+活性值较治疗前降低(P< 0.01).对照组治疗前后CD4+、CD4+/CD8+、NK值均无明显变化.治疗组患者体内IgG、IgA的表达水平在治疗后显著高于对照组患者(P<0.01).结论 泛福舒联合体位引流可减少支气管扩张患者每日咳嗽及咳痰量,降低呼吸道再次感染发病率,增强机体免疫力.且泛福舒药物耐受性好,副反应小.%Objective To investigate the clinical effect of Broncho-Vaxom combined with postural drainage in the treatment of bronchiectasis.Methods 78 cases of bronchiectasis whose acute phase condition was under control after treatment,were randomly double-blind divided into two groups.Control group received postural drainage,and treatment group received Broncho-Vaxom capsule combined with postural drainage.Compared immune indexes after 3 months and clinical symptoms including cough and expectoration,number of respiratory infection during 6 months between two groups.Results Cough and expectoration symptoms after treatment in treatment group were better than those in control group (P=0.046),the number of respiratory tract infection was lower (P=3.43× 1012).Expression levels of CD4+,CD4+/CD8+,NK after treatment improved significantly in treatment group (P<0.01),expression level of CD8+ decreased (P<0.01).Expression levels of CD4+,CD4+/CD8+,NK in control group

  1. Clinical treatment analysis on 36 patients with bronchiectasis complicated with asthma%支气管扩张合并哮喘36例临床治疗分析

    Institute of Scientific and Technical Information of China (English)

    刘锋; 钟丽球

    2013-01-01

    目的:观察我院收治的36例支气管扩张合并哮喘患者的临床治疗效果,并分析其治疗方案。方法选取我院2010年3月~2013年2月收治的支气管扩张并哮喘患者36例,对其使用抗菌、化痰等药物进行常规治疗,同时使用糖皮质激素静脉注射与雾化吸入联合应用,辅助以无创正压通气及纤维支气管镜下吸痰等物理治疗,观察其临床治疗效果。结果所有入组患者在治疗后临床症状及体征均有明显的缓解,痰涂片实验室检查及肺功能检测各项指标水平均有明显改善,差异有统计学意义(P<0.05)。结论支气管扩张合并哮喘患者临床治疗难度较高,我院通过对其进行多途径、多机制联合用药治疗并辅以物理治疗,能够有效的缓解其临床症状,改善患者肺功能情况与病情,具有理想的临床应用效果与价值。%Objective To observe the clinical therapeutic effect of 36 patients with bronchiectasis complicated with asthma in our hospital, and analyze its treatment plan. Methods Thirty-six patients with bronchiectasis complicated with asthma admitted into our hospital from March 2010 to February 2013 were selected. All patients were treated with antimicrobial and reducing phlegm drugs routine treatment, as well as glucocorticoid intravenous injection combined with aerosol inhalation therapy, and aided by noninvasive positive pressure ventilation and bronchoscopic suctioning phlegm physical therapy. The clinical therapeutic effect of patients was observed. Results The clinical symptoms and signs of all patients relieved obviously after the treatment, and the sputum smear laboratory examination and level of pulmonary function testing indexes improved obviously, the differences had statistical significance between before and after the treatment(P<0.05). Conclusion The clinical treatment of bronchiectasis complicated with asthma is difficult, which treated by multi ways

  2. Adesividade e purulência de secreções respiratórias: implicações no transporte mucociliar em pacientes com bronquiectasias Adhesiveness and purulence of respiratory secretions: implications for mucociliary transport in patients with bronchiectasis

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    Joana Tambascio

    2010-10-01

    Full Text Available OBJETIVO: Analisar e comparar as propriedades de transporte de secreções respiratórias, classificadas através de parâmetros selecionados, de indivíduos com bronquiectasias não secundárias à fibrose cística. MÉTODOS: Foram avaliadas amostras de muco respiratório, classificadas como com propriedades de superfície adesivas ou não adesivas, assim como com aspecto mucoide ou purulento, de 35 participantes com bronquiectasias não secundárias à fibrose cística, quanto a velocidade relativa de transporte (VRT, deslocamento em máquina simuladora de tosse (MST e ângulo de contato (AC. Para as comparações propostas, foram utilizados modelos de ANOVA, com nível de significância estabelecido em 5%. RESULTADOS: Houve uma diminuição significativa no deslocamento em MST, assim como um aumento significativo no AC, das amostras adesivas quando comparadas às não adesivas (6,52 ± 1,88 cm vs. 8,93 ± 2,81 cm e 27,08 ± 6,13º vs. 22,53 ± 5,92º, respectivamente; p OBJECTIVE: To analyze and compare the transport properties of respiratory secretions, classified by selected parameters, in individuals with bronchiectasis unrelated to cystic fibrosis. METHODS: We collected mucus samples from 35 individuals with bronchiectasis unrelated to cystic fibrosis. The samples were first classified by their surface properties (adhesive or nonadhesive, as well as by their aspect (mucoid or purulent. We then tested the samples regarding relative transport velocity (RTV, displacement in a simulated cough machine (SCM, and contact angle (CA. For the proposed comparisons, we used ANOVA models, with a level of significance set at 5%. RESULTS: In comparison with nonadhesive samples, adhesive samples showed significantly less displacement in the SCM, as well as a significantly higher CA (6.52 ± 1.88 cm vs. 8.93 ± 2.81 cm and 27.08 ± 6.13º vs. 22.53 ± 5.92º, respectively; p < 0.05 for both. The same was true in the comparison between purulent and mucoid

  3. 118例支气管扩张合并感染患者的病原学及药敏分析%Analysis of etiology and antibiotic susceptibility in infection complicated in bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    申辉; 韩明锋; 腾小宝; 赵凤德; 时靖峰

    2015-01-01

    Objective To investigate the etiology and antibiotic susceptibility in patients with bronchiectasis complicated by severe infection treated in Fuyang No. 2 hospital in 2013 to indicate the use of antibiotics. Methods Data of sputum culture and antibiotic suscepti-bility of 118 patients with boron complicated by severe infection were retrospectively analyzed. Results Positive sputum culture was obtained in 68 patients with the positive rate of 57. 6%. There were 66 stains of gram negative bacteria(97. 0%). Pseudomonas aeruginosa were the most common pathogens and had high sensitivity with cefozolin of III,IV,quinolones,aminoglycosides,carbopenems. Conclusion Gram-negative bacteria,especially pseudomonas aeruginosa are the main pathogens found in bronchiectasis patients. Before the report of suscepti-bility results,the cefozolin of III,IV,quinolones,aminoglycosides,carbopenems are experiential options.%目的:分析118例支气管扩张合并感染住院患者的病原学及药敏情况,以指导临床抗菌药物的应用。方法对因支气管扩张合并感染需要住院的118例患者的痰培养和药敏结果进行回顾分析。结果118例痰培养68例阳性,阳性率为57.6%,其中革兰阴性菌66株,占97.0%,最常见的病原菌为铜绿假单孢菌,对第三、四代头孢菌素、氟喹诺酮类、氨基糖甙类、碳青霉烯类抗菌药物敏感。结论支气管扩张患者病原菌分布以革兰阴性杆菌为主,尤其铜绿假单孢菌感染。在药敏结果未报告前,可经验选择第三、四代头孢菌素、碳青霉烯类、氟喹诺酮类或氨基糖甙类抗菌药物。

  4. Clinical Pharmacist’s Pharmacy Practice for One Patient with Bronchiectasis Complicating with Infection%临床药师参与1例支气管扩张并感染患者的药学实践

    Institute of Scientific and Technical Information of China (English)

    梅昭; 刘晶; 金桂兰

    2014-01-01

    OBJECTIVE:To explore the role of clinical pharmacist in clinical treatment for one patient with bronchiectasis com-plicating with infection. METHODS:The clinical pharmacist assisted clinician in making individualized treatment plan for the pa-tient based on the relevant guidelines for diagnosis and treatment of bronchiectasis and the results of etiological test,monitored the possible adverse drug reactions and provided rational pharmaceutical care and health education programs. RESULTS:Based on sus-ceptibility test result and patient’s renal function,clinical pharmacist made a dosage regimen of amikacin(0.3 g)plus ciprofloxa-cin(0.4 g)by ivgtt every 12 h which was adopted by clinicians and achieved good treatment effect. The patient was discharged in stable condition. CONCLUSIONS:Clinical pharmacist’involvement in clinical drug treatment practice can help improve clinical medical treatment level.%目的:探讨临床药师在1例支气管扩张并感染患者治疗中的作用。方法:临床药师协助医师,根据支气管扩张症诊疗相关指南及病原学结果为患者制订个体化治疗方案,关注患者在用药期间可能出现的不良反应,提供合理的药学服务和健康教育计划。结果:依据患者的药敏结果和肾功能情况,将给药方案调整为阿米卡星0.3 g、静脉滴注、q12h+环丙沙星0.4 g、静脉滴注、q12h,临床医师采纳了药师的建议,取得了良好的治疗效果,患者病情稳定出院。结论:临床药师参与临床药物治疗实践,有利于提高临床药物治疗水平。

  5. Nocardiose pulmonar em portador de doença pulmonar obstrutiva crônica e bronquiectasias Pulmonary nocardiosis in a patient with chronic obstructive pulmonary disease and bronchiectasis

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    Miguel Abidon Aidê

    2008-11-01

    Full Text Available Relatamos o caso de um paciente com doença pulmonar obstrutiva crônica e bronquiectasias, em uso crônico de corticosteróides, que desenvolveu nocardiose pulmonar, sob a forma de múltiplos nódulos pulmonares escavados. Os sintomas principais foram a tosse produtiva com escarro purulento, febre e dispnéia A radiografia simples e a tomografia computadorizada do tórax mostravam nódulos em ambos os pulmões, alguns escavados. O exame direto de escarro e a cultura mostraram a presença de Nocardia spp. A paciente foi tratada com imipenem e cilastatina, com excelente resposta clínica.We report the case of a patient with chronic obstructive pulmonary disease and bronchiectasis, chronically using corticosteroids, who acquired pulmonary nocardiosis, which presented as multiple cavitated nodules. The principal symptoms were fever, dyspnea and productive cough with purulent sputum. Chest X-ray and computed tomography of the chest revealed nodules, some of which were cavitated, in both lungs. Sputum smear microscopy and culture revealed the presence of Nocardia spp. The patient was treated with imipenem and cilastatin, which produced an excellent clinical response.

  6. A influência de bactérias patogênicas na transportabilidade do escarro e na qualidade de vida de portadores de bronquiectasia The influence of pathogenic bacteria on transportability of sputum and quality of life among patients with bronchiectasis

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    RC Zanchet

    2006-12-01

    Full Text Available INTRODUÇÃO: A qualidade de vida pode estar relacionada com o estado clínico do paciente, com o nível de infecção e com o microorganismo que o infecta. OBJETIVO: Analisar o perfil bacteriológico do escarro de pacientes com bronquiectasia e avaliar seu efeito no transporte in vitro e na qualidade de vida dos pacientes. MÉTODOS: Pacientes com bronquiectasia foram avaliados por questionários de qualidade de vida, cultura bacteriana e transporte in vitro do escarro. RESULTADOS: Foram incluídos 19 pacientes com bronquiectasia, com média de idade de 38,6 ± 16 anos. O grupo de portadores de bactérias potencialmente patogênicas, com 10 pacientes (grupo I, foi comparado ao grupo de portadores de bactérias não patogênicas, com 9 pacientes (grupo II. O grupo I teve menor velocidade relativa e maior deslocamento por tosse que o grupo II (p INTRODUCTION: Patients' quality of life may be related to their clinical status and level of infection, and to the infecting microorganism. OBJECTIVE: To analyze the bacteriological profile of sputum from patients with bronchiectasis and to determine the effect of such bacteria on in vitro transport and patients' quality of life. METHODS: Patients with bronchiectasis were evaluated by means of quality-of-life questionnaires and sputum bacterial culturing and in vitro transport. RESULTS: Nineteen patients with bronchiectasis (mean age: 38.6 ± 16 years were included in the study. A group of 10 patients with potentially pathogenic bacteria (group I was compared with a group of 9 patients with nonpathogenic bacteria (group II. Group I presented lower relative transport velocity and greater displacement per cough maneuver than did group II (p < 0.05. Using the St. George's Hospital respiratory questionnaire, group I presented poorer quality of life in the impact domain (p < 0.05. Using the World Health Organization's Quality-of-Life brief questionnaire, group I also presented poorer quality of life in the

  7. Observation on the effect of phentolamine combined with pituitrin in the treatment of bronchiectasis with massive hemoptysis in eldly patients%酚妥拉明联合垂体后叶素在老年支气管扩张伴大咯血病症治疗中的效果观察

    Institute of Scientific and Technical Information of China (English)

    陈娟娟

    2016-01-01

    目的:分析酚妥拉明与垂体后叶素的联合疗法在老年支气管扩张伴大咯血治疗中的应用效果.方法:收治老年支气管扩张伴大咯血患者120例,随机平分为两组,均给予常规对症治疗,对照组在此基础上采用酚妥拉明治疗,观察组则采用酚妥拉明联合垂体后叶素治疗.统计两组治疗有效率,对比两组咯血时间、咯血量与不良反应情况.结果:观察组治疗有效率 95.0%,高于对照组的 78.3%;观察组咯血时间、总咯血量、不良反应率低于对照组,P<0.05.结论:在老年支气管扩张伴大咯血的治疗中,酚妥拉明与垂体后叶素的联合疗法能够有效地提高治疗有效率,不良反应少.%Objective:To analyze the effect of phentolamine combined with pituitrin in the treatment of bronchiectasis with massive hemoptysis in eldly patients.Methods:120 eldly patients with bronchiectasis and massive hemoptysis were selected,they were randomly divided into the two groups,all patients were treated with conventional symptomatic treatment, patients in the control group were treated with phentolamine on the basis of conventional therapy,patients in the observation group were treated with phentolamine combined with pituitrin treatment.We counted the treatment efficiency of the two groups,and compared the hemoptysis time,the amount of hemoptysis and adverse reactions.Results:The effective rate of the observation group 95% was higher than that of the control group of 78.3%.The hemoptysis time,the total amount of hemoptysis,the rate of adverse reaction of the observation group were lower than the control group,P<0.05.Conclusion:In the treatment of eldly patients with bronchiectasis and massive hemoptysis,phentolamine combined with pituitrin therapy can effectively improve the efficiency of treatment and with less adverse reactions.

  8. Distância percorrida no teste de caminhada de seis minutos não se relaciona com qualidade de vida em pacientes com bronquiectasias não fibrocísticas Six-minute walk distance is not related to quality of life in patients with non-cystic fibrosis bronchiectasis

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    Patrícia Santos Jacques

    2012-06-01

    Full Text Available OBJETIVO: Avaliar o desempenho físico de pacientes com bronquiectasias não fibrocísticas no teste de caminhada de seis minutos (TC6 e investigar sua associação com a qualidade de vida (QV. Secundariamente, analisar a associação entre a distância percorrida no TC6 (DTC6 com achados clínicos e espirométricos para se identificar preditores para esse desempenho. MÉTODOS: Estudo transversal envolvendo pacientes com bronquiectasias não fibrocísticas, com idade > 18 anos, pelo menos um sintoma respiratório por > 2 anos e VEF1 OBJECTIVE: To evaluate physical performance on the six-minute walk test (6MWT in patients with non-cystic fibrosis bronchiectasis and to investigate its relationship with quality of life (QoL. To identify predictors of exercise performance, we also investigated whether six-minute walk distance (6MWD is associated with clinical and spirometric findings. METHODS: This was a cross-sectional study involving patients with non-cystic fibrosis bronchiectasis (age, > 18 years, with at least one respiratory symptom for > 2 years and an FEV1 < 70% of predicted. Patients underwent clinical evaluation, pulmonary function tests, the 6MWT, and QoL assessment with the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36. RESULTS: We included 70 patients (48 females. Mean age was 54.5 ± 17.7 years, and mean FEV1 was 44.9 ± 14.5% of predicted. The patients were divided into two groups: 6MWD-low (6MWD below the predicted lower limit; n = 23; and 6MWD-norm (normal 6MWD; n = 47. The following variables were significantly lower in the 6MWD-low group than in the 6MWD-norm group: age; age at diagnosis of bronchiectasis; proportion of former smokers; body mass index (BMI; FEV1% of predicted; and MEP% of predicted. There were no significant differences in the SF-36 scores between the groups. In the logistic regression model, lower age and lower BMI were significantly associated with lower 6MWD. CONCLUSIONS: In this sample, there

  9. Efeitos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos e na remoção de secreção de pacientes com bronquiectasia Effects of ELTGOL and Flutter VRP1® on the dynamic and static pulmonary volumes and on the secretion clearance of patients with bronchiectasis

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    Fernando S. Guimarães

    2012-04-01

    Full Text Available Contextualização: Embora a fisioterapia respiratória seja considerada fundamental para o tratamento de pacientes hipersecretivos, há poucas evidências acerca de seus efeitos fisiológicos e terapêuticos em indivíduos com bronquiectasia. Objetivos: Avaliar os efeitos fisiológicos imediatos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos em pacientes com bronquiectasia e, secundariamente, determinar o efeito dessas técnicas na remoção de secreção brônquica. Métodos: Participaram do estudo pacientes com diagnóstico clínico e radiológico de bronquiectasia. Os pacientes foram submetidos a três intervenções de forma randomizada e com um intervalo (washout de uma semana entre elas. Inicialmente os pacientes inalaram dois jatos de 100µcg de salbutamol. Após 5 minutos de tosse iniciais e após 5 minutos de tosse que sucederam o protocolo controle e as intervenções (ELTGOL e Flutter®, os pacientes realizaram as avaliações dos volumes pulmonares dinâmicos e estáticos por meio da espirometria e pletismografia corporal. A secreção expectorada foi coletada durante as intervenções e durante a segunda série de tosse, sendo quantificada por meio de seu peso seco. Resultados: Foram avaliados dez pacientes, dois do sexo masculino e oito do sexo feminino (média de idade de 55,9±18,1 anos. Após a utilização do Flutter® e da ELTGOL, observou-se diminuição significativa do volume residual (VR, da capacidade residual funcional (CRF e da CPT (pBackground: Although respiratory physical therapy is considered fundamental in the treatment of hypersecretive patients, there is little evidence of its physiological and therapeutic effects in bronchiectasis patients. Objective: To evaluate the acute physiological effects of ELTGOL and Flutter VRP1® in dynamic and static lung volumes in patients with bronchiectasis and, secondarily, to study the effect of these techniques in sputum elimination. Methods: Patients

  10. 探讨垂体后叶素联合酚妥拉明治疗支气管扩张咯血的疗效%To Explore the Effect of Pituitrin Combined With Phentolamine in the Treatment of Bronchiectasis With Hemoptysis by Posterior Pituitary

    Institute of Scientific and Technical Information of China (English)

    王丽敏

    2015-01-01

    Objective Pituitrin combined with phentolamine in the treatment of bronchiectasis with hemoptysis by exploring pituitary after effect. Methods In our hospital were randomly selected for bronchiectasis hemoptysis 80 patients were divided into observation group and control group. Pituitary were used during the two groups of patients treated leaf after intravenous infusion. The observation group patients in the drug combined with phentolamine. The incidence of the two groups were compared the therapeutic effect and adverse reaction. Results The therapeutic effect of observation group was better than that of control group (P<0.05). Conclusion Patients were treated during the use of pituitrin and phentolamine administration, reduce the incidence of adverse reactions of patients, improve the treatment effect, recommended clinical application.%目的:探究垂体后叶素联合酚妥拉明治疗支气管扩张咯血的效果。方法随机抽取我院支气管扩张喀血患者80例平均分为观察组与对照组。两组患者接受治疗期间均使用垂体后叶素静脉输液。观察组患者在此药物基础上加用酚妥拉明。对比两组患者的治疗效果以及不良反应发生情况。结果观察组治疗效果明显优于对照组(P<0.05)。结论患者接受治疗期间使用垂体叶素联合酚妥拉明用药,减少患者不良反应发生率,提高了治疗效果。

  11. Long Term Effect of the Treatment Method of Strengthening Vital Qi, Transforming Phlegm and Clearing Heat for 46 Cases of Bronchiectasis%扶正化痰清热法治疗支气管扩张症46例远期疗效观察

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    黄海茵; 彭晓敏; 宋花玲; 吴银根; 杨佩兰; 汤杰; 郎莹莹; 张谊; 宋文宝; 姚亮; 王振伟; 潘夏萍

    2012-01-01

    目的 观察扶正化痰清热法对支气管扩张稳定期(支扩)的远期疗效.方法 将来自3个中心的100例支扩患者随机分为对照组和中药组各50例,对照组予西医常规治疗(主要为胸部体位引流法,若遇急性加重可短期应用抗生素),中药组予西医常规治疗加中药支扩稳定方辨证加减.观察两组患者治疗前及治疗开始1年后急性加重次数、肺功能[包括用力肺活量(FVC)、第一秒用力呼气容积(FEV1)],并根据圣乔治问卷(SGRQ)进行生活质量评分.结果 治疗后中药组患者急性加重次数较前明显减少(P<0.01),而对照组患者急性加重次数无明显变化(P>0.05),两组急性加重次数变化率差异有统计学意义(P<0.01).治疗后,中药组SGRQ症状、活动、心理及总分较治疗前均明显减少(P<0.05或P<0.01),而对照组治疗后各项评分改善均不明显(P>0.05);治疗后中药组与对照组比较,除症状评分外,其他3项评分差异均有统计学意义(P<0.01);治疗前后评分差值比较,两组活动评分、症状、心理及总分差异均有统计学意义(P<0.05或P<0.01).治疗后,对照组FVC、FVC%、FEV1、FEV1%均明显降低(P<0.05),中药组FVC%下降(P<0.05).中药组治疗后FEV1值趋于稳定,与对照组比较差异有统计学意义(P<0.05),中药组治疗前后FEV1、FEV1%变化率与对照组比较差异有统计学意义(P<0.01).结论 根据扶正化痰清热法辨证施治,能减少支扩患者年急性加重次数,提高生活质量,减缓肺功能FEV1的下降,提高远期疗效,且安全性良好.%Objective To observe the long term effect of strengthening vital qi, transforming phlegm and clearing heat for stable bronchiectasis. Methods One hundred cases of stable bronchiectasis from three clinical centers were randomized into the control group and Chinese medication group, with 50 in each. The control group was treated with conventional western medication

  12. The derivation and validation of a scoring system for identifying patients with bronchiectasis at risk of exacerbations%支气管扩张症急性加重风险分层评分系统的构建及验证

    Institute of Scientific and Technical Information of China (English)

    李杰; 焦瑞; 温林芳; 杜小兵; 高杨; 李晓云; 陈勇; 武元星; 刘双

    2016-01-01

    Objective:To create and verify a scoring system for identifying patients with bronchiectasis at risk of exacerbations.Methods Derivation of the scoring system used data from a retrospective cohort study enrolling 228 patients with bronchiectasis.Multivariable logistic regression analysis was performed to identify independent predictors associated with exacerbations.β-coefficients derived from the independent predictors in our logistic regression model were applied to create a scoring system (Total score was 8).The scoring system was then validated in a prospective cohort enrolling 334 patients with bronchiectasis.Results The derivation study showed that age ≥ 60 years (OR =2.583,95% CI:1.188-5.613),BMI < 18.5 kg/m2 (OR =2.991,95% CI:1.112-8.042),high medical research council dyspnea score (OR =7.905,95% CI:4.288-8.309),Pseudomonas aeruginosa colonization (OR =3.227,95% CI:1.041-9.004),the lobes involved on high-resolution computed tomography ≥3 (OR =3.179,95% CI:1.449 ~ 6.976),prior intensive care unit admissions (OR =2.499,95% CI:1.301-4.801),and FEV1 < 50% predicted (OR =2.497,95% CI:1.421-5.080) were the independent predictors associated with exacerbations.The scoring system predicted exacerbations with an area under the receiver operator characteristic curve (AUC) of 0.79 (95% confidence interval,0.74-0.84).In the validation cohorts,the total score ranged 0 to 6.There was significant difference in exacerbation frequency and quality of life between patients classified as low (0-2),intermediate (3-4),and high (5-8) risks by the scoring system (P < 0.05).A higher score was associated with higher risk of exacerbations and poorer quality of life.Conclusion Our scoring system was an efficient clinical predictive tool to identify patients with bronchiectasis at risk of exacerbations.It may be useful for early prevention of bronchiectasis exacerbations and for proper management.%目的:建立并验证针对支气管扩张症急性加重

  13. Avaliação da resposta clínica ao uso de antibióticos por via oral e via inalatória em pacientes portadores de bronquiectasias Evaluation of clinical response in the antibiotics use by inhalatory and oral ways in patientes with bronchiectasis

    Directory of Open Access Journals (Sweden)

    L. S. de Lima

    1999-07-01

    microbial load present in the sputum. METHOD. To evaluate the bronchiectasis patients with pictures of infectious exacerbations response to the treatment with antibiotic by oral way (roxitromicin 300mg/day for 21 days and in cases of failure of this schema the use of antibiotic by inhalatory way (gentamicin 80mg/2 times day for 21 days, 28 patients were evaluated a special ambulatory, some signs and respiratory symptoms according to the "Cotes modified scale" (sputum, cough, bronchospasm and dyspnea. STATISTICAL ANALYSIS. We used: Kappa concordant test and McNemar test for discordation in the evaluation of the degree of signal and respiratory symptoms, Wilcoxon test for periods without infection, Fisher test for the collaterals effects presented, G of Cochran test for the personal history analysis. RESULTS. The personal history did not influence the evolution of bronchopulmonary infeccion, the evaluated signs and symptoms had significant improvement except dyspnea that stayed the same in 80% of the cases. DISCUSSION. The group that had used the antibiotics schema by inhalatory way after oral scheme failure had a significant longer period without bronchopulmonary infection but with superior collateral effects without clinicals repercussions. CONCLUSION. The use of inhalatory antibiotic in infectious exacerbations in patients with bronchiectasis was better than the oral way.

  14. Efficacy and safety of piperacillin-tazobactam in treatment of bronchiectasis with Pseudomonas aeruginosa infections%哌拉西林/他唑巴坦治疗支气管扩张合并铜绿假单胞菌感染的疗效与安全性评价

    Institute of Scientific and Technical Information of China (English)

    刘学东; 魏风芹; 栾念旭; 魏东

    2013-01-01

    目的 评价哌拉西林/他唑巴坦静脉注射对支气管扩张合并铜绿假单胞菌感染患者的临床疗效和安全性.方法 确诊为支气管扩张合并铜绿假单胞菌感染患者106例,随机分为哌拉西林/他唑巴坦(治疗组)56例和哌拉西林/舒巴坦(对照组)50例;治疗组给予哌拉西林/他唑巴坦以2.5g,2次/d静脉滴注,对照组给予哌拉西林/舒巴坦3.0g,2次/d静脉滴注;疗程均为10~14 d,所有入选病例均完成治疗,未因药物不良反应而中止治疗.结果 哌拉西林/他唑巴坦治疗组痊愈21例,显效30例,总有效率为91.07%;对照组痊愈15例,显效25例,总有效率为80.00%;治疗组治疗有效率高于对照组,差异有统计学意义(P<0.05),哌拉西林/他唑巴坦治疗组临床疗效更好,两组不良反应无明显差异.结论 哌拉西林/他唑巴坦静脉注射安全、有效地治疗支气管扩张合并铜绿假单胞菌感染患者,且具有低附加损害的特点,无1例患者出现伪膜性肠炎及二重真菌感染,值得临床推广.%OBJECTIVE To evaluoate the ofticoiy and fatety of pioerallin-tazo baetam in treat ment of brochiectasis with pseudo monas aeruginosa infections. METHODS A total of 106 patients who were diagnosed as bronchiectasis with P. aeruginosa infections were randomly divided into the treatment group with piperacillin-tazobactam (n = 56) and the control group with piperacillin-sulbactam (n = 50). The treatment group was treated with 2. 5 g of piperacillin-tazobactam, twice a day for intravenous infusion, while the control group was treated with 3. 0g of piperacillin-sulbactam, twice a day for intravenous infusion, with the treatment course of 10-14 days. All the participants finished the treatment, and no cases stopped the treatment due to the antibiotics adverse reactions. RESULTS In the treatment group , there were 21 cases cured and 30 cases valid with the total effective rate of 91. 07%; in the control, there were 15

  15. Influência da técnica de pressão expiratória positiva oscilante utilizando pressões expiratórias pré-determinadas na viscosidade e na transportabilidade do escarro em pacientes com bronquiectasia Influence that oscillating positive expiratory pressure using predetermined expiratory pressures has on the viscosity and transportability of sputum in patients with bronchiectasis

    Directory of Open Access Journals (Sweden)

    Ercy Mara Cipulo Ramos

    2009-12-01

    Full Text Available OBJETIVO: Verificar a efetividade da técnica de pressão expiratória positiva oscilante (PEPO utilizando pressões expiratórias pré-determinadas sobre a viscosidade e a transportabilidade do escarro em pacientes com bronquiectasia. MÉTODOS: Foram incluídos no estudo 15 pacientes estáveis com bronquiectasia (7 homens; média de idade = 53 ± 16 anos, submetidos a duas intervenções PEPO consecutivas, com 24 h de intervalo entre si, utilizando pressões expiratórias de 15 cmH2O (P15 e 25 cmH2O (P25. O protocolo consistiu de tosse voluntária; nova expectoração voluntária após 20 min, denominado tempo zero (T0; repouso de 10 min; e utilização da técnica em duas séries de 10 min (S1 e S2 de PEPO em P15 e P25, com intervalo de 10 min entre si. A viscosidade e transportabilidade do escarro foram avaliadas pela viscosimetria, velocidade relativa de transporte no palato de rã, deslocamento em máquina simuladora de tosse e ângulo de adesão. As amostras de escarro foram coletadas em T0, após S1 e após S2. Testes estatísticos específicos foram aplicados de acordo com a distribuição dos dados. RESULTADOS: Houve diminuição significante da viscosidade do escarro após S1 em P15 e após S2 em P25. Não houve diferenças significantes entre todas as amostras para a transportabilidade. CONCLUSÕES: Houve diminuição da viscosidade do escarro quando a PEPO foi realizada em P15 e P25, o que sugere que não seja necessário gerar alta pressão expiratória para obter o resultado desejado.OBJECTIVE: To determine the effectiveness of oscillating positive expiratory pressure (OPEP using predetermined expiratory pressures on the viscosity and transportability of sputum in patients with bronchiectasis. METHODS: The study involved 15 stable patients with bronchiectasis (7 males; mean age = 53 ± 16 years, submitted to two consecutive OPEP interventions, with a 24-h interval between the two, using positive expiratory pressures set at 15 cmH2O

  16. 通支饮对稳定期支气管扩张痰热壅肺证生活质量的影响%Influence of Tongzhi Decotion on Life Quality of Patients with Syndrome of Phlegm -heat Obstructing Lung in Sable Bronchiectasis

    Institute of Scientific and Technical Information of China (English)

    赵杜鹃; 陈秀妹

    2012-01-01

    Objective;The present study was to determine the effects of Tongzhi decotion on life quality of patients with the syndrome of phlegm - heat obstructing lung in stable bronchiectasis. Methods,70 standard patients were included, we conducted parallel - group study to examine the effects of 90 - day treatment with Tongzhi decotion or Ambroxol Hydrochloride tablets. Results , The group with Tongzhi decotion had a significant improvement in total symptom scores, single symptom scores, total response rate and health status compared to the control group. Conclusion: Our findings suggest that Tongzhi decotion can lead to a significant improvement in clinical symptoms and quality life.%目的:观察通支饮治疗稳定期支气管扩张痰热壅肺证的临床疗效.方法:将70例临床稳定期支气管扩张痰热壅肺证病例,随机分为治疗组和对照组,治疗组予中药通支饮治疗,对照组予盐酸氨溴索片口服,共3个月.结果:两组在中医证候积分、单个症状积分、总疗效、圣乔治呼吸问卷积分方面均有显著性(P<0.05).结论:中药通支饮能改善稳定期支气管扩张痰热壅肺证患者的临床症状,同时显著提高患者的生活质量.

  17. Systematic Review of Phentolamine Combined with Pituitrin Versus Phentolamine Alone in The Treatment of Bronchiectasis Hemoptysis%酚妥拉明联合垂体后叶素治疗支气管扩张咯血的系统评价

    Institute of Scientific and Technical Information of China (English)

    龙霞; 王芊入; 徐珽

    2015-01-01

    Objective To systematically review the effectiveness and safety of phentolamine combined with pituitrin versus phentolamine alone in the treatment of bronchiectasis hemoptysis. Methods Databases including PubMed、EMbase, The Cochrane Library ( Issue 11, 2014 ) , CNKI, WanFang Data and VIP were electronically searched from inception to October 2014, for the randomized controlled trials ( RCT ) on phentolamine combined with pituitrin versus phentolamine alone in the treatment of bronchiectasis hemoptysis. Meta-analysis was performed using RevMan 5. 2. 3 software. Results 14 RCTs were included. The results of meta-analysis indicated that phento-lamine combined with pituitrin versus phentolamine alone can significantly increase the efficiency rate [ RR=1. 24, 95% CI(1. 11, 1. 40), P=0. 000 3 ] and the effective rate [ RR=1. 36, 95% CI(1. 16, 1. 60), P=0. 000 1 ] , reduce the incidence of invalid rate [ RR=0. 31, 95% CI(0. 22, 0. 44), P ﹤ 0. 000 01 ] , shorten the hemoptysis stopping or remission time [ MD= -2. 01, 95% CI(-2. 32,-1. 70), P ﹤ 0. 000 01 ];phentolamine combined with pituitrin versus phentolamine alone can not increase the incidence rate of chest tightness [ RR=1. 25, 95% CI(0. 34, 4. 59), P=0. 74 ] and dizziness [ RR=0. 25, 95% CI(0. 03, 2. 18), P=0. 21 ] . Conclusion Phento-lamine combined with pituitrin versus phentolamine alone can significantly increase the rate of efficiency and effectivity, ruduce the in-cidence of invalid rate, shorten the hemoptysis stopping or remission time, and will not increase the incidence rate of chest tightness and dizziness.%目的:系统评价酚妥拉明联合垂体后叶素治疗支气管扩张咯血的疗效及安全性。方法计算机检索PubMed,EMbase,The Cochrane Library(2014年第11期),CNKI,WanFang Data和VIP数据库,截至2014年10月,纳入酚妥拉明联合垂体后叶素对比单用酚妥拉明治疗支气管扩张咯血的相关随机对照试验(RCT),采用RevMan 5.2.3软件

  18. Tuberculosis, bronchiectasis, and infertility: what ailed George Orwell?

    Science.gov (United States)

    Ross, John J

    2005-12-01

    In the last and most productive years of his life, George Orwell struggled with pulmonary tuberculosis, dying at the dawn of the era of chemotherapy. His case history illustrates clinical aspects of tuberculosis with contemporary relevance: the role of poverty in its spread, the limited efficacy of monotherapy, the potential toxicity of treatment, and the prominence of cachexia as a terminal symptom. Orwell's ordeals with collapse therapy may have influenced the portrayal of the tortures of Winston Smith in the novel 1984. I discuss unifying diagnoses for Orwell's respiratory problems and apparent infertility, including tuberculous epididymitis, Young syndrome, immotile cilia syndrome, and cystic fibrosis.

  19. SINUSITIS, BRONCHIECTASIS, AND FLATUS IN A SUMATRAN ORANGUTAN (PONGO ABELII): COULD THIS BE CYSTIC FIBROSIS?

    Science.gov (United States)

    Stringer, Elizabeth; Cossaboon, Cindy; Han, Sushan; Taylor-Cousar, Jennifer L

    2016-03-01

    A 31-yr-old male Sumatran orangutan (Pongo abelii) presented with 14 yr of chronic purulent nasal drainage and cough with intermittent exacerbation of symptoms requiring systemic antibiotic treatment. He was diagnosed with a cystic fibrosis (CF)-like condition. Evaluation consisted of bronchoscopy with bronchoalveolar lavage, culture, and computed tomography scanning of the sinuses and chest. Although the presence of low fecal elastase activity increased the suspicion for a diagnosis of CF, pilocarpine iontophoresis with sweat collection and analysis ("sweat testing") was inconclusive. Medical management included twice-daily nebulization with bronchodilators and alternating month inhaled antibiotics, pancreatic enzyme replacement therapy, and simethicone as needed. Sinopulmonary and gastrointestinal symptoms improved substantially with treatment. Several years later, the animal died acutely of colonic volvulus. Necropsy and histopathology confirmed CF-like lung disease with chronic air sacculitis.

  20. Effectiveness of treatment with high-frequency chest wall oscillation in patients with bronchiectasis

    OpenAIRE

    Nicolini, Antonello; Cardini, Federica; Landucci, Norma; Lanata, Sergio; Ferrari-Bravo, Maura; Barlascini, Cornelius

    2013-01-01

    Background High-frequency airway clearance (HFCWC) assist devices generate either positive or negative trans-respiratory pressure excursions to produce high-frequency, small-volume oscillations in the airways. HFCWC can lead to changes in volume of 15–57 ml and in flow up to 1.6 L/s, which generate minimal coughing to mobilize secretions. The typical treatment lasts 20–30 minutes, and consists of short periods of compression at different frequencies, separated by coughing. The aim of this stu...

  1. The Epidemiology of Chronic Suppurative Lung Disease and Bronchiectasis in Children and Adolescents

    Science.gov (United States)

    McCallum, Gabrielle B.; Binks, Michael J.

    2017-01-01

    In the modern era, the global burden of childhood chronic suppurative lung disease (CSLD) remains poorly captured by the literature. What is clear, however, is that CSLD is essentially a disease of poverty. Disadvantaged children from indigenous and low- and middle-income populations had a substantially higher burden of CSLD, generally infectious in etiology and of a more severe nature, than children in high-income countries. A universal issue was the delay in diagnosis and the inconsistent reporting of clinical features. Importantly, infection-related CSLD is largely preventable. A considerable research and clinical effort is needed to identify modifiable risk factors and socioeconomic determinants of CSLD and provide robust evidence to guide optimal prevention and management strategies. The purpose of this review was to update the international literature on the epidemiology, etiology, and clinical features of pediatric CSLD. PMID:28265556

  2. Kartagener′s syndrome

    Directory of Open Access Journals (Sweden)

    Pradip V Potdar

    2012-01-01

    Full Text Available Bronchiectasis may have varied etiologies. In patients presenting in middle or old age with bronchiectasis, congenital and hereditary causes of bronchiectasis are not commonly encountered. In majority of adult patients, bronchiectasis follows necrotizing infection. We report a case of Kartagener′s syndrome in a female patient diagnosed in her sixties.

  3. Disease: H00892 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00892 Bronchiectasis with or without elevated sweat chloride Bronchiectasis is a condition in which the air...ways are permanently dilated due to recurrent inflammation or infection. In many ca

  4. The Effect of Lt to Rt Shunt Using Veno-veno-arterial Extracorporeal Membrane Oxygenation (ECMO) on Coronary Oxygenation in Lung Transplantation Patients

    Science.gov (United States)

    2016-08-03

    Interstitial Pulmonary Fibrosis ARDS; COPD (Chronic Obstructive Pulmonary Disease); Bronchiectasis; Lymphangioleiomyomatosis; Primary Pulmonary Hypertension; ARDS (Acute Respiratory Distress Syndrome)

  5. Bronquiectasia localizada e multissegmentar: perfil clínico-epidemiológico e resultado do tratamento cirúrgico em 67 casos Localized and multisegmental bronchiectasis: clinical-epidemiological profile and surgical treatment results in 67 cases

    OpenAIRE

    ANTERO GOMES NETO; MARCOS LIMA DE MEDEIROS; JOSÉ MAURO MENDES GIFONI

    2001-01-01

    A bronquiectasia, doença rara no Primeiro Mundo, tem alta prevalência nos países em desenvolvimento. No Brasil, as principais causas são infecções respiratórias, virais ou bacterianas na infância e tuberculose. A antibioticoterapia diminui consideravelmente a morbidade, mas nos pacientes com sintomatologia persistente, a ressecção cirúrgica oferece maior possibilidade de cura ou melhor qualidade de vida. Objetivos: Avaliar prospectivamente o perfil clínico-epidemiológico de pacientes com bron...

  6. 酚妥拉明联合垂体后叶素治疗支气管扩张咯血的疗效观察%Curative Effect of Phentolamine Combined Pituitrin for Bronchiectasis with Hemoptysis

    Institute of Scientific and Technical Information of China (English)

    李强; 徐晓飞; 范洪峰

    2012-01-01

    目的 观察垂体后叶素联合酚妥拉明治疗支气管扩张咯血的疗效.方法 将支气管扩张咯血患者60例随机单盲法分为治疗组和对照组各30例,两组在抗感染治疗基础上,均予止咳对症及恰当的体位引流等,对照组予垂体后叶素持续静脉使用,输液速度维持在0.1 U/(kg·h)水平.治疗组在上述应用垂体后叶素以0.1 U/(kg·h)同时加用酚妥拉明以0.08 mg/min持续静脉滴注.对比两组疗效和不良反应.结果 治疗组总有效率90%(27/30)高于对照组的83%(25/30),差异有统计学意义(x2=8.27,P<0.05).治疗组咯血停止时间平均(3.6±1.3)d,和对照组的(4.7±1.5)d相比亦明显减少,差异有统计学意义(t=3.25,P<0.05).对照组发现有不同程度的副反应,发生率为.46.7%(14/30).治疗组未出现明显的药物不良反应.结论 垂体后叶素和酚妥拉明联用治疗支气管扩张咯血快速高效,不良反应少,值得临床推广.%Objective To study the curative effect of phentolamine combined with pituitrin in the treatment of bronchietasis with hemoptysis. Methods All 60 cases were randomly divided into treatment group (30 cases, treated with phentolamine combined pituitary vasopressin) and control group (30 cases, treated with pituitary vasopressin). The efficiency and adverse effects were observed. Results The total effective rate was 90% (27/30) in the treatment group and were higher than that(83% ,25/ 30) in the control group (Χ2 = 8.27,P<0.05) ;Time to stop bleeding was(3.6 ± 1.3)days in treatment group and (4.7 ± 1.5) days in the control group(t =3. 25, P <0. 05);There was significant difference between them. Conclusion The phentolamine combined pituitary vasopressin,with less adverse reaction,is effective for the bronchodilation with hemoptysis.

  7. Curative effect of pituitary vasopressin combined Phentolamine treatment bronchiectasis with massive hemoptysis%垂体后叶素联合酚妥拉明治疗支气管扩张大咯血的疗效观察

    Institute of Scientific and Technical Information of China (English)

    杨建彪

    2011-01-01

    Objective To study the curative effect and adverse reactions in bronehiectasis with massive hemoptysis.Methods 100 cases were randomly divided into pituitary vasopressin combined phentolamine treatment (treatment group)50 cases and phentolamine treatment(control group)50 cases.The hemptysis condition,hemostatic time and adverse reaction during the administration were detected.Results The total effective rate 94.0%(47/50)in treatment group were hishter than that control group 82.0%(41/50)(χ2=3.845,P<0.05);Time to stop bleeding(3.2 ±1.0)days in treatment group were lower than the control group(5.0±1.4)days(t=2.134,P<0.05);Adverse reactions in treatment group were 10 cases(20.0%),compared with control group 11 patients(22.0%)there were no significant difference(χ2=1.897,P>0.05).Conclusion Pituitary vasopressin combined phentolamine to treatment massive hemoptysis had significant clinical effect with less adverse reaction.%目的 探讨垂体后叶素联合酚妥拉明治疗支气管扩张大咯血的疗效和不良反应.方法 将100例支气管扩张大咯血患者随机分为酚妥拉明联合垂体后叶素序贯静脉泵入组(治疗组)50例和酚妥拉明组(对照组)50例,用药期间密切监测两组患者的咯血变化情况、止血时间及药物的不良反应.结果 治疗组总有效率为94.0%(47/50),高于对照组的82.0%(41/50)(χ2=3.845,P<0.05);治疗组止血时间(3.2±1.0)d,低于对照组的(5.0±1.4)d(t=2.134,P<0.05);治疗组出现不良反应10例(20.0%),与对照组的11例(22.0%)比较,差异无统计学意义(χ2=1.897,P>0.05).结论 垂体后叶素联合酚妥拉明治疗支气管扩张大咯血临床疗效显著,不良反应少.

  8. Curative effect analysis of treating senile bronchiectasis and mucus hypersecretion by Erdosteine%厄多司坦对老年支气管扩张症及黏掖分泌过多的疗效分析

    Institute of Scientific and Technical Information of China (English)

    唐忆贫

    2012-01-01

    目的:探讨厄多司坦治疗患有支气管扩张症及黏液分泌过多的临床效果分析.方法:抽取80例在本院就诊的患有支气管扩张症及黏液分泌过多的老年患者,将其随机分为A、B两组各40例.A组患者采用胸部物理治疗;B组患者在A组基础上采用厄多司坦进行治疗.结果:两组患者在治疗后的VAS评分、MP、MVP、呼吸肌力、用力肺活量、第1秒用力呼气量均较治疗前有明显的改善,差异有统计学意义(P<0.05);B组患者上述指标的改善程度明显高于A组,差异有统计学意义(P<0.05).结论:采用厄多司坦对患有支气管扩张症及黏液分泌过多的老年患者进行治疗的临床效果十分明显,不会造成特殊的严重的并发症和不良反应现象,可以做为今后临床对该类患者进行治疗的首选方法,值得进一步使用和推广.

  9. Bronquiectasias, estudio de 36 pacientes intervenidos en el hospital “Dr. Rafael Ángel Calderón Guardia” Bronchiectasis, Results of Surgical Intervention in 36 Patients

    Directory of Open Access Journals (Sweden)

    José Alberto Mainieri-Hidalgo

    2011-09-01

    Full Text Available Objetivo: Analizar el resultado de la cirugía por bronquiectasias en el Servicio de Cirugía de Tórax del Hospital Calderón Guardia. Método: Se revisaron los expedientes clínicos de 36 pacientes operados, con un total de 44 intervenciones por bronquiectasias. Resultados: Se encontró una prevalencia muy significativa de la enfermedad en mujeres, con una relación de 11 a 1. La etiología fue en 10 casos el antecedente de tuberculosis, en uno el síndrome de Kartagener, y en 25 no se logró establecer. Todos los pacientes tenían antecedente de tos y expectoración mucopurulenta de larga evolución, y 29 habían sido clasificados como asmáticos, aunque 10 no tenían ningún antecedente familiar. La radiografía de tórax fue anormal en todos los casos, mostrando áreas de fibrosis, infiltrados o nódulos, pero no fue útil para establecer el diagnóstico de bronquiectasias, contrario a la TAC, que en todos mostró las lesiones bronquiales. El resultado de la cirugía, contrario a lo esperado, no logró establecer un indicador de éxito, pues pacientes con lesiones muy localizadas continuaron expectorando e infectándose, y otros con patología difusa y bilateral, mejoraron sustancialmente. Conclusiones: La cirugía por bronquiectasias está indicada cuando el tratamiento médico no logra controlar satisfactoriamente los cuadros infecciosos bronquiales. Existe franca posibilidad de mejoría, pero no un indicador de éxito para el procedimiento quirúrgico.Aim: To analyze the results of surgical interventions due to bronchiectasias at the Thoracic Surgery Department of the Dr. R. A. Calderón Guardia Hospital. Methods: In order to review the results of 44 surgical interventions due to bronchiectasias at the Thoracic Surgery Department of Calderón Guardia Hospital, the clinical files of 36 patients were examined. Results: A significant prevalence of the disease in female patients was observed, in proportion of 11 to 1. In most cases, causal determination was not established due to lack of documentation in the clinical history annotations. In 10 cases there was a history of tuberculosis. One case corresponded to a Kartagener syndrome as causal elements of the disease. All patients exhibited a history of coughing and long evolution of mucopurulent expectoration. Twenty nive patients were catalogued as asthmatic even though ten of them lacked a family history of such disease. Thoracic X-Ray results were abnormal in all cases, presenting fibrosis, infiltrates or nodules, but were insufficient to provide diagnosis. CT scan results, in comparison, were effective in demonstrating the presence of bronchiectasias in all cases. Surgical intervention was decided upon when the affected area was localized in a specific part of the lung or when infectious processes became frequent and too difficult to control with medical treatment. Conclusion: Results of surgery demonstrated that there was no effective indicator for success rates; some patients with particularly localized lesions continued to suffer from infection and expectoration, while others with diffuse and bilateral pathology demonstrated substantial improvements.

  10. Nursing experience of 1 cases of diabetes in the treatment of hemoptysis caused by bronchiectasis%支气管扩张咯血伴糖尿病1例行介入治疗的护理体会

    Institute of Scientific and Technical Information of China (English)

    栾颖; 杨大志; 刘国卓

    2014-01-01

    Bronchiectasia is due to the chronic suppurative inflammation and fibrosis of the trachea and the surrounding lung tissue that lead to deformation of the trachea and sustained expansion through the bronchial wall muscles and elastic tissue damage. Main symptoms of the Bronchiectasia include chronic cough, cough a lot of phlegm and recurrent hemoptysis. interventional therapy is the application of self dingle technology via femoral artery selective bronchial artery intubation, and determine the bleeding and blood vessels, local application of hemostatic agents, with gelatin sponge line so as to achieve the aim of the bleeding artery embolism. We admitted a bronchiectasia hemoptysis patient with diabetes on november 17, 2013, and the interventional treatment for the bleeding has been performed on november 18. the patient recovered well after treatment and care, now report as follows.%支气管扩张是由于气管及周围肺组织慢性化脓性炎症和纤维化,使支气管壁的肌肉和弹性组织破坏导致气管变形及持久扩张,主要症状有慢性咳嗽、咳大量脓痰和反复咯血。介入治疗是应用赛尔丁格儿技术经股动脉选择性支气管动脉插管,确定出血部位和血管,局部应用止血剂,再用明胶海绵行动脉栓塞从而达到止血的目的[1]。我院2013年11月17日收治一位支气管扩张咯血伴糖尿病的患者,该患者于18日行介入治疗止血,经治疗和护理后患者恢复良好,现报告如下。

  11. 鼻出血误诊为支气管扩张、上消化道出血(附8例分析)%Epistaxis Misdiagnosed as Bronchiectasis or Upper Gastrointestinal Bleeding: An analysis on 8 Cases

    Institute of Scientific and Technical Information of China (English)

    朱冠龙; 韩朝晖; 陈荣; 黄思海

    2008-01-01

    回顾性分析8例误诊的鼻出血患者的临床资料,寻找引起误诊的原因.结果 出血部位隐蔽、症状不典型、体检及病史采集不详细、分析欠周到是误诊主因.对患者应仔细采集和分析病史,并行详细体格检查,必要时行鼻内窥镜、支气管镜、胃镜、CT等辅助检查,有助于减少和避免误诊.

  12. Evolving practice: X-linked agammaglobulinemia and lung transplantation.

    Science.gov (United States)

    Barnes, S; Kotecha, S; Douglass, J A; Paul, E; Hore-Lacy, F; Hore-Lacey, F; Stirling, R; Snell, G I; Westall, G P

    2015-04-01

    X-linked agammaglobulinemia (XLA) is a rare primary humoral immunodeficiency syndrome characterized by agammaglobulinemia, recurrent infections and bronchiectasis. Despite the association with end-stage bronchiectasis, the literature on XLA and lung transplantation is extremely limited. We report a series of 6 XLA patients with bronchiectasis who underwent lung transplantation. Short-term outcomes were excellent however long-term outcomes were disappointing with a high incidence of pulmonary sepsis and chronic lung allograft dysfunction (CLAD).

  13. Extraintestinal manifestations of inflammatory bowel disease: epidemiology, diagnosis, and management

    DEFF Research Database (Denmark)

    Larsen, Signe; Bendtzen, Klaus; Nielsen, Ole Haagen

    2010-01-01

    ', 'bronchiectasis', 'bronchitis', 'cutaneous manifestations', 'erythema nodosum', 'extraintestinal manifestations', 'hyperhomocysteinemia', 'infliximab', 'iridocyclitis', 'lung disease', 'ocular manifestations', 'osteomalacia', 'pancreatitis', 'primary sclerosing cholangitis', 'renal stones', 'sulfasalazine...

  14. 20 CFR Appendix 3 to Part 220 - Railroad Retirement Board Occupational Disability Standards

    Science.gov (United States)

    2010-04-01

    ... 2 S Distant 12.8 1 S Oral Cavity/Pharyngeal: Local 76.2 2 S Regional 40.9 2 S Distant 18.7 1 S... which are described below. A. Imaging studies—These studies can include MRI, CAT scan, myelogram, or... Bronchiectasis demonstrated Recommended. Chest CAT scan Bronchiectasis demonstrated Recommended....

  15. A influência de bactérias patogênicas na transportabilidade do escarro e na qualidade de vida de portadores de bronquiectasia The influence of pathogenic bacteria on transportability of sputum and quality of life among patients with bronchiectasis

    OpenAIRE

    RC Zanchet; AC Magalhães; AF Correia; Feijó,G

    2006-01-01

    INTRODUÇÃO: A qualidade de vida pode estar relacionada com o estado clínico do paciente, com o nível de infecção e com o microorganismo que o infecta. OBJETIVO: Analisar o perfil bacteriológico do escarro de pacientes com bronquiectasia e avaliar seu efeito no transporte in vitro e na qualidade de vida dos pacientes. MÉTODOS: Pacientes com bronquiectasia foram avaliados por questionários de qualidade de vida, cultura bacteriana e transporte in vitro do escarro. RESULTADOS: Foram incluídos 19 ...

  16. Analysis of Clinical Efficacy of Pituitrin and Phentolamine in Treatment of Patients with Bronchiectasis with Hemoptysis%垂体后叶素联合酚妥拉明治疗支气管扩张合并咯血患者的临床疗效分析

    Institute of Scientific and Technical Information of China (English)

    纪铜镇; 尹纪祯; 安宁

    2014-01-01

    目的:探讨采用垂体后叶素联合酚妥拉明治疗支气管扩张合并咯血患者的临床疗效。方法按照数字随机表法将我院近年来收治的86例支气管扩张合并咯血患者平均分为观察和对照2组,所有患者均给予镇咳、化痰、抗感染、对症支持等常规治疗,观察组患者在此基础上应用垂体后叶素联合酚妥拉明治疗,比较两组患者的治疗总有效率。结果观察组患者治疗总有效率为95.3%,显著高于对照组患者的76.7%,组间差异可见统计学意义(P<0.05)。观察组患者用药过程中2例出现腹痛,2例出现胸闷,患者均能耐受,未影响治疗。结论垂体后叶素与酚妥拉明联合应用是一种治疗支气管扩张合并咯血患者的理想方法,应在临床大力推广使用。%Objective To investigate the clinical effect of pituitrin combined with phentolamine in the treatment of patients with bronchiectasis with hemoptysis. Methods In our hospital in recent years in 86 cases of bronchiectasis with hemoptysis were divided into observation and control group 2 in accordance with the digital random table method, all patients were given the antitussive, expectorant, anti infection, symptomatic and supportive treatment, observation of pituitary application group patients based on Pituitrin and phentolamine treatment, comparison two groups of patients and the total efifciency. Results In the observation group, the total effective rate was 95.3%, signiifcantly higher than the control group of 76.7%patients, the differences between groups showed statistical signiifcance(P<0.05). Observation group patients in 2 patients had abdominal pain, 2 cases of chest tightness, patients can be well tolerated, did not affect the treatment. Conclusion Pituitrin and phentolamine combined application is an ideal method for the treatment of patients with bronchiectasis with hemoptysis, should be popularized in clinic.

  17. The relationship between cystic fibrosis and bronchiectasis and its research implications in Chinese%囊性肺纤维化与支气管扩张症的关系以及在中国人群中的研究价值

    Institute of Scientific and Technical Information of China (English)

    李惠萍; 何国钧

    2004-01-01

    囊性肺纤维化(cystic fibrosis,CF)是一种在欧洲和北美高加索人种中高发的常染色体隐性遗传性疾病。美国高加索人的发病率为1/3300;CF基因携带者达到1/33(3%)。在非洲人及亚裔人群,CF被认为极为“罕见”。亚裔美国人CF发病率为1/32400(0.30‰);CF基因携带者为1/90。因此,CF的研究主要集中在欧美国家的高加索人群中。

  18. 成年非CF支气管扩张患者呼吸道菌群与临床测量指标间的相关性分析%Analysis of correlation between clinical measures of adult patients with non-CF bronchiectasis and airway microbiota composition

    Institute of Scientific and Technical Information of China (English)

    杨畅; 冯燕; 王新卫; 刘新年

    2015-01-01

    目的 采用非培养法研究成年非CF支气管扩张患者呼吸道菌群特点及与临床指标间的相关性.方法 入选23例非CF支气管扩张患者(实验组,n=23)和15例健康志愿者(对照组,n=15),记录所有研究对象的一般临床指标,并收集痰液标本和支气管肺泡灌洗液标本.对痰液标本进行传统细菌培养和16S rDNA测序,对支气管肺泡灌洗液标本行16S rDNA测序.分析受试者呼吸道菌群特点及其与患者临床指标间的关系.结果 痰标本焦磷酸测序检测到铜绿假单胞菌和流感嗜血杆菌的频率要高于标准培养法报告的频率.两组均显示,痰标本比BAL标本提供了更多的测序序列.细菌群落多样性与FEV1呈正的线性相关.Mantel检验显示,菌落组成的相似性和FEV1间有显著相关性,全部菌种与LCS、痰中性粒细胞数和性别间有显著相关性.结论 非CF支气管扩张患者下呼吸道菌群的多样性与临床检测指标间有相关性.

  19. Developing Optimal Parameters for Hyperpolarized Noble Gas and Inert Fluorinated Gas MRI of Lung Disorders

    Science.gov (United States)

    2016-04-19

    Lung Transplant; Lung Resection; Lung Cancer; Asthma; Cystic Fibrosis; Chronic Obstructive Pulmonary Disease; Emphysema; Mesothelioma; Asbestosis; Pulmonary Embolism; Interstitial Lung Disease; Pulmonary Fibrosis; Bronchiectasis; Seasonal Allergies; Cold Virus; Lung Infection; Pulmonary Hypertension; Pulmonary Dysplasia; Obstructive Sleep Apnea

  20. Coinfection of Pulmonary Hydatid Cyst and Aspergilloma : Case Report and Systematic Review

    NARCIS (Netherlands)

    Aliyali, M.; Badali, H.; Shokohi, T.; Moazeni, M.; Nosrati, A.; Godazandeh, G.; Dolatabadi, S.; Nabili, M.

    2016-01-01

    Aspergilloma infection consists of a mass of fungal hyphae, inflammatory cells, fibrin, mucus, and tissue debris and can colonize lung cavities due to underlying diseases such as tuberculosis, sarcoidosis, bronchiectasis, cavitary lung cancer, neoplasms, ankylosing spondylitis, bronchial cysts, and

  1. Etiologies, Investigations and Outcomes of Patients Presenting With Hemoptysis

    Science.gov (United States)

    2016-09-22

    Hemoptysis; Haemoptysis; Lung Disease; Pneumonia; Tuberculosis; Bronchiectasis; Respiratory Tract Infections; Respiratory Tract Diseases; Bronchitis; Mycobacterium Infections; Bronchial Disease; Pulmonary Hemorrhage; Signs and Symptoms; Signs and Symptoms, Respiratory; Pathologic Processes; Mycosis; Hemorrhage; Lung Cancer; Pulmonary Embolism; Arteriovenous Fistula

  2. Geç Tanı Konulan Nadir Bir Kartagener Sendromu Olgusu

    OpenAIRE

    NURAL, Serkan; HALICI, Bilal; Günay, Ersin

    2015-01-01

    Kartagener’s syndrome is an autosomal recessive inherited, primary ciliary dyskinesia syndrome, which is characterized by situs inversus, bronchiectasis, and chronic sinusitis. Symptoms usually emerge during childhood period. Ninety percent of cases are diagnosed prior to 15 years of age. In patients with Kartagener’s syndrome, respiratory infections are encountered such as chronic coughing, mucoid sputum, and recurrent pneumonia attacks. Bronchiectasis develops as a result of recurrent lung ...

  3. Assessment of CF lung disease using motion corrected PROPELLER MRI: a comparison with CT

    Energy Technology Data Exchange (ETDEWEB)

    Ciet, Pierluigi [General Hospital Ca' Foncello, Radiology Department, Treviso (Italy); Sophia Children' s Hospital, Pediatric Pulmonology Erasmus MC, Rotterdam (Netherlands); Erasmus MC, Radiology, Rotterdam (Netherlands); Serra, Goffredo; Catalano, Carlo [University of Rome ' ' Sapienza' ' , Radiology, Rome (Italy); Bertolo, Silvia; Morana, Giovanni [General Hospital Ca' Foncello, Radiology Department, Treviso (Italy); Spronk, Sandra [Erasmus MC, Radiology, Rotterdam (Netherlands); Erasmus MC, Epidemiology, Rotterdam (Netherlands); Ros, Mirco [Ca' Foncello Hospital, Pediatrics, Treviso (Italy); Fraioli, Francesco [University College London (UCL), Institute of Nuclear Medicine, London (United Kingdom); Quattrucci, Serena [University of Rome Sapienza, Pediatrics, Rome (Italy); Assael, M.B. [Azienda Ospedaliera di Verona, Verona CF Center, Verona (Italy); Pomerri, Fabio [University of Padova, Department of Medicine-DIMED, Padova (Italy); Tiddens, Harm A.W.M. [Sophia Children' s Hospital, Pediatric Pulmonology Erasmus MC, Rotterdam (Netherlands); Erasmus MC, Radiology, Rotterdam (Netherlands)

    2016-03-15

    To date, PROPELLER MRI, a breathing-motion-insensitive technique, has not been assessed for cystic fibrosis (CF) lung disease. We compared this technique to CT for assessing CF lung disease in children and adults. Thirty-eight stable CF patients (median 21 years, range 6-51 years, 22 female) underwent MRI and CT on the same day. Study protocol included respiratory-triggered PROPELLER MRI and volumetric CT end-inspiratory and -expiratory acquisitions. Two observers scored the images using the CF-MRI and CF-CT systems. Scores were compared with intra-class correlation coefficient (ICC) and Bland-Altman plots. The sensitivity and specificity of MRI versus CT were calculated. MRI sensitivity for detecting severe CF bronchiectasis was 0.33 (CI 0.09-0.57), while specificity was 100 % (CI 0.88-1). ICCs for bronchiectasis and trapped air were as follows: MRI-bronchiectasis (0.79); CT-bronchiectasis (0.85); MRI-trapped air (0.51); CT-trapped air (0.87). Bland-Altman plots showed an MRI tendency to overestimate the severity of bronchiectasis in mild CF disease and underestimate bronchiectasis in severe disease. Motion correction in PROPELLER MRI does not improve assessment of CF lung disease compared to CT. However, the good inter- and intra-observer agreement and the high specificity suggest that MRI might play a role in the short-term follow-up of CF lung disease (i.e. pulmonary exacerbations). (orig.)

  4. Progressing features of atypical mycobacterial infection in the lung on conventional and high resolution CT (HRCT) images

    Energy Technology Data Exchange (ETDEWEB)

    Tanaka, Daizo; Niwatsukino, Hiroshi; Nakajo, Masayuki [Kagoshima Univ. (Japan). Faculty of Medicine; Oyama, Takao

    2001-10-01

    The aim of this study was to clarify the localization of abnormalities within secondary pulmonary lobules and the changes in follow-up studies of pulmonary atypical mycobacterial infection (AMI) by conventional and high-resolution computed tomography (HRCT). Forty-six patients (16 men and 30 women; 43-84 years) with pulmonary AMI (M. intracellulare 36; M. avium 10) in the lung were examined by conventional and HRCT. In peripheral zones, all patients had the nodule located in the terminal or lobular bronchiole, and most of the patients also had nodules accompanied with a wedge-shaped or linear shadow connected with the pleura. In the follow-up scans, new centrilobular nodules appeared in other segments, and consolidation or ground-glass pattern appeared newly and was preceded by nodules. Bronchiectasis became more severe in five of 38 follow-up patients. The common HRCT findings of AMI were centrilobular, peribronchovascular nodules, bronchiectasis, consolidation, and pleural thickening/adhesion. The nodules frequently connected with the pleura. The initial and follow-up studies suggest that the disease may begin in the terminal bronchiole or as preexisting bronchiectasis and spread transbronchially along the draining bronchus or towards the pleura to produce lesions such as new nodules, cavities, consolidation, pleuritis, and bronchiectasis, or more severe bronchiectasis. (author)

  5. Kartagener Syndrome: A Rare Genetic Disorder

    Directory of Open Access Journals (Sweden)

    Kunjan Shakya

    2009-01-01

    Full Text Available Kartagener Syndrome is a rare autosomal recessive disorder consisting of triad of sinusitis, bronchiectasis and situs inversus with dextrocardia. It is the subset of disorder called primary ciliary dyskinesia in which the cilia have abnormal structure and/or function resulting in multisystem diseases of various severity. Clinical manifestations include lifelong, chronic upper and lower respiratory tract diseases secondary to ineffective mucociliary clearance. Early diagnosis and management of chest infections can prevent irreversible damage to lungs and prevent potential lifelong complications. This case report is on a patient who presented with long standing history of sinusitis, bronchiectasis and on examination situs inversus with dextrocardia. Key Words:bronchiectasis, dextrocardia, kartagener syndrome, primary ciliary dyskinesia, situs inversus

  6. Allergic bronchopulmonary aspergillosis in an adult with Kartagener syndrome.

    Science.gov (United States)

    Sehgal, Inderpaul Singh; Dhooria, Sahajal; Bal, Amanjit; Agarwal, Ritesh

    2015-08-06

    Allergic bronchopulmonary aspergillosis (ABPA) is a pulmonary disorder resulting from immune responses directed against inhaled Aspergillus fumigatus antigens. It manifests with poorly controlled asthma, fleeting pulmonary opacities and structural lung damage in the form of bronchiectasis. Initially defined in individuals suffering from bronchial asthma and cystic fibrosis, it has also been described in patients with other structural lung disorders such as chronic obstructive pulmonary disease, pulmonary tuberculosis, idiopathic bronchiectasis and others. Kartagener syndrome is a manifestation of primary ciliary dyskinesia characterised by the presence of dextrocardia, bronchiectasis and chronic sinusitis. We report a case of ABPA in an adult suffering from Kartagener syndrome. We also performed a systematic review of the literature on the association between Kartagener syndrome and ABPA.

  7. CT in childhood allergic bronchopulmonary aspergillosis

    Energy Technology Data Exchange (ETDEWEB)

    Shah, A.; Bhagat, R.; Panchal, N. (Delhi Univ. (India). Vallabhabhai Patel Chest Inst.); Pant, C.S. (Institute of Nuclear Medicine and Allied Sciences, Delhi (India). Imaging Div.)

    1992-06-01

    CT of the thorax done during acute severe asthma in two paediatric patients demonstrated central bronchiectasis, a sine qua non for the diagnosis of allergic bronchopulmonary aspergillosis. Bronchography, regarded as the gold standard, was done subsequently on recovery. A comparative segmental analysis revealed that CT was able to identify immediately 24 of 27 segments which showed central bronchiectasis on bronchography. Early diagnosis with the aid of CT enabled immediate intervention which may have helped to prevent further lung damage in the paediatric patients. (orig.).

  8. Kartagener syndrome: a rare genetic disorder.

    Science.gov (United States)

    Shakya, K

    2009-01-01

    Kartagener Syndrome is a rare autosomal recessive disorder consisting of triad of sinusitis, bronchiectasis and situs inversus with dextrocardia. It is the subset of disorder called primary ciliary dyskinesia in which the cilia have abnormal structure and/or function resulting in multisystem diseases of various severity. Clinical manifestations include lifelong, chronic upper and lower respiratory tract diseases secondary to ineffective mucociliary clearance. Early diagnosis and management of chest infections can prevent irreversible damage to lungs and prevent potential lifelong complications. This case report is on a patient who presented with long standing history of sinusitis, bronchiectasis and on examination situs inversus with dextrocardia.

  9. Progressive increase in cavitation with the evolution of fungus ball: A clue to the diagnosis of chronic necrotizing pulmonary aspergillosis

    OpenAIRE

    Prasad R.; Garg Sanjay

    2009-01-01

    Chronic necrotizing pulmonary aspergillosis (CNPA) is an uncommon pulmonary infection seen in the patients with chronic obstructive pulmonary disease, bronchiectasis, pneumoconiosis, diabetes mellitus, alcoholism, poor nutrition or low dose corticosteroid therapy. Here, we are presenting a case of CNPA with diabetes mellitus that was misdiagnosed as pulmonary tuberculosis.

  10. Progressive increase in cavitation with the evolution of fungus ball: A clue to the diagnosis of chronic necrotizing pulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    Prasad R

    2009-01-01

    Full Text Available Chronic necrotizing pulmonary aspergillosis (CNPA is an uncommon pulmonary infection seen in the patients with chronic obstructive pulmonary disease, bronchiectasis, pneumoconiosis, diabetes mellitus, alcoholism, poor nutrition or low dose corticosteroid therapy. Here, we are presenting a case of CNPA with diabetes mellitus that was misdiagnosed as pulmonary tuberculosis.

  11. HRCT features in a 5-year-old child with follicular bronchiolitis

    Energy Technology Data Exchange (ETDEWEB)

    Reittner, P.; Fotter, R.; Lindbichler, F.; Tillich, M. [Department of Radiology, Karl-Franzens University and University Hospital Graz, Auenbruggerplatz 9, A-8036 Graz (Austria); Varga, E.M.; Zach, M. [Department of Pediatrics, Karl-Franzens University and University Hospital Graz, Graz (Austria); Popper, H. [Department of Pathology, Karl-Franzens University and University Hospital Graz, Graz (Austria)

    1997-11-01

    High-resolution CT (HRCT) is the most sensitive radiographic method to image small airways disease. We discuss the HRCT features of follicular bronchiolitis in a 5-year-old boy and correlate them with the histopathological findings. The changes described include centrilobular nodules, bronchiectasis and bronchiolectasis, branching opacities and areas of reduced lung attenuation. (orig.) With 2 figs., 5 refs.

  12. Tracheobronchomegaly (Mounier-Kuhn syndrome) - case report and review of the literature; Traqueobroncomegalia (sindrome de Mounier-Kuhn) - relato de caso e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Nobrega, Bruno Barcelos da; Figueiredo, Sizenildo da Silva; Ribeiro, Ronei Edmar; Teixeira, Kim-Ir-Sem Santos [Goias Univ., Goiania, GO (Brazil). Hospital das Clinicas. Dept. de Diagnostico por Imagem e Anatomia Patologica]. E-mail: brunoradiol@hotmail.com; Cavalcante, Luciana Porto [Goias Univ., Goiania, GO (Brazil). Faculdade de Medicina

    2002-06-01

    Tracheobronchomegaly is a rare condition of uncertain etiology characterized by marked dilatation of the trachea and main bronchi, bronchiectasis and recurrent respiratory tract infections. Clinical presentation is nonspecific and the diagnosis is usually made by imaging methods. We report a case of a patient with tracheobronchomegaly. Diagnosis was confirmed by plain X-ray films and computed tomography of the chest. (author)

  13. Health Risk Assessment of Consuming Deer from Aberdeen Proving Ground, Maryland. Report and Appendices A-D

    Science.gov (United States)

    1995-05-01

    acrocyanosis, angina pectoris, hypertension, myocardia! infarction, mesenicric thrombosis, systemic occlusive arterial disease, bronchiectasis, and...Columbia, MS. pp. 13-24. Borgono, J.M.; Vincent , P.; Venturino, H.; Infante, A. 1977. Arsenic in the drinking water of the city of Antofagast

  14. Successful treatment of Bacillus cereus infection with ciprofloxacin.

    OpenAIRE

    Gascoigne, A.D.; Richards, J.; Gould, K.; Gibson, G. J.

    1991-01-01

    Bacillus cereus is rarely a pulmonary pathogen but may cause pneumonia in immunocompromised patients. A patient with bronchiectasis and no recognisable immunodeficiency had this organism isolated during two infective exacerbations, once from respiratory secretions and once by blood culture. Ciprofloxacin treatment was effective on both occasions.

  15. Posterior Circulation Stroke After Bronchial Artery Embolization. A Rare but Serious Complication

    Energy Technology Data Exchange (ETDEWEB)

    Laborda, Alicia [Universidad de Zaragoza, Departamento de Pediatria, Radiologia y Medicina Fisica, Grupo de Investigacion en Tecnicas Minimamente Invasivas, GITMI (Spain); Tejero, Carlos [Hospital Clinico Universitario Lozano Blesa, Servicio de Neurologia (Spain); Fredes, Arturo, E-mail: fredesarturo@gmail.com [Universidad de Zaragoza, Hospital Quiron, Departamento de Pediatria, Radiologia y Medicina Fisica, Grupo de Investigacion en Tecnicas Minimamente Invasivas, GITMI (Spain); Cebrian, Luis; Guelbenzu, Santiago; Gregorio, Miguel Angel de, E-mail: mgregori@unizar.es [Universidad de Zaragoza, Departamento de Pediatria, Radiologia y Medicina Fisica, Grupo de Investigacion en Tecnicas Minimamente Invasivas, GITMI (Spain)

    2013-06-15

    Bronchial artery embolization (BAE) is the treatment of choice for massive hemoptysis with rare complications that generally are mild and transient. There are few references in the medical literature with acute cerebral embolization as a complication of BAE. We report a case of intracranial posterior territory infarctions as a complication BAE in a patient with hemoptysis due to bronchiectasis.

  16. Mucus hypersecretion in the airway

    Institute of Scientific and Technical Information of China (English)

    WANG Ke; WEN Fu-qiang; XU Dan

    2008-01-01

    @@ Mucus hypersecretion is a distinguishing feature of Chronic intlammation diseases,such as asthma,1chronic bronchitis.2 bronchiectasis3 and cystic fibrosis.4Mucus hypersecretion leads to impairment of mucociliary clearance,abnormal bacterial plantation,mucus plug in the airway,and dysfunction of gas exchange.5

  17. Adenocarcinoma of the Cervix Uteri and Endometrium Combined With the Kartagener Syndrome on FDG PET/CT.

    Science.gov (United States)

    Zhang, Yin; Chen, Yue; Huang, Zhanwen; Zhou, Fan

    2015-11-01

    A 45-year-old woman with pathologically confirmed adenocarcinoma of the cervix uteri and endometrium underwent FDG PET/CT for staging. No metastasis was found. However, the images revealed bronchiectasis, sinusitis, and situs inversus totalis, which are the triad of Kartagener syndrome.

  18. Linkage analysis localises a Kartagener syndrome gene to a 3.5 cM region on chromosome 15q24-25

    NARCIS (Netherlands)

    Geremek, M; Zietkiewicz, E; Diehl, S R; Alizadeh, B Z; Wijmenga, C; Witt, M

    2006-01-01

    BACKGROUND: Primary ciliary dyskinesia (PCD) is a genetic disorder caused by ciliary immotility/dysmotility due to ultrastructural defects of the cilia. Kartagener syndrome (KS), a subtype of PCD, is characterised by situs inversus accompanying the typical PCD symptoms of bronchiectasis and chronic

  19. A case of Kartagener syndrome with rhinolalia clausa.

    Science.gov (United States)

    Raoufi, Mohammed; Sator, Hicham; Lahma, Jawad; El Ayoubi, Ali; Nitassi, Sophia; Oujilal, Abdelilah; Benbouzid, Mohammed Anas; Essakalli, Leila; Elouazzani, Hanane; Rhorfi, Ismail Abderrahmane; Abid, Ahmed

    2016-01-01

    Kartagener syndrome is an autosomal recessive genetic ciliary disorder comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. It's the one of primary ciliary dyskinesia disorders with manifestations present from childhood. Most patients of PCD have situs inversus. We present a case of 18 year-old women with recurrent lower and upper respiratory tracts infections, and rhinolalia clausa.

  20. [Kartagener sindrome (primary ciliary dyskinesia). Report of a case and literature review].

    Science.gov (United States)

    Pino Rivero, V; Pardo Romero, G; Iglesias González, R J; Rodríguez Carmona, M; del Castillo Beneyto, F

    2007-01-01

    Kartagener syndrome (a clinical variant of primary ciliary dyskinesia) is a recessive autossomical disease characterized by the triad of chronic sinusitis, bronchiectasis and situs inversus with dextrocardia. We report one case described in a 8 years old boy who besides presented a seromucous otitis and bronchitis of repetition. Finally we performed a short bibliographic review at respect of this uncommon pathology.

  1. Linkage analysis localises a Kartagener syndrome gene to a 3.5 cM region on chromosome 15q24-25.

    NARCIS (Netherlands)

    Geremek, M.; Zietkiewicz, E.; Diehl, S.R.; Alizadeh, B.Z.; Wijmenga, C.; Witt, M.

    2006-01-01

    Background: Primary ciliary dyskinesia (PCD) is a genetic disorder caused by ciliary immotility/dysmotility due to ultrastructural defects of the cilia. Kartagener syndrome (KS), a subtype of PCD, is characterised by situs inversus accompanying the typical PCD symptoms of bronchiectasis and chronic

  2. [Epidural anesthesia and analgesia in the perioperative treatment of a patient with Kartagener syndrome].

    Science.gov (United States)

    Errando, C L; Sifre, C; López-Alarcón, D

    1998-12-01

    Kartagener's syndrome is an inherited disease characterized by a triad of symptoms--bronchiectasis, situs inversus and sinusitis--and is classified as an immotile cilia syndrome. Patients may experience specific airway problems when undergoing anesthesia for surgical procedures. We report the case of a woman with Kartagener's syndrome who underwent surgery under epidural anesthesia with postoperative epidural analgesia, both techniques proving successful.

  3. Linkage analysis localises a Kartagener syndrome gene to a 3.5 cM region on chromosome 15q24-25.

    NARCIS (Netherlands)

    Geremek, M.; Zietkiewicz, E.; Diehl, S.R.; Alizadeh, B.Z.; Wijmenga, C.; Witt, M.

    2006-01-01

    BACKGROUND: Primary ciliary dyskinesia (PCD) is a genetic disorder caused by ciliary immotility/dysmotility due to ultrastructural defects of the cilia. Kartagener syndrome (KS), a subtype of PCD, is characterised by situs inversus accompanying the typical PCD symptoms of bronchiectasis and chronic

  4. Surgical management of bilateral bronchiectases: results in 29 patients.

    Science.gov (United States)

    Aghajanzadeh, Manucher; Sarshad, Ali; Amani, Hosin; Alavy, Ali

    2006-06-01

    Bronchiectasis is a major cause of morbidity and mortality in developing countries. Staged bilateral segmental resection of the lungs is performed in selected patients. Our experience of surgical removal of 87 bilateral bronchiectases in 29 patients during an 11-year period was reviewed retrospectively. High-resolution computed tomography was performed preoperatively in all patients to locate the anatomic sites of bronchiectasis. The mortality and morbidity of the surgical procedure, clinical symptoms, age distribution, etiology, bacteriology, and operative procedures were analyzed. There were 22 males (76%) and 7 females (24%), aged 5 to 60 years, with a mean age of 30 years. Complications developed in 11 patients (38%); atelectasia was the most common (14%). There was one hospital death. Clinical symptoms disappeared in 19 (66%) patients, improved in 5 (17%), and were unchanged in 4 (14%). Staged bilateral resection for bronchiectases can be performed at any age with acceptable morbidity and mortality.

  5. A case of Kartagener′s syndrome: Importance of early diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Sanjay Gupta

    2013-01-01

    Full Text Available Kartagener′s syndrome is a very rare congenital malformation comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. Primary ciliary dyskinesia is a genetic disorder with manifestations present from early life and this distinguishes it from acquired mucociliary disorders. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus are having Kartagener syndrome. We present a case of 12 year old boy with sinusitis, situs inversus and bronchiectasis. The correct diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

  6. A case of Kartagener′s syndrome: Importance of early diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Sanjay Gupta

    2012-01-01

    Full Text Available Kartagener′s syndrome is a very rare congenital malformation comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. Primary ciliary dyskinesia is a genetic disorder with manifestations present from early life and this distinguishes it from acquired mucociliary disorders. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus are having Kartagener syndrome. We present a case of 12 year old boy with sinusitis, situs inversus and bronchiectasis. The correct diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

  7. [Primary ciliary dyskinesia: clinical and genetic aspects].

    Science.gov (United States)

    D'Auria, E; Palazzo, S; Argirò, S; El, Oksha S; Riva, E

    2012-01-01

    Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous disease, characterized by ciliary disfunction and impaired mucociliary clearance, resulting in a range of clinical manifestations such as chronic bronchitis, bronchiectasis, chronic rhino-sinusitis, chronic otitis media, situs viscerum inversus in almost 40-50% of cases and male infertility. The triad situs viscerum inversus, bronchiectasis and sinusitis is known as Kartagener syndrome. Up to now little is known about genetic, diagnostic and therapeutic aspects of primary motile ciliary diseases in children: for this reason, diagnosis is generally delayed and almost all treatments for PCD are not based on randomized studies but extrapolated from cystic fibrosis guidelines. The aim of this review is to propose to pediatricians a summary of current clinical and diagnostic evidence to obtain better knoledwge of this condition. The earlier diagnosis and the right treatment are both crucial to improve the prognosis of PCD.

  8. Primary ciliary dyskinesia: clinical and genetic aspects

    Directory of Open Access Journals (Sweden)

    E. D’Auria

    2012-06-01

    Full Text Available Primary ciliary dyskinesia (PCD is a rare, genetically heterogeneous disease, characterized by ciliary disfunction and impaired mucociliary clearance, resulting in a range of clinical manifestations such as chronic bronchitis, bronchiectasis, chronic rhino-sinusitis, chronic otitis media, situs viscerum inversus in almost 40-50% of cases and male infertility. The triad situs viscerum inversus, bronchiectasis and sinusitis is known as Kartagener syndrome. Up to now little is known about genetic, diagnostic and therapeutic aspects of primary motile ciliary diseases in children: for this reason, diagnosis is generally delayed and almost all treatments for PCD are not based on randomized studies but extrapolated from cystic fibrosis guidelines. The aim of this review is to propose to pediatricians a summary of current clinical and diagnostic evidence to obtain better knoledwge of this condition. The earlier diagnosis and the right treatment are both crucial to improve the prognosis of PCD.

  9. Multiple bronchoceles in a non-asthmatic patient with allergic bronchopulmonary aspergillosis.

    Science.gov (United States)

    Amin, Muhammad Umar; Mahmood, Rabia

    2008-09-01

    Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction due to a fungus, Aspergillus fumigatus. It is typically seen in patients with long-standing asthma. Our patient was a non-asthmatic 18 years old male who presented with chronic cough for 2 years. Peripheral blood eosinophilia and elevated scrum IgE were observed. His x-ray chest revealed v-shaped opacity in the left upper lobe close to the hilum. High resolution computed tomographic scan of the chest revealed multiple dilated bronchi filled with mucous (bronchoceles) and central bronchiectasis (CB) involving main segmental bronchi. Central bronchiectasis (CB) was typical of ABPA but bronchocele formation was a rare manifestation of the disease. The patient was managed with oral prednisolone and was relieved of his symptoms. Occurrence of ABPA in non-asthmatics is very rare and deserves reporting.

  10. Asian perspective in surgery: thoracic surgery in Turkey

    OpenAIRE

    Turna, Akif

    2016-01-01

    Turkey with a population of 78 million is located between Asia and Europe geographically and culturally. There are 577 active pure thoracic surgeon and 37 thoracic surgery teaching units. Thoracic surgeons usually deal with lung cancer patients due to relatively higher rate of tobacco usage as well as inflammatory diseases such as pulmonary hydatid disease, bronchiectasis and empyema. Minimally invasive thoracic surgery has been a new approach which is being adapted by increasingly more surge...

  11. Primary ciliary dyskinesia: Kartagener syndrome in a family with a novel DNAH5 gene mutation and variable phenotypes

    OpenAIRE

    2015-01-01

    Background: Primary ciliary dyskinesia is a genetically heterogeneous autosomal recessive disorder with variable clinical manifestations, including chronic rhinosinusitis, otitis media, bronchitis, pneumonia, bronchiectasis, situs inversus totalis, reduced fertility in female patients and male infertility. The condition occurs as a result of abnormal ciliary structure and function. It is presented in early life with an estimated incidence of approximately 1/16,000–20,000. About 50% of the aff...

  12. Kroniske lungeforandringer hos børn med langvarig produktiv hoste

    DEFF Research Database (Denmark)

    Foghsgaard, Jakob; Nir, Marta; Marthin, June K;

    2009-01-01

    A productive (sounding) cough is always abnormal, and suppurative lung disease should be considered. A chronic suppurative cough may be associated with the destruction of the bronchial wall (bronchiectasis). The most commonly identifiable cause of suppurative cough is cystic fibrosis. This article......, which is accompamied by an illustrative CT-scan, describes two paediatric cases of cystic fibrosis and primary ciliary dyskinesia and suggests appropriate avenues of clinical investigation, when chronic suppurative cough presents in children. Udgivelsesdato: 2009-Jan-26...

  13. Mounier Kuhn syndrome in adult: a case report

    Directory of Open Access Journals (Sweden)

    Nabanita Deka

    2015-04-01

    Full Text Available Mounier-Kuhn syndrome or tracheobronchomegaly is a rare congenital abnormality of the trachea and main bronchi characterized by marked cystic dilatation of the tracheobronchial tree, associated with tracheal diverticulosis, bronchiectasis and recurrent lower respiratory tract infections. We report a case of 22 years adult male with history of recurrent lower respiratory tract infection. [Int J Res Med Sci 2015; 3(4.000: 987-989

  14. Acute chemical pneumonitis caused by nitric acid inhalation: case report

    Energy Technology Data Exchange (ETDEWEB)

    Choe, Hyung Shim; Lee, In Jae; Ko, Eun Young; Lee, Jae Young; Kim, Hyun Beom; Hwang, Dae Hyun; Lee, Kwan Seop; Lee, Yul; Bae, Sang Hoon [Hallym University Sacred Heart Hospital, Anyang (Korea, Republic of)

    2003-06-01

    Chemical pneumonitis induced by nitric acid inhalation is a rare clinical condition. The previously reported radiologic findings of this disease include acute permeability pulmonary edema, delayed bronchiolitis obliterans, and bronchiectasis. In very few published rare radiologic reports has this disease manifested as acute alveolar injury; we report a case of acute chemical pneumonitis induced by nitric acid inhalation which at radiography manifested as bilateral perihilar consolidation and ground-glass attenuation, suggesting acute alveolar injury.

  15. Effects of Techa River Radiation Contamination on the Reproductive Function of Residents

    Science.gov (United States)

    2006-11-01

    general somatic diseases such as diseases of the respiratory organs (bronchiectasis), diabetes , hepatic, and renal insufficiency. The method of choice...the diagnosis of CRS in some of the patients was infantilism , i.e., retard- ed and insufficient sexual development. As is indicated in a previous...legs and left forearm after a railway disaster. The two remaining women, twins born in 1944, were diagnosed with infantilism , which is evidently the

  16. Evaluation of the safety of high-frequency chest wall oscillation (HFCWO) therapy in blunt thoracic trauma patients

    OpenAIRE

    Becker Brian; Ney Arthur L; Palmer Cassandra A; Anderson Casandra A; Schaffel Steven D; Quickel Robert R

    2008-01-01

    Abstract Background Airway clearance is frequently needed by patients suffering from blunt chest wall trauma. High Frequency Chest Wall Oscillation (HFCWO) has been shown to be effective in helping to clear secretions from the lungs of patients with cystic fibrosis, bronchiectasis, asthma, primary ciliary dyskinesia, emphysema, COPD, and many others. Chest wall trauma patients are at increased risk for development of pulmonary complications related to airway clearance. These patients frequent...

  17. Investigating the Respiratory Health of Deployed Military Personnel

    Science.gov (United States)

    2011-10-01

    emphysema (1% vs. 1%) and asthma (1% vs. 1%) observed. The authors suggested that specifi c exposures rather than deployment may be a determinant of...of chronic pulmonary diagnoses to include asthma , emphysema , chronic bronchitis, chronic obstructive pulmonary disease, bronchiectasis, sarcoidosis...mod- est correlation in self-reported symptoms of asthma and bron- chitis in a cohort of 1,560 veterans based on oil fi re proximity, but the fi

  18. Exophiala Pneumonia Presenting with a Cough Productive of Black Sputum

    OpenAIRE

    2015-01-01

    Exophiala species are black, yeast-like molds that can cause subcutaneous cysts as well as disseminated disease. Isolated pneumonia due to Exophiala species is extremely uncommon. We report a case of isolated Exophiala pneumonia in a patient with bronchiectasis who presented with worsening dyspnea and a cough productive of black sputum. The production of black sputum, known as melanoptysis, is an uncommon physical finding with a limited differential diagnosis. To our knowledge, this is the fi...

  19. Biological Effects of Short, High-Level Exposure to Gases: Sulfur Dioxide.

    Science.gov (United States)

    1980-05-01

    days, starting with 250 ppm on day 1. 24 𔄁 doses of sulfur dioxide, with inflammation, bronchiectasis, I bronchiolectasis, and alveolitis , but, unlike...dioxide inhalation was discontinued. The second subject had a previous history of allergic response to sulfur-containing medications. When she was exposed...to sulfur dioxide from a drainpipe near her home, extreme skin reactions resulted. Clinical tests revealed allergic response to a variety of sulfur

  20. Right bochdalek hernia associated with kartagener syndrome: developmental and clinical observations.

    Science.gov (United States)

    Romeo, Carmelo; Turiaco, Nunzio; Gitto, Eloisa; Borruto, Francesca Astra; Santoro, Giuseppe

    2013-06-01

    We present a novel case of the association of right-sided Bochdalek hernia, a diaphragmatic life-threatening malformation, and Kartagener syndrome, which is characterized by congenital bronchiectasis, chronic sinusitis, and situs inversus. The developmental and clinical findings are discussed. When an association of diaphragmatic hernia with situs viscerum inversus is encountered, physicians should be mindful of the possibility of Kartagener syndrome because this condition could significantly affect the morbidity of the patient.

  1. Evaluation of residual functional lung volume on Tc-99m DTPA aerosol ventilation and Tc-99m MAA perfusion scintigraphy in primary ciliary dyskinesia (Kartagener syndrome).

    Science.gov (United States)

    Chen, Yu-Wen; Chang, Chin-Chuan; Lai, Yung-Chuang; Lu, Chia-Ying; Dai, Zen-Kong

    2008-12-01

    Kartagener syndrome is diagnosed as sinusitis, bronchitis (bronchiectasis), and situs inversus by the clinical features. It is a subclass of primary ciliary dyskinesia (PCD) disease. A 12-year-old girl who had frequent upper and lower airway infections since birth, which was confirmed as Kartagener syndrome by HRCT imaging. We present the residual functional lung volume and mucociliary clearance findings seen on Tc-99m DTPA aerosol ventilation and Tc-99m MAA perfusion scintigraphy.

  2. Bronchiolitis as a feature of kartagener syndrome: a case report.

    Science.gov (United States)

    Ozkaya, Sevket; Sahin, Unal; Gumus, Aziz; Taşç, Filiz; Cnarka, Halit; Yavuz, Asiye

    2011-01-01

    Kartagener syndrome (KS), also known as immotile cilia syndrome or as a primary ciliary dyskinesia, is characterized by the triad of situs inversus, bronchiectasis, and chronic pansinusitis. A few studies reported that diffuse bronchiolitis might be one of the characteristic features of the lung in KS. We aimed to present the radiologic characteristics of KS, including diffuse bronchiolitis, sinus aplasia, and situs inversus totalis in a single case.

  3. KARTAGENER’S SYNDROME : A CASE SERIES STUDY

    Directory of Open Access Journals (Sweden)

    Prakash

    2015-08-01

    Full Text Available Kartagener’s Syndrome is a rare Autosomal recessive disorder presents as a triad of Sinusitis, Bronchiectasis and Situs inversus. Chronic Rhino sinusitis is a common condition seen is ENT OPD routinely. Of the several causes of Sinusitis, Kartagener’s is rare entity and can remain undetected. This case series is to emphasize the diagnosis of the disease and alert the patient about it.

  4. CT findings of swyer-james-macleod syndrome in adults: are there any different findings with aging

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Mi Jin; Kim, Joung Sook; Kim, Ji Young; Kim, Soung Hee; Jeong, Myeong Ja; Kim, Soo Hyun; Kim, Jae Hyung [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Bae, Sang Jin [National Police Hospital, Seoul (Korea, Republic of); Woo, Jeong Joo [Eulji Hospital, Eulji University College of Medicine, Daejeon (Korea, Republic of)

    2007-02-15

    We wanted to evaluate whether there is any different finding on CT with aging for the patients suffering with adult Swyer-James-MacLeod Syndrome (SJMS). We included 11 patients (7 males and 4 females) who underwent chest CT scan among 18 patients who were suspected of suffering with SJMS on chest radiographs. The range of age was from 28 to 85 years (mean: 58.5). We evaluated the diameter of both the main pulmonary artery (MPA) with its ratio, and the diameter of the pulmonary trunk (PT) to evaluate the possibility of pulmonary arterial hypertension, and the presence or absence of bronchiectasis. We also evaluated the relationships between these findings and aging. SJMS affected the left lung in 10 of 11 patients. The mean diameter of the main pulmonary artery of the normal lung was 2.5 cm and it was 1.6 cm in the involved site. The mean ratio of the normal MPA diameter to the involved one was 1.6 and this did not correlate with age ({rho} > 0.1). The mean diameter of the pulmonary trunk was 2.8 cm and this increased with age ({rho} < 0.05). There was bronchiectasis in 6 of 11 patients, and the ratio of bronchiectasis did not correlate with age ({rho} > 0.5). SJMS absolutely affected the left lung much more than right lung. All the patients demonstrated about 1.6 times the compensatory hypertrophy of MPA of the normal lung compared with that of the affected lung on chest CT, which was irrespective of age. The presence or absence of bronchiectasis has no correlation with age.

  5. Cystic fibrosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Si Hyun; Lee, Hyun Ju; Kim, Ji Hye; Park, Chol Heui [Gachon Medical School, Inchon (Korea, Republic of)

    2002-12-01

    Cystic fibrosis is an autosomal recessive genetic disease. Among Caucasians, it is the most common cause of pulmonary insufficiency during the first three decades of life. The prevalence of cystic fibrosis varies according to ethnic origin: it is common among Caucasians but rare among Asians. We report a case in which cystic fibrosis with bronchiectasis and hyperaeration was revealed by high-resolution CT, and mutation of the cystic fibrosis conductance transmembrane regulator gene (CFTR) by DNA analysis.

  6. Persistent and Recurrent Bacterial Bronchitis—A Paradigm Shift in Our Understanding of Chronic Respiratory Disease

    Science.gov (United States)

    Ishak, Alya; Everard, Mark L.

    2017-01-01

    The recent recognition that the conducting airways are not “sterile” and that they have their own dynamic microbiome, together with the rapid advances in our understanding of microbial biofilms and their roles in the causation of respiratory diseases (such as chronic bronchitis, sinusitis, and chronic otitis media), permit us to update the “vicious circle” hypothesis of the causation of bronchiectasis. This proposes that chronic inflammation driven by persistent bacterial bronchitis (PBB) causes damage to both the epithelium, resulting in impaired mucociliary clearance, and to the airway wall, which eventually manifests as bronchiectasis. The link between a “chronic bronchitis” and a persistence of bacterial pathogens, such as non-typable Haemophilus influenzae, was first made more than 100 years ago, and its probable role in the causation of bronchiectasis was proposed soon afterward. The recognition that the “usual suspects” are adept at forming biofilms and hence are able to persist and dominate the normal dynamically changing “healthy microbiome” of the conducting airways provides an explanation for the chronic colonization of the bronchi and for the associated chronic neutrophil-dominated inflammation characteristic of a PBB. Understanding the complex interaction between the host and the microbial communities of the conducting airways in health and disease will be a key component in optimizing pulmonary health in the future. PMID:28261574

  7. Fibrotic idiopathic interstitial pneumonias: HRCT findings that predict mortality

    Energy Technology Data Exchange (ETDEWEB)

    Edey, Anthony J.; Hansell, David M. [The Royal Brompton Hospital, Department of Radiology, London (United Kingdom); Devaraj, Anand A. [St. George' s NHS Foundation Trust, Department of Radiology, Tooting (United Kingdom); Barker, Robert P. [Frimley Park Hosptal, Department of Radiology, Frimley, Surrey (United Kingdom); Nicholson, Andrew G. [The Royal Brompton Hospital, Department of Histopathology, London (United Kingdom); Wells, Athol U. [The Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom)

    2011-08-15

    The study aims were to identify CT features that predict outcome of fibrotic idiopathic interstitial pneumonia (IIP) when information from lung biopsy data is unavailable. HRCTs of 146 consecutive patients presenting with fibrotic IIP were studied. Visual estimates were made of the extent of abnormal lung and proportional contribution of fine and coarse reticulation, microcystic (cysts {<=}4 mm) and macrocystic honeycombing. A score for severity of traction bronchiectasis was also assigned. Using death as our primary outcome measure, variables were analysed using the Cox proportional hazards model. CT features predictive of a worse outcome were coarse reticulation, microcystic and macrocystic honeycombing, as well as overall extent of lung abnormality (p < 0.001). Importantly, increased severity of traction bronchiectasis, corrected for extent of parenchymal abnormality, was predictive of poor prognosis regardless of the background pattern of abnormal lung (HR = 1.04, CI = 1.03-1.06, p < 0.001). On bivariate Cox analysis microcystic honeycombing was a more powerful determinant of a poor prognosis than macrocystic honeycombing. In fibrotic IIPs we have shown that increasingly severe traction bronchiectasis is indicative of higher mortality irrespective of the HRCT pattern and extent of disease. Extent of microcystic honeycombing is a more powerful determinant of outcome than macrocystic honeycombing. (orig.)

  8. The Mounier-Kuhn syndrome

    Directory of Open Access Journals (Sweden)

    Milić Rade

    2010-01-01

    Full Text Available Background. The Mounier-Kuhn syndrome (MKS or tracheobronchomegaly (TBM is a rare condition of unknown frequency, up to now about 100 cases have been reported. It presents by marked dilatation of the trachea and major bronchi, recurrent respiratory infections and consecutive bronchiectasis and scars in lung parenchyme. Sometimes enlargement of transversal colon may be present. Diagnosis is usually made radiologically. Cases report. We reviewed two patients 77 and 72 years old with typical clinical presentation and enlarged upper airways, in whom diagnosis of MKS was established by chest multislice computed tomography (MSCT. Transversal diameter of trachea was 30 mm in the first patient and 33 mm in the other one. Complications of syndrome (tracheal diverticulosis in the first patient, and pulmonary fibrosis, bulous emphysema and bronchiectasis in both patients also were seen. Lung function tests showed mixed ventilation disorder, and disturbance of respiratory gases values in arterial blood samples. Conclusion. The Mounier-Kuhn syndrome is rare disorder, although diagnosis is often missed. Clinical presentation is similar to chronic obstructive pulmonary disease or bronchiectasis. Computed tomography is gold standard for diagnosis. Therapy is presumely supportive.

  9. Computed Tomography Findings of Pulmonary Mycobacterium simiae Infection

    Science.gov (United States)

    Baghizadeh, Ayeh; Farnia, Poopak

    2017-01-01

    Nontuberculous mycobacterial (NTM) pulmonary infections can be quite similar to tuberculosis, both clinically and radiologically. However, the treatment protocol is not similar. Mycobacterium simiae is a rare cause of NTM pulmonary infection. Herein, we aimed to evaluate and compare the computed tomography (CT) scan findings of M. simiae infection in lungs. For this reason, thirty-four patients (n = 34) with M. simiae lung infection were retrospectively evaluated. Diagnosis was confirmed by American Thoracic Society (ATS) guidelines and CT scans were reviewed in both lung and mediastinal windows. The average age of patients was 63 ± 14.54 years and 52.9% were male. The majority of patients had cough (91.2%) and sputum production (76.5%). Clinically, 41.2% of patients had previous history of TB (14/34), 38.2% had cardiac diseases (13/34), and 35.3% had diabetes mellitus (12/34). The most common CT findings in our study were nodular lesions (100%) and bronchiectasis (85.29%). Regarding the severity, grade I bronchiectasis was the most prevalent. Other prominent findings were tree-in-bud sign (88.2%), consolidation (52.94%), and lobar fibrosis and volume loss (67.6%). There was no significant zonal distribution of findings. In conclusion, nodular lesions and bronchiectasis are the most frequent features in CT scan of M. simiae pulmonary infection.

  10. What did we learn from two decades of chest computed tomography in cystic fibrosis?

    Energy Technology Data Exchange (ETDEWEB)

    Tiddens, Harm A.W.M. [Erasmus MC-Sophia Children' s Hospital, Department of Pediatric Pulmonology, Rotterdam (Netherlands); Erasmus MC, Department of Radiology, Rotterdam (Netherlands); Rosenow, Tim [Erasmus MC-Sophia Children' s Hospital, Department of Pediatric Pulmonology, Rotterdam (Netherlands); The University of Western Australia, Telethon Institute for Child Health Research, Perth (Australia); The University of Western Australia, School of Paediatrics and Child Health Research, Perth (Australia)

    2014-12-15

    Despite our current treatment, many cystic fibrosis (CF) patients still show progressive bronchiectasis and small airways disease. Adequate detection and monitoring of progression of these structural abnormalities is needed to personalize treatment to the severity of CF lung disease of the patient. Chest computed tomography (CT) is the gold standard to diagnose and monitor bronchiectasis. Many studies have been done to validate the role of chest CT in CF and to improve the protocols. From these studies it became clear that for correct interpretation of the severity of bronchiectasis and small airways disease standardization of lung volume for the inspiratory and expiratory CT scan acquisition is needed. The risk related to the radiation exposure of a chest CT scan every second year is considered low. Automated and quantitative image analysis systems are developed to improve the reliability and sensitivity of assessments of structural lung changes in CF, particularly in early life. In this paper an overview is given of the lessons learned from two decades of monitoring CF lung disease using chest CT. (orig.)

  11. Kartagener syndrome

    Directory of Open Access Journals (Sweden)

    Nedaa Skeik

    2011-01-01

    Full Text Available Nedaa Skeik1–3, Fadi I Jabr41Mayo Clinic, Rochester, MN, USA; 2Dartmouth Medical School, Hannover, NH, USA; 3New York Medical College, New York, NY, USA; 4Horizon Medical Center, Hospital Medicine, Dickson, TN, USAAbstract: Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by pseudomonal infection. Situs inversus can be seen in about 50% of cases. Diagnosis can be made by tests to prove impaired cilia function, biopsy, and genetic studies. Treatment is supportive. In severe cases, the prognosis can be fatal if bilateral lung transplantation is delayed. We present a case of a 66-year-old woman with chronic recurrent upper respiratory infections, pseudomonal pneumonia, and chronic bronchiectasis who presented with acute respiratory failure. She was diagnosed with Kartagener syndrome based on her clinical presentation and genetic studies. She expired on ventilator with refractory respiratory and multiorgan failure.Keywords: chronic obstructive pulmonary disease, bronchiectasis, immotile cilia syndrome, situs inversus

  12. Drug-sensitive tuberculosis, multidrug-resistant tuberculosis, and nontuberculous mycobacterial pulmonary disease in nonAIDS adults: comparisons of thin-section CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Myung Jin; Lee, Kyung Soo; Kim, Tae Sung; Kim, Sung Mok [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea); Koh, Won-Jung; Kwon, O Jung [Sungkyunkwan University School of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Seoul (Korea); Kang, Eun Young [Korea University Guro Hospital, Department of Diagnostic Radiology, Korea University College of Medicine, Seoul (Korea); Kim, Seonwoo [Sungkyunkwan University School of Medicine, Biostatistics Unit of the Samsung Biomedical Research Institute, Samsung Medical Center, Seoul (Korea)

    2006-09-15

    The aim of this work was to compare thin-section CT (TSCT) findings of drug-sensitive (DS) tuberculosis (TB), multidrug-resistant (MDR) TB, and nontuberculous mycobacterial (NTM) pulmonary disease in nonAIDS adults. During 2003, 216 (113 DS TB, 35 MDR TB, and 68 NTM) patients with smear-positive sputum for acid-fast bacilli (AFB), and who were subsequently confirmed to have mycobacterial pulmonary disease, underwent thoracic TSCT. The frequency of lung lesion patterns on TSCT and patients' demographic data were compared. The commonest TSCT findings were tree-in-bud opacities and nodules. On a per-person basis, significant differences were found in the frequency of multiple cavities and bronchiectasis (P<0.001, chi-square test and multiple logistic regression analysis). Multiple cavities were more frequent in MDR TB than in the other two groups and extensive bronchiectasis in NTM disease (multiple logistic regression analysis). Patients with MDR TB were younger than those with DS TB or NTM disease (P<0.001, multiple logistic regression analysis). Previous tuberculosis treatment history was significantly more frequent in patients with MDR TB or NTM disease (P<0.001, chi-square test and multiple logistic regression analysis). In patients with positive sputum AFB, multiple cavities, young age, and previous tuberculosis treatment history imply MDR TB, whereas extensive bronchiectasis, old age, and previous tuberculosis treatment history NTM disease. (orig.)

  13. Chronic hypersensitivity pneumonitis: high resolution computed tomography patterns and pulmonary function indices as prognostic determinants

    Energy Technology Data Exchange (ETDEWEB)

    Walsh, Simon L.F.; Devaraj, Anand; Hansell, David M. [Royal Brompton Hospital, Department of Radiology, London (United Kingdom); Sverzellati, Nicola [University of Parma, Department of Clinical Sciences, Section of Radiology, Parma (Italy); Wells, Athol U. [Royal Brompton Hospital, Interstitial Lung Diseases Unit, London (United Kingdom)

    2012-08-15

    To investigate high resolution computed tomography (HRCT) and pulmonary function indices (PFTs) for determining prognosis in patients with chronic fibrotic hypersensitivity pneumonitis (CHP). Case records, PFTs (FEV{sub 1}, FVC and DLco) and HRCTs of ninety-two patients with chronic hypersensitivity pneumonitis were evaluated. HRCT studies were scored by two observers for total disease extent, ground-glass opacification, fine and coarse reticulation, microcystic and macrocystic honeycombing, centrilobular emphysema and consolidation. Traction bronchiectasis within each pattern was graded. Using Cox proportional hazards regression models the prognostic strength of individual HRCT patterns and pulmonary function test variables were determined. There were forty two deaths during the study period. Increasing severity of traction bronchiectasis was the strongest predictor of mortality (HR 1.10, P < 0.001, 95%CI 1.04-1.16). Increasing global interstitial disease extent (HR 1.02, P = 0.02, 95%CI 1.00-1.03), microcystic honeycombing (HR 1.09, P = 0.019, 95%CI 1.01-1.17) and macrocystic honeycombing (HR 1.06, P < 0.01, 95%CI 1.01-1.10) were also independent predictors of mortality. In contrast, no individual PFT variable was predictive of mortality once HRCT patterns were accounted for. HRCT patterns, in particular, severity of traction bronchiectasis and extent of honeycombing are superior to pulmonary function tests for predicting mortality in patients with CHP. (orig.)

  14. Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care

    Energy Technology Data Exchange (ETDEWEB)

    Jain, K. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S.P.G. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom)], E-mail: s.padley@ic.ac.uk; Goldstraw, E.J.; Kidd, S.J. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom); Hogg, C.; Biggart, E.; Bush, A. [Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-10-15

    Aim: To investigate the clinical range and severity of radiological findings in a cohort of patients with primary ciliary dyskinesia (PCD) receiving tertiary care. Materials and methods: The case notes and clinical test results of 89 children attending the paediatric respiratory disease clinic at our institution were retrospectively analysed. Demographic details including age at diagnosis and common presenting signs and symptoms were studied. Results of chest radiographs, microscopy, and high-resolution computed tomography (HRCT) for quantification of lung damage were analysed. Results: In a cohort of 89 children with PCD, a presentation chest radiograph was available in 62% of patients (n = 55), with all but one demonstrating changes of bronchial wall thickening. HRCT of the lungs, available in 26 patients, were scored using the system described by Brody et al. analysing five specific features of lung disease, including bronchiectasis, mucus plugging, peribronchial thickening, parenchymal changes of consolidation, and ground-glass density, and focal air-trapping in each lobe. Peribronchial thickening was observed using HRCT in 25 patients, while 20 patients had bronchiectasis. Severity scores were highest for the middle and the lingular lobes. Conclusion: The radiographic findings of the largest reported cohort of patients with PCD are presented, with associated clinical findings. Dextrocardia remains the commonest finding on chest radiography. HRCT demonstrates peribronchial thickening and bronchiectasis, which is most marked in the lower zones. Radiological scoring techniques developed for assessment of cystic fibrosis can also be applied for the assessment of disease severity in this patient population.

  15. Bronchopulmonary infection with lophomonas blattarum: two cases report and literature review

    Institute of Scientific and Technical Information of China (English)

    Yao Guozhong

    2008-01-01

    Objective: To improve the recognition and diagnosis on the bronchopulmonary infection with Lophomonas blattarum (L. blattarum). Methods: The clinical characteristics of 2 patients diagnosed and treated in our hospital were reported, and 42 cases that had been reported from years 1993 to 2007 are analyzed. Results: In our report, the firs tpatient attacked serious asthma time after time, the second patient suffered from bronchiectasis with a protracte dinfection course. Forty-four cases all have pathogen examination and parasitic expertise. The most common symptoms are fever, cough and expectoration. 1/3 of the patients have increased acidophilic granulocyte in peripheral blood. Chest X film and CT scanning suggest changes were similar to pneumonia. Chronic cases are manifested with bronchial asthma, bronchiectasis and pulmonary abscess. L. blattarum found in phlegm or specimen collected by bronchoscopy provides the most reliable evidence for the diagnosis of this disease. Conclusion: Bronchopulmonary L. blattarum infection is a new kind of diseases. The clinical manifestations are similar to pneumonia, asthma, bronchiectasis infection or pulmonary abscess. L. blattarum found in sputum smear, bronchoscopic brush smear, bronchoscopic biopsy smear, or bronchoalceolar lavage under microscope is the foundation of the diagnosis. The pathogen species has not been finally confirmed. It is still unclear how the pathogen exists in the natural environment, how to transmit to persons and what kind of people would suffer from the disease more easily. Treatment only with antibiotics is not effective to this disease. Metronidazole with dosage of 0.5 g per time and twice per day was effective to most patients, the period of treatment need to last 14-38 d, but multidrug resistance case had been reported.

  16. Kimura disease:single case report and review of literature%Kimura 病1例并文献复习

    Institute of Scientific and Technical Information of China (English)

    张挪富; 杨新艳; 叶枫; 顾莹莹; 钟南山

    2010-01-01

    目的 提高对少见疾病Kimura病的认识.方法 分析广州医学院第一附属医院广州呼吸疾病研究所于2008年7月29日收治的1例Kimura病伴支气管扩张患者的临床和病理资料,并复习相关文献.结果 患者,女,18岁,16年前接种卡介苗后反复咳嗽、咳痰、出现全身多发性包块,临床特征主要是血嗜酸性淋巴细胞增多、头颈部包块及淋巴结肿大和支气管扩张.颈部皮下肿物活检符合嗜酸性淋巴肉芽肿,对糖皮质激素治疗有效.结论 Kimura病属于少见病,合并支气管扩张是首例报道.%Objective To recognize the rare Kimura disease(KD).Methods The clinical and pathological features of a KD patient with bronchiectasis admitted to our hospital on July 29,2008 was analyzed and related literature was reviewed.Results The patient was 18-years-old,female.Repeated cough,expectoration and systemic multiple mass appeared after bacillus Calmette-Guerin Vaccine inoculation 16 years ago.The main clinical features included enlarged lymph nodes and mass in soft tissue of the head and neck region,eosinophilia and bronchiectasis.The biopsy of subcutaneous mass of neck was consistent with pathological features of eosinophylic lymphoid granuloma.The patient was sensitive to glucocorticoid therapy.Conclusions KD is a rare disorder.We present the first case of KD with bronchiectasis.

  17. Late lung parenchymal changes on HRCT in children with mycoplasma pneumonia

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Soo Hyeon; Kim, Joung Sook; Yoon, Jung Hee; Hur, Gham; Kim, Chang Gun [Inje Univ. Sanggye Paik Hospital, Seoul (Korea, Republic of)

    1999-08-01

    To evaluate late lung parenchymal change, as seen on high-resolution CT(HRCT) in children with mycoplasma pneumonia. Twenty-three patients [15 boys and 8 girls aged two to 13 (mean, 6) years] with mycoplasma pneumonia underwent HRCT four to 39 (mean, 10) months after initial infection. Using increased mycoplasma antibody titer( > 1;640) mycoplasma pneumonia was diagnosed, and patients were divided into two groups : high titer group (antibody titer > 1:5120), and lower titer group ( < 1:5120). CT scans were performed using 2mm collimation and 5-10mm interval from apex to diaphragm. In seven patients who were cooperative, both inspiratory scans were obtained at a window width of 1600 HU and level of 700. HRCT findings of mosaic low attenuations and changes in bronchioles and bronchial walls were assessed by three radiologists and correlated with initial chest radiographic findings. On HRCT, 17 of 23 patients (74%) demonstrated abnormal findings. These included mosaic attenuation of lung density alone in 11 of 17 patients (65%), mosaic attenuation associated with bronchiectasis in five(29%), and bronchiectasis only in one(6%). Mosaic attenuation was more accentuated on expiratory scans than on inspiratory. These findings were obtained in 10 of 12 high titer group and in 7 of 11 in the lower titer group. In 15 of 23 patients(65%), involved areas seen on HRCT exactly corresponded with initially involved areas seen on chest radiographs (CXR). Two patients in whom findings on initial CXR were normal showed mosaic attenuation on HRCT. Six patients in whom such findings were abnormal showed normal findings on HRCT, a fact which reflected their complete recovery. The most common late parenchymal change in mycoplasma pneumonia, as seen on HRCT, was mosaic attenuation of lung density followed by bronchiectasis. The latter is presumably due to bronchiolitis obliterans, a well-known complication. We believe that HRCT is very useful for the evaluation of long-term sequelae of

  18. High-resolution CT in patients with chronic airflow obstruction: correlation with clinical diagnosis and pulmonary function test

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Ki Taek; Kang, Eun Young; Rhee, Ji Yong; Kim, Jin Hyung; Choi, Jung Ah; Cho, Jae Yoen; Oh, Yu Whan; Suh, Won Hyuck [College of Medicine, Korea University, Seoul (Korea, Republic of)

    2000-06-01

    To determine the utility of HRCT in the diagnosis of chronic airflow obstruction and to correlate the morphologic abnormalities revealed by this modality with functional impairment in patients with chronic airflow obstruction. This study involved 80 patients with chronic airflow obstruction who underwent HRCT and a pulmonary function test. Final clinical diagnosis in these patients was determined by a chest physician on the basis of clinical features, bronchoscopy, pulmonary function test, and HRCT. In order to diagnose and determine the extent of areas of decreased attenuation revealed by HRCT (the CT score), the findings of HRCT were retrospectively reviewed by two radiologists, who reached a consensus. Clinical and HRCT diagnoses were then compared, and the rate of agreement between them was calculated. The relationship between the extent of areas of decreased attenuation revealed by HRCT and by FEV1/FVC was evaluated using Correl's account and Student's unpaired t-test. The agreement rate between clinical and HRCT diagnoses was 77.5% (62/80). The rates for bronchiectasis (88.9%, 24/27), emphysema (93.9%, 31/33), and bronchiolitis obliterans (100%, 6/6) were considerably higher than those for chronic bronchitis and bronchial asthma. The correlation rate between CT score and FEV1/FVC was significant in bronchiectasis (p less than 0.05; r: -0.76) and bronchiolitis obliterans (p less than 0.01; r: -0.66), but not in cases involving emphysema, bronchial asthma, or chronic bronchitis (p greater than 0.05). HRCT is valuable in the diagnosis and prediction of physiologic impairment in patients with bronchiectasis and bronchiolitis obliterans, but has limited value in those with emphysema, chronic bronchitis or asthma. (author)

  19. Persistent asthma in adults: comparison of high resolution computed tomography of the lungs after one year of follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Cukier, Alberto; Stelmach, Rafael; Kavakama, Jorge Issamu; Terra Filho, Mario; Vargas, Francisco [Sao Paulo Univ., SP (Brazil). Instituto do Coracao (InCor). Div. de Doencas Respiratorias

    2001-06-01

    The aims of this study were to evaluate the role of high resolution computed tomography of the thorax in detecting abnormalities in chronic asthmatic patients and to determine the behavior of these lesions after at least one year. Fourteen persistent asthmatic patients with a mean forced expiratory volume in 1-second that was 63% of predicted and a mean forced expiratory volume in 1-second /forced vital capacity of 60% had two high resolution computed tomography separated by an interval of at least one year. All 14 patients had abnormalities on both scans. The most common abnormality was bronchial wall thickening, which was present in all patients on both computed tomography. Bronchiectasis was suggested on the first computed tomography in 5 of the 14 (36%) patients, but on follow-up, the bronchial dilatation had disappeared in 2 and diminished in a third. Only one patient had any emphysematous changes; a minimal persistent area of para septal emphysema was present on both scans. In 3 patients, a 'mosaic' appearance was observed on the first scan, and this persisted on the follow-up computed tomography. Two patients had persistent areas of mucoid impaction. In a third patient, mucus plugging was detected only on the second computed tomography. We conclude that there are many abnormalities on the high resolution computed tomography of patients with persistent asthma. Changes suggestive of bronchiectasis, namely bronchial dilatation, frequently resolve spontaneously. Therefore, the diagnosis of bronchiectasis by high resolution computed tomography in asthmatic patients must be made with caution, since bronchial dilatation can be reversible or can represent false dilatation. Non smoking chronic asthmatic subjects in this study had no evidence of centrilobular or panacinar emphysema. (author)

  20. Predominant pathogen competition and core microbiota divergence in chronic airway infection.

    Science.gov (United States)

    Rogers, Geraint B; van der Gast, Christopher J; Serisier, David J

    2015-01-01

    Chronic bacterial lung infections associated with non-cystic fibrosis bronchiectasis represent a substantial and growing health-care burden. Where Pseudomonas aeruginosa is the numerically dominant species within these infections, prognosis is significantly worse. However, in many individuals, Haemophilus influenzae predominates, a scenario associated with less severe disease. The mechanisms that determine which pathogen is most abundant are not known. We hypothesised that the distribution of H. influenzae and P. aeruginosa would be consistent with strong interspecific competition effects. Further, we hypothesised that where P. aeruginosa is predominant, it is associated with a distinct 'accessory microbiota' that reflects a significant interaction between this pathogen and the wider bacterial community. To test these hypotheses, we analysed 16S rRNA gene pyrosequencing data generated previously from 60 adult bronchiectasis patients, whose airway microbiota was dominated by either P. aeruginosa or H. influenzae. The relative abundances of the two dominant species in their respective groups were not significantly different, and when present in the opposite pathogen group the two species were found to be in very low abundance, if at all. These findings are consistent with strong competition effects, moving towards competitive exclusion. Ordination analysis indicated that the distribution of the core microbiota associated with each pathogen, readjusted after removal of the dominant species, was significantly divergent (analysis of similarity (ANOSIM), R=0.07, P=0.019). Taken together, these findings suggest that both interspecific competition and also direct and/or indirect interactions between the predominant species and the wider bacterial community may contribute to the predominance of P. aeruginosa in a subset of bronchiectasis lung infections.

  1. An unusual cause of spontaneous pneumothorax: the Mounier-Kuhn syndrome.

    LENUS (Irish Health Repository)

    Kent, B D

    2011-05-01

    We present the case of a 54-year old woman referred to our service with an unusual presentation of an under-diagnosed condition. A life-long non-smoker, she was referred to respiratory services by our emergency department with a left sided pneumothorax, progressive dyspnoea on exertion, and recurrent chest infections. Subsequent investigation yielded findings consistent with Mounier-Kuhn syndrome (Tracheobronchomegaly), a condition characterised by marked dilatation of the proximal airways, recurrent chest infection, and consequent emphysema and bronchiectasis. Although rarely diagnosed, some degree of Mounier-Kuhn syndrome may occur in up to 1 in 500 adults.

  2. Swyer- James -MacLeod syndrome presenting as hemoptysis in an adult

    Directory of Open Access Journals (Sweden)

    Santosh Kumar

    2012-06-01

    Full Text Available Swyer-James/MacLeod syndrome is an uncommon disease with characteristic radiological feature of unilateral hyperlucency due to loss of pulmonary vasculature and air trapping. Typically, this disorder is diagnosed in childhood during evaluations for recurrent respiratory infections. Here, we report a case in a 30-year-old adult female who presented with dyspnoea, cough with expectoration and recurrent hemoptysis due to associated bronchiectasis. This case highlights the importance of computed tomography in the diagnostic workup of recurrent hemoptysis in pulmonary tuberculosis epidemic countries like India.

  3. Histopathological Analogies in Chronic Pulmonary Lesions between Cattle and Humans: Basis for an Alternative Animal Model

    Science.gov (United States)

    Ramírez-Romero, Rafael; Nevárez-Garza, Alicia M.; Rodríguez-Tovar, Luis E.; Wong-González, Alfredo; Ledezma-Torres, Rogelio A.; Hernández-Vidal, Gustavo

    2012-01-01

    Most of the natural cases of pneumonia in feedlot cattle are characterized by a longer clinical course due to chronic lung lesions. Microscopically, these lesions include interstitial fibroplasia, bronchitis, bronchiectasis, bronchiolitis obliterans, and epithelial metaplasia of the airways. Herein, the aim was to review, under a medical perspective, the pathologic mechanisms operating in these chronic pneumonic lesions in calves. Based on the similarities of these changes to those reported in bronchiolitis obliterans/organising pneumonia (BO/OP) and chronic obstructive pulmonary disease (COPD) in human beings, calves are proposed as an alternative animal model. PMID:22629176

  4. Histopathological Analogies in Chronic Pulmonary Lesions between Cattle and Humans: Basis for an Alternative Animal Model

    Directory of Open Access Journals (Sweden)

    Rafael Ramírez-Romero

    2012-01-01

    Full Text Available Most of the natural cases of pneumonia in feedlot cattle are characterized by a longer clinical course due to chronic lung lesions. Microscopically, these lesions include interstitial fibroplasia, bronchitis, bronchiectasis, bronchiolitis obliterans, and epithelial metaplasia of the airways. Herein, the aim was to review, under a medical perspective, the pathologic mechanisms operating in these chronic pneumonic lesions in calves. Based on the similarities of these changes to those reported in bronchiolitis obliterans/organising pneumonia (BO/OP and chronic obstructive pulmonary disease (COPD in human beings, calves are proposed as an alternative animal model.

  5. [Pulmonary medicine 2012: news for the general practitioner].

    Science.gov (United States)

    Gex, G; Petitpierre, N; Charbonnier, F; Rochat, T

    2013-01-16

    This review reports on papers published in 2012 that will most likely impact on daily medical practice in four different areas of pulmonary medicine. How should treatment of asthma with inhaled corticosteroids be adjusted on the long run? Should idiopathic pulmonary fibrosis receive treatment with immunosuppressive drugs? Is a long-term treatment with azithromycine for bronchiectasis supported by evidence, apart from patients with cystic fibrosis? And finally, can treatment of obstructive sleep apnea with continuous positive pressure (CPAP) prevent the occurrence of new, systemic hypertension?

  6. Asian perspective in surgery: thoracic surgery in Turkey.

    Science.gov (United States)

    Turna, Akif

    2016-08-01

    Turkey with a population of 78 million is located between Asia and Europe geographically and culturally. There are 577 active pure thoracic surgeon and 37 thoracic surgery teaching units. Thoracic surgeons usually deal with lung cancer patients due to relatively higher rate of tobacco usage as well as inflammatory diseases such as pulmonary hydatid disease, bronchiectasis and empyema. Minimally invasive thoracic surgery has been a new approach which is being adapted by increasingly more surgeons. There are a number of reasons to predict that the number of thoracic surgical cases will be increased and new generation of thoracic surgeons will be operating more minimally invasive resectional surgeries for most lung cancer in future.

  7. A Presentation of Massive Hemoptysis in a Patient with Churg-Strauss Syndrome

    Directory of Open Access Journals (Sweden)

    Fadi Hikmat

    2014-01-01

    Full Text Available Given that Churg-Strauss syndrome is a systemic small-vessel vasculitis, it is not usually considered in patients who present with massive hemoptysis, which is typically caused by bronchiectasis, cancer or, in some cases, aberrant bronchial arteries. This article, however, describes a novel case involving a 50-year-old Churg-Strauss patient who presented with sudden-onset massive hemoptysis. Details of the physical examination, laboratory investigations and several imaging studies, including computed tomography, bronchoscopy and three-dimensional imaging, are presented.

  8. An unusual cause of spontaneous pneumothorax: the Mounier-Kuhn syndrome.

    LENUS (Irish Health Repository)

    Kent, B D

    2012-02-01

    We present the case of a 54-year old woman referred to our service with an unusual presentation of an under-diagnosed condition. A life-long non-smoker, she was referred to respiratory services by our emergency department with a left sided pneumothorax, progressive dyspnoea on exertion, and recurrent chest infections. Subsequent investigation yielded findings consistent with Mounier-Kuhn syndrome (Tracheobronchomegaly), a condition characterised by marked dilatation of the proximal airways, recurrent chest infection, and consequent emphysema and bronchiectasis. Although rarely diagnosed, some degree of Mounier-Kuhn syndrome may occur in up to 1 in 500 adults.

  9. Kartagener综合征1例报道并文献复习%Kartagener syndrome: A case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    王娜; 薛新颖; 薛庆亮; 张丽娜; 郭丽娜; 汪建新

    2011-01-01

    To study the clinical features, diagnosis and treatment of Kartagener syndrome(KS). Methods One case of KS diagnosed in our hospital was retrospectively analyzed and its related literature was reviewed. Results The patient was a 16year old female with repeated respiratory infection since her childhood. CT showed bronchiectasis accompanying infection and situs perversus. Symptoms of the patient were improved after expectant treatment. KS is a hereditary disease of autosome. Its most common symptoms are cough, expectoration, and nasal discharge. Chest CT scan usually shows bronchiectasis, situs perversus, and nasosinusiti, with expectant treatment as its main therapy. Conclusion KS is a rare disease which can be easily misdiagnosed as bronchiectasis or nasosinusitis. Clinical diagnosis of KS is mainly based on the triad of bronchiectasis, situs perversus and nasosinusitis. Early diagnosis and treatment of KS can prevent its complications and achieve a better prognosis.%目的 探讨Kartagener综合征(Kartagener Syndrome,KS)的临床特点、诊断和治疗方法.方法 报道1例KS病例,结合文献进行回顾性分析.结果 患者女性,16岁,自幼易反复患呼吸道感染,CT示支气管扩张并感染、全内脏转位,经对 症治疗后症状好转.KS是一种常染色体遗传性疾病,临床上以咳嗽、咳痰、流脓涕等症状最为常见,CT常提示支气管扩 张、内脏转位、鼻窦炎.对症治疗为主要治疗方法.结论 KS是一种少见疾病,临床上极易误诊为"支气管扩张、鼻窦炎",完全型KS的临床诊断主要依据"支气管扩张、内脏转位、鼻窦炎"三联征.如果能做到早期诊断、早期治疗并预防感染 等并发症,预后一般良好.

  10. Kartagener’s syndrome: A clinical reappraisal with two case reports

    Directory of Open Access Journals (Sweden)

    Apoorva Kumar Pandey

    2014-11-01

    Full Text Available Kartagener’s syndrome is a rare congenital disorder consisting of sinusitis, bronchiectasis with situs inversus and is associated with infertility. It is the subgroup of disorder called primary ciliary dyskinesia in which well defined morphological or functional abnormalities of cilia result in sinopulmonary involvement with varying severity. Clinical manifestations involve chronic and/or recurrent respiratory infections with much heterogeneity in multisystem involvement. Early diagnosis and management of this condition help to prevent irreversible lung damage and prevent chronic lifelong sequelae.

  11. A case report of Mounier-Kuhn syndrome

    Directory of Open Access Journals (Sweden)

    Keerthivasan Sivanmani

    2017-01-01

    Full Text Available Mounier-Kuhn syndrome is a congenital abnormality characterized by tracheobronchomegaly as a result of atrophy or absence of elastic fibers and thinning of smooth muscle layer in trachea and main bronchi. The usual presentation is one of recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis. We describe a case of an elderly man who presented with a recurrent respiratory infection who was subsequently diagnosed as Mounier-Kuhn syndrome.

  12. 肺心病患者睡眠低氧血症特点及氧疗和无创正压通气的应用价值%Hypoxemia features of sleep of pulmonary heart disease and practical value of oxygen therapy and positive pressure ventilation with no trauma

    Institute of Scientific and Technical Information of China (English)

    翟玮; 周利臣; 黄席珍

    2002-01-01

    1 Subjects and method 12 patients of pure pulmonary heart disease,10 males,2 females,aged form 54~ 70 years old(mean age:60 years old).Among these patients,there were 9 cases with long term smoking history,7 cases with chronic bronchitis and pulmonary emphysema,1 case with remote pulmonary tuberculosis,1 case with chronic bronchiectasis,2 cases with pulmonary interstitial fibrosis,1 case with chronic organized pneumonia.Weighing index(21~ 24)kg/m2.Sleep hypoxencic follow international sleep medical diagnosis criterion and pulmonary heart disease follow diagnosis and treatment standard of COPD made by respiratory China Medical College.

  13. Plastic Bronchitis.

    Science.gov (United States)

    Rubin, Bruce K

    2016-09-01

    Plastic bronchitis is an uncommon and probably underrecognized disorder, diagnosed by the expectoration or bronchoscopic removal of firm, cohesive, branching casts. It should not be confused with purulent mucous plugging of the airway as seen in patients with cystic fibrosis or bronchiectasis. Few medications have been shown to be effective and some are now recognized as potentially harmful. Current research directions in plastic bronchitis research include understanding the genetics of lymphatic development and maldevelopment, determining how abnormal lymphatic malformations contribute to cast formation, and developing new treatments.

  14. Consolidation with a twisted appearance along the airways: a report of five cases of interstitial pneumonia.

    Science.gov (United States)

    Watanabe, Etsuko; Kawamura, Tetsuji; Mochizuki, Yoshiro; Nakahara, Yasuharu; Sasaki, Shin; Okamoto, Akira; Higashino, Takanori

    2014-05-01

    High-resolution CT showed areas of airspace consolidation with a twisted appearance of the airways, along with areas of peribronchial ground-glass attenuation and traction bronchiectasis, in five patients with interstitial pneumonia. These areas of airspace consolidation were termed "twisted consolidation" (TwC). The five patients included two patients receiving treatment for rheumatoid arthritis (RA), one patient with newly diagnosed RA, and one patient who subsequently showed RA. Three patients showed improvement after steroid administration. An association of TwC with RA is suspected, but further studies are necessary.

  15. Yellow Nail Syndrome - a Case Report

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    Paravina Mirjana

    2015-06-01

    Full Text Available Yellow nail syndrome is a rare disease of unknown etiology. It is clinically characterized by a triad of yellow nails, lymphedema at one or more sites, and chronic respiratory disease (bronchitis, bronchiectasis and rhinosinusitis. All nails may be affected, but some may be spared. The nail plates are yellowish green, thickened, occasionally with transverse ridging and onycholysis, with increased longitudinal and transversal over-curvature, with partial or complete separation of the nail plate from the nail bed, without lunula and cuticle and slow nail growth rate. The lymphedema is usually peripheral, affecting the lower limbs, or in the form of pleural effusion.

  16. Giant Purulent Pericarditis with Cardiac Tamponade Due to Streptococcus intermedius Rapidly Progressing to Constriction.

    Science.gov (United States)

    Tigen, Elif T; Sari, Ibrahim; Ak, Koray; Sert, Sena; Tigen, Kursat; Korten, Volkan

    2015-08-01

    Purulent pericardial effusion, although rare, is a life-threatening condition usually produced by the extension of a nearby bacterial infection locus or by blood dissemination in the immune-suppressed subjects or in the course of cardiothoracic surgery. Because clinical features of purulent pericardial effusion are often nonspecific, it can cause delay in diagnosis. Therefore, a high index of suspicion is required for timely diagnosis and management. Herein, we describe a case of giant purulent pericardial effusion due to Streptococcus intermedius with the history of bronchiectasis and pneumonia, which was successfully treated with pericardiocentesis via parasternal approach, appropriate antibiotics, and pericardiectomy.

  17. Early pulmonary involvement of anti-CADM-140 autoantibody-positive rapidly progressive interstitial lung disease preceding typical cutaneous symptoms.

    Science.gov (United States)

    Tamai, Koji; Tachikawa, Ryo; Otsuka, Kyoko; Ueda, Hiroyuki; Hosono, Yuji; Tomii, Keisuke

    2014-01-01

    We herein report a patient with clinically amyopathic dermatomyositis (CADM) who developed anti-CADM-140 autoantibody in association with rapidly progressive interstitial lung disease (RP-ILD). Chest high-resolution computed tomography (HRCT) revealed early pulmonary involvement preceding typical cutaneous lesions. Primary lesions of patchy peribronchial opacity developed ground-glass opacity and consolidation with architectural distortion and traction bronchiectasis. The possibility of anti-CADM-140 autoantibody-associated RP-ILD should be considered when patchy peribronchial opacity of an unknown cause is visible on chest HRCT.

  18. Diagnosis of Adult Patients with Cystic Fibrosis.

    Science.gov (United States)

    Nick, Jerry A; Nichols, David P

    2016-03-01

    The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.

  19. Congenital tracheoesophageal fistula: A rare and late presentation in adult patient

    Directory of Open Access Journals (Sweden)

    Waseem M Hajjar

    2012-01-01

    Full Text Available Congenital H-type tracheoesophageal fistula (TEF in adults is a rare presentation and can test the diagnostic acumen of a surgeon, endoscopist, and the radiologist. These undetected fistulas may present as chronic lung disease of unknown origin because repeated aspirations can lead to recurrent lung infections and bronchiectasis. Congenital TEFs should be considered in the diagnosis of infants and young adults with recurrent respiratory distress and/or infections. Here, we present the successful management of this rare case in an adult patient.

  20. Exophiala Pneumonia Presenting with a Cough Productive of Black Sputum

    Directory of Open Access Journals (Sweden)

    Yehuda Z. Cohen

    2015-01-01

    Full Text Available Exophiala species are black, yeast-like molds that can cause subcutaneous cysts as well as disseminated disease. Isolated pneumonia due to Exophiala species is extremely uncommon. We report a case of isolated Exophiala pneumonia in a patient with bronchiectasis who presented with worsening dyspnea and a cough productive of black sputum. The production of black sputum, known as melanoptysis, is an uncommon physical finding with a limited differential diagnosis. To our knowledge, this is the first reported case of Exophiala pneumonia presenting with a cough productive of black sputum.

  1. Fungal infections of the lung in children

    Energy Technology Data Exchange (ETDEWEB)

    Toma, Paolo; Colafati, Giovanna Stefania; D' Andrea, Maria Luisa [IRCCS Bambino Gesu Children' s Hospital, Department of Imaging, Rome (Italy); Bertaina, Alice; Mastronuzzi, Angela [IRCCS Bambino Gesu Children' s Hospital, Department of Pediatric Hematology/Oncology and Transfusion Medicine, Rome (Italy); Castagnola, Elio [IRCCS Istituto Giannina Gaslini, Department of Infective Diseases, Genoa (Italy); Finocchi, Andrea [IRCCS Bambino Gesu Children' s Hospital, Department of Pediatrics, Rome (Italy); Lucidi, Vincenzina [IRCCS Bambino Gesu Children' s Hospital, Cystic Fibrosis Center, Rome (Italy); Granata, Claudio [IRCCS Istituto Giannina Gaslini, Department of Pediatric Radiology, Genoa (Italy)

    2016-12-15

    Fungal infections of the lungs are relatively common and potentially life-threatening conditions in immunocompromised children. The role of imaging in children with lung mycosis is to delineate the extension of pulmonary involvement, to assess response to therapy, and to monitor for adverse sequelae such as bronchiectasis and cavitation. The aim of this paper is to show imaging findings in a series of patients with fungal pneumonia from two tertiary children's hospitals, to discuss differential diagnoses and to show how imaging findings can vary depending on the host immune response. (orig.)

  2. KARTAGENER’S SYNDROME

    Directory of Open Access Journals (Sweden)

    Swati M

    2014-11-01

    Full Text Available : BACKGROUND: Kartagener syndrome (a clinical variant of primary ciliary dyskinesia is an autosomal recessive disease characterized by the triad of chronic sinusitis, bronchiectasis and situs inversus with dextrocardia. CASE CHARACTERISTICS: A 11-year-old boy presenting with chronic cough with expectoration requiring frequent nebulisations. OUTCOME: Early diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

  3. Cerebral Abscess and Extraaxial Empyema in a Patient with Kartagener Syndrome

    Directory of Open Access Journals (Sweden)

    Idil Gunes Tatar

    2013-06-01

    Full Text Available The triad of situs inversus totalis, bronchiectasis and sinusitis is known as Kartagener syndrome which is among the diseases with ciliopathies. Herein we present a case of cerebral abscess and extraaxial empyema detected in a 21-year-old male patient with Kartagener syndrome and a 10-year history of substance abuse. Preoperative CT, MRI findings and postoperative complications are presented with clinical and radiological review of primary ciliary dyskinesia. The consideration of primary ciliary dyskinesia in the differential diagnosis of frequent occurence of cough, rhinitis and otitis media in children is crucial since early diagnosis is known to affect the short term and long term morbidity.

  4. Coronary artery bypass surgery in a patient with Kartagener syndrome: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Bougioukas Ioannis

    2010-08-01

    Full Text Available Abstract Kartagener syndrome consists of congenital bronchiectasis, sinusitis, and total situs inversus in half of the patients. A patient diagnosed with Kartagener syndrome was reffered to our department due to 3-vessel coronary disease. An off-pump coronary artery bypass operation was performed using both internal thoracic arteries and a saphenous vein graft. We performed a literature review for cases with Kartagener syndrome, coronary surgery and dextrocardia. Although a few cases of dextrocardia were found in the literature, no case of Kartagener syndrome was mentioned.

  5. [Renal cell carcinoma in patient with situs inversus and Kartagener syndrome].

    Science.gov (United States)

    Jiménez Verdejo, A; Martínez Torres, J L; Palao Yago, F; Tinaut Ranera, J; Arrabal Martín, M; Miján Ortiz, J L; Zuluaga Gómez, A

    2000-02-01

    A case of a adenocarcinoma renal in a patient with situs inversus complete to which was associated bronchiectasis and chronic sinusitis (kartagener's syndrome) is reported. It is the third case described in the international literature of a renal cells carcinoma in a patient with situs inversus totalis and the first in patient the one which has the triada classic of the kartagener's syndrome plus sterility. The tumor was discovered in a way incidental upon accomplishing a TAC toracoabdominal and was solved through nefrectomia for lumbotomy approach.

  6. Cardiac surgery for Kartagener syndrome.

    Science.gov (United States)

    Tkebuchava, T; von Segesser, L K; Niederhäuser, U; Bauersfeld, U; Turina, M

    1997-01-01

    Two patients (one girl, one boy) with Kartagener syndrome (situs inversus, bronchiectasis, sinusitis), despite pulmonary problems and associated congenital cardiac anomalies, were operated on at the ages of 4 years and 7 years, respectively. They had had previous palliative treatment at the age of 3 months and 1.3 years, respectively. Both postoperative periods after total correction were without significant complications. Long-term follow-up was available for 9 and 19 years, respectively, with no manifestations of heart insufficiency. Both patients are physically active, and neither requires cardiac medication. Patients with Kartagener syndrome and associated congenital cardiac anomalies can successfully undergo multiple cardiac operations with good long-term outcome.

  7. A STUDY OF KARTAGENER’S SYNDROME PATIENTS AMONG

    Directory of Open Access Journals (Sweden)

    Panchasheelan

    2014-06-01

    Full Text Available Situs inversus totalis (SIT entails a mirror-image reversal of all the asymmetrical structures of the body. Many people with situs inversus are unaware of the condition. Kartagener′s syndrome (KS is a subset of a larger group of ciliary motility disorders called primary ciliary dyskinesias, comprising a triad of situs inversus, bronchiectasis and sinusitis. We found 6 cases of Situs inversus totalis (SIT over one year period among admitted patients in our department at ESIC MC PGIMSR and on evaluation 2patients were found to have Kartagener′s syndrome (KS. As the syndrome is rare we hereby report the manifestations and findings in detail

  8. Coronary artery bypass surgery in a patient with Kartagener syndrome: a case report and literature review.

    Science.gov (United States)

    Bougioukas, Ioannis; Mikroulis, Dimitrios; Danner, Bernhard; Lawal, Lukman; Eleftheriadis, Savvas; Bougioukas, George; Didilis, Vassilios

    2010-08-26

    Kartagener syndrome consists of congenital bronchiectasis, sinusitis, and total situs inversus in half of the patients. A patient diagnosed with Kartagener syndrome was referred to our department due to 3-vessel coronary disease. An off-pump coronary artery bypass operation was performed using both internal thoracic arteries and a saphenous vein graft. We performed a literature review for cases with Kartagener syndrome, coronary surgery and dextrocardia. Although a few cases of dextrocardia were found in the literature, no case of Kartagener syndrome was mentioned.

  9. An unusual presentation of immotile-cilia syndrome with azoospermia: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Dixit Ramakant

    2009-01-01

    Full Text Available Immotile-cilia syndrome is a rare disorder characterized by chronic recurrent sino-pulmonary infection, impaired tracheobronchial clearance, situs inversus in about 50% of cases, and living but immotile spermatozoa of normal morphology in semen analysis. In this report, we describe an unusual presentation of immotile-cilia syndrome with azoospermia in a 32-year-old male patient. The diagnosis was based on history of recurrent respiratory tract infection, bronchiectasis, maxillary sinusitis, hypoplasia of frontal sinuses, dextrocardia with situs inversus, impaired nasal mucociliary clearance, etc. Semen analysis revealed azoospermia without any evidence of obstruction in epididymides or vas deference. Normal spermatogenesis was seen on testicular biopsy.

  10. Azoospermia in two brothers with Kartagener syndrome:a family history and diagnosis

    Institute of Scientific and Technical Information of China (English)

    Yue Huan-xun; Zhang Xun; Jiang Min; Li Fu-ping; Ning Gang; Tian Yu; Lin Li; Zhang Si-xiao

    2008-01-01

    0This article describes 2 infertile brothers of asoospermia equally associated with co-existing sinusitis,bronchiectasis,and situs inversus and their family history.These two cases were diagnosed as Kartagener syndrome(KS).The patients were not offspring of kin marriages but their family members with scattered bronchi-pulmouary infections were discovered.No one manifests azoospermia,dextrocardia or situs inversus in the paternal family ex-cept two cases reported.No abnormalities were found in their mother-side family.KS should be differentiated from Young's syndrome,cystic fibrosis(CF),and other diseases such as Kallmann's syndrome in male infertility clinic.

  11. [Recurrent periods of respiratory tract infections in a 22-year-old].

    Science.gov (United States)

    Weinreich, Ulla Møller; Alstrup, Aage Kristian Olsen; Frost, Majbritt; Iyer, Victor Vishwanath; Bertelsen, Henrik Christian; Clausen, Paul; Jensen, Trine Hammer

    2014-12-01

    A 22-year-old male with recurrent periods of coughing and nasal discharge was unable to work and cooperate. A bronchoscopy revealed high amounts of leucocytes and no eosinofils, acute inflammation and > 105/ml Streptococcus pneumoniae susceptible to penicillin. The symptoms relapsed after penicillin and at the age of 24 the patient was CT-scanned which revealed bilateral sinusitis, mastoiditis and bronchiectasis. Treatment with azithromycin and a weight loss programme (from 156 kg) improved the health of the patient, who was an orangutan. This highlights the benefit of cooperation between medical doctors and veterinarians.

  12. Respiratory changes, hand fingers edema and yellow nails in a 94-year-old woman

    Directory of Open Access Journals (Sweden)

    Vitorino Modesto dos Santos

    2015-10-01

    Full Text Available A 94-year-old woman, with antecedent of chronic bronchitis, bronchiectasis, recurrent pneumonitis, arterial hypertension and chronic renal failure was admitted to control an episode of cardiac and respiratory insufficiency. Yellow nail changes and a tendency to pincer nails developed in her hand and toe fingers, preceded by longstanding course of respiratory diseases with pleural involvement. Laboratory tests detected moderate anemia and mildly elevated levels of urea and creatinine, thyroid function was normal. This case study is about yellow nail syndrome in the absence of ankle lymphedema, and affecting a woman of the oldest-old age group with renal failure.

  13. Yellow Nail Syndrome: Dystrophic Nails, Peripheral Lymphedema and Chronic Cough

    Directory of Open Access Journals (Sweden)

    Christian Dornia

    2011-01-01

    Full Text Available A case involving a 41-year-old man with yellow nail syndrome (YNS is reported. YNS is a rare disorder characterized by yellow, dystrophic nails, peripheral lymphedema and bronchiectasis with recurrent lower respiratory tract infections. YNS is often misdiagnosed because the syndrome is not well known. An interdisciplinary approach is required to recognize and collate the components of the syndrome accurately. Correct diagnosis is of utmost clinical importance because YNS can occur secondary to malignancies and autoimmune disorders. Hence, the diagnosis of YNS must prompt further investigation.

  14. Left bronchial artery arising from a replaced left hepatic artery in a patient with massive hemoptysis

    Energy Technology Data Exchange (ETDEWEB)

    Khil, Eun Kyung; Lee, Jae Myung [Dept. of Radiology, Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon (Korea, Republic of)

    2015-09-15

    A 70-year-old man with a 3-year history of bronchiectasis presented with massive hemoptysis that had lasted for 3 days. In our attempt to perform bronchial artery embolization, upper abdominal angiography was required to locate the left bronchial artery, which in this case was of anomalous origin, arising from a replaced left hepatic artery, which arose from the left gastric artery-a very unusual anatomical variant. We performed embolization with polyvinyl alcohol particles, and the patient's symptoms resolved completely, with no additional complications after conservative treatment.

  15. Cardiac and Respiratory Disease in Aged Horses.

    Science.gov (United States)

    Marr, Celia M

    2016-08-01

    Respiratory and cardiac diseases are common in older horses. Advancing age is a specific risk factor for cardiac murmurs and these are more likely in males and small horses. Airway inflammation is the most common respiratory diagnosis. Recurrent airway obstruction can lead to irreversible structural change and bronchiectasis; with chronic hypoxia, right heart dysfunction and failure can develop. Valvular heart disease most often affects the aortic and/or the mitral valve. Management of comorbidity is an essential element of the therapeutic approach to cardiac and respiratory disease in older equids.

  16. Bloom syndrome with lung involvement

    Directory of Open Access Journals (Sweden)

    Nair Girija

    2009-01-01

    Full Text Available We report a case of a 24-year old male presented with cough and breathlessness with diabetes mellitus and diagnosed as a case of bloom syndrome. He was a product of consanguineous marriage, having short stature, dolicocephaly, polydactyly, prominent nose with telangiectasia face. The respiratory system examination revealed bilateral coarse crepitations and wheezes and the chest X-ray revealed emphysema with right middle zone inhomogenous opacity. Also, CT thorax examination revealed bilateral cystic bronchiectasis with bronchiolitis obliterans. Bloom′s syndrome was diagnosed on the basis of clinical features.

  17. Bloom syndrome with lung involvement.

    Science.gov (United States)

    Nair, Girija; Lobo, Ivona; Jayalaksmi, T K; Uppe, Abhay; Jindal, Savita; Chandra, Abhishek; Swami, Shivani

    2009-07-01

    We report a case of a 24-year old male presented with cough and breathlessness with diabetes mellitus and diagnosed as a case of bloom syndrome. He was a product of consanguineous marriage, having short stature, dolicocephaly, polydactyly, prominent nose with telangiectasia face. The respiratory system examination revealed bilateral coarse crepitations and wheezes and the chest X-ray revealed emphysema with right middle zone inhomogenous opacity. Also, CT thorax examination revealed bilateral cystic bronchiectasis with bronchiolitis obliterans. Bloom's syndrome was diagnosed on the basis of clinical features.

  18. Inhaled adrenergics and anticholinergics in obstructive lung disease: do they enhance mucociliary clearance?

    Science.gov (United States)

    Restrepo, Ruben D

    2007-09-01

    Pulmonary mucociliary clearance is an essential defense mechanism against bacteria and particulate matter. Mucociliary dysfunction is an important feature of obstructive lung diseases such as chronic obstructive pulmonary disease, asthma, cystic fibrosis, and bronchiectasis. This dysfunction in airway clearance is associated with accelerated loss of lung function in patients with obstructive lung disease. The involvement of the cholinergic and adrenergic neural pathways in the pathophysiology of mucus hypersecretion suggests the potential therapeutic role of bronchodilators as mucoactive agents. Although anticholinergics and adrenergic agonist bronchodilators have been routinely used, alone or in combination, to enhance mucociliary clearance in patients with obstructive lung disease, the existing evidence does not consistently show clinical effectiveness.

  19. Diffuse polypoid lesions of the digestive tract in severe hypogammaglobulinemia.

    Science.gov (United States)

    Jurcuţ, C; Jinga, Mariana; Costache, Raluca; Vasilescu, Florina; Stănescu-Popp, Alina; Copaci, I

    2011-01-01

    We present a case report of diffuse polypoid lesions of the digestive tract in which multiple endoscopic techniques (upper endoscopy, colonoscopy, and capsule endoscopy) were performed in order to complete and refine the diagnosis in a patient with severe hypogammaglobulinemia with recurrent infections, bronchiectasies and diffuse nodular lymphoid hyperplasia of the digestive tract. In patients with hypogammaglobulinemia, the endoscopic evaluation of the digestive tract might be mandatory, even in asymptomatic patients. The complete assessment should include the upper endoscopy and colonoscopy with multiple biopsies and, if possible, capsule endoscopy.

  20. Pulmonary infection caused by Mycobacterium kansasii: findings on computed tomography of the chest

    Energy Technology Data Exchange (ETDEWEB)

    Mogami, Roberto; Lopes, Agnaldo Jose; Marca, Patricia Gomes Cytrangulo de, E-mail: agnaldolopes.uerj@gmail.com [Universidade do Estado do Rio de Janeiro (UERJ), RJ (Brazil); Goldenberg, Telma; Mello, Fernanda Carvalho de Queiroz [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil)

    2016-07-15

    Objective: To describe the main tomography findings in patients diagnosed with pulmonary infection caused by Mycobacterium kansasii. Materials and Methods: Retrospective study of computed tomography scans of 19 patients with pulmonary infection by M. kansasii. Results: Of the 19 patients evaluated, 10 (52.6%) were male and 9 (47.4%) were female. The mean age of the patients was 58 years (range, 33-76 years). Computed tomography findings were as follows: architectural distortion, in 17 patients (89.5%); reticular opacities and bronchiectasis, in 16 (84.2%); cavities, in 14 (73.7%); centrilobular nodules, in 13 (68.4%); small consolidations, in 10 (52.6%); atelectasis and large consolidations, in 9 (47.4%); subpleural blebs and emphysema, in 6 (31.6%); and adenopathy, in 1 (5.3%). Conclusion: There was a predominance of cavities, as well as of involvement of the small and large airways. The airway disease was characterized by bronchiectasis and bronchiolitis presenting as centrilobular nodules. (author)

  1. CT predictors of mortality in pathology confirmed ARDS

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Jonathan H.; Greene, Reginald E.; Shepard, Jo-Anne O.; Digumarthy, Subba R. [Massachusetts General Hospital, Department of Radiology, Boston, MA (United States); Kradin, Richard L. [Massachusetts General Hospital, Department of Pathology and Pulmonary Medicine, Boston, MA (United States)

    2011-04-15

    To identify CT findings that predict mortality in acute respiratory distress syndrome (ARDS) and to identify CT findings that differentiate diffuse alveolar damage (DAD) from DAD with prominent histopathological features of organizing pneumonia (DAD-OP). Twenty-eight patients with ARDS (corroborated by open biopsy) and chest CT within 2 weeks of biopsy were included in our study. Differences in CT findings in patients with survivors versus nonsurvivors as well as for DAD versus DAD-OP were compared using Fisher's exact test. Lung involvement of greater than 80%, RA/LA ratio >1, and varicoid traction bronchiectasis were statistically more common in nonsurvivors than in survivors (respective p values of 0.001, 0.008, and 0.038). PA dilation greater than 3 cm and RV/LV ratio greater than 0.9 were also more common in nonsurvivors than in survivors but these factors did not achieve significance. CT findings did not differentiate DAD from DAD-OP. Our study suggests that >80% of lung involvement, RA/LA ratio >1, and varicoid bronchiectasis predict mortality in patients with ARDS/DAD. Signs of right-sided heart failure (PA dilation greater than 3 cm and RV/LV ratio greater than 0.9) approached significance. CT findings did not differentiate DAD from DAD-OP. (orig.)

  2. HIGH-Resolution CT in Chronic Pulmonary Changes after Mustard Gas Exposure

    Energy Technology Data Exchange (ETDEWEB)

    Bagheri, M.H.; Mostafavi, S.H. [Shiraz Univ. of Medical Siences (Iran, Islamic Republic of). Dept. of Radiology; Hosseini, S.K. [Shiraz Univ. of Medical Siences (Iran, Islamic Republic of). Dept. of Internal Medicine; Alavi, S.A. [Medical Center for Chemical Warfare Victims Foundation, Shiraz (Iran, Islamic Republic of)

    2003-05-01

    Purpose: To identify the findings of high-resolution CT (HRCT) of the lung in patients with previous sulfur mustard gas exposure, and to correlate these findings with clinical and chest X-ray (CXR) results. Material and Methods: 50 consecutive patients were studied prospectively. The clinical data were recorded. Standard p.a. CXR and HRCT of the lung and spirometry were performed. The findings of CXR, HRCT and clinical and spirometry results were scored between 0 and 3 according to the severity of the findings. Results: HRCT abnormality was detected in all 50 patients (100%), while CXR was abnormal in 40 patients (80%). The most common HRCT findings was airway abnormalities (bronchial wall thickening in 100% of cases). Other important findings were suggestive of interstitial lung disease (ILD) (80%), bronchiectasis (26%), and emphysema (24%). A statistically significant correlation was found between the severity of clinical presentation and that of the HCTR scores in patients with bronchiectasis, bronchitis and ILD (p< 0.05), but not with severity scores of HRCT in patients with emphysema. No significant correlation was found between severity scores of CXR findings. HRCT evidence of bronchial wall thickening and with a lower frequency ILD were present despite normal CXR in 20% of the patients. Conclusion: The results of this study suggest that bronchial wall thickening, ILD and emphysema are common chronic pulmonary sequelae of sulfur mustard injury. HRCT of the chest should be considered as the imaging modality of choice in chemical war injury.

  3. Inflammatory mechanisms and treatment of obstructive airway diseases with neutrophilic bronchitis.

    Science.gov (United States)

    Simpson, Jodie L; Phipps, Simon; Gibson, Peter G

    2009-10-01

    Obstructive airway diseases such as asthma and chronic obstructive pulmonary disease (COPD) are major global health issues. Although considered as distinct diseases, airway inflammation is a key underlying pathophysiological process in asthma, COPD and bronchiectasis. Persistent neutrophilic airway inflammation (neutrophilic bronchitis) occurs with innate immune activation and is a feature of each of these airway diseases. Little is known about the mechanisms leading to neutrophilic bronchitis and few treatments are effective in reducing neutrophil accumulation in the airways. There is a similar pattern of inflammatory mediator release and toll like receptor 2 expression in asthma, COPD and bronchiectasis. We propose the existence of an active amplification mechanism, an effector arm of the innate immune system, involving toll like receptor 2, operating in persistent neutrophilic bronchitis. Neutrophil persistence in the airways can occur through a number of mechanisms such as impaired apoptosis, efferocytosis and mucus hypersecretion, all of which are impaired in airways disease. Impairment of neutrophil clearance results in a reduced ability to respond to bacterial infection. Persistent activation of airway neutrophils may result in the persistent activation of the innate immune system resulting in further airway insult. Current therapies are limited for the treatment of neutrophilic bronchitis; possible treatments being investigated include theophylline, statins, antagonists of pro-inflammatory cytokines and macrolide antibiotics. Macrolides have shown great promise in their ability to reduce airway inflammation, and can reduce airway neutrophils, levels of CXCL8 and neutrophil proteases in the airways. Studies also show improvements in quality of life and exacerbation rates in airways diseases.

  4. FINDINGS OF CHEST RADIOGRAPH AND SPIRAL COMPUTED TOMOGRAPHY IN SWYER-JAMES SYNDROME

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Objective To evaluate the value of X-ray and spiral computed tomography (SCT) in the diagnosis of SwyerJames syndrome (SJS).Methods A total of 28 patients, 12 males and 16 females, were studied retrospectively. Ages ranged from 11 to 57 years, the mean age was 32 years. All patients underwent inspiratory chest X-ray films, 5 with expiratory chest films and 1 with bronchogram. Furthermore, inspiratory and expiratory SCT scans were performed. The SCT findings were analyzed and compared with X-ray films.Results SCT demonstrated 56 lobes with hyperlucency and diminished vascularity. The size of 51 lobes were smaller and 5 were normal. X-ray films showed that hyperlucency was only in 29 lobes, in which 19 lobes were smallsized and the other 10 lobes normal. There were 56 lobes with air-trapping on expiratory SCT scans, but only 5 lobes with air-trapping on expiratory X-ray films. Bronchogram in 1 case demonstrated bronchiectasis and bronchiolitis obliterans. SCT showed 24 patients with bronchiectasis, 9 patients with tuberculosis, 10 patients with bronchiolitis, and 2 with segmental collapse. Conclusion SCT scan is superior to chest radiography in the diagnosis and differential diagnosis of SJS.

  5. Can acute interstitial pneumonia be differentiated from bronchiolitis obliterans organizing pneumonia by high-resolution CT?

    Energy Technology Data Exchange (ETDEWEB)

    Mihara, Naoki; Johkoh, Takeshi [Osaka Univ., Suita (Japan). Medical School; Ichikado, Kazuya (and others)

    2000-10-01

    In the early stages, clinical and chest radiographic findings of acute interstitial pneumonia (AIP) are often similar to those of bronchiolitis obliterans organizing pneumonia (BOOP). However, patients with AIP have a poor prognosis, while those with BOOP can achieve a complete recovery after corticosteroid therapy. The objective of this study was to identify differences in high-resolution CT (HRCT) findings between the two diseases. The study included 27 patients with AIP and 14 with BOOP who were histologically diagnosed [open-lung biopsy (n=7), autopsy (n=17), transbronchial lung biopsy (n=17)]. The frequency and distribution of various HRCT findings for each disease were retrospectively evaluated. Traction bronchiectasis, interlobular septal thickening, and intralobular reticular opacities were significantly more prevalent in AIP (92.6%, 85.2%, and 59.3%, respectively) than in BOOP (42.9%, 35.7%, and 14.3%, respectively) (p<0.01). Parenchymal nodules and peripheral distribution were more prevalent in BOOP (28.6% and 57.1%, respectively) than in AIP (7.4% and 14.8%, respectively) (p<0.01). Areas with ground-glass attenuation, air-space consolidation, and architectural distortion were common in both AIP and BOOP. For a differential diagnosis of AIP and BOOP, special attention should be given to the following HRCT findings: traction bronchiectasis, interlobular septal thickening, intralobular reticular opacities, parenchymal nodules, pleural effusion, and peripheral zone predominance. (author)

  6. Bronchiolitis obliterans following exposure to sulfur mustard: chest high resolution computed tomography

    Energy Technology Data Exchange (ETDEWEB)

    Ghanei, Mostafa E-mail: m.ghanei@bmsu.ac.ir; Mokhtari, Majid; Mohammad, Mehdi Mir; Aslani, Jafar

    2004-11-01

    Background: Pulmonary complications are known to occur in over half of the patients exposed to sulfur mustard (SM). Chemical weapons of mass destruction (WMD) including SM were used by Iraq during Iran-Iraq war between 1983 and 1989. We undertook this study to evaluate the chest high resolution computerized tomography (HRCT) as a diagnostic tool in patients with documented exposure to SM and chronic respiratory symptoms. Method: The medical records of 155 patients exposed to SM during Iran-Iraq war and suffered respiratory complications were reviewed. Chest HRCTs of these patients were examined. Ten healthy controls with no history of exposure to HD were matched for age, gender, and chest HRCT protocol applied. Results: Fifty chest HRCTs of these patients were randomly selected for this study. The most frequent findings were; air trapping 38 (76%), bronchiectasis 37 (74%), mosaic parenchymal attenuation (MPA) 36 (72%), irregular and dilated major airways 33 (66%) bronchial wall thickening (BWT) 45 (90%), and interlobular septal wall thickening (SWT) 13 (26%), respectively. Air trapping in one patient (10%) was the only positive finding in the control group. Conclusions: Chest HRCT findings of bronchiectasis, air trapping, MPA, SWT, and BWT were seen in our patients 15 years after exposure to HD. These findings suggest the diagnosis of bronchiolitis obliterans (BO). We did not encounter chest HRCT features consistent with pulmonary fibrosis.

  7. Paragonimiasis: first case reported in Brazil

    Directory of Open Access Journals (Sweden)

    Antônio Carlos Moreira Lemos

    2007-02-01

    Full Text Available The authors present a case from a 59 years old white female Brazilian patient, based in Salvador-Bahia, Brazil's northeastern side area, who experienced irritative cough and progressive dyspnea, and, after 18 months, was admitted to a hospital with respiratory insufficiency. The physical exam showed diffuse rales in both hemithoraces. Initial leukogram showed 14,400 cells/mL with 14% of eosinophils and chest X-ray showed peribronchovascular infiltrate, predominating in the lower half of the lung fields, and small opaque nodules. The high-resolution computed tomography scan of the chest (HRCT presented compatible pattern with airways disease, especially from the small airways, with air trapping, tree sprouting images, central lobular nodules and bronchiectasis, making the results compatible with bronchiolitis and bronchiectasis. The transbroncho biopsy unveiled granulomatous lesion with necrosis, where was noticed a structure compatible to a parasitic case, and the research of the parasite eggs in the sputum was positive to paragonimus. After the praziquantel use, the patient presented a thick ferruginous expectoration and the result for BAAR examination was positive. The PCR exam and the sputum culture confirmed M. tuberculosis, and then the treatment for M. tuberculosis was initiated. The authors warn that this infection may have been a consequence of economics globalization process, where the importation of parasitized crustaceans might be the cause. However, there is the need of an accurate examination for the possibility of paragonimus specimens in this area of Brazil.

  8. A new autosomal dominant eye and lung syndrome linked to mutations in TIMP3 gene

    Science.gov (United States)

    Meunier, Isabelle; Bocquet, Béatrice; Labesse, Gilles; Zeitz, Christina; Defoort-Dhellemmes, Sabine; Lacroux, Annie; Mauget-Faysse, Martine; Drumare, Isabelle; Gamez, Anne-Sophie; Mathieu, Cyril; Marquette, Virginie; Sagot, Lola; Dhaenens, Claire-Marie; Arndt, Carl; Carroll, Patrick; Remy-Jardin, Martine; Cohen, Salomon Yves; Sahel, José-Alain; Puech, Bernard; Audo, Isabelle; Mrejen, Sarah; Hamel, Christian P.

    2016-01-01

    To revisit the autosomal dominant Sorsby fundus dystrophy (SFD) as a syndromic condition including late-onset pulmonary disease. We report clinical and imaging data of ten affected individuals from 2 unrelated families with SFD and carrying heterozygous TIMP3 mutations (c.572A > G, p.Y191C, exon 5, in family 1 and c.113C > G, p.S38C, exon 1, in family 2). In family 1, all SFD patients older than 50 (two generations) had also a severe emphysema, despite no history of smoking or asthma. In the preceding generation, the mother died of pulmonary emphysema and she was blind after the age of 50. Her two great-grandsons (<20 years), had abnormal Bruch Membrane thickness, a sign of eye disease. In family 2, eye and lung diseases were also associated in two generations, both occurred later, and lung disease was moderate (bronchiectasis). This is the first report of a syndromic SFD in line with the mouse model uncovering the role of TIMP3 in human lung morphogenesis and functions. The TIMP3 gene should be screened in familial pulmonary diseases with bronchiectasis, associated with a medical history of visual loss. In addition, SFD patients should be advised to avoid tobacco consumption, to practice sports, and to undergo regular pulmonary examinations. PMID:27601084

  9. Clinical spectrum of primary ciliary dyskinesia in childhood

    Science.gov (United States)

    Fretzayas, Andrew; Moustaki, Maria

    2016-01-01

    Although the triad of bronchiectasis, sinusitis and situs inversus was first described by Kartagener in 1933, the clinical spectrum of primary ciliary dyskinesia is still under investigation. Heterotaxy defects as well as upper and lower respiratory tract symptoms are the main manifestations in childhood. It is now recognized that situs inversus is encountered in only half of patients. The first lower respiratory symptoms may be present from infancy as neonatal respiratory distress. The most common lower airway manifestations are chronic wet cough, recurrent pneumonia and therapy resistant wheezing. Patients are at risk of developing bronchiectasis which may even be the presenting finding due to delayed diagnosis. Upper respiratory tract infections such as nasal congestion, nasal drainage and recurrent sinusitis as well as otologic manifestations such as otitis media or otorrhea with conductive hearing loss are also often encountered. It seems that the type of ciliary ultrastructure defects and the involved mutated genes are associated to some extent to the clinical profile. The disease, even in nowadays, is not recognized at an early age and the primary care clinician should have knowledge of its clinical spectrum in order to select appropriately the children who need further investigation for the diagnosis of this disorder. PMID:26862502

  10. Medical image of the week: Kartagener syndrome

    Directory of Open Access Journals (Sweden)

    Chan NH

    2013-10-01

    Full Text Available No abstract available. Article truncated at 150 words. A 52 year old woman was admitted for dyspnea and fatigue. Kartagener syndrome had been diagnosed at age 3 with situs inversus totalis (Figures 1 and 2. She has bronchiectasis (Figure 3 with chronic Pseudomonas colonization, chronic sinusitis, and nasal polyposis. Kartagener syndrome is a type of primary cilia dyskinesia or immotile-cilia syndrome. When primary ciliary dyskinesia is combined with situs inversus it is known as Kartagener syndrome (KS after the Swiss internist who recognized the association of situs inversus, bronchiectasis and sinusitis (1. It is popular in case presentations especially with the chest x-ray or CT scans deliberated presented inverted. KS is an autosomal recessive disorder of the ciliary axoneme with incomplete penetrance and extensive heterogeneity (2. The typical ciliary axoneme consists of 2 central microtubules surrounded by 9 microtubular doublets. Patients with primary ciliary dyskinesia exhibit a wide range of defects in ciliary ultrastructure and motility, which ultimately …

  11. Medical image of the week: Kartagener syndrome

    Directory of Open Access Journals (Sweden)

    Das D

    2015-06-01

    Full Text Available No abstract available. Article truncated at 150 words. A 65-year-old woman presented with 7 days of productive cough and the new onset sharp central chest pain. She has a known history of chronic sinusitis and COPD after being a 50 pack-year smoker. On examination, her blood pressure was 116/70 with a heart rate of 86 (sinus rhythm and oxygen saturations were 93% on 4L/min by nasal cannula. She had bilateral expiratory wheezes with reduced air entry on the left side. An AP chest x-ray revealed dextrocardia with a left sided tension pneumothorax (Figure 1A. Our patient was stabilized with an urgent chest tube insertion and taken for a CT chest and abdomen. CT chest indicated diffuse bronchiectasis (Figure 1B, arrow with a CT of the abdomen showing reversal of major abdominal organs (Figure 1C. First described in 1933, the triad of chronic sinusitis, bronchiectasis, and situs inversus is classic for Kartagener syndrome (1. Otherwise known as primary ciliary ...

  12. Recent advances in primary ciliary dyskinesia.

    Science.gov (United States)

    Takeuchi, Kazuhiko; Kitano, Masako; Ishinaga, Hajime; Kobayashi, Masayoshi; Ogawa, Satoru; Nakatani, Kaname; Masuda, Sawako; Nagao, Mizuho; Fujisawa, Takao

    2016-06-01

    Primary ciliary dyskinesia (PCD) is a genetic disease inherited in an autosomal recessive manner. The prevalence of PCD is estimated to be 1 in 20,000 live births. Congenital abnormality of the primary cilia results in situs inversus in 50% of patients. Decreased function of motile cilia causes chronic rhinosinusitis, otitis media with effusion, bronchiectasis and infertility. Cases with situs inversus are considered to show "Kartagener's syndrome", and diagnosis is not difficult. However, in cases without situs inversus, the diagnosis is much more troublesome. PCD without situs inversus is thus probably underdiagnosed. Prolonged chronic cough represents an important symptom that is seen in most patients. The diagnosis of PCD requires the presence of the characteristic clinical phenotypes and either: (1) specific ciliary ultrastructural defects identified by transmission electron microscopy in biopsy samples of respiratory epithelium; or (2) identification of mutation in one of the genes known to be associated with PCD. Nasal nitric oxide concentration is extremely low in PCD, and this could be useful for screening of the disease. At present, no fundamental therapies are available for PCD. Diagnosis in the early stages is important to prevent progression of bronchiectasis and deterioration of lung function by guidance for daily life, immunization, cessation of smoking and prompt therapy at the time of respiratory tract infection. Since PCD is inherited in an autosomal-recessive manner, genetic counseling is necessary after definite diagnosis.

  13. Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome.

    Science.gov (United States)

    Leigh, Margaret W; Pittman, Jessica E; Carson, Johnny L; Ferkol, Thomas W; Dell, Sharon D; Davis, Stephanie D; Knowles, Michael R; Zariwala, Maimoona A

    2009-07-01

    Primary ciliary dyskinesia is a genetically heterogeneous disorder of motile cilia. Most of the disease-causing mutations identified to date involve the heavy (dynein axonemal heavy chain 5) or intermediate(dynein axonemal intermediate chain 1) chain dynein genes in ciliary outer dynein arms, although a few mutations have been noted in other genes. Clinical molecular genetic testing for primary ciliary dyskinesia is available for the most common mutations. The respiratory manifestations of primary ciliary dyskinesia (chronic bronchitis leading to bronchiectasis, chronic rhino-sinusitis, and chronic otitis media)reflect impaired mucociliary clearance owing to defective axonemal structure. Ciliary ultrastructural analysis in most patients (>80%) reveals defective dynein arms, although defects in other axonemal components have also been observed. Approximately 50% of patients with primary ciliary dyskinesia have laterality defects (including situs inversus totalis and, less commonly, heterotaxy, and congenital heart disease),reflecting dysfunction of embryological nodal cilia. Male infertility is common and reflects defects in sperm tail axonemes. Most patients with primary ciliary dyskinesia have a history of neonatal respiratory distress, suggesting that motile cilia play a role in fluid clearance during the transition from a fetal to neonatal lung. Ciliopathies involving sensory cilia, including autosomal dominant or recessive polycystic kidney disease, Bardet-Biedl syndrome, and Alstrom syndrome, may have chronic respiratory symptoms and even bronchiectasis suggesting clinical overlap with primary ciliary dyskinesia.

  14. Bronchial Artery Embolization for Massive Hemoptysis: a Retrospective Study

    Directory of Open Access Journals (Sweden)

    Ali Fani

    2013-05-01

    Full Text Available   Introduction: To assess the efficacy and safety of bronchial artery embolization in the treatment of massive hemoptysis.   Materials and Methods: A retrospective study on 46 patients (26 males and 20 females who were referred to the Razavi Hospital from April 2009 to May 2012 with massive hemoptysis and had bronchial artery embolization procedures. General characteristics of the patients including age, gender, etiology, and thorax computed tomograms, findings of bronchial angiographic, results of the embolization, complications related to bronchial artery embolization and clinical outcome during follow-up were reviewed. Results: The etiology included previous pulmonary tuberculosis in 20 cases, previous tuberculosis with bronchiectasis in 16 cases, bronchiectasis in 6 cases, and active pulmonary tuberculosis in one case. No identifiable causes could be detected in three patients. Moreover, massive hemoptysis was successfully and immediately controlled following the embolization procedure in all patients. One patient developed recurrent hemoptysis during one month following the procedure and was treated by re-embolization. No major procedure–related complication such as bronchial infarction was identified However none of the patientsexperienced neurological complications. Conclusion: Bronchial artery embolization is a safe and effective means of controlling massive hemoptysis and should be regarded as the first-line treatment for this condition.

  15. Serial micro-CT assessment of the therapeutic effects of rosiglitazone in a bleomycin-induced lung fibrosis mouse model

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Eun Jung; Jin, Gong Yong; Bok, Se Mi; Han, Young Min; Lee, Young Sun; Jung, Myung Ja; Kwon, Keun Sang [Research Institute of Clinical Medicine of Chonbuk National University, Biomedical Research Institute of Chonbuk National University Hospital, Institute for Medical Sciences, Jeonju (Korea, Republic of)

    2014-08-15

    The aim of this study was to assess the therapeutic effects of rosiglitazone with serial micro-CT findings before and after rosiglitazone administration in a lung fibrosis mouse model induced with bleomycin. We instilled the bleomycin solution directly into the trachea in twenty mice (female, C57BL/6 mice). After the instillation with bleomycin, mice were closely observed for 3 weeks and then all mice were scanned using micro-CT without sacrifice. At 3 weeks, the mice were treated with rosiglitazone on days 21 to 27 if they had abnormal CT findings (n = 9, 45%). For the mice treated with rosiglitazone, we performed micro-CT with mouse sacrifice 2 weeks after the rosiglitazone treatment completion. We assessed the abnormal CT findings (ground glass attenuation, consolidation, bronchiectasis, reticular opacity, and honeycombing) using a five-point scale at 3 and 6 weeks using Wilcoxon-signed ranked test. The micro-CT findings were correlated with the histopathologic results. One out of nine (11.1%) mice improved completely. In terms of consolidation, all mice (100%) showed marked decrease from 3.1 ± 1.4 at 3 weeks to 0.9 ± 0.9 at 6 weeks (p = 0.006). At 6 weeks, mild bronchiectasis (n = 6, 66.7%), mild reticular opacity (n 7, 77.8%) and mild honeycomb patterns (n = 3, 33.3%) appeared. A serial micro-CT enables the evaluation of drug effects in a lung fibrosis mouse model.

  16. Pulmonary infection caused by Mycobacterium kansasii: findings on computed tomography of the chest*

    Science.gov (United States)

    Mogami, Roberto; Goldenberg, Telma; de Marca, Patricia Gomes Cytrangulo; Mello, Fernanda Carvalho de Queiroz; Lopes, Agnaldo José

    2016-01-01

    Objective To describe the main tomography findings in patients diagnosed with pulmonary infection caused by Mycobacterium kansasii. Materials and Methods Retrospective study of computed tomography scans of 19 patients with pulmonary infection by M. kansasii. Results Of the 19 patients evaluated, 10 (52.6%) were male and 9 (47.4%) were female. The mean age of the patients was 58 years (range, 33-76 years). Computed tomography findings were as follows: architectural distortion, in 17 patients (89.5%); reticular opacities and bronchiectasis, in 16 (84.2%); cavities, in 14 (73.7%); centrilobular nodules, in 13 (68.4%); small consolidations, in 10 (52.6%); atelectasis and large consolidations, in 9 (47.4%); subpleural blebs and emphysema, in 6 (31.6%); and adenopathy, in 1 (5.3%). Conclusion There was a predominance of cavities, as well as of involvement of the small and large airways. The airway disease was characterized by bronchiectasis and bronchiolitis presenting as centrilobular nodules. PMID:27777472

  17. A Pseudomonas aeruginosa toxin that hijacks the host ubiquitin proteolytic system.

    Directory of Open Access Journals (Sweden)

    Jennifer M Bomberger

    2011-03-01

    Full Text Available Pseudomonas aeruginosa (P. aeruginosa is an opportunistic pathogen chronically infecting the lungs of patients with chronic obstructive pulmonary disease (COPD, pneumonia, cystic fibrosis (CF, and bronchiectasis. Cif (PA2934, a bacterial toxin secreted in outer membrane vesicles (OMV by P. aeruginosa, reduces CFTR-mediated chloride secretion by human airway epithelial cells, a key driving force for mucociliary clearance. The aim of this study was to investigate the mechanism whereby Cif reduces CFTR-mediated chloride secretion. Cif redirected endocytosed CFTR from recycling endosomes to lysosomes by stabilizing an inhibitory effect of G3BP1 on the deubiquitinating enzyme (DUB, USP10, thereby reducing USP10-mediated deubiquitination of CFTR and increasing the degradation of CFTR in lysosomes. This is the first example of a bacterial toxin that regulates the activity of a host DUB. These data suggest that the ability of P. aeruginosa to chronically infect the lungs of patients with COPD, pneumonia, CF, and bronchiectasis is due in part to the secretion of OMV containing Cif, which inhibits CFTR-mediated chloride secretion and thereby reduces the mucociliary clearance of pathogens.

  18. A Pseudomonas aeruginosa toxin that hijacks the host ubiquitin proteolytic system.

    Science.gov (United States)

    Bomberger, Jennifer M; Ye, Siying; Maceachran, Daniel P; Koeppen, Katja; Barnaby, Roxanna L; O'Toole, George A; Stanton, Bruce A

    2011-03-01

    Pseudomonas aeruginosa (P. aeruginosa) is an opportunistic pathogen chronically infecting the lungs of patients with chronic obstructive pulmonary disease (COPD), pneumonia, cystic fibrosis (CF), and bronchiectasis. Cif (PA2934), a bacterial toxin secreted in outer membrane vesicles (OMV) by P. aeruginosa, reduces CFTR-mediated chloride secretion by human airway epithelial cells, a key driving force for mucociliary clearance. The aim of this study was to investigate the mechanism whereby Cif reduces CFTR-mediated chloride secretion. Cif redirected endocytosed CFTR from recycling endosomes to lysosomes by stabilizing an inhibitory effect of G3BP1 on the deubiquitinating enzyme (DUB), USP10, thereby reducing USP10-mediated deubiquitination of CFTR and increasing the degradation of CFTR in lysosomes. This is the first example of a bacterial toxin that regulates the activity of a host DUB. These data suggest that the ability of P. aeruginosa to chronically infect the lungs of patients with COPD, pneumonia, CF, and bronchiectasis is due in part to the secretion of OMV containing Cif, which inhibits CFTR-mediated chloride secretion and thereby reduces the mucociliary clearance of pathogens.

  19. 变应性支气管肺曲菌病误诊原因及影像特点分析%Misdiagnosis Cause and Imaging Feature of Allergic Bronchopulmonary Aspergillosis

    Institute of Scientific and Technical Information of China (English)

    蔡志钢; 靳伟; 张卓然

    2011-01-01

    Objective To investigate the clinical features and manifests of chest CT of allergic bronchopulmonary aspergilosis (ABPA) to reduce the misdiagnosis. Methods The clinical and eikonic data of 4 patients with ABPA diagnosed in our hospital were retrospectively analyzed. Results 4 patients had different degrees of cough and expectoration, and they were misdiagnosed as bronchiectasis with infection, bronchial asthma, pulmonary tuberculosis, and pneumonia respectively. Chest CT mainly showed central bronchiectasis and bronchial mucus plug forming. Peripheral blood eosinophils and serum IgE increased. Serum IgE-Af showed medium saitivity. Intracutaneous test of aspergillus fumigatus antigen showed quick positive reaction. All the patients were diagnosed as having ABPA. Conclusion The diagnosis of ABPA can be suggested when central bronchiectasis and bronchial mucus plug forming appear in multiple lung fields in the patients with long term of gasping and cough, and combined serological test is necessary in diagnosis.%目的 探讨变应性肺曲菌病(allergic bronchopulmonary aspergilosis,ABPA)的临床特点及胸部CT表现,减少误诊误治.方法 回顾性分析我院明确诊断4例ABPA临床及影像学资料.结果 4例均有不同程度的咳嗽、咳痰,分别误诊为支气管扩张并感染、支气管哮喘、肺结核和肺炎.胸部CT主要表现为中心性支气管扩张和支气管黏液栓形成;实验室检查表现为外周血嗜酸粒细胞增高,血清IgE升高,血清IgE-Af中度敏感,烟曲菌抗原皮内试验呈速发反应阳性,均确诊ABPA.结论 在长期喘息、咳嗽的患者中出现中上肺野多发的中心性支气管扩张及支气管腔内黏液栓形成可提示ABPA,确诊需结合血清学检查.

  20. HRCT findings of asthmatic children under maintenance therapy

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Hyun Sook; Park, Jai Soung; Goo, Dong Erk; Lee, Hae Kyung; Kwon, Kui Hyang; Choi, Deuk Lin; Pyun, Bok Yang [Soonchunhyang University Hospital, Seoul (Korea, Republic of)

    2000-05-01

    The purpose of this study was to evaluate the HRCT findings of bronchial asthma during maintenance bronchodilator therapy and to determine whether there were irreversible bronchial changes occurred in pediatric patients with this condition. HRCT findings of the lung in 21 asthmatic children (14 boys and 7 girls aged between 3.5 and 13.8 (mean: 7.7) years) who were receiving maintenance bronchodilator therapy were retrospectively studied. At the time of CT examination, 16 were receiving nonsteroid bronchodilator therapy only, and five were receiving both bronchodilator and steroid therapy. Thirteen patients were defined as allergic and eight were nonallergic. The clinical severity of chronic asthma was graded as severe in seven cases, and moderate in 14. The duration of the disease ranged from 4 months to 6 years (mean 3.2 years). HRCT was performed in 19 cases for evaluation of the atelectasis, hyperinflation, and prominent bronchovascular bundles seen on plain radiographs, and in two cases for evaluation following acute exacerbation. A CT W-2000 scanner (Hitachi Medical Co. Tokyo, Japan) was used during the end inspiratory phase, and in addition, ten patients were scanned during the expiratory phase. Scans were reviewed for evidence of bronchial thickening, bronchiectasis, emphysema, abnormal density, mucus plugs, and other morphological abnormalities. The presence of bronchial wall thickening or air trapping was evaluated according to the duration, severity and type of asthma. Among the 21 patients, 7 (33.3%) had normal HRCT findings, while in 14 (66.7%), bronchial wall thickening was demonstrated. Eleven of the 14 patients with bronchial wall thickening(78.6%) also had air trapping. No patient was suffering from bronchiectasis or emphysema. There were no statistically significant correlations between the presence of bronchial wall thickening or air trapping and the duration of the disease, its severity, or type of asthma. There was, however, a statistically

  1. Investigation of common pathogenic bacteria and drug resistance in gerontism with bronchieetasis concurrent infection.%老年支气管扩张合并感染的常见病原菌及其耐药性调查

    Institute of Scientific and Technical Information of China (English)

    许立; 张锡林

    2011-01-01

    目的 调查分析老年支气管扩张合并感染的常见病原菌及其耐药情况,为临床合理应用抗菌药物提供依据.方法 对189例老年支气管扩张合并感染的住院患者的痰培养和药敏结果进行回顾性调查分析.结果 189例患者病原菌检测阳性122例,阳性率为64.6%,病原菌以革兰阴性杆菌为和真菌为主,分别占59.5%和10.8%;主要致病的革兰阴性杆菌对头孢呋辛、氨苄西林等药物耐药率高,对β-内酰胺类、碳青酶烯类、氨基糖甙类和喹诺酮类药物的耐药率相对较低.结论 老年支气管扩张合并感染病原菌以革兰阴性杆菌和真菌为主要条件致病菌,主要致病菌耐药率高,建议在药敏指导下用药.在未取得细菌药敏结果前可根据其病原菌分布特点经验性选择抗生素,宜采用联合抗菌药物治疗.%To investigate common pathogenic bacteria and drug resistance m gerontism with bronchiectasis concurrent infection, to make reasonable use of antibacterials. Methods Retrospectively analyzed sputum culture and drug sensitivity of 189 old patients with bronchiectasis concurrent infection. Results 122 people had masculine pathogenic bacteria, the masculine rate was 64.6%, then the rate of gram negative bacilli (GNB) was 59.5%, fungus was 10.8%; the drug resistance rate of GNB to Cefuroxime and Ampicillin was high, but to β-lactam antibiotics, carbapenem antibiotics, aminoglycosides and quinolones was low. Conclusion The main pathogenic bacteria in gerontism with bronchiectasis concurrent infection was gram negative bacilli and fungus, the rate of drug resistance was high, used antibiotics on the basis of drug sensitivity, selected antibiotics on the basis of the disposition feature of pathogenic bacteria, there was the best to use combination antibacterial drug therapy.

  2. Genetic factors contributing to human primary ciliary dyskinesia and male infertility.

    Science.gov (United States)

    Ji, Zhi-Yong; Sha, Yan-Wei; Ding, Lu; Li, Ping

    2016-06-07

    Primary ciliary dyskinesia (PCD) is an autosomal-recessive disorder resulting from the loss of normal ciliary function. Symptoms include neonatal respiratory distress, chronic sinusitis, bronchiectasis, situs inversus, and infertility. However, only 15 PCD-associated genes have been identified to cause male infertility to date. Owing to the genetic heterogeneity of PCD, comprehensive molecular genetic testing is not considered the standard of care. Here, we provide an update of the progress on the identification of genetic factors related to PCD associated with male infertility, summarizing the underlying molecular mechanisms, and discuss the clinical implications of these findings. Further research in this field will impact the diagnostic strategy for male infertility, enabling clinicians to provide patients with informed genetic counseling, and help to adopt the best course of treatment for developing directly targeted personalized medicine.

  3. AN INTERESTING CASE OF PNEUMOTHORAX IN RHEUMATOID ARTHRITIS

    Directory of Open Access Journals (Sweden)

    Shruthi

    2014-11-01

    Full Text Available : Rheumatoid Arthritis is a multisystem disease with pulmonary manifestations including idiopathic pulmonary fibrosis, obliterative bronchiolitis, pneumonitis, bronchiectasis as well as pneumothorax. Cases of rheumatoid arthritis with active tuberculosis disease have been documented as well as reactivation of latent tuberculosis as a result of methotrexate therapy with or without systemic steroids has led to a renewed interest in the association of the two diseases. We report the case of rheumatoid arthritis in a young female patient who presented with cough and fever of fortnight duration and rapidly developed spontaneous pneumothorax that eventually required surgical pneumonectomy. CONCLUSION: Even though patients with rheumatoid arthritis can present with spontaneous pneumothorax due to rupture of rheumatic nodules, Common diseases like tuberculosis should be kept in mind while treating pulmonary complaints in patients with rheumatoid arthritis, especially those who are on methotrexate therapy

  4. The non-antibacterial effects and possible clinical applications of macrolide antibiotics

    Institute of Scientific and Technical Information of China (English)

    TONG Shi-liang; WANG Yuan-liang; GAO Wen-juan

    2006-01-01

    As one group of the most widely used oral antibacterial drugs, macrolides have been clinically applied for more than 50 years because of their safety and efficacy. Recently, the non-antibacterial applications of macrolides are frequently reported, such as the therapy of malignant tumor, exudative pleurisy, pulmonary fibrosis, rheumatoid arthritis, bronchiectasis, asthma, gastrointestinal peristalsis disorders, and so on with new pharmacological effects, including reverse of tumor resistance, anti-chemical adherence, anti-fibrosis, and modulations of gastrointestinal motility as well as immune activity. The in-depth research of marcolides' non-antibiotic actions can not only extend the application fields of macrolides, but provide a new way to treat some refractory diseases.

  5. The clinical significance of HRCT in evaluation of patients with rheumatoid arthritis-associated interstitial lung disease: a report from China.

    Science.gov (United States)

    Zou, Yu-Qiong; Li, Ya-Song; Ding, Xiao-Nan; Ying, Zhen-Hua

    2012-03-01

    The objective of this study is to describe the interstitial lung disease (ILD) in rheumatoid arthritis (RA) patients of China, and to study clinical significance of high-resolution computed tomography (HRCT) in evaluation and treatment. One hundred and ten Chinese patients (79 women and 31 man) diagnosed with RA between December 2008 to November 2009 were analyzed. According to the HRCT, 47 (42.73%) RA patients were diagnosed as ILD. Old age, smoking and pulmonary rales were closely related to ILD (P pulmonary nodules (11.82%), emphysema (9.09%), bronchiectasis (3.64%), subpleural nodules (11.82%) and pleural thickening (24.55%). In treatment, honeycombing and subpleural nodules were more common in patients with methotrexate (MTX) and/or leflunomide treatment than without (P 0.05). Pulmonary involvement is common in RA patients, and it is suggested that HRCT could be a sensitive and useful way in evaluating the lung of RA patients.

  6. Clinical application of exhaled nitric oxide measurement in pediatric lung diseases

    Directory of Open Access Journals (Sweden)

    Manna Angelo

    2012-12-01

    Full Text Available Summary Fractional exhaled nitric oxide (FeNO is a non invasive method for assessing the inflammatory status of children with airway disease. Different ways to measure FeNO levels are currently available. The possibility of measuring FeNO levels in an office setting even in young children, and the commercial availability of portable devices, support the routine use of FeNO determination in the daily pediatric practice. Although many confounding factors may affect its measurement, FeNO is now widely used in the management of children with asthma, and seems to provide significantly higher diagnostic accuracy than lung function or bronchial challenge tests. The role of FeNO in airway infection (e.g. viral bronchiolitis and common acquired pneumonia, in bronchiectasis, or in cases with diffuse lung disease is less clear. This review focuses on the most recent advances and the current clinical applications of FeNO measurement in pediatric lung disease.

  7. The significance of ultrastructural abnormalities of human cilia.

    Science.gov (United States)

    Fox, B; Bull, T B; Makey, A R; Rawbone, R

    1981-12-01

    The electronmicroscopic structure of cilia was studied from the inferior turbinate of the nose in 22 adults, and in 84 biopsies from the bronchial tree of 40 adults. The incidence of compound cilia and abnormal microtubular structures was assessed. There were significant variations in the incidence of abnormalities in different parts of the airways and even within different areas of the same electronmicroscopic section. The focal nature of differences in structure of cilia indicate that abnormalities found in a single biopsy do not necessarily reflect a generalized change in the bronchial tree. Thus, such a finding should not be used as evidence that the abnormalities of cilia are the cause of decrease in mucociliary clearance or that they play a role in the pathogenesis of bronchiectasis and sinusitis.

  8. The involvement of glycosaminoglycans in airway disease associated with cystic fibrosis.

    LENUS (Irish Health Repository)

    Reeves, Emer P

    2012-02-01

    Individuals with cystic fibrosis (CF) present with severe airway destruction and extensive bronchiectasis. It has been assumed that these structural airway changes have occurred secondary to infection and inflammation, but recent studies suggest that glycosaminoglycan (GAG) remodelling may be an important independent parallel process. Evidence is accumulating that not only the concentration, but also sulphation of GAGs is markedly increased in CF bronchial cells and tissues. Increased expression of GAGs and, in particular, heparan sulphate, has been linked to a sustained inflammatory response and neutrophil recruitment to the CF airways. This present review discusses the biological role of GAGs in the lung, as well as their involvement in CF respiratory disease, and their potential as therapeutic targets.

  9. 咳嗽从瘀血论治经验%The experiences of the treatment on cough from blood stasis

    Institute of Scientific and Technical Information of China (English)

    马献中; 王世彪; 王保平

    2014-01-01

    Cough lung disease is one of the most common syndromes caused by many reasons, cough, a cough and internal cough points, most doctors more responsibility in the lung, spleen, liver, kidney damage, or due to feel the wind, cold, heat, dryness, fire evil and hair, or phlegm due to spleen deficiency, liver fire attacking lung, deficiency of kidney qi and other causes. In recent years, the study found, blood stasis caused one of the main causes of chronic cough, especially in chronic cough patients. Our clinical treatment of chronic bronchitis, cough asthma, pulmonary heart disease, bronchiectasis patients, each with blood stasis of TCM, often obtain good effect. The blood stasis cough experience summarized as follows.

  10. Kartagener's syndrome presented with nasal obstruction: A case report

    Directory of Open Access Journals (Sweden)

    Suna Asilsoy

    2014-08-01

    Full Text Available The nasal polyposis is a chronic inflammatory process of the nasal mucosa. Although it is rare in children, there may be also association with cystic fibrosis and primary ciliary dyskinesia. About 50% of primary ciliary dyskinesia patients develop situs inversus and it is known as Kartagener's syndrome. The Kartagener's sydrome is a rare autosomal recessive disorder characterized by sinusitis, bronchiectasis, situs inversus. Clinically, patients present to the otolaryngologist with nasal obstruction. We as pediatricians, should consider nasal polyposis as a rare cause of nasal obstruction in children. In the presence of recurrent upper and lower respiratory tract infections accompanying nasal polyposis, Kartagener's syndrome must be kept in mind as a rare reason. [Cukurova Med J 2014; 39(4.000: 942-945

  11. Tracheobronchomegaly with multiple diverticula: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Chan Sup; Kim, Joung Sook [College of Medicine, Inha University, Incheon (Korea, Republic of)

    1993-01-15

    Chest radiograhs revealed evidences of dilatation of the trachea and both main bronchi, multiple air bubbles around the trachea and focal bronchopneumonia in right lower lobe. Tracheobronchomegaly is a rare condition characterized by marked dilatation of the trachea and main bronchi and frequently associated with tracheal diverticulosis, bronchiectasis, and recurrent lower respiratory tract infection. Mounier-Kuhn, in 1932, was the first to associate the presence of tracheobronchomegaly with the clinical syndrome of chronic, recurrent respiratory tract infection. Tracheobronchomegaly is believed to be extremely rare. To date, only 92 cases have been reported in the literatures. We recently encountered a case of tracheobronchomegaly associated with multiple diverticula formation. The diagnosis was made by chest radiography, computed tomography (CT), bronchography, and CT taken immediately after the bronchography.

  12. Correlation between computed tomographic and magnetic resonance imaging findings of parenchymal lung diseases

    Energy Technology Data Exchange (ETDEWEB)

    Barreto, Miriam Menna; Rafful, Patricia Piazza [Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro (Brazil); Rodrigues, Rosana Souza [Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro (Brazil); D’Or Institute for Research and Education, Rio de Janeiro, RJ (Brazil); Zanetti, Gláucia [Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro (Brazil); Hochhegger, Bruno [Complexo Hospitalar Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, RS (Brazil); Souza, Arthur Soares [Department of Radiology, Medical School of Rio Preto (FAMERP) and Ultra X, São José do Rio Preto, SP (Brazil); Guimarães, Marcos Duarte [Department of Imaging, Hospital AC Camargo, São Paulo, SP (Brazil); Marchiori, Edson, E-mail: edmarchiori@gmail.com [Department of Radiology, Federal University of Rio de Janeiro, Rio de Janeiro (Brazil)

    2013-09-15

    Computed tomography (CT) is considered to be the gold standard method for the assessment of morphological changes in the pulmonary parenchyma. Although its spatial resolution is lower than that of CT, MRI offers the advantage of characterizing different aspects of tissue based on the degree of contrast on T1-weighted image (WI) and T2-WI. In this article, we describe and correlate the MRI and CT features of several common patterns of parenchymal lung disease (air trapping, atelectasis, bronchiectasis, cavitation, consolidation, emphysema, ground-glass opacities, halo sign, interlobular septal thickening, masses, mycetoma, nodules, progressive massive fibrosis, reverse halo sign and tree-in-bud pattern). MRI may be an alternative modality for the collection of morphological and functional information useful for the management of parenchymal lung disease, which would help reduce the number of chest CT scans and radiation exposure required in patients with a variety of conditions.

  13. What is the importance of classifying Aspergillus disease in cystic fibrosis patients?

    Science.gov (United States)

    Jones, Andrew M; Horsley, Alex; Denning, David W

    2014-08-01

    Aspergillus species are commonly isolated from lower respiratory tract samples of patients with cystic fibrosis (CF) and markers of immunological sensation to Aspergillus are frequently encountered in this group of patients; however, the contribution of Aspergillus to CF lung disease outside of the typical complications of ABPA and aspergilloma formation remains largely unclear. Patients with CF show discretely different responses to Aspergillus, though the underlying reasons for this variation are unknown. Recent work has begun to allow us to categorize patient responses to Aspergillus based upon molecular markers of infection and immune sensitization. Aspergillus sensitization and/or airway infection is associated with worse FEV1, in CF and other patients (asthma, chronic obstructive pulmonary disease, bronchiectasis). Classification of different clinical phenotypes of Aspergillus will enable future studies to determine the natural history of different manifestations of Aspergillus disease and evaluate the effects of intervention with antifungal therapy.

  14. High-attenuation mucus plugs on MDCT in a child with cystic fibrosis: potential cause and differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Morozov, Andrey; Brown, Shanaree [Indiana University Medical School, Indianapolis, IN (United States); Applegate, Kimberly E. [Riley Hospital for Children, Department of Radiology, Indiana University Medical Center, Indianapolis, IN (United States); Howenstine, Michelle [Riley Hospital for Children, Department of Pulmonology, Indiana University Medical Center, Indianapolis, IN (United States)

    2007-06-15

    High-attenuation mucus plugging is a rare finding in both adults and children. When it occurs, the field of differential diagnoses is typically quite small and includes acute hemorrhage, aspiration of radiodense material, and allergic bronchopulmonary aspergillosis (ABPA). The last of these three diagnoses is the most difficult to make, although ABPA is more commonly seen in children with cystic fibrosis (CF) or asthma. ABPA is radiographically characterized by recurrent mucus plugging, atelectasis, and central bronchiectasis. Thus far, high-attenuation mucus plugs have only been reported in adults. We report a rare case of a child with CF who had high-attenuation mucus plugs and atelectasis that raised the possibility of ABPA. We discuss the differential diagnoses of this finding and the role of multidetector CT in these children. (orig.)

  15. Clinical Management of Acute Interstitial Pneumonia: A Case Report

    Directory of Open Access Journals (Sweden)

    Yang Xia

    2012-01-01

    Full Text Available We describe a 51-year-old woman who was admitted to hospital because of cough and expectoration accompanied with general fatigue and progressive dyspnea. Chest HRCT scan showed areas of ground glass attenuation, consolidation, and traction bronchiectasis in bilateral bases of lungs. BAL fluid test and transbronchial lung biopsy failed to offer insightful evidence for diagnosis. She was clinically diagnosed with acute interstitial pneumonia (AIP. Treatment with mechanical ventilation and intravenous application of methylprednisolone (80 mg/day showed poor clinical response and thus was followed by steroid pulse therapy (500 mg/day, 3 days. However, she died of respiratory dysfunction eventually. Autopsy showed diffuse alveolar damage associated with hyaline membrane formation, pulmonary interstitial, immature collagen edema, and focal type II pneumocyte hyperplasia.

  16. Organising pneumonia in common variable immunodeficiency.

    Science.gov (United States)

    Boujaoude, Ziad; Arya, Rohan; Rafferty, William; Dammert, Pedro

    2013-06-07

    Common variable immunodeficiency (CVID) is the most common of the primary immunodeficiency disorders. Pulmonary manifestations are characterised by recurrent rhinosinusitis, respiratory tract infections and bronchiectasis. Less commonly the lung may be affected by lymphoid disorders and sarcoid-like granulomas. Organising pneumonia (OP) is a rare pulmonary manifestation. We report the case of a 32-year-old woman with CVID who presented with fever, dyspnoea and persistent lung infiltrates despite antibiotic therapy. CT of the chest showed bilateral patchy alveolar infiltrates. Pulmonary function tests revealed moderate restriction and reduction in diffusion capacity. Initial bronchoscopy with transbronchial biopsies did not yield a diagnosis but surgical lung biopsies identified OP. Significant clinical, radiographic and physiological improvement was achieved after institution of corticosteroid therapy.

  17. [Hot topics in respiratory infections].

    Science.gov (United States)

    de Souza-Galvao, M Luiza; García-Martínez, Miguel Ángel; Sanz, Francisco; Blanquer, José

    2011-01-01

    We review the most interesting articles on respiratory infections published in the last trimester of 2009 and in 2010. Notable publications in bronchiectasis were the Guidelines of the British Thoracic Society, as well as several articles on the natural course of the process, the impact of exacerbations on the course of the disease, and treatment with inhaled antibiotics. Other notable publications were the SEPAR-SEIMC consensus document for the management of tuberculosis and articles on the use of interferon-gamma in the diagnosis of tuberculosis infection. The new recommendations of the Spanish Society of Pneumology and Thoracic Surgery on community-acquired pneumonia have recently been published. Equally important are studies on the viral etiology of community-acquired pneumonia, the impact of corticosteroid treatment in pneumonia, the duration of antibiotic therapy and preventive measures in both community-acquired and nosocomial pneumonia.

  18. Evidence from Chile that arsenic in drinking water may increase mortality from pulmonary tuberculosis.

    Science.gov (United States)

    Smith, Allan H; Marshall, Guillermo; Yuan, Yan; Liaw, Jane; Ferreccio, Catterina; Steinmaus, Craig

    2011-02-15

    Arsenic in drinking water causes increased mortality from several cancers, ischemic heart disease, bronchiectasis, and other diseases. This paper presents the first evidence relating arsenic exposure to pulmonary tuberculosis, by estimating mortality rate ratios for Region II of Chile compared with Region V for the years 1958-2000. The authors compared mortality rate ratios with time patterns of arsenic exposure, which increased abruptly in 1958 in Region II and then declined starting in 1971. Tuberculosis mortality rate ratios in men started increasing in 1968, 10 years after high arsenic exposure commenced. The peak male 5-year mortality rate ratio occurred during 1982-1986 (rate ratio = 2.1, 95% confidence interval: 1.7, 2.6; P cause of chronic lung disease. Finding weaker associations in women is unsurprising, because this is true of most arsenic-caused health effects. Confirmatory evidence is needed from other arsenic-exposed populations.

  19. Animal models for diseases of respiratory system

    Directory of Open Access Journals (Sweden)

    R. Adil

    2012-07-01

    Full Text Available Latest trends in understanding of respiratory diseases in human beings can be derived from thorough clinical studies of these diseases occurring in man, but conducting such studies in man is difficult in terms of experimental manipulation. In the last 2 decades, various types of experimental respiratory disease models has been developed and utilized by investigators, which have contributed a lot to the understanding of respiratory diseases in man, but only little investigation has been done on the naturally occurring pulmonary diseases of animals as potential models which could have added to our knowledge. There are certain selected examples of spontaneous pulmonary disease in animals that may serve as exploitable models for human chronic bronchitis, bronchiectasis, emphysema, interstitial lung disease, hypersensitivity pneumonitis, hyaline membrane disease, and bronchial asthma.

  20. CT features of lymphobronchial tuberculosis in children, including complications and associated abnormalities

    Energy Technology Data Exchange (ETDEWEB)

    Lucas, Susan; Andronikou, Savvas [Faculty of Health Sciences, University of the Witwatersrand, Department of Radiology, Johannesburg (South Africa); Goussard, Pierre; Gie, Robert [Stellenbosch University, Department Paediatrics, Stellenbosch (South Africa)

    2012-08-15

    Lymphobronchial tuberculosis (TB) is tuberculous lymphadenopathy involving the airways, which is particularly common in children. To describe CT findings of lymphobronchial TB in children, the parenchymal complications and associated abnormalities. CT scans of children with lymphobronchial TB were reviewed retrospectively. Lymphadenopathy, bronchial narrowing, parenchymal complications and associations were documented. Infants comprised 51% of patients. The commonest site of lymphadenopathy was the subcarinal mediastinum (97% of patients). Bronchial compression was seen in all children (259 bronchi, of these 28% the bronchus intermedius) with severe or complete stenosis in 23% of affected bronchi. Parenchymal complications were present in 94% of patients, including consolidation (88%), breakdown (42%), air trapping (38%), expansile pneumonia (28%), collapse (17%) and bronchiectasis (9%), all predominantly on the right side (63%). Associated abnormalities included ovoid lesions, miliary nodules, pleural disease and intracavitary bodies. Airway compression was more severe in infants and most commonly involved the bronchus intermedius. Numerous parenchymal complications were documented, all showing right-side predominance. (orig.)

  1. Recognizing asthma mimics and asthma complications.

    Science.gov (United States)

    Amundson, Dennis; Seda, Gilbert; Daheshia, Massoud

    2011-10-01

    Asthma is a chronic inflammatory disorder of the airways characterized by airflow obstruction, bronchial hyperreactivity, and underlying inflammation. Two common reasons asthmatics fail standard therapy are incorrect diagnosis and failure to recognize underlying contributing factors. A correct diagnosis of asthma is of great importance to military practitioners since misdiagnosis or uncontrolled asthma affects an individual's operational readiness or determines whether one can receive a medical waiver to enlist in military service. This article presents four cases of patients with dyspnea that have conditions which mimic asthma or complicate asthma management: vocal cord dysfunction misdiagnosed as asthma, respiratory bronchiolitis interstitial lung disease mistaken as asthma, difficult-to-control asthma because of bronchiectasis and allergic bronchopulmonary aspergillosis, and difficult and fatal asthma. Asthma is contrasted to other respiratory disorders, and an outlined approach to asthma diagnosis and management is presented using the Global Initiative for Asthma guidelines.

  2. Sarcoidosis of the upper and lower airways.

    Science.gov (United States)

    Morgenthau, Adam S; Teirstein, Alvin S

    2011-12-01

    Sarcoidosis is a systemic granulomatous disease of undetermined etiology characterized by a variable clinical presentation and disease course. Although clinical granulomatous inflammation may occur within any organ system, more than 90% of sarcoidosis patients have lung disease. Sarcoidosis is considered an interstitial lung disease that is frequently characterized by restrictive physiologic dysfunction on pulmonary function tests. However, sarcoidosis also involves the airways (large and small), causing obstructive airways disease. It is one of a few interstitial lung diseases that affects the entire length of the respiratory tract - from the nose to the terminal bronchioles - and causes a broad spectrum of airways dysfunction. This article examines airway dysfunction in sarcoidosis. The anatomical structure of the airways is the organizational framework for our discussion. We discuss sarcoidosis involving the nose, sinuses, nasal passages, larynx, trachea, bronchi and small airways. Common complications of airways disease, such as, atelectasis, fibrosis, bullous leions, bronchiectasis, cavitary lesions and mycetomas, are also reviewed.

  3. Inflammation and its genesis in cystic fibrosis.

    Science.gov (United States)

    Nichols, David P; Chmiel, James F

    2015-10-01

    The host inflammatory response in cystic fibrosis (CF) lung disease has long been recognized as a central pathological feature and an important therapeutic target. Indeed, many believe that bronchiectasis results largely from the oxidative and proteolytic damage comprised within an exuberant airway inflammatory response that is dominated by neutrophils. In this review, we address the longstanding argument of whether or not the inflammatory response is directly attributable to impairment of the cystic fibrosis transmembrane conductance regulator or only secondary to airway obstruction and chronic bacterial infection and challenge the importance of this distinction in the context of therapy. We also review the centrality of neutrophils in CF lung pathophysiology and highlight more recent data that suggest the importance of other cell types and signaling beyond NF-κB activation. We discuss how protease and redox imbalance are critical factors in CF airway inflammation and end by reviewing some of the more promising therapeutic approaches now under development.

  4. Computer-based image analysis in radiological diagnostics and image-guided therapy 3D-Reconstruction, contrast medium dynamics, surface analysis, radiation therapy and multi-modal image fusion

    CERN Document Server

    Beier, J

    2001-01-01

    This book deals with substantial subjects of postprocessing and analysis of radiological image data, a particular emphasis was put on pulmonary themes. For a multitude of purposes the developed methods and procedures can directly be transferred to other non-pulmonary applications. The work presented here is structured in 14 chapters, each describing a selected complex of research. The chapter order reflects the sequence of the processing steps starting from artefact reduction, segmentation, visualization, analysis, therapy planning and image fusion up to multimedia archiving. In particular, this includes virtual endoscopy with three different scene viewers (Chap. 6), visualizations of the lung disease bronchiectasis (Chap. 7), surface structure analysis of pulmonary tumors (Chap. 8), quantification of contrast medium dynamics from temporal 2D and 3D image sequences (Chap. 9) as well as multimodality image fusion of arbitrary tomographical data using several visualization techniques (Chap. 12). Thus, the softw...

  5. Role of inhaled amphotericin in allergic bronchopulmonary aspergillosis

    Directory of Open Access Journals (Sweden)

    I S Sehgal

    2014-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is an immunological pulmonary disorder caused by immune reactions mounted against the ubiquitous fungus Aspergillus fumigatus. The disease clinically manifests with poorly controlled asthma, hemoptysis, systemic manifestations like fever, anorexia and weight loss, fleeting pulmonary opacities and bronchiectasis. The natural course of the disease is characterized by repeated episodes of exacerbations. Almost 30-40% of the patients require prolonged therapy, which currently consists of corticosteroids and anti-fungal azoles; both these agents have significant adverse reactions. Amphotericin B administered via the inhaled route can achieve a high concentration in the small airways with minimal systemic side-effects. Nebulized amphotericin B has been used in the management of invasive pulmonary aspergillosis. The aim of this review is to study the utility of inhaled amphotericin in ABPA.

  6. Hypersensitivity pneumonitis (extrinsic allergic alveolitis): high-resolution computed tomography findings; Pneumonite por hipersensibilidade (alveolite alergica extrinseca): achados na tomografia computadorizada de alta resolucao

    Energy Technology Data Exchange (ETDEWEB)

    Almeida Junior, Jose Guiomar de; Marchiori, Edson [Universidade Federal Fluminense, Niteroi, RJ (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia]. E-mail: edmarchiori@zipmail.com.br; Escuissato, Dante L. [Parana Univ., Curitiba, PR (Brazil). Dept. de Radiologia; Souza Junior, Arthur Soares [Faculdade de Medicina de Sao Jose do Rio Preto (FAMERP), SP (Brazil). Dept. de Radiologia; Gasparetto, Emerson L. [Parana Univ., Curitiba, PR (Brazil). Hospital de Clinicas; Nobre, Luiz Felipe [Santa Catarina Univ., Florianopolis, SC (Brazil); Irion, Klaus L. [Santa Casa de Misericordia de Porto Alegre, RS (Brazil). Pavilhao Pereira Filho. Servico de Radiologia

    2003-12-01

    Hypersensitivity pneumonitis, or extrinsic allergic alveolitis, is an immunologic disease of the lungs caused by inhaled chemicals or organics allergens. A lymphocytic inflammatory response in the peripheral airways and surrounding interstitial tissue occurs. In this study the high-resolution computed tomography findings of 13 patients with hypersensitivity pneumonitis were analyzed and discussed. The most frequent high-resolution computed tomography findings were: ground-glass opacities (92.3%), centrilobular nodules (38.4%) and air trapping (38.4%). Other findings included bronchiectasis (23.1%), consolidation (23.1%), crazy paving (7.7%), parenchymal bands (15.4%), linear opacities (7.7%), architectural distortion (7.7%), tracheal dilatation (7.7%), intralobular reticulate (7.7%), honeycombing (7.7%), emphysema (7.7%) and atelectasis (7.7%). In two of the 13 patients there was fibrosis (architectural distortion and honeycombing), which represents the chronic phase of the disease. (author)

  7. Allergic bronchopulmonary aspergillosis in garden waste (compost) collectors--occupational implications.

    Science.gov (United States)

    Poole, C J M; Wong, M

    2013-10-01

    The separation of rotting garden material from general domestic waste and its collection for processing in industrial composting sites is a relatively new industry in the UK. Two cases of allergic bronchopulmonary aspergillosis and the results of health surveillance are described in a team of 28 garden waste (compost) collectors. A few cases of extrinsic allergic alveolitis due to Aspergillus fumigatus have previously been reported in compost workers. In the absence of any guidance from research and to prevent similar cases of a potentially serious illness, we advise that new starters to the job of collecting or processing compost are screened for asthma and aspergillus sensitivity, cystic fibrosis, bronchiectasis and immunodeficiency if their exposure to high levels of Aspergillus sp cannot be controlled. Annual health surveillance for these workers is also recommended.

  8. Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome.

    Science.gov (United States)

    Kim, Jung Hoon; Song, Won Jun; Jun, Ji Eun; Ryu, Duck Hyun; Lee, Ji Eun; Jeong, Ho Jung; Jeong, Suk Hyeon; Kang, Hyung Koo; Kim, Jung Soo; Lee, Hyun; Chon, Hae Ri; Jeon, Kyeongman; Kim, Dohun; Kim, Jhingook; Koh, Won-Jung

    2014-09-01

    Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.

  9. Anaesthetic Management of a Patient with Synchronous Kartagener Syndrome and Biliary Atresia.

    Science.gov (United States)

    Kendigelen, Pınar; Tütüncü, Ayşe Çiğdem; Erbabacan, Şafak Emre; Kaya, Güner; Altındaş, Fatiş

    2015-06-01

    Kartagener syndrome is an autosomal recessive disorder characterized by primary ciliary dyskinesia accompanied by sinusitis, bronchiectasis, and situs inversus. Synchronous extrahepatic biliary atresia and Kartagener syndrome are very rare. During the preoperative preparation of patients with Kartagener syndrome, special attention is required for the respiratory and cardiovascular system. It is important to provide suitable anaesthetic management to avoid problems because of ciliary dysfunction in the perioperative period. Further, maintaining an effective pain control with regional anaesthetic methods reduces the risk of pulmonary complications. Infants with biliary atresia operated earlier have a higher chance of survival. Hepatic dysfunction and decrease in plasma proteins are important for the kinetics of drugs. In this presentation, the anaesthetic management of patients with synchronous Kartagener syndrome and biliary atresia, both of which are rare diseases, is evaluated.

  10. Amyotrophic lateral sclerosis in a patient with Kartagener syndrome.

    Science.gov (United States)

    Yamashita, Satoshi; Migita, Akie; Hayashi, Kenyu; Hirahara, Tomoo; Kimura, En; Maeda, Yasushi; Hirano, Teruyuki; Uchino, Makoto

    2010-08-01

    We present a case of a patient with clinically definite ALS, who had earlier suffered from Kartagener syndrome, which is characterized by the triad comprising chronic sinusitis, bronchiectasis, and situs inversus. Recent linkage and mutational analyses identified several genes that are responsible for Kartagener syndrome. Most of them encode subunits of axonemal dyneins, highlighting the importance of dynein motors to ciliary motility. Recent data indicate that defects in cytoplasmic dynein-mediated retrograde axonal transport are involved in the etiology of ALS. Genes encoding the dynein heavy chain of cytoplasmic and outer arm axonemal dyneins are reported to have similar sequences in their central and 3'-end regions. Although a causal link between ALS and Kartagener syndrome has not yet been definitely established, the precise relationship between disrupted axonemal dynein function in Kartagener syndrome and motor neuron death should be investigated.

  11. Kartagener′s syndrome: A case series

    Directory of Open Access Journals (Sweden)

    Mayank Mishra

    2012-01-01

    Full Text Available Kartagener′s syndrome is a rare, autosomal recessive genetic ciliary disorder comprising the triad of situs inversus, chronic sinusitis, and bronchiectasis. The basic problem lies in the defective movement of cilia, leading to recurrent chest infections, ear/nose/throat symptoms, and infertility. We hereby report three unusual cases of this rare entity - an infertile male with azoospermia in whom Bochdalek′s diaphragmatic hernia coexisted, another case of an infertile female, and a third of an infertile male with oligospermia. The need for a high index of suspicion to make an early diagnosis cannot be overemphasized in such patients so that wherever possible, options for timely treatment of infertility may be offered and unnecessary evaluation of symptoms is avoided.

  12. [Difficult airway management for general anesthesia in two patients with Kartagener syndrome].

    Science.gov (United States)

    Andoh, Taiki; Momota, Yoshihiro; Murata, Kenji; Kotani, Junichiro

    2010-05-01

    Two patients with Kartagener syndrome were managed under general anesthesia by nasal intubation for sagital split ramus osteotomy. Many episodes of expectoration were encountered in the former patient's perioperative period and the expectoration discharge was inadequate by postoperative nausea, leading to trouble in airway management. In the second patient, tube collapse was caused after intubation by serious nasal cavity strangulation, and re-intubation was necessary. Expectoration is seen resulting from decreased ciliary function with bronchiectasis during anesthetic management of patients with Kartagener syndrome. It is important to prevent lung complications by nausea prevention and pain killing in the postoperative period, in addition to proper suctioning in the perioperative period. Furthermore, there is nasal cavity narrowing by chronic sinusitis. When performing nasal intubation, the difficult airway management is required.

  13. [Kartagener syndrome and papillary thyroid carcinoma: an unusual combination].

    Science.gov (United States)

    Ren, Jingyuan; Wang, Xurui; He, Zhongyin

    2015-11-01

    A case of a papillary thyroid carcinoma in a patient with situs inversus with associated bronchiectasis and chronic sinusitis (Kartagener's syndrome) is reported. A 61-year-old male patient has the symptoms of nasal obstruction. nasal purulent discharge and headache for 2 years. Physical examination: right nasal purulent in right nasal cavity and multiple lychee-like opaque mass in right middle meatus. A nodule, one centimeter in diameter, locates in the upper pole of right thyroid. Evidence of full situs inversus viscerum can be confirmmed by chest radiographs and ultrasound doppler. Pathology: right nasal polyps, the right small papillary thyroid cancer. TEM Tip primary ciliary dyskinesia. Clinical diagnosis: Kartagener syndrome, papillary thyroid carcinoma (T1a N0 M0, I period), chronic sinusitis-nasal polyps.

  14. Adenosquamous carcinoma of paranasal sinuses and Kartagener syndrome: an unusual combination.

    Science.gov (United States)

    Naqvi, Syeda Uzma; Hussain, Syed Iqbal; Quadri, Shaheen

    2014-03-01

    A 34 years old non-smoker male patient reported with growth of right maxillary region which on histopathology confirmed adenosquamous carcinoma of nose and paranasal sinus. Patient also had total situs inversus including dextrocardia, bronchiectasis and sinusitis. His blood group was AB negative. This association of Kartagener syndrome with adenosquamous carcinoma of paranasal sinuses has never been reported. Carcinoma of paranasal sinuses accounts only 0.3% of all cancers. Adenosquamous carcinoma makes only 2% of the nose and paranasal sinuses tumours. Kartagener syndrome, AB negative blood group and adenosquamous carcinoma of paranasal sinuses all are extremely rare clinical conditions found in populations and the combination of all three in the same patient have never been reported to the best of authors' knowledge.

  15. An unusual regression of the symptoms of Kartagener syndrome.

    Science.gov (United States)

    Serapinas, Danielius; Staikūnienė, Jūratė; Barkauskienė, Diana; Jackutė, Jurgita; Sakalauskas, Raimundas

    2013-01-01

    Kartagener's syndrome is a rare autosomal-recessive genetic disease with progressive damage of the respiratory system and situs inversus. Although the management of patients with Kartagener's syndrome remains uncertain and evidence is limited, it is important to follow up these patients with an adequate and shared care system. This report presents a clinical case of Kartagener's syndrome in a 25-year-old woman. Computed tomography showed dextrocardia and bronchiectasis. Abdominal X-ray and ultrasound confirmed situs inversus totalis. After 7 years, good treatment results were achieved: lung function improved and radiological findings showed no changes. The present case discusses the complex interrelationship between the genetic variation and a proper nonspecific management of Kartagener's syndrome.

  16. A Case of the Kartagener Syndrome Accompanied by Pectus Carinatus Deformity

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    Mahmut Tokur

    2013-10-01

    Full Text Available     A seven year old female patient diagnosed as a Kartagener Syndrom (KS by the findings of clinical and radiological, was brought to the hospital with the complaints such as expectoration sputum often, cough and fever. Situs inversus totalis, pansinusitis, destroyed right lower lobe, bronchiectasis in the left middle lobe and pectus carinatus deformity were detected in the physical examination and radiological investigations of the patient. Although long-term medical therapy pulmonary symptoms couldn’t get under control. Because of this right lower lobectomy was performed to the patient. In the literature it is mentioned that KS can be with rare togetherness but patient with thoracic deformities were not encountered.

  17. STUDY OF POLYSOMNO G RAPHY IN CURE POST - TUBERCULOSIS PATIENTS

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    Bhise Dhondiba

    2015-08-01

    Full Text Available Patients with obstructive sleep apnea (OSA have various Comorbidities like cardiovascular, cerebrovascular diseases and metabolic disorders. There is an association between pulmonary hypertension and obstructive sleep apnea. Obstructive sleep apnea is associated with nocturnal arterial oxygen desaturation. During apnea the increa sed vagal tone induces hypoxia and by mechanical effects of obstructive sleep apnea associated with intrathoracic pressure swings leads to bradycardia and acute increase in pulmonary artery pressure. Patients with coronary artery disease may develop myocar dial ischemia during apneas. Post - tuberculosis patients can lead to pulmonary sequelae that are characterized by impairments in the bronchial and parenchymal structure. The structural changes include bronchovascular distortions, bronchiectasis, emphysema a nd fibrosis. This leads to greater ventilation perfusion (V/Q mismatch and hypoxia.

  18. Imaging of community-acquired pneumonia: Roles of imaging examinations, imaging diagnosis of specific pathogens and discrimination from noninfectious diseases

    Institute of Scientific and Technical Information of China (English)

    Atsushi; Nambu; Katsura; Ozawa; Noriko; Kobayashi; Masao; Tago

    2014-01-01

    This article reviews roles of imaging examinations in the management of community-acquired pneumonia(CAP), imaging diagnosis of specific CAP and discrimination between CAP and noninfectious diseases. Chest radiography is usually enough to confirm the diagnosis of CAP, whereas computed tomography is required to suggest specific pathogens and to discriminate from noninfectious diseases. Mycoplasma pneumoniae pneumonia, tuberculosis, Pneumocystis jirovecii pneumonia and some cases of viral pneumonia sometimes show specific imaging findings. Peribronchial nodules, especially tree-in-bud appearance, are fairly specific for infection. Evidences of organization, such as concavity of the opacities, traction bronchiectasis, visualization of air bronchograms over the entire length of the bronchi, or mild parenchymal distortion are suggestive of organizing pneumonia. We will introduce tips to effectively make use of imaging examinations in the management of CAP.

  19. Achalasia cardia presenting with bilateral broncheactasis in a child

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    Bhavana Venkata Nagabhushana Rao

    2016-01-01

    Full Text Available Achalasia cardia usually presents in the age group of 25 to 60 and rare in children. Dysphagia, regurgitation, vomiting and chest pain are the cardinal symptoms. If they present mainly with respiratory symptoms diagnosis may be difficult and delayed. Bilateral bronchiectasis is rarely reported in patients with achalasia both in adults and children. Sometimes children may be erroneously diagnosed as eating disorder as both may present with similar symptoms hence information that achalasia can occur in children reduces such risk. In those children presenting with chronic respiratory symptoms it is prudent to look for esophageal motility disorders. CT scan, endoscopy, manometry are useful diagnostic tools but gold standard is barium esophgogram to diagnose Achalasia. Chest postural drainage may be delayed in bronchiectatic patients with achalasia. Heller myotomy is standard surgical treatment for children. Untreated Patients may end up with megaesophagus, a progressively dilated esophagus. [Int J Res Med Sci 2016; 4(1.000: 314-316

  20. Colistin bladder instillation, an alternative way of treating multi-resistant Acinetobacter urinary tract infection: a case series and review of literature.

    Science.gov (United States)

    Giua, R; Pedone, C; Cortese, L; Antonelli Incalzi, R

    2014-02-01

    The multiresistant Acinetobacter species bacteria are frequently involved in urinary or respiratory tract infections, and one of the most effective drugs, colistine, is associated with significant nephrotoxicity and neurotoxicity. Given that very high concentrations of colistine into biological fluids are safe for the human organism, attempts have been made at delivering the drug topically, by aerosol, or, occasionally, intratechally or intraventricularly for meningitis. These topical treatments could eradicate the Pseudomonas sp. from the lung of patients with cystic fibrosis or bronchiectasis and the Acinetobacter baumannii from lung and meninges. However, only one case of colistin topic treatment in urinary tract infection is described. We report a case series of three patients successfully undergone colistin bladder instillations for multi drug resistant Acinetobacter urinary tract infection, and we review the literature about colistin topic treatment.

  1. Medical image of the week: CREST plus ILD

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    Oliva I

    2013-06-01

    Full Text Available A 60 year old female with a history of fibromyalgia presented with dyspnea and skin changes, predominantly on the hands. Physical exam and imaging showed classic findings of limited cutaneous systemic sclerosis (scleroderma CREST syndrome. Calcinosis cutis (Figure 1A, Raynaud’s (not shown but endorsed by the patient, Esophageal dysmotility (Figure 1B, dilated esophagus, Sclerodactyly (Figure 1C, and Teleganectasias (Figure 1D were all present. Ground glass opacities were seen predominantly in the bilateral lower lung zones, associated with increased reticular markings (Figure 2A, and traction bronchiectasis (Figure 2B. Pulmonary involvement is noted in the majority of scleroderma patients. Interstitial lung disease (ILD is common and often portends a poor prognosis.

  2. Diagnostic role of magnetic resonance angiography in Swyer James syndrome: Case series of two cases

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    Parashari Umesh

    2010-01-01

    Full Text Available Swyer James syndrome is a rare syndrome which occurs due to viral illness in early childhood. The post infective obliterative bronchiolitis results in arrest of lung growth and alveolarization with reduced vascularity resulting in classical radiological features. We describe two cases of patients fulfilling all the criteria of the syndrome - 1 Unilateral hyperlucent small lung in chest radiograph with air trapping on expiration, small ipsilateral hila and pulmonary artery. 2 Diffuse decrease in attenuation of lung parenchyma with bronchiectasis and reduction in vascularity. 3 Unilateral pruned tree appearance on angiography (MRA. The clinical presentation was recurrent chest infection in a child and infrequent bouts of hemoptysis in a middle aged female. The study demonstrates the role of magnetic resonance angiography in diagnosing the condition.

  3. Aerosolized Antibiotics.

    Science.gov (United States)

    Restrepo, Marcos I; Keyt, Holly; Reyes, Luis F

    2015-06-01

    Administration of medications via aerosolization is potentially an ideal strategy to treat airway diseases. This delivery method ensures high concentrations of the medication in the targeted tissues, the airways, with generally lower systemic absorption and systemic adverse effects. Aerosolized antibiotics have been tested as treatment for bacterial infections in patients with cystic fibrosis (CF), non-CF bronchiectasis (NCFB), and ventilator-associated pneumonia (VAP). The most successful application of this to date is treatment of infections in patients with CF. It has been hypothesized that similar success would be seen in NCFB and in difficult-to-treat hospital-acquired infections such as VAP. This review summarizes the available evidence supporting the use of aerosolized antibiotics and addresses the specific considerations that clinicians should recognize when prescribing an aerosolized antibiotic for patients with CF, NCFB, and VAP.

  4. [A rare case of primary ciliary dyskinesia with heterotaxy].

    Science.gov (United States)

    Quintela, Cátia; Meireles, Cláudia; Bettencourt, Maria João; Ribeirinho, Augusto; Bentes, Teresa

    2009-01-01

    Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78 -year -old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it.

  5. Evaluation of Anti-TBGL Antibody in the Diagnosis of Tuberculosis Patients in China

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    Jingge Zhao

    2015-01-01

    Full Text Available Tuberculous glycolipid (TBGL is a component of the Mycobacterium tuberculosis cell wall, and anti-TBGL antibodies are used for serodiagnosis of tuberculosis. Anti-TBGL IgG and IgA levels were measured in 45 pulmonary TB patients (PTB, 26 extra-pulmonary TB patients (ETB, 16 AIDS-TB patients, and 58 healthy controls (HC including 39 health care workers (HW and 19 newly enrolled students (ST. Anti-TBGL IgG measurements yielded 68.9% and 46.2% sensitivity in PTB and ETB, respectively, and 81.0% specificity. However, anti-TBGL IgA measurements were significantly less sensitive in detecting ETB than PTB (15.4% versus 46.7% sensitivity but showed up to 89.7% specificity. Samples from AIDS-TB patients exhibited low reaction of anti-TBGL IgG and IgA with 6.3% and 12.5% sensitivity, respectively. Unlike anti-lipoarabinomannan (LAM IgG that was found to elevate in sputum smearpositive subjects, anti-TBGL IgG and IgA elevated in those with cavitation and bronchiectasis, respectively. Anti-TBGL IgG in cavitary TB yielded 78.2% sensitivity compared to 57.1% in those otherwise. Meanwhile, higher anti-TBGL IgA titers were observed in HW than in ST, and increasing anti-TBGL IgG titers were observed in HW on follow-up. Therefore, higher anti-TBGL antibody titers are present in patients presenting cavities and bronchiectasis and subjects under TB exposure risk.

  6. Fourier-based linear systems description of free-breathing pulmonary magnetic resonance imaging

    Science.gov (United States)

    Capaldi, D. P. I.; Svenningsen, S.; Cunningham, I. A.; Parraga, G.

    2015-03-01

    Fourier-decomposition of free-breathing pulmonary magnetic resonance imaging (FDMRI) was recently piloted as a way to provide rapid quantitative pulmonary maps of ventilation and perfusion without the use of exogenous contrast agents. This method exploits fast pulmonary MRI acquisition of free-breathing proton (1H) pulmonary images and non-rigid registration to compensate for changes in position and shape of the thorax associated with breathing. In this way, ventilation imaging using conventional MRI systems can be undertaken but there has been no systematic evaluation of fundamental image quality measurements based on linear systems theory. We investigated the performance of free-breathing pulmonary ventilation imaging using a Fourier-based linear system description of each operation required to generate FDMRI ventilation maps. Twelve subjects with chronic obstructive pulmonary disease (COPD) or bronchiectasis underwent pulmonary function tests and MRI. Non-rigid registration was used to co-register the temporal series of pulmonary images. Pulmonary voxel intensities were aligned along a time axis and discrete Fourier transforms were performed on the periodic signal intensity pattern to generate frequency spectra. We determined the signal-to-noise ratio (SNR) of the FDMRI ventilation maps using a conventional approach (SNRC) and using the Fourier-based description (SNRF). Mean SNR was 4.7 ± 1.3 for subjects with bronchiectasis and 3.4 ± 1.8, for COPD subjects (p>.05). SNRF was significantly different than SNRC (p<.01). SNRF was approximately 50% of SNRC suggesting that the linear system model well-estimates the current approach.

  7. Surgical treatment of right middle lobe syndrome in children

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    A Sehitogullari

    2012-01-01

    Results: The mean age of the patients was 10.5 years (range, 5 to 15 years. Twelve patients were boys and eight were girls. The most frequent symptom was chronic cough (75%. Hemoptysis was present in two (10% patients. One patient was being treated for asthma. 25% positive cultures were identified among the patients. Streptococcus pneumoniae was the most frequently identified agent in sputum. All patients underwent chest computed tomography. There were bronchiectasis in 11 (55% patients, atelectasis and bronchiectasis in five (25% patients, and destroyed lung in four (20% patients. A narrowed middle lobe bronchus was shown in 15 (75% patients. Bronchoscopy was performed in 18 (90% patients. Stenosis due to external compression was seen in 12 (60% patients, hyperemia and bronchitis in two (10% patients, granulation tissue in two (10% patients, and dense secretions in two (10% patients. A history of doctor-diagnosed tuberculosis was present in two (10% patients. These patients had completed antituberculous treatment. The patients had been symptomatic for the last 1 to 10 years (mean, 4 years and had received several medical treatments. All patients (totally 20 patients underwent right middle lobe resection. In one patient, a bronchial abnormality was found intraoperatively. One patient died on postoperative day 10 due to a brain abscess. Three other patients had postoperative complications (15%. Mean duration of follow-up of the patients was 4.5 years (range, 2 months to 12 years. Seventeen patients were asymptomatic, and two patients had improved. Conclusions: Children with right middle lobe syndrome unresponsive to medical treatment should undergo early lobe resection to avoid serious complications and the progression of the disease to other segments or lobes.

  8. Multidrug resistant tuberculosis versus non-tuberculous mycobacterial infections: a CT-scan challenge

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    Kahkouee, Shahram; Esmi, Elham; Moghadam, Azadeh; Karam, Mehrdad Bakhshayesh; Mosadegh, Leila; Salek, Solmaz; Tabarsi, Payam, E-mail: bestlala@yahoo.com [Chronic Respiratory Disease Research Center, NRITLD, Masih Daneshvari Hospital, Shahid Beheshti University of Medical Science, Tehran (Iran, Islamic Republic of)

    2013-03-15

    Introduction: clinical, laboratory and imaging findings in patients with multidrug resistant tuberculosis (MDR-TB) and non-tuberculosis mycobacterium (NTM) are similar, and the majority of these patients present with positive smear for Acid Fast Bacilli (ADB) and no response to first line anti-TB treatment, so sputum culture and PCR are necessary, especially in NTM. Objective: In this study we evaluate more details of imaging findings to help earlier diagnosis of pathogens. Materials and methods: 66 patients with positive smear for AFB and no response to first line anti-TB drugs were divided into two groups by PCR and culture: MDR-TB (43 patients) and NTM (23 patients). Age, sex, history of anti-TB treatment, smoking and CT-scan findings (parenchymal, pleural and mediastinal variables) by details and lobar distribution were analyzed. Results: mean age of NTM patients was slightly higher (52 versus 45) and there is no significant difference in sex and smoking. In MDR-TB group, history of anti-TB treatment and evidence of chronic pulmonary disease such as calcified and fibrodestructed parenchyma, volume loss and pleural thickening were higher significantly. Cavities in MDR-TB were thick wall in the background of consolidation, while NTM cavities were more thin-walled with adjacent satellite nodules in same segment or lobe. Prevalence of bronchiectasis was similar in both groups, while bronchiectasis in MDR-TB group was in fibrobronchiectatic background in upper lobes, and in NTM group the distribution was more uniform with slightly middle lobes predominance. Prevalence and distribution of nodular infiltrations were similar more in Tree in Buds and scattered pattern. Calcified or non-calcified lymph nodes and also pleural changes were more frequent in MDR-TB but prevalence of lymphadenopathy was mildly higher in NTM. (author)

  9. Diffusion weighted imaging in cystic fibrosis disease: beyond morphological imaging

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    Ciet, Pierluigi [Erasmus Medical Center, Department of Radiology, Rotterdam (Netherlands); Erasmus Medical Center - Sophia Children' s Hospital, Department of Paediatrics, Respiratory Medicine and Allergology, P.O. Box 2060, Rotterdam, Zuid-Holland (Netherlands); Ca' Foncello - General Hospital, Department of Radiology, Treviso (Italy); Serra, Goffredo; Catalano, Carlo [University of Rome ' ' Sapienza' ' , Department of Radiology, Rome (Italy); Andrinopoulou, Eleni Rosalina [Erasmus Medical Center, Department of Biostatistics, Rotterdam (Netherlands); Bertolo, Silvia; Morana, Giovanni [Ca' Foncello - General Hospital, Department of Radiology, Treviso (Italy); Ros, Mirco [Ca' Foncello Hospital, Department of Pediatrics, Treviso (Italy); Colagrande, Stefano [University of Florence - Azienda Ospedaliero-Universitaria Careggi, Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit n. 2, Florence (Italy); Tiddens, Harm A.W.M. [Erasmus Medical Center, Department of Radiology, Rotterdam (Netherlands); Erasmus Medical Center - Sophia Children' s Hospital, Department of Paediatrics, Respiratory Medicine and Allergology, P.O. Box 2060, Rotterdam, Zuid-Holland (Netherlands)

    2016-11-15

    To explore the feasibility of diffusion-weighted imaging (DWI) to assess inflammatory lung changes in patients with Cystic Fibrosis (CF) CF patients referred for their annual check-up had spirometry, chest-CT and MRI on the same day. MRI was performed in a 1.5 T scanner with BLADE and EPI-DWI sequences (b = 0-600 s/mm{sup 2}). End-inspiratory and end-expiratory scans were acquired in multi-row scanners. DWI was scored with an established semi-quantitative scoring system. DWI score was correlated to CT sub-scores for bronchiectasis (CF-CT{sub BE}), mucus (CF-CT{sub mucus}), total score (CF-CT{sub total-score}), FEV{sub 1}, and BMI. T-test was used to assess differences between patients with and without DWI-hotspots. Thirty-three CF patients were enrolled (mean 21 years, range 6-51, 19 female). 4 % (SD 2.6, range 1.5-12.9) of total CF-CT alterations presented DWI-hotspots. DWI-hotspots coincided with mucus plugging (60 %), consolidation (30 %) and bronchiectasis (10 %). DWI{sub total-score} correlated (all p < 0.0001) positively to CF-CT{sub BE} (r = 0.757), CF-CT{sub mucus} (r = 0.759) and CF-CT{sub total-score} (r = 0.79); and negatively to FEV{sub 1} (r = 0.688). FEV{sub 1} was significantly higher (p < 0.0001) in patients without DWI-hotspots. DWI-hotspots strongly correlated with radiological and clinical parameters of lung disease severity. Future validation studies are needed to establish the exact nature of DWI-hotspots in CF patients. (orig.)

  10. Bronchiolitis in Kartagener's syndrome.

    Science.gov (United States)

    Homma, S; Kawabata, M; Kishi, K; Tsuboi, E; Narui, K; Nakatani, T; Saiki, S; Nakata, K

    1999-12-01

    The association of diffuse bronchiolitis in patients with Kartagener's syndrome (KS) has not been reported previously. The aim of this study was to present the morphological characteristics of bronchiolitis in patients with KS. Eight patients (four males, four females; mean age 37.9+/-18.7 yrs), clinically diagnosed as KS with the classical triad of chronic pansinusitis, bronchiectasis and situs in versus with dextrocardia, were evaluated. Routine chest radiography showed bronchiectasis and dextrocardia in all patients. Chest computed tomography (CT) showed diffuse centrilobular small nodules up to 2 mm in diameter throughout both lungs in six out of eight patients. Pulmonary function tests revealed marked obstructive impairment in all patients (forced expiratory volume in one second 57.0+/-11.3%, residual volume/total lung capacity 45.+/-12.7%, maximum midexpiratory flow 0.92+/-0.72 L x s(-1), forced vital capacity 74.1+/-12.2% (all mean +/- SD)). The examination of cilial movement of the bronchus revealed immotility in all of the five patients examined. The ultrastructure showed ciliary dynein arm defects in all patients. Histopathological examination of lung specimens obtained at autopsy or by video-assisted thoracoscopic surgery showed obliterative thickening of the walls of the membranous bronchioli with infiltration of lymphocytes, plasma cells and neutrophils, but most of the distal respiratory bronchioli were spared and alveolar spaces were overinflated. Pathologically, the diffuse centrilobular small nodules on the chest CT mainly corresponded to membranous bronchiolitis. This is the first report demonstrating that the association of diffuse bronchiolitis might be one of the characteristic features of the lung in Kartagener's syndrome.

  11. Perfil nutricional de pacientes candidatos ao transplante de pulmão Nutritional profile of lung transplant candidates

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    Sabrina Monteiro Pereira de Souza

    2009-03-01

    circumference and triceps skinfold thickness (TST were determined during the first outpatient visit. RESULTS: We included 117 patients, 69 of which (59% were male. The mean age of the participants was 42.5 ± 15.2 years. The most prevalent underlying disease, seen in 29 patients (24.8%, was pulmonary emphysema. The nutritional profile was considered normal in 48.3% of the patients with pulmonary emphysema, 55% of those with cystic fibrosis, 56% of those with bronchiectasis and 50% of those with other lung diseases. The majority (51.7% of the patients with pulmonary fibrosis were classified as overweight. The TSTs indicated that the risk of depletion was highest (64.7% among the patients with cystic fibrosis, followed by those with bronchiectasis (52.6%. CONCLUSIONS: Patients with pulmonary fibrosis presented the highest BMIs, although the corresponding TSTs and MAMCs were normal. Patients with cystic fibrosis and bronchiectasis presented the highest prevalence of nutritional depletion, based on TST and MAMC.

  12. COPD phenotypes on computed tomography and its correlation with selected lung function variables in severe patients

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    da Silva SMD

    2016-03-01

    Full Text Available Silvia Maria Doria da Silva, Ilma Aparecida Paschoal, Eduardo Mello De Capitani, Marcos Mello Moreira, Luciana Campanatti Palhares, Mônica Corso PereiraPneumology Service, Department of Internal Medicine, School of Medical Sciences, State University of Campinas (UNICAMP, Campinas, São Paulo, BrazilBackground: Computed tomography (CT phenotypic characterization helps in understanding the clinical diversity of chronic obstructive pulmonary disease (COPD patients, but its clinical relevance and its relationship with functional features are not clarified. Volumetric capnography (VC uses the principle of gas washout and analyzes the pattern of CO2 elimination as a function of expired volume. The main variables analyzed were end-tidal concentration of carbon dioxide (ETCO2, Slope of phase 2 (Slp2, and Slope of phase 3 (Slp3 of capnogram, the curve which represents the total amount of CO2 eliminated by the lungs during each breath.Objective: To investigate, in a group of patients with severe COPD, if the phenotypic analysis by CT could identify different subsets of patients, and if there was an association of CT findings and functional variables.Subjects and methods: Sixty-five patients with COPD Gold III–IV were admitted for clinical evaluation, high-resolution CT, and functional evaluation (spirometry, 6-minute walk test [6MWT], and VC. The presence and profusion of tomography findings were evaluated, and later, the patients were identified as having emphysema (EMP or airway disease (AWD phenotype. EMP and AWD groups were compared; tomography findings scores were evaluated versus spirometric, 6MWT, and VC variables.Results: Bronchiectasis was found in 33.8% and peribronchial thickening in 69.2% of the 65 patients. Structural findings of airways had no significant correlation with spirometric variables. Air trapping and EMP were strongly correlated with VC variables, but in opposite directions. There was some overlap between the EMP and AWD

  13. Risk factors for chronic obstructive pulmonary disease among never-smokers in Korea

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    Lee SJ

    2015-03-01

    Full Text Available Seok Jeong Lee,1 Seo Woo Kim,1 Kyoung Ae Kong,2 Yon Ju Ryu,1 Jin Hwa Lee,1 Jung Hyun Chang1 1Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, 2Department of Clinical Trial Center, School of Medicine, Ewha Womans University, Seoul, Republic of Korea Background: Chronic obstructive pulmonary disease (COPD patients include those who have never smoked. However, risk factors other than smoking in never-smokers have not been elucidated sufficiently. This study investigated the risk factors for COPD among never-smokers in Korea using population-based data. Methods: The data were retrieved from the Korean National Health and Nutrition Survey IV conducted from 2007 to 2009. Among subjects aged 40 years or older who underwent appropriate pulmonary function tests, never-smokers not diagnosed with asthma and not showing a restrictive pattern on pulmonary function tests were enrolled. Risk factors of COPD in never-smokers were analyzed using logistic regression models. Results: Among 24,871 participants in the representative Korean cohort, 3,473 never-smokers were enrolled. COPD patients accounted for 7.6% of the never-smokers. In the logistic regression analysis, low education status (odds ratio [OR]: 2.0; 95% confidence interval [CI]: 1.2–3.2, occupational exposure (OR: 2.6; 95% CI: 1.3–5.3, a history of tuberculosis (OR: 4.5; 95% CI: 2.3–8.7, bronchiectasis (OR: 6.0; 95% CI: 1.4–25.4, male sex (OR: 4.2; 95% CI: 2.6–6.7, advanced age (60–69 years vs 40–49 years; OR: 3.8; 95% CI: 2.0–7.0, and being underweight (body mass index <18.5 vs 18.0–24.9 kg/m2; OR: 3.1; 95% CI: 1.0–9.4 were associated with the development of COPD. Conclusion: Low education status, manual labor, a history of tuberculosis and bronchiectasis, as well as male sex, advanced age and being underweight were risk factors for COPD in Korean never-smokers. Keywords: socioeconomic status, chronic obstructive pulmonary disease, never-smoker

  14. Lung parenchymal change after the resolution of adenovirus pneumonia : chest radiographs and high-resolution CT findings

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    Yoon, Jung Hee; Kim, Joung Sook; Kim, Chang Kuen; Kang, Seung Pyung; Lee, Soo Hyun; Hur Gham [Inje Univ. Sanggye Paik Hospital, Seoul (Korea, Republic of)

    1998-07-01

    To evaluate lung parenchymal change as seen on chest radiographs and high-resolution CT (HRCT) after the resolution of adenovirus pneumonia (a common cause of lower respiratory infection in infants and children),and the usefulness of HRCT during follow-up. Material and Methods : Four to 13(mean, 8) months after recovery, ten patients infected with adenovirus pneumonia underwent HRCT and chest radiographs. Eight were boys and two were girls, and their mean age was 26(range, 14-45) months. Adenovirus pneumonia had been confirmed by viral isolation in culture or serologic test. CT scanning was performed during quiet breathing ; collimation was 2 mm and the interval from apex to diaphragm was 5-10 mm. Lung settings were 1600 HU (window width) and -700 HU(level). CT findings were assessed and compared with chest radiographs by two chest radiologists, who reached a consensus. The patients were clinically followed up for one year. Result : On chest radiographs, hyperlucent lung was seen in 8 of 10 patients (80%) ; in one other there was partial collapse, and in one, findings were normal. The most common HRCT finding was a mosaic pattern of lung attenuation with decreased pulmonary vascularity in the area of lower attenuation ; this was seen in 8 of 10 patients (80%). Other findings were partial collapse, bronchiectasis, and bronchial wall thickening, each seen in two patients, and reticulonodular density, seen in one. In two patients HRCT findings were normal ; in one of these, chest findings were normal but a mosaic pattern of lung attenuation was found in all lobes. During follow-up, three patients wheezed continuously. Conclusion : In cases of adenovirus pneumonia, HRCT demonstrated more specific parenchymal change than did chest radiographs ; a mosaic pattern of lung attenuation was seen, with decreased pulmonary vascularity in areas of lower attenuation ; bronchiectasis,bronchial wall thickening, and reticulo-odular density were also noted. These findings were

  15. Mosaic pattern of lung attenuation on thin-section CT : review of 31 cases

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    Choi, Young Hi; An, Jee Hyun; Lee, Kye Young; Jee, Young Koo; Lee, Young Seok [Dankook Univ. College of Medicine, Choan (Korea, Republic of)

    1998-07-01

    To correlate radiologic findings with clinical findings in patients with a mosaic pattern of lung attenuation, as seen on thin-section CT. Materials and Methods : Thirty-one cases in which a mosaic pattern of lung attenuation was detected on combined expiratory and inspiratory scans of thin-section CT were retrospectively analyzed. Cases involving infiltrative lung disease were excluded. Both thin-section CT and clinical findings we reanalyzed and the relationship between the extent of the area of hyperlucency -as seen on expiratory scan- and physiologic parameters was evaluated. The subjects were 10 men and 21 women ranged in age from 25 to 76 (mean 50)years. Results : Twenty-nine patients with small airway disease, [chronic bronchitis and/or bronchiolitis(n=11),bronchiectasis(n=8), bronchial asthma(n=8), mycoplasmic pneumonitis(n=1) and hypersensitive pneumonitis(n=1),] and two patients with pulmonary vascular disease, [chronic pulmonary thromboembolism(n=1) and stenosis of the left upper pulmonary artery(n=1)] were included in our study. Commonly associated thin-section CT findings in the cases involving small airway disease(n=29) were bronchial wall thickening(n=25), nodular opacity(n=25), bronchial and bronchiolar dilatation(n=20) and small branching opacity(n=16). These findings were not observed in two patients with pulmonary vascular disease, though bronchial wall thickening was seen in the patient with chronic pulmonary thromboembolism. At expiratory scan level, there was statistical correlation between FEV1/FVC and the number of pulmonary segments(r= 0.982, p<0.05), but no correlation between FEV1/FVC and the percentage area of hyperlucency(r=0.803, p>0.05). Conclusion: The mosaic pattern of lung attenuation seen on thin-section CT is indicative of various diseases, involving small airways such as bronchiolitis, bronchitis, bronchiectasis and bronchial asthma, and vascular lung disease. Bronchial wall thickening and nodular opacity can be associated with

  16. A pilot study of the impact of high-frequency chest wall oscillation in chronic obstructive pulmonary disease patients with mucus hypersecretion

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    Chakravorty I

    2011-12-01

    Full Text Available Indranil Chakravorty1, Kamaljit Chahal2, Gillian Austin21St George's Hospital, London, 2East and North Hertfordshire NHS Trust, Lister Hospital and Primary Care Trust, Stevenage, Hertfordshire, UKIntroduction: Chronic obstructive pulmonary disease (COPD patients with mucus hypersecretion tend to demonstrate increased frequency of infective exacerbations and a steeper slope of decline in lung function. Enhanced mucociliary clearance with high-frequency chest wall oscillation (HFCWO devices previously used in cystic fibrosis and bronchiectasis patients may offer the opportunity for community-based, self-managed therapy to improve quality of life and lung function.Study design and methods: A randomized controlled crossover pilot study of HFCWO compared with conventional treatment was conducted in 22 patients with moderate to severe COPD and mucus hypersecretion. Patients spent 4 weeks using an HFCWO (SmartVest® device and 4 weeks in a conventional phase with a 2-week washout. Eleven patients started with HFCWO and changed to conventional treatment, whereas the other eleven patients started conventional treatment and crossed over to HFCWO.Results: The patients were elderly with a mean age of 71 (standard deviation [SD] 10 years and were at the upper end of the normal range of body mass index (25 [SD 4.2] kg/m2. The majority of patients had moderate to severe COPD with a mean percentage predicted forced expiratory volume in 1 second of 41 (SD 15.6 and percentage predicted forced vital capacity of 73 (SD 17.7. Baseline sputum production was negatively correlated to lung function and positively to St George's Respiratory Questionnaire. Symptom scores and St George's Respiratory Questionnaire symptom dimension improved significantly (-8, P < 0.05. Sputum production showed a declining trend in the HFCWO phase, although not reaching statistical significance. The HFCWO device was well tolerated with good reported compliance.Conclusion: This pilot study

  17. Volumetric capnography for the evaluation of chronic airways diseases

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    Veronez L

    2014-09-01

    Full Text Available Liliani de Fátima Veronez,1 Monica Corso Pereira,2 Silvia Maria Doria da Silva,2 Luisa Affi Barcaui,2 Eduardo Mello De Capitani,2 Marcos Mello Moreira,2 Ilma Aparecida Paschoalz2 1Department of Physical Therapy, University of Votuporanga (Educational Foundation of Votuporanga, Votuporanga, 2Department of Internal Medicine, School of Medical Sciences, State University of Campinas (UNICAMP, Campinas, Sao Paulo, BrazilBackground: Obstructive lung diseases of different etiologies present with progressive peripheral airway involvement. The peripheral airways, known as the silent lung zone, are not adequately evaluated with conventional function tests. The principle of gas washout has been used to detect pulmonary ventilation inhomogeneity and to estimate the location of the underlying disease process. Volumetric capnography (VC analyzes the pattern of CO2 elimination as a function of expired volume.Objective: To measure normalized phase 3 slopes with VC in patients with non-cystic fibrosis bronchiectasis (NCB and in bronchitic patients with chronic obstructive pulmonary disease (COPD in order to compare the slopes obtained for the groups.Methods: NCB and severe COPD were enrolled sequentially from an outpatient clinic (Hospital of the State University of Campinas. A control group was established for the NCB group, paired by sex and age. All subjects performed spirometry, VC, and the 6-Minute Walk Test (6MWT. Two comparisons were made: NCB group versus its control group, and NCB group versus COPD group. The project was approved by the ethical committee of the institution. Statistical tests used were Wilcoxon or Student’s t-test; P<0.05 was considered to be a statistically significant difference.Results: Concerning the NCB group (N=20 versus the control group (N=20, significant differences were found in body mass index and in several functional variables (spirometric, VC, 6MWT with worse results observed in the NCB group. In the comparison between

  18. Morpho-Functional 1H-MRI of the Lung in COPD: Short-Term Test-Retest Reliability.

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    Bertram J Jobst

    Full Text Available Non-invasive end-points for interventional trials and tailored treatment regimes in chronic obstructive pulmonary disease (COPD for monitoring regionally different manifestations of lung disease instead of global assessment of lung function with spirometry would be valuable. Proton nuclear magnetic resonance imaging (1H-MRI allows for a radiation-free assessment of regional structure and function. The aim of this study was to evaluate the short-term reproducibility of a comprehensive morpho-functional lung MRI protocol in COPD.20 prospectively enrolled COPD patients (GOLD I-IV underwent 1H-MRI of the lung at 1.5T on two consecutive days, including sequences for morphology, 4D contrast-enhanced perfusion, and respiratory mechanics. Image quality and COPD-related morphological and functional changes were evaluated in consensus by three chest radiologists using a dedicated MRI-based visual scoring system. Test-retest reliability was calculated per each individual lung lobe for the extent of large airway (bronchiectasis, wall thickening, mucus plugging and small airway abnormalities (tree in bud, peripheral bronchiectasis, mucus plugging, consolidations, nodules, parenchymal defects and perfusion defects. The presence of tracheal narrowing, dystelectasis, pleural effusion, pulmonary trunk ectasia, right ventricular enlargement and, finally, motion patterns of diaphragma and chest wall were addressed.Median global scores [10(Q1:8.00;Q3:16.00 vs.11(Q1:6.00;Q3:15.00] as well as category subscores were similar between both timepoints, and kappa statistics indicated "almost perfect" global agreement (ĸ = 0.86, 95%CI = 0.81-0.91. Most subscores showed at least "substantial" agreement of MRI1 and MRI2 (ĸ = 0.64-1.00, whereas the agreement for the diagnosis of dystelectasis/effusion (ĸ = 0.42, 95%CI = 0.00-0.93 was "moderate" and of tracheal abnormalities (ĸ = 0.21, 95%CI = 0.00-0.75 "fair". Most MRI acquisitions showed at least diagnostic quality at

  19. [Domiciliary noninvasive positive pressure ventilation in chronic alveolar hypoventilation].

    Science.gov (United States)

    Casas, J P; Robles, A M; Pereyra, M A; Abbona, H L; López, A M

    2000-01-01

    Effectiveness of treatment with domiciliary nocturnal noninvasive positive pressure ventilation is analyzed in a group of patients with chronic alveolar hypoventilation of different etiologies. It was applied with two levels of pressure (BiPAP) via nasal mask. Criteria for evaluation were symptomatology and improvement in gas exchange. Data were analyzed by Student t tests. A total of 13 patients were included, mean age 55.7 range 20 to 76 years (5 male 8 female). Main diagnosis was tuberculosis in 6, four of them having had surgical procedure (thoracoplasty 2, frenicectomy 1 and neumonectomy 1), myopathy 3 (myasthenia gravis 1, muscular dystrophy 1 and diaphragmatic paralysis 1), obesity-hypoventilation syndrome 1, escoliosis 1, bronchiectasis 1 and cystic fibrosis 1. These last two patients were on waiting list for lung transplantation. At the moment of consultation, the symptoms were: dysnea 13/13 (100%), astenia 13/13 (100%), hypersomnolency 10/13 (77%), cephalea 9/13 (69%), leg edema 6/13 (46%), loss of memory 6/13 (46%). Regarding gas exchange, they showed hypoxemia and hypercapnia. Mean follow up was of 2.2 years (range 6 months to 4 years). Within the year, all 13 patients became less dyspneic. Astenia, hypersomnolency, cephalea, leg edema and memory loss disappeared. Improvement in gas exchange was: PaO2/FiO2 from 269 +/- 65.4 (basal) to 336.7 +/- 75.3 post-treatment (p = 0.0018). PaCO2 from 70.77 +/- 25.48 mmHg (basal) to 46.77 +/- 8.14 mmHg (p = 0.0013). Ventilatory support was discontinued en 5 patients: three because of pneumonia requiring intubation and conventional mechanical ventilation, two of them died and one is still with tracheostomy; One patient with bronchiectasis and one with cystic fibrosis were transplanted. The remaining eight patients are stable. In conclusion, chronic alveolar hypoventilation can be effectively treated with domiciliary nocturnal noninvasive ventilation. Long term improvement in symptomatology and arterial blood gases

  20. Correlation of delta high-resolution computed tomography (HRCT) score with delta clinical variables in early systemic sclerosis (SSc) patients

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    Euathrongchit, Juntima; Wattanawittawas, Pittaporn; Kasitanon, Nuntana

    2016-01-01

    Background The correlation of changes (delta: Δ) of high-resolution computed tomography (HRCT) score with the Δ of other clinical variables has not been well studied. The purpose of this study was to determine the correlation of Δ HRCT score with Δ percent predicted forced vital capacity (%pFVC), Δ modified Rodnan Skin Score (mRSS), Δ erythrocyte sedimentation rate (ESR), and Δ percent of oxygen saturation at room air (%SpO2) in patients with early systemic sclerosis (SSc). Methods We used an inception cohort of early-SSc patients seen at the Rheumatology Clinic, Chiang Mai University, Thailand, between January 2010 and June 2014. All patients underwent HRCT at study entry and every 12 months thereafter. Thirty-one SSc patients who underwent pulmonary function test (PFT) within 12 weeks of their corresponding HRCT at baseline and last visit were identified. The extent of ground glass (GG), lung fibrosis (Fib), bronchiectasis (B), and honeycombing (HC) was scored, and then aggregated to produce a total (t) HRCT score. Results Mean ± SD age and disease duration from non-Raynaud’s phenomenon (NRP) to undergo HRCT at baseline were 52.2±8.8 years and 11.7±7.1 months, respectively. Seventeen (54.8%) patients were female and 20 (64.5%) were classified as dcSSc. The mean ± SD interval between the two HRCT tests was 16.0±7.2 months. The Δ HRCT scores [total fibrosis scores (t-Fib), total bronchiectasis scores (t-B), and total HRCT score (t-HRCT) scores] and Δ mRSS, but not Δ %pFVC, showed significant change over the observation period. We found significant correlation of Δ total honeycombing scores (t-HC) with Δ ESR (r=−0.44, P<0.05), and Δ t-Fib with Δ %SpO2 (r=−0.38, P<0.05). However, no significant correlation of any Δ HRCT scores with Δ %pFVC and Δ mRSS were observed. Conclusions In this study, the changes in the HRCT scores were greater than %pFVC; this, along with their correlations with the changes in ESR and %SpO2, suggest that HRCT

  1. Who Needs to Be Allocated in ICU after Thoracic Surgery? An Observational Study

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    Liana Pinheiro

    2016-01-01

    Full Text Available Background. The effective use of ICU care after lung resections has not been completely studied. The aims of this study were to identify predictive factors for effective use of ICU admission after lung resection and to develop a risk composite measure to predict its effective use. Methods. 120 adult patients undergoing elective lung resection were enrolled in an observational prospective cohort study. Preoperative evaluation and intraoperative assessment were recorded. In the postoperative period, patients were stratified into two groups according to the effective and ineffective use of ICU. The use of ICU care was considered effective if a patient experienced one or more of the following: maintenance of controlled ventilation or reintubation; acute respiratory failure; hemodynamic instability or shock; and presence of intraoperative or postanesthesia complications. Results. Thirty patients met the criteria for effective use of ICU care. Logistic regression analysis identified three independent predictors of effective use of ICU care: surgery for bronchiectasis, pneumonectomy, and age ≥ 57 years. In the absence of any predictors the risk of effective need of ICU care was 6%. Risk increased to 25–30%, 66–71%, and 93% with the presence of one, two, or three predictors, respectively. Conclusion. ICU care is not routinely necessary for all patients undergoing lung resection.

  2. Toxicity risk of non-target organs at risk receiving low-dose radiation: case report

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    Chen Yu-Jen

    2009-12-01

    Full Text Available Abstract The spine is the most common site for bone metastases. Radiation therapy is a common treatment for palliation of pain and for prevention or treatment of spinal cord compression. Helical tomotherapy (HT, a new image-guided intensity modulated radiotherapy (IMRT, delivers highly conformal dose distributions and provides an impressive ability to spare adjacent organs at risk, thus increasing the local control of spinal column metastases and decreasing the potential risk of critical organs under treatment. However, there are a lot of non-target organs at risk (OARs occupied by low dose with underestimate in this modern rotational IMRT treatment. Herein, we report a case of a pathologic compression fracture of the T9 vertebra in a 55-year-old patient with cholangiocarcinoma. The patient underwent HT at a dose of 30 Gy/10 fractions delivered to T8-T10 for symptom relief. Two weeks after the radiotherapy had been completed, the first course of chemotherapy comprising gemcitabine, fluorouracil, and leucovorin was administered. After two weeks of chemotherapy, however, the patient developed progressive dyspnea. A computed tomography scan of the chest revealed an interstitial pattern with traction bronchiectasis, diffuse ground-glass opacities, and cystic change with fibrosis. Acute radiation pneumonitis was diagnosed. Oncologists should be alert to the potential risk of radiation toxicities caused by low dose off-targets and abscopal effects even with highly conformal radiotherapy.

  3. Interstitial lung disease in patients with rheumatoid arthritis: spontaneous and drug induced.

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    Hallowell, Robert W; Horton, Maureen R

    2014-03-01

    Rheumatoid arthritis (RA) is an inflammatory autoimmune disease characterized by the destruction of articular joint structures. RA is a systemic condition that often affects multiple organs, including the heart, lungs, and kidneys. Pulmonary complications of RA are relatively common and include pleural effusion, rheumatoid nodules, bronchiectasis, obliterative bronchiolitis, and opportunistic infections. Interstitial lung disease (ILD) is a common occurrence in patients with RA, and can range in severity from an asymptomatic incidental finding to a rapidly progressing life-threatening event. Usual interstitial pneumonia and non-specific interstitial pneumonia are the two most common patterns, though others have been reported. Various disease-modifying anti-rheumatic drugs-in particular, methotrexate and the tumor necrosis factor-alpha inhibitors-have been associated with RA-ILD in numerous case reports and case series, though it is often difficult to distinguish association from causality. Treatment for RA-ILD typically involves the use of high-dose corticosteroids, often in conjunction with alternative immunosuppressant agents such as azathioprine or mycophenolate mofetil, and outcomes vary widely depending on the initial pattern of lung disease. Additional research into the mechanisms driving RA-ILD is needed to guide future therapy.

  4. Usual interstitial pneumonia in rheumatoid arthritis-associated interstitial lung disease.

    Science.gov (United States)

    Kim, E J; Elicker, B M; Maldonado, F; Webb, W R; Ryu, J H; Van Uden, J H; Lee, J S; King, T E; Collard, H R

    2010-06-01

    Interstitial lung disease is a common manifestation of rheumatoid arthritis; however, little is known about factors that influence its prognosis. The aim of the present study was to determine whether or not the usual interstitial pneumonia pattern found on high-resolution computed tomography (HRCT) is of prognostic significance in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Patients with RA-ILD were identified retrospectively (n = 82). The relationship of a definite usual interstitial pneumonia pattern on HRCT to survival was determined and compared to that in a cohort of patients with radiologically diagnosed idiopathic pulmonary fibrosis (n = 51). A definite usual interstitial pneumonia pattern was seen in 20 (24%) out of 82 patients with RA-ILD. These patients showed worse survival than those without this pattern (median survival 3.2 versus 6.6 yrs), and a similar survival to those with idiopathic pulmonary fibrosis. On multivariate analysis, a definite usual interstitial pneumonia pattern on HRCT was associated with worse survival (hazard ratio of 2.3). Analysis of specific HRCT features demonstrated that traction bronchiectasis and honeycomb fibrosis were associated with worse survival (hazard ratio of 2.6 and 2.1, respectively). Female sex (hazard ratio of 0.30) and a higher baseline diffusing capacity of the lung for carbon monoxide (hazard ratio of 0.96) were associated with better survival. A definite usual interstitial pneumonia pattern on HRCT has important prognostic implications in RA-ILD.

  5. [Airway clearance techniques in chronic obstructive pulmonary syndrome : 2011 update].

    Science.gov (United States)

    Opdekamp, C

    2011-09-01

    For many years the airway clearance techniques used in chest physical therapy were assimilated with the singular technique of postural drainage, percussions and vibrations. However the side effects and counter indications and the lack of scientific proof regarding this technique have forced reflection and development of other techniques more comfortable and without deleterious effects. If all these techniques show a high efficiency in terms of improved mucociliary clearance, the literature is unanimous on how little effect these techniques have in the short and the long-term with regards to lung function and arterial blood gases. In view of the scientific literature, it is clear that the airway clearance techniques don't have the same recognition concerning their efficiency in all obstructive pulmonary diseases. As the cornerstone in the management of cystic fibrosis, the efficiency of the bronchial hygiene techniques are in general poorly documented in the management of the non-cystic fibrosis bronchiectasis, bronchitis or emphysema. The use of the chest physical therapy seems more to do with the interpretation of the imagery and symptomatology. The airway clearance techniques should be individualised according to symptoms, the amount of expectorated mucus and the objectives signs of secretions retention or subjective signs of difficulty expectorating secretions with progression of the disease.

  6. Changes in structural lung disease in cystic fibrosis children over 4 years as evaluated by high-resolution computed tomography

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    Carpio, Carlos; Alvarez-Sala, Rodolfo; Prados, Concepcion [University Hospital La Paz, La Paz Hospital Research Institute, Department of Respiratory Medicine, Madrid (Spain); Albi, Gustavo [Nino de Jesus Children' s Hospital, Department of Radiology, Madrid (Spain); Rayon-Aledo, Jose Carlos; Caballero, Paloma [University Hospital La Princesa, Department of Radiology, Madrid (Spain); Giron, Rosa [University Hospital La Princesa, Department of Respiratory Medicine, Madrid (Spain)

    2015-12-15

    To compare the worsening of structural lung disease on high-resolution computed tomography (HRCT) with changes in spirometry results in cystic fibrosis (CF) patients, and analyse factors associated with the worsening of structural lung disease over time. A total of 31 CF subjects (mean age 11.03 ± 3.67 years old) were prospectively evaluated by two HRCT and spirometry tests performed 4 years apart. HRCT abnormalities were scored using the Bhalla scoring system. Comparisons between changes on HRCT and spirometry were made for all patients, and also for groups categorized by age, sex, genotypic alterations and lung obstruction. The mean HRCT Bhalla scoring, forced expiratory volume in 1 s (FEV{sub 1} %pred.) and forced vital capacity (FVC %pred.) were 7.92 ± 3.59, 87.76 ± 20.52 and 96.54 ± 15.12, respectively. There was a significant deterioration in the Bhalla score (p < 0.01) and in certain categories: severity of bronchiectasis, peribronchial thickening, mucous plugging and bronchial divisions. Females had a more pronounced worsening of the Bhalla score than males (p = 0.048). No change over time was found in FEV{sub 1} and FVC. Only sex was associated with a deterioration in HRCT. HRCT Bhalla scoring changes statistically significantly over 4 years, but spirometry results do not. Worsening on HRCT is more evident in females. (orig.)

  7. Accuracy of high-resolution CT in distinguishing between Pneumocystis carinii pneumonia and non-Pneumocystis carinii pneumonia in AIDS patients

    Energy Technology Data Exchange (ETDEWEB)

    Hidalgo, A.; Mauleon, S.; Andreu, J.; Caceres, J. [Department of Radiology, Hospital General Universitari Vall d' Hebron, Universitari Vall d' Hebron, Universitat Autonoma de Barcelona, Paseo Vall d' Hebron 119-129, 08035 Barcelona (Spain); Falco, V.; Crespo, M.; Ribera, E.; Pahissa, A. [Department of Medicine, Service of Infectious Diseases, Hospital General Universitari Vall d' Hebron, Universitari Vall d' Hebron, Universitat Autonoma de Barcelona, Paseo Vall d' Hebron 119-129, 08035 Barcelona (Spain)

    2003-05-01

    The aim of this study was to assess the value of high-resolution CT in distinguishing between Pneumocystis carinii and non-Pneumocystis carinii pneumonia (PCP) in patients HIV-positive and high risk to have PCP. We performed a prospective study in 30 patients with <200 CD4 lymphocytes, clinical symptoms of pulmonary disease and chest X-ray non-conclusive for pulmonary infection. Evaluated CT findings included ground-glass opacities, reticulation, tree-in-bud appearance, consolidation, cystic lesions, bronchiectasis and lymphadenopathies. The diagnosis of ''examination suggestive of PCP'' was applied to cases showing a diffuse or predominant ground-glass pattern in the upper fields, associated or not with reticulations and small cystic lesions. The sensitivity, specificity, positive predictive value and negative predictive value of high-resolution computed tomography (HRCT) for the diagnosis of PCP was 100, 83.3, 90.5 and 100%, respectively. Pneumocystis carinii pneumonia was not demonstrated in any of the cases classified as ''examination not suggestive of PCP''. Significant small airway disease was not observed in any of the PCP cases. We conclude that HRCT is a reliable method for differentiating PCP from other infectious processes in HIV-positive patients and a good method to rule our PCP. Its inclusion in the diagnostic algorithm of lung infections is justified in these patients. (orig.)

  8. Who Needs to Be Allocated in ICU after Thoracic Surgery? An Observational Study

    Science.gov (United States)

    Pinheiro, Liana; Faresin, Sonia Maria

    2016-01-01

    Background. The effective use of ICU care after lung resections has not been completely studied. The aims of this study were to identify predictive factors for effective use of ICU admission after lung resection and to develop a risk composite measure to predict its effective use. Methods. 120 adult patients undergoing elective lung resection were enrolled in an observational prospective cohort study. Preoperative evaluation and intraoperative assessment were recorded. In the postoperative period, patients were stratified into two groups according to the effective and ineffective use of ICU. The use of ICU care was considered effective if a patient experienced one or more of the following: maintenance of controlled ventilation or reintubation; acute respiratory failure; hemodynamic instability or shock; and presence of intraoperative or postanesthesia complications. Results. Thirty patients met the criteria for effective use of ICU care. Logistic regression analysis identified three independent predictors of effective use of ICU care: surgery for bronchiectasis, pneumonectomy, and age ≥ 57 years. In the absence of any predictors the risk of effective need of ICU care was 6%. Risk increased to 25–30%, 66–71%, and 93% with the presence of one, two, or three predictors, respectively. Conclusion. ICU care is not routinely necessary for all patients undergoing lung resection. PMID:27493477

  9. RECURRENT/PERSISTENT PNEUMONIA AMONG CHILDREN IN UPPER EGYPT

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    Khaled Saad

    2013-04-01

    Full Text Available Abstract Objectives: Recurrent/persistent pneumonia in children continues to be a major challenge for the paediatricians. We aimed to determine the prevalence and underlying causes of recurrent/persistent pneumonia in children in Upper Egypt. Settings: Assiut University Children Hospital, Assiut, Egypt.   Methods: Patients with pneumonia admitted to the hospital during 2 years were investigated (microbiological, biochemical, immunological and radiological tests for recurrent/persistent pneumonia to determine its prevalence and to find out the underlying causes.   Results: 113 out of 1228 patients (9.2% met the diagnosis of recurrent/persistent pneumonia. Identified causes were;  aspiration syndromes (17.7%, pulmonary TB (14%, congenital heart disease (11.5%, bronchial asthma (9.7%, immune deficiency disorders (8.8% and vitamin D deficiency rickets (7%. Other causes included; congenital anomalies of the respiratory tract, interstitial lung diseases, bronchiectasis, and sickle cell anemia. No predisposing factors could be identified in 15% of cases. Conclusion: Approximately 1 in 10 children with pneumonia in our locality had recurrent/persistent pneumonia. The most frequent underlying cause for recurrent/persistent pneumonia in children in Upper Egypt is aspiration syndromes, followed by pulmonary TB.

  10. [Crohn's disease associated with focal pulmonare lesion].

    Science.gov (United States)

    Tagle, Martín; Barriga, José; Piñeiro, Andrés

    2003-01-01

    40 year-old male recently diagnosed with Crohn's disease. A routine chest X ray showed a round, well defined opacity in right lung field. A chest CT scan confirmed the finding and also described bronchiectasis. Patient had no respiratory symptoms. He was prescribed with oral sulfasalazine and corticosteroids with rapid improvement of intestinal symptoms as well as resolution of the pulmonary opacity. We describe the clinical presentation of a male newly diagnosed with Crohn's disease who was found to have an asymptomatic pulmonary lesion on imaging studies. Pulmonary complications have been previously described in inflamatory bowel disease being more common in ulcerative colitis than in Crohn's disease; these can involve the lung parenchyma, the tracheobronchial tree, and the pleura. The true prevalence and etiology of these lesions is currently unknown and are not necessarily associated with bowel disease activity. Abnormal pulmonary functions test have been reported during inflammatory bowel disease exacerbations, and although pulmonary findings can present with a variety of symptoms, subclinical presentations have also been described. Pulmonary manifestations are usually steoid-responsive, as was the case in our patients.

  11. Shulman disease (eosinophilic fasciitis) in X-linked agammaglobulinemia.

    Science.gov (United States)

    Pituch-Noworolska, A; Mach-Tomalska, H; Szaflarska, A; Adamek, D

    2016-06-01

    X-linked agammaglobulinemia (XLA) diagnosed in the first year of life is an immunodeficiency with a life-long indication for substitution of immunoglobulins, due to lack of B lymphocytes in the periphery. The decrease of bacterial infection frequency and severity is an effect of immunoglobulin replacement. However, in the majority of patients bronchiectasis and chronic sinusitis with an overgrown mucous membrane develop despite regular substitution. Autoimmune diseases as co-existing diseases in XLA are noted in a few patients presenting symptoms associated with arthritis, scleroderma and myositis. Our patient was diagnosed with XLA in the first year of life, followed by regular substitution of immunoglobulins. The symptoms of pain, edema of muscles of the right shank with skin edema and discoloration after mild injury were noted in a 13-year-old boy. Shulman disease was diagnosed after 6 months of symptoms, based on histopathology of muscle and skin biopsy. Before the diagnosis, non-steroid anti-inflammatory drugs (NSAID) were used with a transient effect. After the diagnosis, therapy included steroids, immunoglobulins in a high dose and immunosuppression, with improvement of clinical symptoms. During methotrexate (MTX) therapy the patient developed two episodes of pneumonia, so mycophenolate mofetil (MMF) was used, with a similar effect. Now, with this therapy, the symptoms are mild and stable without progression.

  12. THE CLINICAL SPECTRUM OF RESPIRATORY DISEASES IN PATIENTS WITH PRIMARY ANTIBODY DEFICIENCY

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    A. Aghamohammadi

    2000-08-01

    Full Text Available Primary Humoral Immunodeficiencies (PHID are currently increasingly being recognized. Patients with PHID frequently show respiratory complications.The objectives of the study is to determine the clinical spectrum of respiratory diseases in patients with PHID."We extracted data from the clinical files of patients with PHID, diagnosed according to WHO criteria. We encountered 125 patients (84 males, with the diagnosis of primary antibody deficiency including common-variable immunodeficiency (64 pts, x-linked agammaglobulinemia (29 pts, IgA deficiency (20 pts, IgG-subc!ass deficiency (8 pts, and hyper-IgM syndrome (4 pts. The mean age of the patients at the time of study was 11 years. In the evolution of their disease, 92 cases (73.6% developed upper respiratory tract infections, among which acute otitis media (68 pts, 54.4%, sinusitis (61 pts, 48.8%, and pharyngitis (12 pts, 10.4% were found to be the most frequent. Among the lower respiratory tract infections, pneumonia was the most common occurance (91 pts, 72.8%. The other lower respiratory tract complications were: bronchiectasis (22 pts, 17.6%, bronchitis (8 pts, tuberculosis (6 pts, lung abscess (4 pts, and Pneumocystis carinii pneumonia (2 pts.Respiratory infections constitute the most common presenting symptom of patients with primary humoral immunodeficiency. There may be some differences in the type and frequency of infections in each of these disorders.

  13. Stimulation effect of wide type CFTR chloride channel by the naturally occurring flavonoid tangeretin.

    Science.gov (United States)

    Jiang, Yu; Yu, Bo; Wang, Xue; Sui, Yujie; Zhang, Yaofang; Yang, Shuang; Yang, Hong; Ma, Tonghui

    2014-12-01

    Cystic fibrosis transmembrane conductance regulator (CFTR) is a cAMP-activated chloride channel expressed in the apical membrane of serous epithelial cells. Both deficiency and overactivation of CFTR may cause fluid and salt secretion related diseases. In the present study, we identified tangeretin from Pericarpium Citri Reticulatae Viride as a CFTR activator using high-throughput screening based on FRT cell-based fluorescence assay. The activation effect of tangeretin on CFTR chloride channel and the possible underlying mechanisms were investigated. Fluorescence quenching tests showed that tangeretin dose- and time-dependently activated CFTR chloride channel, the activity had rapid and reversible characteristics and the activation effect could be completely reversed by the CFTR specific blocker CFTRinh-172. Primary mechanism studies indicated that the activation effect of tangeretin on CFTR chloride channel was FSK dependent as well as had additional effect with FSK and IBMX suggesting that tangeretin activates CFTR by direct interacting with the protein. Ex-vivo tests revealed that tangeretin could accelerate the speed of the submucosal gland fluid secretion. Short-circuit current measurement demonstrated that tangeretin activated rat colonic mucosa chloride current. Thus, CFTR Cl(-) channel is a molecular target of natural compound tangeretin. Tangeretin may have potential use for the treatment of CFTR-related diseases like cystic fibrosis, bronchiectasis and habitual constipation.

  14. High-resolution computed tomography and histopathological findings in hypersensitivity pneumonitis: a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Torres, Pedro Paulo Teixeira e Silva; Moreira, Marise Amaral Reboucas; Silva, Daniela Graner Schuwartz Tannus; Moreira, Maria Auxiliadora do Carmo [Universidade Federal de Goias (UFG), Goiania, GO (Brazil); Gama, Roberta Rodrigues Monteiro da [Hospital do Cancer de Barretos, Barretos, SP (Brazil); Sugita, Denis Masashi, E-mail: pedroptstorres@yahoo.com.br [Anapolis Unievangelica, Anapolis, GO (Brazil)

    2016-03-15

    Hypersensitivity pneumonitis is a diffuse interstitial and granulomatous lung disease caused by the inhalation of any one of a number of antigens. The objective of this study was to illustrate the spectrum of abnormalities in high-resolution computed tomography and histopathological findings related to hypersensitivity pneumonitis. We retrospectively evaluated patients who had been diagnosed with hypersensitivity pneumonitis (on the basis of clinical-radiological or clinical-radiological-pathological correlations) and had undergone lung biopsy. Hypersensitivity pneumonitis is clinically divided into acute, subacute, and chronic forms; high-resolution computed tomography findings correlate with the time of exposure; and the two occasionally overlap. In the subacute form, centrilobular micronodules, ground glass opacities, and air trapping are characteristic high-resolution computed tomography findings, whereas histopathology shows lymphocytic inflammatory infiltrates, bronchiolitis, variable degrees of organizing pneumonia, and giant cells. In the chronic form, high-resolution computed tomography shows traction bronchiectasis, honeycombing, and lung fibrosis, the last also being seen in the biopsy sample. A definitive diagnosis of hypersensitivity pneumonitis can be made only through a multidisciplinary approach, by correlating clinical findings, exposure history, high-resolution computed tomography findings, and lung biopsy findings. (author)

  15. Imaging of cystic fibrosis lung disease and clinical interpretation

    Energy Technology Data Exchange (ETDEWEB)

    Wielpuetz, M.O.; Eichinger, M.; Kauczor, H.U. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Biederer, J. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Gross-Gerau Community Hospital (Germany). Radiologie Darmstadt; Wege, S. [Heidelberg University Hospital (Germany). Dept. of Pulmonology and Respiratory Medicine; Stahl, M.; Sommerburg, O. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Mall, M.A. [Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Div. of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center; Heidelberg University Hospital (Germany). Dept. of Translational Pulmonology; Puderbach, M. [Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology; Translational Lung Research Center Heidelberg (TLRC) (Germany); Heidelberg University Hospital (Germany). Dept. of Diagnostic and Interventional Radiology with Nuclear Medicine; Hufeland Hospital, Bad Langensalza (Germany). Dept. of Diagnostic and Interventional Radiology

    2016-09-15

    Progressive lung disease in cystic fibrosis (CF) is the life-limiting factor of this autosomal recessive genetic disorder. Increasing implementation of CF newborn screening allows for a diagnosis even in pre-symptomatic stages. Improvements in therapy have led to a significant improvement in survival, the majority now being of adult age. Imaging provides detailed information on the regional distribution of CF lung disease, hence longitudinal imaging is recommended for disease monitoring in the clinical routine. Chest X-ray (CXR), computed tomography (CT) and magnetic resonance imaging (MRI) are now available as routine modalities, each with individual strengths and drawbacks, which need to be considered when choosing the optimal modality adapted to the clinical situation of the patient. CT stands out with the highest morphological detail and has often been a substitute for CXR for regular severity monitoring at specialized centers. Multidetector CT data can be post-processed with dedicated software for a detailed measurement of airway dimensions and bronchiectasis and potentially a more objective and precise grading of disease severity. However, changing to CT was inseparably accompanied by an increase in radiation exposure of CF patients, a young population with high sensitivity to ionizing radiation and lifetime accumulation of dose. MRI as a cross-sectional imaging modality free of ionizing radiation can depict morphological hallmarks of CF lung disease at lower spatial resolution but excels with comprehensive functional lung imaging, with time-resolved perfusion imaging currently being most valuable.

  16. Pro-Con Debate: Protracted Bacterial Bronchitis as a Cause of Chronic Cough in Children.

    Science.gov (United States)

    Bidiwala, Aneela; Krilov, Leonard R; Pirzada, Melodi; Patel, Sameer J

    2015-08-01

    Pro: Children with chronic cough present a diagnostic challenge. Protracted bacterial bronchitis (PBB) is a chronic, persistent bacterial infection of conducting airways defined by the presence of cough for longer than 4 weeks that resolves with antimicrobial therapy and without an alternative diagnosis. The diagnosis is made by the findings of increased bronchial secretions and edema of the lower airways on flexible bronchoscopy and positive cultures on bronchoalveolar lavage. It is speculated that an initial respiratory insult such as viral infection disrupts normal surface morphology and ciliary function, which leads to chronic self-perpetuating inflammation with the formation of bacterial biofilms, leading to PBB. PBB is often misdiagnosed as asthma, leading to inappropriate and excessive use of steroids. The importance of timely diagnosis should be emphasized due to the potential that PBB may be a precursor to chronic suppurative lung disease or bronchiectasis if left untreated; however, every patient should be adequately assessed to exclude other causes of chronic cough. Con: Clinical criteria for the diagnosis of PBB are nonspecific and may not distinguish it from other known causes of chronic cough, including viral infections. Benefits from antibiotic therapy (particularly prolonged therapy) have not been demonstrated. Respiratory conditions are the most common reason for antibiotic prescriptions during ambulatory visits in the United States, and many of these prescriptions are inappropriate and/or unnecessary. The proposed diagnostic criteria and recommendations for the treatment of PBB will lead to unnecessary overuse of antibiotics.

  17. High-resolution computed tomography and histopathological findings in hypersensitivity pneumonitis: a pictorial essay*

    Science.gov (United States)

    Torres, Pedro Paulo Teixeira e Silva; Moreira, Marise Amaral Rebouças; Silva, Daniela Graner Schuwartz Tannus; da Gama, Roberta Rodrigues Monteiro; Sugita, Denis Masashi; Moreira, Maria Auxiliadora do Carmo

    2016-01-01

    Hypersensitivity pneumonitis is a diffuse interstitial and granulomatous lung disease caused by the inhalation of any one of a number of antigens. The objective of this study was to illustrate the spectrum of abnormalities in high-resolution computed tomography and histopathological findings related to hypersensitivity pneumonitis. We retrospectively evaluated patients who had been diagnosed with hypersensitivity pneumonitis (on the basis of clinical-radiological or clinical-radiological-pathological correlations) and had undergone lung biopsy. Hypersensitivity pneumonitis is clinically divided into acute, subacute, and chronic forms; high-resolution computed tomography findings correlate with the time of exposure; and the two occasionally overlap. In the subacute form, centrilobular micronodules, ground-glass opacities, and air trapping are characteristic high-resolution computed tomography findings, whereas histopathology shows lymphocytic inflammatory infiltrates, bronchiolitis, variable degrees of organizing pneumonia, and giant cells. In the chronic form, high-resolution computed tomography shows traction bronchiectasis, honeycombing, and lung fibrosis, the last also being seen in the biopsy sample. A definitive diagnosis of hypersensitivity pneumonitis can be made only through a multidisciplinary approach, by correlating clinical findings, exposure history, high-resolution computed tomography findings, and lung biopsy findings. PMID:27141134

  18. The Lung Immune Response to Nontypeable Haemophilus influenzae (Lung Immunity to NTHi).

    Science.gov (United States)

    King, Paul T; Sharma, Roleen

    2015-01-01

    Haemophilus influenzae is divided into typeable or nontypeable strains based on the presence or absence of a polysaccharide capsule. The typeable strains (such as type b) are an important cause of systemic infection, whilst the nontypeable strains (designated as NTHi) are predominantly respiratory mucosal pathogens. NTHi is present as part of the normal microbiome in the nasopharynx, from where it may spread down to the lower respiratory tract. In this context it is no longer a commensal and becomes an important respiratory pathogen associated with a range of common conditions including bronchitis, bronchiectasis, pneumonia, and particularly chronic obstructive pulmonary disease. NTHi induces a strong inflammatory response in the respiratory tract with activation of immune responses, which often fail to clear the bacteria from the lung. This results in recurrent/persistent infection and chronic inflammation with consequent lung pathology. This review will summarise the current literature about the lung immune response to nontypeable Haemophilus influenzae, a topic that has important implications for patient management.

  19. What is chronic cough in children?

    Directory of Open Access Journals (Sweden)

    Iulia eIOAN

    2014-08-01

    Full Text Available The cough reflex is modulated throughout growth and development. Cough – but not expiration reflex – appears to be absent at birth, but increases with maturation. Thus, acute cough is the most frequent respiratory symptom during the first few years of life. Later on, the pubertal development seems to play a significant role in changing of the cough threshold during childhood and adolescence resulting in sex-related differences in cough reflex sensitivity in adulthood. Asthma is the major cause of chronic cough in children. Prolonged acute cough is usually related to the long-lasting effects of a previous viral airway infection or to the particular entity called protracted bacterial bronchitis. Cough pointers and type may orient towards specific aetiologies, such as barking cough in croup or tracheomalacia, paroxystic whooping cough in Pertussis. Cough is productive in protracted bacterial bronchitis, sinusitis or bronchiectasis. Cough is usually associated with wheeze or dyspnea on exertion in asthma; however, it may be the sole symptom in cough variant asthma. Thus, paediatric cough has particularities differentiating it from adult cough, so the approach and management should be developmentally specific.

  20. Update on Pediatric Cough.

    Science.gov (United States)

    Kantar, Ahmad

    2016-02-01

    Despite the high prevalence of cough in children, the topic has been poorly researched. Although pediatricians recognize that chronic cough in children is different from that in adults, this difference seems less recognizable to other health professionals. During childhood, the respiratory tract and nervous system undergo a series of anatomical and physiological maturation processes that influence the cough reflex. Additionally, immunological responses undergo developmental and memorial processes that make infection and congenital abnormalities the overwhelming cause of cough in children. The lack of comprehensive clinical data regarding chronic cough in children has initially required pediatricians to adopt an adult approach to the problem. In the last 10 years, however, research has led to the reconsideration of the etiology of chronic cough in children. Currently, attention has focused on protracted bacterial bronchitis as a major cause of chronic cough in preschool-aged children and as a possible precursor of bronchiectasis. New research horizons are emerging for both the treatment and prevention of particular causes of chronic cough in children.

  1. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases

    Energy Technology Data Exchange (ETDEWEB)

    Griffin, N. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)]. E-mail: nyreegriffin@hotmail.com; Mansfield, L. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Redmond, K.C. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Dusmet, M. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Goldstraw, P. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Mittal, T.K. [Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S. [Royal Brompton and Harefield NHS Trust, London (United Kingdom)

    2007-03-15

    Aim: To highlight the variation in clinical manifestations, imaging and management of four cases of unilateral pulmonary artery agenesis presenting in adulthood. Method: Four patients with unilateral pulmonary artery agenesis were referred to our institution between 1995 and 2005. They underwent a series of investigations, including chest radiography, echocardiography, ventilation perfusion scintigraphy, angiography, computed tomography (CT) and magnetic resonance imaging (MRI). Results: Two of the four patients had absence of the right main pulmonary artery, whilst the remaining two patients had absence of the left main pulmonary artery. One patient showed a restrictive defect on pulmonary function tests. Two patients who had ventilation perfusion scintigraphy showed absent perfusion and reduced ventilation on the affected side. Angiography (where performed), CT and MRI confirmed the anatomy and the presence of multiple collaterals. Bronchiectasis was demonstrated on CT in two patients, with one also demonstrating a mosaic attenuation pattern. One patient had an incidental lung tumour on the side of the agenesis, which was diagnosed as a chondroid hamartoma on histology. Three of the four patients eventually underwent resection of the affected lung. Conclusion: Isolated unilateral pulmonary artery agenesis has a non-specific presentation. Awareness of this condition can lead to earlier diagnosis, with cross-sectional imaging making an important contribution.

  2. Childhood allergic bronchopulmonary aspergillosis presenting as a middle lobe syndrome.

    Science.gov (United States)

    Shah, Ashok; Gera, Kamal; Panjabi, Chandramani

    2016-01-01

    Allergic bronchopulmonary aspergillosis (ABPA) is infrequently documented in children with asthma. Although collapse is not uncommon, middle lobe syndrome (MLS) as a presentation of ABPA is rather a rarity. A 9-year-old female child with asthma presented with increase in intensity of symptoms along with a right midzone patchy consolidation on a chest radiograph. In addition, an ill-defined opacity abutting the right cardiac border with loss of cardiac silhouette was noted. A right lateral view confirmed a MLS, which was further corroborated by high resolution computed tomography. Central bronchiectasis was also observed, which prompted a work-up for ABPA. The child met 7/8 major diagnostic criteria for ABPA. She was then initiated on oral prednisolone that resulted in a marked clinical improvement within a fortnight. Radiological clearance occurred at 3 months with inflation of the middle lobe. ABPA presenting with MLS in a child is yet to be reported. A high index of suspicion is required to establish the diagnosis of ABPA in a child presenting with MLS. This would obviate the invasive investigations usually done to ascertain the cause of MLS.

  3. Pseudomonas aeruginosa pyocyanin causes airway goblet cell hyperplasia and metaplasia and mucus hypersecretion by inactivating the transcriptional factor FoxA2.

    Science.gov (United States)

    Hao, Yonghua; Kuang, Zhizhou; Walling, Brent E; Bhatia, Shikha; Sivaguru, Mayandi; Chen, Yin; Gaskins, H Rex; Lau, Gee W

    2012-03-01

    The redox-active exotoxin pyocyanin (PCN) can be recovered in 100 µM concentrations in the sputa of bronchiectasis patients chronically infected with Pseudomonas aeruginosa (PA). However, the importance of PCN within bronchiectatic airways colonized by PA remains unrecognized. Recently, we have shown that PCN is required for chronic PA lung infection in mice, and that chronic instillation of PCN induces goblet cell hyperplasia (GCH), pulmonary fibrosis, emphysema and influx of immune cells in mouse airways. Many of these pathological features are strikingly similar to the mouse airways devoid of functional FoxA2, a transcriptional repressor of GCH and mucus biosynthesis. In this study, we postulate that PCN causes and exacerbates GCH and mucus hypersecretion in bronchiectatic airways chronically infected by PA by inactivating FoxA2. We demonstrate that PCN represses the expression of FoxA2 in mouse airways and in bronchial epithelial cells cultured at an air-liquid interface or conventionally, resulting in GCH, increased MUC5B mucin gene expression and mucus hypersecretion. Immunohistochemical and inhibitor studies indicate that PCN upregulates the expression of Stat6 and EGFR, both of which in turn repress the expression of FoxA2. These studies demonstrate that PCN induces GCH and mucus hypersecretion by inactivating FoxA2.

  4. Diagnosing α1-antitrypsin deficiency: how to improve the current algorithm

    Directory of Open Access Journals (Sweden)

    Noel G. McElvaney

    2015-03-01

    Full Text Available Over the past 10–15 years, the diagnosis of α1-antitrypsin deficiency (AATD has markedly improved as a result of increasing awareness and the publication of diagnostic recommendations by the American Thoracic Society (ATS/European Respiratory Society (ERS. Nevertheless, the condition remains substantially underdiagnosed. Furthermore, when AATD is diagnosed there is a delay before treatment is introduced. This may help explain why AATD is the fourth most common cause of lung transplantation. Clearly we need to do better. The ATS/ERS recommend testing high-risk groups, such as: all chronic obstructive pulmonary disease patients; all nonresponsive asthmatic adults/adolescents; all cases of cryptogenic cirrhosis/liver disease; subjects with granulomatosis with polyangitis; bronchiectasis of unknown aetiology; panniculitis and first-degree relatives of patients with AATD. In terms of laboratory diagnosis, measurement of α1-antitrypsin levels will identify patients with protein deficiency, but cannot differentiate between the various genetic subtypes of AATD. Phenotyping is the current gold standard for detecting rare variants of AATD (except null variants, while advances in molecular diagnostics are making genotyping more effective. An accurate diagnosis facilitates the physician's ability to actively intervene with measures such as smoking cessation and perhaps augmentation therapy, and it will also help provide a better understanding of the natural history of the disease.

  5. Bronchiolitis: adopting a unifying definition and a comprehensive etiological classification.

    Science.gov (United States)

    Papiris, Spyros A; Malagari, Katerina; Manali, Effrosyni D; Kolilekas, Likurgos; Triantafillidou, Christina; Baou, Katerina; Rontogianni, Dimitra; Bouros, Demosthenes; Kagouridis, Konstantinos

    2013-06-01

    Bronchiolitis is an inflammatory and potentially fibrosing condition affecting mainly the intralobular conducting and transitional small airways. Secondary bronchiolitis participates in disease process of the airways and/or the surrounding lobular structures in the setting of several already defined clinical entities, mostly of known etiology, and occurs commonly. Primary or idiopathic bronchiolitis dominates and characterizes distinct clinical entities, all of unknown etiology, and occurs rarely. Secondary bronchiolitis regards infections, hypersensitivity disorders, the whole spectrum of smoking-related disorders, toxic fumes and gas inhalation, chronic aspiration, particle inhalation, drug-induced bronchiolar toxicities, sarcoidosis and neoplasms. Idiopathic or primary bronchiolitis defines clinicopathologic entities sufficiently different to be designated as separate disease entities and include cryptogenic constrictive bronchiolitis, diffuse panbronchiolitis, diffuse idiopathic pulmonary neuroendocrine cell hyperplasia, neuroendocrine hyperplasia in infants, bronchiolitis obliterans syndrome in lung and allogeneic hematopoietic cell transplantation, connective tissue disorders, inflammatory bowel disease and bronchiolitis obliterans organizing pneumonia. Most of the above are pathological descriptions used as clinical diagnosis. Acute bronchiolitis, though potentially life threatening, usually regresses. Any etiology chronic bronchiolitis contributes to morbidity and/or mortality if it persists and/or progresses to diffuse airway narrowing and distortion or complete obliteration. Bronchiolitis in specific settings leads to bronchiolectasis, resulting in bronchiectasis.

  6. Intracellular Secretory Leukoprotease Inhibitor Modulates Inositol 1,4,5-Triphosphate Generation and Exerts an Anti-Inflammatory Effect on Neutrophils of Individuals with Cystic Fibrosis and Chronic Obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Emer P. Reeves

    2013-01-01

    Full Text Available Secretory leukoprotease inhibitor (SLPI is an anti-inflammatory protein present in respiratory secretions. Whilst epithelial cell SLPI is extensively studied, neutrophil associated SLPI is poorly characterised. Neutrophil function including chemotaxis and degranulation of proteolytic enzymes involves changes in cytosolic calcium (Ca2+ levels which is mediated by production of inositol 1,4,5-triphosphate (IP3 in response to G-protein-coupled receptor (GPCR stimuli. The aim of this study was to investigate the intracellular function of SLPI and the mechanism-based modulation of neutrophil function by this antiprotease. Neutrophils were isolated from healthy controls (n=10, individuals with cystic fibrosis (CF (n=5 or chronic obstructive pulmonary disease (COPD (n=5. Recombinant human SLPI significantly inhibited fMet-Leu-Phe (fMLP and interleukin(IL-8 induced neutrophil chemotaxis (P<0.05 and decreased degranulation of matrix metalloprotease-9 (MMP-9, hCAP-18, and myeloperoxidase (MPO (P<0.05. The mechanism of inhibition involved modulation of cytosolic IP3 production and downstream Ca2+ flux. The described attenuation of Ca2+ flux was overcome by inclusion of exogenous IP3 in electropermeabilized cells. Inhibition of IP3 generation and Ca2+ flux by SLPI may represent a novel anti-inflammatory mechanism, thus strengthening the attractiveness of SLPI as a potential therapeutic molecule in inflammatory airway disease associated with excessive neutrophil influx including CF, non-CF bronchiectasis, and COPD.

  7. The potential of liposome–encapsulated ciprofloxacin as a tularemia therapy

    Directory of Open Access Journals (Sweden)

    Karleigh Ann Hamblin

    2014-06-01

    Full Text Available Liposome-encapsulation has been suggested as method to improve the efficacy of ciprofloxacin against the intracellular pathogen, Francisella tularensis. Early work with a prototype formulation, evaluated for use against the F. tularensis live vaccine strain, showed that a single dose of liposomal ciprofloxacin given by the intranasal or inhalational route could provide protection in a mouse model of pneumonic tularemia. Liposomal ciprofloxacin offered better protection than ciprofloxacin given by the same routes. Liposomal ciprofloxacin has been further developed by Aradigm Corporation for Pseudomonas aeruginosa infections in patients with cystic fibrosis and non-cystic fibrosis bronchiectasis. This advanced development formulation is safe, effective and well tolerated in human clinical trials. Further evaluation of the advanced liposomal ciprofloxacin formulation against the highly virulent F. tularensis Schu S4 strain has shown that aerosolised CFI (Ciprofloxacin encapsulated in liposomes for inhalation provides significantly better protection than oral ciprofloxacin. Thus, liposomal ciprofloxacin is a promising treatment for tularemia and further research with the aim of enabling licensure under the animal rule is warranted.

  8. The potential of liposome-encapsulated ciprofloxacin as a tularemia therapy.

    Science.gov (United States)

    Hamblin, Karleigh A; Wong, Jonathan P; Blanchard, James D; Atkins, Helen S

    2014-01-01

    Liposome-encapsulation has been suggested as method to improve the efficacy of ciprofloxacin against the intracellular pathogen, Francisella tularensis. Early work with a prototype formulation, evaluated for use against the F. tularensis live vaccine strain, showed that a single dose of liposomal ciprofloxacin given by the intranasal or inhalational route could provide protection in a mouse model of pneumonic tularemia. Liposomal ciprofloxacin offered better protection than ciprofloxacin given by the same routes. Liposomal ciprofloxacin has been further developed by Aradigm Corporation for Pseudomonas aeruginosa infections in patients with cystic fibrosis and non-cystic fibrosis bronchiectasis. This advanced development formulation is safe, effective and well tolerated in human clinical trials. Further evaluation of the advanced liposomal ciprofloxacin formulation against the highly virulent F. tularensis Schu S4 strain has shown that aerosolized CFI (Ciprofloxacin encapsulated in liposomes for inhalation) provides significantly better protection than oral ciprofloxacin. Thus, liposomal ciprofloxacin is a promising treatment for tularemia and further research with the aim of enabling licensure under the animal rule is warranted.

  9. Radioiodine uptake in inactive pulmonary tuberculosis

    Energy Technology Data Exchange (ETDEWEB)

    Bakheet, S.M.; Powe, J.; Al Suhaibani, H. [Department of Radiology, King Faisal Specialist Hospital and Research Centre, Riyadh (Saudi Arabia); Hammami, M.M.; Bazarbashi, M. [Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh (Saudi Arabia)

    1999-06-01

    Radioiodine may accumulate at sites of inflammation or infection. We have seen such accumulation in six thyroid cancer patients with a history of previously treated pulmonary tuberculosis. We also review the causes of false-positive radioiodine uptake in lung infection/inflammation. Eight foci of radioiodine uptake were seen on six iodine-123 diagnostic scans. In three foci, the uptake was focal and indistinguishable from thyroid cancer pulmonary metastases from thyroid cancer. In the remaining foci, the uptake appeared nonsegmental, linear or lobar, suggesting a false-positive finding. The uptake was unchanged, variable in appearance or non-persistent on follow-up scans and less extensive than the fibrocystic changes seen on chest radiographs. In the two patients studied, thyroid hormone level did not affect the radioiodine lung uptake and there was congruent gallium-67 uptake. None of the patients had any evidence of thyroid cancer recurrence or of reactivation of tuberculosis and only two patients had chronic intermittent chest symptoms. Severe bronchiectasis, active tuberculosis, acute bronchitis, respiratory bronchiolitis, rheumatoid arthritis-associated lung disease and fungal infection such as Allescheria boydii and aspergillosis can lead to different patterns of radioiodine chest uptake mimicking pulmonary metastases. Pulmonary scarring secondary to tuberculosis may predispose to localized radioiodine accumulation even in the absence of clinically evident active infection. False-positive radioiodine uptake due to pulmonary infection/inflammation should be considered in thyroid cancer patients prior to the diagnosis of pulmonary metastases. (orig.) With 4 figs., 1 tab., 9 refs.

  10. Progressive systemic sclerosis: high-resolution computed tomography findings; Esclerose sistemica progressiva: aspectos na tomografia computadorizada de alta resolucao

    Energy Technology Data Exchange (ETDEWEB)

    Gasparetto, Emerson L.; Pimenta, Rodrigo; Ono, Sergio E.; Escuissato, Dante L. [Parana Univ., Curitiba, PR (Brazil). Hospital de Clinicas. Servico de Radiologia Medica]. E-mail: dante.luiz@onda.com.br; Inoue, Cesar [Parana Univ., Curitiba, PR (Brazil). Faculdade de Medicina

    2005-09-15

    Objective: To describe the high-resolution computed tomography findings in the lung of patients with systemic sclerosis, independently of the respiratory symptoms. Materials and methods: Seventy-three high-resolution computed tomography scans of 44 patients with clinical diagnosis of systemic sclerosis were reviewed and defined by the consensus of two radiologists. Results: Abnormalities were seen in 91.8% (n = 67) of the scans. The most frequent findings were reticular pattern (90.4%), ground-glass opacities (63%), traction bronchiectasis and bronchiolectasis (56.2%), esophageal dilatation (46.6%), honeycombing pattern (28.8%) and signs of pulmonary hypertension (15.6%). In most cases the lesions were bilateral (89%) and symmetrical (58.5%). The lesions were predominantly located in the basal (91.2%) and peripheral (92.2%) regions. Conclusion: In the majority of the patients, progressive systemic sclerosis can cause pulmonary fibrosis mainly characterized by reticular pattern with basal and peripheral distribution on high-resolution computed tomography. (author)

  11. High-resolution CT of the lung (HRCT) in collagen diseases: A prospective study of 73 patients. Hochaufloesende Computertomographie der Lunge (HRCT) bei Kollagenosen: eine prospektive Untersuchung an 73 Patienten

    Energy Technology Data Exchange (ETDEWEB)

    Mueller-Leisse, C. (Klinik fuer Radiologische Diagnostik, RWTH Aachen (Germany)); Bussmann, A.; Mayer, O. (Klinik fuer Radiologische Diagnostik, RWTH Aachen (Germany)); Genth, E.; Guenther, R.W. (Klinik fuer Radiologische Diagnostik, RWTH Aachen (Germany))

    1994-07-01

    To determine pulmonary features of collagenous vascular diseases as assessed by high resolution computed tomography (HRCT) we performed a prospective study of 73 consecutive patients, 44 with rheumatoid arthritis (ra), 11 with progressive systemic sclerosis (pss), 8 with systemic lupus erythematosus (sle), 5 with sjoegren's syndrome, 3 with dermato-/polymyositis and 2 with mixed connective-tissue disease. Pathological lung changes were demonstrated in 70% of patients with ra, 91% with pss, 63% with sle and 60% with the rest. HRCT features included: Intralobular thickening (48%) with a predominance in posterior lower and middle lung areas, pleural thickening (48%) with a predominance in upper lung areas, prominent interlobular septa (37%), subpleural lines (33%), parenchymal bands (33%) with a predominance in lower and anterior lung areas, honeycombing (33%), groundglass pattern (29%) with a predominance in upper and middle, micronodules (18%) with a predominance in upper lung areas and bronchiectasis (14%). HRCT is an important means for the assessment of lung changes associated with collagenous vascular diseases and a definite diagnosis is possible in most cases. (orig.)

  12. [A case of environmental infection with pulmonary Mycobacterium avium complex disease from a residential bathroom of a patient suggested by variable-number tandem-repeat typing of Mycobacterium avium tandem repeat loci].

    Science.gov (United States)

    Taga, Shu; Niimi, Masaki; Kurokawa, Kazuhiro; Nakagawa, Taku; Ogawa, Kenji

    2012-05-01

    A 63-year-old woman was referred to our hospital because of bilateral infiltrations and nodular opacities in her chest radiograph taken in the mass radiography screening in September 2010. The chest computed tomography showed patchy infiltrations with bronchiectasis in the lower lung fields on both sides. She was diagnosed with pulmonary Mycobacterium avium complex (MAC) disease based on the bacteria recovered from the sputum and the bronchoalveolar lavage fluid. To elucidate an environmental MAC source, we investigated her home, and isolated M. avium and M. gordonae from the bathtub and shower tap, respectively, in her residential bathroom. Analysis of the hsp65-PRA variants digested with BamHI and some insertion sequences showed that the clinical strains recovered from sputum and strains from the bathtub were M. avium subsp. hominissuis. A dendrogram of the Mycobacterium avium tandem repeat loci variable-number tandem-repeat (MATR-VNTR) analysis of the MAC strains showed that the bathtub strains formed a polyclonal colonization, and that 1 of the 5 MATR-VNTR patterns was identical to the corresponding pattern of the sputum strain from the patient. In conclusion, we believe that the residential bathroom of the patient was the environmental source of her pulmonary MAC disease, as has been previously reported.

  13. Coincidence of Cystic Fibrosis in Mother and her Child Related to Infertility

    Directory of Open Access Journals (Sweden)

    Alireza Nikzad Jamnani

    2010-01-01

    Full Text Available Cystic fibrosis (CF, the most common life-shortening, hereditary disease in whites, manifestsitself principally in childhood. Patients presenting with CF as adults appear to be different whencompared to patients diagnosed with CF during childhood. Often these patients have been previouslydiagnosed with asthma, chronic bronchitis or emphysema.We present a case of a woman diagnosed with CF at age 37 years. We noticed her finger clubbingduring her son’s hospital admission for CF decompensation. Taking a thorough history, shecomplained of chronic productive cough and was treated for hyper reactive airway disease for manyyears. A Computed Tomography scan was performed which showed bronchiectasis, atelectasis andthe presence of a honey comb pattern in her lung fields. Two sweat tests were performed, both ofwhich were strongly positive. Her CF diagnosis was confirmed.The clinical course of patients receiving a diagnosis of CF in adulthood is largely unknown, butfrequently they have milder disease and a more favorable prognosis. The proportion and number ofpatients with CF diagnosed in adulthood has increased. A large number of these patients present withsubtle symptoms or single-organ disease. Since the majority have pulmonary disease CF should beincluded in the differential diagnosis of chronic respiratory symptoms in adults.

  14. Smoking-related interstitial lung diseases: radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Hidalgo, Alberto [Universidad Autonoma de Barcelona, Department of Radiology, Hospital de Sant Pau, Barcelona (Spain); Hospital de la Santa Creu i Sant Pau, Thoracic Radiology, Department of Radiology, Barcelona (Spain); Franquet, Tomas; Gimenez, Ana; Pineda, Rosa; Madrid, Marta [Universidad Autonoma de Barcelona, Department of Radiology, Hospital de Sant Pau, Barcelona (Spain); Bordes, Ramon [Universidad Autonoma de Barcelona, Department of Pathology, Hospital de Sant Pau, Barcelona (Spain)

    2006-11-15

    Smoking-related interstitial lung diseases (SRILD) are a heterogeneous group of entities of unknown cause. These diseases include desquamative interstitial pneumonia (DIP), respiratory-bronchiolitis-related interstitial lung disease (RB-ILD), pulmonary Langerhans' cell histiocytosis (LCH) and idiopathic pulmonary fibrosis (IPF). High-resolution CT is highly sensitive in the detection of abnormalities in the lung parenchyma and airways. Ground-glass attenuation can occur in DIP and RB-ILD. Whereas DIP is histologically characterized by intra-alveolar pigmented macrophages, RB-ILD shows alveolar macrophages in a patchy peribronchiolar distribution. LCH shows nodular infiltrates on histopathological examination containing varying amounts of characteristic Langerhans' histiocytes. The HRCT findings are characteristically bilateral, symmetrical and diffuse, involving the upper lobe zones with sparing of the costophrenic angles. The most prominent CT features are nodules (sometimes cavitary) measuring 1 to 10 mm in diameter, cysts and areas of ground-glass attenuation. Pathologically, IPF is characterized by its heterogeneity with areas of normal clung, alveolitis and end-stage fibrosis shown in the same biopsy specimen. High-resolution CT findings consist of honeycombing, traction bronchiectasis and intralobular interstitial thickening with subpleural and lower lung predominance. Since coexisting lesions in the same cases have been observed, a better understanding of the different smoking-related interstitial lung diseases (SRILD) allows a more confident and specific diagnosis. (orig.)

  15. Comparison of Chest HRCT in Inspiration and Expiration of patients with Chemical Gas Exposure

    Directory of Open Access Journals (Sweden)

    Kh. Bakhtavar

    2005-08-01

    Full Text Available Background/Objectives: Chemical weapon agents (CWA including Sulfur Mustard (SM, were com-monly used in the Iran-Iraq war and pulmonary complications are known to occur in over half of the exposed patients. Previous studies showed that HRCT in inspiration was normal in most of the patients. In this study comparison between the HRCT findings in deep inspiration and full expiration was carried out. Materials and Method: HRCT in deep inspiration and full expiration in 473 patients with history of chemi-cal gas exposure during the war was performed and the results were compared. The study was prospective during one year since 1382 to 1383. Results: In our study, 366 patients (77% showed normal HRCT in deep inspiration, however in corre-sponding expiration cuts, 54% showed abnormal findings, from which, patchy air trapping was the most common (78%. Other findings in our study were pulmonary fibrotic changes (30%, emphysema (19%, and bronchiectasis (10%. Conclusion: Exposure to SM has pulmonary compli-cations and results in disability in the affected pa-tients. HRCT is normal only in inspiration in most of the affected patients; expiratory HRCT showed patchy air trapping as the most common finding which is suggestive of bronchiolitis obliterans. There-fore, HRCT should be advised to be done both in deep inspiration and full expiration in patients with history of CWA exposure.

  16. Linear opacities on HRCT in bronchiolitis obliterans organising pneumonia

    Energy Technology Data Exchange (ETDEWEB)

    Murphy, J.M.; Flower, C.D.R. [Dept. of Radiology, Addenbrookes Hospital, Univ. of Cambridge (United Kingdom); Schnyder, P.; Leuenberger, P. [Depts. of Radiology and Medicine, University Hospital, CHUV, Lausanne (Switzerland); Verschakelen, J. [Dept. of Radiology, University Hospital, Leuven (Belgium)

    1999-07-01

    The aim of this study was to report the high-resolution computed tomography (HRCT) appearances of linear opacities that may occur in isolation or in combination with other changes in bronchiolitis obliterans organising pneumonia (BOOP). Eleven patients with BOOP and linear opacities on HRCT were identified at three independent teaching hospitals. The HRCT images and clinical course of each patient were reviewed. Two distinct types of linear opacity were identified. The type-1 opacity extended in a radial manner along the line of the bronchi towards the pleura and was usually intimately related to bronchi. The type-2 opacity occurred in a sub-pleural location and bore no relationship to the bronchi. Both types occurred most commonly in the lower lobes, frequently were associated with multi-focal areas of consolidation and usually completely resolved with treatment. There was no associated bronchiectasis, irreversible volume loss or a reticular or honeycomb pattern. In 2 patients linear opacities were the sole abnormality on HRCT. Bronchiolitis obliterans organising pneumonia may occur in a pure ''linear form'' or HRCT may demonstrate linear opacities in addition to multi-focal consolidation. (orig.)

  17. Acute respiratory distress syndrome (ARDS): HRCT findings in survivors

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Jung Im; Park, Seog Hee; Lee, Jae Mun; Song, Jeong Sup; Lee, Kyo Young [The Catholic Univ. College of Medicine, Seoul (Korea, Republic of)

    1999-08-01

    The purpose of this report is to describe the high-resolution computed tomography (HRCT) findings of the lung in survivors of acute respiratory distress syndrome (ARDS). Among eleven patients who survived ARDS for one year, chest radiography and HRCT revealed pulmonary fibrosis in four. Causes of ARDS included pneumonia during pregnancy, near drowning, pneumonia during liver cirrhosis, and postoperative sepsis. Thoracoscopic biopsy and histopathologic correlation were available in one patient. HRCT showed diffuse interlobular septal thickening, ground glass opacity, parenchymal distortion, and traction bronchiectasis. Fuzzy centrilobular nodules were seen in two patients and one patient had multiple, large bullae in the left hemithorax. In all patients, lesions affected the upper and anterior zones of the lung more prominently. The distribution of pulmonary fibrosis was characteristic and reflected the pathogenesis of lung injury; fibrosis was largely due to hyperoxia caused by ventilator care. In one patient, histopathologic correlation showed that imaging findings were accounted for by thickening of the alveolar septum along with infiltration of chronic inflammatory cells and fibrosis. Fuzzy centrilobular nodules corresponded with bronchiolitis.

  18. Pulmonary manifestations of Sjögren's syndrome.

    Science.gov (United States)

    Flament, Thomas; Bigot, Adrien; Chaigne, Benjamin; Henique, Helene; Diot, Elisabeth; Marchand-Adam, Sylvain

    2016-06-01

    In 9-20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD) and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

  19. Pulmonary manifestations of Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Thomas Flament

    2016-06-01

    Full Text Available In 9–20% of cases, Sjögren's syndrome is associated with various respiratory symptoms. The most typical manifestations are chronic interstitial lung disease (ILD and tracheobronchial disease. The most common manifestation of ILD is nonspecific interstitial pneumonia in its fibrosing variant. Other types of ILD, such as organising pneumonia, usual interstitial pneumonia and lymphocytic interstitial pneumonitis, are rare. Their radiological presentation is less distinctive, and definitive diagnosis may require the use of transbronchial or surgical lung biopsy. Corticosteroid therapy is the mainstay of ILD treatment in Sjögren's syndrome, but the use of other immunosuppressive drugs needs to be determined. ILD is a significant cause of death in Sjögren's syndrome. Tracheobronchial disease is common in Sjögren's syndrome, characterised by diffuse lymphocytic infiltration of the airway. It is sometimes responsible for a crippling chronic cough. It can also present in the form of bronchial hyperresponsiveness, bronchiectasis, bronchiolitis or recurrent respiratory infections. The management of these manifestations may require treatment for dryness and/or inflammation of the airways. Airway disease has little effect on respiratory function and is rarely the cause of death in Sjögren's syndrome patients. Rare respiratory complications such as amyloidosis, lymphoma or pulmonary hypertension should not be disregarded in Sjögren's syndrome patients.

  20. [Salmonella typhi vaccination response study reveals defective antibody production selective IgA deficiency patient].

    Science.gov (United States)

    Pleguezuelo, Daniel E; Gianelli, Carla

    2015-01-01

    Selective IgA deficiency (SIgAD) is the most prevalent immunodeficiency worldwide, progressing to common variable immunodeficiency only in few reported cases. We report the case of a Spanish female aged 22 and diagnosed of selective IgA deficiency, a long history of bronchitis, several episodes of pneumonia, bilateral bronchiectasis, normal IgG, IgM, IgG subclasses, and detectable pre-vaccination IgG antibodies against tetanus toxoid and Streptococcus pneumoniae. She was evaluated in our clinic in order to rule out common variable immunodeficiency. We observed good antibody response to tetanus toxoid, absence of circulating switched memory B cells, decreased response to pneumococcal polysaccharide antigens and a lack of response to Salmonella typhi vaccine. Most SIgAD patients presents with upper respiratory tract infections or mild diarrhea. Those with lower tract infections, pneumonia or untreatable diarrhea should follow B-cell subpopulations' study and antibody response to vaccines. Absence of response to Salmonella typhi vaccine allowed us to expose the defective antibody production.

  1. Past, present and future of macrolide therapy for chronic rhinosinusitis in Japan.

    Science.gov (United States)

    Shimizu, Takeshi; Suzaki, Harumi

    2016-04-01

    In 1984, the effectiveness of low-dose, long-term erythromycin treatment (macrolide therapy) for diffuse panbronchiolitis (DPB) was first reported in Japan. The 5-year survival rate for DPB improved from 62.9 to 91.4% after implementation of macrolide therapy. The usefulness of this treatment has since been demonstrated in patients with other chronic airway diseases, such as chronic bronchitis, cystic fibrosis, bronchiectasis, bronchial asthma, and chronic rhinosinusitis (CRS). The new 14-membered macrolides clarithromycin and roxithromycin and the 15-membered macrolide azithromycin are also effective for treating these inflammatory diseases. The mechanism of action of the 14- and 15-membered macrolides may involve anti-inflammatory rather than anti-bacterial activities. Macrolide therapy is now widely used for the treatment of CRS in Japan; it is particularly effective for treating neutrophil-associated CRS and is useful for suppressing mucus hypersecretion. However, macrolide therapy is not effective for eosinophil-predominant CRS, which is characterized by serum and tissue eosinophilia, high serum IgE levels, multiple polyposis, and bronchial asthma. Recent reports have described the clinical efficacy of macrolides in treating other inflammatory diseases and new biological activities (e.g., anti-viral). New macrolide derivatives exhibiting anti-inflammatory but not anti-bacterial activity thus have therapeutic potential as immunomodulatory drugs. The history, current state, and future perspectives of macrolide therapy for treating CRS in Japan will be discussed in this review.

  2. Pleuropulmonary paragonimiasis due to Paragonimus heterotremus: molecular diagnosis, prevalence of infection and clinicoradiological features in an endemic area of northeastern India.

    Science.gov (United States)

    Devi, K Rekha; Narain, Kanwar; Bhattacharya, S; Negmu, K; Agatsuma, Takeshi; Blair, David; Wickramashinghe, S; Mahanta, J

    2007-08-01

    In the northeastern region of India, paragonimiasis is emerging as an important public health problem. However, until now the identity of the species causing human infection has been uncertain and there has been little information on the prevalence and clinicoradiological features of infection in the community. Parasitological and immunological surveys revealed that paragonimiasis was hyperendemic in parts of Arunachal Pradesh. Egg positivity in the sputum was 20.9% and 4.1% in children (age 15 years), respectively. Antibody positivity against excretory-secretory antigen of the adult worm in children and adults was 51.7% and 18.7%, respectively. Chronic cough (97.2%) and haemoptysis (83.3%) were common respiratory symptoms among egg-positive cases. Chest radiography (n=68) images from egg-positive cases showed that air space consolidation (75%), cavitary lesions (14.7%) and mediastinal adenopathy (11.8%) were very frequent. Less frequent findings were nodular lesions, bronchiectasis, mediastinal adenopathy, pleural thickening and pleural effusion. DNA extracted from eggs from the sputum of patients from Arunachal Pradesh was sequenced. Analyses of the second internal transcribed spacer (ITS2) of nuclear rDNA revealed that the species responsible is Paragonimus heterotremus.

  3. Paragonimiasis: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Jeon, Kyung Hyeo; Park, Mi Jung; Bae, Kyung Soo; Choi, Hae Young; Choi, Ho Cheol; Na, Jae Boem; Choi, Dae Seob; Kim, Ho Cheol; Jang, In Seok; Kim, Dong Chul [Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju (Korea, Republic of)

    2013-11-15

    Pleuropulmonary paragonimiasis is a parasitic infection caused by lung flukes including Paragonimus westermani. Paragonimiasis usually occurs from ingestion of raw or improperly cooked freshwater crabs or crayfish. Pleural or lung parenchymal lesions are commonly found on CT or chest radiographs, and radiologic manifestations of pleuropulmonary paragonimiasis vary with the stage of the disease. Early findings include pneumothorax or hydrothorax, focal air-space consolidation, and linear opacities. Later findings include thin-walled cysts, mass-like consolidation, nodules, or bronchiectasis. Pulmonary paragonimiasis often can be mistaken for pulmonary tuberculosis in tuberculosis-endemic areas or lung cancer when it presents as a solitary pulmonary nodule. Intraperitoneal or ectopic lesions such as those in the retroperitoneum can form during migration of a juvenile worm from the small intestine to the lungs. Although the symptoms and signs of pulmonary paragonimiasis are nonspecific, an early diagnosis can be made if radiologists understand the pathogenesis and typical imaging findings of the disease. The purpose of this report was to demonstrate the various imaging findings of pleuropulmonary paragonimiasis and to review articles to help radiologists make a proper diagnosis.

  4. Intraoperative pathology consultation for pulmonary lesions: errors and deferrals.

    Science.gov (United States)

    Akyildiz, Elif Ulker

    2015-01-01

    The accuracy rate of frozen section constitutes an important step of quality assessment step in pathology practice. This study aimed to investigate pulmonary lesions that were incorrectly diagnosed or postponed for routine examination by pathologists at frozen section examination; it also aimed to discuss the reasons for difficult diagnoses and the various clues enabling the correct diagnosis to be made when such lesions are encountered. This study retrospectively reviewed the medical data of the thoracic surgery cases that underwent frozen section examination between 2009 and 2014. Frozen section errors and deferrals were identified in 25 cases. Fourteen (56%) lesions were of pulmonary parenchymal origin and 11 (44%) were of pleural origin. The number of cases in which the pathologists postponed the diagnosis without making any approach was 14. Of these, 9 (64%) were benign lesions such as bronchiectasis, fibrosis anthracosis, chronic inflammatory cell infiltration, chronic pleuritis, and mesothelial proliferation. The number of misdiagnosed cases was 11. Of these, 7 (64%) were of pulmonary and 4 (36%) were of pleural origin. Because the examination techniques of each pathology department may differ from one another, the comparative examination of frozen sections and routine sections would aid in becoming familiar with various pathologies and would be beneficial for pathologists in minimizing their diagnostic errors.

  5. AIDS and lung infection by Mycobacterium xenopi. Role of Computed Tomography; Sindrome da immunodeficienza acquisita e malattie polmonari da Mycobacterium xenopi. Ruolo della Tomografia Computerizzata

    Energy Technology Data Exchange (ETDEWEB)

    Viterbo, V.; Midiri, M.; Stellacci, G.; Angelelli, G.; Rotondo, A. [Bari Univ., Bari (Italy). Dipt. di Medicina Interna e Medicina Pubblica, Ist. di Radiologia; Carbonara, S.; Maggi, P.; Monno, L. [Bari Univ., Bari (Italy). Ist. di Malattie Infettive

    2000-06-01

    Mycobacterium xenopi is one of the most common agents responsible for nontubercolar mycobacterial pulmonary disease on AIDS patients. These lesions have been studied with conventional radiography while CT has been used in patients with aspecific mycobacterioses or non-AIDS pulmonary conditions from Mycobacterium xenopi. 12 AIDS patients were examined. They had pulmonary lesions from Mycobacterium xenopi, patients age ranged 30 to 46 years. All patients had CD4 blood levels lower than 250 cells/mL and Mycobacterium xenopi in the sputum. All patients underwent a standard chest radiograph and a CT examination. CT images were evaluated by three radiologists independently and the definitive diagnosis was made in the presence of a fourth radiologist. Chest CT showed parenchymal consolidation in 66% of cases, associated with bilateral basal bands in 16% of cases. Consolidation was unilateral in 41% of cases and most frequently involved the right lower lobe. Bilateral reticular interstitial involvement was seen in the patients (41%). Micronodules in 1 patient (8%) and mediastinal adenopathy in 33% of cases. Two patients had pre-existing emphysema and 1 had bronchiectasis. The frequency of lung disease from Mycobacterium xenopi has increased because of the spreading of the HIV infection. Such lung lesions in AIDS patients are aspecific in appearance and localization, which the clinical radiologist needs to consider to address treatment planning. The frequent finding of parenchymal consolidation and the absence of cavitary lesions may be referred to the poor capability of AIDS to produce an adequate inflammatory response. The lung lesions tend to distribute in the lower lobes unilaterally. Adenopathy was also a frequent finding. CT plays a fundamental role in studying the chest of these patients because it permits to locate lung lesions with higher accuracy than conventional radiography and to detect adenopathies, micronodules, reticular interstitial involvement and

  6. Follicular bronchiolitis: a rare disease in children

    Science.gov (United States)

    Kanık, Esra Toprak; Yılmaz, Özge; Türkeli, Ahmet; Şahin, Şebnem; Yüksel, Hasan

    2014-01-01

    Follicular bronchiolitis (FB) is a benign progressive lung disease. It is characterized with lymphoplasmocellular infiltration and hyperplastic follicles in the peribronchial areas in the small airways. Follicular bronchiolitis should be considered in cases where chronic cough, recurrent upper respiratory tract infections and progressive dyspnea are observed in children. The diagnosis should be supported by lung biopsy. A 8-year old female patient presented to our hospital with complaints including continuing cough and wheezing. Bilateral extensive rales and rhonchi in the lungs were heard on auscultation and lung graphy revealed reticuloglandular appearance. Bilateral extensive septal thickennings, reticulonodular appearance, patchy bronchiectasis, bronchiolectasis and peribronchial thickennings were found on high-resolution thoracal computarized tomography. A diagnosis of follicular bronchiolitis was made as a result of lung biopsy. Improvement was observed in the complaints and findings of our patient after methylprednisolone treatment. This patient was presented to emphasize rare interstitial lung diseases should also be considered in children who present with a clinical picture of chronic bronchial obstruction and do not respond to standard treatment. PMID:26078687

  7. Bacterial vaccines in chronic obstructive pulmonary disease: effects on clinical outcomes and cytokine levels.

    Science.gov (United States)

    Ruso, Salvador; Marco, Francisco M; Martínez-Carbonell, Juan A; Carratalá, José A

    2015-07-01

    Chronic obstructive pulmonary disease (COPD) is a major cause of mortality and morbidity worldwide. Exacerbation episodes impair lung function leading to disease progression. Levels of inflammation markers correlate with disease severity. Bacterial immunomodulators have shown a beneficial effect in COPD, improving symptoms and reducing the rate of exacerbations. This is an observational prospective study on 30 patients diagnosed with bronchiectasis and COPD, who received bacterial autogenous vaccine for 12 months. The rate of exacerbation, severity of symptoms and lung function were studied at baseline and after treatment. In addition, plasma levels CRP, IL6, IL8, and TNFα were measured. After treatment we found a reduction in mean acute respiratory infections and signs of lung disease. Acute phase proteins IL6 and CRP increased in blood and IL8 decreased. These changes may be related to the repeated injection of inactivated bacteria. Given the implication of these factors in the pathogenesis of COPD, particularly the production of IL8, the causes and consequences of cytokine modulation by bacterial vaccines should be investigated. Vaccination with autogenous vaccines for 1 year can produce a significant clinical improvement in COPD patients, reducing the frequency of exacerbations associated to changes in the profile of markers of inflammation.

  8. Respiratory Disease Related Mortality and Morbidity on an Island of Greece Exposed to Perlite and Bentonite Mining Dust

    Directory of Open Access Journals (Sweden)

    Melina Stoltidis

    2013-10-01

    Full Text Available A morbidity and mortality study took place, focused on Milos Island, where perlite and bentonite mining sites are located. Official data concerning number and cause of deaths, regarding specific respiratory diseases and the total of respiratory diseases, for both Milos Island and the Cyclades Prefecture were used. Standardized Mortality Ratios (SMRs were computed, adjusted specifically for age, gender and calendar year. Tests of linear trend were performed. By means of a predefined questionnaire, the morbidity rates of specific respiratory diseases in Milos, were compared to those of the municipality of Oinofita, an industrial region. Chi-square analysis was used and the confounding factors of age, gender and smoking were taken into account, by estimating binary logistic regression models. The SMRs for Pneumonia and Chronic Obstructive Pulmonary Disease (COPD were found elevated for both genders, although they did not reach statistical significance. For the total of respiratory diseases, a statistically significant SMR was identified regarding the decade 1989–1998. The morbidity study revealed elevated and statistically significant Odds Ratios (ORs, associated with allergic rhinitis, pneumonia, COPD and bronchiectasis. An elevated OR was also identified for asthma. After controlling for age, gender and smoking, the ORs were statistically significant and towards the same direction.

  9. High-resolution CT of nontuberculous mycobacterium infection in adult CF patients: diagnostic accuracy

    Energy Technology Data Exchange (ETDEWEB)

    McEvoy, Sinead; Lavelle, Lisa; Kilcoyne, Aoife; McCarthy, Colin; Dodd, Jonathan D. [St. Vincent' s University Hospital, Department of Radiology, Dublin (Ireland); DeJong, Pim A. [University Medical Center Utrecht, Department of Radiology, Utrecht (Netherlands); Loeve, Martine; Tiddens, Harm A.W.M. [Erasmus MC-Sophia Children' s Hospital, Department of Radiology, Department of Pediatric Pulmonology and Allergology, Rotterdam (Netherlands); McKone, Edward; Gallagher, Charles G. [St. Vincent' s University Hospital, Department of Respiratory Medicine and National Referral Centre for Adult Cystic Fibrosis, Dublin (Ireland)

    2012-12-15

    To determine the diagnostic accuracy of high-resolution computed tomography (HRCT) for the detection of nontuberculous mycobacterium infection (NTM) in adult cystic fibrosis (CF) patients. Twenty-seven CF patients with sputum-culture-proven NTM (NTM+) underwent HRCT. An age, gender and spirometrically matched group of 27 CF patients without NTM (NTM-) was included as controls. Images were randomly and blindly analysed by two readers in consensus and scored using a modified Bhalla scoring system. Significant differences were seen between NTM (+) and NTM (-) patients in the severity of the bronchiectasis subscore [45 % (1.8/4) vs. 35 % (1.4/4), P = 0.029], collapse/consolidation subscore [33 % (1.3/3) vs. 15 % (0.6/3)], tree-in-bud/centrilobular nodules subscore [43 % (1.7/3) vs. 25 % (1.0/3), P = 0.002] and the total CT score [56 % (18.4/33) vs. 46 % (15.2/33), P = 0.002]. Binary logistic regression revealed BMI, peribronchial thickening, collapse/consolidation and tree-in-bud/centrilobular nodules to be predictors of NTM status (R{sup 2} = 0.43). Receiver-operator curve analysis of the regression model showed an area under the curve of 0.89, P < 0.0001. In adults with CF, seven or more bronchopulmonary segments showing tree-in-bud/centrilobular nodules on HRCT is highly suggestive of NTM colonisation. (orig.)

  10. Description and validation of a scoring system for tomosynthesis in pulmonary cystic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Vult von Steyern, Kristina; Bjoerkman-Burtscher, Isabella M.; Bozovic, Gracijela; Wiklund, Marie; Geijer, Mats [Skaane University Hospital, Lund University, Centre for Medical Imaging and Physiology, Lund (Sweden); Hoeglund, Peter [Skaane University Hospital, Competence Centre for Clinical Research, Lund (Sweden)

    2012-12-15

    To design and validate a scoring system for tomosynthesis (digital tomography) in pulmonary cystic fibrosis. A scoring system dedicated to tomosynthesis in pulmonary cystic fibrosis was designed. Three radiologists independently scored 88 pairs of radiographs and tomosynthesis examinations of the chest in 60 patients with cystic fibrosis and 7 oncology patients. Radiographs were scored according to the Brasfield scoring system and tomosynthesis examinations were scored using the new scoring system. Observer agreements for the tomosynthesis score were almost perfect for the total score with square-weighted kappa >0.90, and generally substantial to almost perfect for subscores. Correlation between the tomosynthesis score and the Brasfield score was good for the three observers (Kendall's rank correlation tau 0.68, 0.77 and 0.78). Tomosynthesis was generally scored higher as a percentage of the maximum score. Observer agreements for the total score for Brasfield score were almost perfect (square-weighted kappa 0.80, 0.81 and 0.85). The tomosynthesis scoring system seems robust and correlates well with the Brasfield score. Compared with radiography, tomosynthesis is more sensitive to cystic fibrosis changes, especially bronchiectasis and mucus plugging, and the new tomosynthesis scoring system offers the possibility of more detailed and accurate scoring of disease severity. (orig.)

  11. Magnetomotive optical coherence elastography for relating lung structure and function in cystic fibrosis

    Science.gov (United States)

    Chhetri, Raghav K.; Carpenter, Jerome; Superfine, Richard; Randell, Scott H.; Oldenburg, Amy L.

    2010-02-01

    Cystic fibrosis (CF) is a genetic defect in the cystic fibrosis transmembrane conductance regulator protein and is the most common life-limiting genetic condition affecting the Caucasian population. It is an autosomal recessive, monogenic inherited disorder characterized by failure of airway host defense against bacterial infection, which results in bronchiectasis, the breakdown of airway wall extracellular matrix (ECM). In this study, we show that the in vitro models consisting of human tracheo-bronchial-epithelial (hBE) cells grown on porous supports with embedded magnetic nanoparticles (MNPs) at an air-liquid interface are suitable for long term, non-invasive assessment of ECM remodeling using magnetomotive optical coherence elastography (MMOCE). The morphology of ex vivo CF and normal lung tissues using OCT and correlative study with histology is also examined. We also demonstrate a quantitative measure of normal and CF airway elasticity using MMOCE. The improved understanding of pathologic changes in CF lung structure and function and the novel method of longitudinal in vitro ECM assessment demonstrated in this study may lead to new in vivo imaging and elastography methods to monitor disease progression and treatment in cystic fibrosis.

  12. Pulmonary echinococcal cyst with a filamentous fungus co-infection.

    Science.gov (United States)

    Pandey, P; Dixit, A K; Tanwar, A; Mahajan, N C

    2013-09-01

    Fungal infections are known to colonize the pre-existing lung cavities formed as a result of diseases like tuberculosis, sarcoidosis, bronchiectasis and cavitatary neoplasia, mostly encountered in immunocompromised patients. Pulmonary echinococcal cysts have been reported coexistent with cryptococcosis and other saprophytic mycosis, but the coexistence of aspergillosis and echinococcal cyst is extremely rare and occasionally been reported in English literature. Active invasion and proliferation of the fungi in the laminated ectocyst of the echinococcal cyst is very unusual. We report a case of 60 years old immunocompetent female, presented with cough, chest pain and shortness of breath. The chest X-ray showed a large thick walled cavity in the lower and mid zone of right lung with positive water lily sign. Surgical enucleation of the echinococcal cyst revealed aspergilloma involving the cavity with massive invasion of laminated ectocyst by filamentous fungus, morphologically resembling an Aspergillus species and was further treated with Itraconazole for 3 months. This unique coexistence of active pulmonary echinococcosis and aspergillosis is being reported because of its rarity and clinical importance for its management.

  13. Patterns of airway involvement in inflammatory bowel diseases

    Institute of Scientific and Technical Information of China (English)

    Ilias; Papanikolaou; Konstantinos; Kagouridis; Spyros; A; Papiris

    2014-01-01

    Extraintestinal manifestations occur commonly in inflammatory bowel diseases(IBD). Pulmonary manifestations(PM) of IBD may be divided in airway disorders, interstitial lung disorders, serositis, pulmonary vasculitis, necrobiotic nodules, drug-induced lung disease, thromboembolic lung disease and enteropulmonary fistulas. Pulmonary involvement may often be asymptomatic and detected solely on the basis of abnormal screening tests. The common embryonic origin of the intestine and the lungs from the primitive foregut, the co-existence of mucosa associated lymphoid tissue in both organs, autoimmunity, smoking and bacterial translocation from the colon to the lungs may all be involved in the pathogenesis of PM in IBD. PM are mainly detected by pulmonary function tests and highresolution computed tomography. This review will focus on the involvement of the airways in the context of IBD, especially stenoses of the large airways, tracheo-bronchitis, bronchiectasis, bronchitis, mucoid impaction, bronchial granulomas, bronchiolitis, bronchiolitis obliterans syndrome and the co-existence of IBD with asthma, chronic obstructive pulmonary disease, sarcoidosis and a1-antitrypsin deficiency.

  14. Changes in CT imaging of monkey pulmonary damage induced by rush decompression%极快速减压致猴肺损伤的CT影像学变化

    Institute of Scientific and Technical Information of China (English)

    杨学东; 周建光; 洪伟; 张志良; 马戈; 叶喜林; 温阿明

    2013-01-01

    Objective To evaluate changes in CT imaging of pulmonary damage induced by rush decompression in monkeys.Methods Two adult monkeys received CT scanning 2 hours after rush decompression,and then observations were made on the pulmonary CT imaging in the monkeys.Results Rush decompression could induce pulmonary damage to various extents.CT scanning revealed the following manifestations:patchy shadows in the lungs,pulmonary cysts,pneumothorax,mediastinal emphysema and bronchiectasis.Conclusions CT scanning was a quick and effective method for the detection and diagnosis of pulmonary lesion induced by rush decompression.%目的 探讨极快速减压致猴肺损伤的CT表现.方法 成年猴2只,极快速减压后2h内进行CT扫描,观察猴肺的CT表现.结果 极快速减压可引起不同程度的肺损伤,CT主要表现为肺片状阴影、肺气囊肿、气胸、纵隔气肿和气管支气管扩张等.结论 CT检查快速、准确,是诊断极快速减压性肺损伤的有效方法.

  15. Clinical and Immunological Features of Common Variable Immunodeficiency in China

    Institute of Scientific and Technical Information of China (English)

    Lian-Jun Lin; Yu-Chuan Wang; Xin-Min Liu

    2015-01-01

    Background:Common variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes.The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment.Methods:Clinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively.Results:The median age at onset was 11-year-old (range 4-51 years).The median age at diagnosis was 14.5-year-old (range 5-66 years).The average time of delay in diagnosis was 5.3 years (range 1-41 years).The most common main complaint was fever due to infections (35 cases,87.5%).Pneumonia (28 cases,70%) was the most common type of infections.Bronchiectasis was present in 6 patients (15%).Autoimmune disease was detected in 6 cases of CVID,and malignancy in 2 cases.The median total serum levels of IgG,IgA,and IgM at diagnosis were 1.07 g/L,0.07 g/L,and 0.28 g/L,respectively.The percentages ofCD3/CD19+ B-cells were 1%-3.14%.Conclusions:Infection is the most frequent presentation of CVID.Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis.Regular and sufficient substitution with Ig is recommended.

  16. Tomographic Aspects of Advanced Active Pulmonary Tuberculosis and Evaluation of Sequelae following Treatment

    Directory of Open Access Journals (Sweden)

    Rafael Barcelos Capone

    2017-01-01

    Full Text Available Objectives. To evaluate tomographic changes in pulmonary tuberculosis (TB, degree of agreement among three radiologists regarding tomographic diagnoses, and sequelae following treatment. Methods. Cross-sectional and descriptive study of 74 TB patients confirmed by sputum culture and chest computed tomography before (CT1 and 6 months after (CT2 drug therapy. Results were performed by three radiologists blinded to clinical and laboratory results. Results. Main findings in CT1 included nodules indicating the presence of a tree-in-bud pattern in 93% of cases, ill-defined nodules in 84% of cases, consolidation in 77% of cases, architectural distortion in 71% of cases, cavitary lesions in 62% of cases, and ground glass opacities in 37% of cases. Airway involvement, characterized by increased thickness and dilatation of the bronchial walls, occurred in 93% of cases. Pleural involvement occurred in 54%. There was an agreement on active TB among the three radiologists in 85% of cases. The results in CT2 indicated the presence of architectural distortion in 91% of cases and cylindrical bronchiectasis in 86%. Conclusions. The study established a tomographic pattern for diagnosis of active TB characterized by the presence of airway nodules, consolidation, architectural distortion, and cavitary lesions, and an almost complete degree of agreement (Kappa was observed among the radiologists (0.85. CT after treatment assists in defining the cure.

  17. Macrolide antibiotics and the risk of cardiac arrhythmias.

    Science.gov (United States)

    Albert, Richard K; Schuller, Joseph L

    2014-05-15

    Randomized, controlled trials have demonstrated that chronic therapy with macrolide antibiotics reduces the morbidity of patients with cystic fibrosis, non-cystic fibrosis bronchiectasis, chronic obstructive pulmonary disease, and nontuberculous mycobacterial infections. Lower levels of evidence indicate that chronic macrolides are also effective in treating patients with panbronchiolitis, bronchiolitis obliterans, and rejection after lung transplant. Macrolides are known to cause torsade des pointes and other ventricular arrhythmias, and a recent observational study prompted the FDA to strengthen the Warnings and Precautions section of azithromycin drug labels. This summary describes the electrophysiological effects of macrolides, reviews literature indicating that the large majority of subjects experiencing cardiac arrhythmias from macrolides have coexisting risk factors and that the incidence of arrhythmias in absence of coexisting risk factors is very low, examines recently published studies describing the relative risk of arrhythmias from macrolides, and concludes that this risk has been overestimated and suggests an approach to patient evaluation that should reduce the relative risk and the incidence of arrhythmias to the point that chronic macrolides can be used safely in the majority of subjects for whom they are recommended.

  18. Interstitial lung disease in an adult with Fanconi anemia: Clues to the pathogenesis

    Energy Technology Data Exchange (ETDEWEB)

    Rubinstein, W.S.; Wenger, S.L.; Hoffman, R.M. [Univ. of Pittsburgh, PA (United States)] [and others

    1997-03-31

    We have studied a 38-year-old man with a prior diagnosis of Holt-Oram syndrome, who presented with diabetes mellitus. He had recently taken prednisone for idiopathic interstitial lung disease and trimethoprim-sulfamethoxazole for sinusitis. Thrombocytopenia progressed to pancytopenia. The patient had skeletal, cardiac, renal, cutaneous, endocrine, hepatic, neurologic, and hematologic manifestations of Fanconi anemia (FA). Chest radiographs showed increased interstitial markings at age 25, dyspnea began in his late 20s, and he stopped smoking at age 32. At age 38, computerized tomography showed bilateral upper lobe fibrosis, lower lobe honeycombing, and bronchiectasis. Pulmonary function tests, compromised at age 29, showed a moderately severe obstructive and restrictive pattern by age 38. Serum alpha-1 antitrypsin level was 224 (normal 85-213) mg/dL and PI phenotype was M1. Karyotype was 46,X-Y with a marked increase in chromosome aberrations induced in vitro by diepoxybutane. The early onset and degree of pulmonary disease in this patient cannot be fully explained by environmental or known genetic causes. The International Fanconi Anemia Registry (IFAR) contains no example of a similar pulmonary presentation. Gene-environment (ecogenetic) interactions in FA seem evident in the final phenotype. The pathogenic mechanism of lung involvement in FA may relate to oxidative injury and cytokine anomalies. 49 refs., 2 figs., 1 tab.

  19. Lung transplantation: overall approach regarding its major aspects

    Science.gov (United States)

    de Camargo, Priscila Cilene León Bueno; Teixeira, Ricardo Henrique de Oliveira Braga; Carraro, Rafael Medeiros; Campos, Silvia Vidal; Afonso, José Eduardo; Costa, André Nathan; Fernandes, Lucas Matos; Abdalla, Luis Gustavo; Samano, Marcos Naoyuki; Pêgo-Fernandes, Paulo Manuel

    2015-01-01

    ABSTRACT Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation. Lung transplantation is most often indicated in cases of COPD, cystic fibrosis, interstitial lung disease, non-cystic fibrosis bronchiectasis, and pulmonary hypertension. This comprehensive review aimed to address the major aspects of lung transplantation: indications, contraindications, evaluation of transplant candidates, evaluation of donor candidates, management of transplant recipients, and major complications. To that end, we based our research on the International Society for Heart and Lung Transplantation guidelines and on the protocols used by our Lung Transplant Group in the city of São Paulo, Brazil. PMID:26785965

  20. Severe lung injuries due to SO/sub 2/ inhalation

    Energy Technology Data Exchange (ETDEWEB)

    Wunderlich, P.; Leupold, W.; Mittenzwey, K.W.; Rupprecht, E.

    1982-01-01

    By accident a 12 year old boy had to spend 4 hours in a SO/sub 2/-enriched atmosphere (concentration 13.8 mg per cubicmeter = 4.8 ppm). The course of the following intoxication was tetraphasic: 1. acute irritation of the mucous membranes of the upper airways and of the eye: rhinopharyngitis, laryngitis and bronchitis, conjunctivitis and corneal lesions (duration: 5 days), 2. symptom free interval (duration: 3 days), 3. subacute destruction of the low airways and the lung: destructing bronchitis, bronchiolitis, alveolitis, emphysema of the lung, mediastinum and skin, gradual development of bronchiectasis (duration: 9-12 months) and 4. gradual transition into terminal scarification: emphysema of the lung, continuous partial respiratory insufficiency with combined, especially obstructive disturbance of ventilation (without alterations observed during the last 4 years). On occasions of this fateful and therapeutically hardly influenced course recommendations are given for initial general and topical administration of very high doses of corticosteroids in each case of inhalative intoxication as the only measure which probably would have prevented it.

  1. Automatic 2D segmentation of airways in thorax computed tomography images; Segmentacao automatica 2D de vias aereas em imagens de tomografia computadorizada do torax

    Energy Technology Data Exchange (ETDEWEB)

    Cavalcante, Tarique da Silveira; Cortez, Paulo Cesar; Almeida, Thomaz Maia de, E-mail: tarique@lesc.ufc.br [Universidade Federal do Ceara (UFC), Fortaleza, CE (Brazil). Dept. de Engenharia de Teleinformatica; Felix, John Hebert da Silva [Universidade da Integracao Internacional da Lusofonia Afro-Brasileira (UNILAB), Redencao, CE (Brazil). Departamento de Energias; Holanda, Marcelo Alcantara [Universidade Federal do Ceara (UFC), Fortaleza, CE (Brazil). Fac. de Medicina

    2013-07-01

    Introduction: much of the world population is affected by pulmonary diseases, such as the bronchial asthma, bronchitis and bronchiectasis. The bronchial diagnosis is based on the airways state. In this sense, the automatic segmentation of the airways in Computed Tomography (CT) scans is a critical step in the aid to diagnosis of these diseases. Methods: this paper evaluates algorithms for airway automatic segmentation, using Neural Network Multilayer Perceptron (MLP) and Lung Densities Analysis (LDA) for detecting airways, along with Region Growing (RG), Active Contour Method (ACM) Balloon and Topology Adaptive to segment them. Results: we obtained results in three stages: comparative analysis of the detection algorithms MLP and LDA, with a gold standard acquired by three physicians with expertise in CT imaging of the chest; comparative analysis of segmentation algorithms ACM Balloon, ACM Topology Adaptive, MLP and RG; and evaluation of possible combinations between segmentation and detection algorithms, resulting in the complete method for automatic segmentation of the airways in 2D. Conclusion: the low incidence of false negative and the significant reduction of false positive, results in similarity coefficient and sensitivity exceeding 91% and 87% respectively, for a combination of algorithms with satisfactory segmentation quality. (author)

  2. KARTAGENER’S SYNDROME PRESENTED AS PARANASAL POLYPOSIS WITH RECURRENT EPISTAXIS: A RARE CLINICAL CASE REPORT

    Directory of Open Access Journals (Sweden)

    Palaparti Jayakar Babu

    2012-02-01

    Full Text Available Genetically determined syndromes of ciliary dyskinesia prevent normal transport of mucus from the bronchial tree to the mouth and result in serious impairment of lung defence system.male infertility was sometimes associated with Immotile spermatozoa. Approximately half of patients with Primary Ciliary Dyskinesia have full triad of kartgeners’s syndrome, give history of recurrent sinusitis and lower respiratory tract infection from early life to adulthood. Kartagener’s syndrome has been considered to be a sub group in a heterogenous collection of disorders to which Immotile Ciliary Syndrome or Dyskinitic cilia syndrome have been applied. There may also be a link with retinitis pigmentosa and hearing loss. Kartagener’s syndrome with paranasal polyposis is a uncommon presentation shown in our case. We report an adult female of 23 of age having Recurrent sinusitis,Bronchiectasis and Dextrocardia with Situs inversus and with Paranasal polyposis showing recurrent epistaxis. Conclusion: Kartagener’s syndrome with paranasal sinusitis is common but paranasal polyposis with epistaxis is uncommon way of presentation. [National J of Med Res 2012; 2(1.000: 99-101

  3. Complete Kartagener's syndrome with left pneumothorax and Ⅰ breath prostrate: a case report and literature review%完全性Kartagener综合征合并左侧气胸、Ⅰ型呼吸衰竭1例并文献复习

    Institute of Scientific and Technical Information of China (English)

    常双喜; 卢春玲; 禹彩霞; 李宝珠

    2014-01-01

    目的 分析Kartagener综合征的临床特点及诊治方法.方法 分析1例Kartagener综合征患者的诊治过程及文献复习.结果 患者,女,47岁,因反复出现呼吸道症状入院,诊断Kartagener综合征,经过对症治疗症状缓解.结论 Kartagener综合征属于先天性常染色体隐性遗传性疾病,全内脏反位、支气管扩张、鼻窦炎三联征可以明确诊断,诊断主要以影像学检查为依据,无特殊治疗方法,主要为对症治疗.%Objective To analyze the clinical features,diagnosis,and treatment of Kartagener's syndrome (KS).Methods One case of KS was reported and the related literatures were reviewed.Results The patient with KS was a 47 years old female who had been admitted to hospital several times because of recurrent respiratory symptoms,which were relieved after symptomatic treatment.Conclusions KS is belong to congenital autosomal recessive hereditary disease.Imaging findings play important roles in the diagnosis of KS,if it has the classical triad of visceral inversion,bronchiectasis,and accessory sinusitis.Symptomatic treatment is the main methods.

  4. Primary ciliary dyskinesia: considerations regarding six cases of Kartagener syndrome.

    Science.gov (United States)

    Ortega, Hugo Alejandro Vega; Vega, Nelson de Araujo; Santos, Bruno Quirino Dos; Maia, Guilherme Tavares da Silva

    2007-01-01

    Primary ciliary dyskinesia (PCD), previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1:20,000 to 1:60,000. Since PCD causes deficiency or even stasis of the transport of secretions throughout the respiratory tract, it favors the growth of viruses and bacteria. As a result, patients have lifelong chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. Bronchiectasis and other chronic conditions infections can be the end result of the irreversible bronchial alterations, leading to chronic cor pulmonale and its consequences. Only half of the patients affected by PDC present all of the symptoms, a condition designated complete KS, compared with incomplete KS, typically defined as cases in which situs inversus does not occur. The diagnosis is made clinically and confirmed through transmission electron microscopy. Since there is no specific therapy for PCD, it is recommended that, upon diagnosis, secondary infections be treated with potent antibiotics and prophylactic interventions be implemented. In this paper, we report six cases of PCD (five cases of complete KS and one case of KS) and review the related literature, focusing on the diagnostic, therapeutic and clinical aspects of this disease.

  5. Axonemal dynein intermediate-chain gene (DNAI1) mutations result in situs inversus and primary ciliary dyskinesia (Kartagener syndrome).

    Science.gov (United States)

    Guichard, C; Harricane, M C; Lafitte, J J; Godard, P; Zaegel, M; Tack, V; Lalau, G; Bouvagnet, P

    2001-04-01

    Kartagener syndrome (KS) is a trilogy of symptoms (nasal polyps, bronchiectasis, and situs inversus totalis) that is associated with ultrastructural anomalies of cilia of epithelial cells covering the upper and lower respiratory tracts and spermatozoa flagellae. The axonemal dynein intermediate-chain gene 1 (DNAI1), which has been demonstrated to be responsible for a case of primary ciliary dyskinesia (PCD) without situs inversus, was screened for mutation in a series of 34 patients with KS. We identified compound heterozygous DNAI1 gene defects in three independent patients and in two of their siblings who presented with PCD and situs solitus (i.e., normal position of inner organs). Strikingly, these five patients share one mutant allele (splice defect), which is identical to one of the mutant DNAI1 alleles found in the patient with PCD, reported elsewhere. Finally, this study demonstrates a link between ciliary function and situs determination, since compound mutation heterozygosity in DNAI1 results in PCD with situs solitus or situs inversus (KS).

  6. Foreign bodies in the torax

    Directory of Open Access Journals (Sweden)

    Ćerimagić Zenaida

    2007-01-01

    Full Text Available Background. Metallic foreign bodies in the lung could be recognized using radiography. Non-metallic foreign bodies make difficulties because they are not as dense as metals. The aim of this report was to present the case of nonmetallic foreign bodies in the lung. Case report. A soldier of the Federation Army of Bosnia and Hertzegovina, injured in the explosion in 1998 was presented. The soldier was subjected to thoracic drainage and surgery tretment of the wound. After one year, the pulmologist treated him with tuberculostatics because of hemoptysis and pulmonary infiltration shown by a radiogram. This therapy had no effects, and the pulmologist presented this case to the thoracic surgeon who made thoracotomy removing a piece of wood (13 × 2 × 0.7 cm, 20 different-size pieces of wood, and a piece of textile from the lung. Conclusion. Patients with penetrating explosive lung injuries without metallic foreign bodies shown by a radiogram, with complications as hemoptisis, lung abscess, bronchiectasis, obstruction of the bronchus, chronic pneumonia should be subjected to thoracotomy for removing suspected foreign bodies without resecting the lung.

  7. Paraquat induced lung injury: long-term follow-up of HRCT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Young Tong; Kim, Hyun Cheol; Bae, Won Kyung; Kim, Il Young; Im, Han Hyek [Soonchunhyang Univ., Chunan (Korea, Republic of)

    2004-03-01

    To determine the long-term follow-up CT findings of paraquat-induced lung injury. Six patients who ingested paraquat underwent sequential follow-up CT scanning during a period of at least six months, and the results were analysed. Scans were obtained 1-6 (mean, 3.3) time during a 7-84 (mean, 25.7) months period, and the findings at 1-2 months, 3-12 months, 1-2 years, 2-3 years and more than above 7 years after poisoning were analyzed. We observed irregular-shaped areas of consolidation with traction bronchiectasis at 1-2 months (5/5), irregular-shaped consolidation and ground-glass opacity (5/5) at 3-12 months, and irregular-shaped consolidations/ground-glass opacity (4/5) and focal honeycombing (1/5) one year later. In the same patients, follow-up CT scans showed that some areas of focal consolidation could not be visualized and the radio-opacity of the lesions had decreased. The HRCT findings of paraquat-induced lung injury were irregular shaped areas of consolidation 1-2 months after ingestion, and irregular-shaped consolidation and ground-glass opacity or focal honeycombing 3-12 months later. At this thim slight improvement was observed.

  8. Molecular Basis for Mucolytic Therapy

    Directory of Open Access Journals (Sweden)

    Bonnie Dasgupta

    1995-01-01

    Full Text Available Airway mucus is a complex, viscoelastic gel that has a three-dimensional structure. It is composed of water, mucous glycoproteins, low molecular weight ions, proteins and lipids. The three-dimensional structure of the mucous gel depends on a number of forms of bonding, such as ionic bonds and disulphide bridges. Airway obstruction in cystic fibrosis (CF lung disease is accompanied by the accumulation of thick and viscous secretions resulting from chronic infection and inflammation, promoting recurrent exacerbations. The normal, free-flow ing airway mucus becomes thick and purulent in patients suffering from CF lung disease. Therefore, current approaches to the treatment of CF include strategics for changing the physical properties of pulmonary secretions with the goal of improving airway clearance. Some of the same strategies may be applicable in the larger group of patients with chronic obstructive airway diseases, including bronchiectasis and chronic obstructive pulmonary disease. This paper reviews various approaches to mucolysis based on the molecular nature of crosslinking and bonding in mucin gels. A brief review of the structure and biochemistry of airway mucus is followed by a discussion of the various physical and biochemical approaches to mucolysis. Seven representative mucotropic modalities are presented: N-acetylcysteine; urea; hypertonic saline; recombinant human DNase; gelsolin; oscillation; and surfactants. Each of these mucotropic modalities acts on a different component within the mucous gel. Finally, the possibilities of mucolytic synergism among these various agents are conside red.

  9. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.

    LENUS (Irish Health Repository)

    Rogan, Mark P

    2012-02-01

    Recent advances in basic science have greatly expanded our understanding of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR), the chloride and bicarbonate channel that is encoded by the gene, which is mutated in patients with CF. We review the structure, function, biosynthetic processing, and intracellular trafficking of CFTR and discuss the five classes of mutations and their impact on the CF phenotype. The therapeutic discussion is focused on the significant progress toward CFTR mutation-specific therapies. We review the results of encouraging clinical trials examining orally administered therapeutics, including agents that promote read-through of class I mutations (premature termination codons); correctors, which overcome the CFTR misfolding that characterizes the common class II mutation F508del; and potentiators, which enhance the function of class III or IV mutated CFTR at the plasma membrane. Long-term outcomes from successful mutation-specific treatments could finally answer the question that has been lingering since and even before the CFTR gene discovery: Will therapies that specifically restore CFTR-mediated chloride secretion slow or arrest the deleterious cascade of events leading to chronic infection, bronchiectasis, and end-stage lung disease?

  10. Allergic bronchopulmonary aspergillosis: a rare cause of pleural effusion.

    LENUS (Irish Health Repository)

    O'Connor, T M

    2012-02-03

    Aspergillus fumigatus is one of the most ubiquitous of the airborne saprophytic fungi. Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis, and is characterized by hypersensitivity to chronic colonization of the airways with A. fumigatus. We report the case of a patient with ABPA presenting with pleural effusion. A 27-year-old male was referred with recurrent right pleural effusion. Past medical history was remarkable for asthma, allergic sinusitis, and recurrent pleurisy. Investigations revealed peripheral eosinophilia with elevated serum immunoglobulin E and bilateral pleural effusions with bilateral upper lobe proximal bronchiectasis. Precipitating serum antibodies to A. fumigatus were positive and the A. fumigatus immediate skin test yielded a positive reaction. A diagnosis of ABPA associated with bilateral pleural effusions was made and the patient was commenced on prednisolone. At review, the patient\\'s symptoms had considerably improved and his pleural effusions had resolved. ABPA may present with diverse atypical syndromes, including paratracheal and hilar adenopathy, obstructive lung collapse, pneumothorax and bronchopleural fistula, and allergic sinusitis. Allergic bronchopulmonary aspergillosis is a rare cause of pleural effusion and must be considered in the differential diagnosis of patients presenting with a pleural effusion, in particular those with a history of asthma.

  11. From a single whole exome read to notions of clinical screening: primary ciliary dyskinesia and RSPH9 p.Lys268del in the Arabian Peninsula.

    Science.gov (United States)

    Alsaadi, Muslim M; Gaunt, Tom R; Boustred, Christopher R; Guthrie, Philip A I; Liu, Xuan; Lenzi, Luca; Rainbow, Lucille; Hall, Neil; Alharbi, Khalid K; Day, Ian N M

    2012-05-01

    Primary ciliary dyskinesia (PCD) is a genetic disorder, usually autosomal recessive, causing early respiratory disease and later subfertility. Whole exome sequencing may enable efficient analysis for locus heterogeneous disorders such as PCD. We whole-exome-sequenced one consanguineous Saudi Arabian with clinically diagnosed PCD and normal laterality, to attempt ab initio molecular diagnosis. We reviewed 13 known PCD genes and potentially autozygous regions (extended homozygosity) for homozygous exon deletions, non-dbSNP codon, splice-site base variants or small indels. Homozygous non-dbSNP changes were also reviewed exome-wide. One single molecular read representing RSPH9 p.Lys268del was observed, with no wild-type reads, and a notable deficiency of mapped reads at this location. Among all observations, RSPH9 was the strongest candidate for causality. Searching unmapped reads revealed seven more mutant reads. Direct assay for p.Lys268del (MboII digest) confirmed homozygosity in the affected individual, then confirmed homozygosity in three siblings with bronchiectasis. Our finding in southwest Saudi Arabia indicates that p.Lys268del, previously observed in two Bedouin families (Israel, UAE), is geographically widespread in the Arabian Peninsula. Analogous with cystic fibrosis CFTR p.Phe508del, screening for RSPH9 p.Lys268del (which lacks sentinel dextrocardia) in those at risk would help in early diagnosis, tailored clinical management, genetic counselling and primary prevention.

  12. Clinical Pearls in pediatric infections.

    Science.gov (United States)

    Singhi, Sunit; Mathew, Joseph; Jindal, Atul; Verma, Sanjay

    2011-12-01

    This series of Clinical Pearls presents four cases presenting with infection. Each of these cases had clinical clues to the correct diagnosis, which could be picked up on meticulous history, clinical examination, or basic laboratory investigations. The authors highlight the important lessons to be learnt from each case. The first is a 7 year old boy with recurrent respiratory tract infections since early life. Clinical examination revealed the presence of dextrocardia and situs inversus and bronchiectasis leading to a diagnosis of Primary Ciliary Dyskinesia. The second case is a 1.5-month-old infant who presented with meningitis and increasing head size since birth. CSF examination and CT scanning led to the correct diagnosis of congenital Toxoplasmosis. The next case is an infant with high grade fever and neck swelling. He had the rare Lemierre's syndrome comprising of oro-pharyngeal infection, suppurative thrompbophlebitis of the internal jugular vein and systemic dissemination of septic emboli. The fourth case is a 2-year-old infant with recurrent respiratory tract infections and discharging neck swellings from early life. Repeated testing for tuberculosis was negative. The diagnosis was Chronic granulomatous disease. The authors describe the clinical approach and investigations in these cases; along with an outline of the management.

  13. Síndrome da unha amarela Yellow nail syndrome

    Directory of Open Access Journals (Sweden)

    Renato Maciel

    2005-10-01

    Full Text Available A síndrome da unha amarela é uma entidade clínica rara caracterizada por três achados principais: alterações distróficas e de coloração das unhas, linfedema e derrame pleural. Bronquiectasias e rinossinusite crônica têm sido freqüentemente associadas. Relatamos o caso de uma paciente com os achados completos da síndrome, com derrame pleural bilateral e que estava em tratamento de tuberculose pulmonar havia nove meses. Na sua história familiar havia a descrição de dois casos semelhantes, em irmã e irmão.Yellow nail syndrome is a rare disorder characterized by three main features: discoloration of the nails, together with dystrophic alterations; lymphedema; and pleural effusion. It is often accompanied by bronchiectasis and chronic rhinosinusitis. Herein, we report a case of the complete syndrome with bilateral pleural effusion in a patient under treatment for pulmonary tuberculosis for nine months. There was a family history of two similar cases in siblings.

  14. Chronic interstitial pneumonia with honeycombing in coal workers

    Energy Technology Data Exchange (ETDEWEB)

    Brichet, A.; Tonnel, A.B.; Brambilla, E.; Devouassoux, G.; Remy-Jardin, M.; Copin, M.C.; Wallaert, B. [A. Calmette Hospital, Lille (France)

    2002-10-01

    Coal worker's pneumoconiosis (CWP) results from coal mine dust inhalation. The paper reports the presence of a chronic interstitial pneumonia (CIP) with honeycombing in 38 cases of coal miners, with or without CWP. The 38 patients were selected on the basis of clinical criteria which are unusual in CWP, i.e. fine inspiratory crackles and severe dyspnea. There were 37 men and one woman; mean age was 67.5 {+-} 9.1 years. Thirty-two were smokers. Duration of exposure was 26.7 {+-} 9.9 years. All the patients had clinical examination, chest radiography, computed tomography (CT), lung function, laboratory investigations, wedged fiberoptic bronchoscopy with bronchoalveolar lavage (BAL). In eight cases, lung specimens were obtained. Seventeen out of 38 had finger clubbing. 17 had radiological signs of CWP limited to the upper lobes or diffusely distributed. CT showed honeycombing (36 cases), and/or ground glass opacities (30 cases) with traction bronchiectasis (8 cases) predominant in the lower lobes. BAL analysis demonstrated an increased percentage of neutrophils (9.4% {+-} 6). Lung function showed a restrictive pattern associated with a decreased DLCO and hypoxemia. Lung specimens demonstrated in 2 cases a homogenous interstitial fibrosis of intra-alveolar septum with an accumulation of immune and inflammatory cells without temporal variation and with obvious honeycombing. The 6 other cases showed features of usual interstitial pneumonia. These cases, should alert other clinicians to a possible association between CIP with honeycombing and coal dust exposure, with or without associated CWP.

  15. Sulphur Mustard Poisoning and Its Complications in Iranian Veterans

    Directory of Open Access Journals (Sweden)

    Beeta Balali-Mood

    2009-09-01

    Full Text Available Sulphur mustard is a chemical warfare agent, which was largelyused during the World War One and in Iraq-Iran conflict. It mayalso be used as a chemical terrorism agent. Therefore, medicalprofessions should have sufficient knowledge and be preparedfor medical intervention of any such chemical attack.Sulphur mustard exerts direct toxic effects on the eyes, skin,and respiratory tract, with subsequent systemic actions on thenervous, immunologic, hematologic, digestive, and reproductivesystems. It is an alkylating agent that affects DNA synthesis andthus, delayed complications have been considered since theWorld War One. Cases of malignancies in the target organs particularlyin hematopoietic, respiratory, and digestive systemswere reported. Common delayed respiratory complications includechronic bronchitis, bronchiectasis, frequent bronchopneumonia,and pulmonary fibrosis, all of which tend to deterioratewith time. Severe dry skin, delayed keratitis, and reduction ofnatural killer cells with subsequent increased risk of infectionsand malignancies are also among the most distressing long-termconsequences of sulphur mustard intoxication. However, despiteextensive research that has been conducted on Iranian veteransduring the past decades, major gaps continue to remain in thesulphur mustard literature. Immunological and neurological dysfunctionsand the relationship between exposure to sulphur mustardand mutagenicity, carcinogenicity, and teratogenicity areimportant fields that require further studies, particularly on Iranianveterans with chronic health problems caused by sulphurmustard poisoning. There is also a paucity of information on themedical management of acute and delayed toxic effects of sulphurmustard poisoning, a subject that greatly challenges themedical professions.

  16. Antibiotic prophylaxis in primary immune deficiency disorders.

    Science.gov (United States)

    Kuruvilla, Merin; de la Morena, Maria Teresa

    2013-01-01

    Long-term prophylactic antibiotics are being widely implemented as primary or adjunctive therapy in primary immune deficiencies. This practice has transformed clinical outcomes in the setting of chronic granulomatous disease, complement deficiencies, Mendelian susceptibility to mycobacterial disease, Wiskott-Aldrich syndrome, hyper-IgE syndrome, Toll signaling defects, and prevented Pneumocystis in patients with T-cell deficiencies. Yet, controlled trials are few in the context of primary antibody deficiency syndromes, and most of this practice has been extrapolated from data in patients who are immune competent and with recurrent acute otitis media, chronic rhinosinusitis, cystic fibrosis, and bronchiectasis. The paucity of guidelines on the subject is reflected in recent surveys among practicing immunologists that highlight differences of habit regarding this treatment. Such discrepancies reinforce the lack of standard protocols on the subject. This review will provide evidence for the use of antibiotic prophylaxis in various primary immune deficiency populations, especially highlighting the role antibiotic prophylaxis in primary antibody deficiency syndromes. We also discussed the relationship of long-term antibiotic use and the prevalence of resistant pathogens. Overall, examination of available data on the use of prophylactic antibiotics in antibody deficiency syndromes merit future investigation in well-designed multicenter prospective trials because this population has few other management options.

  17. Common variable immunodeficiency diagnosed during the treatment of bronchial asthma: Unusual cause of wheezing

    Science.gov (United States)

    Akaba, Tomohiro; Kondo, Mitsuko; Toriyama, Midori; Kubo, Ayako; Hara, Kaori; Yamada, Takeshi; Yoshinaga, Kentaro; Tamaoki, Jun

    2015-01-01

    Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency in adults and children. We herein report a case of CVID, who was misdiagnosed with asthma due to wheezing episodes and relatively late onset. A 51-year-old woman had suffered from recurrent upper and lower airway infection for recent 2 years. She repeated wheezing attacks and was treated as asthma exacerbation triggered by infection. She was referred to our hospital for investigation and treatment. Lung function tests showed no reversibility of FEV1 by β-adrenergic agonist, but the increase of V50/V25. Chest CT showed slight to moderate bronchial wall thickening and bronchiectasis. After that, she suffered from pneumonia with wheezing attacks twice a month, and immunodeficiency was strongly suspected. Her blood tests showed marked decreases of all classes of immunoglobulin and nearly lack of memory B cells, NKT cells and plasmacytoid dendritic cells. She was diagnosed with CVID, and was treated with replacement of gammaglobulin. Thereafter, her wheezing episodes with infection were remarkably improved. Because the delay of diagnosis with CVID likely causes poor mortality and morbidity, a possibility of CVID should be considered in patients with frequent asthma-like symptoms due to recurrent airway infection. PMID:26744651

  18. Common variable immunodeficiency diagnosed during the treatment of bronchial asthma: Unusual cause of wheezing

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    Tomohiro Akaba

    2015-01-01

    Full Text Available Common variable immunodeficiency (CVID is the most frequent primary immunodeficiency in adults and children. We herein report a case of CVID, who was misdiagnosed with asthma due to wheezing episodes and relatively late onset. A 51-year-old woman had suffered from recurrent upper and lower airway infection for recent 2 years. She repeated wheezing attacks and was treated as asthma exacerbation triggered by infection. She was referred to our hospital for investigation and treatment. Lung function tests showed no reversibility of FEV1 by β-adrenergic agonist, but the increase of V50/V25. Chest CT showed slight to moderate bronchial wall thickening and bronchiectasis. After that, she suffered from pneumonia with wheezing attacks twice a month, and immunodeficiency was strongly suspected. Her blood tests showed marked decreases of all classes of immunoglobulin and nearly lack of memory B cells, NKT cells and plasmacytoid dendritic cells. She was diagnosed with CVID, and was treated with replacement of gammaglobulin. Thereafter, her wheezing episodes with infection were remarkably improved. Because the delay of diagnosis with CVID likely causes poor mortality and morbidity, a possibility of CVID should be considered in patients with frequent asthma-like symptoms due to recurrent airway infection.

  19. Tobramycin Inhalation Powder™: a novel drug delivery system for treating chronic Pseudomonas aeruginosa infection in cystic fibrosis.

    Science.gov (United States)

    Parkins, Michael D; Elborn, J Stuart

    2011-10-01

    Lung disease in cystic fibrosis (CF) is typified by the development of chronic airways infection culminating in bronchiectasis and progression to end-stage respiratory disease. Pseudomonas aeruginosa, a ubiquitous gram-negative bacteria, is the archetypical CF pathogen and is associated with an accelerated clinical decline. The development and widespread use of chronic suppressive aerosolized antibacterial therapies, in particular Tobramycin Inhalation Solution (TIS), in CF has contributed to reduced lung function decline and improved survival. However, the requirement for the aerosolization of these agents through nebulizers has been associated with increased treatment burden, reduced quality of life and remain a barrier to broader uptake. Tobramycin Inhalation Powder (TIP™) has been developed by Novartis with the express purpose of delivering the same benefits as TIS in a time-effective manner. Administered via the T-326™ (Novartis) Inhaler in four individual 28-mg capsules, TIP can be administered in a quarter of the time of traditional nebulizers and is inherently portable. In clinical studies, TIP has been shown to be safe, result in equivalent or superior reductions in P. aeruginosa sputum density and produce similar improvements in pulmonary function. TIP offers significant advantages in time saving, portability and convenience over traditional nebulized TIS with comparable clinical outcomes for individuals with CF.

  20. Quantitative computed tomography analysis of the airways in patients with cystic fibrosis using automated software: correlation with spirometry in the evaluation of severity*

    Science.gov (United States)

    Santos, Marcel Koenigkam; Cruvinel, Danilo Lemos; de Menezes, Marcelo Bezerra; Teixeira, Sara Reis; Vianna, Elcio de Oliveira; Elias Júnior, Jorge; Martinez, José Antonio Baddini

    2016-01-01

    Objective To perform a quantitative analysis of the airways using automated software, in computed tomography images of patients with cystic fibrosis, correlating the results with spirometric findings. Materials and Methods Thirty-four patients with cystic fibrosis were studied-20 males and 14 females; mean age 18 ± 9 years-divided into two groups according to the spirometry findings: group I (n = 21), without severe airflow obstruction (forced expiratory volume in first second [FEV1] > 50% predicted), and group II (n = 13), with severe obstruction (FEV1 ≤ 50% predicted). The following tracheobronchial tree parameters were obtained automatically: bronchial diameter, area, thickness, and wall attenuation. Results On average, 52 bronchi per patient were studied. The number of bronchi analyzed was higher in group II. The correlation with spirometry findings, especially between the relative wall thickness of third to eighth bronchial generation and predicted FEV1, was better in group I. Conclusion Quantitative analysis of the airways by computed tomography can be useful for assessing disease severity in cystic fibrosis patients. In patients with severe airflow obstruction, the number of bronchi studied by the method is higher, indicating more bronchiectasis. In patients without severe obstruction, the relative bronchial wall thickness showed a good correlation with the predicted FEV1. PMID:28100929

  1. BIIL 284 reduces neutrophils numbers but increases P. aeruginosa bacteraemia and inflammation in mouse lungs

    Science.gov (United States)

    Döring, Gerd; Bragonzi, Alessandra; Paroni, Moira; Aktürk, Firdevs-Fatma; Cigana, Cristina; Schmidt, Annika; Gilpin, Deirdre; Heyder, Susanne; Born, Torsten; Smaczny, Christina; Kohlhäufl, Martin; Wagner, Thomas O. F.; Loebinger, Michael R.; Bilton, Diana; Tunney, Michael M.; Elborn, J. Stuart; Pier, Gerald B.; Konstan, Michael W.; Ulrich, Martina

    2014-01-01

    Background A clinical study to investigate the leukotriene B4 (LTB4)-receptor antagonist BIIL 284 in cystic fibrosis (CF) patients was prematurely terminated due to a significantly increased risk of adverse pulmonary events. We aimed to establish the effect of BIIL284 in models of Pseudomonas aeruginosa lung infection, thereby contributing to a better understanding of what could have led to adverse pulmonary events in CF patients. Methods P. aeruginosa DNA in the blood of CF patients during and after acute pulmonary exacerbations and in stable patients with non-CF bronchiectasis (NCFB) and healthy individuals was assessed by PCR. The effect of BIIL 284 treatment was tested in an agar beads murine model of Pseudomonas aeruginosa lung infection. Bacterial count and inflammation were evaluated in lung and other organs. Result Most CF patients (98%) and all patients with NCFB and healthy individuals had negative P. aeruginosa DNA in their blood. Similarly, the P. aeruginosa-infected mice showed bacterial counts in the lung but not blood or spleen. BIIL 284 treatment decreased pulmonary neutrophils and increased P. aeruginosa numbers in mouse lungs leading to significantly higher bacteremia rates and lung inflammation compared to placebo treated animals. Conclusions Decreased airway neutrophils induced lung proliferation and severe bacteraemia in a murine model of P. aeruginosa lung infection. These data suggest that caution should be taken when administering anti-inflammatory compounds to patients with bacterial infections. PMID:24183915

  2. Nonspecific Interstitial Pneumonia: What Is the Optimal Approach to Management?

    Science.gov (United States)

    Tomassetti, Sara; Ryu, Jay H; Piciucchi, Sara; Chilosi, Marco; Poletti, Venerino

    2016-06-01

    We reviewed current aspects of the clinical and pathogenic profile of nonspecific interstitial pneumonia (NSIP), to better elucidate the complex issue of management and treatment options for NSIP patients. Recent findings suggest that idiopathic NSIP is a complex clinical entity with a disease spectrum that includes at least three different phenotypes: NSIP associated with autoimmune features, emphysema, and familial interstitial lung disease. This distinction, based mainly on clinical findings, may be of critical importance when it comes to making a decision on patients' management. This hypothesis warrants further studies. Currently, two major radiologic-pathologic different profiles have been well established. First, the "inflammatory type" characterized by prominent lymphocytic inflammation both on biopsy and bronchoalveolar lavage (BAL), and high-resolution computed tomography (HRCT) with mixed NSIP/organizing pneumonia pattern that tends to have a better response to corticosteroid and immunosuppressive treatment. Second, the "highly fibrotic" subgroup that shows prominent reticular changes and traction bronchiectasis by HRCT, high fibrotic background on biopsy, and no lymphocytosis on BAL. The latter fibrotic NSIP is the subgroup with less potential to respond to immunosuppressive treatment and a marginal risk to evolve into "full-blown idiopathic pulmonary fibrosis." The management of patients with fibrotic, progressive, and immunosuppressive treatment, refractory NSIP remains uncertain, and further studies are needed to address the role of antifibrotic drug in this settings. Oxygen therapy, pulmonary rehabilitation, and lung transplantation are of importance in the current management of severe, progressive, and refractory NSIP patients.

  3. Respiratory disease related mortality and morbidity on an island of Greece exposed to perlite and bentonite mining dust.

    Science.gov (United States)

    Sampatakakis, Stefanos; Linos, Athena; Papadimitriou, Eleni; Petralias, Athanasios; Dalma, Archontoula; Papasaranti, Eirini Saranti; Christoforidou, Eleni; Stoltidis, Melina

    2013-10-14

    A morbidity and mortality study took place, focused on Milos Island, where perlite and bentonite mining sites are located. Official data concerning number and cause of deaths, regarding specific respiratory diseases and the total of respiratory diseases, for both Milos Island and the Cyclades Prefecture were used. Standardized Mortality Ratios (SMRs) were computed, adjusted specifically for age, gender and calendar year. Tests of linear trend were performed. By means of a predefined questionnaire, the morbidity rates of specific respiratory diseases in Milos, were compared to those of the municipality of Oinofita, an industrial region. Chi-square analysis was used and the confounding factors of age, gender and smoking were taken into account, by estimating binary logistic regression models. The SMRs for Pneumonia and Chronic Obstructive Pulmonary Disease (COPD) were found elevated for both genders, although they did not reach statistical significance. For the total of respiratory diseases, a statistically significant SMR was identified regarding the decade 1989-1998. The morbidity study revealed elevated and statistically significant Odds Ratios (ORs), associated with allergic rhinitis, pneumonia, COPD and bronchiectasis. An elevated OR was also identified for asthma. After controlling for age, gender and smoking, the ORs were statistically significant and towards the same direction.

  4. Chronic health effects of sulphur mustard exposure with special reference to Iranian veterans

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    B Balali-Mood

    2008-01-01

    Full Text Available The widespread use of sulphur mustard (SM as an incapacitating chemical warfare agent in the past century has proved its long-lasting toxic effects. It may also be used as a chemical terrorist agent. Therefore, all health professionals should have sufficient knowledge and be prepared for any such chemical attack. SM exerts direct toxic effects on the eyes, skin, and respiratory tissue, with subsequent systemic action on the nervous, immunological, haematological, digestive, and reproductive systems. SM is an alkylating agent that affects DNA synthesis, and, thus, delayed complications have been seen since the First World War. Cases of malignancies in the target organs, particularly in haematopoietic, respiratory, and digestive systems, have been reported. Important delayed respiratory complications include chronic bronchitis, bronchiectasis, frequent bronchopneumonia, and pulmonary fibrosis, all of which tend to deteriorate with time. Severe dry skin, delayed keratitis, and reduction of natural killer cells with subsequent increased risk of infections and malignancies are also among the most distressing long-term consequences of SM intoxication. However, despite a lot of research over the past decades on Iranian veterans, there are still major gaps in the SM literature. Immunological and neurological dysfunction, as well as the relationship between SM exposure and mutagenicity, carcinogenicity, and teratogenicity are important fields that require further studies, particularly on Iranian veterans with chronic health effects of SM poisoning. There is also a paucity of information on the medical management of acute and delayed toxic effects of SM poisoning—a subject that greatly challenges health care specialists.

  5. Characterisation of COPD heterogeneity in the ECLIPSE cohort

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    Agusti Alvar

    2010-09-01

    Full Text Available Abstract Background Chronic obstructive pulmonary disease (COPD is a complex condition with pulmonary and extra-pulmonary manifestations. This study describes the heterogeneity of COPD in a large and well characterised and controlled COPD cohort (ECLIPSE. Methods We studied 2164 clinically stable COPD patients, 337 smokers with normal lung function and 245 never smokers. In these individuals, we measured clinical parameters, nutritional status, spirometry, exercise tolerance, and amount of emphysema by computed tomography. Results COPD patients were slightly older than controls and had more pack years of smoking than smokers with normal lung function. Co-morbidities were more prevalent in COPD patients than in controls, and occurred to the same extent irrespective of the GOLD stage. The severity of airflow limitation in COPD patients was poorly related to the degree of breathlessness, health status, presence of co-morbidity, exercise capacity and number of exacerbations reported in the year before the study. The distribution of these variables within each GOLD stage was wide. Even in subjects with severe airflow obstruction, a substantial proportion did not report symptoms, exacerbations or exercise limitation. The amount of emphysema increased with GOLD severity. The prevalence of bronchiectasis was low (4% but also increased with GOLD stage. Some gender differences were also identified. Conclusions The clinical manifestations of COPD are highly variable and the degree of airflow limitation does not capture the heterogeneity of the disease.

  6. Prevalence and Characteristics of Lung Involvement on High Resolution Computed Tomography in Patients with Ankylosing Spondylitis: A Systematic Review

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    Abdellah El Maghraoui

    2012-01-01

    Full Text Available To determine the prevalence of lung involvement and the spectrum of abnormalities revealed on HRCT in patients with AS, a systematic literature review was conducted in the Medline database up to May 2009 and in the abstracts of rheumatology scientific meetings (2006–2008. A hand search of references was also performed. Among the 264 selected articles, 10 articles (303 patients allowed a calculation of the prevalence of lung abnormalities on thoracic HRCT in AS. A total of 185 patients (61% had an abnormal thoracic HRCT: upper lobe fibrosis in 21 (6.9%, emphysema in 55 (18.1%, bronchiectasis in 33 (10.8%, and ground glass attenuation in 34 (11.2%. Non specific interstitial abnormalities were observed in 101 (33% patients. The most common observed abnormalities were pleural thickening (52%, parenchymal bands (45% and interlobular septal thickening (30%. Only the prevalence of upper lobe fibrosis increased significantly with disease duration (3 studies. Mild and non-specific interstitial abnormalities on thoracic HRCT are common in patients with AS, even in patients with early disease and without respiratory symptoms.

  7. Prevalence and characteristics of lung involvement on high resolution computed tomography in patients with ankylosing spondylitis: a systematic review.

    Science.gov (United States)

    El Maghraoui, Abdellah; Dehhaoui, Mohamed

    2012-01-01

    To determine the prevalence of lung involvement and the spectrum of abnormalities revealed on HRCT in patients with AS, a systematic literature review was conducted in the Medline database up to May 2009 and in the abstracts of rheumatology scientific meetings (2006-2008). A hand search of references was also performed. Among the 264 selected articles, 10 articles (303 patients) allowed a calculation of the prevalence of lung abnormalities on thoracic HRCT in AS. A total of 185 patients (61%) had an abnormal thoracic HRCT: upper lobe fibrosis in 21 (6.9%), emphysema in 55 (18.1%), bronchiectasis in 33 (10.8%), and ground glass attenuation in 34 (11.2%). Non specific interstitial abnormalities were observed in 101 (33%) patients. The most common observed abnormalities were pleural thickening (52%), parenchymal bands (45%) and interlobular septal thickening (30%). Only the prevalence of upper lobe fibrosis increased significantly with disease duration (3 studies). Mild and non-specific interstitial abnormalities on thoracic HRCT are common in patients with AS, even in patients with early disease and without respiratory symptoms.

  8. Isolated idiopathic chronic pancreatitis associated with a compound heterozygosity for two mutations of the CFTR gene.

    Science.gov (United States)

    Reboul, Marie-Pierre; Laharie, David; Amouretti, Michel; Lacombe, Didier; Iron, Albert

    2003-01-01

    We report the case of a patient suffering from idiopathic chronic pancreatitis (ICP) and compound heterozygous for mutations G542X and S1235R of the cystic fibrosis transmembrane regulator (CFTR) gene. The patient had normal sweat test and no other clinical sign usually linked with a typical or moderate pathology (bronchiectasis, nasal polyposis, congenital absence of the vas deferens) of the CFTR gene. G542X is a severe mutation, which is usually found in classical cystic fibrosis when associated with other severe mutations. S1235R is a quite rare abnormality recently reported as being potentially pathogenic when combined in trans with a second CF mutation. Our case is quite similar to the only other six patients in the literature in whom only the pancreas is affected and who bear a rare mutation with moderate effect. The history and the clinical features of our patient indicate an unambiguous isolated ICP in which the presence of the S1235R mutation--in trans with regard to G542X--is likely responsible for the ICP phenotype. This case could throw light on some of the as yet poorly known abnormalities of the CFTR gene in the ICP phenotype.

  9. Fungal allergy in asthma-state of the art and research needs.

    Science.gov (United States)

    Denning, David W; Pashley, Catherine; Hartl, Domink; Wardlaw, Andrew; Godet, Cendrine; Del Giacco, Stefano; Delhaes, Laurence; Sergejeva, Svetlana

    2014-01-01

    Sensitization to fungi and long term or uncontrolled fungal infection are associated with poor control of asthma, the likelihood of more severe disease and complications such as bronchiectasis and chronic pulmonary aspergillosis. Modelling suggests that >6.5 million people have severe asthma with fungal sensitizations (SAFS), up to 50% of adult asthmatics attending secondary care have fungal sensitization, and an estimated 4.8 million adults have allergic bronchopulmonary aspergillosis (ABPA). There is much uncertainty about which fungi and fungal allergens are relevant to asthma, the natural history of sensitisation to fungi, if there is an exposure response relationship for fungal allergy, and the pathogenesis and frequency of exacerbations and complications. Genetic associations have been described but only weakly linked to phenotypes. The evidence base for most management strategies in ABPA, SAFS and related conditions is weak. Yet straightforward clinical practice guidelines for management are required. The role of environmental monitoring and optimal means of controlling disease to prevent disability and complications are not yet clear. In this paper we set out the key evidence supporting the role of fungal exposure, sensitisation and infection in asthmatics, what is understood about pathogenesis and natural history and identify the numerous areas for research studies.

  10. Clinical heterogeneity of dominant chronic mucocutaneous candidiasis disease: presenting as treatment-resistant candidiasis and chronic lung disease.

    Science.gov (United States)

    Dotta, Laura; Scomodon, Omar; Padoan, Rita; Timpano, Silviana; Plebani, Alessandro; Soresina, Annarosa; Lougaris, Vassilios; Concolino, Daniela; Nicoletti, Angela; Giardino, Giuliana; Licari, Amelia; Marseglia, Gianluigi; Pignata, Claudio; Tamassia, Nicola; Facchetti, Fabio; Vairo, Donatella; Badolato, Raffaele

    2016-03-01

    In gain-of-function STAT1 mutations, chronic mucocutaneous candidiasis disease (CMCD) represents the phenotypic manifestation of a complex immunodeficiency characterized by clinical and immunological heterogeneity. We aimed to study clinical manifestations, long-term complications, molecular basis, and immune profile of patients with dominant CMCD. We identified nine patients with heterozygous mutations in STAT1, including novel amino acid substitutions (L283M, L351F, L400V). High risk of azole-resistance was observed, particularly when intermittent regimens of antifungal treatment or use of suboptimal dosage occurs. We report a case of Cryptococcosis and various bacterial and viral infections. Risk of developing bronchiectasis in early childhood or gradually evolving to chronic lung disease in adolescent or adult ages emerges. Lymphopenia is variable, likely progressing by adulthood. We conclude that continuous antifungal prophylaxis associated to drug monitoring might prevent resistance to treatment; prompt diagnosis and therapy of lung disease might control long-term progression; careful monitoring of lymphopenia-related infections might improve prognosis.

  11. The best of respiratory infections from the 2015 European Respiratory Society International Congress

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    Eva Polverino

    2016-07-01

    Full Text Available The breadth and quality of scientific presentations on clinical and translational research into respiratory infections at the 2015 European Respiratory Society (ERS International Congress in Amsterdam, the Netherlands, establishes this area as one of the leadings fields in pulmonology. The host–pathogen relationship in chronic obstructive pulmonary disease, and the impact of comorbidities and chronic treatment on clinical outcomes in patients with pneumonia were studied. Various communications were dedicated to bronchiectasis and, in particular, to different prognostic and clinical aspects of this disease, including chronic infection with Pseudomonas and inhaled antibiotic therapy. Recent data from the World Health Organization showed that Europe has the highest number of multidrug-resistant tuberculosis cases and the poorest countries have the least access to suitable treatments. Latent tuberculosis and different screening programmes were also discussed with particular attention to risk factors such as HIV infection and diabetes. Several biomarkers were proposed to distinguish between active tuberculosis and latent infection. Major treatment trials were discussed (REMOX, RIFQUIN and STREAM. The possibility of once-weekly treatment in the continuation phase (RIAQUIN was especially exciting. The continuing rise of Mycobacterium abscessus as a significant pathogen was noted. This article reviews some of the best contributions from the Respiratory Infections Assembly to the 2015 ERS International Congress.

  12. The best of respiratory infections from the 2015 European Respiratory Society International Congress.

    Science.gov (United States)

    Polverino, Eva; Bothamley, Graham H; Goletti, Delia; Heyckendorf, Jan; Sotgiu, Giovanni; Aliberti, Stefano

    2016-07-01

    The breadth and quality of scientific presentations on clinical and translational research into respiratory infections at the 2015 European Respiratory Society (ERS) International Congress in Amsterdam, the Netherlands, establishes this area as one of the leadings fields in pulmonology. The host-pathogen relationship in chronic obstructive pulmonary disease, and the impact of comorbidities and chronic treatment on clinical outcomes in patients with pneumonia were studied. Various communications were dedicated to bronchiectasis and, in particular, to different prognostic and clinical aspects of this disease, including chronic infection with Pseudomonas and inhaled antibiotic therapy. Recent data from the World Health Organization showed that Europe has the highest number of multidrug-resistant tuberculosis cases and the poorest countries have the least access to suitable treatments. Latent tuberculosis and different screening programmes were also discussed with particular attention to risk factors such as HIV infection and diabetes. Several biomarkers were proposed to distinguish between active tuberculosis and latent infection. Major treatment trials were discussed (REMOX, RIFQUIN and STREAM). The possibility of once-weekly treatment in the continuation phase (RIAQUIN) was especially exciting. The continuing rise of Mycobacterium abscessus as a significant pathogen was noted. This article reviews some of the best contributions from the Respiratory Infections Assembly to the 2015 ERS International Congress.

  13. COEXISTENCE YELLOW NAIL SYNDROME WITH SYSTEMIC SYMPTOMS - PRESENTATION OF CASES

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    Brzeziński Piotr

    2010-10-01

    Full Text Available Nail changes can accompany many systemic diseases and very often indicate the ongoing systemic process of illness. The yellow nail syndrome (YNS is very rare clinical entity characterized by marked thickening and yellow to yellow-green discoloration of the nails. Congenitally hypoplastic lymphostasis plays a major role in the clinical manifestation of that disease. Syndrome includes pleural effusions, lymphedema and yellow dystrophic nails. The pathogenesis stays still unknown.Aim: Presentation the coexistence of YNS with the systemic symptoms by analyzing cases of 3 patients.Material and methods: The analysis involved 3 patients with YNS (2 women and 1 man aged from 43 to 48 years.Results: We confirmed 3 cases of YNS, with the characteristic nails changes (yellow-greenish discoloration, absence of lunula, etc.. None of the patients had a family history of YNS. All suffered from chronic diseases: the first patient suffered from lymphedema and diabetes mellitus, second - from rheumatoid arthritis and the third complained of a chronic caught and sinusitis. All YNS`s symptoms occurred in the patients` forties. We observed fingers and toes involvement on 7-8 nails in each patient.Conclusions: The YNS offen associated with systemic disease, most commonly lymphedema and bronchiectasis. However, the literature describes some connections with carcinoma and autoimmune diseases. Therefore, each patient with YNS should be examined for cancer detection and stay under periodic medical control.

  14. How to adapt the pulmonary rehabilitation programme to patients with chronic respiratory disease other than COPD

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    Anne E. Holland

    2013-12-01

    Full Text Available Dyspnoea, fatigue, reduced exercise tolerance, peripheral muscle dysfunction and mood disorders are common features of many chronic respiratory disorders. Pulmonary rehabilitation successfully treats these manifestations in chronic obstructive pulmonary disease (COPD and emerging evidence suggests that these benefits could be extended to other chronic respiratory conditions, although adaptations to the standard programme format may be required. Whilst the benefits of exercise training are well established in asthma, pulmonary rehabilitation can also provide evidence-based interventions including breathing techniques and self-management training. In interstitial lung disease, a small number of trials show improved exercise capacity, symptoms and quality of life following pulmonary rehabilitation, which is a positive development for patients who may have few treatment options. In pulmonary arterial hypertension, exercise training is safe and effective if patients are stable on medical therapy and close supervision is provided. Pulmonary rehabilitation for bronchiectasis, including exercise training and airway clearance techniques, improves exercise capacity and quality of life. In nonsmall cell lung cancer, a comprehensive interdisciplinary approach is required to ensure the success of pulmonary rehabilitation following surgery. Pulmonary rehabilitation programmes provide important and underutilised opportunities to improve the integrated care of people with chronic respiratory disorders other than COPD.

  15. Immunopathology and Immunogenetics of Allergic Bronchopulmonary Aspergillosis

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    Alan P. Knutsen

    2011-01-01

    Full Text Available Allergic bronchopulmonary aspergillosis (ABPA is a Th2 hypersensitivity lung disease in response to Aspergillus fumigatus that affects asthmatic and cystic fibrosis (CF patients. Sensitization to A. fumigatus is common in both atopic asthmatic and CF patients, yet only 1%–2% of asthmatic and 7%–9% of CF patients develop ABPA. ABPA is characterized by wheezing and pulmonary infiltrates which may lead to pulmonary fibrosis and/or bronchiectasis. The inflammatory response is characterized by Th2 responses to Aspergillus allergens, increased serum IgE, and eosinophilia. A number of genetic risks have recently been identified in the development of ABPA. These include HLA-DR and HLA-DQ, IL-4 receptor alpha chain (IL-4RA polymorphisms, IL-10 −1082GA promoter polymorphisms, surfactant protein A2 (SP-A2 polymorphisms, and cystic fibrosis transmembrane conductance regulator gene (CFTR mutations. The studies indicate that ABPA patients are genetically at risk to develop skewed and heightened Th2 responses to A. fumigatus antigens. These genetic risk studies and their consequences of elevated biologic markers may aid in identifying asthmatic and CF patients who are at risk to the development of ABPA. Furthermore, these studies suggest that immune modulation with medications such as anti-IgE, anti-IL-4, and/or IL-13 monoclonal antibodies may be helpful in the treatment of ABPA.

  16. First report of c. 1499G>C mutation in a 6-month-child with cystic fibrosis

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    Abbas Sahami

    2014-01-01

    Full Text Available So far, more than 1800 mutations identified in the cystic fibrosis transmembrane conductance regulator (CFTR gene. In this case report, we presented first report of c. 1499G>C mutation in a 6-month-old girl with cystic fibrosis (CF diagnosis. A 6-month-old girl with weakness and meconium Ileus referred to the pediatric clinic in Ilam, in the west of Iran. Patient′s skin was dark and suffered from bronchiectasis. The sweat test was performed, and the concentration of chloride and sodium in patient′s sweat was 130-135 mmol/L and 125-128 mmol/L, respectively. The exon 10 mutation analysis of a CF patient was performed. CFTR mutation analysis revealed the identification of 2 mutations in patient, the mutations were p.F508del (ΔF508 and c. 1499G>C (cd500, respectively. The mutation c. 1499G>C (cd500 were found for the first time in the world. Assessing this mutation in future study and genetic investigation is recommended.

  17. Host-microbe interactions in distal airways: relevance to chronic airway diseases.

    Science.gov (United States)

    Martin, Clémence; Burgel, Pierre-Régis; Lepage, Patricia; Andréjak, Claire; de Blic, Jacques; Bourdin, Arnaud; Brouard, Jacques; Chanez, Pascal; Dalphin, Jean-Charles; Deslée, Gaetan; Deschildre, Antoine; Gosset, Philippe; Touqui, Lhousseine; Dusser, Daniel

    2015-03-01

    This article is the summary of a workshop, which took place in November 2013, on the roles of microorganisms in chronic respiratory diseases. Until recently, it was assumed that lower airways were sterile in healthy individuals. However, it has long been acknowledged that microorganisms could be identified in distal airway secretions from patients with various respiratory diseases, including cystic fibrosis (CF) and non-CF bronchiectasis, chronic obstructive pulmonary disease, asthma and other chronic airway diseases (e.g. post-transplantation bronchiolitis obliterans). These microorganisms were sometimes considered as infectious agents that triggered host immune responses and contributed to disease onset and/or progression; alternatively, microorganisms were often considered as colonisers, which were considered unlikely to play roles in disease pathophysiology. These concepts were developed at a time when the identification of microorganisms relied on culture-based methods. Importantly, the majority of microorganisms cannot be cultured using conventional methods, and the use of novel culture-independent methods that rely on the identification of microorganism genomes has revealed that healthy distal airways display a complex flora called the airway microbiota. The present article reviews some aspects of current literature on host-microbe (mostly bacteria and viruses) interactions in healthy and diseased airways, with a special focus on distal airways.

  18. Host–microbe interactions in distal airways: relevance to chronic airway diseases

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    Clémence Martin

    2015-03-01

    Full Text Available This article is the summary of a workshop, which took place in November 2013, on the roles of microorganisms in chronic respiratory diseases. Until recently, it was assumed that lower airways were sterile in healthy individuals. However, it has long been acknowledged that microorganisms could be identified in distal airway secretions from patients with various respiratory diseases, including cystic fibrosis (CF and non-CF bronchiectasis, chronic obstructive pulmonary disease, asthma and other chronic airway diseases (e.g. post-transplantation bronchiolitis obliterans. These microorganisms were sometimes considered as infectious agents that triggered host immune responses and contributed to disease onset and/or progression; alternatively, microorganisms were often considered as colonisers, which were considered unlikely to play roles in disease pathophysiology. These concepts were developed at a time when the identification of microorganisms relied on culture-based methods. Importantly, the majority of microorganisms cannot be cultured using conventional methods, and the use of novel culture-independent methods that rely on the identification of microorganism genomes has revealed that healthy distal airways display a complex flora called the airway microbiota. The present article reviews some aspects of current literature on host–microbe (mostly bacteria and viruses interactions in healthy and diseased airways, with a special focus on distal airways.

  19. August 2014 Phoenix pulmonary journal club: the use of macrolide antibiotics in chronic respiratory disease

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    Robbins RA

    2014-08-01

    Full Text Available No abstract available. Article truncated after 150 words. This month's journal club reviewed the role of macrolide antibiotics in chronic respiratory disease. Macrolide usage was suggested from observational studies in Japan in diffuse panbroncholitis, a disorder associated with chronic respiratory infection, usually Pseudomonas aeruginosa (1. Clinical improvement was noted despite doses of antibiotics well below the minimal inhibitory concentration (MIC of the antibiotic. This suggested the antibiotic was likely working by an anti-inflammatory effect. These observations were extended to cystic fibrosis (CF where prophylactic macrolide therapy in CF patients infected with Pseudomonas has become standard therapy (2. More recently, low dose macrolide therapy has been applied to non-CF lung diseases such as chronic obstructive pulmonary disease (COPD, bronchiectasis and asthma. Time did not permit a review of all studies so a representative sample was discussed. In patients with COPD, the four randomized, placebo-controlled trials reviewed all suggested that chronic therapy with macrolide antibiotics reduced COPD exacerbations (3-5. This ...

  20. Impact of multidetector CT-angiography on the emergency management of severe hemoptysis

    Energy Technology Data Exchange (ETDEWEB)

    Chalumeau-Lemoine, Ludivine [Service de Pneumologie et Réanimation, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France); Khalil, Antoine, E-mail: antoine_khalil@yahoo.fr [Service de Radiologie, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France); Pathological Angiogenesis and Vessel Normalization, Center for Interdisciplinary Research in Biology, CNRS UMR 7241/INSERM U1050, Collège de France, Paris (France); Prigent, Hélène [Service de Pneumologie et Réanimation, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France); Carette, Marie-France [Service de Radiologie, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France); Université Pierre et Marie Curie, Paris VI (France); Fartoukh, Muriel [Service de Pneumologie et Réanimation, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France); Université Pierre et Marie Curie, Paris VI (France); Parrot, Antoine [Service de Pneumologie et Réanimation, Hôpital Tenon, HUEP, APHP, 4 rue de la Chine, 75020 Paris (France)

    2013-11-01

    Background: Multidetector CT-angiography (MDCTA) is commonly used in patients with severe haemoptysis requiring admission to intensive care unit. However, the impact of MDCTA on the management of severe haemoptysis in emergency setting is poorly evaluated. Methods: We prospectively compared data provided by clinical bedside evaluation (clinical examination, chest-X-ray and fiberoptic bronchoscopy) to MDCTA data in terms of lateralization, location of the bleeding site, etiology as well as impact on the treatment choice. Results: Over a 13-month period, 87 patients (men n = 58, median age = 61 years, median haemoptysis expectorated volume = 180 mL) were included. Etiology was mainly (67%) bronchiectasis, tuberculosis sequelae and tumor. MDCTA and clinical bedside evaluation were equally effective in determining lateralization (87.4% and 93.1%, respectively, p = 0.23) and location (85% and 82.7%, respectively, p = 0.82) of the bleeding site. MDCTA was significantly more accurate than the clinical bedside strategy in determining the haemoptysis cause (86% and 70%, respectively, p = 0.007). Moreover, MDCTA suggested the involvement of systemic arteries as bleeding mechanism in 92% of cases, leading to the modification of the treatment initially considered after bedside evaluation in 21.8% of patients. Conclusion: MDCTA provides useful information for the management of patients with severe haemoptysis, especially in the treatment choice. Thus, in the absence of emergency fiberoptic bronchoscopy (FOB) requirement for airways management, MDCTA should be the first-line procedure performed in emergency clinical setting.

  1. Imagem em tuberculose pulmonar Pulmonary tuberculosis imaging

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    SIDNEY BOMBARDA

    2001-11-01

    with the active disease, such as centrilobular nodules segmentarily distributed, thick-walled cavities, thickened bronchial or bronchiolar walls, bronchiectasis and lymphadenopathy can be observed by computerized tomography. Thin-walled cavities, traction bronchiectasis, parenchymal bands, emphysema and mosaic pattern are signs suggestive of inactive disease. Gallium-67 citrate scyntigraphy is a complementary method useful in the detection of infectious diseases, including tuberculosis, especially in immunocompromised patients. Inhalation / perfusion analyses are used in the pre-operative assessment of patients carrying tuberculosis sequelaes and multiresistant tuberculosis. Positron emission tomography with fluorine-18 labeled deoxyglucose allows the detection of the inflammatory process that takes place during the active stage of tuberculosis and may persist, not so intense, after specific treatment is over. Imaging methods are valuable tools to be used in the diagnosis and follow up of pulmonary tuberculosis.

  2. Kartagener综合征一例并文献复习%Case report of Kartagener syndrome and literature review

    Institute of Scientific and Technical Information of China (English)

    邹艳; 翟雪松

    2011-01-01

    目的 探讨Kanagener综合征的临床特点、诊断标准,以提高对Kartagener综合征认识.方法 Kartagener综合征1例.患儿,女,10岁余.因反复咳嗽1年,咯血4次入院.回顾性分析该患儿临床资料,检索国内外文献,进行总结分析.结果 患儿隐匿起病,主要表现为反复咳嗽,咳脓痰,伴咯血.CT示双肺支扩伴感染,全内脏反位,鼻窦炎.经抗感染对症治疗1周后,临床症状消失出院.检索国内外文献发现,Kartagener综合征以全内脏反位,支气管扩张,副鼻窦炎三联征为诊断标准.治疗以对症治疗为主.结论 Kartagener综合征是一种罕见的常染色体隐性遗传病,加强对该病的认识,有利于提高对该病的诊断率,减少误诊率.对该病的致病基因、机制的进一步研究,可能提供更好的诊断和治疗方法.%Objective: To explore the clinical characteristics and the diagnosis standard of Kartagener Syndrome to enhance the understanding. Methods: One case with Kartagener Syndrome was reported and relevant literatures were reviewed . A 10 years old girl was admitted to our hospital with a history of cough repeatedly for one year, hemoptysis four times. Review analysis the clinical material of this girl and retrieves the domestic and foreign literature. Results: The patient went into hiding sickness, and the main manifestations included cough repeatedly, vomica, accompanied by the hemoptysis. CT images of the patients showed visceral inversion of entire internal organs, the bronchiectasis and nasosinusitis were seen in this patient. After the treatment of anti - infection for 10 days, clinical symptoms vanished . The domestic and foreign documents showed that the Kartagener syndrome take visceral inversion of entire internal organs, the bronchiectasis and nasosinusitis as the diagnosis standard. Symptomatic treatment is just right for primary treatment. Conclusions: Kartagener Syndrome is one kind of rare euchromosome recessive hereditary

  3. Aspergilosis broncopulmonar alérgica. Complicaciones poco usuales de la afección ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS. UNUSUAL COMPLICATIONS

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    EDGARDO CARRASCO C.

    2004-01-01

    being diagnosed them ABPA. Their clinical and radiological characteristics, as well as their immunological tests (skin test, Elisa IgG + for A. fumigatus gave ground for ABPA diagnosis. In both cases MAI pulmonary infection -confirmed by two positive cultures of MAI- was associated to clinical and radiological worsening. Patients were succesfully treated with claritromycin and ethambutol during 12 months; this treatment was associated to streptomycin and minocycline for the first two months. MAI infection is a rare ABPA´s complication. This association should be suspected if a high resolution CT scan shows nodules associated to diffuse bronchiectasis located beyond the central bronchiectasis (typical lesion in non-complicated ABPA. MAI pulmonary infection should be confirmed by MAI cultures in sputum or in bronchoalveolar lavage fluid

  4. 儿童闭塞性细支气管炎的临床表现与高分辨率CT特点%Clinical manifestations and high resolution CT features of bronchiolitis obliterans

    Institute of Scientific and Technical Information of China (English)

    周俊霖; 丁山; 朱晓华; 郑巍

    2012-01-01

    Objective To study the clinical manifestations and high resolution CT( HRCT) characteristics of bronchiolitis obliter-ans(BO) , so that to improve the understanding of it. Methods Clinical and CT data in 27 patients(22 boys and 5 girls, aged from 2 months to 5 years, and mean age 15. 4 months) with clinical confirmation of BO were retrospectively analysed. All the patients pres-ented with persistent cough and asthma. All cases underwent chest radiograph, HRCT examinations and blood gas analysis. 10 ca-ses and 5 cases underwent bronchoscopy and pulmonary function test, respectively. Results In 27 cases,24 cases were post-infec-tions BO and the others were premature infants after injury of lungs. Hypoxemia in 14 cases and respiratory failure in 4 cases were showed by blood gas analysis. Ten cases who underwent bronchoscopy showed chronic endobronchial infection. HRCT features in-cluded direct signs (peripheral bronchiectasis and bronchial wall thickening in 16 cases, centrilobular nodules in 5 cases) and indirect signs(bronchiectasis and bronchial wall thickening in 20 cases, air trapping signs in 5 cases, mosaic perfusion signs in 25 cases, con-solidation in 10 cases and atelectasis in 5 cases). Conclusion In most of the patients with BO present following infections. HRCT is of important value in the early diagnosis and evaluation of outcome of BO.%目的 探讨闭塞性支气管炎(BO)的临床表现与高分辨率CT(HRCT)特点,提高对本病的认识.方法 回顾性分析27例经临床诊断证实的儿童闭塞性细支气管炎的临床和CT资料,年龄2个月~5岁,平均15.4月,男22例,女5例.反复咳喘病程均在6周以上.27例患儿均行X线胸片,肺部高分辨率CT及血气分析.10例行电子纤维支气管镜检查,5例行肺功能检查.结果 27例确诊为BO患儿,3例为早产儿肺损伤后,余24例为感染后.血气分析示低氧血症14例,呼吸衰竭4例.10例行电子纤维支气管镜检查均提示有支气管内膜慢性感

  5. Non-tuberculous mycobacterial lung disease: diagnosis based on computed tomography of the chest

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    Kwak, Nakwon; Han, Sung Koo; Yim, Jae-Joon [Seoul National University College of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, 101 Daehak-Ro, Jongno-Gu, Seoul (Korea, Republic of); Lee, Chang Hyun; Lee, Hyun-Ju [Seoul National University College of Medicine, Department of Radiology, and Institute of Radiation Medicine, Seoul (Korea, Republic of); Kang, Young Ae [Yonsei University College of Medicine, Division of Pulmonology, Department of Internal Medicine, Severance Hospital, Institute of Chest Diseases, Seoul (Korea, Republic of); Lee, Jae Ho [Seoul National University Bundang Hospital, Department of Internal Medicine, Seongnam, Gyeonggi-do (Korea, Republic of)

    2016-12-15

    To elucidate the accuracy and inter-observer agreement of non-tuberculous mycobacterial lung disease (NTM-LD) diagnosis based on chest CT findings. Two chest radiologists and two pulmonologists interpreted chest CTs of 66 patients with NTM-LD, 33 with pulmonary tuberculosis and 33 with non-cystic fibrosis bronchiectasis. These observers selected one of these diagnoses for each case without knowing any clinical information except age and sex. Sensitivity and specificity were calculated according to degree of observer confidence. Inter-observer agreement was assessed using Fleiss' κ values. Multiple logistic regression was performed to elucidate which radiological features led to the correct diagnosis. The sensitivity of NTM-LD diagnosis was 56.4 % (95 % CI 47.9-64.7) and specificity 80.3 % (73.1-86.0). The specificity of NTM-LD diagnosis increased with confidence: 44.4 % (20.5-71.3) for possible, 77.4 % (67.4-85.0) for probable, 95.2 % (87.2-98.2) for definite (P < 0.001) diagnoses. Inter-observer agreement for NTM-LD diagnosis was moderate (κ = 0.453). Tree-in-bud pattern (adjusted odds ratio [aOR] 6.24, P < 0.001), consolidation (aOR 1.92, P = 0.036) and atelectasis (aOR 3.73, P < 0.001) were associated with correct NTM-LD diagnoses, whereas presence of pleural effusion (aOR 0.05, P < 0.001) led to false diagnoses. NTM-LD diagnosis based on chest CT findings is specific but not sensitive. (orig.)

  6. O muco traqueobrônquico humano mantido em temperatura ambiente e suas propriedades físico-químicas Physicochemical properties of human tracheobronchial sputum maintained at room temperature

    Directory of Open Access Journals (Sweden)

    Renata Claudia Zanchet

    2007-02-01

    Full Text Available OBJETIVO: Verificar a influência da permanência em temperatura ambiente na análise da transportabilidade por ação ciliar e por tosse e do ângulo de contato do muco traqueobrônquico. MÉTODOS: Foi coletado muco hialino de 30 indivíduos sem doença pulmonar, e purulento de vinte pacientes com bronquiectasia. As amostras foram analisadas logo após a coleta e novamente após 24 h. RESULTADOS: Para o muco purulento, após 24 h em temperatura ambiente, houve aumento no deslocamento por tosse (96 ± 50 vs. 118 ± 61 mm e diminuição do ângulo de contato (32 ± 6 vs. 27 ± 6 graus (p OBJECTIVE: To evaluate the effect that maintaining tracheobronchial sputum at room temperature has on the analysis of ciliary transport and cough, as well as on the contact angle. METHODS: Hyaline sputum was collected from 30 individuals without pulmonary diseases, and purulent sputum was collected from patients with bronchiectasis. The samples were analyzed immediately after collection and again after 24 h. RESULTS: After 24 h at room temperature, the purulent sputum presented an increase in cough-induced dislodgment (96 ± 50 vs. 118 ± 61 mm and a decrease in the contact angle (32 ± 6 vs. 27 ± 6 degrees (p < 0.05. For the hyaline sputum, there were no alterations in the parameters analyzed. CONCLUSION: Hyaline tracheobronchial sputum can be stored in room temperature for 24 h without presenting alterations in ciliary transport or contact angle. However, purulent sputum should not be stored at room temperature for many hours, since ciliary transport and contact angle might be altered as a result.

  7. Computed tomography of pulmonary changes in rheumatoid arthritis: carcinoembryonic antigen (CEA) as a marker of airway disease.

    Science.gov (United States)

    Koch, Milene Caroline; Pereira, Ivânio Alves; Nobre, Luiz Felipe Souza; Neves, Fabricio Souza

    2016-04-01

    Rheumatoid arthritis (RA) classically affects the joints, but can present extra-articular manifestations, including pulmonary disease. The present study aimed to identify possible risk factors or laboratory markers for lung involvement in RA, particularly the presence of rheumatoid factor (RF), anti-citrullinated peptide antibodies (ACPA), and tumor markers, by correlating them with changes observed on chest high-resolution computerized tomography (HRCT). This cross-sectional study involved RA patients who were examined and questioned by a specialist physician and later subjected to chest HRCT and blood collection for measurement of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), rheumatoid factor (RF), ACPA (anti-vimentin and/or anti-CCP3), and the tumor markers carcinoembryonic antigen (CEA), CA 125, CA 15-3, and CA 19-9. A total of 96 patients underwent chest HRCT. The most frequent findings were bronchial thickening (27/28.1 %) and bronchiectasis (25/26 %). RF was present in 63.2 % of patients (55/87), and ACPA (anti-vimentin or anti-CCP3) was present in 72.7 % of patients (64/88). CEA levels were high in 14 non-smokers (37.8 %) and 23 smokers (62.2 %). CA-19-9 levels were high in 6 of 86 patients (7.0 %), CA 15-3 levels were high in 3 of 85 patients (3.5 %), and CA 125 levels were high in 4 of 75 patients (5.3 %). Multivariate analysis indicated a statistically significant association between high CEA levels and the presence of airway changes in patients with RA (p = 0.048). CEA can serve as a predictor of lung disease in RA and can help identify individuals who require more detailed examination for the presence of respiratory disorders.

  8. The subclinical involvement of the lung in rheumatoid arthritis: evaluation by high-resolution computed tomography

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    E. Bichi Secchi

    2011-09-01

    Full Text Available Pulmonary involvement is one of the most frequent extra-articular manifestations of rheumatoid arthritis (RA and represents a serious complication, being the second cause of death after infection. High-resolution computed tomography (HRCT, owing to its increased sensitivity and diagnostic accuracy respect to the conventional chest radiograph (CXR, allows to detect pulmonary abnormalities in RA patients more frequently than CXR. The aim of this study was to assess pulmonary involvement by HRCT in lifelong non-smoking RA patients without symptoms and clinical signs of pulmonary disease. Seventy-two patients (54 women and 18 men with a mean age of 56.8±10.4 years (range, 40- 77 years and mean duration of disease of 6.9±4.7 years (range, 2-12 years entered the study. 52/72 (72% were positive for rheumatoid factor (> 20 UI/ml. Standard CXR and HRCT were carried out in each patient. CXR showed a mild interstitial fibrosis in 7 patients (9.7%, whereas HRCT demonstrated pulmonary abnormalities in an higher number of them (22/72 = 30.5%. The most frequent abnormal findings on HRCT were irregular pleural margins (13.8% and septal/subpleural lines (18%, both compatible with pulmonary fibrosis. Ground-glass opacities were found in 8.3% of the patients. Pulmonary nodules (diameter, range 0,5-2 cm predominantly located in the subpleural portions of the lung, were demonstrated in the same percentage (8.3% of patients. Small airway involvement, represented by bronchiectasis/bronchioloectasis, was shown in 15.2% of patients. Subpleural cysts were present in two cases (2.8%. No patient had evidence of honeycombing on HRCT. In conclusion, HRCT is an accurate, non-invasive and safe method of diagnosing lung abnormalities in RA patients without signs and clinical symptoms of pulmonary disease...

  9. Pulmonary function test: its correlation with pulmonary high-resolution computed tomography in patients with rheumatoid arthritis.

    Science.gov (United States)

    Leonel, Daza; Lucia, Cervantes; A, Muñiz; Martha-Alicia, Hernández; Blanca, Murillo

    2012-07-01

    Our objective was to try to evaluate lung affection and to correlate an easier and cheaper method with the high-resolution computed tomography (HRCT) findings in patients with RA. Thirty-six RA patients were selected for HRCT lung scan (twelve patients with altered pulmonary function test (PFT) and 24 with normal PFT). The American Thoracic Society criteria were followed for the pulmonary test. Clinical and laboratory variables were recorded. A statistical analysis was done by Kaplan-Meyer survival curve and ROC curve. When HRCT was evaluated in all patients, only sixteen had an HRCT normal and twenty patients showed some radiologic alteration under HRCT such as: pleural thickness, bronchiectasis, interstitial pattern, micro-nodules pattern, ground-glass opacity, and a reticular pattern. A logistic regression showed that methotrexate use, evolution of the disease (beta 0.018), and FEV1 (beta 0.89) were statistically associated with HRCT alterations. A projection of patients, free from event (HRCT lung scan altered), was obtained through a Kaplan-Meyer analysis, using FEV1 as a predictor over time. The curve shows that in the next 240 months (20 years) nearly 40% of the patients with rheumatoid arthritis will have FEV1 values less than 80% of the normal values predicted for the same age and sex. The FEV1 values have demonstrated a good correlation between PFT and HRCT lung scan. Therefore, they provide an accessible tool for tracking early pulmonary alterations. Methotrexate use and time evolution of the disease have been associated with altered FEV1.

  10. MICROBIOLOGICAL CHARACTERISATION OF Haemophilus influenzae STRAINS ISOLATED FROM PATIENTS WITH INVASIVE AND RESPIRATORY DISEASES

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    Tomislav Kostyanev

    2010-01-01

    Full Text Available A total of 175 H. influenzae strains were collected between 1994 and 2009 from all aged patient groups. The strains were isolated from patients with invasive and community-acquired respiratory tract infections. All strains were identified according to standard microbiological methods. Serotyping was done by a coagglutination test and by molecular PCR capsular genotyping. Beta-lactamase production was determined by the chromogenic cephalosporin test with nitrocephin as substrate. Most of the isolated H. influenzae strains were from children under 5 years of age (57.7%. Overall, 61 strains belonged to serotype b (34.9% by the means of PCR capsular typing, 1 strain was type f, and 113 isolates (64.6% were non-typeable (non-encapsulated H. influenzae. Among the infants and children with meningitis or other invasive infections, aged 2 month to 5 years, all strains, except one, were serotype b. In respiratory tract infections (pneumonia, otitis media, sinusitis and people with chronic pulmonary diseases - exacerbations of COPD, bronchiectasis, cystic fibrosis the most common - 96.5% were non-typeable strains in both groups children and adults. Overall, the prevalence of beta-lactamase production was 19.4%. But, it was much higher for invasive strains from CSF isolates - 37.7%, 25% in blood samples, and 37.5% in otitis media causative strains. Beta-lactamase production was less frequent in respiratory tract isolates - in sputum 13.3% and in URT samples - 2.3%. The rate of beta-lactamase production in CSF isolates has not changed for the last 10 years.PCR capsular genotyping method has to be performed for all non-b-type strains. The implementation of Hib vaccine in our country will be accompanied by a reduction in invasive diseases caused by H. influenzae type b in children, but it is not useful in preventing infections caused by non-typeable H. influenzae strains.

  11. Correlation between pretreatment or follow-up CT findings and therapeutic effect of autologous peripheral blood stem cell transplantation for interstitial pneumonia associated with systemic sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Yabuuchi, Hidetake, E-mail: yabuuchi@shs.kyushu-u.ac.jp [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Matsuo, Yoshio [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Tsukamoto, Hiroshi [Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Sunami, Shunya; Kamitani, Takeshi [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Sakai, Shuji [Department of Health Sciences, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Hatakenaka, Masamitsu [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Nagafuji, Koji; Horiuchi, Takahiko; Harada, Mine; Akashi, Koichi [Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan); Honda, Hiroshi [Department of Clinical Radiology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582 (Japan)

    2011-08-15

    Purpose: To evaluate what is useful among various parameters including CT findings, laboratory parameters (%VC, %DLco, KL-6), patients related data (age, sex, duration of disease) to discriminate between responder and non-responder in patients who received autologous peripheral blood stem cell transplantation (auto-PBSCT) for interstitial pneumonia (IP) with systemic sclerosis (SSc). Method: Auto-PBSCT and follow-up of at least one year by chest CT, serum KL-6, %VC, and %DLco were performed in 15 patients for IP with SSc. Analyzed CT findings included extent of ground-glass opacity (GGO), intralobular reticular opacity, number of segments that showed traction bronchiectasis, and presence of honeycombing. We regarded the therapeutic response of patients as responders when TLC or VC increase over 10% or DLco increase more than 15%, otherwise we have classified as non-responder. We applied univariate and multivariate analyses to find the significant indicators to discriminate responders from non-responders. P < 0.05 was considered statistically significant. Results: Univariate and multivariate analyses showed that the significant parameter to discriminate responders from non-responders were pretreatment KL-6, presence of honeycombing, extent of GGO, and early change in extent of GGO. Among them, extent of GGO and early change in extent of GGO were the strongest discriminators between responders and non-responders (P = 0.001, 0.001, respectively). Conclusion: Several CT findings and pretreatment KL-6 may be useful to discriminate between responder and non-responder in patients who received auto-PBSCT for IP with SSc.

  12. Lung morphology assessment of cystic fibrosis using MRI with ultra-short echo time at submillimeter spatial resolution

    Energy Technology Data Exchange (ETDEWEB)

    Dournes, Gael [University of Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); CHU de Bordeaux, Service d' Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d' Exploration Fonctionnelle Respiratoire, Pessac (France); Centre de Recherche Cardio-thoracique de Bordeaux, INSERM, U1045, Universite de Bordeaux, Bordeaux (France); Menut, Fanny [CHU de Bordeaux, Service d' Imagerie de la Femme et de l' Enfant, Unite de Pneumologie pediatrique, Bordeaux (France); Macey, Julie; Montaudon, Michel; Berger, Patrick; Laurent, Francois [University of Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); CHU de Bordeaux, Service d' Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d' Exploration Fonctionnelle Respiratoire, Pessac (France); Fayon, Michael [University of Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); INSERM, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); CHU de Bordeaux, Service d' Imagerie de la Femme et de l' Enfant, Unite de Pneumologie pediatrique, Bordeaux (France); Chateil, Jean-Francois [CHU de Bordeaux, Service d' Imagerie de la Femme et de l' Enfant, Unite de Pneumologie pediatrique, Bordeaux (France); University of Bordeaux, Centre de Resonance Magnetique des Systemes Biologiques, Bordeaux (France); Salel, Marjorie; Corneloup, Olivier [University of Bordeaux, Centre de Recherche Cardio-Thoracique de Bordeaux, Bordeaux (France); CHU de Bordeaux, Service d' Imagerie Thoracique et Cardiovasculaire, Service des Maladies Respiratoires, Service d' Exploration Fonctionnelle Respiratoire, Pessac (France)

    2016-11-15

    We hypothesized that non-contrast-enhanced PETRA (pointwise encoding time reduction with radial acquisition) MR (magnetic resonance) sequencing could be an alternative to unenhanced computed tomography (CT) in assessing cystic fibrosis (CF) lung structural alterations, as well as compared agreements and concordances with those of conventional T1-weighted and T2-weighted sequences. Thirty consecutive CF patients completed both CT and MRI the same day. No contrast injection was used. Agreement in identifying structural alterations was evaluated at the segmental level using a kappa test. Intraclass correlation coefficients (ICC) and Bland-Altman analysis were used to assess concordances and reproducibility in Helbich-Bhalla disease severity scoring. Agreement between PETRA and CT was higher than that of T1- or T2-weighted sequences, notably in assessing the segmental presence of bronchiectasis (Kappa = 0.83; 0.51; 0.49, respectively). The concordance in Helbich-Bhalla scores was very good using PETRA (ICC = 0.97), independently from its magnitude (mean difference (MD) = -0.3 [-2.8; 2.2]), whereas scoring was underestimated using both conventional T1 and T2 sequences (MD = -3.6 [-7.4; 0.1]) and MD = -4.6 [-8.2; -1.0], respectively. Intra- and interobserver reproducibility were very good for all imaging modalities (ICC = 0.86-0.98). PETRA showed higher agreement in describing CF lung morphological changes than that of conventional sequences, whereas the Helbich-Bhalla scoring matched closely with that of CT. (orig.)

  13. Long-term effects of mustard gas on respiratory system of Iranian veterans after Iraq-Iran war: a review

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    Razavi Seyed Mansour

    2013-06-01

    Full Text Available 【Abstract】To review long-term respiratory effects of mustard gas on Iranian veterans having undergone Iraq-Iran war. Electronic databases of Scopus, Medline, ISI, IranMedex, and Irandoc sites were searched. We accepted articles published in scientific journals as a quality criterion. The main pathogenic factors are free radical mediators. Preva-lence of pulmonary involvement is approximately 42.5%. The most common complaints are cough and dyspnea. Major respiratory complications are chronic obstructive pulmo-nary disease, bronchiectasis, and asthma. Spirometry re-sults can reveal restrictive and obstructive pulmonary disease. Plain chest X-ray does not help in about 50% of lung diseases. High-resolution CT of the lung is the best modality for diagnostic assessment of parenchymal lung and bronchi. There is no definite curative treatment for mus-tard lung. The effective treatment regimens consist of oxy-gen administration, use of vaporized moist air, respiratory physiotherapy, administration of mucolytic agents, bronchodilators, corticosteroids, and long-acting beta-2 agonists, antioxidants, surfactant, magnesium ions, thera-peutic bronchoscopy, laser therapy, placement of respira-tory stents, early tracheostomy in laryngospasm, and ulti-mately lung transplantation. High-resolution CT of the lung is the most accurate modality for the evaluation of the lung parenchyma and bronchi. The treatment efficacy of patients exposed to mustard gas depends on patient conditions (acute or chronic, upper or lower respiratory tract involvement. There are various treatment protocols, but unfortunately none of them is definitely curable. Key words: Lung injury; Chemical warfare; Mustard gas

  14. Incidence and clinical outcome of renal amyloidosis: A retrospective study

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    Emad Abdallah

    2013-01-01

    Full Text Available The kidneys are affected in almost all patients with amyloid A in secondary amyloidosis (AA amyloidosis but less frequently in immunoglobulin light chains in primary systemic amyloidosis (AL amyloidosis. In this study, we present the incidence, etiology, clinical manifestations, biochemical features and clinical course of renal amyloidosis. We conducted a retrospective study on a group of 40 cases with renal biopsy-proven amyloidosis. They constituted 2.5% of the total cases of renal biopsies performed in the Theodor Bilharz Research Institute, Cairo, Egypt, during the period from February 2003 to May 2009. The mean age (30 males, ten females was 36.51 ± 10.32 years. Thirty-two of the cases had secondary AA amyloidosis and eight cases had primary AL amyloidosis. The causes of secondary amyloidosis were as follows: 12 (30% familial Mediterranean fever (FMF, eight (20% pulmonary tuberculosis, four (10% chronic osteomyelitis, four (10% bronchiectasis, three (7% rheumatoid arthritis and one (2% rheumatic heart disease. The eight cases of primary AL amyloidosis comprised of five cases that were associated with myloma (13% and three (8% cases that were idiopathic. Among the 23 patients with AA amyloidosis, after six months of treatment with colchicine, the proteinuria improved, serum albumin level increased and edema disappeared in 13 patients. In four cases of AA amyloidosis who were clinically and biochemically normal after cholchicine therapy, a second renal biopsy disclosed decreased amyloid deposition compared with the first biopsy. In the three renal transplanted patients who had amyloidosis secondary to FMF and were treated with colchicines, AA amyloidosis did not recur in the transplanted kidney. It might be possible that in AL amyloidosis, treatment with methotrexate, melphalan and prednisolone may improve survival. The incidence of renal amyloidosis is increasing and colchicine can be used in secondary amyloidosis as it may have an effect

  15. Long-term effects of mustard gas on respiratory system of Iranian veterans after Iraq-Iran war: a review

    Institute of Scientific and Technical Information of China (English)

    Seyed Mansour Razavi; Mostafa Ghanei; Payman Salamati; Mehdi Safiabadi

    2013-01-01

    To review long-term respiratory effects of mustard gas on Iranian veterans having undergone IraqIran war.Electronic databases of Scopus,Medline,ISI,IranMedex,and Irandoc sites.were searched.We accepted articles published in scientific journals as a quality criterion.The main pathogenic factors are free radical mediators.Prevalence of pulmonary involvement is approximately 42.5%.The most common complaints are cough and dyspnea.Major respiratory complications are chronic obstructive pulmonary disease,bronchiectasis,and asthma.Spirometry resuits can reveal restrictive and obstructive pulmonary disease.Plain chest X-ray does not help in about 50% of lung diseases.High-resolution CT of the lung is the best modality for diagnostic assessment of parenchymal lung and bronchi.There is no definite curative treatment for mustard lung.The effective treatment regimens consist of oxygen administration,use of vaporized moist air,respiratory physiotherapy,administration of mucolytic agents,bronchodilators,corticosteroids,and long-acting beta-2 agonists,antioxidants,surfactant,magnesium ions,therapeutic bronchoscopy,laser therapy,placement of respiratory stents,early tracheostomy in laryngospasm,and ultimately lung transplantation.High-resolution CT of the lung is the most accurate modality for the evaluation of the lung parenchyma and bronchi.The treatment efficacy of patients exposed to mustard gas depends on patient conditions (acute or chronic,upper or lower respiratory tract involvement).There are various treatment protocols,but unfortunately none of them is defmitely curable.

  16. Evaluation of chronic arsenic poisoning due to consumption of contaminated ground water in West Bengal, India

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    Asutosh Ghosh

    2013-01-01

    Full Text Available Background: Chronic arsenic poisoning is an important public health problem and most notable in West Bengal and Bangladesh. In this study different systemic manifestations in chronic arsenic poisoning were evaluated. Methods: A nonrandomized, controlled, cross-sectional, observational study was carried out in Arsenic Clinic, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, over a period of 1 year 4 months. Seventy-three cases diagnosed clinically, consuming water containing arsenic ≥50 μg/L and having hair and nail arsenic level >0.6 μg/L, were included. Special investigations included routine parameters and organ-specific tests. Arsenic levels in the drinking water, hair, and nail were measured in all. Twenty-five nonsmoker healthy controls were evaluated. Results: Murshidabad and districts adjacent to Kolkata, West Bengal, were mostly affected. Middle-aged males were the common sufferers. Skin involvement was the commonest manifestation (100%, followed by hepatomegaly [23 (31.5%] with or without transaminitis [7 (9.58%]/portal hypertension [9 (12.33%]. Restrictive abnormality in spirometry [11 (15.06%], bronchiectasis [4 (5.47%], interstitial fibrosis [2 (2.73%], bronchogenic carcinoma [2 (2.73%], oromucosal plaque [7 (9.58%], nail hypertrophy [10 (13.69%], alopecia [8 (10.95%], neuropathy [5 (6.84%], and Electrocardiography abnormalities [5 (6.84%] were also observed. Conclusions: Mucocutaneous and nail lesions, hepatomegaly, and restrictive change in spirometry were the common and significant findings. Other manifestations were characteristic but insignificant.

  17. Ciliary genes are down-regulated in bronchial tissue of primary ciliary dyskinesia patients.

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    Maciej Geremek

    Full Text Available Primary ciliary dyskinesia (PCD is a rare, genetically heterogeneous disease characterized by recurrent respiratory tract infections, sinusitis, bronchiectasis and male infertility. The pulmonary phenotype in PCD is caused by the impaired motility of cilia in the respiratory epithelium, due to ultrastructural defects of these organelles. We hypothesized that defects of multi-protein ciliary complexes should be reflected by gene expression changes in the respiratory epithelium. We have previously found that large group of genes functionally related to cilia share highly correlated expression pattern in PCD bronchial tissue. Here we performed an explorative analysis of differential gene expression in the bronchial tissue from six PCD patients and nine non-PCD controls, using Illumina HumanRef-12 Whole Genome BeadChips. We observed 1323 genes with at least 2-fold difference in the mean expression level between the two groups (t-test p-value <0.05. Annotation analysis showed that the genes down-regulated in PCD biopsies (602 were significantly enriched for terms related to cilia, whereas the up-regulated genes (721 were significantly enriched for terms related to cell cycle and mitosis. We assembled a list of human genes predicted to encode ciliary proteins, components of outer dynein arms, inner dynein arms, radial spokes, and intraflagellar transport proteins. A significant down-regulation of the expression of genes from all the four groups was observed in PCD, compared to non-PCD biopsies. Our data suggest that a coordinated down-regulation of the ciliome genes plays an important role in the molecular pathomechanism of PCD.

  18. Occurrence of allergic bronchopulmonary mycosis in patients with asthma: An Eastern India experience

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    Sarkar Anirban

    2010-01-01

    Full Text Available Background: Allergic bronchopulmonary mycosis (ABPM is a clinical syndrome associated with immune sensitivity to various fungi notably Aspergillus spp. that colonize the airways of asthmatics. Early diagnosis and treatment with systemic corticosteroids is the key in preventing the progression of the disease to irreversible lung fibrosis. Aims: To study the occurrence of ABPM among asthma patients with fungal sensitization attending a chest clinic of a tertiary hospital of eastern India. The clinico-radiological and aetiological profiles are also described. Materials and Methods: All consecutive patients with asthma presenting to the chest clinic over a period of one year were screened for cutaneous hypersensitivity to 12 common fungal antigens. The skin test positive cases were further evaluated for ABPM using standard criteria. Results: One hundred and twenty-six asthma patients were screened using twelve common fungal antigens; forty patients (31.74% were found to be skin test positive, and ABPM was diagnosed in ten patients (7.93%. Of the 10 cases of ABPM, nine cases were those of allergic bronchopulmonary aspergillosis (ABPA and one case was identified as caused by sensitization to Penicillium spp. A majority of the cases of ABPM had advanced disease and had significantly lower FEV1 compared to non-ABPM skin test positive asthmatics. Central bronchiectasis on high resolution CT scan was the most sensitive and specific among the diagnostic parameters. Conclusion: There is a significant prevalence of ABPM in asthma patients attending our hospital and this reinforces the need to screen asthma patients for fungal sensitisation. This will help in early diagnosis and prevention of irreversible lung damage.

  19. 呼出气冷凝液中pH测定的影响因素及pH检测的临床意义%Influential factors and clinical significance of pH in exhaled breath condensate

    Institute of Scientific and Technical Information of China (English)

    余苏云; 刘翱

    2009-01-01

    呼出气冷凝液检测技术为一项新型的呼吸道炎症状态检测方法.近年来国外对其研究进展很快,临床应用得到了很大发展,而其中以pH值检测技术应用最为广泛,目前广泛应用于哮喘、慢性阻塞性肺疾病、睡眠呼吸暂停综合征、肺癌、肺间质纤维化、结核、支气管扩张、囊性纤维化等多种呼吸系统疾病.本文把握国外相关研究的最新进展,重点综述了呼出气冷凝液标本收集、贮存、检测等环节对pH值的影响凶素及其在常见呼吸系统疾病中的临床应用.%The technique of exhaled breath condensate is a new detective means for respiratory tract inflammation.It is more and more extensively applied in clinic because of the quick study abroad recently.However,the detectivc technique of pH is the most extensive one in exhaled breath condensate,and it is widely applied for asthma,chronic obstructive pulmonary disease,sleep apnea syndrome,lung cancer,pulmonary interstitial fibrosis,tuberculosis,bronchiectasis,cystic fibrosis and others.This article grasps the latest progress of relative study,reviews the influential factors of pH in exhaled breath condensate,including specimen collection,storage and detection,and the clinical application in the common diseases of respiratory system.

  20. Hyperpolarized {sup 3}helium magnetic resonance ventilation imaging of the lung in cystic fibrosis: comparison with high resolution CT and spirometry

    Energy Technology Data Exchange (ETDEWEB)

    McMahon, Colm J.; Dodd, Jonathan D.; Skehan, Stephen J.; Masterson, James B. [St. Vincent' s University Hospital, Department of Radiology, Dublin (Ireland); Hill, Catherine; Woodhouse, Neil; Wild, Jim M.; Fichele, Stan [Royal Hallamshire Hospital, The Unit of Academic Radiology, University of Sheffield, Sheffield (United Kingdom); Gallagher, Charles G. [St. Vincent' s University Hospital, Department of National Referral Centre for Adult Cystic Fibrosis, Dublin (Ireland); Beek, Edwin J.R. van [Royal Hallamshire Hospital, The Unit of Academic Radiology, University of Sheffield, Sheffield (United Kingdom); University of Iowa, Department of Radiology, Carver College of Medicine, Iowa City, IA (United States)

    2006-11-15

    The purpose of this study was to compare hyperpolarized {sup 3}helium magnetic resonance imaging ({sup 3}He MRI) of the lungs in adults with cystic fibrosis (CF) with high-resolution computed tomography (HRCT) and spirometry. Eight patients with stable CF prospectively underwent {sup 3}He MRI, HRCT, and spirometry within 1 week. Three-dimensional (3D) gradient-echo sequence was used during an 18-s breath-hold following inhalation of hyperpolarized {sup 3}He. Each lung was divided into six zones; {sup 3}He MRI was scored as percentage ventilation per lung zone. HRCT was scored using a modified Bhalla scoring system. Univariate (Spearman rank) and multivariate correlations were performed between {sup 3}He MRI, HRCT, and spirometry. Results are expressed as mean{+-}SD (range). Spirometry is expressed as percent predicted. There were four men and four women, mean age=31.9{+-}9 (20-46). Mean forced expiratory volume in 1 s (FEV){sub 1}=52%{+-}29 (27-93). Mean {sup 3}He MRI score=74%{+-}25 (55-100). Mean HRCT score=48.8{+-}24 (13.5-83). The correlation between {sup 3}He MRI and HRCT was strong (R={+-}0.89, p<0.001). Bronchiectasis was the only independent predictor of {sup 3}He MRI; {sup 3}He MRI correlated better with FEV{sub 1} and forced vital capacity (FVC) (R=0.86 and 0.93, p<0.01, respectively) than HRCT (R={+-}0.72 and {+-}0.81, p<0.05, respectively). This study showed that {sup 3}He MRI correlates strongly with structural HRCT abnormalities and is a stronger correlate of spirometry than HRCT in CF. (orig.)

  1. Mathematical model of mean age, mean arsenic dietary dose and age-specific prevalence rate from endemic chronic arsenic poisoning: a human toxicology study

    Energy Technology Data Exchange (ETDEWEB)

    Zald' ivar, R.; Ghai, G.L.

    1980-01-01

    The aim of this investigation was to develop a mathematical model of mean age, mean arsenic dietary dose, and age-specific prevalence rate for endemic chronic arsenic poisoning. Data on mean age (years), mean arsenic dietary dose (mg/kg body weight/day), and age-specific prevalence rate per 100,000 population for endemic chronic arsenic poisoning in Antofagasta Commune, northern Chile, for the 1968 to 1971 period, were collected. Endemic chronic arsenic poisoning means here chronic arsenical dermatosis associated with marked or sever symptoms (or signs) of chronic arsenic poisoning (chronic diarrhea, hepatic cirrohsis, chronic bronchitis, bronchiectasis, recurrent broncho-pneumonia, cardiomegaly, systemic occlusive arterial disease, cerebral thrombosis, etc.). There was a strong positive correlation between age-specific pevalence rate per 100,000 population and mean arsenic dose (r = + 0.9593) and a negative correlation between prevalence rate and mean age (r = 0.8789). These findings show that the prevalence rate declines with the advancing age and increases with the increase of arsenic dose. A multiple linear regression model E(y) = alpha + beta X1 + gamma X2, where y represents the age-specific prevalence rate per 100,000 population, X1 the mean arsenic dose, and X2 the mean age, was fitted to the data. The estimates of the parameters (alpha, beta, and gamma) were obtained by minimizing the residual sum of squares sigma(y - alpha - beta X1 - gamma X2)2. The following multiple linear regression equation was obtained: Y = 202.161 + 8452.455 X1 - 2.394 X2. Of the total variability in the prevalence rate, 96.22 percent was accounted for by the multiple regression.

  2. Respiratory risks from household air pollution in low and middle income countries.

    Science.gov (United States)

    Gordon, Stephen B; Bruce, Nigel G; Grigg, Jonathan; Hibberd, Patricia L; Kurmi, Om P; Lam, Kin-bong Hubert; Mortimer, Kevin; Asante, Kwaku Poku; Balakrishnan, Kalpana; Balmes, John; Bar-Zeev, Naor; Bates, Michael N; Breysse, Patrick N; Buist, Sonia; Chen, Zhengming; Havens, Deborah; Jack, Darby; Jindal, Surinder; Kan, Haidong; Mehta, Sumi; Moschovis, Peter; Naeher, Luke; Patel, Archana; Perez-Padilla, Rogelio; Pope, Daniel; Rylance, Jamie; Semple, Sean; Martin, William J

    2014-10-01

    A third of the world's population uses solid fuel derived from plant material (biomass) or coal for cooking, heating, or lighting. These fuels are smoky, often used in an open fire or simple stove with incomplete combustion, and result in a large amount of household air pollution when smoke is poorly vented. Air pollution is the biggest environmental cause of death worldwide, with household air pollution accounting for about 3·5-4 million deaths every year. Women and children living in severe poverty have the greatest exposures to household air pollution. In this Commission, we review evidence for the association between household air pollution and respiratory infections, respiratory tract cancers, and chronic lung diseases. Respiratory infections (comprising both upper and lower respiratory tract infections with viruses, bacteria, and mycobacteria) have all been associated with exposure to household air pollution. Respiratory tract cancers, including both nasopharyngeal cancer and lung cancer, are strongly associated with pollution from coal burning and further data are needed about other solid fuels. Chronic lung diseases, including chronic obstructive pulmonary disease and bronchiectasis in women, are associated with solid fuel use for cooking, and the damaging effects of exposure to household air pollution in early life on lung development are yet to be fully described. We also review appropriate ways to measure exposure to household air pollution, as well as study design issues and potential effective interventions to prevent these disease burdens. Measurement of household air pollution needs individual, rather than fixed in place, monitoring because exposure varies by age, gender, location, and household role. Women and children are particularly susceptible to the toxic effects of pollution and are exposed to the highest concentrations. Interventions should target these high-risk groups and be of sufficient quality to make the air clean. To make clean energy

  3. Pulmonary fibrosis and emphysema: Is the emphysema type associated with the pattern of fibrosis?

    Institute of Scientific and Technical Information of China (English)

    Anastasia; Oikonomou; Paraskevi; Mintzopoulou; Argyris; Tzouvelekis; Petros; Zezos; George; Zacharis; Anastasios; Koutsopoulos; Demosthenes; Bouros; Panos; Prassopoulos

    2015-01-01

    AIM: To investigate whether the predominant emphysema type is associated with the high resolution computed tomography(HRCT) pattern of fibrosis in combined pulmonary fibrosis and emphysema(CPFE).METHODS: Fifty-three smokers with upper lobe emphysema and lower lobe pulmonary fibrosis on- HRCT-were retrospectively evaluated. Patients were stratified into 3 groups according to the predominant type of emphysema: Centrilobular(CLE), paraseptal(PSE), CLE = PSE. Patients were also stratified into 3 other groups according to the predominant type of fibrosis on HRCT: Typical usual interstitial pneumonia(UIP), probable UIP and nonspecific interstitial pneumonia(NSIP). HRCTs were scored at 5 predetermined levels for the coarseness of fibrosis(Coarseness), extent of emphysema(emphysema), extent of interstitial lung disease(Tot Ext ILD), extent of reticular pattern not otherwise specified(Ret NOS), extent of ground glass opacity with traction bronchiectasis(extG GOBx), extent of pure ground glass opacity and extent of honeycombing. HRCT mean scores, pulmonary function tests, diffusion capacity(DLCO) and systolic pulmonary arterial pressure were compared among the groups.RESULTS: The predominant type of emphysema was strongly correlated with the predominant type of fibrosis. The centrilobular emphysema group exhibited a significantly higher extent of emphysema(P P > 0.000). Although the pulmonary arterial pressure was higher in typical UIP group relative to the NSIP group, the difference was not statistically significant.CONCLUSION: In CPFE patients, paraseptal emphysema is associated more with UIP-HRCT pattern and higher extent of fibrosis than centrilobular emphysema.

  4. High resolution computed tomography of the chest in cystic fibrosis (CF): is simplification of scoring systems feasible?

    Energy Technology Data Exchange (ETDEWEB)

    Oikonomou, Anastasia; Prassopoulos, Panos [University Hospital of Alexandroupolis, Democritus University of Thrace, Department of Radiology, Dragana, Alexandroupolis (Greece); Tsanakas, John; Hatziagorou, Elpis; Kirvassilis, Fotios [Ippokratio Hospital of Thessaloniki, Aristotle University of Thessaloniki, 3d Department of Pediatrics, Thessaloniki (Greece); Efremidis, Stavros [University Hospital of Ioannina, University of Ioannina, Department of Radiology, Ioannina (Greece)

    2008-03-15

    The purpose of this study was to simplify HRCT scoring systems (SS) for CF by selecting representative HRCT parameters. Forty-two consecutive patients with CF underwent baseline and follow-up chest HRCT. Three radiologists evaluated 84 HRCTs employing five SS. ''Simplified'' HRCT SS were formed by selection of parameters exhibiting statistically significant relations with FEV1. Pulmonary function tests (PFTs) and nutrition (IBW%) were recorded. Regression analysis, Pearson correlation and T-test were used for statistical analysis. Three HRCT parameters were selected for the formation of ''simplified'' HRCT SS (severity of bronchiectasis, bronchial wall thickening, atelectasis-consolidation) using regression analysis. There was excellent correlation between each ''simplified'' and corresponding complete score (0.892 < r < 0.0967, p < 0.0001) or the remaining four complete scores (0.786 < r < 0.961, p < 0.0001). Strong correlation was found among the five ''simplified'' scores (0.803 < r < 0.997, p < 0.0001). Comparing baseline complete and ''simplified'' scores with corresponding follow-up ones, significant worsening was observed (p < 0.0001). PFTs and IBW% did not change significantly. HRCT scores correlated moderately with FVC and FEV1, but there was no correlation with FEF25-75 and IBW%. ''Simplified'' HRCT SS are as reliable as the complete ones and detect progression of lung disease earlier than clinical parameters. They are easy to use and could be adopted in clinical practice. (orig.)

  5. Pseudomonas aeruginosa elastase provides an escape from phagocytosis by degrading the pulmonary surfactant protein-A.

    Directory of Open Access Journals (Sweden)

    Zhizhou Kuang

    Full Text Available Pseudomonas aeruginosa is an opportunistic pathogen that causes both acute pneumonitis in immunocompromised patients and chronic lung infections in individuals with cystic fibrosis and other bronchiectasis. Over 75% of clinical isolates of P. aeruginosa secrete elastase B (LasB, an elastolytic metalloproteinase that is encoded by the lasB gene. Previously, in vitro studies have demonstrated that LasB degrades a number of components in both the innate and adaptive immune systems. These include surfactant proteins, antibacterial peptides, cytokines, chemokines and immunoglobulins. However, the contribution of LasB to lung infection by P. aeruginosa and to inactivation of pulmonary innate immunity in vivo needs more clarification. In this study, we examined the mechanisms underlying enhanced clearance of the ΔlasB mutant in mouse lungs. The ΔlasB mutant was attenuated in virulence when compared to the wild-type strain PAO1 during lung infection in SP-A+/+ mice. However, the ΔlasB mutant was as virulent as PAO1 in the lungs of SP-A⁻/⁻ mice. Detailed analysis showed that the ΔlasB mutant was more susceptible to SP-A-mediated opsonization but not membrane permeabilization. In vitro and in vivo phagocytosis experiments revealed that SP-A augmented the phagocytosis of ΔlasB mutant bacteria more efficiently than the isogenic wild-type PAO1. The ΔlasB mutant was found to have a severely reduced ability to degrade SP-A, consequently making it unable to evade opsonization by the collectin during phagocytosis. These results suggest that P. aeruginosa LasB protects against SP-A-mediated opsonization by degrading the collectin.

  6. Performance of long-term CT monitoring in diagnosing bronchiolitis obliterans after lung transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Berstad, Audun E. [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway)]. E-mail: a.e.berstad@medisin.uio.no; Aalokken, Trond Mogens [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway); Kolbenstvedt, Alf [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway); Bjortuft, Oystein [Department of Thoracic Medicine, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway)

    2006-04-15

    Aim: The purpose of the study was to evaluate the ability of CT, including expiratory scans with minimum intensity projection in predicting the development of bronchiolitis obliterans syndrome after lung transplantation. Materials and methods: Forty consecutive patients, 29 bilateral and 11 single lung transplanted, were followed-up with regular scans for a median of 36 months. Air trapping was evaluated on expiratory scans constructed from two short spiral scans with minimum intensity projection-technique, one at the level of the carina and the other midway between the right diaphragm and the carina. Air trapping was scored on a 16-point scale. Bronchiolitis obliterans syndrome was diagnosed according to established clinical criteria and quantified spirometrically. Results: Bronchiolitis obliterans syndrome developed in 17 patients (43%) after a median of 12 months. Air trapping and bronchiectasis was seen before the diagnosis of bronchiolitis obliterans syndrome in only two and one patient, respectively. Interobserver agreement for air trapping score was good (kappa = 0.65). Air trapping scores performed significantly better than that achieved by chance alone in determining the presence of bronchiolitis obliterans syndrome (P = 0.0025). An air trapping score of 4 or more provided the best results with regard to sensitivity and specificity in diagnosing bronchiolitis obliterans syndrome. The sensitivity, specificity, positive and negative predictive values of an air trapping of 4 or more in the diagnosis of bronchiolitis obliterans syndrome were 77, 74, 68 and 81%, respectively. Conclusion: Expiratory CT scans with minimum intensity projection-reconstruction did not predict the development of bronchiolitis obliterans syndrome in most patients. The findings seriously limit the clinical usefulness of long-term CT monitoring for diagnosing bronchiolitis obliterans syndrome after lung transplantation.

  7. 602 Cvid: A Common but Still Underdiagnosed Disease

    Science.gov (United States)

    Gallego, Claudia; González-Díaz, Sandra; del Carmen Zarate, Maria; Arias-Cruz, Alfredo; Garcia-Calderin, Diego; Yanneth Mejia Salas, Karla; Calva, Maricruz; Alfredo Dominguez Sansores, Luis

    2012-01-01

    Background Among the more than 150 different forms of Primary Immunodeficiency Diseases (PID) the CVID is the most common symptomatic primary immunodeficiency, present mainly in adults. There is a failure of B cells to develop and differentiate into plasma cells; at consequent a reduction of the production of one or more isotypes of antibody can also affected Cell-mediated immunity. Common manifestations included recurrent bacterial infections, that typically involve the upper and lower respiratory tract. Some patients are highly prone to autoimmune manifestations, lymphoid hyperplasia, and tumors. Methods We presented 3 cases of CVID with a variety of clinical presentation, evolution and complications related to delayed diagnosis. Results A 34 year old male presented chronic diarrhea, weight loss, malnutrition and recurrent upper respiratory infections; digestive tract endoscopy and biopsy was reported with villous atrophy, chronic inflammation and low grade non-Hodgkin's lymphoma B cell. Unfortunately this patient refused the use of gamma globulin treatment, had a high morbidity, and finally the patient died. The case of a nurse with clinical manifestation of recurrent rinosinusitis and pneumonia, which was diagnosed as IDCV 17 years later, after she developed pulmonary bronchiectasis. Fortunately the disease is under control and she is actually under treatment with intravenous immunoglobulin. Finally, the case of a 44 year old female, who suffered from recurrent upper respiratory infections, additionally had a thyroid gland tumor associated which affecting the thyroid function. Conclusions In the 3 cases had low levels of all immunoglobulin as a hallmark. The clinician must be suspecting this condition in all adults with recurrent infectious disease who have gastrointestinal symptoms or who are detected a malignant disease. Early diagnosis and correct treatment are critical in preventing tissue damage, long-term sequelae and death. Replacement with intravenous

  8. Risk of lung cancer in patients with gastro-esophageal reflux disease: a population-based cohort study

    Science.gov (United States)

    Hsu, Chi-Kuei; Lai, Chih-Cheng

    2016-01-01

    This large-scale, controlled cohort study estimated the risks of lung cancer in patients with gastro-esophageal reflux disease (GERD) in Taiwan. We conducted this population-based study using data from the National Health Insurance Research Database of Taiwan during the period from 1997 to 2010. Patients with GERD were diagnosed using endoscopy, and controls were matched to patients with GERD at a ratio of 1:4. We identified 15,412 patients with GERD and 60,957 controls. Compared with the controls, the patients with GERD had higher rates of osteoporosis, diabetes mellitus, asthma, chronic obstructive pulmonary disease, pneumonia, bronchiectasis, depression, anxiety, hypertension, dyslipidemia, chronic liver disease, congestive heart failure, atrial fibrillation, stroke, chronic kidney disease, and coronary artery disease (all P < .05). A total of 85 patients had lung cancer among patients with GERD during the follow-up of 42,555 person-years, and the rate of lung cancer was 0.0020 per person-year. By contrast, 232 patients had lung cancer among patients without GERD during the follow-up of 175,319 person-years, and the rate of lung cancer was 0.0013 per person-year. By using stepwise Cox regression model, the overall incidence of lung cancer remained significantly higher in the patients with GERD than in the controls (hazard ratio, 1.53; 95% CI [1.19–1.98]). The cumulative incidence of lung cancer was higher in the patients with GERD than in the controls (P = .0012). In conclusion, our large population-based cohort study provides evidence that GERD may increase the risk of lung cancer in Asians. PMID:28028458

  9. Cystic Fibrosis Involving Multisystem: A Case Report and Literature Review%囊性纤维化累及多系统一例

    Institute of Scientific and Technical Information of China (English)

    王犇; 杨丽

    2012-01-01

    目的 提高对囊性纤维化的认识.方法 2011年11月收治1例自幼有临床表现的囊性纤维化患者,回顾其诊断及治疗经过,复习相关文献总结其临床特征、诊疗进展及预后评价.结果 囊性纤维化起病年龄较早,患者自幼年起即反复出现肺、消化道、肝脏等多系统病变,最终导致多器官功能衰竭.结论 应提高对囊性纤维化的识别度,对于发病年龄过早、反复发作的严重支气管扩张,伴随生长发育延迟、肝硬化等临床征象应注意对囊性纤维化的筛查.%Objective To know more about cystic fibrosis. Methods A patient with cystic fibrosis with typical clinical features was diagnosed and treated. We retrospectively analyzed and evaluate the clinical characteristics, diagnosis and treatment, and the prognosis. Results The onset of cystic fibrosis was early. This patient had repeating multisystem diseases including lung, pancreas, digestive system, liver, etc from a child, and she finally had multiple organ failure. Conclusion We should know more about cystic fibrosis, and should screen the patients with early onset and repeating severe bronchiectasis with delaying growing development and hepatocirrhosis.

  10. Clinical characteristics of 41 patients with intractable asthma

    Directory of Open Access Journals (Sweden)

    Li BAI

    2011-09-01

    Full Text Available Objective To explore the clinical characteristics of intractable asthma,and to provide new knowledge for diagnosis and treatment of the disease.Methods Forty one patients with intractable asthma,admitted to the Institute of Respiratory Disease,Xinqiao Hospital of Third Military Medical University from Jan.2009 to Dec.2010,were included in present study.Spirometry tests were performed for all the 41 patients.Cell classification and counting were done in the induced sputum of 37 patients,and 34 patients underwent high-resolution chest computed tomography(HRCT.Results Incomplete reversibility of airflow obstruction(FEV1/FVC 0.03 of the total cells,13(35.1% and increased neutrophils( > 0.61 of total cells,6(16.2% showed increased both eosinophils and neutrophils,and only that of one patient showed normal percentage of the eosinophils and neutrophils.Chest HRCT of 34 patients showed thickening of bronchial wall in visible segment in 28 cases(82.3%,and in 22 cases(64.7% thickening of bronchial wall in secondary segments was accompanied with narrowed bronchus lumen,cylindrical bronchiectasis was predominant in 7 patients,and centrilobular emphysema was seen in 5 patients.Conclusion Airway remodeling,incomplete reversibility of airflow obstruction,airway inflammation appear to be the major clinical characteristics of intractable asthma.Combined use of chest HRCT,spirometry test,and cellular analysis of induced sputum may be helpful for identifying intractable asthma,and they provide the basis for individualized strategies to manage the disease.

  11. Epidemiology and costs of hospital care for COPD in Puglia

    Directory of Open Access Journals (Sweden)

    Moretti Anna

    2011-10-01

    Full Text Available Abstract Background and aims Chronic obstructive pulmonary disease (COPD is currently the 5th cause of morbidity and mortality in the developed world and represents a substantial economic and social burden. The aim of this study is to report on hospital admissions and related costs of hospital treatment for COPD in the Puglia Region of Italy in the years 2005-2007. Materials and methods Patients were selected who were hospitalized between 01/01/2005 and 31/12/2007 with ICD-9-CM code: 490.xx: bronchitis not specified as acute or chronic; 491.xx: chronic bronchitis; 492.xx: emphysema; 493.xx: asthma; 494.xx: bronchiectasis; 496.xx: chronic airway obstruction not elsewhere classified; 518.81: acute respiratory failure as principal or secondary diagnosis. Results In the period 2005-2007, there were 73,721 hospital admissions for COPD registered in Puglia (25,690 in 2005; 24,153 in 2006 and 23,878 in 2007 of which 34.3% were women, with no significant variation in the three years. There appears to be a negative trend in hospitalisations in Puglia for chronic bronchitis with ratios decreasing from 359.4 per 100,000 population in 2005 to 307.9 per 100,000 in 2007. The overall cost of COPD for Apulian hospital trusts was €272,293,182.85 over the 3-year period. Conclusions Analysis of the data for hospital care, its costs and performance may be an important indicator of the efficacy of community care. In particular, the lack of reduction in admissions for COPD should lead decision makers to question both the appropriateness and quality of the care given.

  12. Invasive Aspergillus infections in hospitalized patients with chronic lung disease

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    Wessolossky M

    2013-05-01

    Full Text Available Mireya Wessolossky,1 Verna L Welch,2 Ajanta Sen,1 Tara M Babu,1 David R Luke21Division of Infectious Diseases, University of Massachusetts Medical School, Worcester, MA, USA; 2Medical Affairs, Pfizer Inc, Collegeville, PA, USABackground: Although invasive pulmonary aspergillosis (IPA is more prevalent in immunocompromised patients, critical care clinicians need to be aware of the occurrence of IPA in the nontraditional host, such as a patient with chronic lung disease. The purpose of this study was to describe the IPA patient with chronic lung disease and compare the data with that of immunocompromised patients.Methods: The records of 351 patients with Aspergillus were evaluated in this single-center, retrospective study for evidence and outcomes of IPA. The outcomes of 57 patients with chronic lung disease and 56 immunocompromised patients were compared. Patients with chronic lung disease were defined by one of the following descriptive terms: emphysema, asthma, idiopathic lung disease, bronchitis, bronchiectasis, sarcoid, or pulmonary leukostasis.Results: Baseline demographics were similar between the two groups. Patients with chronic lung disease were primarily defined by emphysema (61% and asthma (18%, and immunocompromised patients primarily had malignancies (27% and bone marrow transplants (14%. A higher proportion of patients with chronic lung disease had a diagnosis of IPA by bronchoalveolar lavage versus the immunocompromised group (P < 0.03. The major risk factors for IPA were found to be steroid use in the chronic lung disease group and neutropenia and prior surgical procedures in the immunocompromised group. Overall, 53% and 69% of chronic lung disease and immunocompromised patients were cured (P = 0.14; 55% of chronic lung patients and 47% of immunocompromised patients survived one month (P = 0.75.Conclusion: Nontraditional patients with IPA, such as those with chronic lung disease, have outcomes and mortality similar to that in the

  13. A challenge to the seven widely believed concepts of COPD

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    Al-Kassmimi FA

    2013-01-01

    Full Text Available Feisal A Al-Kassimi, Esam H AlhamadDivision of Pulmonology, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi ArabiaAbstract: This review proposes a critical reassessment (based entirely on published evidence of the following seven common beliefs about chronic obstructive pulmonary disease (COPD: (1 COPD is one disease. (2 There is a valid definition for COPD. (The current definition includes cases of irreversible asthma and bronchiectasis, and occasionally, other obstructive lung conditions. (3 Irreversible asthma in smokers and COPD cannot be differentiated. (4 A “chronic bronchitis” form of COPD exists and is characterized by blue bloater status and normal carbon monoxide diffusion studies. (5 Phenotyping has no bearing on medication choice in COPD. (6 Computerized scoring of lung attenuation on CT scans can diagnose emphysema. (Emphysema scores overlap in irreversible asthma and COPD; however, qualitative visual changes may be useful for differentiation. (7 A definable entity called the overlap (of COPD and asthma syndrome exists. Conflict over the abovementioned points denies patients proper phenotype-guided therapy and encourages a multidrug approach to COPD management. The recently coined term, overlap syndrome, invites a double-barreled therapy aimed at asthma and COPD, despite the absence of any agreement about how to define the syndrome and the lack of any related drug trials (in the area of inhaled corticosteroids. A diagnosis of COPD is associated with high morbidity and escalating costs, suggesting the need for a thorough new examination of the evidence.Keywords: asthma, computerized tomography, COPD, global initiative for chronic obstructive lung disease, overlap syndrome

  14. Prevalence of lung structure abnormalities in patients with acromegaly and their relationship with gas exchange: cross-sectional analytical study with a control group

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    Marcelo Palmeira Rodrigues

    Full Text Available CONTEXT AND OBJECTIVE: Different functional respiratory alterations have been described in acromegaly, but their relationship with pulmonary tissue abnormalities is unknown. The objective of this study was to observe possible changes in lung structure and explain their relationship with gas exchange abnormalities. DESIGN AND SETTING: Cross-sectional analytical study with a control group, conducted at a university hospital. METHODS: The study included 36 patients with acromegaly and 24 controls who were all assessed through high-resolution computed tomography of the thorax (CT. Arterial blood gas, effort oximetry and serum growth hormone (GH and insulin-like growth factor I (IGF-1 were also assessed in the patients with acromegaly. RESULTS: The abnormalities found in the CT scan were not statistically different between the acromegaly and control groups: mild cylindrical bronchiectasis (P = 0.59, linear opacity (P = 0.29, nodular opacity (P = 0.28, increased attenuation (frosted glass; P = 0.48 and decreased attenuation (emphysema; P = 0.32. Radiographic abnormalities were not associated with serum GH and IGF-1. Hypoxemia was present in seven patients; however, in six of them, the hypoxemia could be explained by underlying clinical conditions other than acromegaly: chronic obstructive pulmonary disease in two, obesity in two, bronchial infection in one and asthma in one. CONCLUSION: No changes in lung structure were detected through thorax tomography in comparison with the control subjects. The functional respiratory alterations found were largely explained by alternative diagnoses or had subclinical manifestations, without any plausible relationship with lung structural factors.

  15. Evaluation of the safety of high-frequency chest wall oscillation (HFCWO therapy in blunt thoracic trauma patients

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    Becker Brian

    2008-10-01

    Full Text Available Abstract Background Airway clearance is frequently needed by patients suffering from blunt chest wall trauma. High Frequency Chest Wall Oscillation (HFCWO has been shown to be effective in helping to clear secretions from the lungs of patients with cystic fibrosis, bronchiectasis, asthma, primary ciliary dyskinesia, emphysema, COPD, and many others. Chest wall trauma patients are at increased risk for development of pulmonary complications related to airway clearance. These patients frequently have chest tubes, drains, catheters, etc. which could become dislodged during HFCWO. This prospective observational study was conducted to determine if HFCWO treatment, as provided by The Vest™ Airway Clearance System (Hill-Rom, Saint Paul, MN, was safe and well tolerated by these patients. Methods Twenty-five blunt thoracic trauma patients were entered into the study. These patients were consented. Each patient was prescribed 2, 15 minute HFCWO treatments per day using The Vest® Airway Clearance System (Hill-Rom, Inc., St Paul, MN. The Vest® system was set to a frequency of 10–12 Hz and a pressure of 2–3 (arbitrary unit. Physiological parameters were measured before, during, and after treatment. Patients were free to refuse or terminate a treatment early for any reason. Results No chest tubes, lines, drains or catheters were dislodged as a result of treatment. One patient with flail chest had a chest tube placed after one treatment due to increasing serous effusion. No treatments were missed and continued without further incident. Post treatment survey showed 76% experienced mild or no pain and more productive cough. Thirty days after discharge there were no deaths or hospital re-admissions. Conclusion This study suggests that HFCWO treatment is safe for trauma patients with lung and chest wall injuries. These findings support further work to demonstrate the airway clearance benefits of HFCWO treatment.

  16. Thoracotomies in children.

    Science.gov (United States)

    Findik, Gokturk; Gezer, Suat; Sirmali, Mehmet; Turut, Hasan; Aydogdu, Koray; Tastepe, Irfan; Karaoglanoglu, Nurettin; Kaya, Sadi

    2008-06-01

    Thoracotomies in children have been less extensively studied, as the incidence of diseases necessitating thoracotomies is low in the pediatric age group. This study reviews childhood thoracic diseases, thoracotomy approaches, indications, and complications. Surgical procedures and complications of a total of 196 children below 16 years of age who underwent thoracotomy for various reasons at the Department of Thoracic Surgery, Ataturk Chest Diseases and Chest Surgery Training and Research Hospital, between January 2000 and December 2004, were reviewed in this study. Out of the 196 patients, 77 were female (39%) and 119 (61%) were male. The most commonly encountered indications for surgery were hydatid cyst (35%), bronchiectasis (25%), chronic nonspecific pleuritis (13%), chest wall deformities (10%), and mediastinal cystic formations and masses (10%). The other indications included tuberculosis (3%), aspergilloma (0.5%), fibrohyalinized cyst (0.5%), resection of trachea (0.5%), bronchogenic cyst (0.5%), inflammatory pseudo-tumor (0.5%), sequestration (1%), lipoblastoma (0.5%), and eosinophilic granuloma (1%). Out of the 196 patients, 176 underwent lateral thoracotomy and 20 patients with a chest wall deformity underwent midsternal incision. Complications were seen in 35 patients (18%): atelectasia and secretory retention (54%), wound infection (17%), hemorrhage (3%), chylothorax (3%), intrathoracic space (3%), and postoperative extended air leakage (20%). The mean hospital stay was 15 days and we did not encounter any mortality. The physiology and anatomy of the respiratory system and especially the respiratory control mechanism in pediatric patients vary from those of the adults, resulting in a more morbid course after thoracic surgery in children. Despite severe postoperative pain, posterolateral thoracotomy is the preferred approach in adults because of an advanced intrathoracic exposure and easy manipulation. On the other hand, lower pain threshold and the

  17. HRCT of the lung in collagen vascular diseases; HRCT der Lunge bei Kollagenosen

    Energy Technology Data Exchange (ETDEWEB)

    Diederich, S. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany); Roos, N. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany); Schmitz-Linneweber, B. [Medizinische Klinik B, Westfaelische Wilhelms-Univ., Muenster (Germany); Gaubitz, M. [Medizinische Klinik B, Westfaelische Wilhelms-Univ., Muenster (Germany); Peters, P.E. [Inst. fuer Klinische Radiologie, Westfaelische Wilhelms-Univ., Muenster (Germany)

    1996-07-01

    Collagen vascular diseases, representing systemic soft tissue disorders, may cause a broad spectrum of pathologic changes of the respiratory tract. The type and extent of manifestations can vary considerably among individuals and entities. This survey describes the chest radiographic and, in particular, high-resolution computed tomographic and, in particular, high-resolution computed tomographic (HRCT) findings of individual lesions of the respiratory tract. It includes fibrosing alveolitis (alveolitis, interstitial pneumonia, pulmonary fibrosis) and bronchial (bronchitis/bronchiolitis, bronchiectasis), pleural and vascular manifestations, as well as lymphadenopathy and abnormalities related to therapy. We present typical patterns of changes in progressive systemic sclerosis (PSS, scleroderma), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD, Sharp syndrome), Sjoegren syndrome, overlap syndrome and rheumatoid arthritis (RA). Furthermore, we describe findings which are specific for individual entities such as esophageal involvement in PSS, acute pneumonitis and pulmonary hemorrhage in SLE, lymphoproliferative disease in Sjoegren syndrome and necrobiotic nodules in RA. (orig.) [Deutsch] Die Kollagenosen koennen als systemische Bindegewebserkrankungen auch zu einem breiten Spektrum pathologischer Veraenderungen am Respirationstrakt fuehren, wobei sich Art und Ausmass der Manifestationen innerhalb einzelner Entitaeten und zwischen verschiedenen Krankheitsbildern erheblich unterscheiden koennen. In der vorliegenden Uebersicht werden die entsprechenden Befunde von Thoraxuebersichtsaufnahme und insbesondere hochaufloesender Computertomographie (HRCT) beschrieben. Beruecksichtigt werden dabei die fibrosierende Alveolitis (Alveolitis, interstitielle Pneumonie, Lungenfibrose), bronchiale (Bronchitis/Bronchiolitis, Bronchiektasen), pleurale und vaskulaere Manifestationen sowie Lymphadenopathie und therapie-induzierte Befunde. Typische Befundmuster

  18. Risk of lung cancer in patients with gastro-esophageal reflux disease: a population-based cohort study

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    Chi-Kuei Hsu

    2016-12-01

    Full Text Available This large-scale, controlled cohort study estimated the risks of lung cancer in patients with gastro-esophageal reflux disease (GERD in Taiwan. We conducted this population-based study using data from the National Health Insurance Research Database of Taiwan during the period from 1997 to 2010. Patients with GERD were diagnosed using endoscopy, and controls were matched to patients with GERD at a ratio of 1:4. We identified 15,412 patients with GERD and 60,957 controls. Compared with the controls, the patients with GERD had higher rates of osteoporosis, diabetes mellitus, asthma, chronic obstructive pulmonary disease, pneumonia, bronchiectasis, depression, anxiety, hypertension, dyslipidemia, chronic liver disease, congestive heart failure, atrial fibrillation, stroke, chronic kidney disease, and coronary artery disease (all P < .05. A total of 85 patients had lung cancer among patients with GERD during the follow-up of 42,555 person-years, and the rate of lung cancer was 0.0020 per person-year. By contrast, 232 patients had lung cancer among patients without GERD during the follow-up of 175,319 person-years, and the rate of lung cancer was 0.0013 per person-year. By using stepwise Cox regression model, the overall incidence of lung cancer remained significantly higher in the patients with GERD than in the controls (hazard ratio, 1.53; 95% CI [1.19–1.98]. The cumulative incidence of lung cancer was higher in the patients with GERD than in the controls (P = .0012. In conclusion, our large population-based cohort study provides evidence that GERD may increase the risk of lung cancer in Asians.

  19. Quantification of neutrophil migration into the lungs of patients with chronic obstructive pulmonary disease

    Energy Technology Data Exchange (ETDEWEB)

    Ruparelia, Prina; Summers, Charlotte; Chilvers, Edwin R. [University of Cambridge School of Clinical Medicine, Department of Respiratory Medicine, Cambridge (United Kingdom); Szczepura, Katherine R. [University of Cambridge School of Clinical Medicine, Department of Radiology, Cambridge (United Kingdom); Solanki, Chandra K.; Balan, Kottekkattu [Cambridge University Hospitals NHS Foundation Trust, Nuclear Medicine, Addenbrooke' s Hospital, Cambridge (United Kingdom); Newbold, Paul [AstraZeneca R and D Charnwood, Loughborough (United Kingdom); Bilton, Diana [Papworth Hospital NHS Foundation Trust, Cystic Fibrosis and Lung Defence Unit, Papworth Everard (United Kingdom); Peters, A.M. [University of Cambridge School of Clinical Medicine, Department of Radiology, Cambridge (United Kingdom); Brighton Sussex Medical School, Brighton (United Kingdom)

    2011-05-15

    To quantify neutrophil migration into the lungs of patients with chronic pulmonary obstructive disease (COPD). Neutrophil loss via airways was assessed by dedicated whole-body counting 45 min, 24 h and 2, 4, 7 and 10 days after injection of very small activities of {sup 111}In-labelled neutrophils in 12 healthy nonsmokers, 5 healthy smokers, 16 patients with COPD (of whom 7 were ex-smokers) and 10 patients with bronchiectasis. Lung accumulation of {sup 99m}Tc-labelled neutrophils was assessed by sequential SPECT and Patlak analysis in six COPD patients and three healthy nonsmoking subjects. Whole body {sup 111}In counts, expressed as percentages of 24 h counts, decreased in all subjects. Losses at 7 days (mean {+-} SD) were similar in healthy nonsmoking subjects (5.5 {+-} 1.5%), smoking subjects (6.5 {+-} 4.4%) and ex-smoking COPD patients (5.8 {+-} 1.5%). In contrast, currently smoking COPD patients showed higher losses (8.0 {+-} 3.0%) than healthy nonsmokers (p = 0.03). Two bronchiectatic patients lost 25% and 26%, indicating active disease; mean loss in the remaining eight was 6.9 {+-} 2.5%. The rate of accumulation of {sup 99m}Tc-neutrophils in the lungs, determined by sequential SPECT, was increased in COPD patients (0.030-0.073 min{sup -1}) compared with healthy nonsmokers (0-0.002 min{sup -1}; p = 0.02). In patients with COPD, sequential SPECT showed increased lung accumulation of {sup 99m}Tc-labelled neutrophils, while whole-body counting demonstrated subsequent higher losses of {sup 111}In-labelled neutrophils in patients who continued to smoke. Sequential SPECT as a means of quantifying neutrophil migration deserves further evaluation. (orig.)

  20. Cystic fibrosis in adults. Short-term and long-term reproducibility of the Brody score for lung morphology in low-dose MDCT scans

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    Weber, K.; Paolini, M.; Schmitz, M.; Coppenrath, E.; Reiser, M.; Mueller-Lisse, U.G. [Ludwig-Maximilians-Univ. Muenchen (Germany). Inst. fuer Klinische Radiologie; Fischer, R.; Huber, R. [Ludwig-Maximilians-Univ. Muenchen (Germany). Medical Hospital V

    2014-01-15

    Purpose: The semi-quantitative Brody score measures the severity of cystic fibrosis (CF)-related lung disease. We investigated the short-term (28 - 60 days) and long-term (2 - 7 years) intra- and inter-observer reproducibility of the Brody score in low-dose multidetector row computed tomography examinations performed in inspiration (LDCTs) of adult CF patients. Materials and Methods: Composite Brody scores and respective underlying bronchiectasis, mucus plugging, peribronchial thickening, parenchymal opacity, and hyperinflation subscores were evaluated twice (time interval, 1 - 84 months) by each of 3 independent radiologists (1 - 20 years of professional diagnostic radiology experience) in LDCTs (4 - 64 rows, 120 KVp, 10 - 15 mAs/slice, CTDIw approx. 1.0 mGy, effective dose approx. 0.5 mSv) of 15 adult patients with CF-related lung disease (8 female, 7 male, age, 18 - 50 years, mean, 33 years). Results: The average reproducibility of the Brody score was within +/-7 % (range, 2 - 30 %) between radiologists, and +/-6 % (3 - 12 %) within radiologists (short-term, 28 - 60 days, 4 %, 0 - 12 %, long-term, 2 - 7 years, 12 %, 1 - 36 %). For the different subscores, the reproducibility was within +/-25 % (15 - 41 %) between radiologists and +/-23 % (12 - 46 %) within radiologists. Conclusion: The Brody score shows high average inter-observer reproducibility in LDCTs of adult CF patients. The Brody score also demonstrates high average intra-observer reproducibility if subsequent assessments are made within 28 - 61 days. With time intervals of 2 - 7 years between subsequent evaluations, however, intra-observer reproducibility decreases. Respective subscores each demonstrate lower intra- and inter-observer reproducibility than does the composite Brody score. (orig.)

  1. Human infection with a novel avian-origin influenza A (H7N9) virus: serial chest radiographic and CT findings

    Institute of Scientific and Technical Information of China (English)

    Dai Jian; Zhou Xianmei; Dong Danjiang; Liu Yin; Gu Qin; Zhu Bin; Wu Chao

    2014-01-01

    Background Rapidly progressive pneumonia infection with H7N9 virus is a novel disease,and limited information is available concerning serial chest radiographic and computed tomography (CT) findings.The aim of this study was to evaluate the changes in serial radiologic findings in patients with H7N9 pneumonia.Methods The two institutional ethics review boards approved this retrospective study.This study included 10 patients with H7N9 pneumonia.All patients underwent chest radiologic examinations at different time points.Serial radiologic images were systematically analyzed.Results All patients showed abnormal results on initial chest radiography and CT.The initial radiographic abnormalities were unilateral (n=9) and bilateral (n=1),including ground-glass opacities (GGOs) (n=5) and consolidation (n=5).The initial CT findings consisted of unilateral (n=6) and bilateral (n=4),including consolidation (n=10),GGOs (n=10),reticular opacities (n=2),and pleural effusion (n=3).Follow-up radiologic findings showed rapid development of consolidation or GGOs within two weeks after illness onset.Pneumomediastinum with secondary subcutaneous emphysema and pneumothorax were noted in two patients.Follow-up high resolution computed tomography (HRCT) after two weeks showed slow improvement in both size and opacity of the lesions.On HRCT after discharge,patients had substantial residual lesions such as irregular linear opacities,reticular opacities,parenchymal bands,traction bronchiectasis,and cystic lesions.Conclusions The most common radiologic findings at presentation are multifocal or diffuse areas of consolidation and GGOs in H7N9 pneumonia.HRCT in sequence can show more changes in rapid progression of disease and a slow decrease of both size and opacity of the lesions plays an important role in the evaluation of H7N9 pneumonia.

  2. Pulmonary infections caused by non-tuberculous mycobacteria−single centre experience

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    Adžić-Vukičević Tatjana N.

    2013-01-01

    Full Text Available Introduction. Non-tuberculosis mycobacteria are environmental organisms found in soil and water worldwide, and an infection caused by non-tuberculosis mycobacteria is less frequently found than the one associated with Mycobacterium tuberculosis. This study was designed to evaluate data relating to non-tuberculosis mycobacteria in patients with clinical importance. Material and Methods. Of 12 patients (pts admitted to the Department of Pulmonology, Clinical Centre of Serbia in Belgrade during 2010- 2011, seven (58.33% were men and five (41.67% were women. Bacteriological and radiographic findings, co−morbidity, treatment management and outcome were evaluated from medical records. Results. Using GenoType® Mycobacterium CM/AS (Hain Lifescience assays for identification of isolated cultures of NTM M.xenopi was found in six (50% pts, M.avium complex in two (16.67% pts, M.kansasii and M.xenopi in one (8.33%, M. gordone, M.abscessus and M.peregrinum in one (8.33% patient each. Cavitary lesions were most frequently determined on high resolution computed tomography in five (41.67% pts, followed with consolidation in two (16.67% pts, cavitation with fibronodular lesions and bronchiectasis in one (8.33% patient each. Comorbidities were noticed in nine (75% pts, with chronic obstructive pulmonary diseases most frequently found in six (50% pts. According to American Thoracic Society definition and criteria, treatment was administered in nine (75% pts. Conclusion. In order to find the right treatment, it is important to identify non-tuberculosis mycobacteria lung infection by culture methods, at least two positive, accompanied with high resolution computed tomography changes.

  3. Allergic bronchopulmonary aspergillosis: A review of 42 patients from a tertiary care center in India

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    Prasad R

    2009-01-01

    Full Text Available Objective: To study the clinical, radiological, and laboratory profile in patients of allergic bronchopulmonary aspergillosis (ABPA. Materials and Methods: Retrospective analysis of 42 cases of ABPA, diagnosed over a period of 10 years from 1995 to 2005, for their clinical, radiological, and laboratory profiles. Results: Of 42 ABPA patients, 27 were men and 17 were women. Their mean age at the time of diagnosis was 31.2 years and mean duration of illness was 12.2 years. Breathlessness was the chief symptom. Other allergic disorders existed in 17 (40.5% patients, and family history suggestive of allergic disease was present in 22 (52.4% patients. Most common chest radiographic finding was fleeting pulmonary shadows in 28 (66.7% patients. High resolution CT thorax revealed central bronchiectasis as predominant finding. Peripheral blood eosinophilia more than 1000 cells/µl, Type I and type III cutaneous reactivity to Aspergillus antigen, elevated serum titers of total IgE antibody, A. fumigatus specific IgE and IgG antibodies, and serum precipitin against A. fumigatus were positive in majority of patients, who underwent these tests. Thirty eight (90.5% patients had had history of antitubercular treatment during the course of their illness. All 42 patients met at least four criteria for the diagnosis of ABPA. Conclusion: Any patient of bronchial asthma, presenting with recurrent shadows in chest radiograph and high peripheral blood eosinophilia, should be investigated for ABPA. Efforts need to be intensified to improve the awareness level among general physicians for early diagnosis and prompt treatment of this disease to avoid misuse of antitubercular drugs.

  4. High resolution CT diagnosis of Kartagener syndrome:a report of the three cases and literature review of 118 cases%Kartagener综合征的高分辨率CT诊断(附3例报告及118例文献回顾)

    Institute of Scientific and Technical Information of China (English)

    李周强; 董养珍; 王生海; 宋鹏; 耿冀洲

    2012-01-01

    Objective To explore the clinical and high resolution CT(HRCT) findings of Kartagener syndrome in order to improve the understanding of the disease and its early diagnosis. Methods The clinical data and HRCT chest findings of three cases of Kartagener syndrome were presented. The clinical data and the lung CT manifestations of 118 cases reported in the literature were statistically analyzed. Results Three cases and 118 literature review cases all showed lung capsule columnar bronchiectasis and cent-rilobular nodules scattered in the original middle lobe and the two lower lobes, visceral inversion, and the maxillary sinusitis with repeated respiratory infections. Conclusion Chest HRCT findings of Kartagener syndrome is characteristics,and the diagnosis can be made by combining with the clinical manifestations.%目的 探讨Kartagener综合征的临床及高分辨率CT(HRCT)表现特征,提高对该病的认识及早期诊断.方法 报道3例Kartagener综合征的临床资料和胸部HRCT表现,并对118例文献的临床资料、肺部CT征象进行统计分析.结果 3例与118例文献均表现有肺部原位中叶及两下肺囊柱状支气管扩张及两肺散在小叶中央结节影、内脏全部或部分转位、上颌窦炎三联症及反复呼吸道感染.结论 Kartagener综合征胸部HRCT具有特征性,结合其临床特点是诊断的主要依据.

  5. Kartagener 综合征2例及文献复习%Kartagener syndrome:a report of 2 cases and review of the literature

    Institute of Scientific and Technical Information of China (English)

    杨海华; 李善群; 金先桥; 龙丰

    2015-01-01

    Objective The aim of this study is to improve the understanding of Kartagener syndrome by analysing the clinical characteristics of patients with Kartagener syndrome.Methods To analyse the clinical characteristics and treatment of Kartagener syndrome,a retrospective study about clinical data of two patients with Kartagener syndrome combined with review of the literatures was done. Results The two patients were sister and brother relationship,whose parents were consanguineous marriage.Both of the two patients have bronchiectasis,visceral transposition and sinusitis triple feature which are the evidences to make a definite diagnosis of Kartagener syndrome.Conclusions Kartagener syndrome is a rare disease.Consanguineous marriage can increase the risk of offspring suffering from Kartagener syndrome.Improving the understanding of Kartagener syndrome is conducive to its early diagnosis and treatment.%目的:分析 Kartagener 综合征的临床特点,以提高临床医师对 Kartagener 综合征的认识。方法回顾性分析2例 Kartagener 综合征患者的临床资料并结合文献复习,分析 Kartagener 综合征的临床特点和治疗方法。结果2例患者为姐弟关系,其父母为近亲结婚。2例患者均有确诊 Kartagener综合征的支气管扩张、内脏转位、副鼻窦炎三联征等依据。结论 Kartagener 综合征是一种罕见疾病,近亲结婚可使后代 Kartagener 综合征的发病率增加,提高对 Kartagener 综合征的认识有利于其早期诊断和治疗。

  6. Kartagener综合征3例分析并文献复习%Kartagener syndrome: three cases report and literature review

    Institute of Scientific and Technical Information of China (English)

    顾扬; 吴峰

    2009-01-01

    Objective To improve the early diagnosis and treatment of Kartagener syndrome.Methods The clinical presentation, radiography, ulfrasound cardiogram,electrocardiogram changes of 3 patients were studied,and the relatated literature was reviewed. Results Kartagener syndrome is a rare autosomal recessive genetic disease, recognized by the three symptoms of chronic sinusitis, bronchiectasis,situs inversus. The most common initial symptoms are cough, sputum and sinusitis. Conclusion If the patients with situs irversus have the clinical presentations and radiography of brouchiefasis,it should be thought of Katagener syndrome.%目的 供临床医师提高对Kartagener综合征早期诊断和治疗.方法 通过3例病史、临床表现、影像学、彩超、心电图等检查明确诊断进行分析和文献复习.结果 Kartagener综合征是一种少见的常染色体隐性遗传性疾病.症状为:副鼻窦炎一支气管扩张一内脏转位三联征,其临床表现为反复咳嗽、咯脓痰、咯血等肺部表现,同时伴副鼻窦炎.结论 在内脏转位的患者中若出现支气管扩张症状及影像学表现,应想到Kartagener综合征的可能.

  7. Respiratory tract complications of Kartagener syndrome:two case report%Kartagener 综合征呼吸道症状2例诊治分析

    Institute of Scientific and Technical Information of China (English)

    陈澄; 张秀琴; 黄建安

    2014-01-01

    目的:探讨Kartagener 综合征呼吸道症状的有效治疗措施及日常管理。方法回顾性分析2例Kartagener综合征患者临床资料并结合文献复习,分析Kartagener综合征呼吸道症状的病因、诊断和治疗方法。结果Kartagener综合征患者因基因突变致纤毛结构和功能异常,从而出现慢性鼻窦炎、支气管扩张、内脏反位三联征,针对其呼吸道症状的有效治疗可能减轻支气管、肺疾病的发展。结论 Kartagener 综合征的呼吸道症状应予以终生治疗,及早确诊并选择最合适的治疗方式,有益于疾病的转归。%Objective To explore the appropriate treatment and management in respiratory tract complications of Kartagener syndrome .Methods Retrospective analysis of clinical data of patients with Kartagener syndrome in two cases and review literature , analysis of the cause of Kartagener syndrome , respiratory symptoms , diagnosis and treatment .Results Kartagener syndrome due to mutations caused by abnormal structure and function of cilia , which appeared chronic sinusitis , bronchiectasis , situs inversus triad , may relieve bronchial effective treatment for their respiratory symptoms , lung disease de-velopment .Conclusion Kartagener syndrome should be lifelong treatment , early diagnosis and choose the most appropriate treatment, beneficial outcome of the disease .

  8. A database of annotated promoters of genes associated with common respiratory and related diseases

    KAUST Repository

    Chowdhary, Rajesh

    2012-07-01

    Many genes have been implicated in the pathogenesis of common respiratory and related diseases (RRDs), yet the underlying mechanisms are largely unknown. Differential gene expression patterns in diseased and healthy individuals suggest that RRDs affect or are affected by modified transcription regulation programs. It is thus crucial to characterize implicated genes in terms of transcriptional regulation. For this purpose, we conducted a promoter analysis of genes associated with 11 common RRDs including allergic rhinitis, asthma, bronchiectasis, bronchiolitis, bronchitis, chronic obstructive pulmonary disease, cystic fibrosis, emphysema, eczema, psoriasis, and urticaria, many of which are thought to be genetically related. The objective of the present study was to obtain deeper insight into the transcriptional regulation of these disease-associated genes by annotating their promoter regions with transcription factors (TFs) and TF binding sites (TFBSs). We discovered many TFs that are significantly enriched in the target disease groups including associations that have been documented in the literature. We also identified a number of putative TFs/TFBSs that appear to be novel. The results of our analysis are provided in an online database that is freely accessible to researchers at http://www.respiratorygenomics.com. Promoter-associated TFBS information and related genomic features, such as histone modification sites, microsatellites, CpG islands, and SNPs, are graphically summarized in the database. Users can compare and contrast underlying mechanisms of specific RRDs relative to candidate genes, TFs, gene ontology terms, micro-RNAs, and biological pathways for the conduct of metaanalyses. This database represents a novel, useful resource for RRD researchers. Copyright © 2012 by the American Thoracic Society.

  9. The prevalence of pulmonary tuberculosis in Jeonbug Province

    Energy Technology Data Exchange (ETDEWEB)

    Rhee, S. J.; Moon, M. C.; Song, H. Y.; Choi, K. C. [Jeonbug Natinal University College of Medicine, Jeonju (Korea, Republic of)

    1981-12-15

    A photofluorographic mass survey of P-A chest had been done to the 13136 residents (male 6264, female 6872) in Jeonbug province, Korea for about 2 years from May 5, to Dec.19, 1978 and from Mar. 5, to Dec. 22, 1979. The results are as follows: 1. The prevalence rate of all active pulmonary tuberculosis is 5.3%. 2. The prevalence rate of male(8.8%) is 4.2 times higher than that of female (2.1%). 3. The prevalence rates of all old age groups above fifty years are higher than that of remainder under fifty years of age. Of these, 61-70 years old age group is highest in prevalence rate. 4. According to extent of active pulmonary tuberculosis, 77.5% is minimal, 15.4% is moderately advanced, and 7.1% is far advanced. Inactive pulmonary tuberculosis are 39 cases (0.3% to objective population). 5. In the incidence of the involved side, right side is about 2 times higher than the left, and involvement of both sides is increased in moderately and far advance pulmonary tuberculosis. In all pulmonary tuberculosis, one or both upper lung fields are most commonly involved. The incidences of cavity in moderately and far advanced pulmonary tuberculosis show no significant difference. 6. The incidences of other intrathoracic lesions are as follows: suspected hypertensive heart disease 2.4%, dextrocardia with situs inversus 0.04%, pleural calcification 0.4%, pleural thickening 0.2%, pleural effusion 0.1%, pneumonia 0.02%, bronchiectasis 0.1%, lung abscess 0.02%, C.O.P.D. 0.2%, suspected lung tumor 0.06%, pneumothorax 0.0076%, and suspected mediastinal tumor 0.02%.

  10. Twenty-five-year outbreak of Pseudomonas aeruginosa infecting individuals with cystic fibrosis: identification of the prairie epidemic strain.

    Science.gov (United States)

    Parkins, Michael D; Glezerson, Bryan A; Sibley, Christopher D; Sibley, Kristen A; Duong, Jessica; Purighalla, Swathi; Mody, Christopher H; Workentine, Matthew L; Storey, Douglas G; Surette, Michael G; Rabin, Harvey R

    2014-04-01

    Transmissible strains of Pseudomonas aeruginosa have been described for cystic fibrosis (CF) and may be associated with a worse prognosis. Using a comprehensive strain biobank spanning 3 decades, we sought to determine the prevalence and stability of chronic P. aeruginosa infection in an adult population. P. aeruginosa isolates from sputum samples collected at initial enrollment in our adult clinic and at the most recent clinic visit were examined by a combination of pulsed-field gel electrophoresis and multilocus sequence typing and compared against a collection of established transmissible and local non-CF bronchiectasis (nCFB) isolates. A total of 372 isolates from 107 patients, spanning 674 patient-years, including 66 patients with matched isolates from initial and final encounters, were screened. A novel clone with increased antibacterial resistance, termed the prairie epidemic strain (PES), was found in 29% (31/107 patients) of chronically infected patients referred from multiple prairie-based CF centers. This isolate was not found in those diagnosed with CF as adults or in a control population with nCFB. While 90% (60/66 patients) of patients had stable infection over a mean of 10.8 years, five patients experienced strain displacement of unique isolates, with PES occurring within 2 years of transitioning to adult care. PES has been present in our cohort since at least 1987, is unique to CF, generally establishes chronic infection during childhood, and has been found in patients at the time of transition of patients from multiple prairie-based CF clinics, suggesting broad endemicity. Studies are under way to evaluate the clinical implications of PES infection.

  11. Practical management of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Kishaba, Tomoo

    2015-07-22

    Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease.

  12. Inspiratory and expiratory HRCT findings in Behcet's disease and correlation with pulmonary function tests

    Energy Technology Data Exchange (ETDEWEB)

    Oezer, Caner [Department of Radiology, Mersin University, Faculty of Medicine, Mersin (Turkey)]. E-mail: cozer@mersin.edu.tr; Duce, Meltem Nass [Department of Radiology, Mersin University, Faculty of Medicine, Mersin (Turkey); Ulubas, Bahar [Department of Respiratory Disease, Mersin University, Faculty of Medicine, Mersin (Turkey); Bicer, Ali [Department of Physical Medicine and Rehabilitation, Mersin University, Faculty of Medicine, Mersin (Turkey); Tuersen, Uemit [Department of Dermatology, Mersin University, Faculty of Medicine, Mersin (Turkey); Apaydin, F. Demir [Department of Radiology, Mersin University, Faculty of Medicine, Mersin (Turkey); Yildiz, Altan [Department of Radiology, Mersin University, Faculty of Medicine, Mersin (Turkey); Camdeviren, Handan [Department of Biostatistics, Mersin University, Faculty of Medicine, Mersin (Turkey)

    2005-10-01

    Purpose: The purpose of our study was to describe the pulmonary parenchymal changes of Behcet's disease using high-resolution computed tomography and to correlate them with pulmonary function tests. Materials and methods: Thirty-four patients with Behcet's disease (18 men, 16 women), 3 of whom were symptomatic, were included as the study group. Four of 34 patients were smokers. Twenty asymptomatic volunteers (12 men, 8 women), 4 of whom were smokers, constituted the control group. The pulmonary function tests and high-resolution computed tomography were performed for both groups. Results: Inspiratory high-resolution computed tomography findings were abnormal in nine patients (26.5%) of the study group. In eight patients, there were multiple abnormalities, whereas one patient had only one abnormality. Pleural thickening and irregularities, major fissure thickening, emphysematous changes, bronchiectasis, parenchymal bands, and irregular densities, and parenchymal nodules were the encountered abnormalities. Inspiratory high-resolution computed tomography scans were normal in the control group. On expiratory scans, there was statistically significant difference between study group and control group when air trapping, especially grades 3 and 4, was compared (P < 0.01). Pulmonary function tests of both the study and the control groups were in normal ranges, and there was no statistically significant difference between the two groups according to pulmonary function tests (P > 0.05). Discussion and conclusion: High-resolution computed tomography is sensitive in the demonstration of pulmonary changes in patients with Behcet's disease. End-expiratory high-resolution computed tomography examination is very useful and necessary to show the presence of air trapping, thus the presence of small airway disease, even if the patient is asymptomatic or has normal pulmonary function tests.

  13. Mycobacterium abscessus glycopeptidolipid prevents respiratory epithelial TLR2 signaling as measured by HβD2 gene expression and IL-8 release.

    Directory of Open Access Journals (Sweden)

    Lisa B Davidson

    Full Text Available Mycobacterium abscessus has emerged as an important cause of lung infection, particularly in patients with bronchiectasis. Innate immune responses must be highly effective at preventing infection with M. abscessus because it is a ubiquitous environmental saprophyte and normal hosts are not commonly infected. M. abscessus exists as either a glycopeptidolipid (GPL expressing variant (smooth phenotype in which GPL masks underlying bioactive cell wall lipids, or as a variant lacking GPL which is immunostimulatory and invasive in macrophage infection models. Respiratory epithelium has been increasingly recognized as playing an important role in the innate immune response to pulmonary pathogens. Respiratory epithelial cells express toll-like receptors (TLRs which mediate the innate immune response to pulmonary pathogens. Both interleukin-8 (IL-8 and human β-defensin 2 (HβD2 are expressed by respiratory epithelial cells in response to toll-like receptor 2 (TLR2 receptor stimulation. In this study, we demonstrate that respiratory epithelial cells respond to M. abscessus variants lacking GPL with expression of IL-8 and HβD2. Furthermore, we demonstrate that this interaction is mediated through TLR2. Conversely, M. abscessus expressing GPL does not stimulate expression of IL-8 or HβD2 by respiratory epithelial cells which is consistent with "masking" of underlying bioactive cell wall lipids by GPL. Because GPL-expressing smooth variants are the predominant phenotype existing in the environment, this provides an explanation whereby initial M. abscessus colonization of abnormal lung airways escapes detection by the innate immune system.

  14. Profile of a Brazilian population with severe chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Yaksic Mateo Sainz

    2003-01-01

    Full Text Available Chronic obstructive pulmonary disease (COPD is a public health problem. Tobacco smoking is the major cause, but not the only one. Air pollution, exposure to chemicals, environmental smoke exposure, and passive smoking are among other contributing causes; being viral and bacterial infections also risk factors. Gender and weight are associated to the severity of the disease. Co-morbidity is frequent. OBJECTIVE: To characterize a population of COPD outpatients followed at an outsourced medical service. METHODS: Questionnaires were applied to patients with COPD. The data included gender, age, weight, body mass index (BMI, oxygen delivery users, and FEV1, exposure to tobacco smoke, exposure to wood smoke, history of tuberculosis and co-morbid diseases. RESULTS: Of the 70 patients enrolled in the study, 70% (49 were men with an average age of 64 ± 10 years, average weight of 63 ± 16 kg and average BMI of 22 ± 5 kg/m². Mean FEV1 was 35 ± 14% and 45.7% were oxygen dependent. Nine (12.8% patients never smoked, while 78.8% had quit tobacco smoking, (38 ± 11 pack/years was the average. Nine (12.8% smoked corn husk cigarettes. Eighteen (25.7% were exposed to wood smoke. Eleven (15.7% patients had tuberculosis, 5.7% complained of asthma symptoms, 2.8% had bronchiectasis, 11.4% diabetes mellitus, 51.4% hypertension, and 20% Cor pulmonale. CONCLUSION: Other possible COPD etiologies must be investigated. Determinants of the pulmonary injury could be environmental smoke exposure associated to former infections. Men with low BMI are typically representative of this severe patient population. Hypertension and Cor Pulmonale are frequent co-morbidity factors.

  15. A PROSPECTIVE STUDY OF ELECTROCARDIOGRAPHIC CHANGES IN COPD

    Directory of Open Access Journals (Sweden)

    Jimnaz

    2014-05-01

    Full Text Available OBJECTIVES OF STUDY: To study the electrocardiographic findings in Chronic obstructive pulmonary disease. To correlate these findings with duration, severity of the disease and pulmonary function test. MATERIALS AND METHODS: This study consists of 50 patients selected randomly from the cases admitted in the medical wards with symptoms suggestive of airway obstruction of more than 2 years duration, and in whom clinical diagnosis of chronic obstructive pulmonary disease was made. All these patients were subjected to Spirometric tests, the values of forced expiratory volume in first second (FEV1 less than 80% of the expected value, which does not alter significantly after bronchodilator inhalation (<200ml were included in this study. The cases like Bronchial asthma, Pulmonary tuberculosis, Bronchiectasis, Cor pulmonale in failure, Cardiac illness, hypertension, and diabetes mellitus were excluded from the study. RESULT: 50 patients of chronic obstructive pulmonary disease were studied Majority of patient had moderate airflow obstruction. The commonest ECG changes were P wave axis ≥+900, QRS axis ≥ + 90and P wave height in L2 ≥ 2.5mm. R wave in V6 < 5 mm and R/S ratio in V5 V6 ≤1 were seen less commonly. Unifocal right ventricular ectopics and RBBB were seen rarely. CONCLUSION: E.C.G. changes correlate significantly with low value of FEV1/FVC ratio. The commonest ECG changes were P wave axis ≥+900, QRS axis ≥ + 90 and P wave height in L2 ≥2.5mm. R wave in V6 <5 mm and R/S ratio in V5 V6 ≤1 were seen less commonly. Unifocal right ventricular ectopics and RBBB were seen rarely.

  16. Idiopathic non-specific interstitial pneumonia.

    Science.gov (United States)

    Belloli, Elizabeth A; Beckford, Rosemarie; Hadley, Ryan; Flaherty, Kevin R

    2016-02-01

    Non-specific interstitial pneumonia (NSIP) is an interstitial lung disease that may be idiopathic or secondary to connective tissue disease, toxins or numerous other causes. Idiopathic NSIP is a rare diagnosis and requires exclusion of these other possible causes. Patients typically present in mid-adulthood with dyspnoea, cough and often constitutional symptoms including fever and fatigue. The disease has a female predominance, and more than 50% of patients have never smoked. Physical exam features mild hypoxaemia and inspiratory rales. Pulmonary function tests demonstrate restriction and a low diffusing capacity for carbon monoxide. High-resolution computed tomography abnormalities include predominantly lower lobe subpleural reticular changes, traction bronchiectasis and ground-glass opacities; honeycombing is rarely seen. An evaluation of the underlying pathology is necessary for a firm diagnosis. Histologically, alveolar and interstitial mononuclear cell inflammation and fibrosis are seen in a temporally uniform pattern with preserved underlying alveolar architecture. NSIP must be differentiated from other parenchymal lung diseases including idiopathic pulmonary fibrosis and hypersensitivity pneumonitis. A thorough exposure history and assessment for underlying connective tissue diseases are highly important, as positive findings in these categories would likely denote a case of secondary NSIP. A multi-disciplinary discussion that includes pulmonologist(s), radiologist(s) and pathologist(s) assists in reaching a consensus diagnosis and improves diagnostic accuracy. Treatment of idiopathic NSIP, although not well proven, is generally instituted in the form of immunosuppression. Prognosis is favourable compared with idiopathic pulmonary fibrosis, although the diagnosis still carries an attributable mortality. Herein we will summarize the clinical characteristics and management of idiopathic NSIP.

  17. Combined anti CXC receptors 1 and 2 therapy is a promising anti-inflammatory treatment for respiratory diseases by reducing neutrophil migration and activation.

    Science.gov (United States)

    Planagumà, A; Domènech, T; Pont, M; Calama, E; García-González, V; López, R; Aulí, M; López, M; Fonquerna, S; Ramos, I; de Alba, J; Nueda, A; Prats, N; Segarra, V; Miralpeix, M; Lehner, M D

    2015-10-01

    Neutrophil infiltration and activation in the lung are important pathophysiological features in COPD, severe asthma and bronchiectasis mostly mediated by CXCL8 and CXCL1 via CXCR1 and CXCR2. No thorough study to date has been performed to compare the anti-inflammatory effect profile of dual CXCR1/2 vs. selective CXCR2 antagonists in relevant human neutrophil assays and pulmonary inflammation models. Dual CXCR1/2 (SCH527123, diaminocyclobutandione-1) and selective CXCR2 (SB265610, thiopyrimidine-1) antagonist activity and receptor residence time were determined by [(35)S]GTPγS binding in human (h)- and guinea pig (gp)-CXCR1 and CXCR2 overexpressing membranes. h-neutrophil chemotaxis, degranulation and ROS production were established using CXCL8 or CXCL1 to evaluate dual CXCR1/2- or selective CXCR2-dependent activities. LPS-induced lung inflammation in gp was selected to assess in vivo potency. Dual CXCR1/2 antagonists blocked both CXCL8 and CXCL1-induced h-neutrophil functions and [(35)S]GTPγS binding. In contrary, selective CXCR2 antagonists displayed significantly reduced potency in CXCL8 -mediated h-neutrophil responses despite being active in CXCR2 assays. Upon LPS challenge in gp, administration of SCH527123 inhibited the increase of neutrophils in BALF, modestly reduced blood neutrophils and induced minor neutrophil accumulation in bone marrow. Differentiation of CXCR1/2 vs. CXCR2 antagonists could not be extended to in vivo due to differences in CXCR1 receptor homology between h and gp. Dual CXCR1/2 therapy may represent a promising anti-inflammatory treatment for respiratory diseases reducing more effectively neutrophil migration and activation in the lung than a CXCR2 selective treatment. However, the in vivo confirmation of this claim is still missing due to species differences in CXCR1.

  18. Accurate measurement of respiratory airway wall thickness in CT images using a signal restoration technique

    Science.gov (United States)

    Park, Sang Joon; Kim, Tae Jung; Kim, Kwang Gi; Lee, Sang Ho; Goo, Jin Mo; Kim, Jong Hyo

    2008-03-01

    Airway wall thickness (AWT) is an important bio-marker for evaluation of pulmonary diseases such as chronic bronchitis, bronchiectasis. While an image-based analysis of the airway tree can provide precise and valuable airway size information, quantitative measurement of AWT in Multidetector-Row Computed Tomography (MDCT) images involves various sources of error and uncertainty. So we have developed an accurate AWT measurement technique for small airways with three-dimensional (3-D) approach. To evaluate performance of these techniques, we used a set of acryl tube phantom was made to mimic small airways to have three different sizes of wall diameter (4.20, 1.79, 1.24 mm) and wall thickness (1.84, 1.22, 0.67 mm). The phantom was imaged with MDCT using standard reconstruction kernel (Sensation 16, Siemens, Erlangen). The pixel size was 0.488 mm × 0.488 mm × 0.75 mm in x, y, and z direction respectively. The images were magnified in 5 times using cubic B-spline interpolation, and line profiles were obtained for each tube. To recover faithful line profile from the blurred images, the line profiles were deconvolved with a point spread kernel of the MDCT which was estimated using the ideal tube profile and image line profile. The inner diameter, outer diameter, and wall thickness of each tube were obtained with full-width-half-maximum (FWHM) method for the line profiles before and after deconvolution processing. Results show that significant improvement was achieved over the conventional FWHM method in the measurement of AWT.

  19. Diagnosis and treatment of male infertility associated with immotile ciliasyndrome: a report of 6 cases

    Institute of Scientific and Technical Information of China (English)

    Fei Qian-jin; Huang Xue-feng; Ye Bi-lu

    2011-01-01

    Objective:To investigate the clinical characteristics,diagnosis and treatment of male infertility associated with immotile cilia syndrome (ICS).Methods:The clinical data of six cases of male infertility associated with ICS were reviewed retrospectively.Results:The clinical features in all cases included chronic or recurrent infections of the upper and lower airways and male infertility.Sinusitis,bronchitis and bronchiectasis were found in all cases and situs inversus totalis found in two cases.Sex hormone levels,chromosome karyotype and Y chromosome microdeletion (AZF) in all cases were normal.All cases were diagnosed as severe asthenospermia by routine semen analysis.Five cases had no motile spermatozoa in semen,while there were less than 0.2% of motile sperm in one case.The total sperm count and sperm viability were normal in four cases and there were very few immotile sperm in two cases.Transmission electron microscopic examination of sperm flagellum revealed disarrangement or a partial absence of 9 + 2 microtubules and/or an absence of the dynein arms in six cases.The bronchial cilia in one case showed to be devoid of inner dynein arms.Five cases underwent six intracytoplasmic sperm injection (ICSI) cycles and the rates of fertilization,embryo cleavage and good quality embryos were 50.0%,69.2% and 55.6%,respectively.Two clinical pregnancies and one chemical pregnancy were achieved,with one birth of a healthy baby boy.Conclusions:The ultrastructural defect of cilia or flagellum is the most important diagnostic criteria of ICS.ICSI is an effective treatment for male infertility associated with ICS.

  20. Application of Irwin diagnostic procedures for chronic cough

    Directory of Open Access Journals (Sweden)

    Guo-fang FENG

    2011-07-01

    Full Text Available Objective To explore the advantages and disadvantages of the diagnostic procedures suggested by the Irwin group,and summarize the experiences in diagnosis and treatment,and to beter understand the etiology,diagnosis,differential diagnosis and treatment of chronic cough.Methods Data of 118 patients,who were finally diagnosed as chronic cough according to the diagnostic procedures suggested by Irwin group in the First Affiliated Hospital of General Hospital of PLA in 2009,were retrospectivety analyzed.Results With the Irwin diagnostic procedures of chronic cough,118 patients were diagnosed definitely.The final diagnostic rate reached 100%.The duration to reach the final diagnosis was 1 day to 30 days.Of the 118 patients with chronic cough,113 were cured(96%.32 cases(27.1% were caused by asthma and related diseases(allergic rhinitis,allergic pharyngitis,cough variant asthma and eosinophilic bronchitis;28 cases(23.7% were due to upper airway cough syndrome and related diseases(chronic rhinitis,sinusitis,pharyngeal bursitis,postnasal drip syndrome,chronic laryngitis,and vocal cord polyps;23 cases(19.5% due to gastroesophageal reflux disease;14 cases(11.9% due to lower respiratory tract infection and related diseases(endobronchial tuberculosis,pulmonary tuberculosis,endotracheal mucosal adenocarcinoma,lung cancer,bronchiectasis,and pulmonary fibrosis;10 cases(8.5% due to cardiac insafficiency;6 cases(5.1% due to administration of angiotensin-converting enzyme inhibitor(ACEI-like antihypertensive agents;3 cases(2.5% were psychogenic cough,and 2 cases(1.7% were induced by other causes.Conclusions The Irwin diagnosis of chronic cough is a comprehensive and thorough procedure,and it should be used with delibcration in clinic.The etiology of chronic cough is complicated,mainly including asthma and related diseases,sinusitis and upper airway cough syndrome,and gastroesophageal reflux disease.

  1. A novel CFTR mutation found in a Chinese patient with cystic fibrosis

    Institute of Scientific and Technical Information of China (English)

    2006-01-01

    Background Cystic fibrosis (CF) is rare in Chinese. We investigated the mutations in the gene of cystic fibrosis transmembrane conductance regulator (CFTR) in a Chinese CF patient and reviewed the clinical features, gene mutations in Chinese CF cases. Methods Blood samples were collected from a previously reported CF girl and her parents. The 24 coding exons of CFTR of the proband were amplified and sequenced. Results A Chinese girl of 16 years old was diagnosed as CF at the age of 14. She had recurrent productive cough with bronchiectasis in bilateral upper lobes, parasinusitis and otitis media, but without pancreatic involvement. Her sweat chloride was (108.9 ±3.3) mmol/L. A heterozygous novel missense mutation of 699 C→A which results in the amino acid change of N189K was identified in exon 5. In addition, a heterozygous 3821-3823 delT mutation in exon 19 was found in CFTR. The mutation 699C→A was inherited from her father, and the 3821-3823delT mutation was from her mother. Twenty patients with CF in Chinese reported from 1974 to 2004 were also reviewed. DelF508 mutation was not found in the nine cases whose CFTR mutations were analyzed. Conclusions The CF proband carries two heterozygous mutations (699C→A and 3821-3823delT) in CFTR. 699C→A mutation is a novel mutation which is not reported previously. Review of reported Chinese cases suggests that the genotype of Chinese CF may be different from those of white cases. More studies are needed to understand the spectra of CFTR and clinical CF features in Chinese.

  2. Simple image-based no-wash method for quantitative detection of surface expressed CFTR.

    Science.gov (United States)

    Larsen, Mads Breum; Hu, Jennifer; Frizzell, Raymond A; Watkins, Simon C

    2016-03-01

    Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasians. It is caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene, which encodes an apical membrane anion channel that is required for regulating the volume and composition of epithelial secretions. The most common CFTR mutation, present on at least one allele in >90% of CF patients, deletes phenylalanine at position 508 (F508del), which causes the protein to misfold. Endoplasmic reticulum (ER) quality control elicits the degradation of mutant CFTR, compromising its trafficking to the epithelial cell apical membrane. The absence of functional CFTR leads to depletion of airway surface liquid, impaired clearance of mucus and bacteria from the lung, and predisposes to recurrent infections. Ultimately, respiratory failure results from inflammation and bronchiectasis. Although high throughput screening has identified small molecules that can restore the anion transport function of F508del CFTR, they correct less than 15% of WT CFTR activity, yielding insufficient clinical benefit. To date, most primary CF drug discovery assays have employed measurements of CFTR's anion transport function, a method that depends on the recruitment of a functional CFTR to the cell surface, involves multiple wash steps, and relies on a signal that saturates rapidly. Screening efforts have also included assays for detection of extracellularly HA-tagged or HRP-tagged CFTR, which require multiple washing steps. We have recently developed tools and cell lines that report the correction of mutant CFTR trafficking by currently available small molecules, and have extended this assay to the 96-well format. This new and simple no-wash assay of F508del CFTR at the cell surface may permit the discovery of more efficacious drugs, and hopefully thereby prevent the catastrophic effects of this disease. In addition, the modular design of this platform should make it useful for other diseases where loss

  3. Interstitial lung disease in anti-synthetase syndrome: Initial and follow-up CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Debray, Marie-Pierre, E-mail: marie-pierre.debray@bch.aphp.fr [AP-HP, Bichat-Claude Bernard Hospital, Department of Radiology, 46, rue Henri Huchard, 75877 Paris Cedex 18 (France); Borie, Raphael, E-mail: raphael.borie@bch.aphp.fr [AP-HP, Bichat-Claude Bernard Hospital, Department of Pneumology A and Centre de Compétence Maladies Pulmonaires rares, DHU Fire 46, rue Henri Huchard, 75877 Paris Cedex 18 (France); Inserm, U1152, Paris (France); Revel, Marie-Pierre, E-mail: marie-pierre.revel@htd.aphp.fr [AP-HP, Cochin Hospital, Department of Radiology, 27, Rue du Fg Saint Jacques, 75679 Paris Cedex 14 (France); Naccache, Jean-Marc, E-mail: jean-marc.naccache@tnn.aphp.fr [AP-HP, Avicenne Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, Bobigny (France); AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris (France); Khalil, Antoine, E-mail: antoine.khalil@tnn.aphp.fr [AP-HP, Tenon Hospital, Department of Radiology, 4, rue de la Chine, 75020 Paris (France); Toper, Cécile, E-mail: cecile.toper@gmail.com [AP-HP, Tenon Hospital, Department of Pneumology and Centre de Compétence Maladies Pulmonaires rares, 4, rue de la Chine, 75020 Paris (France); Israel-Biet, Dominique, E-mail: dominique.israel-biet@egp.aphp.fr [Université Paris Descartes and AP-HP, Department of Pneumology, Georges Pompidou European Hospital, 20, rue Leblanc, 75015 Paris (France); and others

    2015-03-15

    Purpose: To describe the initial and follow-up CT features of interstitial lung disease associated with anti-synthetase syndrome (AS-ILD). Materials and methods: Two independent thoracic radiologists retrospectively analysed thin-section CT images obtained at diagnosis of AS-ILD in 33 patients (17 positive for anti-Jo1, 13 for anti-PL12, and three for anti-PL7 antibodies). They evaluated the pattern, distribution and extent of the CT abnormalities. They also evaluated the change in findings during follow-up (median 27 months; range 13–167 months) in 26 patients. Results: At diagnosis, ground-glass opacities (100%), reticulations (87%) and traction bronchiectasis (76%) were the most common CT findings. Consolidations were present in 45% of patients. A non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP) or mixed NSIP-OP CT pattern were observed in 15 out of 33 (45%), seven out of 33 (21%) and eight out of 33 (24%) patients, respectively, whereas the CT pattern was indeterminate in three patients. During follow-up, consolidations decreased or disappeared in 11 out of 12 patients (92%), among which seven within the first 6 months, but honeycombing progressed or appeared in ten out of 26 patients (38%) and overall disease extent increased in nine out of 26 patients (35%). Conclusion: CT features at diagnosis of AS-ILD mainly suggest NSIP and OP, isolated or in combination. Consolidations decrease or disappear in most cases but the disease may progress to fibrosis in more than one third of patients.

  4. 初治活动性继发性肺结核的HRCT影像研究%The high-resolution CT imaging of active secondary pulmonary tuberculosis in the primary therapy

    Institute of Scientific and Technical Information of China (English)

    吕岩; 李成海; 谢汝明; 王岳; 周震; 宁锋钢; 周新华; 贺伟

    2015-01-01

    Objective To study the high-resolution CT imaging feature of active secondary pulmonary tuberculosis in the primary therapy.Methods High-resolution CT imaging of 323 cases with active secondary pulmonary tuberculosis in the primary therapy in Beijing Chest Hospital, Capital Medical University were collected, and the lesion distribution and morphological characteristics were observed, respectively. The imaging of high-resolution CT in pulmonary tuberculosis of negative and positive sputum for tuberculous bacterium were compared, the difference of count data in two groups were analyzed by Chi-square test and Logistic regression.Results Among the 323 cases, the lesions of 86 (26.6%) case located in single lung lobe, the lesions of 237 (73.4%) case located in more lung lobe and more segment, mostly in S1, S2 and S6. High-resolution CT manifestation: centrilobular nodles, there were alveolar nodules in 282 case and "tree-in-bud" sign in 193 case, 45 cases with "reversed halo sign" among them, reversed halos with nodular walls and nodules inside the halos were observed. There were 293 (90.7%) lobular and subsegmental consolidation, 115 (35.6%) segmental comsolidation, 22 (6.8%) lobe consolidation , of which, the CT ifndings showed small patchy low-density areas in the lesions, and with bronchiectasis and cavity in part lesions. There were 37 spherical or mass lesions, > 3 cm, the contrast-enhanced CT scanning showed that ring enhancement and uneven enhancement appeared small patchy low-density areas and boundaries were vague. There were 123 interstitial abnormalities, intralobular, include