Bronchiolitis obliterans organising pneumonia syndrome presenting with neutrophilia in bronchoalveolar lavage fluid after breast-conserving therapy

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Chiba, Sahoko; Jinta, Torahiko; Chohnabayashi, Naohiko; Fujie, Toshihide; Sumi, Yuki; Inase, Naohiko

2012-01-01

A 61-year-old female presented with a dry cough and fever 4 months after tangential radiation therapy (RT) following conserving surgery for breast cancer. Chest radiography and CT demonstrated consolidation with air bronchogram outside the irradiated area. Neutrophil granulocytes were abundant in bronchoalveolar lavage fluid (BALF) (39.6% of total cells), and transbronchial lung biopsy revealed organising pneumonia (OP) histologically. Antibiotic therapy had no effect, but corticosteroid therapy brought about clinical improvement. Her condition was diagnosed as bronchiolitis obliterans OP (BOOP) syndrome. Lymphocytic BALF has been identified as a characteristic of BOOP syndrome induced after RT for breast cancer. The BALF in this case, however, was neutrophilic. In our analysis of differential cell counts in the BALF of 24 patients with BOOP syndrome, the BALF was neutrophilic (>5%) in 16 (76%) cases, and the neutrophilia was severe in some of those patients. PMID:22605699

Acute cellular rejection is a risk factor for bronchiolitis obliterans syndrome independent of post-transplant baseline FEV1

DEFF Research Database (Denmark)

Burton, C.M.; Iversen, M.; Carlsen, J.

2009-01-01

BACKGROUND: Post-transplant baseline forced expiratory volume in 1 second (FEV(1)) constitutes a systematic bias in analyses of bronchiolitis obliterans syndrome (BOS). This retrospective study evaluates risk factors for BOS adjusting for the confounding of post-transplant baseline FEV(1). METHODS......-specific hazard of BOS (hazard ratio 1.4, confidence interval 1.1 to 1.8, p = 0.009). The absolute value of baseline FEV(1) was a significant confounder in all survival and competing risk analyses of BOS (p ... an independent risk factor for the development of BOS after adjusting for the confounding of post-transplant baseline FEV(1) Udgivelsesdato: 2009/9...

Rituximab-Induced Bronchiolitis Obliterans Organizing Pneumonia

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Ahmet B. Ergin

2012-01-01

Full Text Available Rituximab-induced lung disease (R-ILD is a rare entity that should be considered in patients treated with rituximab who present with dyspnea, fever, and cough, but no clear evidence of infection. A variety of pathologic findings have been described in this setting. Bronchiolitis obliterans organizing pneumonia (BOOP is the most common clinicopathologic diagnosis, followed by interstitial pneumonitis, acute respiratory distress syndrome (ARDS, and hypersensitivity pneumonitis. Prompt diagnosis and treatment with corticosteroids are essential as discussed by Wagner et al. (2007. Here we present a case of an 82-year-old man who was treated with rituximab for recurrent marginal zone lymphoma. After the first infusion of rituximab, he reported fever, chills, and dyspnea. On computed tomography imaging, he was found to have bilateral patchy infiltrates, consistent with BOOP on biopsy. In our patient, BOOP was caused by single-agent rituximab, in the first week after the first infusion of rituximab. We reviewed the relevant literature to clarify the different presentations and characteristics of R-ILD and raise awareness of this relatively overlooked entity.

Bronchiolitis obliterans syndrome after allogeneic hematopoietic SCT: phenotypes and prognosis.

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Bergeron, A; Godet, C; Chevret, S; Lorillon, G; Peffault de Latour, R; de Revel, T; Robin, M; Ribaud, P; Socié, G; Tazi, A

2013-06-01

Bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic SCT (HSCT) is recognized as a new-onset obstructive lung defect (OLD) in pulmonary function testing and is related to pulmonary chronic GVHD. Little is known about the different phenotypes of patients with BOS and their outcomes. We reviewed the data of all allogeneic HSCT recipients referred to our pulmonary department for a non-infectious bronchial disease between 1999 and 2010. We identified 103 patients (BOS (n=77), asthma (n=11) and chronic bronchitis (n=15)). In patients with BOS, we identified two functional phenotypes: a typical OLD, that is, forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio <0.7 (n=53), and an atypical OLD with a concomitant decrease in the FEV1 <80% and FVC <80% predicted with a normal total lung capacity (n=24). The typical OLD was characterized by more severe FEV1 and fewer centrilobular nodules on the computed tomography scan. The FEV1 was not significantly affected during the follow-up, regardless of the phenotype. In addition to acute and extensive chronic GVHD, only the occurrence of BOS soon after transplantation and the intentional treatment of BOS with steroids were associated with a poor survival. The determination of patient subgroups should be explored to improve the management of this condition.

Quinine-induced bronchiolitis obliterans and organizing pneumonia

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Masoodur Rahman

2012-01-01

Full Text Available Quinine is a vital anti-malarial drug used in the management of resistant Falciparum malaria. There are previous reports of quinine-induced pulmonary edema and infiltrates. We report the first case of biopsy-proven bronchiolitis obliterans organizing pneumonia (BOOP, confirmed by the Naranjo Adverse Drug Reaction Probability Scale (NADRS of 5 and a severity scale of 5, secondary to intravenous quinine, in a 15-year-old girl with Plasmodium falciparum infection after a visit to Kenya. Clinical course of the patient followed by review of the literature and appropriate medical interventions for quinine-induced BOOP are suggested.

Diffuse micronodular pattern of bronchiolitis obliterans organizing pneumonia: a case report

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Lee, In Jae; Jang, Seung Hun; Min, Kwang Seon; Whang, Im Kyung; Lee, Yul; Bae, Sang Hoon [Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang (Korea, Republic of)

2006-10-15

The typical radiographic findings of bronchiolitis obliterans organizing pneumonia (BOOP) are known to be patchy air-space consolidation that is often subpleural, and with or without ground-glass opacities. However, there are scant radiologic reports about the micronodular pattern of BOOP. We report here on a case of BOOP that manifested as diffusely scattered ill-defined centrilobular micronodules on HRCT.

Bronchiolitis obliterans after allo-SCT: clinical criteria and treatment options

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Uhlving, H H; Buchvald, F; Heilmann, C J

2012-01-01

Bronchiolitis obliterans (BO) following allogeneic haematopoietic SCT (HSCT) is a serious complication affecting 1.7-26% of the patients, with a reported mortality rate of 21-100%. It is considered a manifestation of chronic graft-versus-host disease, but our knowledge of aetiology and pathogenes...... treatment options for BO and presents the treatment results with HDPM in a paediatric national HSCT-cohort.Bone Marrow Transplantation advance online publication, 29 August 2011; doi:10.1038/bmt.2011.161....

Bilateral pulmonary nodules in an adult patient with bronchiolitis obliterans-organising pneumonia.

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Kopanakis, Antonios; Golias, Christos; Pantentalakis, George; Patentalakis, Michalis; Mermigkis, Charalampos; Mermigkis, Christos; Charalabopoulos, Alexandros; Peschos, Dimitrios; Batistatou, Anna; Charalabopoulos, Konstantinos

2009-01-01

A 58-year-old male ex-smoker was admitted to hospital because of nodular infiltrates on chest x rays. He was complaining of fatigue, dyspnoea with exertion, low grade fever and weight loss. Physical examination was unremarkable. Bronchoscopy was inconclusive but revealed endobronchial lesions of chronic active inflammation. The diagnosis of cryptogenic organising pneumonitis bronchiolitis obliterans-organising pneumonia (COP-BOOP) was established by open lung biopsy. Proliferative bronchiolitis with regions of organising pneumonia is the characteristic feature of COP. The radiological picture of bilateral pulmonary nodules is an infrequent manifestation of COP. Lung biopsy, open or with video assistance thoracic surgery, is recommended to confirm the diagnosis.

Bronchiolitis obliterans organising pneumonia: a consequence of breast radiotherapy.

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Fahim, Ahmed; Campbell, Anne P; Hart, Simon Paul

2012-01-18

The authors describe a case of 51-year-old woman who presented with breathlessness following radiotherapy for breast carcinoma. A chest radiograph and thoracic CT scan revealed extensive airspace consolidation affecting right upper and lower lobes. A trans-bronchial biopsy revealed evidence of foamy macrophages and fibroblastic plugs within alveoli, consistent with organising pneumonia. Indirect immunofluorescence microscopy revealed evidence of antiepithelial antibodies. Gradual but complete resolution occurred without any specific treatment. This case highlights the importance of considering radiation induced bronchiolitis obliterans organising pneumonia in the context of parenchymal shadowing following radiotherapy. Although corticosteroids are widely recommended for treatment, this case illustrates that organising pneumonia may resolve spontaneously.

Associação de bronquiolite obliterante pós-infecciosa e hemossiderose pulmonar na infância Postinfectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in childhood

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Leonardo Araújo Pinto

2006-12-01

Full Text Available Descreve-se uma apresentação rara de bronquiolite obliterante associada a hemossiderose pulmonar, em paciente de nove anos com sintomas respiratórios persistentes iniciados após episódio de bronquiolite aguda grave aos sete meses. Após o episódio agudo, apresentou sintomas respiratórios persistentes, piorando significativamente aos sete anos, quando começou a apresentar dificuldade respiratória em pequenos esforços. A tomografia computadorizada de tórax demonstrou achados compatíveis com bronquiolite obliterante. A biópsia pulmonar a céu aberto demonstrou numerosos macrófagos corados com hemossiderina, além dos achados compatíveis com bronquiolite obliterante. O diagnóstico de hemossiderose pulmonar pode estar ocasionalmente associado a bronquiolite obliterante em crianças com seqüela pós-viral grave.In the present report, we describe an unusual presentation of post-infectious bronchiolitis obliterans accompanied by pulmonary hemosiderosis in a nine-year-old boy with persistent respiratory symptoms subsequent to an episode of acute bronchiolitis occurring at the age of seven months. After the episode, the persistent respiratory symptoms worsened significantly, and, by the age of seven, the patient began to have difficulty breathing after minimal exertion. Computed tomography of the chest presented findings consistent with bronchiolitis obliterans. Open lung biopsy revealed numerous hemosiderin-laden macrophages, as well as other findings consistent with bronchiolitis obliterans. Pulmonary hemosiderosis can occasionally be accompanied by bronchiolitis obliterans in children with severe sequelae after an episode of viral infection.

Radiological findings in seven patients of bronchiolitis obliterans with organized pneumonia

International Nuclear Information System (INIS)

Serrano Gotarredona, M.P.; Fernandez-Cruz, J.; Jimenez-Ruiz, M.

1995-01-01

We present seven patients, five women and two men, between 40 and 71 years old, who were diagnosed between 1983 and 1993 as having bronchiolitis obliterans with organized pneumonia (BOOP). Five of the patients were treated at Hospital Virgen del Rocio and two at Hospital Nuestra Se=A4ora de Valme. The histological, clinical and radiological features are described. All the patients responded favorable to corticosteroid therapy, and there have been no recurrences. We point out the predominance of patchy alveolar infiltrates which, in three cases proved to be migratory. We analyze the computerized tomography (CT) findings in three patients, comparing our observations with those reported in the literature. (Author)

Development of a Multivariate Prediction Model for Early-Onset Bronchiolitis Obliterans Syndrome and Restrictive Allograft Syndrome in Lung Transplantation

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Angela Koutsokera

2017-07-01

Full Text Available BackgroundChronic lung allograft dysfunction and its main phenotypes, bronchiolitis obliterans syndrome (BOS and restrictive allograft syndrome (RAS, are major causes of mortality after lung transplantation (LT. RAS and early-onset BOS, developing within 3 years after LT, are associated with particularly inferior clinical outcomes. Prediction models for early-onset BOS and RAS have not been previously described.MethodsLT recipients of the French and Swiss transplant cohorts were eligible for inclusion in the SysCLAD cohort if they were alive with at least 2 years of follow-up but less than 3 years, or if they died or were retransplanted at any time less than 3 years. These patients were assessed for early-onset BOS, RAS, or stable allograft function by an adjudication committee. Baseline characteristics, data on surgery, immunosuppression, and year-1 follow-up were collected. Prediction models for BOS and RAS were developed using multivariate logistic regression and multivariate multinomial analysis.ResultsAmong patients fulfilling the eligibility criteria, we identified 149 stable, 51 BOS, and 30 RAS subjects. The best prediction model for early-onset BOS and RAS included the underlying diagnosis, induction treatment, immunosuppression, and year-1 class II donor-specific antibodies (DSAs. Within this model, class II DSAs were associated with BOS and RAS, whereas pre-LT diagnoses of interstitial lung disease and chronic obstructive pulmonary disease were associated with RAS.ConclusionAlthough these findings need further validation, results indicate that specific baseline and year-1 parameters may serve as predictors of BOS or RAS by 3 years post-LT. Their identification may allow intervention or guide risk stratification, aiming for an individualized patient management approach.

Protection against bronchiolitis obliterans syndrome is associated with allograft CCR7+ CD45RA- T regulatory cells.

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Aric L Gregson

2010-06-01

Full Text Available Bronchiolitis obliterans syndrome (BOS is the major obstacle to long-term survival after lung transplantation, yet markers for early detection and intervention are currently lacking. Given the role of regulatory T cells (Treg in modulation of immunity, we hypothesized that frequencies of Treg in bronchoalveolar lavage fluid (BALF after lung transplantation would predict subsequent development of BOS. Seventy BALF specimens obtained from 47 lung transplant recipients were analyzed for Treg lymphocyte subsets by flow cytometry, in parallel with ELISA measurements of chemokines. Allograft biopsy tissue was stained for chemokines of interest. Treg were essentially all CD45RA(-, and total Treg frequency did not correlate to BOS outcome. The majority of Treg were CCR4(+ and CD103(- and neither of these subsets correlated to risk for BOS. In contrast, higher percentages of CCR7(+ Treg correlated to reduced risk of BOS. Additionally, the CCR7 ligand CCL21 correlated with CCR7(+ Treg frequency and inversely with BOS. Higher frequencies of CCR7(+ CD3(+CD4(+CD25(hiFoxp3(+CD45RA(- lymphocytes in lung allografts is associated with protection against subsequent development of BOS, suggesting that this subset of putative Treg may down-modulate alloimmunity. CCL21 may be pivotal for the recruitment of this distinct subset to the lung allograft and thereby decrease the risk for chronic rejection.

Chest health surveillance utility in the early detection of bronchiolitis obliterans syndrome in children after allo-SCT.

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Gassas, A; Craig-Barnes, H; Dell, S; Doyle, J; Schechter, T; Sung, L; Egeler, M; Palaniyar, N

2013-06-01

To prospectively assess whether periodic chest health surveillance is beneficial for the early detection of bronchiolitis obliterans syndrome (BOS) in children after allo-SCT. Children up to 18 years of age receiving allo-SCT from September 2009 to September 2011 were included. Surveillance consisted of the following: a 7-item respiratory system questionnaire of cough, wheeze and shortness of breath; focused physical examination; and pulmonary function test (PFT) conducted before SCT and at 1, 3, 6, 9, 12, 18 and 24 months after SCT. Thirty-nine patients were enrolled. Five children developed BOS at a median time of 192 days (range 94-282). Positive response comparisons between the BOS group vs the non-BOS group were NS for history questionnaire (P=0.2), heart rate (P=0.3), respiratory rate (P=0.3) and oxygen saturation monitoring (P=0.8). Differences between the two groups for chest auscultation and PFT were statistically significant (P=0.03 and P=0.01, respectively). However, chest auscultation in the BOS group was only positive after BOS diagnosis. PFT reduction was evident in the asymptomatic phase (BOS group 33%; non-BOS group 4.5%, P=0.01). Changes in PFT, but not history/physical examination, allow the early detection of BOS in children after SCT. Our study is limited by the small sample size.

Oxygen-sensitive 3He-MRI in bronchiolitis obliterans after lung transplantation

International Nuclear Information System (INIS)

Gast, Klaus K.; Biedermann, Alexander; Herweling, Annette; Schreiber, Wolfgang G.; Schmiedeskamp, Joerg; Mayer, Eckhard; Heussel, Claus P.; Markstaller, Klaus; Eberle, Balthasar; Kauczor, Hans-Ulrich

2008-01-01

Oxygen-sensitive 3 He-MRI was studied for the detection of differences in intrapulmonary oxygen partial pressure (pO 2 ) between patients with normal lung transplants and those with bronchiolitis obliterans syndrome (BOS). Using software developed in-house, oxygen-sensitive 3 He-MRI datasets from patients with normal lung grafts (n = 8) and with BOS (n = 6) were evaluated quantitatively. Datasets were acquired on a 1.5-T system using a spoiled gradient echo pulse sequence. Underlying diseases were pulmonary emphysema (n 10 datasets) and fibrosis (n = 4). BOS status was verified by pulmonary function tests. Additionally, 3 He-MRI was assessed blindedly for ventilation defects. Median intrapulmonary pO 2 in patients with normal lung grafts was 146 mbar compared with 108 mbar in patients with BOS. Homogeneity of pO2 distribution was greater in normal grafts (standard deviation pO2 34 versus 43 mbar). Median oxygen decrease rate during breath hold was higher in unaffected patients (-1.75 mbar/s versus -0.38 mbar/s). Normal grafts showed fewer ventilation defects (5% versus 28%, medians). Oxygen-sensitive 3 He-MRI appears capable of demonstrating differences of intrapulmonary pO2 between normal lung grafts and grafts affected by BOS. Oxygen-sensitive 3 He-MRI may add helpful regional information to other diagnostic techniques for the assessment and follow-up of lung transplant recipients. (orig.)

Development of ELISA-detected anti-HLA antibodies precedes the development of bronchiolitis obliterans syndrome and correlates with progressive decline in pulmonary function after lung transplantation.

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Jaramillo, A; Smith, M A; Phelan, D; Sundaresan, S; Trulock, E P; Lynch, J P; Cooper, J D; Patterson, G A; Mohanakumar, T

1999-04-27

Development of anti-HLA antibodies after lung transplantation (LT) is thought to play an important role in the etiology of bronchiolitis obliterans syndrome (BOS). However, a cause-effect relationship between anti-HLA antibodies and BOS has not been established. This study was conducted to determine the temporal relationship between the development of anti-HLA antibodies and BOS after LT, and to determine the antigenic specificity of the antibodies developed in BOS patients. Sera from 15 BOS+ LT patients and 12 BOS- LT patients were obtained before LT and collected again at 6, 12, 24, 36, and 48 months after LT. Anti-HLA antibodies were detected by the PRA-STAT ELISA system and by complement-dependent cytotoxicity assays. Anti-HLA reactivity was further characterized by flow cytometry and absorption/elution with human platelets. When analyzed by ELISA, 10 of 15 BOS+ patients developed anti-HLA antibodies, whereas 0 of 12 BOS- patients developed anti-HLA antibodies (PELISA after LT can provide an early identification of an important subset of LT patients with an increased risk of developing BOS.

Interaction between Pseudomonas and CXC Chemokines Increases Risk of Bronchiolitis Obliterans Syndrome and Death in Lung Transplantation

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Wang, Xiaoyan; Weigt, S. Sam; Palchevskiy, Vyacheslav; Lynch, Joseph P.; Ross, David J.; Kubak, Bernard M.; Saggar, Rajan; Fishbein, Michael C.; Ardehali, Abbas; Li, Gang; Elashoff, Robert; Belperio, John A.

2013-01-01

Rationale: Pseudomonas aeruginosa is the most commonly isolated gram-negative bacterium after lung transplantation and has been shown to up-regulate glutamic acid–leucine–arginine–positive (ELR+) CXC chemokines associated with bronchiolitis obliterans syndrome (BOS), but the effect of pseudomonas on BOS and death has not been well defined. Objectives: To determine if the influence of pseudomonas isolation and ELR+ CXC chemokines on the subsequent development of BOS and the occurrence of death is time dependent. Methods: A three-state model was developed to assess the likelihood of transitioning from lung transplant (state 1) to BOS (state 2), from transplant (state 1) to death (state 3), and from BOS (state 2) to death (state 3). This Cox semi-Markovian approach determines state survival rates and cause-specific hazards for movement from one state to another. Measurements and Main Results: The likelihood of transition from transplant to BOS was increased by acute rejection, CXCL5, and the interaction between pseudomonas and CXCL1. The pseudomonas effect in this transition was due to infection rather than colonization. Movement from transplant to death was facilitated by pseudomonas infection and single lung transplant. Transition from BOS to death was affected by the length of time in state 1 and by the interactions between any pseudomonas isolation and CXCL5 and aspergillus, either independently or in combination. Conclusions: Our model demonstrates that common post-transplantation events drive movement from one post-transplantation state to another and influence outcomes differently depending upon when after transplantation they occur. Pseudomonas and the ELR+ CXC chemokines may interact to negatively influence lung transplant outcomes. PMID:23328531

Early Pathologic Findings of Bronchiolitis Obliterans after Allogeneic Hematopoietic Stem Cell Transplantation: A Proposal from a Case

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Rie Nakamoto-Matsubara

2012-01-01

Full Text Available Bronchiolitis obliterans (BO is one of the serious, noninfectious pulmonary complications after allogeneic hematopoietic stem cell transplantation (allo-HSCT. Early diagnosis of BO is usually difficult because patients are often asymptomatic at an initial stage of the disease and pathologic findings are available mostly at the late stages. Therefore, the diagnosis of the disease is based on the pulmonary function test using the National Institute of Health consensus criteria. Here, we report a case of slowly progressive BO. A biopsy specimen at an early stage demonstrated alveolar destruction with lymphocyte infiltration in bronchial walls and mild narrowing of bronchioles without fibrosis, those were strongly indicative of initial pathologic changes of BO. Definitive BO followed, which was proven by both clinical course and autopsy. While alloreactive lymphocytes associated with chronic graft-versus-host disease are believed to initiate BO, we present a rare case that directly implies such a scenario.

Bronchiolitis from nitrous fumes

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Darke, C S; Warrack, A J.N.

1958-01-01

Gases of low solubility (e.g., NO/sub 2/) may not irritate upper respiratory tract but may act insidiously on lower tract producing edema and bronchiolitis. Cases of bronchiolitis (1 resulting in death) from exposure to brown nitrous fumes are reported. There were no or slight symptoms at first but severe ones after about 1 wk. Pathology appears as acute pulmonary edema with damage and desquamation of alveolar epithelium particularly and that of respiratory bronchioles somewhat. Patchy atelectasis, bronchopneumonia and disruption of fibers in cell walls may also occur. Severe cases have obliteration of lumen (bronchiolitis obliterans) with extreme distress and death.

Pathology, toxicology, and latency of irritant gases known to cause bronchiolitis obliterans disease: Does diacetyl fit the pattern?

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Brent D. Kerger

2015-01-01

Full Text Available Bronchiolitis obliterans (BO is a rare disease involving concentric bronchiolar fibrosis that develops rapidly following inhalation of certain irritant gases at sufficiently high acute doses. While there are many potential causes of bronchiolar lesions involved in a variety of chronic lung diseases, failure to clearly define the clinical features and pathological characteristics can lead to ambiguous diagnoses. Irritant gases known to cause BO follow a similar pathologic process and time course of disease onset in humans. Studies of inhaled irritant gases known to cause BO (e.g., chlorine, hydrochloric acid, ammonia, nitrogen oxides, sulfur oxides, sulfur or nitrogen mustards, and phosgene indicate that the time course between causal chemical exposures and development of clinically significant BO disease is typically limited to a few months. The mechanism of toxic action exerted by these irritant gases generally involves widespread and severe injury of the epithelial lining of the bronchioles that leads to acute respiratory symptoms which can include lung edema within days. Repeated exposures to inhaled irritant gases at concentrations insufficient to cause marked respiratory distress or edema may lead to adaptive responses that can reduce or prevent severe bronchiolar fibrotic changes. Risk of BO from irritant gases is driven substantially by toxicokinetics affecting concentrations occurring at the bronchiolar epithelium. Highly soluble irritant gases that cause BO like ammonia generally follow a threshold-dependent cytotoxic mechanism of action that at sufficiently high doses results in severe inflammation of the upper respiratory tract and the bronchiolar epithelium concurrently. This is followed by acute respiratory distress, pulmonary edema, and post inflammatory concentric fibrosis that become clinically obvious within a few months. In contrast, irritant gases with lower solubility like phosgene also follow a threshold-dependent mechanism

Machine Learning Algorithms Utilizing Quantitative CT Features May Predict Eventual Onset of Bronchiolitis Obliterans Syndrome After Lung Transplantation.

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Barbosa, Eduardo J Mortani; Lanclus, Maarten; Vos, Wim; Van Holsbeke, Cedric; De Backer, William; De Backer, Jan; Lee, James

2018-02-19

Long-term survival after lung transplantation (LTx) is limited by bronchiolitis obliterans syndrome (BOS), defined as a sustained decline in forced expiratory volume in the first second (FEV 1 ) not explained by other causes. We assessed whether machine learning (ML) utilizing quantitative computed tomography (qCT) metrics can predict eventual development of BOS. Paired inspiratory-expiratory CT scans of 71 patients who underwent LTx were analyzed retrospectively (BOS [n = 41] versus non-BOS [n = 30]), using at least two different time points. The BOS cohort experienced a reduction in FEV 1 of >10% compared to baseline FEV 1 post LTx. Multifactor analysis correlated declining FEV 1 with qCT features linked to acute inflammation or BOS onset. Student t test and ML were applied on baseline qCT features to identify lung transplant patients at baseline that eventually developed BOS. The FEV 1 decline in the BOS cohort correlated with an increase in the lung volume (P = .027) and in the central airway volume at functional residual capacity (P = .018), not observed in non-BOS patients, whereas the non-BOS cohort experienced a decrease in the central airway volume at total lung capacity with declining FEV 1 (P = .039). Twenty-three baseline qCT parameters could significantly distinguish between non-BOS patients and eventual BOS developers (P machine), we could identify BOS developers at baseline with an accuracy of 85%, using only three qCT parameters. ML utilizing qCT could discern distinct mechanisms driving FEV 1 decline in BOS and non-BOS LTx patients and predict eventual onset of BOS. This approach may become useful to optimize management of LTx patients. Copyright © 2018 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.

Aspiration, Localized Pulmonary Inflammation, and Predictors of Early-Onset Bronchiolitis Obliterans Syndrome after Lung Transplantation

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Fisichella, P Marco; Davis, Christopher S; Lowery, Erin; Ramirez, Luis; Gamelli, Richard L; Kovacs, Elizabeth J

2014-01-01

BACKGROUND We hypothesized that immune mediator concentrations in the bronchoalveolar fluid (BALF) are predictive of bronchiolitis obliterans syndrome (BOS) and demonstrate specific patterns of dysregulation, depending on the presence of acute cellular rejection, BOS, aspiration, and timing of lung transplantation. STUDY DESIGN We prospectively collected 257 BALF samples from 105 lung transplant recipients. The BALF samples were assessed for absolute and differential white blood cell counts and 34 proteins implicated in pulmonary immunity, inflammation, fibrosis, and aspiration. RESULTS There were elevated BALF concentrations of interleukin (IL)-15, IL-17, basic fibroblast growth factor, tumor necrosis factor–α, and myeloperoxidase, and reduced concentrations of α1-antitrypsin, which were predictive of early-onset BOS. Patients with BOS had an increased percentage of BALF lymphocytes and neutrophils, with a reduced percentage of macrophages (p < 0.05). The BALF concentrations of IL-1β; IL-8; interferon-γ–induced protein 10; regulated upon activation, normal T-cell expressed and secreted; neutrophil elastase; and pepsin were higher in patients with BOS (p < 0.05). Among those with BOS, BALF concentrations of IL-1RA; IL-8; eotaxin; interferon-γ–induced protein 10; regulated upon activation, normal T-cell expressed and secreted; myeloperoxidase; and neutrophil elastase were positively correlated with time since transplantation (p < 0.01). Those with worse grades of acute cellular rejection had an increased percentage of lymphocytes in their BALF (p < 0.0001) and reduced BALF concentrations of IL-1β, IL-7, IL-9, IL-12, granulocyte colony-stimulating factor, granulocyte-macrophage colony-stimulating factor, interferon-γ, and vascular endothelial growth factor (p ≤ 0.001). Patients with aspiration based on detectable pepsin had increased percentage of neutrophils (p < 0.001) and reduced BALF concentrations of IL-12 (p < 0.001). CONCLUSIONS The BALF levels

Post-infectious bronchiolitis obliterans: clinical, radiological and pulmonary function sequelae

International Nuclear Information System (INIS)

Chang, A.B.; Masel, J.P.; Masters, B.

1998-01-01

Background. There are few data on clinical, chest radiograph (CXR) or pulmonary function sequelae in children with post-infectious bronchiolitis obliterans (BO) (pulmonary crepitations, abnormalities on CXR, CT, nuclear medicine scans, or bronchography, with a history of past pulmonary infection and in the absence of other underlying pathology). Objective. To analyse the methodology of diagnosis, long-term clinical imaging and pulmonary function sequelae of post-infectious BO in children. Materials and methods. Imaging (CXRs, CT and nuclear lung scans) and clinical histories of 19 children were analysed. Results. Clinical follow-up (mean 6.8 years), revealed a high incidence of continuing problems (asthma and bronchiectasis). Fixed airway obstruction was the most common pulmonary function sequela. The sequelae on follow-up (mean 5.8 years) CXR were classified into five patterns which are illustrated: unilateral hyperlucency of an enlarged lung/part of lung; complete collapse of the affected lobe; unilateral hyperlucency of a small or normal-sized lung; bilateral hyperlucent lungs and a mixed pattern of persistent collapse, hyperlucency and peribronchial thickening. Conclusion. Long-term observations in children with post-infectious BO should be undertaken to detect bronchiectasis and obstructive airway disease. Sequelae evident on CXR, other than those previously described, can be found. Bronchography and/or lung biopsy are not usually required for the diagnosis of post-infectious BO. (orig.)

Sauropus androgynus (L. Merr. Induced Bronchiolitis Obliterans: From Botanical Studies to Toxicology

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Hamidun Bunawan

2015-01-01

Full Text Available Sauropus androgynus L. Merr. is one of the most popular herbs in South Asia, Southeast Asia, and China where it was known as a slimming agent until two outbreaks of pulmonary dysfunction were reported in Taiwan and Japan in 1995 and 2005, respectively. Several studies described that the excessive consumption of Sauropus androgynus could cause drowsiness, constipation, and bronchiolitis obliterans and may lead to respiratory failure. Interestingly, this herb has been used in Malaysia and Indonesia in cooking and is commonly called the “multigreen” or “multivitamin” plant due to its high nutritive value and inexpensive source of dietary protein. The plant is widely used in traditional medicine for wound healing, inducing lactation, relief of urinary disorders, as an antidiabetic cure and also fever reduction. Besides these medicinal uses, the plant can also be used as colouring agent in food. This review will explore and compile the fragmented knowledge available on the botany, ethnobotany, chemical constitutes, pharmacological properties, and toxicological aspects of this plant. This comprehensive review will give readers the fundamental, comprehensive, and current knowledge regarding Sauropus androgynus L. Merr.

Long Term Follow-Up of Sulfur Mustard Related Bronchiolitis Obliterans Treatment

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Hamidreza Abtahi

2016-10-01

Full Text Available Bronchiolitis obliterans (BO is the most remarkable pulmonary sequels of war-related sulfur mustard inhalation. There is little if any data about long-term efficacy of associated BO treatment. Five years spirometric records of three groups of patients with obstructive pulmonary diseases (asthma, COPD, BO and documented sulfur mustard inhalation were evaluated. The BO patients were treated with inhaled Seretide 125-250/25 (2 puffs BID, azithromycin (250 mg, three times/week and N-acetylcysteine (1200-1800/day. Asthma and COPD patients were treated according to existing guidelines. Seventy-three (38 asthma, 16 COPD and 19 BO patients completed the 5 years follow-up. Basal and final FEV1 in BO patients (2.69±0.81 and 2.39±0.65 respectively were not significantly different from COPD patients (2.46±0.56 and 1.96±0.76 respectively. There was also no significant difference between the yearly FEV1 decline in BO patients compared to COPD patients (60±84 cc vs. 99±79 cc respectively, P=0.163. The non-significant difference of FEV1 decline in BO compared to COPD patients suggests the effectiveness of azithromycin, inhaled steroid and N-acetyl cysteine in BO patients. Considering safety and possible effectiveness, this treatment is recommended until more data is available from controlled clinical studies.

Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh

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Alsaghir, Abdullah H.; Al-Mobeireek, Abdulla F.; Al-Jahdali, H.; Al-Etihan, A.; Al-Otair, H.; Al-Dayel, F.

2007-01-01

Because reports of bronchiolitis obliterans organizing pneumonia (BOOP) are lacking from the Middle East, we conducted a retrospective review of all histopathologically proven cases of BOOP over 10-year period at three tertiary care hospitals in Riyadh and describe the clinical features and outcome. Charts at the three hospitals were searched using a specific code for BOOP or cryptogenic organizing pneumonia (COP). Lung specimens have to show histological proof of BOOP with a compatible clinical picture. Chest radiographs and high resolution CT scans were reviewed. Twenty cases of biopsy-proven BOOP had well documented clinical and radiograph data. There were 11 males and 9 females (mean age 58 years; range 42-78). The clinical presentation f BOOP was acute or subacute pneumonia-like illness with cough (85%), fever (70%) dyspnea, (85%) and crackles (80%). The most frequent radiological pattern was a bilateral alveolar infiltrate. The most common abnormality on pulmonary function testing (n=14) was a restrictive pattern (11 patients). Most patients (70%) had no underlying cause (idiopathic BOOP). Other associations included thyroid cancer, rheumatoid arthritis, syphilis and Wegner's granulomatosis. Ten patients (50%) had a complete response to steroids, 6 (30%) had a partial response and 3 (15.8) with secondary BOOP had rapid progressive respiratory failure and died. The clinical presentation of BOOP in our patients is similar to other reported series. A favorable outcome occurs in the majority of cases. However, BOOP may occasionally be associated with a poor prognosis, particularly when associated with an underlying disease. (author)

Differential role of the Fas/Fas ligand apoptotic pathway in inflammation and lung fibrosis associated with reovirus 1/L-induced bronchiolitis obliterans organizing pneumonia and acute respiratory distress syndrome.

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Lopez, Andrea D; Avasarala, Sreedevi; Grewal, Suman; Murali, Anuradha K; London, Lucille

2009-12-15

Bronchiolitis obliterans organizing pneumonia (BOOP) and acute respiratory distress syndrome (ARDS) are two clinically and histologically distinct syndromes sharing the presence of an inflammatory and fibrotic component. Apoptosis via the Fas/Fas ligand (FasL) pathway plays an important role in the development of acute lung injury and fibrosis characteristic of these and other pulmonary inflammatory and fibrotic syndromes. We evaluated the role of apoptosis via the Fas/FasL pathway in the development of pulmonary inflammation and fibrosis in reovirus 1/L-induced BOOP and ARDS. CBA/J mice were intranasally inoculated with saline, 1 x 10(6) (BOOP), or 1 x 10(7) (ARDS) PFU reovirus 1/L, and evaluated at various days postinoculation for in situ apoptosis by TUNEL analysis and Fas/FasL expression. Our results demonstrate the presence of apoptotic cells and up-regulation of Fas/FasL expression in alveolar epithelium and in infiltrating cells during the inflammatory and fibrotic stages of both reovirus 1/L-induced ARDS and BOOP. Treatment of mice with the caspase 8 inhibitor, zIETD-fmk, inhibited apoptosis, inflammation, and fibrotic lesion development in reovirus 1/L-induced BOOP and ARDS. However, CBA/KlJms-Fas(lpr-cg)/J mice, which carry a point mutation in the Fas cytoplasmic region that abolishes the ability of Fas to transduce an apoptotic signal, do not develop pulmonary inflammation and fibrotic lesions associated with reovirus 1/L-induced BOOP, but still develop inflammation and fibrotic lesions associated with reovirus 1/L-induced ARDS. These results suggest a differential role for the Fas/FasL apoptotic pathway in the development of inflammation and fibrotic lesions associated with BOOP and ARDS.

Bronchiolitis obliterans syndrome after single-lung transplantation: impact of time to onset on functional pattern and survival.

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Brugière, Olivier; Pessione, Fabienne; Thabut, Gabriel; Mal, Hervé; Jebrak, Gilles; Lesèche, Guy; Fournier, Michel

2002-06-01

Among risk factors for the progression of bronchiolitis obliterans syndrome (BOS) after lung transplantation (LT), the influence of time to BOS onset is not known. The aim of the study was to assess if BOS occurring earlier after LT is associated with worse functional prognosis and worse graft survival. We retrospectively compared functional outcome and survival of all single-LT (SLT) recipients who had BOS develop during follow-up in our center according to time to onset of BOS ( or = 3 years after transplantation). Among the 29 SLT recipients with BOS identified during the study period, 20 patients had early-onset BOS and 9 patients had late-onset BOS. The mean decline of FEV(1) over time during the first 9 months in patients with early-onset BOS was significantly greater than in patients with of late-onset BOS (p = 0.04). At last follow-up, patients with early-onset BOS had a lower mean FEV(1) value (25% vs 39% of predicted, p = 0.004), a lower mean PaO(2) value (54 mm Hg vs 73 mm Hg, p = 0.0005), a lower 6-min walk test distance (241 m vs 414 m, p = 0.001), a higher Medical Research Council index value (3.6 vs 1.6, p = 0.0001), and a higher percentage of oxygen dependency (90% vs 11%, p = 0.001) compared with patients with late-onset BOS. In addition, graft survival of patients with early-onset BOS was significantly lower than that of patients with late-onset BOS (log-rank test, p = 0.04). There were 18 of 20 graft failures (90%) in the early-onset BOS group, directly attributable to BOS in all cases (deaths [n = 10] or retransplantation [n = 8]). In the late-onset BOS group, graft failure occurred in four of nine patients due to death from extrapulmonary causes in three of four cases. The median duration of follow-up after occurrence of BOS was not statistically different between patients with early-onset BOS and patients with late-onset BOS (31 +/- 28 months and 37 +/- 26 months, respectively; p = not significant). The subgroup of patients who had BOS develop

Bronchiolitis obliterans after allogenic bone marrow transplantation: HRCT findings

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Jung, Jung Im; Jung, Won Sang; Hahn, Seong Tai; Park, Seog Hee; Min, Chang Ki; Kim, Chun Choo

2004-01-01

To evaluate the high resolution computed tomography (HRCT) findings of bronchiolitis obliterans (BO) after bone marrow transplantation (BMT). During the past three years, 11 patients were diagnosed as having BO after BMT when they developed irreversible air flow obstruction, with an FEV 1 value of less than 80% of the baseline value, without any clinical evidence of infection. All 11 patients underwent HRCT, of whom eight also underwent follow-up HRCT. The HRCT images were assessed retrospectively for the presence of decreased lung attenuation, segmental or subsegmental bronchial dilatation, diminution of peripheral vascularity, centrilobular nodules, and branching linear structure on the inspiratory images. The lobar distribution of the decreased lung attenuation and bronchial dilatation was also examined. The presence of air trapping was investigated on the expiratory images. The interval changes of the HRCT findings were evaluated in those patients who had follow-up images. Abnormal HRCT findings were present in all cases; the most common abnormalities were decreased lung attenuation (n=11), subsegmental bronchial dilatation (n=6), diminution of peripheral vascularity (n=6), centrilobular nodules or branching linear structure (n=3), and segmental bronchial dilatation (n=3). Expiratory air trapping was noted in all patients. The decreased lung attenuation and bronchial dilatations were more frequent or extensive in the lower lobes. Interval changes were found in all patients with follow-up HRCT: increased extent of decreased lung attenuation (n=7); newly developed or progressed bronchial dilatation (n=4); and increased lung volume (n=3). HRCT scans are abnormal in patients with BO, with the most commonly observed finding being areas of decreased lung attenuation. While the HRCT findings are not specific, it is believed that their common features can assist in the diagnosis of BO in BMT recipients

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

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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.

Differential Role of the Fas/Fas Ligand Apoptotic Pathway in Inflammation and Lung Fibrosis Associated with Reovirus 1/L-Induced Bronchiolitis Obliterans Organizing Pneumonia and Acute Respiratory Distress Syndrome1

Science.gov (United States)

Lopez, Andrea D.; Avasarala, Sreedevi; Grewal, Suman; Murali, Anuradha K.; London, Lucille

2010-01-01

Bronchiolitis obliterans organizing pneumonia (BOOP) and acute respiratory distress syndrome (ARDS) are two clinically and histologically distinct syndromes sharing the presence of an inflammatory and fibrotic component. Apoptosis via the Fas/Fas ligand (FasL) pathway plays an important role in the development of acute lung injury and fibrosis characteristic of these and other pulmonary inflammatory and fibrotic syndromes. We evaluated the role of apoptosis via the Fas/FasL pathway in the development of pulmonary inflammation and fibrosis in reovirus 1/L-induced BOOP and ARDS. CBA/J mice were intranasally inoculated with saline, 1 × 106 (BOOP), or 1 × 107 (ARDS) PFU reovirus 1/L, and evaluated at various days postinoculation for in situ apoptosis by TUNEL analysis and Fas/FasL expression. Our results demonstrate the presence of apoptotic cells and up-regulation of Fas/FasL expression in alveolar epithelium and in infiltrating cells during the inflammatory and fibrotic stages of both reovirus 1/L-induced ARDS and BOOP. Treatment of mice with the caspase 8 inhibitor, zIETD-fmk, inhibited apoptosis, inflammation, and fibrotic lesion development in reovirus 1/L-induced BOOP and ARDS. However, CBA/KlJms-Faslpr-cg/J mice, which carry a point mutation in the Fas cytoplasmic region that abolishes the ability of Fas to transduce an apoptotic signal, do not develop pulmonary inflammation and fibrotic lesions associated with reovirus 1/L-induced BOOP, but still develop inflammation and fibrotic lesions associated with reovirus 1/L-induced ARDS. These results suggest a differential role for the Fas/FasL apoptotic pathway in the development of inflammation and fibrotic lesions associated with BOOP and ARDS. PMID:20007588

Utility of the inspiratory phase in high-resolution computed tomography evaluations of pediatric patients with bronchiolitis obliterans after allogeneic bone marrow transplant: reducing patient radiation exposure

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Togni Filho, Paulo Henrique; Casagrande, Joao Luiz Marin; Lederman, Henrique Manoel, E-mail: paulotognifilho@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Medicina. Dept. of Diagnostico por Imagem; Universidade de Sao Paulo (InRad/HC/FMUSP), Sao Paulo, SP (Brazil). Hospital das Clinicas. Instituto de Radiologia

2017-03-15

Objective: To evaluate the utility of the inspiratory phase in high-resolution computed tomography (HRCT) of the chest for the diagnosis of post-bone marrow transplantation bronchiolitis obliterans. Materials and Methods: This was a retrospective, observational, cross-sectional study. We selected patients of either gender who underwent bone marrow transplantation and chest HRCT between March 1, 2002 and December 12, 2014. Ages ranged from 3 months to 20.7 years. We included all examinations in which the HRCT was performed appropriately. The examinations were read by two radiologists, one with extensive experience in pediatric radiology and another in the third year of residency, who determined the presence or absence of the following imaging features: air trapping, bronchiectasis, alveolar opacities, nodules, and atelectasis. Results: A total of 222 examinations were evaluated (mean, 5.4 ± 4.5 examinations per patient). The expiratory phase findings were comparable to those obtained in the inspiratory phase, except in one patient, in whom a small uncharacteristic nodule was identified only in the inspiratory phase. Air trapping was identified in a larger number of scans in the expiratory phase than in the inspiratory phase, as was atelectasis, although the difference was statistically significant only for air trapping. Conclusion: In children being evaluated for post-bone marrow transplantation bronchiolitis obliterans, the inspiratory phase can be excluded from the chest HRCT protocol, thus reducing by half the radiation exposure in this population. (author)

Utility of the inspiratory phase in high-resolution computed tomography evaluations of pediatric patients with bronchiolitis obliterans after allogeneic bone marrow transplant: reducing patient radiation exposure

International Nuclear Information System (INIS)

Togni Filho, Paulo Henrique; Casagrande, Joao Luiz Marin; Lederman, Henrique Manoel; Universidade de Sao Paulo

2017-01-01

Objective: To evaluate the utility of the inspiratory phase in high-resolution computed tomography (HRCT) of the chest for the diagnosis of post-bone marrow transplantation bronchiolitis obliterans. Materials and Methods: This was a retrospective, observational, cross-sectional study. We selected patients of either gender who underwent bone marrow transplantation and chest HRCT between March 1, 2002 and December 12, 2014. Ages ranged from 3 months to 20.7 years. We included all examinations in which the HRCT was performed appropriately. The examinations were read by two radiologists, one with extensive experience in pediatric radiology and another in the third year of residency, who determined the presence or absence of the following imaging features: air trapping, bronchiectasis, alveolar opacities, nodules, and atelectasis. Results: A total of 222 examinations were evaluated (mean, 5.4 ± 4.5 examinations per patient). The expiratory phase findings were comparable to those obtained in the inspiratory phase, except in one patient, in whom a small uncharacteristic nodule was identified only in the inspiratory phase. Air trapping was identified in a larger number of scans in the expiratory phase than in the inspiratory phase, as was atelectasis, although the difference was statistically significant only for air trapping. Conclusion: In children being evaluated for post-bone marrow transplantation bronchiolitis obliterans, the inspiratory phase can be excluded from the chest HRCT protocol, thus reducing by half the radiation exposure in this population. (author)

Clinical study of bronchiolitis obliterans organizing pneumonia (BOOP) primed by radiation to the breast cancer

International Nuclear Information System (INIS)

Ozawa, Yuichi; Ida, Masaki; Fujii, Masato; Kato, Masato; Hasegawa, Hirotsugu; Nogimura, Hiroshi; Suda, Takafumi; Enomoto, Noriyuki; Chida, Kingo

2005-01-01

Bronchiolitis obliterans organizing pneumonia (BOOP) primed by radiation therapy for the breast cancer (BOOP-RT/BC) has been recognized recently. In this study, we reported 9 such cases and discussed steroid therapy. Mean radiation dose and interval from completion of radiation therapy to the appearance of BOOP-RT/BC were 47.3 Gy and 5.6 months respectively. BOOP-RT/BC recurrence was observed by 5 episodes in 3 of 9 cases (recurrence rate was 33%). Steroid therapy showed a remarkable improvement in both primary and recurrent disease, and no residual symptom or BOOP shadow was detectable at the end of the observation except for shadows due to radiation fibrosis. All but one patient were prescribed prednisolone, with a mean initial dose of 32.5 mg/day and a mean treatment duration of 46.7 weeks. One patient improved without treatment. No case suffered from respiratory failure which needed mechanical ventilation or was motile. Considering these findings and the fact that almost all patients are middle aged or senior women who are susceptible to osteoporosis, we conclude that steroid therapy for BOOP-RT/BC should be carefully considered. (author)

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

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Mihara, Naoki; Johkoh, Takeshi; Ichikado, Kazuya

2000-01-01

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)

Ventilation/perfusion scintigraphy in children with post-infectious bronchiolitis obliterans: a pilot study.

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Bo-Qia Xie

Full Text Available PURPOSE: Childhood post-infectious bronchiolitis obliterans (BO is an infrequent lung disease leading to narrowing and/or complete obliteration of small airways. Ventilation and perfusion (V/Q scan can provide both regional and global pulmonary information. However, only few retrospective researches investigating post-infectious BO involved V/Q scan, the clinical value of this method is unknown. This preliminary prospective study was aimed to evaluate the correlation of V/Q scan with disease severity, pulmonary function test results, and prognosis in children with post-infectious BO. METHODS: Twenty-five post-infectious BO children (18 boys and 7 girls; mean age, 41 months underwent V/Q scan and pulmonary function tests. Patients were followed after their inclusion. Ventilation index and perfusion index obtained from V/Q scan were used to measure pulmonary abnormalities. Spearman's rank correlation test of ventilation index and perfusion index on disease severity, lung function tests indices, and follow-up results were performed. RESULTS: The median follow-up period was 4.6 years (range, 2.2 to 5.0 years. Ventilation index and perfusion index were both correlated with disease severity (r = 0.72, p<0.01 and r = 0.73, p<0.01, but only ventilation index was related to pulmonary function tests results (all p<0.05. In addition, Spearman test yielded significant correlations between perfusion index and prognosis (r = 0.77, p<0.01, and ventilation index and prognosis (r = 0.63, p = 0.01. CONCLUSIONS: For children with post-infectious BO, the present study preliminarily indicated that the degree of ventilation and perfusion abnormalities evaluated by V/Q scan may be used to assess disease severity, and may be predictive of patient's outcome.

Quantitative computed tomography assessment of graft-versus-host disease-related bronchiolitis obliterans in children: A pilot feasibility study

International Nuclear Information System (INIS)

Kim, Hyun Gi; Shin, Hyun Joo; Kim, Myung-Joon; Lee, Mi-Jung; Kim, Yoon Hee; Sohn, Myung Hyun; Kim, Kyung Won; Lyu, Chuhl Joo

2015-01-01

To suggest a simple method that can quantify air trapping from chest CT in children with graft-versus-host disease (GVHD)-related bronchiolitis obliterans (BO). This institutional review board-approved retrospective study included eight GVHD-related BO patients (age, 6 - 17 years) who underwent both 31 CTs of variable settings and pulmonary function tests (PFT). The attenuation values of lung parenchyma in normal (An) and air trapping (Aa) areas were obtained. Individualized threshold [(An + Aa)/2] and fixed threshold of -950 HU were set for air trapping quantification. Spearman correlation analysis and generalized linear mixed models were used for statistical analysis. The mean value of individualized threshold was -830.2 ± 48.3 HU. The mean air trapping lung volume percentage with individualized threshold and -950 HU were 45.4 ± 18.9 % and 1.4 ± 1.9 %, respectively. The air trapping lung volume percentage with individualized threshold showed a significant negative correlation with the PFT of FEV1/FVC% in all data (γ = -0.795, P <.001) and in the correction of repetition (γ = -0.837, P =.010). We suggest a simple and individualized threshold attenuation setting method for air trapping quantification insusceptible to CT imaging protocols or respiratory phase control in children with GVHD-related BO. (orig.)

Quantitative computed tomography assessment of graft-versus-host disease-related bronchiolitis obliterans in children: A pilot feasibility study

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Kim, Hyun Gi [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Seoul (Korea, Republic of); Ajou University Medical Center, Department of Radiology, Ajou University School of Medicine, Suwon (Korea, Republic of); Shin, Hyun Joo; Kim, Myung-Joon; Lee, Mi-Jung [Yonsei University College of Medicine, Department of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Seoul (Korea, Republic of); Kim, Yoon Hee; Sohn, Myung Hyun; Kim, Kyung Won [Yonsei University College of Medicine, Department of Pediatrics and Institute of Allergy, Severance Children' s Hospital, Seoul (Korea, Republic of); Lyu, Chuhl Joo [Yonsei University College of Medicine, Department of Pediatric Hematology and Oncology, Severance Children' s Hospital, Seoul (Korea, Republic of)

2015-10-15

To suggest a simple method that can quantify air trapping from chest CT in children with graft-versus-host disease (GVHD)-related bronchiolitis obliterans (BO). This institutional review board-approved retrospective study included eight GVHD-related BO patients (age, 6 - 17 years) who underwent both 31 CTs of variable settings and pulmonary function tests (PFT). The attenuation values of lung parenchyma in normal (An) and air trapping (Aa) areas were obtained. Individualized threshold [(An + Aa)/2] and fixed threshold of -950 HU were set for air trapping quantification. Spearman correlation analysis and generalized linear mixed models were used for statistical analysis. The mean value of individualized threshold was -830.2 ± 48.3 HU. The mean air trapping lung volume percentage with individualized threshold and -950 HU were 45.4 ± 18.9 % and 1.4 ± 1.9 %, respectively. The air trapping lung volume percentage with individualized threshold showed a significant negative correlation with the PFT of FEV1/FVC% in all data (γ = -0.795, P <.001) and in the correction of repetition (γ = -0.837, P =.010). We suggest a simple and individualized threshold attenuation setting method for air trapping quantification insusceptible to CT imaging protocols or respiratory phase control in children with GVHD-related BO. (orig.)

Mac Leod's syndrome

International Nuclear Information System (INIS)

Schad, M.; Danesi, C.; Ricci, R.; Galluzzi, S.; Coviello, G.

1988-01-01

Mac Leod's syndrome is a rarely diagnosed disease; that is why an accurate differential diagnosis is needed by means of radiological imaging. This paper is aimed at discussing the differential diagnosis, with a special emphasis on the pathogenesis of the syndrome. The phenomenon of air trapping in absence of central bronchial lesions is a typical radiographic finding. Chest X-ray is performed in both inspiration and expiration. Posterior oblique tomography at 55 grade centigrade of the effected side is also performed. Diffuse bronchiolitis obliterans in infancy or early childhood ia widely accepted pathogenetic pattern. Pulmonary hypoventilation causes vasoconstriction and underdevelopment of pulmonary vessels, that are reduced in caliber. Differential diagnosis includes all the diseases resulting in pulmonary hyperlucency, i.e. pulmonary and pleural alterations, and skeletal anomalies

Swyer-James-Macleod's syndrome. A case report

International Nuclear Information System (INIS)

Pacheco C, Dario; Ojeda Leon, Paulina; Varo Acosta, Humberto; Salcedo Veles, Patricia; Salazar Juan Carlos

1998-01-01

This is a case report about a 67 years-old female patient with respiratory syndrome of 8 years with cough and dyspnoea. End-inspiration crackles in the pulmonary auscultation were found in left hemi thorax. Chest x-ray in expiration and inspiration showed hyperluscency and air trapping in the same hemi-thorax. Chest high-resolution CT revealed a low sized, oligohemic left lung with cylindrical bronchiectasis. Perfusion scintigraphy 99Tc labeled showed markedly left lung hypo-perfusion. Mild obstructive process was found in pulmonary function test. Lung biopsy of lingula reported bronchiolitis obliterans. Considered all the results that were obtained from clinical, x-ray and histopathology, a diagnosis of Swyer-James-Macleods syndrome was made

Bronchiolitis obliterans organizing pneumonia–Experience of a pulmonology ward

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Ana L. Fonseca

2007-05-01

Full Text Available Bronchiolitis obliterans organizing pneumonia (BOOP is a physiopathologic syndrome associating suggestive clinical and imaging features with histopathologic studies showing buds of connective tissue in the lumen of the distal pulmonary airspace.The aim of the study is a retrospective review of all patients with BOOP diagnosed in the Pulmonology Unit of Coimbra Hospital Centre (CHC between 2000 and 2005.Eleven cases (6 female and 5 male with mean age 54.8 years were diagnosed. Ten patients were non-smokers and one was an ex-smoker. The mean duration of symptoms was 62.1 days with the initial symptoms dyspnea (8, cough (7, fever (5 and weight loss (2. Nine patients had been given multiple antibiotics, crackles were heard in 6, fever was detected in 6 and dyspnea in 5. Chest X-ray showed bilateral alveolar opacities in 6, focal consolidation in 3, multiple bilateral nodular opacities in 1 and linear opacities in 1. Lung function, performed in 7 patients, showed a reduction in the diffusion capacity in 5. BAL was performed in 8, and all revealed an increase in the percentage of lymphocytes, with low CD4/CD8 in 4. Diagnosis was obtained by pulmonary biopsy performed by TBLB (7, VATS (2 and TTLB (1. Systemic corticosteroids were given in 9 patients. Evolution was favourable in 10 and one patient died.The authors emphasise the time symptoms took to develop, the failure of multiple antibiotics, agreement between symptoms and imaging with those published in the literature, the increased lymphocytes in the BAL, the usefulness of TBLB and the good response to corticosteroids. Resumo: A bronquiolite obliterante com pneumonia organizada (BOOP é uma entidade clinicopatológica que associa clínica e imagiologia sugestivas à evidência de cilindros de tecido conjuntivo no lúmen dos espaços aéreos distais.Procedeu-se à análise retrospectiva dos casos de BOOP diagnosticados no Serviço de Pneumologia do Centro Hospitalar de Coimbra

A cross-sectional study of lung function and respiratory symptoms among chemical workers producing diacetyl for food flavourings.

NARCIS (Netherlands)

van Rooy, F.G.; Smit, L.A.; Houba, R.; Zaat, V.A.; Rooijackers, J.M.; Heederik, D.J.J.

2009-01-01

OBJECTIVES: Four diacetyl workers were found to have bronchiolitis obliterans syndrome. Exposures, respiratory symptoms, lung function and exposure-response relationships were investigated. METHODS: 175 workers from a plant producing diacetyl between 1960 and 2003 were investigated. Exposure data

Hepatocyte growth factor enhances the inflammation-alleviating effect of umbilical cord-derived mesenchymal stromal cells in a bronchiolitis obliterans model.

Science.gov (United States)

Cao, Xiao-Pei; Han, Dong-Mei; Zhao, Li; Guo, Zi-Kuan; Xiao, Feng-Jun; Zhang, Yi-Kun; Zhang, Xiao-Yan; Wang, Li-Sheng; Wang, Heng-Xiang; Wang, Hua

2016-03-01

Specific and effective therapy for prevention or reversal of bronchiolitis obliterans (BO) is lacking. In this study, we evaluated the therapeutic effect of hepatocyte growth factor (HGF) gene modified mesenchymal stromal cells (MSCs) on BO. A mouse model of experimental BO was established by subcutaneously transplanting the tracheas from C57BL/6 mice into Balb/C recipients, which were then administered saline, Ad-HGF-modified human umbilical cord-MSCs (MSCs-HGF) or Ad-Null-modified MSCs (MSCs-Null). The therapeutic effects of MSCs-Null and MSCs-HGF were evaluated by using fluorescence-activated cell sorting (FACS) for lymphocyte immunophenotype of spleen, enzyme-linked immunosorbent assay (ELISA) and real-time polymerase chain reaction (rt-PCR) for cytokine expression, and histopathological analysis for the transplanted trachea. The histopathologic recovery of allograft tracheas was improved significantly after MSCs-Null and MSCs-HGF treatment and the beneficial effects were particularly observed in MSCs-HGF-treated mice. Furthermore, the allo-transplantation-induced immunophenotype disorders of the spleen, including regulatory T (Treg), T helper (Th)1, Th2 and Th17, were attenuated in both cell-treated groups. MSCs-HGF treatment reduced expression and secretion of inflammation cytokines interferon-gamma (IFN-γ), and increased expression of anti-inflammatory cytokine interleukin (IL)-4 and IL-10. It also decreased the expression level of the profibrosis factor transforming growth factor (TGF)-β. Treatment of BO with HGF gene modified MSCs results in reduction of local inflammation and promotion in recovery of allograft trachea histopathology. These findings might provide an effective therapeutic strategy for BO. Copyright © 2015 International Society for Cellular Therapy. Published by Elsevier Inc. All rights reserved.

The value of ventilation scintigraphy after single lung transplantation

NARCIS (Netherlands)

Ouwens, JP; van der Bij, W; van der Mark, TW; Piers, DA; Koeter, GH

Background: A decrease in forced expiratory volume in 1 second (FEV1) as a diagnostic criterion for bronchiolitis obliterans syndrome (BOS) after single lung transplantation may be influenced significantly by the presence of the native lung. To quantify and to discriminate between the relative

Regional ventilation/perfusion mismatch pattern in patient with Swyer James (MacLeod′s syndrome

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Sait Sager

2014-01-01

Full Text Available Swyer James (McLeod′s syndrome (SJMS is an uncommon disease, which occurs as a result of childhood bronchiolitis obliterans. Patients may not be diagnosed until later in their life. A 46-year-old man underwent ventilation/perfusion scintigraphy for acute onset of dyspnea. The scan showed markedly diminished ventilation and perfusion unilaterally on the right middle and inferior lobes. However, mismatched ventilation-perfusion pattern was shown on the upper right lobe, which was consistent with pulmonary embolism. Unilaterally matched ventilation/perfusion defect can see in SJMS in lung scintigraphy; however, when pulmoner embolism may accompany, scintigraphy should be carefully examined.

Severe chronic bronchiolitis as the presenting feature of primary Sjögren's syndrome.

Science.gov (United States)

Borie, Raphael; Schneider, Sophie; Debray, Marie-Pierre; Adle-Biasssette, Homa; Danel, Claire; Bergeron, Anne; Mariette, Xavier; Aubier, Michel; Papo, Thomas; Crestani, Bruno

2011-01-01

Sjögren's syndrome is a frequent auto-immune disorder with a pulmonary location in almost 10% of the patients. Although bronchial involvement is very common, most patients only complain of cough and this involvement rarely results in severe symptoms or chronic respiratory failure are rarely observed. We describe here 5 patients with severe chronic bronchiolitis revealing primary Sjögren's syndrome. The lung involvement resulted in chronic bronchorrhea, recurrent sinusitis, diffuse bronchiolar nodules with bronchiectasis on the CT scan, and a severe obstructive airway pattern on lung function tests. Improvement was obtained in 4 patients with combination of inhaled corticosteroids, inhaled long acting beta-agonists, and a low dose of erythromycin. Copyright © 2010 Elsevier Ltd. All rights reserved.

A case of bronchiolitis obliterans organising pneumonia associated with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome.

Science.gov (United States)

Hameed, Fawad; Steer, Henry

2017-08-01

A 57-year-old woman with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome presented with recurrent episodes of pneumonia. She was treated with multiple courses of antibiotics with no success. The transbronchial biopsy undertaken via bronchoscopy revealed organising pneumonia (OP). She was treated with steroids and responded well with full clinical recovery and normalisation of her chest X-ray.To our knowledge, this is the first reported case of OP in association with SAPHO syndrome. This case report highlights the importance of considering OP in patients with SAPHO syndrome who present with chest infection. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans: correlation of xenon and CT density values with pulmonary function test results

International Nuclear Information System (INIS)

Goo, Hyun Woo; Yang, Dong Hyun; Seo, Joon Beom; Chae, Eun Jin; Lee, Jeongjin; Hong, Soo-Jong; Yu, Jinho; Kim, Byoung-Ju; Krauss, Bernhard

2010-01-01

Xenon ventilation CT using dual-source and dual-energy technique is a recently introduced, promising functional lung imaging method. To expand its clinical applications evidence of additional diagnostic value of xenon ventilation CT over conventional chest CT is required. To evaluate the usefulness of xenon ventilation CT using dual-source and dual-energy technique in children with bronchiolitis obliterans (BO). Seventeen children (age 7-18 years; 11 boys) with BO underwent xenon ventilation CT using dual-source and dual-energy technique. Xenon and CT density values were measured in normal and hyperlucent lung regions on CT and were compared between the two regions. Volumes of hyperlucent regions and ventilation defects were calculated with thresholds determined by visual and histogram-based analysis. Indexed volumes of hyperlucent lung regions and ventilation defects were correlated with pulmonary function test results. Effective doses of xenon CT were calculated. Xenon (14.6 ± 6.4 HU vs 26.1 ± 6.5 HU; P 25-75 , (γ = -0.68-0.88, P ≤ 0.002). Volume percentages of xenon ventilation defects (35.0 ± 16.4%)] were not significantly different from those of hyperlucent lung regions (38.2 ± 18.6%). However, mismatches between the volume percentages were variable up to 21.4-33.3%. Mean effective dose of xenon CT was 1.9 ± 0.5 mSv. In addition to high-resolution anatomic information, xenon ventilation CT using dual-source and dual-energy technique demonstrates impaired regional ventilation and its heterogeneity accurately in children with BO without additional radiation exposure. (orig.)

Crescentic and ring-shaped opacities. CT features in two cases of bronchiolitis obliterans organizing pneumonia (BOOP)

International Nuclear Information System (INIS)

Voloudaki, A.E.; Bouros, D.E.; Froudarakis, M.E.; Datseris, G.E.; Apostolaki, E.G.; Gourtsoyiannis, N.C.

1996-01-01

Two cases of idiopathic bronchilitis obliterans organizing pneumonia (BOOP) with unusual CT findings are presented. On CT both cases exhibited crescentic and ring-shaped opacities, surrounding areas of groundglass attenuation, and associated with a nodular pattern in one patient and airspace consolidations in the second patient. CT-pathologic correlation dislosed that the central areas of groundglass attenuation corresponded to alveolar septal inflammation, in contrast to the denser periphery where granulomatous tissue in peripheral airspaces predominated. In the broad spectrum of CT findings, BOOP can exhibit specific CT features with regard to the crescentic or ring-shaped opacities with a central groundglass attenuation area. Since these features have not been described in any other disease, they might be characteristic features for the diagnosis of BOOP. (orig.)

Postinfectious bronchiolitis obliterans

Science.gov (United States)

2018-06-01

La bronquiolitis obliterante es una enfermedad pulmonar crónica infrecuente y grave producto de una lesión del tracto respiratorio inferior. En nuestro país, es más frecuente observarla secundaria a una lesión viral grave, en especial, por adenovirus. La bronquiolitis obliterante se caracteriza por la oclusión parcial o total del lumen de los bronquiolos respiratorios y terminales por tejido inflamatorio y fibrosis, que produce la obstrucción crónica de la vía aérea. Este consenso discute el estado actual del conocimiento en las diferentes áreas de la bronquiolitis obliterante secundaria a una lesión infecciosa.

Primary Graft Dysfunction and Long-Term Outcomes Following Lung Transplantation

OpenAIRE

DerHovanessian, Ariss

2012-01-01

Background: Primary graft dysfunction (PGD) is an early complication of lung transplantation associated with poor early outcomes, however less is known about its prolonged effects on morbidity and mortality. We hypothesized that PGD is associated with long-term mortality and chronic rejection in the form of bronchiolitis obliterans syndrome. Methods: A retrospective study of 279 adult lung transplant recipients between 2000 and 2007 was performed. PGD grade was determined both immediately ...

Lung transplantation for bronchiolitis obliterans syndrome after allo-SCT

DEFF Research Database (Denmark)

Holm, A M; Riise, Gerdt; Hansson, Leif Helmuth

2013-01-01

and Finland were recorded and compared with survival data from the Scandiatransplant registry. In total, LTx after allo-SCT had been performed in 13 patients. Allo-SCT was done because of AML (n=6), CML (n=3), ALL (n=2), immunodeficiency (n=1) and aplastic anemia (n=1). All developed clinical c...

Risk factors for hospital admission with RSV bronchiolitis in England: a population-based birth cohort study.

Directory of Open Access Journals (Sweden)

Joanna Murray

Full Text Available To examine the timing and duration of RSV bronchiolitis hospital admission among term and preterm infants in England and to identify risk factors for bronchiolitis admission.A population-based birth cohort with follow-up to age 1 year, using the Hospital Episode Statistics database.71 hospitals across England.We identified 296618 individual birth records from 2007/08 and linked to subsequent hospital admission records during the first year of life.In our cohort there were 7189 hospital admissions with a diagnosis of bronchiolitis, 24.2 admissions per 1000 infants under 1 year (95%CI 23.7-24.8, of which 15% (1050/7189 were born preterm (47.3 bronchiolitis admissions per 1000 preterm infants (95% CI 44.4-50.2. The peak age group for bronchiolitis admissions was infants aged 1 month and the median was age 120 days (IQR = 61-209 days. The median length of stay was 1 day (IQR = 0-3. The relative risk (RR of a bronchiolitis admission was higher among infants with known risk factors for severe RSV infection, including those born preterm (RR = 1.9, 95% CI 1.8-2.0 compared with infants born at term. Other conditions also significantly increased risk of bronchiolitis admission, including Down's syndrome (RR = 2.5, 95% CI 1.7-3.7 and cerebral palsy (RR = 2.4, 95% CI 1.5-4.0.Most (85% of the infants who are admitted to hospital with bronchiolitis in England are born at term, with no known predisposing risk factors for severe RSV infection, although risk of admission is higher in known risk groups. The early age of bronchiolitis admissions has important implications for the potential impact and timing of future active and passive immunisations. More research is needed to explain why babies born with Down's syndrome and cerebral palsy are also at higher risk of hospital admission with RSV bronchiolitis.

Clinical definition of respiratory viral infections in young children and potential bronchiolitis misclassification.

Science.gov (United States)

Megalaa, Rosemary; Perez, Geovanny F; Kilaikode-Cheruveettara, Sasikumar; Kotwal, Nidhi; Rodriguez-Martinez, Carlos E; Nino, Gustavo

2018-01-01

Viral respiratory infections are often grouped as a single respiratory syndrome named 'viral bronchiolitis', independently of the viral etiology or individual risk factors. Clinical trials and guidelines have used a more stringent definition of viral bronchiolitis, including only the first episode of wheezing in children less than 12 months of age without concomitant respiratory comorbidities. There is increasing evidence suggesting that this definition is not being followed by pediatric care providers, but it is unclear to what extent viral respiratory infections are currently misclassified as viral bronchiolitis using standard definitions. We conducted a retrospective analysis of hospitalized young children (≤3 years) due to viral respiratory infections. Bronchiolitis was defined as the first wheezing episode less than 12 months of age. Demographic variables and comorbidities were obtained by electronic medical record review. The study comprised a total of 513 hospitalizations (n=453). Viral bronchiolitis was diagnosed in 144 admissions (28.1%). Notably, we identified that the majority of children diagnosed with bronchiolitis (63%) were misclassified as they had prior episodes of wheezing. Many children with bronchiolitis misclassification had significant comorbidities, including prematurity (51%), neuromuscular conditions (9.8%), and congenital heart disease (9.8%). Misclassification of bronchiolitis is a common problem that may lead to inappropriate management of viral respiratory infections in young children. A comprehensive approach that takes into consideration viral etiology and individual risk factors may lead to a more accurate clinical assessment of this condition and would potentially prevent bronchiolitis misclassification. © American Federation for Medical Research (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Bronquiolite obliterante pós-infecciosa na criança Pos-infectious bronchiolitis obliterans in children

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Ana Luísa Lobo

2007-07-01

Full Text Available Na criança imunocompetente, a bronquiolite obliterante (BO é uma doença rara que geralmente ocorre após uma infecção vírica das vias aéreas inferiores. O diagnóstico de BO era, tradicionalmente, confirmado por biópsia pulmonar. Contudo, a identificação de lesão pulmonar prévia, aliada à evolução clínica típica, radiografia e tomografia computorizada sugestivas, substituíram a necessidade de procedimentos mais invasivos. Os autores fizeram uma revisão dos processos clínicos das crianças, com o diagnóstico de BO (n=10 em seguimento numa consulta externa de pneumologia pediátrica, entre Janeiro de 1997 e Dezembro de 2002, com o objectivo de determinar etiologia, apresentação clínica, alterações imagiológicas, tratamento e evolução mais frequentes. Todas as crianças com o diagnóstico de BO apresentavam tosse e/ou pieira persistentes após o episódio agudo inicial. Na altura, 80% das crianças tinham má evolução ponderal. A idade média de diagnóstico foi de 16 meses. O evento inicial foi uma pneumonia e, em 9 casos, identificou-se o agente causal (cinco adenovírus, três vírus sincicial respiratório, um parainfluenzae e um desconhecido. Na nossa casuística, nenhuma das crianças efectuou biópsia pulmonar, por a apresentação clínica e radiológica ser típica de BO. O seguimento, de 36 meses em média, revelou resolução clínica em 3 crianças e sintomas persistentes em 6. Uma criança morreu por falência respiratória progressiva. O reconhecimento rápido do diagnóstico e o tratamento de suporte, que inclui oxigenioterapia e plano nutricional agressivo, contribuíram para melhorar o estado clínico destas crianças.Bronchiolitis obliterans (BO is a rare disease in immunocompetent children that usually occurs after infection of the lower airways. While a diagnosis of BO was usually confirmed by lung biopsy, identification of prior lung lesion plus a typical clinical course and a suggestive chest X

The Induction of IgM and IgG Antibodies against HLA or MICA after Lung Transplantation

OpenAIRE

Paantjens, Annelieke W. M.; van de Graaf, Ed A.; Kwakkel-van Erp, Johanna M.; Hoefnagel, Tineke; van Ginkel, Walter G. J.; Fakhry, Farzia; van Kessel, Diana A.; van den Bosch, Jules M. M.; Otten, Henny G.

2011-01-01

The production of IgG HLA antibodies after lung transplantation (LTx) is considered to be a major risk factor for the development of chronic rejection, represented by the bronchiolitis obliterans syndrome (BOS). It has recently been observed that elevated levels of IgM HLA antibodies also correlates with the development of chronic rejection in heart and kidney transplantation. This study investigates the relationship between IgM and IgG antibodies against HLA and MICA after lung transplantati...

Local interleukin-10 production during respiratory syncytial virus bronchiolitis is associated with post-bronchiolitis wheeze

Directory of Open Access Journals (Sweden)

Hodemaekers Hennie M

2011-09-01

Full Text Available Abstract Background Respiratory syncytial virus (RSV is the most common cause of bronchiolitis in infants. Following RSV bronchiolitis, 50% of children develop post-bronchiolitis wheeze (PBW. Animal studies have suggested that interleukin (IL-10 plays a critical role in the pathogenesis of RSV bronchiolitis and subsequent airway hyperresponsiveness. Previously, we showed that ex vivo monocyte IL-10 production is a predictor of PBW. Additionally, heterozygosity of the single-nucleotide polymorphism (SNP rs1800872 in the IL10 promoter region was associated with protection against RSV bronchiolitis. Methods This study aimed to determine the in vivo role of IL-10 in RSV pathogenesis and recurrent wheeze in a new cohort of 235 infants hospitalized for RSV bronchiolitis. IL-10 levels in nasopharyngeal aspirates (NPAs were measured at the time of hospitalization and the IL10 SNP rs1800872 genotype was determined. Follow-up data were available for 185 children (79%. Results Local IL-10 levels during RSV infection turned out to be higher in infants that later developed physician diagnosed PBW as compared to infants without PBW in the first year after RSV infection (958 vs 692 pg/ml, p = 0.02. The IL10 promoter SNP rs1800872 was not associated with IL-10 concentration in NPAs. Conclusion The relationship between high local IL-10 levels during the initial RSV infection and physician diagnosed PBW provides further evidence of the importance of the IL-10 response during RSV bronchiolitis.

Impact of long-term treatment with inhaled corticosteroids and bronchodilators on lung function in a patient with post-infectious bronchiolitis obliterans.

Science.gov (United States)

Calabrese, Cecilia; Corcione, Nadia; Rea, Gaetano; Stefanelli, Francesco; Meoli, Ilernando; Vatrella, Alessandro

2016-01-01

Post-infectious bronchiolitis obliterans (PIBO) is a small airways disease characterized by fixed airflow limitation. Therefore, inhaled bronchodilators and corticosteroids are not recommended as maintenance therapy options. The management of PIBO currently consists only of close monitoring of affected patients, aimed at the prevention and early treatment of pulmonary infections. In recent years, there has been an increase in the incidence of PIBO in the pediatric population. Patients with PIBO are characterized by a progressive decline in lung function, accompanied by a decrease in overall functional capacity. Here, we report the case of a relatively young man diagnosed with PIBO and followed for three years. After short- and long-term therapy with an inhaled corticosteroid/long-acting 2 agonist combination, together with an inhaled long-acting antimuscarinic, the patient showed relevant improvement of airway obstruction that had been irreversible at the time of the bronchodilator test. The lung function of the patient worsened when he interrupted the triple inhaled therapy. In addition, a 3-week pulmonary rehabilitation program markedly improved his physical performance. RESUMO A bronquiolite obliterante pós-infecciosa (BOPI) é uma doença das pequenas vias aéreas caracterizada por limitação fixa do fluxo aéreo. Portanto, os broncodilatadores e os corticosteroides inalatórios não são recomendados como opções de terapia de manutenção. Atualmente, o manejo da BOPI consiste apenas de um acompanhamento rigoroso dos pacientes afetados, visando à prevenção e ao tratamento precoce de infecções pulmonares. A incidência de BOPI tem aumentado na população pediátrica nos últimos anos. Os pacientes com BOPI caracterizam-se por um declínio progressivo da função pulmonar, associado a uma diminuição da capacidade funcional global. Relatamos aqui o caso de um homem relativamente jovem diagnosticado com BOPI, acompanhado por três anos. Ap

Bronchiolitis

Science.gov (United States)

... young children and infants. It causes inflammation and congestion in the small airways (bronchioles) of the lung. ... hospitalization. Severe respiratory failure may require that a tube be inserted into the trachea to help the ...

HRCT findings of childhood follicular bronchiolitis

International Nuclear Information System (INIS)

Weinman, Jason P.; Browne, Lorna P.; Manning, David A.; Liptzin, Deborah R.; Krausert, Amanda J.

2017-01-01

Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We found no reports of systematic review of high-resolution computed tomography (HRCT) findings in pediatric follicular bronchiolitis. The purpose of this study was to describe the HRCT findings of follicular bronchiolitis in children and correlate these imaging findings with histopathology. A 5-year retrospective review of all pathology-proven cases of follicular bronchiolitis was performed. Inclusion criteria were age <18 years and an HRCT within 6 months of lung biopsy. HRCTs were reviewed by three observers and scored using the system previously described by Brody et al. Six patients met the inclusion criteria with age range at HRCT of 7-82 months (median: 39.5 months). Pulmonary nodules (n=6) were the most common HRCT finding followed by focal consolidation (n=5), bronchiectasis (n=4) and lymphadenopathy (n=3). Tree and bud opacities and nodules on CT correlated with interstitial lymphocytic infiltrates and discrete lymphoid follicles on pathology. The salient HRCT findings of childhood follicular bronchiolitis are bilateral, lower lung zone predominant pulmonary nodules and bronchiectasis with infantile onset of symptoms. These characteristic HRCT findings help differentiate follicular bronchiolitis from other forms of infantile onset ILD. (orig.)

HRCT findings of childhood follicular bronchiolitis

Energy Technology Data Exchange (ETDEWEB)

Weinman, Jason P.; Browne, Lorna P. [Children' s Hospital Colorado, Department of Radiology, Aurora, CO (United States); Manning, David A. [Children' s Hospital of New Orleans, Department of Radiology, New Orleans, LA (United States); Liptzin, Deborah R. [Children' s Hospital Colorado, Department of Pediatrics, Section of Pediatric Pulmonology, Aurora, CO (United States); Krausert, Amanda J. [New Orleans Forensic Center, New Orleans, LA (United States)

2017-12-15

Follicular bronchiolitis is a lymphoproliferative form of interstitial lung disease (ILD) defined by the presence of peribronchial lymphoid follicles. Follicular bronchiolitis has been associated with viral infection, autoimmune disease and immunodeficiency. The most common clinical manifestation is respiratory distress in infancy followed by a prolonged course with gradual improvement. We found no reports of systematic review of high-resolution computed tomography (HRCT) findings in pediatric follicular bronchiolitis. The purpose of this study was to describe the HRCT findings of follicular bronchiolitis in children and correlate these imaging findings with histopathology. A 5-year retrospective review of all pathology-proven cases of follicular bronchiolitis was performed. Inclusion criteria were age <18 years and an HRCT within 6 months of lung biopsy. HRCTs were reviewed by three observers and scored using the system previously described by Brody et al. Six patients met the inclusion criteria with age range at HRCT of 7-82 months (median: 39.5 months). Pulmonary nodules (n=6) were the most common HRCT finding followed by focal consolidation (n=5), bronchiectasis (n=4) and lymphadenopathy (n=3). Tree and bud opacities and nodules on CT correlated with interstitial lymphocytic infiltrates and discrete lymphoid follicles on pathology. The salient HRCT findings of childhood follicular bronchiolitis are bilateral, lower lung zone predominant pulmonary nodules and bronchiectasis with infantile onset of symptoms. These characteristic HRCT findings help differentiate follicular bronchiolitis from other forms of infantile onset ILD. (orig.)

Evaluating bronchodilator response in pediatric patients with post-infectious bronchiolitis obliterans: use of different criteria for identifying airway reversibility.

Science.gov (United States)

Mattiello, Rita; Vidal, Paula Cristina; Sarria, Edgar Enrique; Pitrez, Paulo Márcio; Stein, Renato Tetelbom; Mocelin, Helena Teresinha; Fischer, Gilberto Bueno; Jones, Marcus Herbert; Pinto, Leonardo Araújo

2016-01-01

Post-infectious bronchiolitis obliterans (PIBO) is a clinical entity that has been classified as constrictive, fixed obstruction of the lumen by fibrotic tissue. However, recent studies using impulse oscillometry have reported bronchodilator responses in PIBO patients. The objective of this study was to evaluate bronchodilator responses in pediatric PIBO patients, comparing different criteria to define the response. We evaluated pediatric patients diagnosed with PIBO and treated at one of two pediatric pulmonology outpatient clinics in the city of Porto Alegre, Brazil. Spirometric parameters were measured in accordance with international recommendations. We included a total of 72 pediatric PIBO patients. The mean pre- and post-bronchodilator values were clearly lower than the reference values for all parameters, especially FEF25-75%. There were post-bronchodilator improvements. When measured as mean percent increases, FEV1 and FEF25-75%, improved by 11% and 20%, respectively. However, when the absolute values were calculated, the mean FEV1 and FEF25-75% both increased by only 0.1 L. We found that age at viral aggression, a family history of asthma, and allergy had no significant effects on bronchodilator responses. Pediatric patients with PIBO have peripheral airway obstruction that is responsive to treatment but is not completely reversible with a bronchodilator. The concept of PIBO as fixed, irreversible obstruction does not seem to apply to this population. Our data suggest that airway obstruction is variable in PIBO patients, a finding that could have major clinical implications. A bronquiolite obliterante pós-infecciosa (BOPI) é uma entidade clínica que tem sido classificada como obstrução fixa e constritiva do lúmen por tecido fibrótico. Entretanto, estudos recentes utilizando oscilometria de impulso relataram resposta ao broncodilatador em pacientes com BOPI. O objetivo deste estudo foi avaliar a resposta broncodilatadora em pacientes pediátricos com

Immunological risk stratification of the bronchiolitis obliterans syndrome after lung transplantation

NARCIS (Netherlands)

Kwakkel - van Erp, J.M.

2011-01-01

The development of chronic allograft rejection after lung transplantation (LTx) is the most common cause of poor long-term survival in lung transplant recipients. This rejection leads to obliteration of the bronchioli. Since this obliteration has a patchy distribution and normal lung tissue obtained

Pulse oximetry in bronchiolitis: is it needed?

Directory of Open Access Journals (Sweden)

Hendaus MA

2015-10-01

Full Text Available Mohamed A Hendaus,1,2 Fatima A Jomha,3 Ahmed H Alhammadi,1,2 1Department of Pediatrics, General Pediatrics Division, Hamad Medical Corporation, 2Weill-Cornell Medical College, Doha, Qatar; 3School of Pharmacy, Lebanese International University, Khiara, Lebanon Abstract: Infants admitted to health-care centers with acute bronchiolitis are frequently monitored with a pulse oximeter, a noninvasive method commonly used for measuring oxygen saturation. The decision to hospitalize children with bronchiolitis has been largely influenced by pulse oximetry, despite its questionable diagnostic value in delineating the severity of the illness. Many health-care providers lack the appropriate clinical fundamentals and limitations of pulse oximetry. This deficiency in knowledge might have been linked to changes in the management of bronchiolitis. The aim of this paper is to provide the current evidence on the role of pulse oximetry in bronchiolitis. We discuss the history, fundamentals of operation, and limitations of the apparatus. A search of the Google Scholar, Embase, Medline, and PubMed databases was carried out for published articles covering the use of pulse oximetry in bronchiolitis. Keywords: bronchiolitis, children, monitor, oxygen

Oxidative and antioxidative status of children with acute bronchiolitis.

Science.gov (United States)

Dundaroz, Rusen; Erenberk, Ufuk; Turel, Ozden; Demir, Aysegul Dogan; Ozkaya, Emin; Erel, Ozcan

2013-01-01

Oxidative stress has been shown to contribute to the pathogenesis of acute and chronic lung inflammatory diseases. This article aimed to evaluate the oxidant/antioxidant status of children with acute bronchiolitis through the measurement of plasma total antioxidant capacity, total oxidant status, and oxidative stress index. Children with acute bronchiolitis admitted to the pediatric emergency department of a university hospital between January and April of 2012 were compared with age-matched healthy controls. Patients with acute bronchiolitis were classified as mild and moderate bronchiolitis. Oxidative and antioxidative status were assessed by measurement of plasma total antioxidant capacity, total oxidant status, and oxidative stress index. Thirty-one children with acute bronchiolitis aged between 3 months and 2 years, and 39 healthy children were included. Total oxidative status (TOS) was higher in patients with acute bronchiolitis than the control group (5.16±1.99 μmol H2O2 versus 3.78±1.78 μmol H2O2 [p=0.004]). Total antioxidant capacity (TAC) was lower in children with bronchiolitis than the control group (2.51±0.37 μmol Trolox eqv/L versus 2.75±0.39 μmol Trolox eqv/L [p=0.013]). Patients with moderate bronchiolitis presented higher TOS levels than those with mild bronchiolitis and the control group (p=0.03, pantioxidant systems is disrupted in children with moderate bronchiolitis, which indicates that this stress factor may have a role in the pathogenesis of the disease. Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

Bronchiolitis - discharge

Science.gov (United States)

... bronchiolitis. Your health care provider may prescribe such medicine for your child. DO NOT give your child decongestant nose drops, antihistamines, or any other cold medicines unless your child's doctor tells you to. When to Call the ...

Seasonal Timing of Infant Bronchiolitis, Apnea and Sudden Unexplained Infant Death.

Directory of Open Access Journals (Sweden)

Chantel D Sloan

Full Text Available Rates of Sudden Unexplained Infant Death (SUID, bronchiolitis, and central apnea increase in winter in temperate climates. Though associations between these three conditions are suggested, more work is required to establish if there is a causal pathway linking bronchiolitis to SUID through inducing central apnea. Utilizing a large population-based cohort of infants studied over a 20-year period (n = 834,595, from birth years 1989-2009, we analyzed ecological associations between timing of SUID cases, bronchiolitis, and apnea healthcare visits. Data were analyzed between 2013 and 2015. We used a Cox Proportional Hazards model to analyze possible interactions between maternal smoking and maternal asthma with infant bronchiolitis on time to SUID. SUID and bronchiolitis both occurred more frequently in winter. An increase in bronchiolitis clinical visits occurred within a few days prior to apnea visits. We found a temporal relationship between infant bronchiolitis and apnea. In contrast, no peak in SUID cases was seen during peaks of bronchiolitis. Among those without any bronchiolitis visits, maternal smoking was associated with an increased risk of SUID: Hazard Ratio (HR of 2.38 (95% CI: 2.11, 2.67, p-value <0.001. Maternal asthma was associated with an increased risk of SUID among infants with at least one bronchiolitis visit: HR of 2.40 (95% CI: 1.04, 5.54, p-value = 0.04. Consistent trends between bronchiolitis, apnea, and SUID were not established due to small numbers of SUID cases. However, interaction analysis revealed potential differential associations of bronchiolitis and SUID by maternal smoking, maternal asthma status.

Prevention and nursing care of the complications occurred in interventional therapy for arteriosclerosis obliterans of lower extremity

International Nuclear Information System (INIS)

Xu Yang; Qi Yuchun; Wang Hua; Han Yajun; Fu Wenli; Fan Rui; Lv Xiaoying

2009-01-01

Objective: To discuss the prevention and nursing care of the perioperative complications occurred in interventional therapy for arteriosclerosis obliterans of lower extremity. Methods: During the period of July 2006 to June 2009, interventional treatment for the arteriosclerosis obliterans of lower extremity was performed in 380 cases. The clinical data and complications were reviewed and analyzed, and the prevention and nursing care of the complications were summarized. Results: Complications occurred in 41 cases. During the surgery, vascular rupture or arterial dissection occurred in 5 cases, hypoglycemia reaction in 3 cases and elevation of blood pressure in 2 cases. The complications,which occurred after the treatment,included acute arterial thrombosis (n=3), deep vein thrombosis (n=2), bleeding of different tissues or organs (n=17), acute myocardial infarction (n=2), pseudoaneurysm (n=2), excessive lower limb perfusion syndrome (n=4) and compression sores (n=1). Conclusion: Detailed information of medical history, careful observation of clinical condition, intensive care of patient, adequate preparation of medical materials, seriously handing over the duty to the next shift and taking one's turn on duty, etc. are all the effective measures to prevent and to reduce the occurrence of complications. (authors)

Diagnosis and management of bronchiolitis.

Science.gov (United States)

2006-10-01

Bronchiolitis is a disorder most commonly caused in infants by viral lower respiratory tract infection. It is the most common lower respiratory infection in this age group. It is characterized by acute inflammation, edema, and necrosis of epithelial cells lining small airways, increased mucus production, and bronchospasm. The American Academy of Pediatrics convened a committee composed of primary care physicians and specialists in the fields of pulmonology, infectious disease, emergency medicine, epidemiology, and medical informatics. The committee partnered with the Agency for Healthcare Research and Quality and the RTI International-University of North Carolina Evidence-Based Practice Center to develop a comprehensive review of the evidence-based literature related to the diagnosis, management, and prevention of bronchiolitis. The resulting evidence report and other sources of data were used to formulate clinical practice guideline recommendations. This guideline addresses the diagnosis of bronchiolitis as well as various therapeutic interventions including bronchodilators, corticosteroids, antiviral and antibacterial agents, hydration, chest physiotherapy, and oxygen. Recommendations are made for prevention of respiratory syncytial virus infection with palivizumab and the control of nosocomial spread of infection. Decisions were made on the basis of a systematic grading of the quality of evidence and strength of recommendation. The clinical practice guideline underwent comprehensive peer review before it was approved by the American Academy of Pediatrics. This clinical practice guideline is not intended as a sole source of guidance in the management of children with bronchiolitis. Rather, it is intended to assist clinicians in decision-making. It is not intended to replace clinical judgment or establish a protocol for the care of all children with this condition. These recommendations may not provide the only appropriate approach to the management of children

A prospective randomised trial comparing nasogastric with intravenous hydration in children with bronchiolitis (protocol) The comparative rehydration in bronchiolitis study (CRIB)

OpenAIRE

Borland Meredith; Acworth Jason; Babl Franz E; Oakley Ed; Kreiser David; Neutze Jocelyn; Theophilos Theane; Donath Susan; South Mike; Davidson Andrew

2010-01-01

Abstract Background Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV) or nasogastric (NG). The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the...

Bronchiolitis: presenting signs and etiologic factors

International Nuclear Information System (INIS)

Hernandez, D.; Pallisa, E.; Sebastia, M. C.; Quiroga, S.; Alvarez-Castells, A.; Caceres, J.

1999-01-01

Bronchiolitis is a disease of the small airways that can effect both the terminal and respiratory bronchioles and the alveoli. The diagnosis is based on histological findings and it is generally divided into two types: proliferative and constrictive. In clinical practice, there are a number of entities, such as infections, connective tissue diseases and lug transplantation, that can results in involvement of the small airways. In high-resolution computed tomography (HRCT), signs of bronchiolitis include centrilobular nodules, bronchiolectasis, mosaic perfusion, parenchymal consolidations and air-trapping. The findings are nonspecific, although the predominant features change depending on the underlying disease. Familiarity with the radiological sings of bronchilitis in HRCT studies avoids incorrect diagnoses and broadens the available knowledge of the morphology of lung pathology. (Author) 16 refs

Sustainability in the AAP Bronchiolitis Quality Improvement Project.

Science.gov (United States)

Shadman, Kristin A; Ralston, Shawn L; Garber, Matthew D; Eickhoff, Jens; Mussman, Grant M; Walley, Susan C; Rice-Conboy, Elizabeth; Coller, Ryan J

2017-11-01

Adherence to American Academy of Pediatrics (AAP) bronchiolitis clinical practice guideline recommendations improved significantly through the AAP's multiinstitutional collaborative, the Bronchiolitis Quality Improvement Project (BQIP). We assessed sustainability of improvements at participating institutions for 1 year following completion of the collaborative. Twenty-one multidisciplinary hospital-based teams provided monthly data for key inpatient bronchiolitis measures during baseline and intervention bronchiolitis seasons. Nine sites provided data in the season following completion of the collaborative. Encounters included children younger than 24 months who were hospitalized for bronchiolitis without comorbid chronic illness, prematurity, or intensive care. Changes between baseline-, intervention-, and sustainability-season data were assessed using generalized linear mixed-effects models with site-specific random effects. Differences between hospital characteristics, baseline performance, and initial improvement between sites that did and did not participate in the sustainability season were compared. A total of 2275 discharges were reviewed, comprising 995 baseline, 877 intervention, and 403 sustainability- season encounters. Improvements in all key bronchiolitis quality measures achieved during the intervention season were maintained during the sustainability season, and orders for intermittent pulse oximetry increased from 40.6% (95% confidence interval [CI], 22.8-61.1) to 79.2% (95% CI, 58.0- 91.3). Sites that did and did not participate in the sustainability season had similar characteristics. BQIP participating sites maintained improvements in key bronchiolitis quality measures for 1 year following the project's completion. This approach, which provided an evidence-based best-practice toolkit while building the quality-improvement capacity of local interdisciplinary teams, may support performance gains that persist beyond the active phase of the

Mechanisms underlying epithelium-dependent relaxation in rat bronchioles

DEFF Research Database (Denmark)

Kroigaard, Christel; Dalsgaard, Thomas; Simonsen, Ulf

2010-01-01

This study investigated the mechanisms underlying epithelium-derived hyperpolarizing factor (EpDHF)-type relaxation in rat bronchioles. Immunohistochemistry was performed, and rat bronchioles and pulmonary arteries were mounted in microvascular myographs for functional studies. An opener of small...... (SK(Ca)) and intermediate (IK(Ca))-conductance calcium-activated potassium channels, NS309 (6,7-dichloro-1H-indole-2,3-dione 3-oxime) was used to induce EpDHF-type relaxation. IK(Ca) and SK(Ca)3 positive immunoreactions were observed mainly in the epithelium and endothelium of bronchioles and arteries......, respectively. In 5-hydroxytryptamine (1 microM)-contracted bronchioles (828 +/- 20 microm, n = 84) and U46619 (0.03 microM)-contracted arteries (720 +/- 24 microm, n = 68), NS309 (0.001-10 microM) induced concentration-dependent relaxations that were reduced by epithelium/endothelium removal and by blocking IK...

Winter air pollution and infant bronchiolitis in Paris.

Science.gov (United States)

Ségala, Claire; Poizeau, David; Mesbah, Mounir; Willems, Sylvie; Maidenberg, Manuel

2008-01-01

Respiratory syncytial virus (RSV) is one of the most common respiratory pathogens in infants and young children. It is not known why some previously healthy infants, when in contact with RSV, develop bronchiolitis whereas others have only mild symptoms. Our study aimed to evaluate the possible association between emergency hospital visits for bronchiolitis and air pollution in the Paris region during four winter seasons. We included children under the age of 3 years who attended emergency room services for bronchiolitis (following standardized definition) during the period 1997-2001. Two series of data from 34 hospitals, the daily number of emergency hospital consultations (n=50857) and the daily number of hospitalizations (n=16588) for bronchiolitis, were analyzed using alternative statistical methods; these were the generalized additive model (GAM) and case-crossover models. After adjustments for public holidays, holidays and meteorological variables the case-crossover model showed that PM10, BS, SO2 and NO2 were positively associated with both consultations and hospitalizations. GAM models, adjusting for long-term trend, seasonality, holiday, public holiday, weekday and meteorological variables, gave similar results for SO2 and PM10. This study shows that air pollution may act as a trigger for the occurrence of acute severe bronchiolitis cases.

International Variation in Asthma and Bronchiolitis Guidelines.

Science.gov (United States)

Bakel, Leigh Anne; Hamid, Jemila; Ewusie, Joycelyne; Liu, Kai; Mussa, Joseph; Straus, Sharon; Parkin, Patricia; Cohen, Eyal

2017-11-01

Guideline recommendations for the same clinical condition may vary. The purpose of this study was to determine the degree of agreement among comparable asthma and bronchiolitis treatment recommendations from guidelines. National and international guidelines were searched by using guideline databases (eg, National Guidelines Clearinghouse: December 16-17, 2014, and January 9, 2015). Guideline recommendations were categorized as (1) recommend, (2) optionally recommend, (3) abstain from recommending, (4) recommend against a treatment, and (5) not addressed by the guideline. The degree of agreement between recommendations was evaluated by using an unweighted and weighted κ score. Pairwise comparisons of the guidelines were evaluated similarly. There were 7 guidelines for asthma and 4 guidelines for bronchiolitis. For asthma, there were 166 recommendation topics, with 69 recommendation topics given in ≥2 guidelines. For bronchiolitis, there were 46 recommendation topics, with 21 recommendation topics provided in ≥2 guidelines. The overall κ for asthma was 0.03, both unweighted (95% confidence interval [CI]: -0.01 to 0.07) and weighted (95% CI: -0.01 to 0.10); for bronchiolitis, it was 0.32 unweighted (95% CI: 0.16 to 0.52) and 0.15 weighted (95% CI: -0.01 to 0.5). Less agreement was found in national and international guidelines for asthma than for bronchiolitis. Additional studies are needed to determine if differences are based on patient preferences and values and economic considerations or if other recommendation-level, guideline-level, and condition-level factors are driving these differences. Copyright © 2017 by the American Academy of Pediatrics.

Pediatric Interstitial Lung Disease Masquerading as Difficult Asthma: Management Dilemmas for Rare Lung Disease in Children

Directory of Open Access Journals (Sweden)

EY Chan

2005-01-01

Full Text Available Idiopathic nontransplant-related childhood bronchiolitis obliterans is an uncommon disease. Most patients present with chronic recurrent dyspnea, cough and wheezing, which are also features of asthma, by far a much more common condition. The present case study reports on a six-year-old girl who presented to a tertiary care centre with recurrent episodes of respiratory distress on a background of baseline tachypnea, chronic hypoxemia and exertional dyspnea. Her past medical history revealed significant lung disease in infancy, including respiratory syncytial virus bronchiolitis and repaired gastroesophageal reflux. She was treated for 'asthma exacerbations' throughout her early childhood years. Bronchiolitis obliterans was subsequently diagnosed with an open lung biopsy. She did not have sustained improvement with systemic corticosteroids, hydroxychloroquine or clarithromycin. Cardiac catheterization confirmed the presence of secondary pulmonary hypertension. Treatment options remain a dilemma for this patient because there is no known effective treatment for this condition, and the natural history is not well understood. The present case demonstrates the need for careful workup in 'atypical asthma', and the urgent need for further research into the rare lung diseases of childhood.

Frequency of pleural effusion in acute bronchiolitis and its effect on prognosis

Directory of Open Access Journals (Sweden)

Yilmaz Ayse E

2011-12-01

Full Text Available Abstract Aim To analyze the frequency of pleural effusion and the effect on prognosis in children with acute bronchiolitis. Methods A total of 69 infants aged 1-24 months with acute bronchiolitis were studied between September 2009 and December 2010. All patients'age, sex, breastfeeding duration, exposure to smoking, history of using vitamin D, symptoms duration, physical examination and laboratory findings were recorded. Bronchiolitis score and predisposing factors that influence the disease process were determined. Thorax ultrasonography was carried out in all patients, who were evaluated on the 3rd and 7th day of the treatment. Results Mean age of patients (43 boys, 26 girls was 11.97 ± 0.69 months (median 11 months. Breastfeeding duration was 8.26 ± 0.56 months (median 8 months. According to bronchiolitis score, 52 patients (75.4% had mild and moderate bronchiolitis and 17 (24.6% had severe bronchiolitis; 34 patients (49.2% had pleural effusion. There was no relation between pleural effusion and symptoms. Frequency of pleural effusion was significantly higher in patients with risk factors. Conclusions This study demonstrated that most of the acute bronchiolitis cases in the infants studied were accompanied by pleural effusion. Pleural effusion in acute bronchiolitis had no effects on prognosis.

Coexistence of axial spondyloarthritis and thromboangiitis obliterans in a young woman

Directory of Open Access Journals (Sweden)

G. Lopalco

2015-06-01

Full Text Available A peculiar coexistence of axial spondyloarthritis and ischemia of the feet and the fourth finger of the left hand in a young woman, who was a heavy smoker, is discussed in this report. This picture was considered within the context of thromboangiitis obliterans. Positivity of anti-nuclear antibodies and mild elevation of inflammatory parameters were noted. Computed tomography angiograms of upper and lower limbs showed luminal narrowing and occlusion of the left humeral, left anterior/posterior tibial and right anterior tibial arteries. Daily iloprost perfusions were started, and smoking cessation was strongly recommended. Coldness and rest pain in the distal extremities improved within a few weeks. The possibility that spondyloarthritis might precede the clinical picture of thromboangiitis obliterans should be considered in heavy smokers.

[Thromboangiitis obliterans (Buerger's disease): update 2015].

Science.gov (United States)

Klein-Weigel, Peter; Volz, Theresa Sophie; Richter, Jutta

2015-10-01

Thromboangiitis obliterans (Buerger's disease) is a vasculitis with undulating clinical course multisegmentarily affecting small and medium-sized arteries and veins. The disease is closely linked to tobacco-use. Increasing knowledge of autoimmunologic mechanisms in the complex pathophyiology of the disease let to the formulation of an autoimmunity-hypothesis now serving as a new paradigma. New treatment options comprise progenitor-cell-therapy, immunoadsorption, use of sendothelin-receptor-blocking agent Bosentan, and prescriptions of antiphosphodiesterase-V-inhibitors. © Georg Thieme Verlag KG Stuttgart · New York.

Computed tomography findings of postoperative complications in lung transplantation

International Nuclear Information System (INIS)

Hochhegger, Bruno; Irion, Klaus Loureiro; Marchiori, Edson; Bello, Rodrigo; Moreira, Jose; Camargo, Jose Jesus; Universidade Federal do Rio de Janeiro

2009-01-01

Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis. (author)

Computed tomography findings of postoperative complications in lung transplantation; Achados tomograficos nas complicacoes pos-operatorias do transplante pulmonar

Energy Technology Data Exchange (ETDEWEB)

Hochhegger, Bruno; Irion, Klaus Loureiro; Marchiori, Edson; Bello, Rodrigo; Moreira, Jose; Camargo, Jose Jesus [Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS (Brazil). Postgraduate Program in Respiratory Sciences; Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Postgraduate Program in Radiological Sciences], e-mail: brunorgs@mail.ufsm.br

2009-03-15

Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis. (author)

Bronchiolitis: what the clinician should know

Directory of Open Access Journals (Sweden)

Roberto Antonucci

2015-10-01

Full Text Available Bronchiolitis is an acute infection of the lower respiratory tract affecting infants and young children, with Respiratory Syncytial Virus (RSV being the most common pathogen. Bronchiolitis is generally a mild disease, but may present with severe signs and symptoms requiring hospitalization. Risk factors including prematurity, bronchopulmonary dysplasia, immunodeficiency and congenital heart defects may predispose patients to develop a severe disease. The diagnosis should be based on clinical evaluation, without supportive radiographic and laboratory studies. Etiological diagnosis may be helpful to decrease the hospital transmission of virus and to avoid inappropriate use of antibiotics.The mainstay of therapy for bronchiolitis is supportive care, which should be directed at maintaining adequate oxygenation, ensuring a proper respiratory toilet, and meeting the requirements of fluids and nutrition. The use of nebulized hypertonic saline should be limited to hospitalized patients. Severe respiratory failure may require mechanical ventilatory support. Neither corticosteroids nor antibiotics offer consistent benefit in the treatment of bronchiolitis, and thus should not be used. A trial of a bronchodilator may be appropriate, but should be continued exclusively if a prompt favorable response occurs. Effective interventions to prevent the spread of RSV infection include hand washing or disinfection by caregivers and contact isolation. The use of palivizumab, a monoclonal antibody directed against RSV, is a safe prophylactic option, but should be restricted to children at high-risk for severe RSV disease, during the epidemic period. Current evidence suggests that early RSV bronchiolitis predisposes children to recurrent wheezing and asthma in the first decade of life.Proceedings of the 11th International Workshop on Neonatology and Satellite Meetings · Cagliari (Italy · October 26th-31st, 2015 · From the womb to the adultGuest Editors: Vassilios

Office-based treatment and outcomes for febrile infants with clinically diagnosed bronchiolitis.

Science.gov (United States)

Luginbuhl, Lynn M; Newman, Thomas B; Pantell, Robert H; Finch, Stacia A; Wasserman, Richard C

2008-11-01

The goals were to describe the (1) frequency of sepsis evaluation and empiric antibiotic treatment, (2) clinical predictors of management, and (3) serious bacterial illness frequency for febrile infants with clinically diagnosed bronchiolitis seen in office settings. The Pediatric Research in Office Settings network conducted a prospective cohort study of 3066 febrile infants (or=38 degrees C) in 219 practices in 44 states. We compared the frequency of sepsis evaluation, parenteral antibiotic treatment, and serious bacterial illness in infants with and without clinically diagnosed bronchiolitis. We identified predictors of sepsis evaluation and parenteral antibiotic treatment in infants with bronchiolitis by using logistic regression models. Practitioners were less likely to perform a complete sepsis evaluation, urine testing, and cerebrospinal fluid culture and to administer parenteral antibiotic treatment for infants with bronchiolitis, compared with those without bronchiolitis. Significant predictors of sepsis evaluation in infants with bronchiolitis included younger age, higher maximal temperature, and respiratory syncytial virus testing. Predictors of parenteral antibiotic use included initial ill appearance, age of urinary tract infection (n = 167). In office settings, serious bacterial illness in young febrile infants with clinically diagnosed bronchiolitis is uncommon. Limited testing for bacterial infections seems to be an appropriate management strategy.

Adult bronchiolitis – A clinical and pathological interpretative classification

Directory of Open Access Journals (Sweden)

D. Madama

2016-03-01

Full Text Available Introduction: Bronchiolitis is a heterogeneous group of diseases of an inflammatory nature, centered on small conducting airways and often associated with other pulmonary disorders. No single classification scheme for bronchiolar diseases has been widely accepted. In this retrospective study, it was decided to apply a new clinical and pathological interpretative classification. Objectives: To propose a new clinical and pathological interpretative classification for adult bronchiolitis, based on statistical analysis of a population of 193 patients with histopathological diagnosis of bronchiolitis. Materials and methods: A retrospective study analyzed the epidemiological characteristics, co-morbidities and radiological findings present in a group of patients with histopathological diagnosis of bronchiolitis. Results: This trial involved 193 cases collected over a period of eleven years; 48 (24.9% patients had simultaneous pulmonary disease; non-pulmonary diseases, such as cardiovascular diseases, type II Diabetes mellitus and dyslipidemia were present in 57 cases. The image study was extremely important in order to integrate clinical and pathological aspects.In this study respiratory bronchiolitis related to smoking dominated. The radiological findings confirmed the secondary nature of the histopathological features, with prevalence of ground-glass patterns, pneumothorax and patterns of interstitial involvement, as described in the literature. It was also verified that clinical behavior of different forms of bronchiolitis was important to distinguish the various types, since they could progress without typical anatomopathological aspects. Conclusion: This trial showed that the vast majority of diagnosis obtained corresponded to bronchiolitis as secondary to pulmonary pathology. In most cases, morphological findings had to be complemented with clinical and radiological characteristics, in order to obtain the final diagnosis. Keywords: Bronchiolitis

Risk of urinary tract infection in infants and children with acute bronchiolitis

Science.gov (United States)

Hendaus, Mohamed A; Alhammadi, Ahmed H; Khalifa, Mohamed S; Muneer, Eshan; Chandra, Prem

2015-01-01

OBJECTIVES: To estimate the prevalence of urinary tract infection in infants and children with bronchiolitis. METHODS: A retrospective cross-sectional study involving patients zero to 24 months of age who were hospitalized with acute bronchiolitis was conducted. RESULTS: A total of 835 paediatric patients with acute bronchiolitis were admitted to the paediatric ward between January 2010 and December 2012. The mean (± SD) age at diagnosis was 3.47±2.99 months. There were 325 (39%) girls and 510 (61%) boys. For the purpose of data analysis, the patient population was divided into three groups: group 1 included children hospitalized with respiratory syncytial virus (RSV) bronchiolitis; group 2 included children hospitalized with clinical bronchiolitis with no virus detected; and group 3 included children hospitalized with clinical bronchiolitis due to a respiratory virus other than RSV. Results revealed that urinary tract infection was present in 10% of patients, and was most common in group 3 (13.4%) followed by group 2 (9.7%), and was least common in group 1 (6%) (P=0.030). CONCLUSIONS: The possibility of a urinary tract infection should be considered in a febrile child with a diagnosis of bronchiolitis, particularly if the trigger is a respiratory virus other than RSV. PMID:26175566

SA Journal of Radiology - Vol 9, No 2 (2005)

African Journals Online (AJOL)

Bronchiolitis obliterans organising pneumonia (BOOP) or cryptogenic organising pneumonia (COP) · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. VK Bhagwandas, R Morar, HD Bhula, 19-21. http://dx.doi.org/10.4102/sajr.v9i2.86 ...

Bronchodilators for bronchiolitis.

Science.gov (United States)

Gadomski, Anne M; Scribani, Melissa B

2014-06-17

Bronchiolitis is an acute, viral lower respiratory tract infection affecting infants and is sometimes treated with bronchodilators. To assess the effects of bronchodilators on clinical outcomes in infants (0 to 12 months) with acute bronchiolitis. We searched CENTRAL 2013, Issue 12, MEDLINE (1966 to January Week 2, 2014) and EMBASE (1998 to January 2014). Randomized controlled trials (RCTs) comparing bronchodilators (other than epinephrine) with placebo for bronchiolitis. Two authors assessed trial quality and extracted data. We obtained unpublished data from trial authors. We included 30 trials (35 data sets) representing 1992 infants with bronchiolitis. In 11 inpatient and 10 outpatient studies, oxygen saturation did not improve with bronchodilators (mean difference (MD) -0.43, 95% confidence interval (CI) -0.92 to 0.06, n = 1242). Outpatient bronchodilator treatment did not reduce the rate of hospitalization (11.9% in bronchodilator group versus 15.9% in placebo group, odds ratio (OR) 0.75, 95% CI 0.46 to 1.21, n = 710). Inpatient bronchodilator treatment did not reduce the duration of hospitalization (MD 0.06, 95% CI -0.27 to 0.39, n = 349).Effect estimates for inpatients (MD -0.62, 95% CI -1.40 to 0.16) were slightly larger than for outpatients (MD -0.25, 95% CI -0.61 to 0.11) for oximetry. Oximetry outcomes showed significant heterogeneity (I(2) statistic = 81%). Including only studies with low risk of bias had little impact on the overall effect size of oximetry (MD -0.38, 95% CI -0.75 to 0.00) but results were close to statistical significance.In eight inpatient studies, there was no change in average clinical score (standardized MD (SMD) -0.14, 95% CI -0.41 to 0.12) with bronchodilators. In nine outpatient studies, the average clinical score decreased slightly with bronchodilators (SMD -0.42, 95% CI -0.79 to -0.06), a statistically significant finding of questionable clinical importance. The clinical score outcome showed significant heterogeneity (I(2

Respiratory Syncytial Virus Bronchiolitis in Children.

Science.gov (United States)

Smith, Dustin K; Seales, Sajeewane; Budzik, Carol

2017-01-15

Bronchiolitis is a common lower respiratory tract infection in infants and young children, and respiratory syncytial virus (RSV) is the most common cause of this infection. RSV is transmitted through contact with respiratory droplets either directly from an infected person or self-inoculation by contaminated secretions on surfaces. Patients with RSV bronchiolitis usually present with two to four days of upper respiratory tract symptoms such as fever, rhinorrhea, and congestion, followed by lower respiratory tract symptoms such as increasing cough, wheezing, and increased respiratory effort. In 2014, the American Academy of Pediatrics updated its clinical practice guideline for diagnosis and management of RSV bronchiolitis to minimize unnecessary diagnostic testing and interventions. Bronchiolitis remains a clinical diagnosis, and diagnostic testing is not routinely recommended. Treatment of RSV infection is mainly supportive, and modalities such as bronchodilators, epinephrine, corticosteroids, hypertonic saline, and antibiotics are generally not useful. Evidence supports using supplemental oxygen to maintain adequate oxygen saturation; however, continuous pulse oximetry is no longer required. The other mainstay of therapy is intravenous or nasogastric administration of fluids for infants who cannot maintain their hydration status with oral fluid intake. Educating parents on reducing the risk of infection is one of the most important things a physician can do to help prevent RSV infection, especially early in life. Children at risk of severe lower respiratory tract infection should receive immunoprophylaxis with palivizumab, a humanized monoclonal antibody, in up to five monthly doses. Prophylaxis guidelines are restricted to infants born before 29 weeks' gestation, infants with chronic lung disease of prematurity, and infants and children with hemodynamically significant heart disease.

Biopsy-verified bronchiolitis obliterans and other noninfectious lung pathologies after allogeneic hematopoietic stem cell transplantation

DEFF Research Database (Denmark)

Uhlving, Hilde Hylland; Andersen, Claus B; Christensen, Ib Jarle

2015-01-01

Institutes of Health's consensus criteria for BO syndrome (BOS) based exclusively on noninvasive measures. We included 44 patients transplanted between 2000 and 2010 who underwent lung biopsy for suspected BO. Of those, 23 were diagnosed with BO and 21 presented other noninfectious pulmonary pathologies...

Predicting Appropriate Admission of Bronchiolitis Patients in the Emergency Department: Rationale and Methods.

Science.gov (United States)

Luo, Gang; Stone, Bryan L; Johnson, Michael D; Nkoy, Flory L

2016-03-07

In young children, bronchiolitis is the most common illness resulting in hospitalization. For children less than age 2, bronchiolitis incurs an annual total inpatient cost of $1.73 billion. Each year in the United States, 287,000 emergency department (ED) visits occur because of bronchiolitis, with a hospital admission rate of 32%-40%. Due to a lack of evidence and objective criteria for managing bronchiolitis, ED disposition decisions (hospital admission or discharge to home) are often made subjectively, resulting in significant practice variation. Studies reviewing admission need suggest that up to 29% of admissions from the ED are unnecessary. About 6% of ED discharges for bronchiolitis result in ED returns with admission. These inappropriate dispositions waste limited health care resources, increase patient and parental distress, expose patients to iatrogenic risks, and worsen outcomes. Existing clinical guidelines for bronchiolitis offer limited improvement in patient outcomes. Methodological shortcomings include that the guidelines provide no specific thresholds for ED decisions to admit or to discharge, have an insufficient level of detail, and do not account for differences in patient and illness characteristics including co-morbidities. Predictive models are frequently used to complement clinical guidelines, reduce practice variation, and improve clinicians' decision making. Used in real time, predictive models can present objective criteria supported by historical data for an individualized disease management plan and guide admission decisions. However, existing predictive models for ED patients with bronchiolitis have limitations, including low accuracy and the assumption that the actual ED disposition decision was appropriate. To date, no operational definition of appropriate admission exists. No model has been built based on appropriate admissions, which include both actual admissions that were necessary and actual ED discharges that were unsafe. The

Treating Bronchiolitis in Infants

Science.gov (United States)

... from RSV Fever and Pain Medicine: How Much to Give Your Child Last Updated 11/21/2015 Source Bronchiolitis and Your Young Child Brochure (Copyright © 2005 American Academy of Pediatrics, Updated 7/2014) The information contained on this Web site should not be used as a substitute ...

A prospective randomised trial comparing nasogastric with intravenous hydration in children with bronchiolitis (protocol) The comparative rehydration in bronchiolitis study (CRIB)

Science.gov (United States)

2010-01-01

Background Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV) or nasogastric (NG). The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the first to provide good quality evidence of whether nasogastric rehydration (NGR) offers benefits over intravenous rehydration (IVR) using the clinically relevant continuous outcome measure of duration of hospital admission. Methods/Design A prospective randomised multi-centre trial in Australia and New Zealand where children between 2 and 12 months of age with bronchiolitis, needing non oral fluid replacement, are randomised to receive either intravenous (IV) or nasogastric (NG) rehydration. 750 patients admitted to participating hospitals will be recruited, and will be followed daily during the admission and by telephone 1 week after discharge. Patients with chronic respiratory, cardiac, or neurological disease; choanal atresia; needing IV fluid resuscitation; needing an IV for other reasons, and those requiring CPAP or ventilation are excluded. The primary endpoint is duration of hospital admission. Secondary outcomes are complications, need for ICU admission, parental satisfaction, and an economic evaluation. Results will be analysed using t-test for continuous data, and chi squared for categorical data. Non parametric data will be log transformed. Discussion This trial will define the role of NGR and IVR in bronchiolitis Trail registration The trial is registered with the Australian and New Zealand Clinical Trials Registry - ACTRN12605000033640 PMID:20515467

A prospective randomised trial comparing nasogastric with intravenous hydration in children with bronchiolitis (protocol The comparative rehydration in bronchiolitis study (CRIB

Directory of Open Access Journals (Sweden)

Borland Meredith

2010-06-01

Full Text Available Abstract Background Bronchiolitis is the most common reason for admission of infants to hospital in developed countries. Fluid replacement therapy is required in about 30% of children admitted with bronchiolitis. There are currently two techniques of fluid replacement therapy that are used with the same frequency-intravenous (IV or nasogastric (NG. The evidence to determine the optimum route of hydration therapy for infants with bronchiolitis is inadequate. This randomised trial will be the first to provide good quality evidence of whether nasogastric rehydration (NGR offers benefits over intravenous rehydration (IVR using the clinically relevant continuous outcome measure of duration of hospital admission. Methods/Design A prospective randomised multi-centre trial in Australia and New Zealand where children between 2 and 12 months of age with bronchiolitis, needing non oral fluid replacement, are randomised to receive either intravenous (IV or nasogastric (NG rehydration. 750 patients admitted to participating hospitals will be recruited, and will be followed daily during the admission and by telephone 1 week after discharge. Patients with chronic respiratory, cardiac, or neurological disease; choanal atresia; needing IV fluid resuscitation; needing an IV for other reasons, and those requiring CPAP or ventilation are excluded. The primary endpoint is duration of hospital admission. Secondary outcomes are complications, need for ICU admission, parental satisfaction, and an economic evaluation. Results will be analysed using t-test for continuous data, and chi squared for categorical data. Non parametric data will be log transformed. Discussion This trial will define the role of NGR and IVR in bronchiolitis Trail registration The trial is registered with the Australian and New Zealand Clinical Trials Registry - ACTRN12605000033640

An international ISHLT/ATS/ERS clinical practice guideline:

DEFF Research Database (Denmark)

Meyer, Keith C; Raghu, Ganesh; Verleden, Geert M

2014-01-01

Bronchiolitis obliterans syndrome (BOS) is a major complication of lung transplantation that is associated with poor survival. The International Society for Heart and Lung Transplantation, American Thoracic Society, and European Respiratory Society convened a committee of international experts...... to March, 2013. The expert committee discussed the available research evidence upon which the updated definition of BOS, identified risk factors and recommendations are based. The committee followed the GRADE (Grading of Recommendation, Assessment, Development and Evaluation) approach to develop specific......, and several risk factors have been identified that have a significant association with the onset of BOS. Currently available therapies have not been proven to result in significant benefit in the prevention or treatment of BOS. Adequately designed and executed randomised controlled trials that properly...

Belatacept for Maintenance Immunosuppression in Lung Transplantation

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Christine Hui PharmD

2014-06-01

Full Text Available Belatacept is a novel immunosuppressant that blocks a T-cell costimulation pathway and is approved for use in adult kidney transplant recipients. Its safety and efficacy have not been established after lung transplantation. We present a case of a lung transplant recipient treated with belatacept. A 56-year-old man underwent bilateral lung retransplantation for bronchiolitis obliterans syndrome (BOS. In the third year posttransplant, he developed hemolytic uremic syndrome (HUS attributed to tacrolimus. Tacrolimus was changed to sirolimus. One month later, he presented with worsening renal function and HUS attributed to sirolimus. Plasmapheresis and steroid pulse were initiated with clinical improvement, and sirolimus was switched to belatacept. He experienced no episodes of cellular rejection but developed recurrent BOS. Complications during treatment included anemia and recurrent pneumonias. The safety and efficacy of belatacept in lung transplantation remains unclear; further studies are needed.

Oxidative and antioxidative status of children with acute bronchiolitis

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Rusen Dundaroz

2013-07-01

Full Text Available Objective: Oxidative stress has been shown to contribute to the pathogenesis of acute and chronic lung inflammatory diseases. This article aimed to evaluate the oxidant/antioxidant status of children with acute bronchiolitis through the measurement of plasma total antioxidant capacity, total oxidant status, and oxidative stress index. Methods: Children with acute bronchiolitis admitted to the pediatric emergency department of a university hospital between January and April of 2012 were compared with age-matched healthy controls. Patients with acute bronchiolitis were classified as mild and moderate bronchiolitis. Oxidative and antioxidative status were assessed by measurement of plasma total antioxidant capacity, total oxidant status, and oxidative stress index. Results: Thirty-one children with acute bronchiolitis aged between 3 months and 2 years, and 39 healthy children were included. Total oxidative status (TOS was higher in patients with acute bronchiolitis than the control group (5.16 ± 1.99 μmol H2O2 versus 3.78 ± 1.78 μmol H2O2 [p = 0.004]. Total antioxidant capacity (TAC was lower in children with bronchiolitis than the control group (2.51 ± 0.37 μmol Trolox eqv/L versus 2.75 ± 0.39 μmol Trolox eqv/L [p = 0.013]. Patients with moderate bronchiolitis presented higher TOS levels than those with mild bronchiolitis and the control group (p = 0.03, p < 0.001, respectively. Patients with moderate bronchiolitis had higher oxidative stress index levels than the control group (p = 0.015. Oxygen saturation level of bronchiolitis patients was inversely correlated with TOS (r = −0.476, p < 0.05. Conclusion: The balance between oxidant and antioxidant systems is disrupted in children with moderate bronchiolitis, which indicates that this stress factor may have a role in the pathogenesis of the disease. Resumo: Objetivo: O estresse oxidativo demonstrou contribuir para a patogênese de doenças pulmonares inflamat

Immunomodulatory constituents of human milk change in response to infant bronchiolitis.

Science.gov (United States)

Bryan, Dani-Louise; Hart, Prue H; Forsyth, Kevin D; Gibson, Robert A

2007-09-01

Although epidemiological evidence is generally supportive of a causal association between respiratory syncytial virus (RSV) bronchiolitis during infancy and the development of persistent wheeze/asthma, if not allergy, the mechanism by which this occurs and an explanation for why all children do not succumb remains to be elucidated. Breast feeding has been found to confer a protective effect against respiratory infections such as RSV bronchiolitis and allergy; however, again there is little direct evidence and no clear mechanism. In this study, we examined whether human milk immunomodulatory factors (cells, cytokines) change in response to clinically diagnosed, severe bronchiolitis in the recipient breast-fed infant. We examined milk from 36 breast feeding mothers of infants hospitalized with bronchiolitis and compared them with milk from 63 mothers of postpartum age-matched healthy controls. Milks from mothers of infants hospitalized with bronchiolitis had significantly greater numbers of viable cells when compared with the milks obtained from mothers of healthy infants (1.3 +/- 0.4 vs. 0.3 +/- 0.03 x 10(6) cells/ml, mean +/- s.e.m.; p respiratory infections as well as a possible contributing factor to the development of persistent wheeze in these infants.

Cerebral angiographic findings in thromboangiitis obliterans

International Nuclear Information System (INIS)

No, Young J.; Lee, Eun M.; Kim, Jong S.; Lee, Deok H.

2005-01-01

Transient ischemic attacks (TIAs) or ischemic stroke may complicate thromboangiitis obliterans (TAO). However, there has been debate regarding the mechanism of ischemic stroke in TAO. We report the case of a patient with TAO who developed repeated TIAs. An angiogram showed multiple alternative areas of arterial occlusions in the distal segments of both middle cerebral arteries. Extensive collateral vessels around the occluded segment were also observed, which resembled the ''tree root'' or ''corkscrew'' vessels described in the peripheral arteries in TAO. Our patient illustrates that cerebral manifestations of TAO may occur with vascular changes that are identical with those encountered in the limb arteries in TAO. (orig.)

Browse Title Index

African Journals Online (AJOL)

Items 51 - 100 of 350 ... South African Journal of Radiology. ... Vol 9, No 2 (2005), Bronchiolitis obliterans organising pneumonia (BOOP) or ... Vol 9, No 4 (2005), Can ulceration in carcinoma of the oesophagus be detected by 99mTc-sucralfate scan? ... encephalopathy with CT confirmation in four children with acute renal ...

Immunomodulatory constituents of human breast milk and immunity from bronchiolitis.

Science.gov (United States)

Li, Chunyu; Liu, Yanbo; Jiang, Yanfang; Xu, Naijun; Lei, Jie

2017-01-14

The mother's immune status can be achieved by genetic and breastfeeding impact descendants of the immune system. The study aimed to determine whether a mother's immune status and breastfeeding practices were related to development of bronchiolitis in her infant. The frequency of T, B and natural kill (NK) cells in patients' blood and their mothers' breast milk was determined using flow cytometry. The concentrations of serum and breast milk IgG and IgD in individual patients and healthy control were determined by enzyme-linked immunosorbent assay (ELISA). The relationships between immunocytes, immunoglobulin and respiratory score (RS) were analyzed by Spearman's rank correlation test. The mothers of bronchiolitis patients had lower IgG concentrations in their breast milk when compared to the mothers of healthy children. There was no significant difference in the frequency of T cells, B cells, and NK cells in samples of breast milk. However, significant decreases of CD3+, CD8+ T cells, as well as significant increases of CD4+ T cells and CD19+ B cells were found in the serum of bronchiolitis infants. There were positive correlation relationships between RS and CD3+, CD4+ T cells, IgG and IgD concentrations. Our data suggested that the mothers of bronchiolitis patients had lower IgG concentration in their breast milk. The breast milk IgG might be absorbed by the breastfeeding infants, which could play important role in resistance of bronchiolitis.

Associations of Nasopharyngeal Metabolome and Microbiome with Severity among Infants with Bronchiolitis. A Multiomic Analysis.

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Stewart, Christopher J; Mansbach, Jonathan M; Wong, Matthew C; Ajami, Nadim J; Petrosino, Joseph F; Camargo, Carlos A; Hasegawa, Kohei

2017-10-01

Bronchiolitis is the most common lower respiratory infection in infants; however, it remains unclear which infants with bronchiolitis will develop severe illness. In addition, although emerging evidence indicates associations of the upper-airway microbiome with bronchiolitis severity, little is known about the mechanisms linking airway microbes and host response to disease severity. To determine the relations among the nasopharyngeal airway metabolome profiles, microbiome profiles, and severity in infants with bronchiolitis. We conducted a multicenter prospective cohort study of infants (age metabolomic and metagenomic (16S ribosomal RNA gene and whole-genome shotgun sequencing) approaches to 144 nasopharyngeal airway samples collected within 24 hours of hospitalization, we determined metabolome and microbiome profiles and their association with higher severity, defined by the use of positive pressure ventilation (i.e., continuous positive airway pressure and/or intubation). Nasopharyngeal airway metabolome profiles significantly differed by bronchiolitis severity (P metabolomics to predict bronchiolitis severity and better understand microbe-host interaction.

Clinical Predictors of Hospital Admission in Children Aged 0-24 Months with Acute Bronchiolitis

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Nucksheeba Aziz

2015-03-01

Full Text Available BACKGROUND Bronchiolitis is a significant cause of acute morbidity in children less than 2 years old and some children with bronchiolitis are admitted to the hospital. AIMS AND OBJECTIVES To identify clinical predictors of hospital admission in children aged 0-24 months with acute bronchiolitis.   METHODS: All children in the age group of 0-24 months presenting with acute bronchiolitis to a dedicated pediatric emergency department of GB pant cantonment children hospital, Govt Medical College Srinagar,   from April 2012-March 2013 were included in the study, provided they met the inclusion criteria. Non-parametric numerical variables were analyzed using Mann Whitney u test. Chi square was used to analyzecategorical variables, p value < 0.05 was considered significant. RESULTS: 763(552(72.3% male, mean age 8.52+/- 3.59 months children (0-24months presented with acute bronchiolitis during the study period. 435(313 (72% male, mean age 6.69+/-3.8 monthspatients were admitted to the hospital. The eight best predictors of admission (age, respiratory rate, heart rate, oxygen saturation, fever, grunt, dehydration and duration of symptoms were determined. CONCLUSION:This study has identified clinical predictors of admission in children aged 0-24 months with acute bronchiolitis. This information can be used as a guide in deciding whether to admit a child with bronchiolitis.

Prevention and treatment of respiratory syncytial virus bronchiolitis and postbronchiolitic wheezing

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Kimpen Jan LL

2002-06-01

Full Text Available Abstract Respiratory syncytial virus (RSV is the primary cause of hospitalization for acute respiratory tract illness in general and specifically for bronchiolitis in young children. The link between RSV bronchiolitis and reactive airway disease is not completely understood, even though RSV bronchiolitis is frequently followed by recurrent episodes of wheezing. Therapy with ribavirin does not appear to significantly reduce long-term respiratory outcome of RSV lower respiratory tract infection, and corticosteroid or bronchodilator therapy may possibly improve outcomes only on a short-term basis. No vaccine against RSV is yet available. It is not known whether prophylaxis with RSV intravenous immune globulin or palivizumab can reduce postbronchiolitic wheezing.

Clinical profile of bronchiolitis in infants younger than 90 days in Saudi Arabia

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Syed Amir Ahmad

2014-01-01

Full Text Available Background: Bronchiolitis is a self-limiting disease of children caused by viral infections of the small airways with a wide spectrum of illness severity. Search of the literature reveals a need for refinement of criteria for testing for concomitant severe bacterial infections as well as appropriate therapeutic interventions for patients <90-day-old diagnosed with clinical bronchiolitis. We believe that a better understanding of the disease spectrum will help optimize health-care delivery to these patients. Aims: The aim of this study was to determine the clinical profile at presentation, disease course and outcome of bronchiolitis in <3-month-old infants who presented to our Pediatric Emergency Department (PED during one disease season. Settings: Retrospective chart review during one bronchiolitis season, from November 1, 2011 to April 20, 2012. Subjects: All <90-day-old infants presenting with clinical bronchiolitis presenting to Urban PED of a tertiary care university hospital during one bronchiolitis season. Materials and Methods: A retrospective chart review based on computer records of all emergency department visits of infants less than 90 days with a clinical diagnosis of bronchiolitis, covering the period between November 1, 2011 and April 30, 2012. Results: Out of the total of 1895 infants <90 days of age, 141 had a clinical diagnosis of bronchiolitis and 35 needed admission to hospital. Blood for culture was obtained from 47 infants, urine for culture was obtained from 46 infants and cerebrospinal fluid for culture was obtained from eight infants. One case of bacteremia was documented, but this was found to be a contaminant. No cases of meningitis occurred among these infants. However, one infant had a positive urine culture consistent with infection (Escherichia coli . Conclusion: Based on the results, it can be conclude that the risk of bacteremia or meningitis among infants <90 days of age with fever and bronchiolitis is low. The

[Obliterative bronchiolitis with organising pneumonia following FOLFOX 4 chemotherapy].

Science.gov (United States)

Dahlqvist, C; Fremault, A; Carrasco, J; Colinet, B

2010-01-01

FOLFOX 4 chemotherapy (5-fluorouracil, leucovorin and oxaliplatin) is the standard adjuvant treatment for stage III colon cancer. The principal secondary effects described are haematological, gastro-intestinal or neurological. A single case of obliterative bronchiolitis with organising pneumonia has been described recently. We report the case of a female patient aged 74 years who, after 12 courses of FOLFOX 4 chemotherapy, developed acute onset of severe shortness of breath and a dry cough but remained afebrile. A thoracic CT-scan showed symmetrical bilateral interstitial infiltration that was reticular in appearance, and predominantly basal and peripheral in distribution. Broncho-alveolar lavage revealed an alveolitis with 9% eosinophils and 4% neutrophils. Transbronchial biopsies showed the appearances of obliterative bronchiolitis with organising pneumonia. Systemic corticosteroid treatment led to a remarkable clinical and functional improvement. To our knowledge, this is the second case of obliterative bronchiolitis with organising pneumonia that has been described following adjuvant treatment based on FOLFOX 4.

Increased Risk of Pneumonia and Bronchiolitis after Bacterial Colonization of the Airways as Neonates

DEFF Research Database (Denmark)

Vissing, Nadja Hawwa; Chawes, Bo Lk; Bisgaard, Hans

2013-01-01

Rationale: The frequency of pneumonia and bronchiolitis exhibits considerable variation in otherwise healthy children, and suspected risk factors explain only a minor proportion of the variation. We hypothesized that alterations in the airway microbiome in early life may be associated with suscep......Rationale: The frequency of pneumonia and bronchiolitis exhibits considerable variation in otherwise healthy children, and suspected risk factors explain only a minor proportion of the variation. We hypothesized that alterations in the airway microbiome in early life may be associated...... with susceptibility to pneumonia and bronchiolitis in young children. Objectives: To investigate the relation between neonatal airway colonization and pneumonia and bronchiolitis during the first three years of life. Methods: Participants comprised children of the COPSAC2000 cohort; a prospective birth cohort study...... of 411 children born to asthmatic mothers. Aspirates from the hypopharynx at age four weeks were cultured for S.pneumoniae, H.influenzae, M.catarrhalis, and S.aureus. Clinical information on pneumonia and bronchiolitis within the first three years of life was prospectively collected by the research...

Occupational (? constrictive bronchiolitis with normal physical, functional and image findings

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Sandra Figueiredo

2009-07-01

Full Text Available Constrictive bronchiolitis is characterized by alterations in the walls of membranous and respiratory bronchioles. These changes lead to concentric narrowing or complete obliteration of the airway lumen. Suspicion of possible bronchiolar disorders may arise from clinical, funcional, and radiologic findings. However, constrictive bronchiolitis may be present even with normal physical, functional and image findings, which turns the diagnosis difficult. A high index of suspicion is necessary to justify invasive tests that lead to pulmonary biopsy. In this report, we describe a patient with cough and dyspnoea, with normal physical, functional and image findings, whose work-up leaded to the diagnosis of constrictive bronchiolitis. Resumo: A bronquiolite constritiva é caracterizada por alterações das paredes dos bronquíolos membranosos e respiratórios. Estas alterações incluem um espectro de alterações que podem variar, desde a inflamação à fibrose concêntrica progressiva, com obstrução completa do lúmen bronquiolar. O diagnóstico pode ser sugerido pela história clínica e por alterações radiológicas e funcionais. No entanto, o exame físico e os exames complementares de diagnóstico podem ser normais, o que dificulta o diagnóstico, sendo necessário um elevado índice de suspeita para se sujeitar o doente a exames invasivos, tal como a biópsia pulmonar cirúrgica. Os autores apresentam um caso clínico de uma doente com quadro arrastado de tosse e dispneia, com exame físico, funcional e imagiológico normais, cujo estudo exaustivo veio a revelar o diagnóstico de bronquiolite constritiva. Key-words: Constrictive bronchiolitis, iron oxide, Palavras-chave: Bronquiolite constritiva, óxido de ferro

Bronchiolitis in US emergency departments 1992 to 2000: epidemiology and practice variation.

Science.gov (United States)

Mansbach, Jonathan M; Emond, Jennifer A; Camargo, Carlos A

2005-04-01

To describe the epidemiology of US emergency department (ED) visits for bronchiolitis, including the characteristics of children presenting to the ED and the variability in bronchiolitis care in the ED. Data were obtained from the 1992 to 2000 National Hospital Ambulatory Medical Care Survey. Cases had International Classification of Diseases, Ninth Revision, Clinical Modification code 466 and were younger than 2 years. National estimates were obtained using assigned patient visit weights; 95% confidence intervals were calculated using the relative standard error of the estimate; analysis used chi2 and logistic regression. From 1992 to 2000, bronchiolitis accounted for approximately 1,868,000 ED visits for children younger than 2 years. Among this same age group, the overall rate was 26 (95% confidence interval 22-31) per 1000 US population and 31 (95% confidence interval 26-36) per 1000 ED visits. These rates were stable over the 9-year period. Comparing children with bronchiolitis to those presenting with other problems, children with bronchiolitis were more likely boys (61% vs. 53%; P = 0.01) and Hispanic (27% vs. 20%; P = 0.008). Therapeutic interventions varied and 19% were admitted to the hospital. The multivariate predictor for receiving systemic steroids was urgent/emergent status at triage (odds ratio 4.0, 1.9-8.4). Multivariate predictors for admission were Hispanic ethnicity (odds ratio 2.3, 1.1-5.0) and urgent/emergent status at triage (odds ratio 3.7, 2.0-6.9). ED visit rates for bronchiolitis among children younger than 2 years were stable between 1992 and 2000. The observed ED practice variation demonstrates that children are receiving medications for which there is little supporting evidence. Boys and Hispanics are at-risk groups for presentation to the ED, and Hispanics are more likely to be hospitalized.

Bronchiolitis-associated encephalopathy in critically-ill infants: an underestimated complication?

Science.gov (United States)

Antonucci, Roberto; Chiappe, Stefano; Porcella, Annalisa; Rosatelli, Daniela; Fanos, Vassilios

2010-05-01

To investigate the bronchiolitis-associated encephalopathy in critically ill infants. The records of infants with severe bronchiolitis admitted to our intensive care unit between 1991 and 2003 were reviewed. Subjects with underlying neurological disorders were excluded. Encephalopathy was defined as occurrence of seizures or at least two nonconvulsive neurologic manifestations. A semistructured telephone interview investigated long-term neurodevelopmental outcome. Twenty-one infants (11 newborns) were enrolled. All patients required oxygen supplementation and 14 required mechanical ventilation. Encephalopathy occurred in 10 infants, six of whom developed seizures. Encephalopathic infants frequently (six of nine) showed transient EEG abnormalities, and occasionally (one of nine) cranial ultrasound abnormalities. A positive respiratory syncytial virus test was found in five of nine encephalopathic infants. One encephalopathic patient died, while 20 infants clinically normalised before discharge and showed a good neurodevelopmental outcome. Acute encephalopathy was frequently observed in our patients with severe bronchiolitis. Long-term prognosis of encephalopathic infants was good.

Inhibiting CXCL12 blocks fibrocyte migration and differentiation and attenuates bronchiolitis obliterans in a murine heterotopic tracheal transplant model.

Science.gov (United States)

Harris, David A; Zhao, Yunge; LaPar, Damien J; Emaminia, Abbas; Steidle, John F; Stoler, Mark; Linden, Joel; Kron, Irving L; Lau, Christine L

2013-03-01

Fibrocytes are integral in the development of fibroproliferative disease after lung transplantation. Undifferentiated fibrocytes (CD45+anti-collagen 1+CXCR4+) preferentially traffic by way of the CXCR4/CXCL12 axis and differentiate into smooth muscle actin-producing (CD45+CXCR4+α-smooth muscle actin+) cells. We postulated that an antibody directed against CXCL12 would attenuate fibrocyte migration and fibro-obliteration of heterotopic tracheal transplant allografts. A total alloantigenic mismatch murine heterotopic tracheal transplant model of obliterative bronchiolitis was used. The mice were treated with either goat-anti-human CXCL12 F(ab')(2) or goat IgG F(ab')(2). Buffy coat, bone marrow, and trachea allografts were collected and analyzed using flow cytometry. Tracheal luminal obliteration was assessed using hematoxylin-eosin and Direct Red 80 collagen stain. Compared with the controls, the anti-CXCL12-treated mice showed a significant decrease in tracheal allograft fibrocyte populations at 7 and 21 days after transplantation. Bone marrow and buffy coat aspirates showed the same trend at 7 days. In the anti-CXCL12-treated mice, there was a 35% decrease in luminal obliteration at 21 days (65% vs 100% obliterated; interquartile range, 38% vs 10%; P = .010) and decreased luminal collagen deposition at 21 and 28 days after transplantation (P = .042 and P = .012, respectively). Understanding the role of fibrocytes in airway fibrosis after lung transplantation could lead to a paradigm shift in treatment strategy. Anti-CXCL12 antibody afforded protection against infiltrating fibrocytes and reduced the deterioration of the tracheal allografts. Thus, the CXCR4/CXCL12 axis is a novel target for the treatment of fibro-obliteration after lung transplantation, and the quantification of fibrocyte populations could provide clinicians with a biomarker of fibrosis, allowing individualized drug therapy. Copyright © 2013 The American Association for Thoracic Surgery. Published

Respiratory mechanics in infants with severe bronchiolitis on controlled mechanical ventilation.

Science.gov (United States)

Cruces, Pablo; González-Dambrauskas, Sebastián; Quilodrán, Julio; Valenzuela, Jorge; Martínez, Javier; Rivero, Natalia; Arias, Pablo; Díaz, Franco

2017-10-06

Analysis of respiratory mechanics during mechanical ventilation (MV) is able to estimate resistive, elastic and inertial components of the working pressure of the respiratory system. Our aim was to discriminate the components of the working pressure of the respiratory system in infants on MV with severe bronchiolitis admitted to two PICU's. Infants younger than 1 year old with acute respiratory failure caused by severe bronchiolitis underwent neuromuscular blockade, tracheal intubation and volume controlled MV. Shortly after intubation studies of pulmonary mechanics were performed using inspiratory and expiratory breath hold. The maximum inspiratory and expiratory flow (QI and QE) as well as peak inspiratory (PIP), plateau (PPL) and total expiratory pressures (tPEEP) were measured. Inspiratory and expiratory resistances (RawI and RawE) and Time Constants (K TI and K TE ) were calculated. We included 16 patients, of median age 2.5 (1-5.8) months. Bronchiolitis due to respiratory syncytial virus was the main etiology (93.8%) and 31.3% had comorbidities. Measured respiratory pressures were PIP 29 (26-31), PPL 24 (20-26), tPEEP 9 [8-11] cmH2O. Elastic component of the working pressure was significantly higher than resistive and both higher than threshold (tPEEP - PEEP) (P mechanics of infants with severe bronchiolitis receiving MV shows that the elastic component of the working pressure of the respiratory system is the most important. The elastic and resistive components in conjunction with flow profile are characteristic of restrictive diseases. A better understanding of lung mechanics in this group of patients may lead to change the traditional ventilatory approach to severe bronchiolitis.

Predictors of Inappropriate Use of Diagnostic Tests and Management of Bronchiolitis

Science.gov (United States)

Sarmiento, Lorena; Rojas-Soto, Gladys E.

2017-01-01

Background The aim of the present study was to determine predictors of inappropriate use of diagnostic tests and management of bronchiolitis in a population of hospitalized infants. Methods In an analytical cross-sectional study, we determined independent predictors of the inappropriate use of diagnostic tests and management of bronchiolitis in a population of hospitalized infants. We defined a composite outcome score as the main outcome variable. Results Of the 303 included patients, 216 (71.3%) experienced an inappropriate use of diagnostic tests and treatment of bronchiolitis. After controlling for potential confounders, it was found that atopic dermatitis (OR 5.30; CI 95% 1.14–24.79; p = 0.034), length of hospital stay (OR 1.48; CI 95% 1.08–2.03; p = 0.015), and the number of siblings (OR 1.92; CI 95% 1.13–3.26; p = 0.015) were independent predictors of an inappropriate use of diagnostic tests and treatment of the disease. Conclusions Inappropriate use of diagnostic tests and treatment of bronchiolitis was a highly prevalent outcome in our population of study. Participants with atopic dermatitis, a longer hospital stay, and a greater number of siblings were at increased risk for inappropriate use of diagnostic tests and management of the disease. PMID:28758127

Lack of effect of zinc sulfate on wheezing after bronchiolitis in infants less than two years

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R Abasi

2014-10-01

Full Text Available Backgrounds & aim: Wheezing after bronchiolitis is known as a risk factor for asthma. No cure or effective vaccine has been identified for such disease. The purpose of this study was to determine the effects of zinc sulfate on persistent wheezing after bronchiolitis in infants less than two years. Methods: The present randomized clinical trial study was conducted on 90 infants under two years of age suffering from the first attack with a clinical diagnosis of bronchiolitis wheezing hospitalized in Imam Sajjad Hospital, Yasuj. All infants were equally placed in test groups and control group. In addition to bronchiolitis usual treatment, test group received 20 mg of elemental zinc for seven days. Patient information including risk factors associated with wheezing and persistent wheezing were compared 48 hours and two weeks after treatment. Data were analyzed using chi-square and t tests. Results: In all 90 infants diagnosed with bronchiolitis, wheezing was observed. After 48 hours and two weeks wheezing was continued in 67 patients (74.4% and 54 patients (60% respectively Conclusion: The results showed Zinc sulfate had no effect on the improvement of wheezing after bronchiolitis.

Clinical spectrum of cryptogenic organising pneumonitis.

Science.gov (United States)

Bellomo, R; Finlay, M; McLaughlin, P; Tai, E

1991-01-01

Cryptogenic organising pneumonitis (bronchiolitis obliterans organising pneumonia) is an uncommon condition that often responds to steroids. It is characterised clinically by constitutional symptoms, pathologically by intra-alveolar organising fibrosis, and radiologically by patchy pulmonary infiltrates. Its full clinical spectrum and course are only partially described and understood. Six patients are described, seen over three years, with considerably diverse clinical and radiological presentations (two had diffuse lung infiltrates, two had peripheral lung infiltrates, and two had localised lobar involvement) and with very varying severity of disease (two with a life threatening illness, three with appreciable subacute constitutional symptoms, and one with mild symptoms). It is concluded that cryptogenic organising pneumonitis can present in various ways. A set of diagnostic criteria are proposed which will help in the recognition of this syndrome, which is probably underdiagnosed. Images PMID:1926023

Disease: H01873 [KEGG MEDICUS

Lifescience Database Archive (English)

Full Text Available ibed in all age groups, the frequency of underlying causes and potential prognoses are different for child...ren and adults. For example, OB in children is most often seen following a severe l...akal SP, Fan LL ... TITLE ... Bronchiolitis obliterans in children. ... JOURNAL ... Curr Opin Pediatr 20:272-8 (2008) DOI:10.1097/MOP.0b013e3282ff62e9

Viral etiology of bronchiolitis among pediatric inpatients in northern Taiwan with emphasis on newly identified respiratory viruses.

Science.gov (United States)

Chen, Yu-Wen; Huang, Yhu-Chering; Ho, Tai-Hua; Huang, Chung-Guei; Tsao, Kuo-Chien; Lin, Tzou-Yien

2014-04-01

Viral etiology of bronchiolitis in children in Taiwan has been fragmentary. We conducted a prospective study to figure out the viral epidemiology of bronchiolitis in Taiwan. From January 2009 to March 2011, a total of 113 children with bronchiolitis, aged culture, antigen test, and polymerase chain reaction. A total of 120 viruses were detected from 113 children. Positive viral etiology was identified in 86 (76%) children. Mixed viral pathogens were found in 28 cases (25%). Respiratory syncytial virus (RSV) was the most common pathogen and was identified in 43.4% of the cases. Human bocavirus (hBoV) was the second most common identified virus (in 19.5%), followed by human metapneumovirus (hMPV), rhinovirus, influenza viruses, and coronavirus OC43. In terms of clinical characteristics, no significant difference was found among the children with bronchiolitis either caused by different single or mixed viral infection. RSV was the most common etiologic agent for children with bronchiolitis in Taiwan. Newly identified viruses, including hMPV and hBoV, were also among the common causative agents. Clinical characteristics were not significantly different among the children with bronchiolitis caused by different viruses. Copyright © 2012. Published by Elsevier B.V.

A multicenter, randomized, controlled trial of dexamethasone for bronchiolitis.

Science.gov (United States)

Corneli, Howard M; Zorc, Joseph J; Mahajan, Prashant; Majahan, Prashant; Shaw, Kathy N; Holubkov, Richard; Reeves, Scott D; Ruddy, Richard M; Malik, Baqir; Nelson, Kyle A; Bregstein, Joan S; Brown, Kathleen M; Denenberg, Matthew N; Lillis, Kathleen A; Cimpello, Lynn Babcock; Tsung, James W; Borgialli, Dominic A; Baskin, Marc N; Teshome, Getachew; Goldstein, Mitchell A; Monroe, David; Dean, J Michael; Kuppermann, Nathan

2007-07-26

Bronchiolitis, the most common infection of the lower respiratory tract in infants, is a leading cause of hospitalization in childhood. Corticosteroids are commonly used to treat bronchiolitis, but evidence of their effectiveness is limited. We conducted a double-blind, randomized trial comparing a single dose of oral dexamethasone (1 mg per kilogram of body weight) with placebo in 600 children (age range, 2 to 12 months) with a first episode of wheezing diagnosed in the emergency department as moderate-to-severe bronchiolitis (defined by a Respiratory Distress Assessment Instrument score > or =6). We enrolled patients at 20 emergency departments during the months of November through April over a 3-year period. The primary outcome was hospital admission after 4 hours of emergency department observation. The secondary outcome was the Respiratory Assessment Change Score (RACS). We also evaluated later outcomes: length of hospital stay, later medical visits or admissions, and adverse events. Baseline characteristics were similar in the two groups. The admission rate was 39.7% for children assigned to dexamethasone, as compared with 41.0% for those assigned to placebo (absolute difference, -1.3%; 95% confidence interval [CI], -9.2 to 6.5). Both groups had respiratory improvement during observation; the mean 4-hour RACS was -5.3 for dexamethasone, as compared with -4.8 for placebo (absolute difference, -0.5; 95% CI, -1.3 to 0.3). Multivariate adjustment did not significantly alter the results, nor were differences detected in later outcomes. In infants with acute moderate-to-severe bronchiolitis who were treated in the emergency department, a single dose of 1 mg of oral dexamethasone per kilogram did not significantly alter the rate of hospital admission, the respiratory status after 4 hours of observation, or later outcomes. (ClinicalTrials.gov number, NCT00119002 [ClinicalTrials.gov].). Copyright 2007 Massachusetts Medical Society.

The Effects of Inhaled Steroids on Recurrent Wheeze After Acute Bronchiolitis

Directory of Open Access Journals (Sweden)

Patricia Green MD

2015-07-01

Full Text Available Background. Acute bronchiolitis infection during infancy is associated with an increased risk of asthma later in life. The objective of this study was to determine if inhaled steroids are effective in preventing the development of recurrent wheeze or asthma following acute bronchiolitis. Methods. Multiple databases and bibliographies of selected references were searched. Inclusion required (a a randomized controlled trial of inhaled steroids and control group, (b at least 2 weeks duration of therapy started during the acute phase of disease, and (c identification of the rate of recurrent wheeze or asthma at least 6 months after therapy. Results. Of 1410 studies reviewed, 8 reports were included in this meta-analysis (748 patients. The overall odds ratio for developing recurrent wheeze or asthma with treatment versus without treatment was 1.02 (95% confidence interval = 0.58-1.81. Conclusions. A course of inhaled steroids after acute bronchiolitis is not effective in preventing recurrent wheeze or asthma.

Obliterative bronchiolitis due to Mycoplasma pneumoniae infection in a child

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Isles, A.F.; Masel, J.; O' Duffy, J.

1987-02-01

A six-year-old girl presented with Mycoplasma pneumoniae involving the right upper and lower lobes. She made a slow but complete recovery with resolution of the radiological changes. She represented 5 years later with a productive cough, recurrent wheezing and physical and radiological signs suggestive of obliterative bronchiolitis. This diagnosis was confirmed by ventilation - perfusion (dV/dt/dQ/dt) lung scan, and bronchography. The case highlights the value of dV/dt/dQ/dt scanning in the diagnosis of obliterative bronchiolitis and confirms the previous reports that mycoplasma infections are not always benign.

3He-MRI in follow-up of lung transplant recipients

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Gast, Klaus Kurt; Zaporozhan, Julia; Ley, Sebastian; Biedermann, Alexander; Knitz, Frank; Eberle, Balthasar; Schmiedeskamp, Joerg; Heussel, Claus-Peter; Mayer, Eckhard; Schreiber, Wolfgang Guenter; Thelen, Manfred; Kauczor, Hans-Ulrich

2004-01-01

The aim of this study was to evaluate the possible contribution of 3 He-MRI to detect obliterative bronchiolitis (OB) in the follow-up of lung transplant recipients. Nine single- and double-lung transplanted patients were studied by an initial and a follow-up 3 He-MRI study. Images were evaluated subjectively by estimation of ventilation defect area and quantitatively by individually adapted threshold segmentation and subsequent calculation of ventilated lung volume. Bronchiolitis obliterans syndrome (BOS) was diagnosed using pulmonary function tests. At 3 He-MRI, OB was suspected if ventilated lung volume had decreased by 10% or more at the follow-up MRI study compared with the initial study. General accordance between pulmonary function testing and 3 He-MRI was good, although subjective evaluation of 3 He-MRI underestimated improvement in ventilation as obtained by pulmonary function tests. The 3 He-MRI indicated OB in 6 cases. According to pulmonary function tests, BOS was diagnosed in 5 cases. All diagnoses of BOS were also detected by 3 He-MRI. In 2 of these 5 cases, 3 He-MRI indicated OB earlier than pulmonary function tests. The results support the hypothesis that 3 He-MRI may be sensitive for early detection of OB and emphasize the need for larger prospective follow-up studies. (orig.)

Complementary and alternative medicine for the treatment of bronchiolitis in infants: A systematic review.

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Kua, Kok Pim; Lee, Shaun Wen Huey

2017-01-01

Bronchiolitis is a common cause of hospitalization among infants. The limited effectiveness of conventional medication has prompted the use of complementary and alternative medicine (CAM) as alternative or adjunctive therapy for the management of bronchiolitis. To determine the effectiveness and safety of CAM for the treatment of bronchiolitis in infants aged less than 2 years. A systematic electronic search was performed in Medline, Embase, CINAHL, AMED, and Cochrane Central Register of Controlled Trials (CENTRAL) from their respective inception to June 30, 2016 for studies evaluating CAM as an intervention to treat bronchiolitis in infants (1 month to 2 years of age). The CAM could be any form of treatment defined by the National Center for Complementary and Integrative Health (NCCIH) and was utilized either as a single agent or adjunctive therapy. The predefined primary outcome was length of hospital stay. Secondary outcomes were time to resolution of bronchiolitis symptoms, adverse events, and all other clinical outcomes reported by the included studies. The review identified 11 studies (8 randomized controlled trials and 3 cohort studies) examining four herbal preparations and four supplements used either as adjunctive or alternative therapy for bronchiolitis in 904 infants. Most studies were of moderate quality. Among six studies reporting on length of stay, a significant benefit was found for Chinese herbal medicine compared to ribavirin in one cohort study (n = 66) and vitamin D compared to placebo in one randomized controlled trial (n = 89). Studies of Chinese herbal medicine (4 studies, n = 365), vitamin D (1 study, n = 89), N-acetylcysteine (1 study, n = 100), and magnesium (2 studies, n = 176) showed some benefits with respect to clinical severity scores, oxygen saturation, and other symptoms, although data were sparse for any single intervention and the outcomes assessed and reported varied across studies. Only five studies reported on adverse events

Unilateral mastitis obliterans presented as a palpable breast mass in a patient with long-standing diabetes mellitus.

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Blay, J; Medina, R; Rausell, N; Fonfria, C; Atares, M; Requeni, L; Vilar, J

2012-01-01

Mastitis obliterans is an uncommon and late manifestation of ductal ectasia. We report a case of a woman with a long-term type 2 diabetes, referred to us because of a palpable right breast mass. Mammography showed an asymmetry in the palpated area. Ultrasonography was consistent with a an irregular, hypoechoic mass with indistinct margins and linear tracts to the skin. The biopsy showed a fibrotic component surrounding dilated galactophore ducts, which were collapsed by an infiltrate of lymphocytes and histiocytes corresponding to mastitis obliterans. The differential diagnosis should be made between diabetic fibrous mastopathy, granulomatous mastitis and lobular carcinoma. In our opinion, the therapeutic approach should depend on the symptomatology and should be individualized for each patient owing to the lack of information on this pathology, adopting therefore a conservative attitude.

Association Between Antibiotic Exposure, Bronchiolitis, and TLR4 (rs1927911) Polymorphisms in Childhood Asthma

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Lee, Eun; Kwon, Ji-Won; Kim, Hyo-Bin; Yu, Ho-Sung; Kang, Mi-Jin; Hong, Kyungmo; Yang, Song I; Jung, Young Ho; Lee, Seung-Hwa; Choi, Kil Young; Shin, Hye Lim; Hong, Seo Ah; Kim, Hyung Young; Seo, Ju-Hee; Kim, Byoung-Ju; Lee, So Yeon; Song, Dae Jin; Kim, Woo-Kyung; Jang, Gwang Cheon; Shim, Jung Yeon

2015-01-01

Purpose The complex interplay between environmental and genetic factors plays an important role in the development of asthma. Several studies have yielded conflicting results regarding the 2 asthma-related risk factors: antibiotic usage during infancy and/or a history of bronchiolitis during early life and the development of asthma. In addition to these risk factors, we also explored the effects of Toll-like receptor 4 (TLR4) polymorphism on the development of childhood asthma. Methods This cross-sectional study involved 7,389 middle school students who were from 8 areas of Seoul, Korea, and completed the International Study of Asthma and Allergies in Childhood questionnaire. The TLR4 polymorphism rs1927911 was genotyped in 1,395 middle school students from two areas using the TaqMan assay. Results Bronchiolitis in the first 2 years of life, antibiotic exposure during the first year of life, and parental history of asthma were independent risk factors for the development of asthma. When combined, antibiotic use and a history of bronchiolitis increased the risk of asthma (adjusted odds ratio [aOR]: 4.64, 95% confidence interval [CI]: 3.09-6.97, P value for interaction=0.02). In subjects with CC genotype of TLR4, antibiotic exposure and a history of bronchiolitis during infancy, the risk of asthma was increased, compared to subjects without these risk factors (aOR: 5.72, 95% CI: 1.74-18.87). Conclusions Early-life antibiotic exposures and a history of bronchiolitis are risk factors for asthma in young adolescents. Polymorphisms of TLR4 modified the influence of these environmental factors. Reducing antibiotic exposure and preventing bronchiolitis during infancy may prevent the development of asthma, especially in genetically susceptible subjects. PMID:25729624

Quality Improvement Initiative to Increase the Use of Nasogastric Hydration in Infants With Bronchiolitis.

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Srinivasan, Mythili; Pruitt, Cassandra; Casey, Erin; Dhaliwal, Keerat; DeSanto, Cori; Markus, Richard; Rosen, Ayelet

2017-08-01

Intravenous (IV) hydration is used primarily in children with bronchiolitis at our institution. Because nasogastric (NG) hydration can provide better nutrition, the goal of our quality improvement (QI) initiative was to increase the rate of NG hydration in eligible children 1 to 23 months old with bronchiolitis by 20% over 6 months. We used Plan-Do-Study-Act cycles to increase the use of NG hydration in eligible children. Interventions included educational and system-based changes and sharing parental feedback with providers. Chart reviews were performed to identify the rates of NG hydration, which were plotted over time in a statistical process control p chart. The balancing measure was the rate of complications in children with NG versus IV hydration. Two hundred and ninety-three children who were hospitalized with bronchiolitis needed supplemental hydration during the QI initiative (January 2016-April 2016). Ninety-one children were candidates for NG hydration, and 53 (58%) received NG hydration. The rates of NG hydration increased from a baseline of 0% pre-QI bronchiolitis season (January 2015-April 2015) to 58% during the initiative. There was no aspiration and no accidental placement of the NG tube into a child's airway. Nine patients (17%) in the NG group had a progression of disease requiring nil per os status, and 6 of these were transferred to the PICU whereas none of those in the IV group were transferred to the PICU. Post-QI initiative, the majority of nurses (63%) and physicians (95%) stated that they are more likely to consider NG hydration in children with bronchiolitis. We successfully increased the rates of NG hydration in eligible children with bronchiolitis by using educational and system-based interventions. Copyright © 2017 by the American Academy of Pediatrics.

Do we really ponder about necessity of intravenous hydration in acute bronchiolitis?

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Y?ld?r?m, ?ule; Kaymaz, Nazan; Topalo?lu, Naci; K?ksal Binneto?lu, Fatih; Tekin, Mustafa; Aylan?, Hakan; Battal, Fatih; G?nüllü, Bur?in

2016-01-01

Objective: The goal was to establish the role of intravenous hydration therapy on mild bronchiolitis. Methods: This was a retrospective case control study. Infants between 1 month and 2 years of age admitted to our general pediatrics ward between June 2012 and June 2013 with a diagnosis of uncomplicated acute bronchiolitis were enrolled to the study. Hospital medical files were reviewed to get information about children personal history, symptoms of the disease, disease severity scores and th...

Nasogastric Hydration in Infants with Bronchiolitis Less Than 2 Months of Age.

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Oakley, Ed; Bata, Sonny; Rengasamy, Sharmila; Krieser, David; Cheek, John; Jachno, Kim; Babl, Franz E

2016-11-01

To determine whether nasogastric hydration can be used in infants less than 2 months of age with bronchiolitis, and characterize the adverse events profile of these infants compared with infants given intravenous (IV) fluid hydration. A descriptive retrospective cohort study of children with bronchiolitis under 2 months of age admitted for hydration at 3 centers over 3 bronchiolitis seasons was done. We determined type of hydration (nasogastric vs IV fluid hydration) and adverse events, intensive care unit admission, and respiratory support. Of 491 infants under 2 months of age admitted with bronchiolitis, 211 (43%) received nonoral hydration: 146 (69%) via nasogastric hydration and 65 (31%) via IV fluid hydration. Adverse events occurred in 27.4% (nasogastric hydration) and 23.1% (IV fluid hydration), difference of 4.3%; 95%CI (-8.2 to 16.9), P = .51. The majority of adverse events were desaturations (21.9% nasogastric hydration vs 21.5% IV fluid hydration, difference 0.4%; [-11.7 to 12.4], P = .95). There were no pulmonary aspirations in either group. Apneas and bradycardias were similar in each group. IV fluid hydration use was positively associated with intensive care unit admission (38.5% IV fluid hydration vs 19.9% nasogastric hydration; difference 18.6%, [5.1-32.1], P = .004); and use of ventilation support (27.7% IV fluid hydration vs 15.1% nasogastric hydration; difference 12.6 [0.3-23], P = .03). Fewer infants changed from nasogastric hydration to IV fluid hydration than from IV fluid hydration to nasogastric hydration (12.3% vs 47.7%; difference -35.4% [-49 to -22], P hydration can be used in the majority of young infants admitted with bronchiolitis. Nasogastric hydration and IV fluid hydration had similar rates of complications. Copyright © 2016 Elsevier Inc. All rights reserved.

Oxidative and antioxidative status of children with acute bronchiolitis

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Rusen Dundaroz

2013-07-01

Conclusion: The balance between oxidant and antioxidant systems is disrupted in children with moderate bronchiolitis, which indicates that this stress factor may have a role in the pathogenesis of the disease.

Availability of platelet scintigraphy for evaluation of arteriosclerosis obliterans

International Nuclear Information System (INIS)

Morimoto, Yoshihisa; Sugimoto, Takashi; Okada, Masayoshi; Mukai, Tomoichiro

1998-01-01

We reviewed the availability of platelet scintigraphy which can detect thrombotic activity to evaluate arteriosclerosis obliterans (ASO). During the past 13 months, 15 cases with ASO were studied. Among them, 6 cases (40%) showed acute aggravations of ischemic symptoms along with abnormal accumulation on scintigram. These cases have instantly more intensive antiplatelet and anticoagulant therapy, and obtained a remarkable recuperation together with disappearance of abnormal accumulation. In contrast, 9 cases without abnormal accumulation had a stable course without any ischemic complaints. In conclusion, platelet scintigraphy is very useful to evaluate the clinical effects of conservative or surgical therapy during the follow-up period in patients with ASO. (author)

[An initiative to reduce the use of unnecessary medication in infants with bronchiolitis in primary care].

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Montejo Fernández, Marta; Benito Manrique, Iñaki; Montiel Eguía, Arantza; Benito Fernández, Javier

2018-05-23

Although evidence-based guidelines for acute bronchiolitis recommend primarily supportive care unnecessary treatments remain well documented. The objective of this study was to analyse a quality improvement initiative to reduce overuse of unnecessary treatments in infants with acute bronchiolitis in primary care settings. To determine the number of unnecessary treatments we reviewed the charts corresponding to infants aged initiative may be applied to the settings where the majority of infants with acute bronchiolitis are attended in western countries. Copyright © 2018. Publicado por Elsevier España, S.L.U.

Maternal milk protects infants against bronchiolitis during the first year of life. Results from an Italian cohort of newborns.

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Lanari, Marcello; Prinelli, Federica; Adorni, Fulvio; Di Santo, Simona; Faldella, Giacomo; Silvestri, Michela; Musicco, Massimo

2013-06-01

Bronchiolitis is one of the primary causes of hospitalization in infancy. We evaluated the effect of breastfeeding on the occurrence of hospitalization for bronchiolitis in the first year of life. In a prospective cohort study, 1,814 newborns of =33 weeks of gestational age (wGA) were enrolled in 30 Italian Neonatology Units and followed-up for 1 year to assess hospitalizations for bronchiolitis. Children were grouped as 'never breastfed' and 'ever breastfed'; these latter were further divided into those 'exclusively breastfed' and 'breastfed associated with milk formula'. The risk of hospitalization for bronchiolitis was evaluated with survival analysis, and hazard ratios (HR) with 95% confidence interval [95% CI] were calculated. Among enrolled newborns 22.9% were 'never breastfed'; in the breastfed group, 65% were 'exclusively breastfed' and 35% were 'breastfed with associated milk formula'. At 12 months of age, the risk of hospitalization for bronchiolitis was significantly higher in the 'never breastfed' group (HR: 1.57; 95% CI: 1.00-2.48). 'Breastfed associated with formula milk' and 'exclusively breastfed' groups were at similar risk of hospitalization for bronchiolitis. This observed protective effect of maternal milk was not explained by the higher prevalence of conditions able to increase the risk of bronchiolitis among 'never breastfed newborns'. Breastfeeding, even in association with formula milk, reduces the risk of hospitalization for bronchiolitis during the first year of life. Encouraging breastfeeding might be an effective/inexpensive measure of prevention of lower respiratory tract infections in infancy. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Etiological diagnosis reduces the use of antibiotics in infants with bronchiolitis

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Ângela Esposito Ferronato

2012-09-01

Full Text Available OBJECTIVE: Acute bronchiolitis is a leading cause of infant hospitalization and is most commonly caused by respiratory syncytial virus. Etiological tests are not required for its diagnosis, but the influence of viral screening on the therapeutic approach for acute bronchiolitis remains unclear. METHODS: A historical cohort was performed to assess the impact of viral screening on drug prescriptions. The study included infants up to one year of age who were hospitalized for bronchiolitis. Virus screening was performed using immunofluorescence assays in nasopharyngeal aspirates. The clinical data were obtained from the patients' medical records. Therapeutic changes were considered to be associated with viral screening when made within 24 hours of the release of the results. RESULTS: The frequency of prescriptions for beta agonists, corticosteroids and antibiotics was high at the time of admission and was similar among the 230 patients. The diagnosis of pneumonia and otitis was associated with the introduction of antibiotics but did not influence antibiotics maintenance after the results of the virus screening were obtained. Changes in the prescriptions were more frequent for the respiratory syncytial virus patients compared to patients who had negative viral screening results (p =0.004, especially the discontinuation of antibiotics (p<0.001. The identification of respiratory syncytial virus was associated with the suspension of antibiotics (p= 0.003, even after adjusting for confounding variables (p = 0.004; however, it did not influence the suspension of beta-agonists or corticosteroids. CONCLUSION: The identification of respiratory syncytial virus in infants with bronchiolitis was independently associated with the discontinuation of antibiotics during hospitalization

Etiological diagnosis reduces the use of antibiotics in infants with bronchiolitis.

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Ferronato, Ângela Esposito; Gilio, Alfredo Elias; Ferraro, Alexandre Archanjo; Paulis, Milena de; Vieira, Sandra E

2012-09-01

Acute bronchiolitis is a leading cause of infant hospitalization and is most commonly caused by respiratory syncytial virus. Etiological tests are not required for its diagnosis, but the influence of viral screening on the therapeutic approach for acute bronchiolitis remains unclear. A historical cohort was performed to assess the impact of viral screening on drug prescriptions. The study included infants up to one year of age who were hospitalized for bronchiolitis. Virus screening was performed using immunofluorescence assays in nasopharyngeal aspirates. The clinical data were obtained from the patients' medical records. Therapeutic changes were considered to be associated with viral screening when made within 24 hours of the release of the results. The frequency of prescriptions for beta agonists, corticosteroids and antibiotics was high at the time of admission and was similar among the 230 patients. The diagnosis of pneumonia and otitis was associated with the introduction of antibiotics but did not influence antibiotics maintenance after the results of the virus screening were obtained. Changes in the prescriptions were more frequent for the respiratory syncytial virus patients compared to patients who had negative viral screening results (p =0.004), especially the discontinuation of antibiotics (p<0.001). The identification of respiratory syncytial virus was associated with the suspension of antibiotics (p= 0.003), even after adjusting for confounding variables (p = 0.004); however, it did not influence the suspension of beta-agonists or corticosteroids. The identification of respiratory syncytial virus in infants with bronchiolitis was independently associated with the discontinuation of antibiotics during hospitalization.

The Use of Albuterol in Young Infants Hospitalized with Acute RSV Bronchiolitis

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Michael T. Del Vecchio

2012-01-01

Full Text Available Objective. To evaluate the effects of albuterol use in young infants admitted with respiratory syncytial virus (RSV bronchiolitis with regards to length of time on supplemental oxygen and length of stay (LOS. To consider the possibility that albuterol use may increase the need for supplemental oxygen and increase LOS. Design, Setting, and Participants. Full-term infants between the ages of 11 days and 90 days (=316 were included in this retrospective study. Infants included were hospitalized with a diagnosis of RSV bronchiolitis at a university-affiliated children’s hospital. Results. In 4 of 5 severity groups, patients who received albuterol required more time on supplemental oxygen and had longer LOS. The differences only reached statistical significance in one of the severity groups in regards to LOS. Conclusions. The use of albuterol does not appear to be useful in the treatment of young infants with RSV bronchiolitis and may actually be harmful, in regards to increased supplemental oxygen need.

Do we really ponder about necessity of intravenous hydration in acute bronchiolitis?

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Yıldırım, Şule; Kaymaz, Nazan; Topaloğlu, Naci; Köksal Binnetoğlu, Fatih; Tekin, Mustafa; Aylanç, Hakan; Battal, Fatih; Gön Uuml Ll Uuml, Burçin

2016-03-30

The goal was to establish the role of intravenous hydration therapy on mild bronchiolitis. This was a retrospective case control study. Infants between 1 month and 2 years of age admitted to our general pediatrics ward between June 2012 and June 2013 with a diagnosis of uncomplicated acute bronchiolitis were enrolled to the study. Hospital medical files were reviewed to get information about children personal history, symptoms of the disease, disease severity scores and their management. Patients were classified into 4 groups according to the management; nebulized short-acting β2-agonist (salbutamol) +hydration; nebulized short-acting β2-agonist (salbutamol); hydration and neither bronchodilator nor hydration. We examined length of stay in the hospital as an outcome measure. A total of 94 infants were studied. There was no significant difference between groups in terms of length of stay in hospital. IV hydration is not effective on length of stay in hospital in mild acute bronchiolitis patients.

Bilateral extensive ductitis obliterans manifested by bloody nipple discharge in a patient with long-term diabetes mellitus.

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Wang, Zhiqin; Leonard, Morton H; Khamapirad, Tuenchit; Castro, Claudia Y

2007-01-01

Ductitis obliterans or mastitis obliterans is a rare late manifestation of mammary ductal ectasia. We describe a long-term diabetic patient who presented with bilateral bloody nipple discharge and poorly defined nodularities around the nipple of both breasts. The ductography showed multiple segments of irregular ductal narrowing and intraluminal filling defects in both breasts. The bilateral resection of the sub-areolar portion of the breast showed exuberant fibrous obliteration of the large- and medium-sized ducts by granulation tissue associated with few histiocytes. Ductal dilatation and intraductal accumulation of histiocytes was also present. This represents a late and florid form of mammary ductal ectasia. Differential diagnostic considerations including fibrocystic changes, diabetic sclerosing lymphocytic lobulitis, idiopathic granulomatous lobular mastitis, and periductal mastitis (Zuska disease) are discussed. Accurate diagnosis can help avoid or limit radical surgeries in this group of patients.

Delayed recurrence of ulcerative colitis manifested by tracheobronchitis, bronchiolitis, and bronchiolectasis

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Iclal Ocak MD,

2017-12-01

Full Text Available Ulcerative colitis can cause inflammation of small and large airways, characterized by mucosal inflammation, tracheobronchial stenosis, bronchiestasis, and bronchiolitis. We present a case of tracheobronchitis and bronchiolitis associated with ulcerative colitis in a 58-year-old nonsmoking man, 17 years after the total colectomy and complete resolution of intestinal findings. Computed tomography demonstrated wall thickening of trachea and left main stem bronchus, and multiple bronchi around the both hilum with mild to moderate stenosis. Fiberoptic bronchial biopsy showed inflammation of the airways, similar to histologic findings of ulcerative colitis within colon. Keywords: Ulcerative colitis, Trachea, Lung

Interstitial inflammatory lesions of the pulmonary allograft: a retrospective analysis of 2697 transbronchial biopsies

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Burton, C.M.; Iversen, M.; Carlsen, J.

2008-01-01

. The strongest histological correlations were between ACR and lymphocytic bronchiolitis (OR 5.1, Pfibrosis (OR 3.2, Ppulmonary hemosiderosis were also more likely to demonstrate the finding of interstitial fibrosis (OR 3.0 and 3.7, P..., incidence and possible associations between commonly identified inflammatory and fibrotic lesions in the pulmonary allograft. METHODS: Retrospective chart review of all transbronchial biopsies performed within the first 2 years of 299 lung-transplanted patients in the period 1996 to 2006. RESULTS: A total...... (BOOP) and interstitial pneumonitis occurred at 4 to 6 weeks, and 6 to 12 weeks, respectively. There was a steady increase in the cumulative proportion of patients with fibrosis and bronchiolitis obliterans, at each successive scheduled surveillance time point beyond 3 months posttransplantation...

Acute viral bronchiolitis and risk of asthma in schoolchildren: analysis of a Brazilian newborn cohort.

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Brandão, Heli V; Vieira, Graciete O; Vieira, Tatiana O; Cruz, Álvaro A; Guimarães, Armênio C; Teles, Carlos; Camargos, Paulo; Cruz, Constança M S

To verify whether the occurrence of acute viral bronchiolitis in the first year of life constitutes a risk factor for asthma at age 6 considering a parental history of asthma. Cross-sectional study in a cohort of live births. A standardized questionnaire of the International Study of Asthma and Allergies in Childhood was applied to the mothers to identify asthma in children at the age of 6 years. Acute viral bronchiolitis diagnosis was performed by maternal report of a medical diagnosis and/or presence of symptoms of coryza accompanied by cough, tachypnea, and dyspnea when participants were 3, 6, 9, and 12 months. Socioeconomic, environmental data, parental history of asthma, and data related to pregnancy were collected in the first 72h of life of the newborn and in prospective home visits by trained interviewers. The association between acute viral bronchiolitis and asthma was evaluated by logistic regression analysis and potential modifier effect of parental history was verified by introducing an interaction term into the adjusted logistic regression model. Prevalence of acute viral bronchiolitis in the first year of life was 68.6% (461). The occurrence of acute viral bronchiolitis was a risk factor for asthma at 6 years of age in children with parental history of asthma OR: 2.66, 95% CI (1.10-6.40), modifier effect p=0.002. Parental history of asthma OR: 2.07, 95% CI (1.29-3.30) and male gender OR: 1.69, 95% CI, (1.06-2.69) were other identified risk factors for asthma. Acute viral bronchiolitis in the first year of life is a risk factor for asthma in children with parental history of asthma. Copyright © 2016. Published by Elsevier Editora Ltda.

Acute viral bronchiolitis and risk of asthma in schoolchildren: analysis of a Brazilian newborn cohort,

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Heli V. Brandão

Full Text Available Abstract Objective: To verify whether the occurrence of acute viral bronchiolitis in the first year of life constitutes a risk factor for asthma at age 6 considering a parental history of asthma. Methods: Cross-sectional study in a cohort of live births. A standardized questionnaire of the International Study of Asthma and Allergies in Childhood was applied to the mothers to identify asthma in children at the age of 6 years. Acute viral bronchiolitis diagnosis was performed by maternal report of a medical diagnosis and/or presence of symptoms of coryza accompanied by cough, tachypnea, and dyspnea when participants were 3, 6, 9, and 12 months. Socioeconomic, environmental data, parental history of asthma, and data related to pregnancy were collected in the first 72 h of life of the newborn and in prospective home visits by trained interviewers. The association between acute viral bronchiolitis and asthma was evaluated by logistic regression analysis and potential modifier effect of parental history was verified by introducing an interaction term into the adjusted logistic regression model. Results: Prevalence of acute viral bronchiolitis in the first year of life was 68.6% (461. The occurrence of acute viral bronchiolitis was a risk factor for asthma at 6 years of age in children with parental history of asthma OR: 2.66, 95% CI (1.10-6.40, modifier effect p = 0.002. Parental history of asthma OR: 2.07, 95% CI (1.29-3.30 and male gender OR: 1.69, 95% CI, (1.06-2.69 were other identified risk factors for asthma. Conclusion: Acute viral bronchiolitis in the first year of life is a risk factor for asthma in children with parental history of asthma.

Acute chemical pneumonitis caused by nitric acid inhalation: case report

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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.

Early extracellular matrix changes are associated with later development of bronchiolitis obliterans syndrome after lung transplantation

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Müller, Catharina; Andersson-Sjöland, Annika; Schultz, Hans Henrik

2017-01-01

are largely unknown. The aim of this study was to identify potential early changes in the extracellular matrix (ECM) in different compartments of the transplanted lung prior to the development of BOS. Methods: Transbronchial biopsies from a cohort of 58 lung transplantation patients at the Copenhagen...... and immunohistochemistry. Results: A time-specific and compartment-specific pattern of ECM changes was detected. Alveolar total collagen (p=0.0190) and small airway biglycan (p=0.0199) increased between 3 and 12 months after transplantation in patients developing BOS, while collagen type IV (p=0.0124) increased...... in patients without BOS. Patients with early-onset BOS mirrored this increase. Patients developing grade 3 BOS showed distinct ECM changes already at 3 months. Patients with BOS with treated acute rejections displayed reduced alveolar total collagen (p=0.0501) and small airway biglycan (p=0.0485) at 3 months...

Preliminary clinical application of SilverHawk directional atherectomy device in arteriosclerosis obliterans of lower extremity

International Nuclear Information System (INIS)

Qian Jun; Jiang Hong; Xu Gang; Yang Xiaohong

2011-01-01

Objective: To evaluate the clinical efficiency and security of SilverHawk directional atherectomy device in treating arteriosclerosis obliterans of lower extremity. Methods: Seven patients with lower extremity ischemia were treated with SilverHawk directional atherectomy. The lesions length varied from 1.8 to 14.5 cm. Clinical symptoms (Fontaine classification) included 4 cases of grade Ⅱ b, I case of grade Ⅲ, 2 cases of grade Ⅳ. The ABI varied from 0.28 to 0.65. Patency was evaluated with color duplex sonography or CTA besides clinical examination during follow-up. Results: Seven occlusive lesions were recanalizated successfully. The technical success (residual stenosis < 30%) rate was 100%. Clinical symptoms were obviously improved or disappeared. The ABI varied from 0.82 to 1.18 (P=0.002), and Fontaine classification included 6 cases of Ⅰ; 1 case of Ⅱ a. Patency rate was 100% and the ABI varied from 0.75 to 1.14 (P=0.938). Fontaine classifications keep consistent 1-6 months after operation. Conclusion: SilverHawk directional atherectomy was a safe and effective way for treatment of lower arteriosclerosis obliteran. (authors)

Granulomatous Bronchiolitis with Necrobiotic Pulmonary Nodules in Chrohn's Disease

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Hugh J Freeman

2004-01-01

Full Text Available A 37-year-old man with extensive Crohn's disease of the stomach, small and large intestine for almost a decade developed respiratory symptoms and radiological findings suggestive of pneumonia that failed to resolve with antibiotic treatment. Computed tomography scanning of his lungs showed extensive changes with cavitated parenchymal nodules. Histological evaluation of an open lung biopsy showed granulomatous bronchiolitis and pulmonary necrobiosis. Treatment with steroids and immunosuppression resulted in complete resolution of his clinical symptoms of pneumonia and abnormal computed tomography imaging changes. Granulomatous bronchiolitis and necrobiotic nodules may be a manifestation of Crohn's disease in the absence of microbial agents, including mycobacteria or fungal agents. While a multiplicity of complex pulmonary changes may occur in Crohn's disease, their clinical recognition and precise pathological definition may be particularly important if treatment with a biological agent, such as infliximab, is being considered.

Diagnostic testing for serious bacterial infections in infants aged 90 days or younger with bronchiolitis.

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Liebelt, E L; Qi, K; Harvey, K

1999-05-01

To describe the different laboratory tests that are performed on young infants aged 90 days or younger with bronchiolitis and to identify historical and clinical predictors of infants on whom laboratory tests are performed. Cross-sectional study whereby information was obtained by retrospective review of medical records from November through March 1992 to 1995 of all infants with a clinical diagnosis of bronchiolitis. Urban pediatric emergency department. Two hundred eleven consecutive infants aged 90 days or younger (median age, 54 days) with 216 episodes of bronchiolitis. Historical and clinical data on each infant in addition to laboratory data that included a white blood cell count, urinalysis, and blood, urine, and cerebrospinal fluid cultures. Two or more laboratory tests (not including chest radiographs) were obtained in 48% of all infants and 78% of febrile infants. Of the 91 infants with a history of a temperature of 38.0 degrees C or more or temperature on presentation of 38.0 degrees C or more, white blood cell counts were obtained in 77%, blood cultures in 75%, urinalyses in 53%, urine cultures in 60%, and analyses-cultures of cerebrospinal fluid in 47%. Febrile infants were 10 times more likely to get at least 2 laboratory tests than afebrile infants (Ppredictors of whether laboratory studies were obtained. History of preterm gestation, aged younger than 28 days, previous antibiotic use, and presence of otitis media were not associated with obtainment of laboratory studies. No cases of bacteremia, urinary tract infection, or meningitis were found among all infants with bronchiolitis who had blood, urine, and/or cerebrospinal fluid cultures. There is wide variability in the diagnostic testing of infants aged 90 days or younger with bronchiolitis. The risks of bacteremia, urinary tract infection, and meningitis in infants with bronchiolitis seems to be low. History or a documented temperature of 38.0 degrees C or more; oxygen saturation of less than 92

A 47-Year-Old Man With Fever, Dry Cough, and a Lung Mass After Redo Lung Transplantation.

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Chaddha, Udit; Patil, Pradnya D; Omar, Ashraf; Walia, Rajat; Panchabhai, Tanmay S

2018-06-01

A 47-year-old man who was a redo double lung transplant recipient (cytomegalovirus [CMV] status: donor positive/recipient positive; Epstein-Barr virus status: donor positive/recipient positive) presented to the hospital with 1 week of generalized malaise, low-grade fevers, and dry cough. His redo lung transplantation was necessitated by bronchiolitis obliterans syndrome, and his previous lung transplantation 5 years earlier was for silicosis-related progressive massive fibrosis. He denied any difficulty breathing or chest pain. There was no history of GI or urinary symptoms, and the patient had no anorexia, weight loss, night sweats, sick contacts, or history of travel. He had a history of 1 earlier episode of CMV viremia that was treated with valganciclovir. His immunosuppressive regimen included tacrolimus, mycophenolate mofetil, and prednisone, and his infection prophylaxis included trimethoprim-sulfamethoxazole, itraconazole, and valganciclovir. Results of a chest radiograph 8 weeks earlier were normal. Copyright © 2017 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.

C Reactive protein and disease severity in bronchiolitis

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S. Costa

2009-01-01

Full Text Available A diagnosis of bronchiolitis is made clinically and the use of supportive laboratory examinations, including the quantification of C reactive protein (CRP, is not well established.The aim of this study was to evaluate the correlation between CRP value and indirect markers of disease severity in patients with bronchiolitis.This study included the patients diagnosed with bronchiolitis admitted to the Pediatrics Department of S. João Hospital in 2006. A retrospective review was made through analysing clinical files.176 patients aged 0 to 36 months (median of 4 months were included. 63.1% were males. CRP level was measured in 94.3% of the patients, with values ranging from zero to 256 mg/L and a median of 11 mg/L.CRP value in this population had a statistically significant relation with admission to Intensive Care Unit (ICU (p=0.008, length of hospital stay (p=0.025 and need for supplementary oxygen during hospital stay (p=0.022.This work raises the hypothesis that the CRP value on admission might be a marker of disease severity and have prognostic significance in patients with bronchiolitis. Further investigation is necessary to validate these results and exclude the potential confounding effect of associated infections. Resumo: O diagnóstico de bronquiolite aguda é essencialmente clínico. A utilidade de exames laboratoriais, em particular do doseamento da proteína C reactiva (PCR, não está bem estabelecida.O objectivo deste trabalho foi avaliar a relação entre o valor da PCR e os marcadores indirectos de gravidade em doentes com diagnóstico de bronquiolite.Foram incluídos no estudo todos os doentes admitidos no Serviço de Pediatria do HSJ durante o ano de 2006 e que tiveram como diagnóstico principal bronquiolite, tendo sido feita uma revisão retrospectiva do processo clínico desses doentes.Foram incluídos 176 doentes com idades compreendidas entre zero e 36 meses (mediana de 4 meses; 63,1% eram do sexo masculino. O doseamento da

Parental preference and perspectives on continuous pulse oximetry in infants and children with bronchiolitis

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Hendaus MA

2018-04-01

Full Text Available Mohamed A Hendaus,1,2 Suzan Nassar,3 Bassil A Leghrouz,3 Ahmed H Alhammadi,1,2 Mohammed Alamri4 1Department of Pediatrics, Section of Academic General Pediatrics, Sidra Medicine, Doha, Qatar; 2Department of Clinical Pediatrics, Weill Cornell Medicine, Doha, Qatar; 3Department of Pediatrics, Hamad General Corporation, Doha, Qatar; 4Pediatric Emergency Center, Hamad General Corporation, Doha, Qatar Objective: The purpose of the study was to investigate parental preference of continuous pulse oximetry in infants and children with bronchiolitis. Materials and methods: A cross-sectional prospective study was conducted at Hamad Medical Corporation in Qatar. Parents of infants and children <24 months old and hospitalized with bronchiolitis were offered an interview survey. Results: A total of 132 questionnaires were completed (response rate 100%. Approximately 90% of participants were 20–40 years of age, and 85% were females. The mean age of children was 7.2±5.8 months. Approximately eight in ten parents supported the idea of continuous pulse oximetry in children with bronchiolitis. Almost 43% of parents believed that continuous pulse-oximetry monitoring would delay their children’s hospital discharge. Interestingly, approximately 85% of caregivers agreed that continuous pulse oximetry had a good impact on their children’s health. In addition, around one in two of the participants stated that good bedside examinations can obviate the need for continuous pulse oximetry. Furthermore, 80% of parents believed that continuous pulse-oximetry monitoring would give the health-care provider a good sense of security regarding the child’s health. Finally, being a male parent was associated with significantly increased risk of reporting unnecessary fatigue, attributed to the sound of continuous pulse oximetry (P=0.031. Conclusion: Continuous pulse-oximetry monitoring in children with bronchiolitis was perceived as reassuring for parents. Involving parents

Viral bronchiolitis in young infants: new perspectives for management and treatment

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Mauricio T. Caballero

Full Text Available Abstract Objective: The aim of this review was to address advances in management and treatment of acute viral bronchiolitis in infants. Sources: A systematic review search was made including all articles published in English between 2010 and 2017, and available in the electronic databases PubMed and Cochrane Central Register of Controlled Trials (CENTRAL and specialized register of the Acute Respiratory Infections Group (Cochrane review group. The following MESH terms in English were included, using different Boolean operators for the search strategy: “bronchiolitis, viral,” “diagnosis,” “epidemiology,” “etiology,” “therapy,” “virology,” “prevention and control,” “respiratory syncytial virus, human.” Additional filters were used. Summary of findings: Few effective interventions are recommended for the management of RSV bronchiolitis in young infants. The main goal is to ensure an adequate oxygen supplementation and fluid balance whenever deemed necessary. Hypertonic saline nebulization is helpful only for hospitalized infants. Numerous antiviral drugs and specific vaccines for RSV are under evaluation and foretell advances in disease management in the near future. Conclusion: A number of promising new technologies are advancing in the field. Until new interventions became feasible, early detection and modification of preventable risk factors is essential to improve outcomes.

Bronchiolitis in Abha, Southwest Saudi Arabia: viral etiology and ...

African Journals Online (AJOL)

Other viruses isolated were: Influenza virus A (11%), influenza virus B (7%), Parainfluenza viruses (18%), parainfluenza virus type 1 (4%), parainfluenza virus type 2 (2%) and parainfluenza virus type 3 (13 %). Conclusions: Respiratory syncytial virus was the most frequent cause of admitted-cases of bronchiolitis, followed ...

RSV-induced bronchiolitis but not upper respiratory tract infection is accompanied by an increased nasal IL-18 response

NARCIS (Netherlands)

van Benten, Inesz J.; van Drunen, Cornelis M.; Koopman, Laurens P.; Kleinjan, Alex; van Middelkoop, Barbara C.; de Waal, Leon; Osterhaus, Albert D. M. E.; Neijens, Herman J.; Fokkens, Wytske J.

2003-01-01

The aim of this study was to investigate potential differences in the local nasal immune response between bronchiolitis and upper respiratory tract infection induced by respiratory syncytial virus (RSV). Nasal brush samples were obtained from 14 infants with RSV bronchiolitis and from 8 infants with

Implementing an Oxygen Supplementation and Monitoring Protocol on Inpatient Pediatric Bronchiolitis: An Exercise in Deimplementation

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Brian LeCleir

2017-01-01

Full Text Available Aim. Our goal in this study is to evaluate the effectiveness of our oxygen (O2 protocol to reduce length of stay (LOS for children hospitalized with bronchiolitis. Methods. In this retrospective cohort study, the outcomes of children ≤ 24 months old that were admitted with bronchiolitis and placed on the O2 protocol were compared to historical controls. The primary outcome was hospital length of stay. Secondary outcomes were duration of O2 supplementation, rates of pediatric intensive care unit transfer, and readmission. Results. Groups were not significantly different in age, gender, and rates of respiratory distress score assessment. Significantly more severely ill patients were in the O2 protocol group. There were no significant differences between control and O2 protocol groups with regard to mean LOS, rates of pediatric intensive care unit transfer, or seven-day readmission rates. By multiple regression analysis, the use of the O2 protocol was associated with a nearly 20% significant decrease in the length of hospitalization (p=0.030. Conclusion. Use of O2 supplementation protocol increased LOS in the more ill patients with bronchiolitis but decreased overall LOS by having a profound effect on patients with mild bronchiolitis.

An automated classification system for the differentiation of obstructive lung diseases based on the textural analysis of HRCT images

International Nuclear Information System (INIS)

Park, Seong Hoon; Seo, Joon Beom; Kim, Nam Kug; Lee, Young Kyung; Kim, Song Soo; Chae, Eun Jin; Lee, June Goo

2007-01-01

To develop an automated classification system for the differentiation of obstructive lung diseases based on the textural analysis of HRCT images, and to evaluate the accuracy and usefulness of the system. For textural analysis, histogram features, gradient features, run length encoding, and a co-occurrence matrix were employed. A Bayesian classifier was used for automated classification. The images (image number n = 256) were selected from the HRCT images obtained from 17 healthy subjects (n = 67), 26 patients with bronchiolitis obliterans (n = 70), 28 patients with mild centrilobular emphysema (n = 65), and 21 patients with panlobular emphysema or severe centrilobular emphysema (n = 63). An five-fold cross-validation method was used to assess the performance of the system. Class-specific sensitivities were analyzed and the overall accuracy of the system was assessed with kappa statistics. The sensitivity of the system for each class was as follows: normal lung 84.9%, bronchiolitis obliterans 83.8%, mild centrilobular emphysema 77.0%, and panlobular emphysema or severe centrilobular emphysema 95.8%. The overall performance for differentiating each disease and the normal lung was satisfactory with a kappa value of 0.779. An automated classification system for the differentiation between obstructive lung diseases based on the textural analysis of HRCT images was developed. The proposed system discriminates well between the various obstructive lung diseases and the normal lung

Advanced sclerosis of the chest wall skin secondary to chronic graft-versus-host disease: a case with severe restrictive lung defect.

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Ödek, Çağlar; Kendirli, Tanil; İleri, Talia; Yaman, Ayhan; Fatih Çakmakli, Hasan; Ince, Elif; İnce, Erdal; Ertem, Mehmet

2014-10-01

Pulmonary chronic graft-versus-host disease (cGvHD) is one of the most common causes of morbidity and mortality after allogeneic hematopoietic stem cell transplantation (aHSCT). Herein, we describe a patient with severe restrictive lung defect secondary to cGvHD. A 21-year-old male patient was admitted to our pediatric intensive care unit (PICU) with pneumonia and respiratory distress. He had a history of aHSCT for chronic myelogeneous leukemia at the age of 17 years. Six months after undergoing aHSCT, he had developed cGvHD involving skin, mouth, eye, lung, liver, and gastrointestinal tract. At the time of PICU admission he had respiratory distress and required ventilation support. Thorax high-resolution computed tomography was consistent with bronchiolitis obliterans. Although bronchiolitis obliterans is an obstructive lung defect, a restrictive pattern became prominent in the clinical course because of the sclerotic chest wall skin. The activity of cGvHD kept increasing despite the therapy and we lost the patient because of severe respiratory distress and massive hemoptysis secondary to bronchiectasis. In conclusion, pulmonary cGvHD can present with restrictive changes related with the advanced sclerosis of the chest wall skin. Performing a fasciotomy or a scar revision for the rigid chest wall in selected patients may improve the patients ventilation.

Mixed Herbal Medicine Induced Diffuse Infiltrative Lung Disease: The HRCT and Histopathologic Findings

International Nuclear Information System (INIS)

Kim, Tae Gyu; Shin, Eun A; Kim, Joung Sook

2010-01-01

The purpose of this study was to evaluate the high-resolution CT (HRCT) and pathologic findings of mixed herbal medicine-induced diffuse interstitial lung disease. Eight patients (6 women and 2 men, age range: 31 to 81 years, mean age: 51.4 years) who presented with cough or dyspnea after taking mixed herbal medicine were included in this study. All the patients underwent plain chest radiography and HRCT. We obtained pathologic specimens from 7 patients via fluoroscopy guided large bore cutting needle biopsy and transbronchial lung biopsy. All the patients were treated with steroid therapy. The most common HRCT finding was bilateral diffuse ground glass opacity (n=7), followed by peribronchial consolidation (n=5) and inter- or intralobular septal thickening (n=2). For the disease distribution, the lower lung zone was dominantly involved. The pathologic results of 7 patients were nonspecific interstitial pneumonia (n=3), bronchiolitis obliterans organizing pneumonia (n=2), hypersensitivity pneumonitis (n=1) and eosinophilic pneumonia (n=1). Irrespective of the pathologic results, all 8 patients improved clinically and radiologically after steroid treatment. The HRCT findings of mixed herbal medicine-induced diffuse infiltrative lung disease were mainly bilateral diffuse ground glass opacity, peribronchial consolidation and dominant involvement of the lower lung zone. Those pathologic findings were nonspecific and the differential diagnosis could include interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, hypersensitivity pneumonitis and eosinophilic pneumonia

Mixed Herbal Medicine Induced Diffuse Infiltrative Lung Disease: The HRCT and Histopathologic Findings

Energy Technology Data Exchange (ETDEWEB)

Kim, Tae Gyu; Shin, Eun A [Sanggye Paik Hospital, Inje University College of Medicine, Seoul (Korea, Republic of); Kim, Joung Sook [Mokdong Hospital, Ewha Womans University College of Medicine, Seoul (Korea, Republic of)

2010-12-15

The purpose of this study was to evaluate the high-resolution CT (HRCT) and pathologic findings of mixed herbal medicine-induced diffuse interstitial lung disease. Eight patients (6 women and 2 men, age range: 31 to 81 years, mean age: 51.4 years) who presented with cough or dyspnea after taking mixed herbal medicine were included in this study. All the patients underwent plain chest radiography and HRCT. We obtained pathologic specimens from 7 patients via fluoroscopy guided large bore cutting needle biopsy and transbronchial lung biopsy. All the patients were treated with steroid therapy. The most common HRCT finding was bilateral diffuse ground glass opacity (n=7), followed by peribronchial consolidation (n=5) and inter- or intralobular septal thickening (n=2). For the disease distribution, the lower lung zone was dominantly involved. The pathologic results of 7 patients were nonspecific interstitial pneumonia (n=3), bronchiolitis obliterans organizing pneumonia (n=2), hypersensitivity pneumonitis (n=1) and eosinophilic pneumonia (n=1). Irrespective of the pathologic results, all 8 patients improved clinically and radiologically after steroid treatment. The HRCT findings of mixed herbal medicine-induced diffuse infiltrative lung disease were mainly bilateral diffuse ground glass opacity, peribronchial consolidation and dominant involvement of the lower lung zone. Those pathologic findings were nonspecific and the differential diagnosis could include interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, hypersensitivity pneumonitis and eosinophilic pneumonia

Fibrosing organising pneumonia.

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Beardsley, Brooke; Rassl, Doris

2013-10-01

Organising pneumonia (otherwise referred to as bronchiolitis obliterans organising pneumonia) is characterised histologically by plugs of granulation tissue, which are present predominantly within small airways, alveolar ducts and peri-bronchiolar alveoli. This pattern is not specific for any disorder or cause, but is one type of inflammatory response to pulmonary injury, which may be seen in a wide variety of clinical conditions. Typically, organising pneumonia responds very well to corticosteroid treatment; however, a small percentage of patients appear to develop progressive fibrosis.

Rib fractures after chest physiotherapy for bronchiolitis or pneumonia in infants

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Chalumeau, Martin [Service de Pneumologie et d' Allergologie Pediatriques, Hopital Necker-Enfants Malades, Paris (France); Service de Pediatrie, Hopital Saint-Vincent-de-Paul, Paris (France); Foix-l' Helias, Laurence; Scheinmann, Pierre; Zuani, Pierre [Service de Pneumologie et d' Allergologie Pediatriques, Hopital Necker-Enfants Malades, Paris (France); Gendrel, Dominique [Service de Pediatrie, Hopital Saint-Vincent-de-Paul, Paris (France); Ducou-le-Pointe, Hubert [Service de Radiologie, Hopital d' Enfants Armand-Trousseau, Avenue du Docteur Arnold Netter, 75571 Paris Cedex 12 (France)

2002-09-01

Heading AbstractBackground. The reported causes of rib fractures in infants are: child abuse, accidental injury, cardiopulmonary resuscitation, bone fragility, birth trauma and severe cough.Objective. To report chest physiotherapy (CPT) as a new cause of rib fractures in five infants.Materials and methods. We retrospectively identified all infants with rib fractures after CPT for bronchiolitis or pneumonia over a 4-year period in two paediatric and one paediatric radiology units in three university hospitals in Paris.Results. Five boys were identified. Their median age was 3 months. None had any other potential cause of rib fractures. The indication for CPT was bronchiolitis in four cases and pneumonia in one. The median number of rib fractures was four (range 1-5). Fractures were located between the 3rd and 8th ribs; they were lateral in four patients and posterior in one; they were unilateral in four patients and bilateral in one. Evolution was favourable in all cases. The prevalence of rib fractures after CPT during the study period was estimated at 1 in 1,000 infants hospitalised for bronchiolitis or pneumonia.Conclusions. CPT should be considered a potential, but very rare cause of rib fractures in infants. It can be of clinical relevance when rib fractures are the only feature suggestive of child abuse. (orig.)

Rib fractures after chest physiotherapy for bronchiolitis or pneumonia in infants

International Nuclear Information System (INIS)

Chalumeau, Martin; Foix-l'Helias, Laurence; Scheinmann, Pierre; Zuani, Pierre; Gendrel, Dominique; Ducou-le-Pointe, Hubert

2002-01-01

Heading AbstractBackground. The reported causes of rib fractures in infants are: child abuse, accidental injury, cardiopulmonary resuscitation, bone fragility, birth trauma and severe cough.Objective. To report chest physiotherapy (CPT) as a new cause of rib fractures in five infants.Materials and methods. We retrospectively identified all infants with rib fractures after CPT for bronchiolitis or pneumonia over a 4-year period in two paediatric and one paediatric radiology units in three university hospitals in Paris.Results. Five boys were identified. Their median age was 3 months. None had any other potential cause of rib fractures. The indication for CPT was bronchiolitis in four cases and pneumonia in one. The median number of rib fractures was four (range 1-5). Fractures were located between the 3rd and 8th ribs; they were lateral in four patients and posterior in one; they were unilateral in four patients and bilateral in one. Evolution was favourable in all cases. The prevalence of rib fractures after CPT during the study period was estimated at 1 in 1,000 infants hospitalised for bronchiolitis or pneumonia.Conclusions. CPT should be considered a potential, but very rare cause of rib fractures in infants. It can be of clinical relevance when rib fractures are the only feature suggestive of child abuse. (orig.)

Frequency, duration and predictors of bronchiolitis episodes of care among infants ≥32 weeks gestation in a large integrated healthcare system: a retrospective cohort study

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Flaherman Valerie J

2012-06-01

Full Text Available Abstract Background Bronchiolitis is common in the first two years of life and is the most frequent cause of hospitalization in this age group. No previous studies have used an episode-of-care analysis to describe the frequency, duration, and predictors of bronchiolitis episodes of care during the first two years. Methods We conducted a retrospective cohort study of 123,264 infants ≥32 weeks gestation born at 6 Northern California Kaiser Permanente hospitals between 1996 and 2002. We used electronic medical records to concatenate hospital, emergency department and outpatient health care encounters for bronchiolitis into discrete episodes of care. We used descriptive statistics to report frequency and duration of bronchiolitis episodes and used logistic regression to assess the effect of gestational age and other clinical and demographic predictors on the outcome of bronchiolitis episodes. Results Among all infants, the rate of bronchiolitis episodes was 162 per 1000 children during the first 2 years of life; approximately 40% required >1 day of medical attention with a mean duration of 7.0 ± 5.9 days. Prematurity was associated with increased risk of bronchiolitis episodes and longer duration. Bronchiolitis episodes rates per 1000 infants were 246 for 32–33 weeks gestational age, 204 for 34–36 weeks, and 148–178 for >36 weeks. Male gender, African-American and Hispanic race/ethnicity, and parental history of asthma were associated with an increased risk of having a bronchiolitis episode and/or longer duration. Conclusions Bronchiolitis episodes of care are frequent during the first two years of life and the duration ranges from 1 to 27 days. Prematurity was associated with more frequent and longer duration of bronchiolitis episodes of care, which may reflect illness severity and/or perceived vulnerability.

Placebo-controlled trial of nebulization with adrenaline in acute bronchiolitis: a quasi-experimental study

International Nuclear Information System (INIS)

Afzal, M.F.; Iqbal, S.M.; Sultan, M.A.

2012-01-01

Background: Bronchiolitis is an acute inflammatory obstruction. of small In children that occurs In first two years of life and is by fever, rhinitis, cough, tachypnoea, expiratory wheeze and increased respiratory effort To study efficacy of nebulized adrenaline compared with placebo in acute bronchiolitis. Quasi-experimental study carried out at Department of aediatrics, King Edward Medical University/ Mayo Hospital, Lahore from October 2006 through March 2007. After consent from parents, sixty children of age between 2 months to 2 years with the first episode consistent with clinical case definition of bronchiolitis were included by using convenient sampling. clinical scoring system was used to grade the severity of disease as well as to monitor the efficacy of intervention. Those having score = 8 were randomly allocated to the two study groups. The information was recorded at 0 minute and effect of each method of treatment was followed for 90 minutes. Results: Our study population was 60 children. The mean age was 11:1:6 Months. Male to female ratio was 1.2: 1. Mean weight of the Children was 9:1:3 kg. Improvement in clinical score, oxygen saturation, and length of hospital at 0 and 90 minutes was noted in both groups but when compared with placebo, there was no Statistically significant difference. Conclusion: There is no difference in the efficacy of nebulization with adrenaline compared with placebo in the management of acute bronchiolitis. (author)

Interferon lambda 1-3 expression in infants hospitalized for RSV or HRV associated bronchiolitis.

Science.gov (United States)

Selvaggi, Carla; Pierangeli, Alessandra; Fabiani, Marco; Spano, Lucia; Nicolai, Ambra; Papoff, Paola; Moretti, Corrado; Midulla, Fabio; Antonelli, Guido; Scagnolari, Carolina

2014-05-01

The airway expression of type III interferons (IFNs) was evaluated in infants hospitalized for respiratory syncytial virus (RSV) or rhinovirus (HRV) bronchiolitis. As an additional objective we sought to determine whether a different expression of IFN lambda 1-3 was associated with different harboring viruses, the clinical course of bronchiolitis or with the levels of well established IFN stimulated genes (ISGs), such as mixovirus resistance A (MxA) and ISG56. The analysis was undertaken in 118 infants with RSV or HRV bronchiolitis. Nasopharyngeal washes were collected for virological studies and molecular analysis of type III IFN responses. RSV elicited higher levels of IFN lambda subtypes when compared with HRV. A similar expression of type III IFN was found in RSVA or RSVB infected infants and in those infected with HRVA or HRVC viruses. Results also indicate that IFN lambda 1 and IFN lambda 2-3 levels were correlated with each other and with MxA and ISG56-mRNAs. In addition, a positive correlation exists between the IFN lambda1 levels and the clinical score index during RSV infection. In particular, higher IFN lambda 1 levels are associated to an increase of respiratory rate. These findings show that differences in the IFN lambda 1-3 levels in infants with RSV or HRV infections are present and that the expression of IFN lambda 1 correlates with the severity of RSV bronchiolitis. Copyright © 2014 The British Infection Association. Published by Elsevier Ltd. All rights reserved.

Neonatal bronchial hyperresponsiveness precedes acute severe viral bronchiolitis in infants

DEFF Research Database (Denmark)

Chawes, Bo L K; Poorisrisak, Porntiva; Johnston, Sebastian L

2012-01-01

Respiratory syncytial virus and other respiratory tract viruses lead to common colds in most infants, whereas a minority develop acute severe bronchiolitis often requiring hospitalization. We hypothesized that such an excessive response to respiratory tract viral infection is caused by host factors...

Impact of hospitalizations for bronchiolitis in preterm infants on long-term health care costs in Italy: a retrospective case-control study

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Roggeri DP

2016-08-01

Full Text Available Daniela Paola Roggeri,1 Alessandro Roggeri,1 Elisa Rossi,2 Salvatore Cataudella,2 Nello Martini,3 1ProCure Solutions, Nembro, Bergamo, 2CINECA Interuniversity Consortium, Bologna, 3Accademia Nazionale di Medicina, Rome, Italy Purpose: Bronchiolitis is an acute inflammatory injury of the bronchioles, and is the most frequent cause of hospitalization for lower respiratory tract infections in preterm infants. This was a retrospective, observational, case-control study conducted in Italy, based on administrative database analysis. The aim of this study was to evaluate differences in health care costs of preterm infants with and without early hospitalization for bronchiolitis. Patients and methods: Preterm infants born in the period between January 1, 2009 and December 31, 2010 and hospitalized for bronchiolitis in the first year of life were selected from the ARNO Observatory database and observed for the first 4 years of life. These preterm infants were compared (paired 1–3 with preterm infants who were not hospitalized for bronchiolitis in the first year of life and with similar characteristics. Only direct health care costs reimbursed by the Italian National Health Service were considered for this study (drugs, hospitalizations, and diagnostic/therapeutic procedures. Results: Of 40,823 newborns in the accrual period, 863 were preterm with no evidence of prophylaxis, and 22 preterm infants were hospitalized for bronchiolitis (cases and paired with 62 controls. Overall, cases had 74% higher average cost per infant in the first 4 years of life than controls (18,624€ versus 10,189€, respectively. The major cost drivers were hospitalizations, accounting for >90% in both the populations. The increase in total yearly health care cost between cases and controls remained substantial even in the fourth year of life for all cost items. A relevant increase in hospitalizations and drug consumption linked to respiratory tract diseases was noted in

Chronic bronchiolitis in non-human primates after prolonged ozone exposure

International Nuclear Information System (INIS)

Eustis, S.L.

1979-01-01

Bonnet monkeys (Macaca radiata) were exposed to 0.0, 0.5, and 0.8 ppM ozone for 7, 28, or 90 consecutive d, eight h per d. Changes in the lung evoked by prolonged oxidant exposure were evaluated by light microscopy, scanning electron microscopy, transmission electron microscopy, autoradiography, and morphometry. Morphological changes were principally characterized as a low-grade chronic respiratory bronchiolitis. Proximal generations of respiratory bronchioles were the most consistently affected region of the pulmonary acinus. Major features of the bronchiolitis were intraluminal accumulations of macrophages and hypertrophy and hyperplasia of cuboidal bronchiolar epithelial cells. The degree of inflammation was greatest at the 0.8 ppM ozone concentration at each exposure period. The number of inflammatory cells observed at 90 d, however, was about one-half that observed at 7 d. Tritiated thymidine labeling and differential cell counts of respiratory bronchiolar epithelium demonstrated a proliferative response and an increase in the proportion of cubiodal bronchiolar cells which presumably function as the progenitor cell during cell renewal and repair of the epithelium. The labeling index remained elevated above control values through 90 d of ozone exposure despite a reduction below 7 d values at both 28 and 90 d. The epithelial hyperplasia persisted through 90 d at a level similar to but slightly above that present at 7 d. Since cell differentials remained shifted at 28 and 90 d, it appears that persistent ozone exposure delayed maturation and transformation of cubiodal bronchiolar cells to their mature phenotype

Herpes simplex virus bronchiolitis in a cannabis user

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Daniel H. Libraty

2014-01-01

Full Text Available Herpes simplex virus (HSV lower respiratory tract infections in adults are uncommon. We present a case of HSV bronchiolitis and pneumonitis in an immunocompetent individual, likely linked to chronic habitual marijuana use and a herpetic orolabial ulcer. The case serves as a reminder to consider HSV as a potential unusual cause of lower respiratory tract infection/inflammation in individuals with chronic habitual marijuana use.

Clinical significance of changes of plasma CGRP and VIP levels in infants with bronchiolitis

International Nuclear Information System (INIS)

Yang Chun; Gu Ling; Zhang Yanjun; Xin Haiyan

2005-01-01

Objective: To explore the clinical significance of changes of calcitonin gene-related peptide (CGRP) and vasoactive intestinal peptide (VIP) levels in infants (2-24months) with bronchiolitis. Methods: Plasma levels of CGRP and VIP were determined with RIA in 31 infants with bronchiolitis both during acute infection and convalescence as well as in 35 controls. Results: Plasma CGRP levels in patients during acute infection were significantly higher than those in patients during convalescence and in controls (P<0.05). Levels of CGRP dropped during convalescence, but still remained significantly higher than those in controls (P<0.05). The reverse was true for the plasma VIP levels. The plasma VIP levels in patients during acute infection were significantly lower than those in patients during convalescence and in controls (P<0.05). During convalescence, the plasma VIP levels rose but remained significantly lower than those in controls (P<0.05). Conclusion: There were dynamic changes of plasma CGRP and VIP levels in the course of infant bronchiolitis and the two peptides played opposite roles. (authors)

Using multifaceted education to improve management in acute viral bronchiolitis.

Science.gov (United States)

Murch, Hannah; Oakley, Juliette; Pierrepoint, Marcus; Powell, Colin

2015-07-01

To establish current bronchiolitis management across hospitals in Wales, improve compliance with national guidelines and standardise evidence-based clinical practice. A complete audit cycle with implementation of a multifaceted education bundle prior to the follow-up audit. Twelve acute paediatric departments between 1 November and 31 December in 2012 and 2013. All infants under 12 months with a clinical diagnosis of bronchiolitis. The first audit assessed management of bronchiolitis with reference to both the Scottish Intercollegiate Guideline Network (SIGN) guidelines and local hospital guidelines. Following analysis and dissemination of these results, an education bundle was implemented nationwide, with completion of the audit cycle to assess change. Compliance with SIGN recommendations for investigation, treatment and discharge. Compliance with the education bundle requirements also assessed in 2013. Data were collected for 1599 infants. The education bundle was delivered in all hospitals. The level of severity, defined by oxygen saturations in air at presentation, length of stay and paediatric intensive care unit transfers, was equivalent for both years. Mean compliance percentage (95% CI) across Wales significantly improved between 2012 and 2013, with compliance with investigations increasing from 50% (46% to 53%) to 71% (68% to 74%), with management increasing from 65% (61% to 68%) to 74% (71% to 77%), and overall compliance improving from 38% (37% to 39%) to 59% (56% to 62%) in 2013. This audit demonstrated a significant improvement in compliance following implementation of our educational bundle. This has enabled improvement in standardised and evidence-based patient care across Wales. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

The severity of acute bronchiolitis in infants was associated with quality of life nine months later.

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Rolfsjord, Leif Bjarte; Skjerven, Håvard Ove; Carlsen, Kai-Håkon; Mowinckel, Petter; Bains, Karen Eline Stensby; Bakkeheim, Egil; Lødrup Carlsen, Karin C

2016-07-01

Acute bronchiolitis in infancy increases the risk of later asthma and reduced health-related quality of life (QoL). We aimed to see whether the severity of acute bronchiolitis in the first year of life was associated with QoL nine months later. The parents of 209 of 404 of children hospitalised for acute bronchiolitis in eight paediatric departments in south-east Norway at a mean four months of age (range 0-12 months) completed the Infant/Toddler Quality of Life Questionnaire sent by mail nine months after the acute illness. Disease severity was measured by length of stay and the need for supportive treatment. Interactions with gender, inclusion age, prematurity, maternal ethnicity and maternal education were examined. Reduced QoL in four domains was associated with increased length of stay and need for ventilatory support. Physical abilities and general health were associated with both severity markers, whereas bodily pain and discomfort and change in health were associated with length of stay. Ventilatory support was more negatively associated with QoL than atopic eczema and also associated with reduced parental emotions and parental time. The severity of acute bronchiolitis in infants was associated with reduced QoL nine months later. ©2016 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.

Principal findings of systematic reviews for the management of acute bronchiolitis in children.

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Castro-Rodriguez, Jose A; Rodriguez-Martinez, Carlos E; Sossa-Briceño, Monica P

2015-09-01

Bronchiolitis is the most common cause of hospitalization among infants during the first 12 months of life, with high direct and indirect cost for health system and families. Different treatment approaches co-exist worldwide resulting in many drugs prescribed, without any proven benefit. Twenty systematic reviews of randomized clinical trials (SRCTs) on management of acute bronchiolitis in children were retrieved through 5 databases and their methodological quality was determined using an AMSTAR tool. Epinephrine showed impact only in short-term outcomes among outpatients (reduced admission at day 1 and improved the clinical score in the first 2 hours, compared to placebo) and inpatients (decreased length of stay (LOS) and improved saturation only in the first 2 hours, compared to nebulized salbutamol, but with high heterogeneity). Nebulized 3% saline among inpatients (but not in the emergency department setting) decreased hospital LOS. In small trials, exogenous surfactant among children may decrease the duration of mechanical ventilation and intensive care unit LOS and had favorable effects on oxygenation and CO2 elimination at 24 hrs. Although several SRCTs are currently available, only few treatments show clinically important improvements. Therefore, it is still difficult to prepare a well-established and accepted guideline for the treatment of acute bronchiolitis. Copyright © 2015 Elsevier Ltd. All rights reserved.

Virus Type and Genomic Load in Acute Bronchiolitis: Severity and Treatment Response With Inhaled Adrenaline.

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Skjerven, Håvard O; Megremis, Spyridon; Papadopoulos, Nikolaos G; Mowinckel, Petter; Carlsen, Kai-Håkon; Lødrup Carlsen, Karin C

2016-03-15

Acute bronchiolitis frequently causes infant hospitalization. Studies on different viruses or viral genomic load and disease severity or treatment effect have had conflicting results. We aimed to investigate whether the presence or concentration of individual or multiple viruses were associated with disease severity in acute bronchiolitis and to evaluate whether detected viruses modified the response to inhaled racemic adrenaline. Nasopharyngeal aspirates were collected from 363 infants with acute bronchiolitis in a randomized, controlled trial that compared inhaled racemic adrenaline versus saline. Virus genome was identified and quantified by polymerase chain reaction analyses. Severity was assessed on the basis of the length of stay and the use of supportive care. Respiratory syncytial virus (83%) and human rhinovirus (34%) were most commonly detected. Seven other viruses were present in 8%-15% of the patients. Two or more viruses (maximum, 7) were detected in 61% of the infants. Virus type or coinfection was not associated with disease severity. A high genomic load of respiratory syncytial virus was associated with a longer length of stay and with an increased frequency of oxygen and ventilatory support use. Treatment effect of inhaled adrenaline was not modified by virus type, load or coinfection. In infants hospitalized with acute bronchiolitis, disease severity was not associated with specific viruses or the total number of viruses detected. A high RSV genomic load was associated with more-severe disease. NCT00817466 and EudraCT 2009-012667-34. © The Author 2015. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.

Racemic epinephrine compared to salbutamol in hospitalized young children with bronchiolitis; a randomized controlled clinical trial [ISRCTN46561076

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LeBlanc John C

2005-05-01

Full Text Available Abstract Background Bronchiolitis is the most common cause of lower respiratory tract illness in infancy, and hospital admission rates appear to be increasing in Canada and the United States. Inhaled beta agonists offer only modest short-term improvement. Trials of racemic epinephrine have shown conflicting results. We sought to determine if administration of racemic epinephrine during hospital stay for bronchiolitis improved respiratory distress, was safe, and shortened length of stay. Methods The study was a randomized, double-blind controlled trial of aerosolized racemic epinephrine compared to salbutamol every one to 4 hours in previously well children aged 6 weeks to ≤ 2 years of age hospitalized with bronchiolitis. The primary outcome was symptom improvement as measured by the Respiratory Distress Assessment Instrument (RDAI; secondary outcomes were length of stay in hospital, adverse events, and report of symptoms by structured parental telephone interview one week after discharge. Results 62 children with a mean age of 6.4 months were enrolled; 80% of children had Respiratory Syncytial Virus (RSV. Racemic epinephrine resulted in significant improvement in wheezing and the total RDAI score on day 2 and over the entire stay (p 0.05. Adverse events were not significantly different in the two arms. At one week post-discharge, over half of parents reported that their child still had a respiratory symptom and 40% had less than normal feeding. Conclusion Racemic epinephrine relieves respiratory distress in hospitalized infants with bronchiolitis and is safe but does not abbreviate hospital stay. Morbidity associated with bronchiolitis as identified by parents persists for at least one week after hospital discharge in most infants.

Correlation and Agreement of Handheld Spirometry with Laboratory Spirometry in Allogeneic Hematopoietic Cell Transplant Recipients.

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Cheng, Guang-Shing; Campbell, Angela P; Xie, Hu; Stednick, Zach; Callais, Cheryl; Leisenring, Wendy M; Englund, Janet A; Chien, Jason W; Boeckh, Michael

2016-05-01

Early detection of subclinical lung function decline may help identify allogeneic hematopoietic cell transplant (HCT) recipients who are at increased risk for late noninfectious pulmonary complications, including bronchiolitis obliterans syndrome. We evaluated the use of handheld spirometry in this population. Allogeneic HCT recipients enrolled in a single-center observational trial performed weekly spirometry with a handheld spirometer for 1 year after transplantation. Participants performed pulmonary function tests in an outpatient laboratory setting at 3 time points: before transplantation, at day 80 after transplantation, and at 1 year after transplantation. Correlation between the 2 methods was assessed by Pearson and Spearman correlations; agreement was assessed using Bland-Altman plots. A total of 437 subjects had evaluable pulmonary function tests. Correlation for forced expiratory volume in 1 second (FEV1) was r = .954 (P spirometry correlated well with laboratory spirometry after allogeneic HCT and may be useful for self-monitoring of patients for early identification of airflow obstruction. Copyright © 2016 American Society for Blood and Marrow Transplantation. All rights reserved.

Esophageal Dysmotility, Gastro-esophageal Reflux Disease, and Lung Transplantation: What Is the Evidence?

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Wood, Richard K

2015-12-01

Lung transplantation is an effective and life-prolonging therapy for patients with advanced lung disease (ALD). However, long-term patient survival following lung transplantation is primarily limited by development of an inflammatory and fibrotic process involving the lung allograft known as bronchiolitis obliterans syndrome (BOS). Although the precise cause of BOS remains uncertain and is likely multifactorial, chronic aspiration of gastro-duodenal contents is one possible contributing factor. Multiple small, cross-sectional studies performed over the past two decades have reported a high prevalence of gastro-esophageal reflux disease (GERD) and esophageal dysmotility in the ALD population and several investigations suggest the prevalence may increase following lung transplantation. More recent studies evaluating the direct effect of gastro-duodenal contents on airways have demonstrated a possible biologic link between GERD and BOS. Despite the recent advances in our understanding of BOS, further investigations are needed to establish GERD as a causative factor in its development. This review will discuss the existing literature that has identified an association of GERD with ALD and post-transplant populations, with a focus on recent advances in the field.

Prune belly syndrome in an Egyptian infant with Down syndrome: A case report

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Metwalley Kotb A

2008-10-01

Full Text Available Abstract Introduction Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare. Case presentation A 4-month-old Egyptian boy was admitted to our institute for management of acute bronchiolitis. He was born at full term by normal vaginal delivery. His mother, a 42-year-Egyptian villager with six other children, had no antenatal or prenatal care. On examination, the boy was found to be hypotonic. In addition to features of Down syndrome, karyotyping confirmed the diagnosis of trisomy 21. Ultrasound examination of the abdomen showed bilateral gross hydronephrosis with megaureter. Micturating cystourethrography showed grade V vesicoureteric reflux bilaterally with no urethral obstruction. Serum creatinine concentration was 90 μmol/litre, serum sodium was 132 mmol/litre and serum potassium was 5.9 mmol/litre. Conclusion We report an Egyptian infant with Down syndrome and prune belly syndrome. The incidence of this association is unknown. Routine antenatal ultrasonography will help in discovering renal anomalies which can be followed postnatally. Postnatal detection of prune belly syndrome necessitates full radiological investigation to detect any renal anomalies. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

Prune belly syndrome in an Egyptian infant with Down syndrome: a case report.

Science.gov (United States)

Metwalley, Kotb A; Farghalley, Hekma S; Abd-Elsayed, Alaa A

2008-10-02

Prune belly syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. The association between prune belly syndrome and Down syndrome is very rare. A 4-month-old Egyptian boy was admitted to our institute for management of acute bronchiolitis. He was born at full term by normal vaginal delivery. His mother, a 42-year-Egyptian villager with six other children, had no antenatal or prenatal care. On examination, the boy was found to be hypotonic. In addition to features of Down syndrome, karyotyping confirmed the diagnosis of trisomy 21. Ultrasound examination of the abdomen showed bilateral gross hydronephrosis with megaureter. Micturating cystourethrography showed grade V vesicoureteric reflux bilaterally with no urethral obstruction. Serum creatinine concentration was 90 mumol/litre, serum sodium was 132 mmol/litre and serum potassium was 5.9 mmol/litre. We report an Egyptian infant with Down syndrome and prune belly syndrome. The incidence of this association is unknown. Routine antenatal ultrasonography will help in discovering renal anomalies which can be followed postnatally. Postnatal detection of prune belly syndrome necessitates full radiological investigation to detect any renal anomalies. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course.

Efficacy of nebulised L-adrenaline with 3% hypertonic saline versus normal saline in bronchiolitis

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Shabnam Sharmin

2016-08-01

Full Text Available Background: Bronchiolitis is one of the most common respiratory diseases requiring hospitalization. Nebulized epineph­rine and salbutamol therapy has been used in different centres with varying results. Objective: The objective of the study was to compare the efficacy of nebulised adrenaline diluted with 3% hypertonic saline with nebulised adrenaline diluted with normal saline in bronchiolitis. Methods: Fifty three infants and young children with bronchiolitis, age ranging from 2 months to 2 years, presenting in the emergency department of Manikganj Sadar Hospital were enrolled in the study. After initial evaluation, patients were randomized to receive either nebulized adrenaline I .5 ml ( 1.5 mg diluted with 2 ml of3% hypertonic saline (group I ornebulised adrenaline 1.5 ml (1.5 mg diluted with 2 ml of normal saline (group II. Patients were evaluated again 30 minutes after nebulization. Results: Twenty eight patients in the group I (hypertonic saline and twenty five in groupII (normal saline were included in the study. After nebulization, mean respiratory rate decreased from 63.7 to 48.1 (p<.01, mean clinical severity score decreased from 8.5 to 3.5 (p<.01 and mean oxygen satw·ation increased 94.7% to 96.9% (p<.01 in group I. In group II, mean respiratory rate decreased from 62.4 to 47.4 (p<.01, mean clinical severity score decreased from 7.2 to 4.1 (p<.01 and mean oxygen saturation increased from 94. 7% to 96. 7% (p<.01. Mean respiratory rate decreased by 16 in group I versus 14.8 (p>.05 in group 11, mean clinical severity score decreased by 4.6 in group versus 3 (p<.05 in group, and mean oxygen saturation increased by 2.2% and 1.9% in group and group respectively. Difference in reduction in clinical severity score was statistically significant , though the changes in respiratory rate and oxygen saturation were not statistically significant. Conclusion: The study concluded that both nebulised adrenaline diluted with 3% hypertonic saline and

IL1RL1 gene variants and nasopharyngeal IL1RL-a levels are associated with severe RSV bronchiolitis: a multicenter cohort study.

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Tina E Faber

Full Text Available Targets for intervention are required for respiratory syncytial virus (RSV bronchiolitis, a common disease during infancy for which no effective treatment exists. Clinical and genetic studies indicate that IL1RL1 plays an important role in the development and exacerbations of asthma. Human IL1RL1 encodes three isoforms, including soluble IL1RL1-a, that can influence IL33 signalling by modifying inflammatory responses to epithelial damage. We hypothesized that IL1RL1 gene variants and soluble IL1RL1-a are associated with severe RSV bronchiolitis.We studied the association between RSV and 3 selected IL1RL1 single-nucleotide polymorphisms rs1921622, rs11685480 or rs1420101 in 81 ventilated and 384 non-ventilated children under 1 year of age hospitalized with primary RSV bronchiolitis in comparison to 930 healthy controls. Severe RSV infection was defined by need for mechanical ventilation. Furthermore, we examined soluble IL1RL1-a concentration in nasopharyngeal aspirates from children hospitalized with primary RSV bronchiolitis. An association between SNP rs1921622 and disease severity was found at the allele and genotype level (p = 0.011 and p = 0.040, respectively. In hospitalized non-ventilated patients, RSV bronchiolitis was not associated with IL1RL1 genotypes. Median concentrations of soluble IL1RL1-a in nasopharyngeal aspirates were >20-fold higher in ventilated infants when compared to non-ventilated infants with RSV (median [and quartiles] 9,357 [936-15,528] pg/ml vs. 405 [112-1,193] pg/ml respectively; p<0.001.We found a genetic link between rs1921622 IL1RL1 polymorphism and disease severity in RSV bronchiolitis. The potential biological role of IL1RL1 in the pathogenesis of severe RSV bronchiolitis was further supported by high local concentrations of IL1RL1 in children with most severe disease. We speculate that IL1RL1a modifies epithelial damage mediated inflammatory responses during RSV bronchiolitis and thus may serve as a

Network Meta-Analysis Comparing the Efficacy of Therapeutic Treatments for Bronchiolitis in Children.

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Guo, Caili; Sun, Xiaomin; Wang, Xiaowen; Guo, Qing; Chen, Dan

2018-01-01

This study aims to compare placebo (PBO) and 7 therapeutic regimens-namely, bronchodilator agents (BAs), hypertonic saline (HS), BA ± HS, corticosteroids (CS), epinephrine (EP), EP ± CS, and EP ± HS-to determine the optimal bronchiolitis treatment. We plotted networks using the curative outcome of several studies and specified the relations among the experiments by using mean difference, standardized mean difference, and corresponding 95% credible interval. The surface under the cumulative ranking curve (SUCRA) was used to separately rank each therapy on clinical severity score (CSS) and length of hospital stay (LHS). This network meta-analysis included 40 articles from 1995 to 2016 concerning the treatment of bronchiolitis in children. All 7 therapeutic regimens displayed no significant difference to PBO with regard to CSS in our study. Among the 7 therapies, BA performed better than CS. As for LHS, EP and EP ± HS had an advantage over PBO. Moreover, EP and EP ± HS were also more efficient than BA. The SUCRA results showed that EP ± CS is most effective, and EP ± HS is second most effective with regard to CSS. With regard to LHS, EP ± HS ranked first, EP ± CS ranked second, and EP ranked third. We recommend EP ± CS and EP ± HS as the first choice for bronchiolitis treatment in children because of their outstanding performance with regard to CSS and LHS. © 2017 American Society for Parenteral and Enteral Nutrition.

Trends in Respiratory Syncytial Virus and Bronchiolitis Hospitalization Rates in High-Risk Infants in a United States Nationally Representative Database, 1997-2012.

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Abigail Doucette

Full Text Available Respiratory syncytial virus (RSV causes significant pediatric morbidity and is the most common cause of bronchiolitis. Bronchiolitis hospitalizations declined among US infants from 2000‒2009; however, rates in infants at high risk for RSV have not been described. This study examined RSV and unspecified bronchiolitis (UB hospitalization rates from 1997‒2012 among US high-risk infants.The Kids' Inpatient Database (KID infant annual RSV (ICD-9 079.6, 466.11, 480.1 and UB (ICD-9 466.19, 466.1 hospitalization rates were estimated using weighted counts. Denominators were based on birth hospitalizations with conditions associated with high-risk for RSV: chronic perinatal respiratory disease (chronic lung disease [CLD]; congenital airway anomalies (CAA; congenital heart disease (CHD; Down syndrome (DS; and other genetic, metabolic, musculoskeletal, and immunodeficiency conditions. Preterm infants could not be identified. Hospitalizations were characterized by mechanical ventilation, inpatient mortality, length of stay, and total cost (2015$. Poisson and linear regression were used to test statistical significance of trends.RSV and UB hospitalization rates were substantially elevated for infants with higher-risk CHD, CLD, CAA and DS without CHD compared with all infants. RSV rates declined by 47.0% in CLD and 49.7% in higher-risk CHD infants; no other declines in high-risk groups were observed. UB rates increased in all high-risk groups except for a 22.5% decrease among higher-risk CHD. Among high-risk infants, mechanical ventilation increased through 2012 to 20.4% and 13.5% of RSV and UB hospitalizations; geometric mean cost increased to $31,742 and $25,962, respectively, and RSV mortality declined to 0.9%.Among high-risk infants between 1997 and 2012, RSV hospitalization rates declined among CLD and higher-risk CHD infants, coincident with widespread RSV immunoprophylaxis use in these populations. UB hospitalization rates increased in all high

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

International Nuclear Information System (INIS)

Reittner, P.; Fotter, R.; Lindbichler, F.; Tillich, M.; Varga, E.M.; Zach, M.; Popper, H.

1997-01-01

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.)

Validation of a pediatric caregiver diary to measure symptoms of postacute respiratory syncytial virus bronchiolitis

DEFF Research Database (Denmark)

Santanello, Nancy C; Norquist, Josephine M; Nelsen, Linda M

2005-01-01

consistent, supporting a unidimensional scale structure. Test-retest reliabilities for the percentage of SFD and CSS were above the recommended cut point of 0.70. Cross-sectional and longitudinal correlations were sizeable and statistically significant, demonstrating construct validity. Hypothesized known......Acute respiratory syncytial virus (RSV)-induced bronchiolitis is often associated with continuing respiratory symptoms following hospitalization. To date, there is no validated objective measure to evaluate symptoms of RSV-induced bronchiolitis. We report on the reliability, validity...... the 4-week treatment period of the reported prospective, placebo-controlled trial of montelukast for treatment of postacute RSV were used to assess reliability (internal consistency and test-retest), construct validity (cross-sectional and longitudinal correlations), discriminant validity (known...

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.

Computed Tomography Imaging findings in Chemical Warfare Victims with pulmonary Complications

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Zahra Salehinezhad

2013-05-01

Full Text Available Introduction: Data on imaging findings in pulmonary complications of chemical agents is scarce. The current study aimed to evaluate radiological findings of late onset pulmonary complications in chemical warfare victims (CWV and to guide pulmonologists in diagnosis of these subjects. Materials and Methods: Ninety- three male CWV were enrolled in this prospective study, 20-25 years (mean=23 after exposure. Demographic and clinical data were recorded. High resolution computed Tomography (HRCT of the lung was performed during inspiration and expiration and was double reported blindly by two radiologists. Final diagnosis was made according to HRCT findings. The HRCT findings, final diagnosis, and distribution of the abnormalities were compared between subjects whom had been exposed to more complex chemical agents used during the second half of the war and simpler agents during the first half. Results: The most frequent HRCT findings were air trapping (56.7% and mosaic attenuation (35.1%. The distribution of abnormalities was mostly local (79.4% and bilateral (73% especially in lower regions (61.3%. The diagnosed respiratory diseases included bronchiolitis obliterans (43%, chronic obstructive pulmonary disease (COPD (27.9%, asthma (23.6%, bronchiectasis (13.9% and interstitial lung disease (ILD (9.6%. Frequency of subjects involved in the second half of the period of war was more than the first period (P-value < 0.05 but the HRCT findings were similar. Conclusions: Bronchiolitis obliterans with picture of focal bilateral air trapping was the most common finding in CWV but asthma appeared to have become a new problem in these subjects.

Cirrosis biliar primaria con neumonía organizada secundaria

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Almonte Batista WM

2017-10-01

Full Text Available Resume: Organized pneumonia, previously called bronchiolitis obliterans with organized pneumonia, is an interstitial lung disea- se that belongs to the group of idiopathic interstitial diseases and was first recognized in 1985. Sometimes it is not rela- ted to a specific cause calleing it in that case cryptogenetic, organized pneumonia but is frequently associated with other etiologies that may be infectious, inflammatory, neoplastic or secondary to drugs, it might also be related to extrapul- monary pathology as is the case of our patient in which is associated with a chronic cholestatic liver disease such as primary biliary cirrhosis.

Organising pneumonia presenting as acute life threatening pulmonary haemorrhage.

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Narasimhaiah, Damodhara Honnavally; Chakravorty, Indranil; Swamy, Rajiv; Prakash, Doraiswamy

2011-11-08

Organising pneumonia, previously called bronchiolitis obliterans organising pneumonia is a clinicopathological entity of unknown aetiology, which has been reported with increasing frequency. Various modes of presentation have been described such as cough, fever, weight loss and alveolar opacities on chest radiograph. Haemoptysis as primary presenting symptom has only rarely been reported. The authors report a case in which massive life-threatening haemoptysis was the major presenting symptom. No aetiology was identified for the haemoptysis and the diagnosis was confirmed on postmortem histology. This case highlights the importance of considering organising pneumonia in the differential diagnosis of acute severe haemoptysis.

CT findings of respiratory bronchiolitis caused by cigarette smoking

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Katagiri, Siro; Osima, K.; Kim, S. [Chiba Tokusyukai Hospital, Funabashi (Japan)

1998-07-01

CT scans were performed in 11 cases of respiratory bronchiolitis caused by cigarette smoking. Characteristics of CT findings were as follows: Remarkable visualization of the branching in peripheral bronchi within secondary lobules, multiple ground-glass opacities of centrilobular or lobular size adjacent to the above mentioned bronchial branching, thickening of the bronchial wall without dilatation, and no or minimal centrilobular emphysema. These characteristic CT findings were observed in all of 11 cases, who are current smokers, and never observed in non-smokers, ex-smokers and patients with apparent centrilobular emphysema. (author)

CT findings of respiratory bronchiolitis caused by cigarette smoking

International Nuclear Information System (INIS)

Katagiri, Siro; Osima, K.; Kim, S.

1998-01-01

CT scans were performed in 11 cases of respiratory bronchiolitis caused by cigarette smoking. Characteristics of CT findings were as follows: Remarkable visualization of the branching in peripheral bronchi within secondary lobules, multiple ground-glass opacities of centrilobular or lobular size adjacent to the above mentioned bronchial branching, thickening of the bronchial wall without dilatation, and no or minimal centrilobular emphysema. These characteristic CT findings were observed in all of 11 cases, who are current smokers, and never observed in non-smokers, ex-smokers and patients with apparent centrilobular emphysema. (author)

Low-dose computed tomography volumetry for subtyping chronic lung allograft dysfunction.

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Saito, Tomohito; Horie, Miho; Sato, Masaaki; Nakajima, Daisuke; Shoushtarizadeh, Hassan; Binnie, Matthew; Azad, Sassan; Hwang, David M; Machuca, Tiago N; Waddell, Thomas K; Singer, Lianne G; Cypel, Marcelo; Liu, Mingyao; Paul, Narinder S; Keshavjee, Shaf

2016-01-01

The long-term success of lung transplantation is challenged by the development of chronic lung allograft dysfunction (CLAD) and its distinct subtypes of bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS). However, the current diagnostic criteria for CLAD subtypes rely on total lung capacity (TLC), which is not always measured during routine post-transplant assessment. Our aim was to investigate the utility of low-dose 3-dimensional computed tomography (CT) lung volumetry for differentiating RAS from BOS. This study was a retrospective evaluation of 63 patients who had developed CLAD after bilateral lung or heart‒lung transplantation between 2006 and 2011, including 44 BOS and 19 RAS cases. Median post-transplant follow-up was 65 months in BOS and 27 months in RAS. The median interval between baseline and the disease-onset time-point for CT volumetry was 11 months in both BOS and RAS. Chronologic changes and diagnostic accuracy of CT lung volume (measured as percent of baseline) were investigated. RAS showed a significant decrease in CT lung volume at disease onset compared with baseline (mean 3,916 ml vs 3,055 ml when excluding opacities, p volumetry is a useful tool to differentiate patients who develop RAS from those who develop BOS. Copyright © 2016 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

Epinephrine Improves the Efficacy of Nebulized Hypertonic Saline in Moderate Bronchiolitis: A Randomised Clinical Trial.

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J Carlos Flores-González

Full Text Available There is no evidence that the epinephrine-3% hypertonic saline combination is more effective than 3% hypertonic saline alone for treating infants hospitalized with acute bronchiolitis. We evaluated the efficacy of nebulized epinephrine in 3% hypertonic saline.We performed a randomized, double-blind, placebo-controlled clinical trial in 208 infants hospitalized with acute moderate bronchiolitis. Infants were randomly assigned to receive nebulized 3% hypertonic saline with either 3 mL of epinephrine or 3 mL of placebo, administered every four hours. The primary outcome measure was the length of hospital stay.A total of 185 infants were analyzed: 94 in the epinephrine plus 3% hypertonic saline group and 91 in the placebo plus 3% hypertonic saline group. Baseline demographic and clinical characteristics were similar in both groups. Length of hospital stay was significantly reduced in the epinephrine group as compared with the placebo group (3.94 ±1.88 days vs. 4.82 ±2.30 days, P = 0.011. Disease severity also decreased significantly earlier in the epinephrine group (P = 0.029 and P = 0.036 on days 3 and 5, respectively.In our setting, nebulized epinephrine in 3% hypertonic saline significantly shortens hospital stay in hospitalized infants with acute moderate bronchiolitis compared to 3% hypertonic saline alone, and improves the clinical scores of severity from the third day of treatment, but not before.EudraCT 2009-016042-57.

Organizing pneumonia: what is it? A conceptual approach and pictorial review.

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Baque-Juston, M; Pellegrin, A; Leroy, S; Marquette, C H; Padovani, B

2014-09-01

Organizing pneumonia (formerly named bronchiolitis obliterans with organizing pneumonia or BOOP) is a clinical, radiological and histological entity that is classified as an Interstitial Lung Disease. The understanding of this family of diseases has seen great progress over the past twenty years. CT presentation of organizing pneumonia is polymorphous but a few patterns have been recently recognized as being more specific to this diagnosis. The aim of this work is to summarize new understandings of the clinical and histological presentation of the disease and to review the most relevant CT features. Copyright © 2014 Éditions françaises de radiologie. Published by Elsevier Masson SAS. All rights reserved.

Republished: Fibrosing organising pneumonia.

Science.gov (United States)

Beardsley, Brooke; Rassl, Doris

2014-08-01

Organising pneumonia (otherwise referred to as bronchiolitis obliterans organising pneumonia) is characterised histologically by plugs of granulation tissue, which are present predominantly within small airways, alveolar ducts and peri-bronchiolar alveoli. This pattern is not specific for any disorder or cause, but is one type of inflammatory response to pulmonary injury, which may be seen in a wide variety of clinical conditions. Typically, organising pneumonia responds very well to corticosteroid treatment; however, a small percentage of patients appear to develop progressive fibrosis. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

The relationship between nasopharyngeal CCL5 and microbiota on disease severity among infants with bronchiolitis.

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Hasegawa, K; Mansbach, J M; Ajami, N J; Petrosino, J F; Freishtat, R J; Teach, S J; Piedra, P A; Camargo, C A

2017-11-01

Emerging evidence suggests that the airway microbiota plays an important role in viral bronchiolitis pathobiology. However, little is known about the combined role of airway microbiota and CCL5 in infants with bronchiolitis. In this multicenter prospective cohort study of 1005 infants (age microbiota profiles with regard to the risk of both intensive care use (P interaction =.02) and hospital length-of-stay ≥3 days (P interaction =.03). Among infants with lower CCL5 levels, the Haemophilus-dominant microbiota profile was associated with a higher risk of intensive care use (OR, 3.20; 95%CI, 1.18-8.68; P=.02) and hospital length-of-stay ≥3 days (OR, 4.14; 95%CI, 2.08-8.24; Pmicrobiota profiles and these severity outcomes (all P≥.10). © 2017 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.

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.

[Clinical signs of dysphagia in infants with acute viral bronchiolitis].

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Barbosa, Lisiane De Rosa; Gomes, Erissandra; Fischer, Gilberto Bueno

2014-09-01

To determine the occurrence of clinical signs of dysphagia in infants with acute viral bronchiolitis, to compare the respiratory parameters during deglutition, and to ensure the intra- and inter- examiners agreement, as well as to accomplish intra and interexaminators concordance of the clinical evaluation of the deglutition. This was a cross-sectional study of 42 infants aged 0-12 months. The clinical evaluation was accompanied by measurements of respiratory rate and pulse oximetry. A score of swallowing disorders was designed to establish associations with other studied variables and to ensure the intra- and interrater agreement of clinical feeding assessments. Caregivers also completed a questionnaire about feeding difficulties. Significance was set at p<0.05. Changes in the oral phase (prolonged pauses) and pharyngeal phase (wheezing, coughing and gagging) of swallowing were found. A significant increase in respiratory rate between pre- and post-feeding times was found, and it was determined that almost half of the infants had tachypnea. An association was observed between the swallowing disorder scores and a decrease in oxygen saturation. Infants whose caregivers reported feeding difficulties during hospitalization stated a significantly greater number of changes in the swallowing evaluation. The intra-rater agreement was considered to be very good. Infants with acute viral bronchiolitis displayed swallowing disorders in addition to changes in respiratory rate and measures of oxygen saturation. It is suggested, therefore, that infants displaying these risk factors have a higher probability of dysphagia. Copyright © 2014 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

Cryptogenic Organising Pneumonia As The Initial Presenting Manifestation of SLE

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Neena Mampilly

2015-07-01

Full Text Available Cryptogenic Organising Pneumonia (COP, also called idiopathic Bronchiolitis Obliterans Organising Pneumonia( BOOP, is a distinct entity among the idiopathic interstitial pneumonias defined histopathologically by intraalveolar buds of granulation tissue. The etiology includes idiopathic, infectious, drug induced radiation induced and connective tissue diseases. Organising pneumonia occurs particularly in patients with dermatomyositis-polymyositis where it may be the presenting manifestation, and rarely in SLE, rheumatoid arthritis, scleroderma and other connective tissue diseases. We describe a 30 yr old lady who initially presented with respiratory symptoms, not responding to antibiotics. She was subsequently diagnosed as SLE and HRCT thorax showed consolidation involving both lung fields. A percutaneous lung biopsy revealed features of Cryptogenic Organising Pneumonia.

Nonatopic asthma is associated with helminth infections and bronchiolitis in poor children.

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Pereira, M U; Sly, P D; Pitrez, P M; Jones, M H; Escouto, D; Dias, A C O; Weiland, S K; Stein, R T

2007-06-01

Asthma is common in urban centres in Latin America, but atopic asthma may not be the main phenotype among children. Helminth infections are highly prevalent in poor populations, and it was hypothesised that they attenuate allergic asthma, whereas other factors are related to the expression of a nonatopic wheeze/asthma phenotype. A total of 1,982 children from Southern Brazil with a mean+/-sd age of 10.1+/-0.76 yrs completed asthma questionnaires, and 1,011 were evaluated for intestinal parasites and atopy using skin-prick tests (SPTs). Wheeze in the previous 12 months was reported by 25.6%, and 9.3% showed current asthma; 13% were SPT-positive and 19.1% were positive for any helminths. Most children with either wheeze or asthma were SPT-negative; however, severe wheeze was more prevalent among the atopic minority. Helminth infections were inversely associated with positive SPT results. Bronchiolitis before the age of 2 yrs was the major independent risk factor for asthma at age 10 yrs; high-load Ascaris infection, a family history of asthma and positive SPT results were also asthma risk factors. Most asthma and wheeze are of the nonatopic phenotype, suggesting that some helminths may exert an attenuating effect on the expression of the atopic portion of the disease, whereas viral bronchiolitis predisposes more specifically to recurrent airway symptoms.

Pulmonary manifestations of Sjögren's syndrome

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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.

How to Treat a Patient with Thromboangiitis Obliterans: A Systematic Review.

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Fazeli, Bahare; Dadgar Moghadam, Maliheh; Niroumand, Shabnam

2018-05-01

To date, there is still no treatment protocol for patients with thromboangiitis obliterans (TAO) who are also afflicted with critical limb ischemia (CLI). Smoking cessation on its own cannot be considered a treatment for the purposes of salvaging a limb of a TAO patient with CLI. The aim of this review was to evaluate different studies of various treatment protocols for avoiding amputation in TAO patients. A systematic search for relevant studies dating from 1990 to the end of 2016 was performed on the PubMed, SCOPUS, and Science Direct databases. Only 24 studies fulfilled the inclusion criteria, of which only one was a randomized controlled trial (RCT). The remaining studies were quasi-experimental with various treatments and follow-up durations. Therefore, meta-analysis was not performed. Judging from the major amputation rates after the suggested treatments were performed, no treatment was particularly effective. This review demonstrated that more standard RCTs are needed to resolve this treatment issue involved in TAO. In addition, because health insurance coverage for TAO patients differs by country, regional cost-benefit and cost-efficacy studies of the suggested treatments for TAO are highly recommended. Copyright © 2018 Elsevier Inc. All rights reserved.

The Role of Human Milk Immunomodulators in Protecting Against Viral Bronchiolitis and Development of Chronic Wheezing Illness.

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Dixon, Dani-Louise

2015-07-07

Infants who are breastfed are at an immunological advantage when compared with formula fed infants, evidenced by decreased incidence of infections and diminished propensity for long term conditions, including chronic wheeze and/or asthma. Exclusive breastfeeding reduces the duration of hospital admission, risk of respiratory failure and requirement for supplemental oxygen in infants hospitalised with bronchiolitis suggesting a potentially protective mechanism. This review examines the evidence and potential pathways for protection by immunomodulatory factors in human milk against the most common viral cause of bronchiolitis, respiratory syncytial virus (RSV), and subsequent recurrent wheeze in infants. Further investigations into the interplay between respiratory virus infections such as RSV and how they affect, and are affected by, human milk immunomodulators is necessary if we are to gain a true understanding of how breastfeeding protects many infants but not all against infections, and how this relates to long-term protection against conditions such as chronic wheezing illness or asthma.

Complicações pulmonares não infecciosas após transplante de células-tronco hematopoiéticas

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Eliane Viana Mancuzo

2010-09-01

evolution of hematopoietic stem cell transplantation receptors.The objective of this study is to review the most frequent non-infection complications associated to hematopoietic stem cell transplantation since the first bone marrow transplantation performed in 1957.A systematic literature review was performed, using the PICO strategy to ask the questions. The descriptors hematopoietic stem cell transplantation, non-infectious pulmonary complications, systematic review, in portuguese and their english correspondents, were used to access the following databases: MEDLINE, EBM, Embase, Cocrane Library, LILACS and SciELO. In this review, 263 studies were identified, from which 31 were selected for full analyzis.The non-infection pulmonary complications most frequently found were: bronchiolitis obliterans, bronchiolitis obliterans organizing pneumonia, pulmonary edema, idiopathic pneumonia syndrome, delayed pulmonary toxicity syndrome, diffuse alveolar hemorrhage, engrafment syndrome and pulmonary cytolitic thrombi.Rev Port Pneumol 2010; XVI (5: 815-828 Palavras-chave: Transplante de células-tronco hematopoiéticas, complicações pulmonares não infecciosas, revisão sistemática, Key-words: Hematopoietic stem cell transplantation, non-infectious pulmonary complications, systematic review

The modified respiratory index score (RIS) guides resource allocation in acute bronchiolitis.

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Chong, Shu-Ling; Teoh, Oon Hoe; Nadkarni, Nivedita; Yeo, Joo Guan; Lwin, Zaw; Ong, Yong-Kwang Gene; Lee, Jan Hau

2017-07-01

Bronchiolitis is a common disease in early childhood with increasing healthcare utilization. We aim to study how well a simple and improved respiratory score (the modified Respiratory Index Score [RIS]) would perform when predicting for a warranted admission. This is an observational prospective study, from June 2015 to December 2015 in a paediatric emergency department (ED) of a large tertiary hospital in Singapore. We included children aged less than 2 years old, presenting with typical symptoms and signs of bronchiolitis but excluded children with four or more previous wheezes, a gestation of hydration, and a hospital stay of 2 days or more. Among 1,818 patients, the median age was 10.8 months (IQR 7.2-15.9). The median modified RIS score was 4.0 (IQR 3.0-5.0). A total of 19 (1.0%) children required respiratory support, 101 (5.6%) received intravenous hydration, and 571 (31.4%) required a hospital stay of 2 days or more. After adjusting for age and duration of illness, a modified RIS score of >4 predicted significantly for a warranted admission (adjusted Odds Ratio: 3.28, 95% confidence interval: 2.62-4.12). The association remained significant among children presenting with their first wheeze. This simple respiratory tool predicts for the need for respiratory support, intravenous hydration, and a significant hospital stay of 2 days or more. Pediatr Pulmonol. 2017; 52:954-961. © 2017 Wiley Periodicals, Inc. © 2017 Wiley Periodicals, Inc.

Relapsing bronchiolitis obliterans organising pneumonia and chronic sarcoidosis in an atopic asthmatic patient.

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Carbonelli, C; Roggeri, A; Cavazza, A; Zompatori, M; Zucchi, L

2008-03-01

Asthma is thought to be a Th2 disease while sarcoidosis is considered a Th1 granulomatous disorder. Organising pneumonia is a histologic pattern of lung injury. When it has no recognisable cause it is defined as cryptogenic organising pneumonia. We herein report the case of a patient with recurrent and steroid sensitive organising pneumonia associated with chronic sarcoidosis in an atopic, moderate persistent asthmatic patient. Each disease has been documented with transbronchial biopsies and recurrence of organising pneumonia was suggested by clinical features and by follow up HRCT which shows distinctive signs even in associated disease. Steroids are the mainstay of therapy for these disorders and especially for the consolidated processes typical of organising pneumonia but prognostic indices for relapse and progression are lacking.

Heliox delivered by high flow nasal cannula improves oxygenation in infants with respiratory syncytial virus acute bronchiolitis

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Wael Seliem

2018-01-01

Conclusion: Transient improvement of oxygenation in infants with RSV acute bronchiolitis during the initial phase of the therapy is associated with heliox when provided with HFNC, may provide a precious time for other therapeutic agents to work or for the disease to resolve naturally, avoiding other aggressive interventions.

Severe respiratory syncytial virus bronchiolitis in infants is associated with reduced airway interferon gamma and substance P.

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Malcolm G Semple

2007-10-01

Full Text Available Severe human respiratory syncytial virus (hRSV bronchiolitis in previously well infants may be due to differences in the innate immune response to hRSV infection.to determine if factors mediating proposed mechanisms for severe bronchiolitis differ with severity of disease.197 infants admitted to hospital with hRSV bronchiolitis were recruited and grouped according to no oxygen requirement (n = 27, oxygen dependence (n = 114 or mechanical ventilation (n = 56. We collected clinical data, nasopharyngeal aspirate (NPA and if ventilated bronchoalveolar lavage (BAL. Interferon-gamma (IFN-gamma, substance P (SP, interleukin 9 (IL-9, urea and hRSV load, were measured in cell free supernatant from NPA and BAL. Multivariate analysis compared independent effects of clinical, virological and immunological variables upon disease severity. IFN-gamma and SP concentrations were lower in NPA from infants who required oxygen or mechanical ventilation. Viral load and IL-9 concentrations were high but did not vary with severity of disease. Independent predictors of severe disease (in diminishing size of effect were low weight on admission, low gestation at birth, low NPA IFN-gamma and NPA SP. Nasal airway sampling appears to be a useful surrogate for distal airway sampling since concentrations of IFN-gamma, SP, IL-9 and viral load in NPA correlate with the same in BAL.Our data support two proposed mechanisms for severe hRSV disease; reduced local IFN-gamma response and SP mediated inflammation. We found large amounts of hRSV and IL-9 in airways secretions from the upper and lower respiratory tract but could not associate these with disease severity.

Hazy increased density in diffuse lung disease

International Nuclear Information System (INIS)

Klein, J.S.; Webb, W.R.; Gamsu, G.; Warnock, M.; Park, C.K.

1989-01-01

In order to determine the significance of ground glass density on high-resolution CT scans of patients with idiopathic pulmonary fibrosis and other lung disorders, the authors have reviewed 200 high-resolution CT studies and found 50 cases demonstrating areas of hazy increased lung density. Disease entities most often associated with this finding included DIP, UIP, alveolar proteinosis, sarcoidosis, and bronchiolitis obliterans/ organizing pneumonia. Pathologic examination revealed either cellular or fluid material lining terminal air spaces, often associated with alveolar wall infiltration and an absence of fibrosis. Gallium scans and bronchoalveolar lavage in some cases showed active inflammation Follow-up high-resolution CT studies in 10 patients showed either change or resolution of the hazy densities, confirming the presence of a reversible parenchymal lesion

Microdialysis as a tool for drug quantification in the bronchioles of anaesthetized pigs

DEFF Research Database (Denmark)

Rottbøll, Lisa Amanda Holm; Friis, Christian

2014-01-01

through a tracheostomy into the distal bronchioles using an insertion tube. Intravenously administered inulin served as a marker of extracellular fluid contamination of PELF. Concentrations of free drug in MD fractions were compared to total and non-protein-bound drug concentrations in plasma. Rising...... equipment, the danofloxacin concentrations in PELF were consistent within the different administration groups. The described MD method for drug quantification in PELF is easily accessible and provides repeatable results. However, trace amount of inulin was detected in the MD fractions, suggesting a local...

Comparison of CPAP and HFNC in Management of Bronchiolitis in Infants and Young Children

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Majken Bisgaard Pedersen

2017-04-01

Full Text Available Continuous positive airway pressure (CPAP has been used in infants with bronchiolitis for decades. Recently, high flow nasal cannula (HFNC therapy was introduced We conducted a retrospective study of treatment with CPAP vs. HFNC between 2013 and 2015, comparing the development in respiratory rate, fraction of inspired oxygen (FiO2 and heart rate, treatment failure, duration of treatment, and length of hospital stay. A sample size of 49 children were included. Median age was 1.9 months. Median baseline pCO2 was 7.4 kPa in both groups, respiratory rate per minute was 57 vs. 58 (CPAP vs. HFNC. Respiratory rate decreased faster in the CPAP group (p < 0.05. FiO2 decreased in the CPAP group and increased in the HFNC group during the first 12 h, whereafter it decreased in both groups. (p < 0.01. Heart rate development was similar in both groups. Twelve children (55% changed systems from HFNC to CPAP due to disease progression. There was no difference in length of treatment, hospital stay, or transmission to intensive care unit between the groups. CPAP was more effective than HFNC in decreasing respiratory rate (RR and FiO2. No differences were observed in length of treatment or complications. Further studies should be conducted to compare the efficacy of the two treatments of bronchiolitis, preferably through prospective randomized trials.

Association of Obstructive Sleep Apnea Syndrome and Buerger's Disease: a Pilot Study

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Gholam Hosein Kazemzadeh

2015-10-01

Full Text Available In this study we evaluated the incidence and severity of obstructive sleep apnea and Obstructive sleep apnea syndrome in patients with thromboangiitis obliterans for reduction of crisis. In 40 patients with Buerger's disease daily sleepiness and risk of Obstructive sleep apnea were evaluated using the Epworth sleeping scale (ESS and the Stop-Bang score. An Apnea-link device was used for evaluation of chest motion, peripheral oxygenation, and nasal airflow during night-time sleep. The apnea/hypopnea index (AHI and respiratory tdisurbance index were used for Obstructive sleep apnea syndrome diagnosis. All subjects were cigarette smokers and 80% were opium addicted. The prevalence of Obstructive sleep apnea (AHI>5 was 80%, but incidence of Obstructive sleep apnea syndrome (AHI>5 + ESS≥10 was 5% (2/40. There was no association between duration or frequency of hospitalization and Obstructive sleep apnea syndrome (P=0.74 and 0.86, respectively. In addition, no correlation between ESS and Stop-Bang scores and AHI was observed (P=0.58 and 0.41, respectively. There was an inverse correlation between smoking rate and AHI (P=0.032, r = −0.48. We did not find an association between Buerger's disease and Obstructive sleep apnea syndrome. Although the AHI was high (80% and daily sleepiness was low. The negative correlation of smoking with AHI and on the other hand daily napping in addiction may be caused by the absence of a clear relationship between Obstructive sleep apnea syndrome and Buerger's disease.

Aging effects on airflow dynamics and lung function in human bronchioles.

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Kim, JongWon; Heise, Rebecca L; Reynolds, Angela M; Pidaparti, Ramana M

2017-01-01

The mortality rate for patients requiring mechanical ventilation is about 35% and this rate increases to about 53% for the elderly. In general, with increasing age, the dynamic lung function and respiratory mechanics are compromised, and several experiments are being conducted to estimate these changes and understand the underlying mechanisms to better treat elderly patients. Human tracheobronchial (G1 ~ G9), bronchioles (G10 ~ G22) and alveolar sacs (G23) geometric models were developed based on reported anatomical dimensions for a 50 and an 80-year-old subject. The aged model was developed by altering the geometry and material properties of the model developed for the 50-year-old. Computational simulations using coupled fluid-solid analysis were performed for geometric models of bronchioles and alveolar sacs under mechanical ventilation to estimate the airflow and lung function characteristics. The airway mechanical characteristics decreased with aging, specifically a 38% pressure drop was observed for the 80-year-old as compared to the 50-year-old. The shear stress on airway walls increased with aging and the highest shear stress was observed in the 80-year-old during inhalation. A 50% increase in peak strain was observed for the 80-year-old as compared to the 50-year-old during exhalation. The simulation results indicate that there is a 41% increase in lung compliance and a 35%-50% change in airway mechanical characteristics for the 80-year-old in comparison to the 50-year-old. Overall, the airway mechanical characteristics as well as lung function are compromised due to aging. Our study demonstrates and quantifies the effects of aging on the airflow dynamics and lung capacity. These changes in the aging lung are important considerations for mechanical ventilation parameters in elderly patients. Realistic geometry and material properties need to be included in the computational models in future studies.

Comparison of Initial Response of Nebulized Salbutamol and Adrenaline in Infants and young Children Admitted with Acute Bronchiolitis.

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Adhikari, S; Thapa, P; Rao, K S; Bk, G

2016-01-01

Background Acute bronchiolitis is common cause of hospitalization in infants and young children. There are widespread variations in the diagnosis and management. Despite the use of bronchodilators for decades, there is lack of consensus for the benefit of one above another. Objective To compare initial response of nebulized adrenaline and salbutamol. Method Children aged two months to two years admitted with acute bronchiolitis in the department of Paediatrics of Manipal teaching hospital, Pokhara, Nepal, from 1st March 2014 to 28th February 2015 were enrolled. Patients fulfilling inclusion criteria received either adrenaline or salbutamol nebulization. Data were collected in a predesigned proforma. Respiratory distress assessment instrument (RDAI) scores were considered primary outcome measure and respiratory rate at 48 hours, duration of hospital stay, requirement of supplemental oxygen and intravenous fluid were considered secondary outcome measure. Result A total of 40 patients were enrolled in each study group. Mean RDAI scores at admission was in 9.75 with (CI- 9.01, 10.49) in adrenaline and 9.77 (CI- 9.05, 10.50) in salbutamol group. There was gradual decline in mean RDAI scores in both the groups over 48 hours to 4.15 (CI- 3.57,4.73) and 4.13 (CI- 3.69,4.56) in adrenaline and salbutamol group respectively. Hospital stay was 5.32 days in adrenaline and 5.68 days in salbutamol group. Patients nebulized with adrenaline required oxygen for 33.30 hours compared with 36.45 hours in salbutamol. Intravenous fluid duration was also less in adrenaline group compared to salbutamol group (33.15 vs 37.80 hours). Conclusion Patients of acute bronchiolitis nebulized with either salbutamol or adrenaline experienced similar decline in RDAI scores in the first 48 hours. Duration of supplementary oxygen and intravenous fluid was less in adrenaline group compared with salbutamol group.

Popcorn worker's lung: In vitro exposure to diacetyl, an ingredient in microwave popcorn butter flavoring, increases reactivity to methacholine

International Nuclear Information System (INIS)

Fedan, J.S.; Dowdy, J.A.; Fedan, K.B.; Hubbs, A.F.

2006-01-01

Workers who inhale microwave popcorn butter flavorings experience decrements in lung function and can develop clinical bronchiolitis obliterans, i.e., 'popcorn worker's lung' (Kreiss, K., Gomaa, A., Kullman, G., Fedan, K., Simoes, E.J., Enright, P.L., 2002. Clinical bronchiolitis obliterans in workers at a microwave-popcorn plant. N. Engl. J. Med. 347, 330-338.). In a rat inhalation model, vapors of an artificial butter flavoring damaged the epithelium of the upper and lower airways (Hubbs, A.F., Battelli, L.A., Goldsmith, W.T., Porter, D.W., Frazer, D., Friend, S., Schwegler-Berry, D., Mercer, R.R., Reynolds, J.S., Grote, A., Castranova, V., Kullman, G., Fedan, J.S., Dowdy, J., Jones, W.G., 2002. Necrosis of nasal and airway epithelium in rats inhaling vapors of artificial butter flavoring. Toxicol. Appl. Pharmacol. 185, 128-135.). Diacetyl, a butter flavoring component, is a major volatile ketone in the popcorn-processing workplace. We investigated the effects of diacetyl on epithelium of guinea pig isolated airway preparations and the effects of diacetyl in vitro on reactivity to bronchoactive agents. In the isolated, perfused trachea preparation, diacetyl added to the intraluminal (mucosal) bath elicited responses that began with contraction (threshold ca. 3 mM) and ended with relaxation. After a 4-h incubation with intraluminal diacetyl (3 mM), contractions to extraluminal (serosal) methacholine (MCh) were slightly increased; however, sensitivity to intraluminally (mucosally) applied MCh was increased by 10-fold. Relaxation responses of MCh (3 x 10 -7 M)-contracted tracheas to extraluminally applied terbutaline and intraluminally applied 120 mM KCl, to evoke epithelium-derived relaxing factor release, were unaffected by diacetyl. Exposure of the tracheal epithelium in Ussing chambers to diacetyl decreased transepithelial potential difference and resistance. These findings suggest that diacetyl exposure compromised epithelial barrier function, leading to

Respiratory syncytial virus (RSV bronchiolitis: comparative study of RSV groups A and B infected children

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Selir M. Straliotto

1994-03-01

Full Text Available The grouping characteristics of 29 respiratory syncitial virus (RSV present in nasopharyngeal cells collectedfrom hospitalized children with bronchiolitis during the 1990RSVseason in Porto Alegre, RS, were analysed. Twenty-two were grouped as belonging to group A and 7 to group B. Cyanosis, oxigen therapy, cough, lenght of hospitalization and atelectasis were observed to be more frequently found within group B infected children. Other clinical signs and symptoms were similarly found in both groups.

High-resolution computed tomography findings in pulmonary complications after bone marrow transplantation: iconographic essay

International Nuclear Information System (INIS)

Gasparetto, Emerson L.; Ono, Sergio E.; Souza, Carolina A.; Escuissato, Dante L.; Rocha, Gabriela de Melo; Inoue, Cezar; Falavigna, Joao M.; Marchiori, Edson; Universidade Federal, Rio de Janeiro

2005-01-01

Bone marrow transplantation has been the treatment of choice for many hematologic diseases. However, pulmonary complications, which may occur in up to 60% of the patients, are the main cause of treatment failure and may be divided in three phases according to the patient's immunity. In the first phase, up to 30 days after the procedure, there is a predominance of non-infectious complications and fungal pneumonia. Viral infections, mainly by cytomegalovirus, are common in the second phase (up to 100 days after bone marrow transplantation). Finally, in the late phase after bone marrow transplantation, non-infectious complications as bronchiolitis obliterans organizing pneumonia and graft-versus-host disease are most commonly seen. The authors present a pictorial essay of the high-resolution computed tomography findings in patients with pulmonary complications after bone marrow transplantation. (author)

Banff study of pathologic changes in lung allograft biopsy specimens with donor-specific antibodies

DEFF Research Database (Denmark)

Wallace, William Dean; Li, Ning; Andersen, Claus B

2016-01-01

a statistically significant difference vs NABs in the setting of acute lung injury, with or without diffuse alveolar damage (p = 0.0008), in the presence of capillary neutrophilic inflammation (p = 0.0014), and in samples with endotheliitis (p = 0.0155). In samples with complement 4d staining, there was a trend......-DSAs, and no antibodies (NABs) present. The significance of each histologic variable was reviewed. RESULTS: We found no statistically significant association with acute cellular rejection, airway inflammation, or bronchiolitis obliterans and the presence or absence of antibodies. However, biopsy specimens with DSAs had...... but no statistically significant difference between specimens associated with DSAs and specimens with NABs. CONCLUSIONS: Capillary inflammation, acute lung injury, and endotheliitis significantly correlated with DSAs. The infrequently observed diffuse staining for complement 4d limits the usefulness of this stain....

Role of long term antibiotics in chronic respiratory diseases.

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Suresh Babu, K; Kastelik, J; Morjaria, J B

2013-06-01

Antibiotics are commonly used in the management of respiratory disorders such as cystic fibrosis (CF), non-CF bronchiectasis, asthma and COPD. In those conditions long-term antibiotics can be delivered as nebulised aerosols or administered orally. In CF, nebulised colomycin or tobramycin improve lung function, reduce number of exacerbations and improve quality of life (QoL). Oral antibiotics, such as macrolides, have acquired wide use not only as anti-microbial agents but also due to their anti-inflammatory and pro-kinetic properties. In CF, macrolides such as azithromycin have been shown to improve the lung function and reduce frequency of infective exacerbations. Similarly macrolides have been shown to have some benefits in COPD including reduction in a number of exacerbations. In asthma, macrolides have been reported to improve some subjective parameters, bronchial hyperresponsiveness and airway inflammation; however have no benefits on lung function or overall asthma control. Macrolides have also been used with beneficial effects in less common disorders such as diffuse panbronchiolitis or post-transplant bronchiolitis obliterans syndrome. In this review we describe our current knowledge the use of long-term antibiotics in conditions such as CF, non-CF bronchiectasis, asthma and COPD together with up-to-date clinical and scientific evidence to support our understanding of the use of antibiotics in those conditions. Copyright © 2013 Elsevier Ltd. All rights reserved.

Relative impact of human leukocyte antigen mismatching and graft ischemic time after lung transplantation.

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Brugière, Olivier; Thabut, Gabriel; Suberbielle, Caroline; Reynaud-Gaubert, Martine; Thomas, Pascal; Pison, Christophe; Saint Raymond, Christel; Mornex, Jean-François; Bertocchi, Michèle; Dromer, Claire; Velly, Jean-François; Stern, Marc; Philippe, Bruno; Dauriat, Gaëlle; Biondi, Giuseppina; Castier, Yves; Fournier, Michel

2008-06-01

Recent data strongly suggest that human leukocyte antigen (HLA) mismatching has a negative impact on development of bronchiolitis obliterans syndrome (BOS) and survival after lung transplantation (LTx). Because HLA matching is sometimes achieved by extending ischemic time in other solid-organ transplantation models and ischemic time is a risk factor per se for death after LTx, we sought to compare the theoretical benefit of HLA matching with the negative impact of lengthened ischemic time. In this collaborative study we compared the relative impact of HLA mismatching and ischemic time on BOS and survival in 182 LTx recipients. Using multivariate analyses, we observed a lower incidence of BOS (hazard ratio [HR] = 1.70, 95% confidence interval [CI]: 1.1 to 2.7, p = 0.03) and enhanced survival (HR = 1.91, 95% CI: 1.24 to 2.92, p = 0.01) in patients with zero or one HLA-A mismatch compared with those having two HLA-A mismatches. This beneficial effect on survival was equivalent to a reduction of ischemic time of 168 minutes. We observed a reduced incidence of BOS and a better survival rate in patients well-matched at the HLA-A locus, associated with an opposite effect of an enhanced ischemic time. This suggests that graft ischemic time should be taken into account in future studies of prospective HLA matching in LTx.

The Induction of IgM and IgG Antibodies against HLA or MICA after Lung Transplantation

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Annelieke W. M. Paantjens

2011-01-01

Full Text Available The production of IgG HLA antibodies after lung transplantation (LTx is considered to be a major risk factor for the development of chronic rejection, represented by the bronchiolitis obliterans syndrome (BOS. It has recently been observed that elevated levels of IgM HLA antibodies also correlates with the development of chronic rejection in heart and kidney transplantation. This study investigates the relationship between IgM and IgG antibodies against HLA and MICA after lung transplantation. Serum was collected from 49 patients once prior to transplantation and monthly for up to 1 year after lung transplantation was analyzed by Luminex to detect IgM and IgG antibodies against HLA and MICA. The presence of either IgM or IgG HLA and/or MICA antibodies prior to or after transplantation was not related to survival, gender, primary disease, or the development of BOS. Additionally, the production of IgG alloantibodies was not preceded by an increase in levels of IgM, and IgM levels were not followed by an increase in IgG. Under current immune suppressive regimen, although the presence of IgM antibodies does not correlate with BOS after LTx, IgM high IgG low HLA class I antibody titers were observed more in patients with BOS compared to patients without BOS.

Temporal Changes in Prescription of Neuropharmacologic Drugs and Utilization of Resources Related to Neurologic Morbidity in Mechanically Ventilated Children With Bronchiolitis.

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Shein, Steven L; Slain, Katherine; Wilson-Costello, Deanne; McKee, Bryan; Rotta, Alexandre T

2017-12-01

Critically ill children with bronchiolitis may require neuropharmacologic medications and support for neuro-functional sequelae, but current practices are not well described. We aimed to describe recent trends in neuropharmacology and utilization of neuro-rehabilitation resources in mechanically ventilated children with bronchiolitis. Analysis of the multicenter Pediatric Health Information System database. Forty-seven U.S. children's hospitals. PICU patients less than 2 years old with bronchiolitis undergoing mechanical ventilation between 2006 and 2015. None. Annual rates of utilization of neuropharmacologic medications (sedatives, analgesics, etc) and of neuro-rehabilitation services (physical therapy, neurologic consultation, etc) over the 10-year study period were compared. Neuropharmacologic medications prescribed on greater than or equal to 2 days were extracted. Utilization of MRI of the brain, neurologic consultation, swallow evaluation, occupational therapy, and physical therapy was also extracted. Among 12,508 subjects, the median age was 2.8 months, ~50% had comorbid conditions, and the median duration of mechanical ventilation was 7 days. The percentage of children prescribed greater than or equal to five drugs/drug classes increased over the study period from 36.5% to 55.8% (p use of greater than or equal to one service (36.3% in 2006 to 59.6% in 2015; p use of greater than or equal to two services (20.8% to 34.8%; p use of vasoactive medications and mortality did not. Prescription of neuropharmacologic agents increased over time using metrics of both overall drug burden and specific drug usage. Concurrently, the utilization of services that evaluate and/or treat neurologic morbidity was common and also increased over time.

Effectiveness of chest physiotherapy in infants hospitalized with acute bronchiolitis: a multicenter, randomized, controlled trial.

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Vincent Gajdos

2010-09-01

Full Text Available Acute bronchiolitis treatment in children and infants is largely supportive, but chest physiotherapy is routinely performed in some countries. In France, national guidelines recommend a specific type of physiotherapy combining the increased exhalation technique (IET and assisted cough (AC. Our objective was to evaluate the efficacy of chest physiotherapy (IET + AC in previously healthy infants hospitalized for a first episode of acute bronchiolitis.We conducted a multicenter, randomized, outcome assessor-blind and parent-blind trial in seven French pediatric departments. We recruited 496 infants hospitalized for first-episode acute bronchiolitis between October 2004 and January 2008. Patients were randomly allocated to receive from physiotherapists three times a day, either IET + AC (intervention group, n=246 or nasal suction (NS, control group, n=250. Only physiotherapists were aware of the allocation group of the infant. The primary outcome was time to recovery, defined as 8 hours without oxygen supplementation associated with minimal or no chest recession, and ingesting more than two-thirds of daily food requirements. Secondary outcomes were intensive care unit admissions, artificial ventilation, antibiotic treatment, description of side effects during procedures, and parental perception of comfort. Statistical analysis was performed on an intent-to-treat basis. Median time to recovery was 2.31 days, (95% confidence interval [CI] 1.97-2.73 for the control group and 2.02 days (95% CI 1.96-2.34 for the intervention group, indicating no significant effect of physiotherapy (hazard ratio [HR]=1.09, 95% CI 0.91-1.31, p=0.33. No treatment by age interaction was found (p=0.97. Frequency of vomiting and transient respiratory destabilization was higher in the IET + AC group during the procedure (relative risk [RR]=10.2, 95% CI 1.3-78.8, p=0.005 and RR=5.4, 95% CI 1.6-18.4, p=0.002, respectively. No difference between groups in bradycardia with or

Dipyridamole thallium-201 single-photon emission computed tomography for prediction of perioperative cardiac events in patients with arteriosclerosis obliterans undergoing vascular surgery

International Nuclear Information System (INIS)

Ziyang, Huang; Komori, Sadayoshi; Sawanobori, Takao

1998-01-01

The aim of the study was to determine whether or not dipyridamole thallium-201 single-photon emission computed tomography ( 201 Tl-SPECT) has significant additive value for predicting perioperative cardiac events in patients with arteriosclerosis obliterans (ASO) undergoing vascular surgery. Routine preoperative 201 Tl-SPECT was performed in 106 consecutive patients with ASO (age 68±8.9 years; 91 men and 15 women). The frequency of reversible defects in a clinical high-risk group (n=44) was significantly higher than in a low-risk group (n=62; 55% vs 24%, p 201 Tl-SPECT data to clinical risk-stratified patients with ASO allows better prediction of perioperative cardiac events. (author)

Elevation of Serum Acid Sphingomyelinase Activity in Children with Acute Respiratory Syncytial Virus Bronchiolitis.

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Yoshida, Shuichiro; Noguchi, Atsuko; Kikuchi, Wataru; Fukaya, Hiroshi; Igarashi, Kiyoshi; Takahashi, Tsutomu

2017-12-01

Acid sphingomyelinase (ASM) is a lysosomal enzyme that hydrolyzes sphingomyelin into ceramide, a bioactive lipid to regulate cellular physiological functions. Thus, ASM activation has been reported as a key event in pathophysiological reactions including inflammation, cytokine release, oxidative stress, and endothelial damage in human diseases. Since ASM activation is associated with extracellular ASM secretion through unknown mechanisms, it can be detected by recognizing the elevation of secretory ASM (S-ASM) activity. Serum S-ASM activity has been reported to increase in chronic diseases, acute cardiac diseases, and systemic inflammatory diseases. However, the serum S-ASM has not been investigated in common acute illness. This study was designed to evaluate serum S-ASM activity in children with common acute illness. Fifty children with common acute illness and five healthy children were included in this study. The patients were categorized into five groups based on clinical diagnoses: acute respiratory syncytial virus (RSV) bronchiolitis, adenovirus infection, streptococcal infection, asthma, and other infections due to unknown origin. The serum S-ASM activity was significantly elevated at 6.9 ± 1.6 nmol/0.1 mL/6 h in the group of acute RSV bronchiolitis patients compared with healthy children who had a mean level of 1.8 ± 0.8 nmol/0.1 mL/6 h (p ASM activity was not significantly elevated. The results suggest an association of ASM activation with RSV infection, a cause for common acute illness. This is the first report to describe the elevation of serum S-ASM activity in respiratory tract infection.

Systematic Review and Meta-Analysis of the Efficacy and Safety of Combined Epinephrine and Corticosteroid Therapy for Acute Bronchiolitis in Infants

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Kok P. Kua

2017-06-01

Full Text Available Objective: To evaluate the effectiveness of combined epinephrine and corticosteroid therapy for acute bronchiolitis in infants.Methods: Four electronic databases (MEDLINE, EMBASE, CINAHL, and CENTRAL were searched from their inception to February 28, 2017 for studies involving infants aged less than 24 months with bronchiolitis which assessed the use of epinephrine and corticosteroid combination therapy. The methodological quality of the included studies was assessed using the Cochrane Collaboration's Risk of Bias Tool. A random-effects meta-analysis was used to pool the effect estimates. The primary outcomes were hospital admission rate and length of hospital stay.Results: Of 1,489 citations identified, 5 randomized controlled trials involving 1,157 patients were included. All studies were of high quality and low risk of bias. Results of the meta-analysis showed no significant differences in the primary outcomes. Hospitalization rate was reduced by combinatorial therapy of epinephrine and corticosteroid in only one out of five studies, whereas pooled data indicated no benefit over epinephrine plus placebo. Clinical severity scores were significantly improved in all five RCTs when assessed individually, but no benefit was observed compared to epinephrine monotherapy when the data were pooled together. Pooled data showed that combination therapy was more effective at improving oxygen saturation level (mean difference: −0.70; 95% confidence interval: −1.17 to −0.22, p = 0.004. There was no difference in the risk of serious adverse events in infants treated with the combined epinephrine and corticosteroid therapy.Conclusions: Combination treatment of epinephrine and dexamethasone was ineffective in reducing hospital admission and length of stay among infants with bronchiolitis.

GENETIC SUSCEPTIBILITY TO RESPIRATORY SYNCYTIAL VIRUS BRONCHIOLITIS IN PRETERM CHILDREN IS ASSOCIATED WITH AIRWAY REMODELING GENES AND INNATE IMMUNE GENES

NARCIS (Netherlands)

Siezen, Christine L. E.; Bont, Louis; Hodemaekers, Hennie M.; Ermers, Marieke J.; Doornbos, Gerda; van't Slot, Ruben; Wijmenga, Ciska; van Hottwelingen, Hans C.; Kimpen, Jan L. L.; Kimman, Tjeerd G.; Hoebee, Barbara; Janssen, Riny

Prematurity is a risk factor for severe respiratory syncytial virus bronchiolitis. We show that genetic factors in innate immune genes (IFNA13, IFNAR2, STAT2. IL27, NFKBIA, C3, IL1RN, TLR5), in innate and adaptive immunity (IFNG), and in airway remodeling genes (ADAM33 and TGFBR1), affect disease

Impact and safety of open lung biopsy in patients with acute respiratory distress syndrome (ARDS).

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Ortiz, G; Garay, M; Mendoza, D; Cardinal-Fernández, P

2018-02-28

Acute respiratory distress syndrome (ARDS) is an inflammatory lung disorder, and its pathological hallmark is diffuse alveolar damage (DAD). Given that open lung biopsy (OLB) can sometimes result in severe side effects, it is rarely performed in patients with ARDS. The aims of this study were to describe: (a) the rate of treatment change associated with the histological result; and (b) the incidence of side effects induced by OLB. A retrospective, single-center, descriptive observational study was carried out in Hospital Santa Clara (Bogotá, Colombia) from February 2007 to January 2014. Critically ill patients over 18 years of age, undergoing invasive mechanical ventilation, diagnosed with ARDS of unknown etiology, and with OLB performed at the bedside. ARDS was diagnosed according to the Berlin definition. DAD was defined by the presence of a hyaline membrane plus at least one of the following: intra-alveolar edema, alveolar type I cell necrosis, alveolar type II cell (cuboidal cells) proliferation progressively covering the denuded alveolar-capillary membrane, interstitial proliferation of fibroblasts and myofibroblasts, or organizing interstitial fibrosis. The rate of treatment change (RTC) was established according to whether the OLB pathology report resulted in: a) the prescription or discontinuation of an antimicrobial; b) the indication of new procedures; c) medical interconsultation; or d) limitation of therapeutic effort. Patients were followed-up until death or hospital discharge. This study was approved by the Ethics Committee. A total of 32 OLBs were performed during the study period; 17 were ruled out as they did not involve ARDS, and 15 were considered for further analysis. A histological diagnosis was reached in 14 of the 15 patients (12 DAD, one case of bronchiolitis obliterans organizing pneumonia and one case of Wegener's granulomatosis with alveolar hemorrhage). The RTC rate was 0.73. The most frequent intervention was discontinuation of

Clinical, radiological, and physiological differences between obliterative bronchiolitis and problematic severe asthma in adolescents and young adults: the early origins of the overlap syndrome?

Science.gov (United States)

Bandeira, Teresa; Negreiro, Filipa; Ferreira, Rosário; Salgueiro, Marisa; Lobo, Luísa; Aguiar, Pedro; Trindade, J C

2011-06-01

Few reports have compared chronic obstructive lung diseases (OLDs) starting in childhood. To describe functional, radiological, and biological features of obliterative bronchiolitis (OB) and further discriminate to problematic severe asthma (PSA) or to diagnose a group with overlapping features. Patients with OB showed a greater degree of obstructive lung defect and higher hyperinflation (P < 0.001). The most frequent high-resolution computed tomography (HRCT) features (increased lung volume, inspiratory decreased attenuation, mosaic pattern, and expiratory air trapping) showed significantly greater scores in OB patients. Patients with PSA have shown a higher frequency of atopy (P < 0.05). ROC curve analysis demonstrated discriminative power for the LF variables, HRCT findings and for atopy between diagnoses. Further analysis released five final variables more accurate for the identification of a third diagnostic group (FVC%t, post-bronchodilator ΔFEV(1) in ml, HRCT mosaic pattern, SPT, and D. pteronyssinus-specific IgE). We found that OB and PSA possess identifiable characteristic features but overlapping values may turn them undistinguishable. Copyright © 2011 Wiley-Liss, Inc.

Fatores de risco associados à hospitalização por bronquiolite aguda no período pós-neonatal Risk factors associated with hospitalization for bronchiolitis in the post-neonatal period

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Elaine P Albernaz

2003-08-01

Full Text Available OBJETIVO: Investigar a relação entre fatores socioeconômicos, características maternas, aleitamento e hospitalização por bronquiolite no período pós-neonatal. MÉTODOS: Estudo caso-controle, aninhado a estudo de coorte de 5.304 crianças nascidas na cidade de Pelotas, RS. O estudo de coorte constitui-se de quatro subestudos, cada um com métodos e logística específicos. As mães foram entrevistadas por meio de questionário padronizado, em visitas hospitalares e domiciliares. Foram definidos como casos as crianças com idade entre 28 e 364 dias, que haviam sido hospitalizadas por bronquiolite. RESULTADOS: De 5.304 crianças da coorte, 113 (2,1% foram hospitalizadas por bronquiolite. A análise multivariada hierarquizada, realizada pela regressão logística, mostrou os seguintes resultados: renda familiar, assim como idade gestacional, estiveram inversamente relacionadas ao risco de hospitalização por bronquiolite. O aleitamento materno desempenhou um fator protetor; crianças com tempo de aleitamento materno inferior a um mês tiveram risco 7 vezes maior de serem hospitalizadas por bronquiolite aguda nos primeiros três meses de vida. O risco de hospitalização por bronquiolite foi 57% maior naquelas expostas ao fumo materno. CONCLUSÕES: A hospitalização por bronquiolite está inversamente relacionada com renda familiar, idade gestacional e tempo de aleitamento materno, e diretamente relacionada com exposição a fumo materno. Não foi evidenciada associação com paridade ou história materna de asma brônquica.OBJECTIVE: To investigate the relationship between socioeconomic factors, maternal characteristics, breastfeeding, and hospitalization for bronchiolitis in the post-neonatal period. METHODS: A nested case-control study with a cohort of 5,304 children born in the city of Pelotas, Brazil, was conducted. The cohort study consisted of four sub-studies with their own methods and logistics. Mothers were interviewed using a

Proteína C reactiva e gravidade da bronquiolite aguda C Reactive protein and disease severity in bronchiolitis

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S Costa

2009-01-01

Full Text Available O diagnóstico de bronquiolite aguda é essencialmente clínico. A utilidade de exames laboratoriais, em particular do doseamento da proteína C reactiva (PCR, não está bem estabelecida. O objectivo deste trabalho foi avaliar a relação entre o valor da PCR e os marcadores indirectos de gravidade em doentes com diagnóstico de bronquiolite. Foram incluídos no estudo todos os doentes admitidos no Serviço de Pediatria do HSJ durante o ano de 2006 e que tiveram como diagnóstico principal bronquiolite, tendo sido feita uma revisão retrospectiva do processo clínico desses doentes. Foram incluídos 176 doentes com idades compreendidas entre zero e 36 meses (mediana de 4 meses; 63,1% eram do sexo masculino. O doseamento da PCR tinha sido efectuado em 94,3% dos doentes, com valores compreendidos entre zero e 256mg/L e com mediana de 11 mg/L. O valor da PCR, na população estudada, apresentou uma relação estatisticamente significativa com a admissão em unidade de cuidados intensivos (UCI (p=0,008, a duração do internamento hospitalar (p=0,025 e a necessidade de oxigenoterapia (p=0,022. Desta forma, este trabalho coloca a hipótese de o valor de PCR poder ser um marcador de gravidade e ter significado prognóstico em doentes com bronquiolite. Mais estudos são necessários para validar estes resultados e esclarecer se existe uma relação real entre as variáveis estudadas ou se esta é produzida pelo efeito confundidor de outras infecções.A diagnosis of bronchiolitis is made clinically and the use of supportive laboratory examinations, including the quantification of C reactive protein (CRP, is not well established. The aim of this study was to evaluate the correlation between CRP value and indirect markers of disease severity in patients with bronchiolitis. This study included the patients diagnosed with bronchiolitis admitted to the Pediatrics Department of S. João Hospital in 2006. A retrospective review was made through analysing

Acute lung injury and persistent small airway disease in a rabbit model of chlorine inhalation

Energy Technology Data Exchange (ETDEWEB)

Musah, Sadiatu; Schlueter, Connie F.; Humphrey, David M. [Department of Environmental and Occupational Health Sciences, School of Public Health and Information Sciences, University of Louisville, Louisville, KY (United States); Powell, Karen S. [Research Resource Facilities, University of Louisville, Louisville, KY (United States); Roberts, Andrew M. [Department of Physiology, University of Louisville, Louisville, KY (United States); Hoyle, Gary W., E-mail: Gary.Hoyle@louisville.edu [Department of Environmental and Occupational Health Sciences, School of Public Health and Information Sciences, University of Louisville, Louisville, KY (United States)

2017-01-15

Chlorine is a pulmonary toxicant to which humans can be exposed through accidents or intentional releases. Acute effects of chlorine inhalation in humans and animal models have been well characterized, but less is known about persistent effects of acute, high-level chlorine exposures. In particular, animal models that reproduce the long-term effects suggested to occur in humans are lacking. Here, we report the development of a rabbit model in which both acute and persistent effects of chlorine inhalation can be assessed. Male New Zealand White rabbits were exposed to chlorine while the lungs were mechanically ventilated. After chlorine exposure, the rabbits were extubated and were allowed to survive for up to 24 h after exposure to 800 ppm chlorine for 4 min to study acute effects or up to 7 days after exposure to 400 ppm for 8 min to study longer term effects. Acute effects observed 6 or 24 h after inhalation of 800 ppm chlorine for 4 min included hypoxemia, pulmonary edema, airway epithelial injury, inflammation, altered baseline lung mechanics, and airway hyperreactivity to inhaled methacholine. Seven days after recovery from inhalation of 400 ppm chlorine for 8 min, rabbits exhibited mild hypoxemia, increased area of pressure–volume loops, and airway hyperreactivity. Lung histology 7 days after chlorine exposure revealed abnormalities in the small airways, including inflammation and sporadic bronchiolitis obliterans lesions. Immunostaining showed a paucity of club and ciliated cells in the epithelium at these sites. These results suggest that small airway disease may be an important component of persistent respiratory abnormalities that occur following acute chlorine exposure. This non-rodent chlorine exposure model should prove useful for studying persistent effects of acute chlorine exposure and for assessing efficacy of countermeasures for chlorine-induced lung injury. - Highlights: • A novel rabbit model of chlorine-induced lung disease was developed. �

Better newborn vitamin D status lowers RSV-associated bronchiolitis in infants.

Science.gov (United States)

Maxwell, Christy S; Carbone, Elena T; Wood, Richard J

2012-09-01

Each year 1.5 million children under the age of 5 years die from pneumonia. In the United States, respiratory syncytial virus (RSV) is the number one cause of bronchiolitis and pneumonia in children under 1 year of age. Low serum 25(OH)D is associated with an increased risk of lower respiratory tract infections (LRTI). Two recent studies have provided important information concerning the association between cord blood 25(OH)D and subsequent risk of developing respiratory infection in very young children. These findings support the need in future studies to determine the extent to which an intervention to change the vitamin D status of mothers during pregnancy can reduce the risk of RSV-associated LRTI in their offspring. An answer to this question would have significant worldwide public health importance given the high prevalence of low vitamin D status worldwide and the high mortality burden accompanying infectious lung diseases in young children. © 2012 International Life Sciences Institute.

Popcorn-worker lung caused by corporate and regulatory negligence: an avoidable tragedy.

Science.gov (United States)

Egilman, David; Mailloux, Caroline; Valentin, Claire

2007-01-01

Diacetyl-containing butter flavor was identified as the cause of an outbreak of bronchiolitis obliterans (BO) and other lung diseases in popcorn-plant workers. Litigation documents show that the outbreak was both predictable and preventable. The industry trade organization was aware of BO cases in workers at butter-flavoring and popcorn-manufacturing plants but often failed to implement industrial hygiene improvements and actively hid pertinent warning information. Due to weaknesses in the organization and mandates of regulatory bodies, organizations such as NIOSH, OSHA, the FDA, particularly the "generally recognized as safe" (GRAS) system, and the EPA failed to detect and prevent the outbreak, which highlights the need for systemic changes in food-product regulation, including the need for corporations to act responsibly, for stronger regulations with active enforcement, for a restructuring of the GRAS system, and for criminal penalties against corporations and professionals who knowingly hide information relevant to worker protection.

Efeito anti-inflamatório dos macrolídeos em doenças pulmonares da infância Anti-inflammatory effects of macrolides in childhood lung diseases

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Fernanda Luisi

2012-12-01

Full Text Available Os macrolídeos são fármacos com efeitos antimicrobianos especialmente contra patógenos intracelulares. Vários estudos têm demonstrado possíveis efeitos anti-inflamatórios dos macrolídeos. Esses medicamentos inibem a produção de algumas interleucinas e podem reduzir a inflamação neutrofílica pulmonar. Ensaios clínicos têm demonstrado efeitos benéficos dos macrolídeos em diversas doenças pulmonares crônicas. O objetivo deste estudo foi revisar os dados recentes da literatura médica sobre os efeitos anti-inflamatórios dos macrolídeos nas doenças respiratórias da infância, através da pesquisa da base de dados Medline (PubMed dos seguintes termos em inglês: "macrolide and cystic fibrosis"; "macrolide and asthma"; "macrolide and bronchiolitis obliterans"; e "macrolide and acute bronchiolitis" Foram selecionados artigos publicados em revistas científicas internacionais entre 2001 e 2012. Estudos clínicos e evidências in vitro comprovam o efeito anti-inflamatório dos macrolídeos em doenças respiratórias. Alguns ensaios clínicos demonstram benefícios na administração de macrolídeos em pacientes com fibrose cística; porém, o risco de resistência bacteriana deve ser considerado na análise desses benefícios. Tais benefícios são controversos em outras doenças respiratórias, e seu uso rotineiro não está indicado. Mais estudos clínicos controlados são necessários para avaliar a eficácia desses medicamentos como anti-inflamatórios. Dessa forma, poderemos definir melhor os benefícios dos macrolídeos no tratamento de cada uma das situações clínicas especificadas.Macrolides are drugs that have antimicrobial effects, especially against intracellular pathogens. Various studies have shown that macrolides might also have anti-inflammatory effects. Macrolides inhibit the production of interleukins and can reduce pulmonary neutrophilic inflammation. Clinical trials have demonstrated beneficial effects of

Parental and household smoking and the increased risk of bronchitis, bronchiolitis and other lower respiratory infections in infancy: systematic review and meta-analysis

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Britton John

2011-01-01

Full Text Available Abstract Background Passive smoke exposure increases the risk of lower respiratory infection (LRI in infants, but the extensive literature on this association has not been systematically reviewed for nearly ten years. The aim of this paper is to provide an updated systematic review and meta-analysis of studies of the association between passive smoking and LRI, and with diagnostic subcategories including bronchiolitis, in infants aged two years and under. Methods We searched MEDLINE and EMBASE (to November 2010, reference lists from publications and abstracts from major conference proceedings to identify all relevant publications. Random effect pooled odds ratios (OR with 95% confidence intervals (CI were estimated. Results We identified 60 studies suitable for inclusion in the meta-analysis. Smoking by either parent or other household members significantly increased the risk of LRI; odds ratios (OR were 1.22 (95% CI 1.10 to 1.35 for paternal smoking, 1.62 (95% CI 1.38 to 1.89 if both parents smoked, and 1.54 (95% CI 1.40 to 1.69 for any household member smoking. Pre-natal maternal smoking (OR 1.24, 95% CI 1.11 to 1.38 had a weaker effect than post-natal smoking (OR 1.58, 95% CI 1.45 to 1.73. The strongest effect was on bronchiolitis, where the risk of any household smoking was increased by an OR of 2.51 (95% CI 1.96 to 3.21. Conclusions Passive smoking in the family home is a major influence on the risk of LRI in infants, and especially on bronchiolitis. Risk is particularly strong in relation to post-natal maternal smoking. Strategies to prevent passive smoke exposure in young children are an urgent public and child health priority.

Parental and household smoking and the increased risk of bronchitis, bronchiolitis and other lower respiratory infections in infancy: systematic review and meta-analysis.

Science.gov (United States)

Jones, Laura L; Hashim, Ahmed; McKeever, Tricia; Cook, Derek G; Britton, John; Leonardi-Bee, Jo

2011-01-10

Passive smoke exposure increases the risk of lower respiratory infection (LRI) in infants, but the extensive literature on this association has not been systematically reviewed for nearly ten years. The aim of this paper is to provide an updated systematic review and meta-analysis of studies of the association between passive smoking and LRI, and with diagnostic subcategories including bronchiolitis, in infants aged two years and under. We searched MEDLINE and EMBASE (to November 2010), reference lists from publications and abstracts from major conference proceedings to identify all relevant publications. Random effect pooled odds ratios (OR) with 95% confidence intervals (CI) were estimated. We identified 60 studies suitable for inclusion in the meta-analysis. Smoking by either parent or other household members significantly increased the risk of LRI; odds ratios (OR) were 1.22 (95% CI 1.10 to 1.35) for paternal smoking, 1.62 (95% CI 1.38 to 1.89) if both parents smoked, and 1.54 (95% CI 1.40 to 1.69) for any household member smoking. Pre-natal maternal smoking (OR 1.24, 95% CI 1.11 to 1.38) had a weaker effect than post-natal smoking (OR 1.58, 95% CI 1.45 to 1.73). The strongest effect was on bronchiolitis, where the risk of any household smoking was increased by an OR of 2.51 (95% CI 1.96 to 3.21). Passive smoking in the family home is a major influence on the risk of LRI in infants, and especially on bronchiolitis. Risk is particularly strong in relation to post-natal maternal smoking. Strategies to prevent passive smoke exposure in young children are an urgent public and child health priority.

Activation of endothelial and epithelial K(Ca) 2.3 calcium-activated potassium channels by NS309 relaxes human small pulmonary arteries and bronchioles

DEFF Research Database (Denmark)

Kroigaard, Christel; Dalsgaard, Thomas; Nielsen, Gorm

2012-01-01

BACKGROUND AND PURPOSE: Small (K(Ca) 2) and intermediate (K(Ca) 3.1) conductance calcium-activated potassium channels (K(Ca) ) may contribute to both epithelium- and endothelium-dependent relaxations, but this has not been established in human pulmonary arteries and bronchioles. Therefore, we inv...... targets for treatment of pulmonary hypertension and chronic obstructive pulmonary disease....

Not all infantile respiratory distress in winter is bronchiolitis: congenital lobar emphysema.

Science.gov (United States)

Taqvi, Laura; Griksaitis, Michael; Eastham, Katherine

2011-10-20

The authors report the case of a 4-week-old male infant presented during the winter period with respiratory distress. He had a 3 day history of cough and coryza, and a 2 day history of breathlessness and reduced feeding. He had evidence of tachypnoea, subcostal recession and hypoxia on examination. An initial diagnosis of bronchiolitis was made. The authors explore how the correct diagnosis of congenital lobar emphysema (CLE) was reached, highlighting key clinical signs and investigations. He had evidence of a hyperinflated right middle lobe, with collapse of right upper and lower lobes and left upper lobe with associated mediastinal shift on chest x-ray (CXR) and CT scan. He was referred to the regional Paediatric Cardiothoracic Centre where right middle lobectomy was performed with complete resolution of his respiratory distress and re-expansion of the compressed lobes on CXR. Current literature concerning CLE is reviewed.

Heliox delivered by high flow nasal cannula improves oxygenation in infants with respiratory syncytial virus acute bronchiolitis

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Wael Seliem

Full Text Available Abstract Objective: The objective of this study is to evaluate the hypothesis that use of heliox would result in improvement of gas exchange when used with high flow nasal cannula in infants with RSV acute bronchiolitis. Methods: All patients that met the inclusion criteria were randomized to either heliox (70:30 or air-oxygen mixture 30% via high flow nasal cannula at 8 L/min for a continuous 24 h. Measurements were taken at baseline, after 2 h, and at the end of the 24 h. Results: This prospective study included 48 patients. After 2 h of treatment with heliox, the oxygen saturation and PaO2 significantly improved when compared with the air-oxygen group, 98.3% vs. 92.9%, 62.0 mmHg vs. 43.6 mmHg (p = 0.04 and 0.01, respectively. Furthermore, PaO2/FiO2 ratio was significantly higher in the heliox group when compared with the air-oxygen group, 206.7 vs. 145.3. Nevertheless, CO2 showed better elimination when heliox was used, without significance. MWCA score dropped significantly in the heliox group, 2.2 points vs. 4.0 points in air-oxygen (p = 0.04, 2 h after starting the therapy. Conclusion: Transient improvement of oxygenation in infants with RSV acute bronchiolitis during the initial phase of the therapy is associated with heliox when provided with HFNC, may provide a precious time for other therapeutic agents to work or for the disease to resolve naturally, avoiding other aggressive interventions.

Clinical study of the relationship between arteriosclerosis obliterans (ASO) and cerebral infarction

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Iwamoto, Toshihiko; Sasaki, Akinori; Yanagawa, Kiyotaka; Mitsugi, Yasushi

1991-01-01

To clarify the relationship between arteriosclerosis obliterans (ASO) and cerebral infarction (CI), brain CT was performed and the risk factors for atherosclerosis were assessed. Thirty-five male and 5 female patients with intermittent claudication and/or leg ulceration were angiographically diagnosed as having ASO. According to CT findings, these patients were divided into three groups [no low-density areas (NLDA), hemorrhage, and infarction (CI)]. CI was subdivided as lacunar, cortical, and watershed infarction. Thirteen patients were in the NLDA group and 26 in the CI group (17 lacunar, 3 cortical and 6 mixed infarcts), indicating a CI incidence of 65%. Comparing the risk factors of the CI group with those of the NLDA group, hypertension (53.8%), diabetes (34.6%), and cigarette smoking (69.2%) was often seen in the CI group, although hypercholesterolemia (53.8%) and ischemic heart disease (42.3%) was the same in both groups. Multivariate analysis revealed that smoking had the strongest effect on the occurrence of CI in ASO patients. Furthermore, the number of combined risk factors (hypertension, diabetes, smoking, hypercholesterolemia) had a significant positive correlation with cortical infarction. As to the chronological relationship between the onset of ASO and CI, CI was present in 14 of 27 ASO patients on CT when the ischemic leg symptoms appeared, while symptomatic cortical infarction preceded ASO in 5 patients. CI patients increased gradually over a decade to 26 out of 40, among whom 16 patients with lacunae had silent infarcts. These findings suggested that ASO is frequently associated with CI, not only due to atherosclerosis of the main trunks of the cerebral vessels, but also due to arteriolosclerosis of the perforating arteries. (author)

Study on Effectiveness of Low Dose Theophylline as Add-on to Inhaled Corticosteroid for Patients with Sulfur Mustard Induced Bronchiolitis

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Yunes Panahi

2013-12-01

Full Text Available Background: Theophylline may reverse steroid resistance and decrease inflammation in patients with chronic pulmonary disease and sulfur mustard (SM induced bronchiolitis. This study was designed to assess the effects of low-dose theophylline on improvement of pulmonary function tests (PFTs of SM exposed patients.Methods: In this comparative observational study, a group of SM-exposed victims during the Iraq-Iran war who were treated with oral slow releasing (SR theophylline, salmetrol, fluxitide, omeprazole and NAC (study group were compared to a group of age and gender matched SM-exposed patients who received same medications except oral SR theophylline (historical control group. PFTs were measured at the beginning of the study and after 8 weeks of the treatment.Results: In total, 33 subjects in the study group and 27 subjects in the control group were studied. Mean (SD age of all subjects was 51 (14.1 years. In the study group, on the 8th week post-treatment, PFTs decreased, though the differences of tests between before and after treatment were not significant. In the control group, all the tests decreased in the same period and these reductions were not also significant. However, the changes in PFTs were significantly different between the two groups. The results of most PFTs in the controls decreased in greater extents compared to theophylline treated patients. This shows that despite theophylline was unable to improve the patients; it was partially able to decelerate the reductions in PFTs.Conclusion: Theophylline may not improve PFTs of SM exposed patients but it may decelerate the progress of the underlying respiratory disease. Further studies in this setting with higher doses of theophylline and longer term of evaluation are needed to better understand the pathophysiological mechanism of SM induced bronchiolitis and the effectiveness of the treatment with theophylline.   How to cite this article: Panahi Y, Poursaleh Z, Amini-Harandi A

Analysis on correlation between overall classification on color doppler ultrasound and clinical stages of atherosclerosis obliterans

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Zhang Dongmei; Liu Meihan; Shi Weidong; Chen Enqi; Li Xinying; Lin Yu

2010-01-01

Objective: To investigate the correlation and the clinical significance between the overall classification on color Doppler ultrasound and the clinical stages of atherosclerosis obliterans (ASO), and evaluate the extent of arterial lesions comprehensively. Methods: 125 patients of ASO, who were divided into three groups of mild, moderate and severe with Color Doppler ultrasound according to differences of occlusion, quantity, degree of stenosis and collateral number, were analyzed with clinical stages, then their associations were studied with Spearman rank analysis. Results: The clinical manifestations of ASO patients who were divided into three groups of mild, moderate and severe according to overall classification on color Doppler ultrasound were respectively gradually serious, which had positive correlations with the stages of I, II and III according to clinical stages. Spearman rank analysis showed that the correlation coefficients (rs)was 0.797 2 between two groups (P<0.01), there was good consistency between the overall classification on color Doppler ultrasound and the clinical stagesof ASO. Conclusion: The overall classification of ASO on color Doppler ultrasound has considered impact of many other factors on the clinical symptoms,such as the level of the local narrow, narrow scope, segments of occlusion and collateral arteries, which divides the lesions more objectively, shows good consistency with the clinical stages. (authors)

Acute respiratory bronchiolitis: an ultrastructural and autoradiographic study of epithelial cell injury and renewal in Rhesus monkeys exposed to ozone

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Castleman, W.L.; Dungworth, D.L.; Schwartz, L.W.; Tyler, W.S.

1980-01-01

The pathogenesis of acute respiratory bronchiolitis was examined in Rhesus monkeys exposed to 0.8 ppM ozone for 4 to 50 hours. Epithelial injury and renewal were qualitatively and quantitatively characterized by correlated techniques of scanning and transmission electron microscopy as well as by light-microscopic autoradiography following labeling with tritiated thymidine. Extensive degeneration and necrosis of Type 1 epithelial cells occurred on the respiratory bronchiolar wall during the initial 4 to 12 hours of exposure. Increased numbers of labeled epithelial cells were present in this region after 18 hours of exposure, and the highest labeling index (18%) was measured after 50 hours of exposure. Most (67 to 80%) of the labeled cells and all the mitotic epithelial cells (22) observed ultrastructurally were cuboidal bronchiolar epithelial cells. Of the labeled epithelial cells, 20 to 33% were Type 2 epithelial cells. After 50 hours of exposure the respiratory bronchiolar epithelium was hyperplastic. The predominant inflammatory cell in respiratory bronchiolar exudate was the alveolar macrophage. Monkeys that were exposed for 50 hours and allowed to recover in unozonized air for 7 days had incomplete resolution of respiratory bronchiolar epithelial hyperplasia. The results indicate that Type 1 epithelial cells lining respiratory bronchioles are the cell types most sensitive to injury and that both cuboidal bronchiolar epithelial cells and Type 2 epithelial cells function as stem cells in epithelial renewal

[Gastro-esophageal reflux and chronic respiratory diseases].

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Dirou, S; Germaud, P; Bruley des Varannes, S; Magnan, A; Blanc, F-X

2015-12-01

Gastroesophageal reflux disease (GERD) frequently occurs in association with chronic respiratory diseases although the casual link is not always clear. Several pathophysiological and experimental factors are considered to support a role for GERD in respiratory disease. Conversely, respiratory diseases and bronchodilator treatment can themselves exacerbate GERD. When cough or severe asthma is being investigated, GERD does not need to be systematically looked for and a therapeutic test with proton pump inhibitors is not always recommended. pH impedance monitoring is now the reference diagnostic tool to detect non acid reflux, a form of reflux for which proton pump inhibitor treatment is ineffective. Recent data have shown a potential role of GERD in idiopathic pulmonary fibrosis and bronchiolitis obliterans following lung transplantation, leading to discussions about the place of surgery in this context. However, studies using pH impedance monitoring are still needed to better understand and manage the association between GERD and chronic respiratory diseases. Copyright © 2015 SPLF. Published by Elsevier Masson SAS. All rights reserved.

Impact of Donor Arterial Partial Pressure of Oxygen on Outcomes After Lung Transplantation in Adult Cystic Fibrosis Recipients.

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Hayes, Don; Kopp, Benjamin T; Kirkby, Stephen E; Reynolds, Susan D; Mansour, Heidi M; Tobias, Joseph D; Tumin, Dmitry

2016-08-01

Donor PaO2 levels are used for assessing organs for lung transplantation (LTx), but survival implications of PaO2 levels in adult cystic fibrosis (CF) patients receiving LTx are unclear. UNOS registry data spanning 2005-2013 were used to test for associations of donor PaO2 with patient survival and bronchiolitis obliterans syndrome (BOS) in adult (age ≥ 18 years) first-time LTx recipients diagnosed with CF. The analysis included 1587 patients, of whom 1420 had complete data for multivariable Cox models. No statistically significant differences among donor PaO2 categories of ≤200, 201-300, 301-400, or >400 mmHg were found in univariate survival analysis (log-rank test p = 0.290). BOS onset did not significantly differ across donor PaO2 categories (Chi-square p = 0.480). Multivariable Cox models of patient survival supported the lack of difference across donor PaO2 categories. Interaction analysis found a modest difference in survival between the two top categories of donor PaO2 when examining patients with body mass index (BMI) in the lowest decile (≤16.5 kg/m(2)). Donor PaO2 was not associated with survival or BOS onset in adult CF patients undergoing LTx. Notwithstanding statistically significant interactions between donor PaO2 and BMI, there was no evidence of post-LTx survival risk associated with donor PaO2 below conventional thresholds in any subgroup of adults with CF.

[Short-term outcomes of lung transplant recipients using organs from brain death donors].

Science.gov (United States)

He, W X; Jiang, C; Liu, X G; Huang, W; Chen, C; Jiang, L; Yang, B; Wu, K; Chen, Q K; Yang, Y; Yu, Y M; Jiang, G N

2016-12-01

Objective: To assess short-term outcomes after lung transplantation with organs procured following brain death. Methods: Between April 2015 and July 2016, all 17 recipients after lung transplantation using organs from brain death donors (DBD) at Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University School of Medicine were enrolled in this study. All patients were male, aging (60±7) years, including 11 chronic obstructive pulmonary disease, 5 idiopathic pulmonary fibrosis, 1 silicosis. Seventeen donors were 16 males and 1 female, with 10 traumatic brain injury, 5 cerebrovascular accident and 2 sudden cardiac death. Of 17 recipients receiving DBD lung transplant, 16 were single lung transplant. Data were collected including intubation duration of mechanical ventilation, hospital length of stay, incidence of pulmonary infection bronchus anastomosis complications, primary graft dysfunction (PGD), and acute rejection, bronchiolitis obliterans syndrome (BOS) as well as mortality of 90-day after lung transplantation. Results: Median duration of intubation were 2 (2) days ( M ( Q R )) in recipients after lung transplantation. The incidence of pulmonary infection and bronchus anastomosis complications were 15/17 and 5/17, respectively. Median length of stay in hospital were 56 (19) days. The ratio of readmission 1 month after discharge were 10/17. Mortality of 90-day post-transplant were 2/17. The incidence of PGD and BOS were 1/17 and 2/17, respectively. Conclusion: Recipients with DBD lung transplantation have an acceptable survival during short-term follow-up, but with higher incidences of complications related to infection post-transplantation.

Comparison of CPAP and HFNC in Management of Bronchiolitis in Infants and Young Children

DEFF Research Database (Denmark)

Pedersen, Majken Bisgaard; Vahlkvist, Signe

2017-01-01

, fraction of inspired oxygen (FiO2) and heart rate, treatment failure, duration of treatment, and length of hospital stay. A sample size of 49 children were included. Median age was 1.9 months. Median baseline pCO₂ was 7.4 kPa in both groups, respiratory rate per minute was 57 vs. 58 (CPAP vs. HFNC......Continuous positive airway pressure (CPAP) has been used in infants with bronchiolitis for decades. Recently, high flow nasal cannula (HFNC) therapy was introduced We conducted a retrospective study of treatment with CPAP vs. HFNC between 2013 and 2015, comparing the development in respiratory rate...... due to disease progression. There was no difference in length of treatment, hospital stay, or transmission to intensive care unit between the groups. CPAP was more effective than HFNC in decreasing respiratory rate (RR) and FiO2. No differences were observed in length of treatment or complications...

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

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Jung, Jung Im; Park, Seog Hee; Lee, Jae Mun; Song, Jeong Sup; Lee, Kyo Young

1999-01-01

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

Analysis of radiation pneumonitis outside the radiation field in breast conserving therapy for early breast cancer

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Ogo, Etsuyo; Fujimoto, Kiminori; Hayabuchi, Naofumi

2002-01-01

In a retrospective study of radiation-induced pulmonary changes for patients with breast conserving therapy for early breast cancer, we sent questionnaires to the main hospitals in Japan. In this study, we analyzed pulmonary changes after tangential whole-breast irradiation. The purpose of this study was to determine the incidence and risk factors for radiation pneumonitis outside the radiation field. The questionnaires included patients data, therapy data, and lung injury information between August 1999 and May 2000. On the first questionnaires, answer letters were received from 107 institutions out of 158 (67.7%). On the second questionnaires, response rate (hospitals which had radiation pneumonitis outside the radiation field) was 21.7% (23/106). We could find no risk factors of this type of pneumonitis. We suggested that lung irradiation might trigger this type of pneumonitis which is clinically similar to BOOP (bronchiolitis obliterans organizing pneumonia). It developed in 1.5-2.1% among the patients with breast conserving surgery and tangential whole-breast irradiation. And it is likely appeared within 6 months after radiotherapy. (author)

Sirolimus-associated interstitial pneumonitis in a liver transplant recipient

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Claire Berrouet, Marie; Aristizabal, Julian Miguel; Restrepo, Juan Carlos; Correa, Gonzalo

2005-01-01

Sirolimus is an immunosuppressive drug that has been used during the past few years. Sirolimus is indicated in rescue therapies and to reduce the secondary toxic effects of calcineurin inhibitors. This drug has been associated with infrequent but severe pulmonary toxicity. Cases of interstitial pneumonitis, bronchiolitis obliterans with organizing pneumonia, and alveolar proteinosis have been described. We describe a case of pulmonary toxicity associated with the use of sirolimus in a 59-yr-old liver transplant recipient. We also review all reported cases of sirolimus-associated lung toxicity among liver transplantation recipients, with the intention of understanding the risk factors, the clinical picture and the outcomes of this complication. Five cases have been reported since January 2000, including the present case. Clinical presentation is similar, with fever, dyspnoea, fatigue, cough, and hemoptysis. Discontinuation of the drug led to resolution of clinical and radiographic findings. Sirolimus-induced pulmonary toxicity is a serious condition and should be considered in the differential diagnosis of liver recipients presenting with respiratory findings. Discontinuation of the drug is associated with resolution of the pulmonary compromise

Evolução e característica de lactantes com bronquiolite viral aguda submetidos à ventilação mecânica em uma unidade de terapia intensiva pediátrica brasileira Outcome and characteristics of infants with acute viral bronchiolitis submitted to mechanical ventilation in a Brazilian pediatric intensive care

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Fernanda Umpierre Bueno

2009-06-01

bronquiolite viral aguda.OBJECTIVE: To describe the characteristics and the outcome of infants with acute viral bronchiolitis submitted to mechanical ventilation. METHODS: We performed a retrospective study enrolling all infants (less than 12 months old admitted with the diagnosis of acute viral bronchiolitis and submitted to mechanical ventilation in an university affiliated Brazilian pediatric intensive care unit between March, 2004 and September, 2006 (3 consecutives winters. The mechanical ventilation parameters' employed on 1st, 2nd, 3rd, 7th day and before extubation were evaluated as well as the evolution (mortality rate, presence of acute respiratory distress syndrome and the prevalence of complications. The groups were compared using the Student t test, the Mann-Whitney U test and the Chi-square test. RESULTS: Fifty-nine infants were included (3.8 ± 2.7 months old, 59% male, with 9.0 ± 9.4 days on mechanical ventilation. Prior mechanical ventilation, non invasive ventilation was instituted in 71% of children. Anemia was observed in 78% of the sample. In 51 infants (86.5% the lower airway obstructive pattern was maintained up to tracheal extubation with a nil mortality and low prevalence of pneumothorax (7.8%. Acute respiratory distress syndrome occurred in 8 infants (13.5%, with higher mortality and a higher prevalence of pneumothorax (62.5%. CONCLUSIONS: The declining mortality in acute viral bronchiolitis is observed even in non developed regions, involving children with high rates of anemia and premature labor. The low mortality is associated with the maintenance of the lower airway obstructive pattern during the period on mechanical ventilation. The development of acute respiratory distress syndrome is associated with increased mortality and higher prevalence of complications, representing the actual challenge in the management of children with severe acute viral bronchiolitis.

Clinical application of lower extremity CTA and lower extremity perfusion CT as a method of diagnostic for lower extremity atherosclerotic obliterans

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Moon, Il Bong; Dong, Kyung Rae [Dept. Radiological Technology, Gwangju Health University, Gwangju (Korea, Republic of); Goo, Eun Hoe [Dept. Radiological Science, Cheongju University, Cheongju (Korea, Republic of)

2016-11-15

The purpose of this study was to assess clinical application of lower extremity CTA and lower extremity perfusion CT as a method of diagnostic for lower extremity atherosclerotic obliterans. From January to July 2016, 30 patients (mean age, 68) were studied with lower extremity CTA and lower extremity perfusion CT. 128 channel multi-detector row CT scans were acquired with a CT scanner (SOMATOM Definition Flash, Siemens medical solution, Germany) of lower extremity perfusion CT and lower extremity CTA. Acquired images were reconstructed with 3D workstation (Leonardo, Siemens, Germany). Site of lower extremity arterial occlusive and stenosis lesions were detected superficial femoral artery 36.6%, popliteal artery 23.4%, external iliac artery 16.7%, common femoral artery 13.3%, peroneal artery 10%. The mean total DLP comparison of lower extremity perfusion CT and lower extremity CTA, 650 mGy-cm and 675 mGy-cm, respectively. Lower extremity perfusion CT and lower extremity CTA were realized that were never be two examination that were exactly the same legions. Future through the development of lower extremity perfusion CT soft ware programs suggest possible clinical applications.

Flock worker's lung: chronic interstitial lung disease in the nylon flocking industry.

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Kern, D G; Crausman, R S; Durand, K T; Nayer, A; Kuhn, C

1998-08-15

Two young men working at a nylon flocking plant in Rhode Island developed interstitial lung disease of unknown cause. Similar clusters at the same company's Canadian plant were reported previously. To define the extent, clinicopathologic features, and potential causes of the apparent disease outbreak. Case-finding survey and retrospective cohort study. Academic occupational medicine program. All workers employed at the Rhode Island plant on or after 15 June 1990. Symptomatic employees had chest radiography, pulmonary function tests, high-resolution computed tomography, and serologic testing. Those with unexplained radiographic or pulmonary function abnormalities underwent bronchoalveolar lavage, lung biopsy, or both. The case definition of "flock worker's lung" required histologic evidence of interstitial lung disease (or lavage evidence of lung inflammation) not explained by another condition. Eight cases of flock worker's lung were identified at the Rhode Island plant. Three cases were characterized by a high proportion of eosinophils (25% to 40%) in lavage fluid. Six of the seven patients who had biopsy had histologic findings of nonspecific interstitial pneumonia, and the seventh had bronchiolitis obliterans organizing pneumonia. All seven of these patients had peribronchovascular interstitial lymphoid nodules, usually with germinal centers, and most had lymphocytic bronchiolitis and interstitial fibrosis. All improved after leaving work. Review of the Canadian tissue specimens showed many similar histologic findings. Among the 165-member study cohort, a 48-fold or greater increase was seen in the sex-adjusted incidence rate of all interstitial lung disease. Work in the nylon flocking industry poses substantial risk for a previously unrecognized occupational interstitial lung disease. Nylon fiber is the suspected cause of this condition.

Local C-Reactive Protein Expression in Obliterative Lesions and the Bronchial Wall in Posttransplant Obliterative Bronchiolitis

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Outi E. Päiväniemi

2009-01-01

Full Text Available The local immunoreactivity of C-reactive protein (CRP was studied in a heterotopic porcine model of posttranplant obliterative bronchiolitis (OB. Bronchial allografts and control autografts were examined serially 2–28 days after subcutaneous transplantation. The autografts stayed patent. In the allografts, proliferation of inflammatory cells (P<.0001 and fibroblasts (P=.02 resulted in occlusion of the bronchial lumens (P<.01. Influx of CD4+ (P<.001 and CD8+ (P<.0001 cells demonstrated allograft immune response. CRP positivity simultaneously increased in the bronchial walls (P<.01, in macrophages, myofibroblasts, and endothelial cells. Local CRP was predictive of features characteristic of OB (R=0.456–0.879, P< .05−P<.0001. Early obliterative lesions also showed CRP positivity, but not mature, collagen-rich obliterative plugs (P<.05. During OB development, CRP is localized in inflammatory cells, myofibroblasts and endothelial cells probably as a part of the local inflammatory response.

Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

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Parissis Haralabos

2010-09-01

Full Text Available Abstract Background To report our experience of a rather uncommon drug interaction, resulting in hemolytic uremic syndrome (HUS. Methods Two consecutive cases of hemolytic uremic syndrome were diagnosed in our service. In both patients the use of macrolides in patients taking Tacrolimus, resulted in high levels of Tacrolimus. Results The first patient was a 48 years old female with Bilateral emphysema. She underwent Single Sequential Lung Transplantation. She developed reperfusion injury requiring prolonged stay. Tacrolimus introduced (Day 51. The patient remained well up till 5 months later; Erythromycin commenced for chest infection. High Tacrolimus levels and a clinical diagnosis of HUS were made. She was treated with plasmapheresis successfully. The second case was a 57 years old female with Emphysema & A1 Antithrypsin deficiency. She underwent Right Single Lung Transplantation. A2 rejection with mild Obliterative Bronchiolitis diagnosed 1 year later and she switched to Tacrolimus. She was admitted to her local Hospital two and a half years later with right middle lobe consolidation. The patient commenced on amoxicillin and clarithromycin. Worsening renal indices, high Tacrolimus levels, hemolytic anemia & low Platelets were detected. HUS diagnosed & treated with plasmapheresis. Conclusions There are 21 cases of HUS following lung transplantation in the literature that may have been induced by high tacrolimus levels. Macrolides in patients taking Cyclosporin or Tacrolimus lead to high levels. Mechanism of action could be glomeruloconstrictor effect with reduced GFR increased production of Endothelin-1 and increased Platelet aggregation.

Long-term results after lung transplantation using organs from circulatory death donors: a propensity score-matched analysis†.

Science.gov (United States)

Sabashnikov, Anton; Patil, Nikhil P; Popov, Aron-Frederik; Soresi, Simona; Zych, Bartlomiej; Weymann, Alexander; Mohite, Prashant N; García Sáez, Diana; Zeriouh, Mohamed; Wahlers, Thorsten; Choi, Yeong-Hoon; Wippermann, Jens; Wittwer, Thorsten; De Robertis, Fabio; Bahrami, Toufan; Amrani, Mohamed; Simon, André R

2016-01-01

Due to organ shortage in lung transplantation (LTx), donation after circulatory death (DCD) has been implemented in several countries, contributing to an increasing number of organs transplanted. We sought to assess long-term outcomes after LTx with organs procured following circulatory death in comparison with those obtained from donors after brain death (DBD). Between January 2007 and November 2013, 302 LTxs were performed in our institution, whereby 60 (19.9%) organs were retrieved from DCD donors. We performed propensity score matching (DCD:DBD = 1:2) based on preoperative donor and recipient factors that were significantly different in univariate analysis. After propensity matching, there were no statistically significant differences between the groups in terms of demographics and preoperative donor and recipient characteristics. There were no significant differences regarding intraoperative variables and total ischaemic time. Patients from the DCD group had significantly higher incidence of primary graft dysfunction grade 3 at the end of the procedure (P = 0.014), and significantly lower pO2/FiO2 ratio during the first 24 h after the procedure (P = 0.018). There was a trend towards higher incidence of the need for postoperative extracorporeal life support in the DCD group. Other postoperative characteristics were comparable. While the overall cumulative survival was not significantly different, the DCD group had significantly poorer results in terms of bronchiolitis obliterans syndrome (BOS)-free survival in the long-term follow-up. Long-term results after LTx with organs procured following DCD are in general comparable with those obtained after DBD LTx. However, patients transplanted using organs from DCD donors have a predisposition for development of BOS in the longer follow-up. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Computed tomography findings of postoperative complications in lung transplantation Achados tomográficos nas complicações pós-operatórias do transplante pulmonar

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Bruno Hochhegger

2009-03-01

Full Text Available Due to the increasing number and improved survival of lung transplant recipients, radiologists should be aware of the imaging features of the postoperative complications that can occur in such patients. The early treatment of complications is important for the long-term survival of lung transplant recipients. Frequently, HRCT plays a central role in the investigation of such complications. Early recognition of the signs of complications allows treatment to be initiated earlier, which improves survival. The aim of this pictorial review was to demonstrate the CT scan appearance of pulmonary complications such as reperfusion edema, acute rejection, infection, pulmonary thromboembolism, chronic rejection, bronchiolitis obliterans syndrome, cryptogenic organizing pneumonia, post-transplant lymphoproliferative disorder, bronchial dehiscence and bronchial stenosis.Com o número cada vez maior e uma melhor sobrevida dos pacientes submetidos ao transplante pulmonar, os radiologistas devem estar cientes das diversas possibilidades de complicações associadas ao transplante de pulmão. O tratamento precoce das complicações é importante para a sobrevida a longo prazo dos receptores de transplante pulmonar. Com frequência, a TCAR desempenha um papel central na investigação de tais complicações. O reconhecimento precoce dos sinais de complicações proporciona um tratamento rápido e melhora a sobrevida. O objetivo desta revisão pictórica foi proporcionar uma visão sobre as complicações mais prevalentes na TC, tais como edema de reperfusão, rejeição aguda, infecção, tromboembolismo pulmonar, rejeição crônica, síndrome da bronquiolite obliterante, pneumonia em organização criptogênica, doença linfoproliferativa pós-transplante, deiscência brônquica e estenose brônquica.

The diagnostic value of gastroesophageal reflux disease (GERD) symptoms and detection of pepsin and bile acids in bronchoalveolar lavage fluid and exhaled breath condensate for identifying lung transplantation patients with GERD-induced aspiration.

Science.gov (United States)

Reder, Nicholas P; Davis, Christopher S; Kovacs, Elizabeth J; Fisichella, P Marco

2014-06-01

Gastroesophageal reflux disease (GERD) is thought to lead to aspiration and bronchiolitis obliterans syndrome after lung transplantation. Unfortunately, the identification of patients with GERD who aspirate still lacks clear diagnostic indicators. The authors hypothesized that symptoms of GERD and detection of pepsin and bile acids in the bronchoalveolar lavage fluid (BAL) and exhaled breath condensate (EBC) are effective for identifying lung transplantation patients with GERD-induced aspiration. From November 2009 to November 2010, 85 lung transplantation patients undergoing surveillance bronchoscopy were prospectively enrolled. For these patients, self-reported symptoms of GERD were correlated with levels of pepsin and bile acids in BAL and EBC and with GERD status assessed by 24-h pH monitoring. The sensitivity and specificity of pepsin and bile acids in BAL and EBC also were compared with the presence of GERD in 24-h pH monitoring. The typical symptoms of GERD (heartburn and regurgitation) had modest sensitivity and specificity for detecting GERD and aspiration. The atypical symptoms of GERD (aspiration and bronchitis) showed better identification of aspiration as measured by detection of pepsin and bile acids in BAL. The sensitivity and specificity of pepsin in BAL compared with GERD by 24-h pH monitoring were respectively 60 and 45 %, whereas the sensitivity and specificity of bile acids in BAL were 67 and 80 %. These data indicate that the measurement of pepsin and bile acids in BAL can provide additional data for identifying lung transplantation patients at risk for GERD-induced aspiration compared with symptoms or 24-h pH monitoring alone. These results support a diagnostic role for detecting markers of aspiration in BAL, but this must be validated in larger studies.

Nocturnal weakly acidic reflux promotes aspiration of bile acids in lung transplant recipients.

Science.gov (United States)

Blondeau, Kathleen; Mertens, Veerle; Vanaudenaerde, Bart A; Verleden, Geert M; Van Raemdonck, Dirk E; Sifrim, Daniel; Dupont, Lieven J

2009-02-01

Gastroesophageal reflux (GER) and aspiration of bile acids have been implicated as non-alloimmune risk factors for the development of bronchiolitis obliterans syndrome (BOS) after lung transplantation. The aim of our study was to investigate the association between GER and gastric aspiration of bile acids and to establish which reflux characteristics may promote aspiration of bile acids into the lungs and may feature as a potential diagnostic tool in identifying lung transplantation (LTx) patients at risk for aspiration. Twenty-four stable LTx recipients were studied 1 year after transplantation. All patients underwent 24-hour ambulatory impedance-pH recording for the detection of acid (pH acidic (pH 4 to 7) reflux. On the same day, bronchoalveolar lavage fluid (BALF) was collected and then analyzed for the presence of bile acids (Bioquant enzymatic assay). Increased GER was detected in 13 patients, of whom 9 had increased acid reflux and 4 had exclusively increased weakly acidic reflux. Sixteen patients had detectable bile acids in the BALF (0.6 [0.4 to 1.5] micromol/liter). The 24-hour esophageal volume exposure was significantly increased in patients with bile acids compared to patients without bile acids in the BALF. Acid exposure and the number of reflux events (total, acid and weakly acidic) were unrelated to the presence of bile acids in the BALF. However, both nocturnal volume exposure and the number of nocturnal weakly acidic reflux events were significantly higher in patients with bile acids in the BALF. Weakly acidic reflux events, especially during the night, are associated with the aspiration of bile acids in LTx recipients and may therefore feature as a potential risk factor for the development of BOS.

Usefulness of preoperative coronary angiography and brain computed tomography in cases of coronary artery disease and cerebrovascular disease undergoing revascularization for arteriosclerosis obliterans

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Sakurada, Tall; Shibata, Yoshiki

2003-01-01

Coronary angiography and brain computed tomography were preoperatively performed to evaluate the clinical condition of coronary artery disease and cerebrovascular disease in 101 patients (mean age, 68.4 years) with revascularization for arteriosclerosis obliterans. Eighty patients had hypertension, 12 had diabetes, and 26 had hyperlipidemia. Seventy-one patients (70.3%) had coronary stenosis. Significant stenoses in major coronary artery branches were confirmed in 35 patients, including 13 patients with old myocardial infarction. Coronary artery bypass grafting and percutaneous coronary angioplasty were performed in 2 and 7 patients with critical stenosis, respectively. Of 57 patients, who underwent brain computed tomography, abnormalities were found in 52 patients (91.2%), including cortical infarction in 9, lacunar infarction in 35, and leukoaraiosis in 27 patients. During the follow-up period 13 patients died (including 3 cases of myocardial infarction and 3 cases of stroke). Actuarial survival rate at 5 years was 80.4%. The influence of ischemic heart disease and cerebrovascular disease on early and late mortality after surgical reconstruction for peripheral occlusive vascular disease is significant. Using visual diagnostic techniques, such as coronary angiography and brain computed tomography, long term survivor should be closely observed for multiple arteriosclerotic vascular diseases. (author)

Usefulness of preoperative coronary angiography and brain computed tomography in cases of coronary artery disease and cerebrovascular disease undergoing revascularization for arteriosclerosis obliterans

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Sakurada, Tall; Shibata, Yoshiki [Southern Tohoku Fukushima Hospital (Japan)

2003-05-01

Coronary angiography and brain computed tomography were preoperatively performed to evaluate the clinical condition of coronary artery disease and cerebrovascular disease in 101 patients (mean age, 68.4 years) with revascularization for arteriosclerosis obliterans. Eighty patients had hypertension, 12 had diabetes, and 26 had hyperlipidemia. Seventy-one patients (70.3%) had coronary stenosis. Significant stenoses in major coronary artery branches were confirmed in 35 patients, including 13 patients with old myocardial infarction. Coronary artery bypass grafting and percutaneous coronary angioplasty were performed in 2 and 7 patients with critical stenosis, respectively. Of 57 patients, who underwent brain computed tomography, abnormalities were found in 52 patients (91.2%), including cortical infarction in 9, lacunar infarction in 35, and leukoaraiosis in 27 patients. During the follow-up period 13 patients died (including 3 cases of myocardial infarction and 3 cases of stroke). Actuarial survival rate at 5 years was 80.4%. The influence of ischemic heart disease and cerebrovascular disease on early and late mortality after surgical reconstruction for peripheral occlusive vascular disease is significant. Using visual diagnostic techniques, such as coronary angiography and brain computed tomography, long term survivor should be closely observed for multiple arteriosclerotic vascular diseases. (author)

Anti-Inflammatory and Tissue Regenerative Effects of Topical Treatment with Ozonated Olive Oil/Vitamin E Acetate in Balanitis Xerotica Obliterans

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Monica Currò

2018-03-01

Full Text Available Balanitis xerotica obliterans (BXO is a chronic inflammatory skin disorder, considered the male genital variant of lichen sclerosus. Anti-inflammatory drugs are commonly used in BXO. We evaluated the effects of an innovative formulation of ozonated olive oil with vitamin E acetate (OZOILE® on the inflammatory status and tissue remodeling in male children with BXO. The mRNA transcripts of proteins involved either in inflammation or in dynamics of tissue regeneration were analyzed by quantitative real-time PCR, in foreskins affected by BXO removed from patients untreated or treated with OZOILE® cream for 7 days before circumcision. We found a significant reduction in mRNA levels of IL-1β, TNF-α, INF–γ, transglutaminase 2 and NOS2 in foreskins treated with OZOILE® in comparison to untreated ones (p < 0.001. No significant differences were observed in NF-κB activation in the specimens obtained from treated and untreated patients. Hence, OZOILE® treatment up-regulated hypoxia-inducible factor (HIF-1alpha, vascular endothelial growth factor (VEGF and E-cadherin gene expression (p < 0.001. The treatment with OZOILE® showed effective results in children affected by BXO by reducing the inflammatory process and stimulating mechanisms for tissue regeneration of the foreskin. A randomized clinical trial on a large number of children affected by BXO might be useful to verify the efficacy of topical treatment with OZOILE®.

Buccal mucosal urethroplasty for balanitis xerotica obliterans related urethral strictures: the outcome of 1 and 2-stage techniques.

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Dubey, Deepak; Sehgal, Anand; Srivastava, Aneesh; Mandhani, Anil; Kapoor, Rakesh; Kumar, Anant

2005-02-01

Balanitis xerotica obliterans (BXO) related strictures are complex and generally managed by 2-staged urethroplasty. We present our results with 1-stage dorsal onlay and 2-stage buccal mucosal urethroplasty for such strictures. Between January 2000 and April 2004, 39 patients underwent buccal mucosal urethroplasty for BXO related anterior urethral strictures. The 25 patients with a salvageable urethral plate (group 1) were treated with 1-stage dorsal onlay urethroplasty using a cosmetic incision. The 14 patients with a severely scarred urethral plate, focally dense segments or active infection (group 2) underwent 2-stage urethroplasty. Outcomes in terms of cosmetic appearance, stricture recurrence and complications in the 2 groups were assessed. At a mean followup of 32.5 months (range 3 to 52) 3 patients (12%) in group 1 had recurrent stricture, of which 2 and 1 were treated with optical urethrotomy and urethral dilation, respectively. All patients had a normal slit-like meatus and none had chordee or erectile dysfunction. Four group 2 patients (28.6%) required stomal revision and 2 had glans cleft narrowing after stage 1 urethroplasty. Following stage 2, 3 patients had recurrent stricture, of whom 2 were treated with optical urethrotomy and 1 underwent repeat urethroplasty. In BXO related strictures with a viable urethral plate 1-stage dorsal onlay buccal mucosal urethroplasty provides excellent intermediate term results. The cosmetic incision described provides a normal, wide caliber, slit-like glans. Two-stage procedures provide satisfactory outcomes but they are associated with a higher revision rate.

HIV-related chronic lung disease in adolescents: are we prepared for the future?

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Goussard, Pierre; Gie, Robert P

2017-12-01

Chronic lung diseases (CLD) are the most chronic disease occurring in adolescents living with human immunodeficiency virus (ALHIV). In ALHIV who received antiretroviral therapy (ART) late in childhood, bronchiectasis, bronchiolitis obliterans and interstitial pneumonitis are common. In adolescents who received ART early in life the spectrum of CLD has changed with asthma and chronic obstructive pulmonary disease being common. Areas covered: The aim of this paper was to review CLD in ALHIV. We conducted a literature review of electronic databases focusing on CLD that were common prior to the introduction of ART (1996-2004), the present situation where ART is widely available (2005 to 2016), and articles which aided us speculating on the impact of HIV-related CLD in adolescents transitioning to adult HIV-clinics. Amongst the approximately 2.1 million adolescents living with HIV, CLD commonly occurs. Awareness of the CLD amongst ALHIV needs to be raised to ensure that disease appropriate treatment is available to these vulnerable adolescents. Expert commentary: As adolescents' transition from pediatric HIV-clinics to adult HIV-clinics the evidence shows that adolescents might not receive optimal care if adult pulmonologists are not aware of the CLD that commonly occur in ALHIV.

Corruption of dendritic cell antigen presentation during acute GVHD leads to regulatory T-cell failure and chronic GVHD.

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Leveque-El Mouttie, Lucie; Koyama, Motoko; Le Texier, Laetitia; Markey, Kate A; Cheong, Melody; Kuns, Rachel D; Lineburg, Katie E; Teal, Bianca E; Alexander, Kylie A; Clouston, Andrew D; Blazar, Bruce R; Hill, Geoffrey R; MacDonald, Kelli P A

2016-08-11

Chronic graft-versus-host disease (cGVHD) is a major cause of late mortality following allogeneic bone marrow transplantation (BMT) and is characterized by tissue fibrosis manifesting as scleroderma and bronchiolitis obliterans. The development of acute GVHD (aGVHD) is a powerful clinical predictor of subsequent cGVHD, suggesting that aGVHD may invoke the immunologic pathways responsible for cGVHD. In preclinical models in which sclerodermatous cGVHD develops after a preceding period of mild aGVHD, we show that antigen presentation within major histocompatibility complex (MHC) class II of donor dendritic cells (DCs) is markedly impaired early after BMT. This is associated with a failure of regulatory T-cell (Treg) homeostasis and cGVHD. Donor DC-restricted deletion of MHC class II phenocopied this Treg deficiency and cGVHD. Moreover, specific depletion of donor Tregs after BMT also induced cGVHD, whereas adoptive transfer of Tregs ameliorated it. These data demonstrate that the defect in Treg homeostasis seen in cGVHD is a causative lesion and is downstream of defective antigen presentation within MHC class II that is induced by aGVHD. © 2016 by The American Society of Hematology.

Prevention of bronchiolitis from the hospital to home: enviromental and pharmacological strategies

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Andrea Dotta

2015-10-01

Full Text Available Bronchiolitis is one of the major cause of mortality and morbidity during infancy, with high hospitalization rate during epidemic season for high risk infants during the first year of life. There is no specific therapy with proven efficacy apart hydration and hypertonic saline. Mild forms can be treated in outpatient setting avoiding a useless hospitalization. Enviromental prevention is crucial in hospital setting, in outpatient ambulatory and at home: for this purpose hand hygiene, tobacco exposure, breastfeeding must be emphasized with families and caregivers. Pharmacological prophylaxis uses a humanized monoclonal antibody (palivizumab shown effective in reducing hospitalization rate in preterm infants < 35 weeks gestational age, infants with bronchopulmonary dysplasia and infants with congenital heart disease. During the last 2 years different recommandations and guidelines confirmed the importance of prophylaxis with palivizumab with some differences between Groups and Societies based on different healthcare systems. In any case it is important a good contact between hospital and all caregivers of these patients to reduce the viral exposure and increase the defense capability of high risk patients. Proceedings of the 11th International Workshop on Neonatology and Satellite Meetings · Cagliari (Italy · October 26th-31st, 2015 · From the womb to the adultGuest Editors: Vassilios Fanos (Cagliari, Italy, Michele Mussap (Genoa, Italy, Antonio Del Vecchio (Bari, Italy, Bo Sun (Shanghai, China, Dorret I. Boomsma (Amsterdam, the Netherlands, Gavino Faa (Cagliari, Italy, Antonio Giordano (Philadelphia, USA

Ingraft chimerism in lung transplantation - a study in a porcine model of obliterative bronchiolitis

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Rubes Jiri

2011-04-01

Full Text Available Abstract Background Bronchial epithelium is a target of the alloimmune response in lung transplantation, and intact epithelium may protect allografts from rejection and obliterative bronchiolitis (OB. Herein we study the influence of chimerism on bronchial epithelium and OB development in pigs. Methods A total of 54 immunosuppressed and unimmunosuppressed bronchial allografts were serially obtained 2-90 days after transplantation. Histology (H&E was assessed and the fluorescence in situ hybridization (FISH method for Y chromosomes using pig-specific DNA-label was used to detect recipient derived cells in graft epithelium and bronchial wall, and donor cell migration to recipient organs. Ingraft chimerism was studied by using male recipients with female donors, whereas donor cell migration to recipient organs was studied using female recipients with male donors. Results Early appearance of recipient-derived cells in the airway epithelium appeared predictive of epithelial destruction (R = 0.610 - 0.671 and p R = 0.698 and p p p Conclusions In this study we demonstrate that early appearance of Y chromosomes in the airway epithelium predicts features characteristic of OB. Chimerism occurred in all allografts, including those without features of OB. Therefore we suggest that ingraft chimerism may be a mechanism involved in the repair of alloimmune-mediated tissue injury after transplantation.

HRCT of the lung in collagen vascular diseases; HRCT der Lunge bei Kollagenosen

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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

Lung structure-respiratory function relationships in experimentally-induced bronchiolitis, bronchopneumonia and interstitial pneumonia in rats

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Mauderly, J L; Madron, E de; Harkema, J R

1988-12-01

Histopathology and respiratory function of rats with three different types and distributions of lower lung inflammation were compared to better understand lung structure-function relationships. Rats were exposed 21 h/day for 7 days to 0.8 ppm ozone (O{sub 3}), sham-exposed as controls, or given 5 mg/kg bacterial endotoxin either intratracheally (ITE) or intraperitoneally (IPE). Respiratory function was measured 24 h after the end of treatment, than the rats were sacrificed and the distribution of inflammation was evaluated morphometrically. Chronic centriacinar inflammation with formation of new respiratory bronchioles caused an obstructive functional impairment in the O{sub 3} rats, which was clearly distinguished from the restrictive impairments resulting from acute inflammation in ITE and IPE rats. Only the magnitudes of changes related to the distribution of inflammation differentiated the ITE and IPE groups. Flow parameters previously thought sensitive to large airway resistance were changed in the O{sub 3} rats. Alveolar luminal inflammatory exudate affected quasistatic compliance more than septal inflammation in ITE and IPE rats. Quasistatic chord compliance was the most sensitive of three indices of pressure-volume relationships. The findings in this study improve the basis for interpreting respiratory function changes of rats. (author)

Respiratory syncytial virus (RSV bronchiolitis: comparative study of RSV groups A and B infected children

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Selir M. Straliotto

1994-03-01

Full Text Available The grouping characteristics of 29 respiratory syncitial virus (RSV present in nasopharyngeal cells collectedfrom hospitalized children with bronchiolitis during the 1990RSVseason in Porto Alegre, RS, were analysed. Twenty-two were grouped as belonging to group A and 7 to group B. Cyanosis, oxigen therapy, cough, lenght of hospitalization and atelectasis were observed to be more frequently found within group B infected children. Other clinical signs and symptoms were similarly found in both groups.Estudos recentes de amostras do vírus respiratório sincicial (VRS usando anticorpos monoclonais distiguiram duas variantes antigênicas, designadas como grupos A e B. Estes grupos foram estudados em 29 secreções de nasofaringe positivas para o VRS, provenientes de crianças hospitalizadas com bronquiolite durante surto de virose por VRS, em Porto Alegre, em 1990. Destas, 22 foram grupadas como pertencentes ao grupo A e 7 ao grupo B. Alguns achados clínicos como cianose, tosse, uso de oxigênio e dias de hospitalização foram mais freqüentemente observados em crianças infectadas coin o grupo B do VRS. Outros sinais e sintomas clínicos foram similarmente encontrados nos 2 grupos.

Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings

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Kim, Yoon Kyung; Lee, Kyung Soo; Chong, Semin; Chung, Myung Jin; Yi, Chin A; Kim, Ha Young [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Seoul (Korea); Chung, Man Pyo [Samsung Medical Center, Sungkyunkwan University School of Medicine, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Seoul (Korea); Han, Joungho [Samsung Medical Center, Sungkyunkwan University School of Medicine, Department of Pathology, Seoul (Korea)

2007-12-15

We tried to assess retrospectively thin-section CT findings of Churg-Strauss syndrome (CSS) in 25 patients and to compare these findings with clinical and histopathologic findings. Of 25 patients, 19 (76%) had parenchymal abnormalities at CT; small nodules (n = 12; 63%), ground-glass opacity (n = 10; 53%), bronchial wall thickening (n = 10; 53%), and consolidation (n = 8; 42%). Parenchymal abnormalities (n = 19) were categorizable as an airway pattern in 11 and an airspace pattern in eight. Patients with an airway pattern (n = 5) had obstructive (n = 3) or combined (n = 2) PFT results, whereas those with an airspace pattern (n = 4) had restrictive (n = 3) or obstructive (n = 1) results. Parenchymal opacities at CT corresponded histologically to areas of eosinophilic pneumonia, necrotizing granulomas, and granulomatous vasculitis; small nodules to eosinophilic bronchiolitis and peribronchiolar vasculitis; and bronchial wall thickening to airway wall eosinophil and lymphocyte infiltration. Patients with airspace pattern responded more readily to treatment than those with airway pattern. CT shows lung parenchymal abnormalities in about three-quarters of CSS patients and these abnormalities can be categorized as airspace or airway patterns. This classification helps predict PFT data, underlying histopathology, and treatment response. (orig.)

Pulmonary involvement in Churg-Strauss syndrome: an analysis of CT, clinical, and pathologic findings

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Kim, Yoon Kyung; Lee, Kyung Soo; Chong, Semin; Chung, Myung Jin; Yi, Chin A.; Kim, Ha Young; Chung, Man Pyo; Han, Joungho

2007-01-01

We tried to assess retrospectively thin-section CT findings of Churg-Strauss syndrome (CSS) in 25 patients and to compare these findings with clinical and histopathologic findings. Of 25 patients, 19 (76%) had parenchymal abnormalities at CT; small nodules (n = 12; 63%), ground-glass opacity (n = 10; 53%), bronchial wall thickening (n = 10; 53%), and consolidation (n = 8; 42%). Parenchymal abnormalities (n = 19) were categorizable as an airway pattern in 11 and an airspace pattern in eight. Patients with an airway pattern (n = 5) had obstructive (n = 3) or combined (n = 2) PFT results, whereas those with an airspace pattern (n = 4) had restrictive (n = 3) or obstructive (n = 1) results. Parenchymal opacities at CT corresponded histologically to areas of eosinophilic pneumonia, necrotizing granulomas, and granulomatous vasculitis; small nodules to eosinophilic bronchiolitis and peribronchiolar vasculitis; and bronchial wall thickening to airway wall eosinophil and lymphocyte infiltration. Patients with airspace pattern responded more readily to treatment than those with airway pattern. CT shows lung parenchymal abnormalities in about three-quarters of CSS patients and these abnormalities can be categorized as airspace or airway patterns. This classification helps predict PFT data, underlying histopathology, and treatment response. (orig.)

Prospective Multicentre Study on the Epidemiology and Current Therapeutic Management of Severe Bronchiolitis in Spain

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Jose C. Flores-González

2017-01-01

Full Text Available Objective. To determine the epidemiology and therapeutic management of patients with severe acute bronchiolitis (AB admitted to paediatric intensive care units (PICUs in Spain. Design. Descriptive, prospective, multicentre study. Setting. Sixteen Spanish PICUs. Patients. Patients with severe AB who required admission to any of the participating PICUs over 1 year. Interventions. Both epidemiological variables and medical treatment received were recorded. Results. A total of 262 patients were recruited; 143 were male (54.6%, with median age of 1 month (0–23. Median stay in the PICU was 7 days (1–46. Sixty patients (23% received no nebuliser treatment, while the rest received a combination of inhalation therapies. One-quarter of patients (24.8% received corticosteroids and 56.5% antibiotic therapy. High-flow oxygen therapy was used in 14.3% and noninvasive ventilation (NIV was used in 75.6%. Endotracheal intubation was required in 24.4% of patients. Younger age, antibiotic therapy, and invasive mechanical ventilation (IMV were risk factors that significantly increased the stay in the PICU. Conclusions. Spanish PICUs continue to routinely use nebulised bronchodilator treatment and corticosteroid therapy. Despite NIV being widely used in this condition, intubation was required in one-quarter of cases. Younger age, antibiotic therapy, and IMV were associated with a longer stay in the PICU.

028. Migratory pneumonia—cryptogenic organizing pneumonia (COP)

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Lagoudi, Kalliopi; Ioannidou, Despoina; Papadaki, Elena; Organtzis, Ioannis; Kostanta, Soultana; Papaioannou, Antonis; Moumtzi, Despoina; Porpodis, Konstantinos; Fouka, Evaggelia

2015-01-01

In this study were presented the clinical and laboratory findings of eight patients with migratory pneumonia, who were hospitalised in our clinic. It is about eight women with average age of 58±13 years with fever, weakness, dry cough, shortness of breath and who already had received antibiotics. Crackles were the most frequent evidence by the auscultation. All patients showed consolidation in chest radiography which resolved completely from the initial area and migrated in different areas. The chest HRCT showed opacity with air bronchogram and ground glass in places. Regarding to the respiratory function, patients showed mild restriction disorder (average values ± SD: FEV1% 83±24, FVC% 86±21, TLC% 82±16, DLco% 74±15). The average price of pO2 was 68+7 mmHg. The findings of BAL were: macrophages 51%±20%, lymphocytes 33.5%±14%, neutrophils 7.5%±3%, eosinophils 7%±8%. From diagnosis, we excluded eosinophilic pneumonia, infectious causes, collagen diseases and vasculitis. The findings of physical examination, chest radiography and the results of the BAL of all of the patients argued for Bronchiolitis obliterans organizing pneumonia (BOOP), the cause of which was not found (cryptogenic organizing pneumonia-COP). All patients responded directly to corticosteroids.

Lateral decubitus HRCT: a simple technique to replace expiratory CT in children with air trapping

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Choi, Soo-Jung; Choi, Byeong-Kyoo; Kim, Hyeon Joo; Lee, Soo Hyun; Choi, Seong Hoon; Park, Sang Joon; Goo, Hyun Woo; Lee, Jin Seong; Yoon, Chong Hyun

2002-01-01

Objective: To evaluate the effectiveness of lateral decubitus high-resolution CT (HRCT) in detecting air trapping in children. Materials and methods: HRCT scans of 21 children with heterogeneous lung attenuation caused by air trapping (n=10) or with infiltrative lung disease (n=11) were reviewed retrospectively. The air-trapping disease included bronchiolitis obliterans (n=7), bronchial obstruction due to mediastinal lymphoma (n=1), endobronchial haemangioma (n=1) and foreign body aspiration (n=1). HRCT was performed in both lateral decubitus positions as well as the supine position. The attenuation (Hounsfield units; HU) was measured in both the hypo- and adjacent hyper-attenuating areas of the heterogeneous lung portion, and the difference of attenuation between these two areas was calculated in the supine and both lateral decubitus scans, respectively. The attenuation differences of the three scans were compared in each group. Results: The attenuation difference was larger in the ipsilateral decubitus (207.95±105.24 HU) scans than in the contralateral (121.25±90.05 HU) or supine (162±94.01 HU) scans in the air-trapping group (P 0.05). Conclusions: Lateral decubitus HRCT is an effective adjunct to standard HRCT in the evaluation of air trapping as a cause of mosaic lung attenuation in uncooperative paediatric patients. (orig.)

GERD related micro-aspiration in chronic mustard-induced pulmonary disorder

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Rasoul Aliannejad

2012-01-01

Full Text Available Background and Aim: Bronchiolitis obliterans (BO is the main pulmonary involvement resulting from sulfur mustard (SM gas exposure that was used against Iranian civilians and military forces during the Iran-Iraq war. The present study aimed to investigate the prevalence of gastro-esophageal reflux (GER and gastric micro-aspiration in SM gas injured patients with chronic pulmonary diseases and recurrent episodes of exacerbations. Materials and Methods: This cross-sectional study was done at Baqiyatallah University of Medical Sciences, Tehran, Iran. Gastric micro-aspiration and GER were assessed in the enrolled patients by assessing bile acids, pepsin and trypsin in their bronchoalveolar lavage fluid. Results: Our result showed that bile acids were found to be high in 21.4% patients, and low in 53.6% of patients. Only in 16% patients, no bile was detected in the BALF. Trypsin and pepsin were detected in BAL fluid of all patients. Conclusion: Most of BO patients after exposure to SM suffer GER, while none the etiologic factors of GER in post lung transplant BO are present. It would be hypothesized that GER per se could be considered as an aggregative factor for exacerbations in patients. Further studies will provide more advances to better understanding of pathophysiological mechanism regarding GER and BO and treatment.

A shift in the collagen V antigenic epitope leads to T helper phenotype switch and immune response to self-antigen leading to chronic lung allograft rejection.

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Tiriveedhi, V; Angaswamy, N; Brand, D; Weber, J; Gelman, A G; Hachem, R; Trulock, E P; Meyers, B; Patterson, G; Mohanakumar, T

2012-01-01

Immune responses to human leucocyte antigen (HLA) and self-antigen collagen V (Col-V) have been proposed in the pathogenesis of chronic rejection (bronchiolitis obliterans syndrome, BOS) following human lung transplantation (LTx). In this study, we defined the role for the shift in immunodominant epitopes of Col-V in inducing T helper phenotype switch leading to immunity to Col-V and BOS. Sera and lavage from BOS(+) LTx recipients with antibodies to Col-V were analysed. Two years prior to BOS, patients developed antibodies to both Col-V,α1(V) and α2(V) chains. However, at clinical diagnosis of BOS, antibodies became restricted to α1(V). Further, lung biopsy from BOS(+) patients bound to antibodies to α1(V), indicating that these epitopes are exposed. Fourteen Col-V peptides [pep1-14, pep1-4 specific to α1(V), pep5-8 to α1,2(V) and pep9-14 to α2(V)] which bind to HLA-DR4 and -DR7, demonstrated that prior to BOS, pep 6, 7, 9, 11 and 14 were immunodominant and induced interleukin (IL)-10. However, at BOS, the response switched to pep1, 4 and 5 and induced interferon (IFN)-γ and IL-17 responses, but not IL-10. The T helper (Th) phenotype switch is accompanied by decreased frequency of regulatory T cells (T(regs) ) in the lavage. LTx recipients with antibodies to α1(V) also demonstrated increased matrix metalloproteinase (MMP) activation with decreased MMP inhibitor, tissue inhibitor of metalloproteinase (TIMP), suggesting that MMP activation may play a role in the exposure of new Col-V antigenic epitopes. We conclude that a shift in immunodominance of self-antigenic determinants of Col-V results in induction of IFN-γ and IL-17 with loss of tolerance leading to autoimmunity to Col-V, which leads to chronic lung allograft rejection. © 2011 The Authors. Clinical and Experimental Immunology © 2011 British Society for Immunology.

Late lung parenchymal changes on HRCT in children with mycoplasma pneumonia

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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

Late lung parenchymal changes on HRCT in children with mycoplasma pneumonia

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Lee, Soo Hyeon; Kim, Joung Sook; Yoon, Jung Hee; Hur, Gham; Kim, Chang Gun

1999-01-01

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

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Background: Balanitis Xerotic obliterans. Balanitis Xerotic obliterans (BXO) or Penile Lichen Sclerosus is a dermatological. Penile Lichen Sclerosus is a dermatological condition affecting the genitalia and associated with chronic, progressive, sclerosing inflammatory dermatosis of unclear etiology. It was first described by ...

Immotile cilia syndrome in an aged dog

International Nuclear Information System (INIS)

Killingsworth, C.R.; Slocombe, R.F.; Wilsman, N.J.

1987-01-01

An 11-year-old Dalmatian was examined and treated for bilateral nasal discharge and cough of 6 months' duration. Response to medical treatment and surgical intervention was unsatisfactory. Histologic examination of lung tissue revealed chronic severe catarrhal bronchitis and bronchiolitis with bronchiectasis. Histologic findings and barium sulfate bronchography indicated abnormal mucociliary clearance in the respiratory tract. Electron microscopy revealed abnormalities or deletions of outer and/or inner dynein arms in 26% of the ciliary profiles from the affected dog. Similar abnormalities were not found in 500 ciliary profiles from age- and gender-matched control dogs

Respiratory bronchiolitis-associated interstitial lung disease secondary to electronic nicotine delivery system use confirmed with open lung biopsy.

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Flower, Mark; Nandakumar, Lakshmy; Singh, Mahendra; Wyld, David; Windsor, Morgan; Fielding, David

2017-05-01

As a modern phenomenon, there is currently limited understanding of the possible toxic effects and broader implications of electronic nicotine delivery systems (ENDS). Large volumes of aerosolized particles are inhaled during "vaping" and there are now an increasing number of case reports demonstrating toxic effects of ENDS, as well as human studies demonstrating impaired lung function in users. This article presents a case of respiratory bronchiolitis interstitial lung disease (RB-ILD) precipitated by vaping in a 33-year-old male with 10 pack years of traditional cigarette and prior treatment for mixed germ cell tumour. The patient had started vaping 10-15 times per day while continuing to smoke 10 traditional cigarettes per day. After 3 months of exposure to e-cigarette vapour, chest computed tomography demonstrated multiple new poorly defined pulmonary nodules with fluffy parenchyma opacification centred along the terminal bronchovascular units. Video-assisted thoracoscopy with lung biopsy of the right upper and right middle lobes was undertaken. The microscopic findings were overall consistent with RB-ILD. This case demonstrates toxicity with use of ENDS on open lung biopsy with resolution of radiographic findings on cessation. We believe that this is the first case where open lung biopsy has demonstrated this and our findings are consistent with RB-ILD.

IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis: A case report.

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Chen, Chiu-Fan; Chu, Kuo-An; Tseng, Yen-Chiang; Wu, Chang-Che; Lai, Ruay-Sheng

2017-12-01

IgG4-related disease is a rare and novel disease entity that tends to involve multiple organs. The pulmonary manifestation of this disease is highly variable and may mimic lung cancer, pneumonia, interstitial lung disease (ILD), sarcoidosis, and so forth. Small airway disease is rarely reported in IgG4-related lung disease (IgG4-RLD). In the current study, we describe a rare case of IgG4-RLD with patterns of ILD and bronchiolitis. A 43-year-old man had chronic cough and dyspnea on exertion for 4 years. Initial chest radiography showed diffuse interstitial infiltration. Follow-up chest computed tomography 4 years later revealed bilateral diffuse centrilobular nodules with tree-in-bud pattern, bronchial wall thickening, and mediastinal lymph nodes. Bilateral diffuse multifocal ground-glass opacities and mosaic attenuation were also observed. Pulmonary function test revealed mixed restrictive and obstructive ventilatory impairment. Video-assisted thoracoscopic surgery (VATS) lung biopsy showed interstitial fibrosis with lymphoplasmacytic infiltration rich in IgG4-positive plasma cells. Serum IgG4 level also showed remarkable elevation. Therefore, IgG4-RLD is confirmed. VATS wedge resection of right upper lobe and mediastinal lymph node. The patient responded well to steroid and immunosuppression therapy, and was regular followed-up in outpatient clinic. IgG4-RLD should be considered not only in ILD, but also in small airway disease. Serum IgG4 level may be a useful tool for screening.

Short communication: Reactivity of diacetyl with cleaning and sanitizing agents.

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Rincon-Delgadillo, M I; Lopez-Hernandez, A; Rankin, S A

2013-01-01

Diacetyl is used to impart a buttery flavor to numerous food products such as sour cream, cottage cheese, vegetable oil-based spreads, baked goods, and beverages. Recent studies have linked exposure to high concentrations of diacetyl and the onset of bronchiolitis obliterans. Due to the reported risks that diacetyl may pose, many food companies have altered practices to reduce worker exposure to diacetyl, including the use of personal respirators, improved air handling systems, and adequate cleaning practices. Commonly used cleaning and sanitizing agents may be reactive with diacetyl; however, the efficacy of these chemicals has not been studied in detail and remains unclear. The objective of this work was to study the reaction chemistry of diacetyl with common industrial cleaning and sanitizing chemicals. The reactions were assessed at equimolar concentrations and analyzed by gas chromatography-mass spectrometry. Peroxyacetic acid was most reactive with diacetyl (95% reduction in diacetyl), followed by sodium hypochlorite (76% reduction), and hydrogen peroxide (26% reduction). Benzalkonium chloride (BAC) did not react with diacetyl. Acetic acid was detected as the main product of reactions of diacetyl with peroxyacetic acid, sodium hypochlorite, and hydrogen peroxide. 1,1-Dichloro-2-propanone and 1,1,1-trichloropropanone were also identified as volatile reaction products in the sodium hypochlorite reactions. Copyright © 2013 American Dairy Science Association. Published by Elsevier Inc. All rights reserved.

Evaluation of the hypersensitivity potential of alternative butter flavorings

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Anderson, Stacey E.; Franko, Jennifer; Wells, J.R.; Lukomska, Ewa; Meade, B. Jean

2015-01-01

Concern has been raised over the association of diacetyl with lung disease clinically resembling bronchiolitis obliterans in food manufacturing workers. This has resulted in the need for identification of alternative chemicals to be used in the manufacturing process. Structurally similar chemicals, 2,3-pentanedione, 2,3-hexanedione, 3,4-hexanedione and 2,3-heptanedione, used as constituents of synthetic flavoring agents have been suggested as potential alternatives for diacetyl, however, immunotoxicity data on these chemicals are limited. The present study evaluated the dermal irritation and sensitization potential of diacetyl alternatives using a murine model. None of the chemicals were identified as dermal irritants when tested at concentrations up to 50%. Similar to diacetyl (EC3 = 17.9%), concentration-dependent increases in lymphocyte proliferation were observed following exposure to all four chemicals, with calculated EC3 values of 15.4% (2,3-pentanedione), 18.2% (2,3-hexanedione), 15.5% (3,4-hexanedione) and 14.1% (2,3-heptanedione). No biologically significant elevations in local or total serum IgE were identified after exposure to 25–50% concentrations of these chemicals. These results demonstrate the potential for development of hypersensitivity responses to these proposed alternative butter flavorings and raise concern about the use of structurally similar replacement chemicals. Additionally, a contaminant with strong sensitization potential was found in varying concentrations in diacetyl obtained from different producers. PMID:24007741

Bronchoscopy for the diagnosis of pulmonary tuberculosis in patients with negative sputum smear microscopy results.

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Jacomelli, Márcia; Silva, Priscila Regina Alves Araújo; Rodrigues, Ascedio Jose; Demarzo, Sergio Eduardo; Seicento, Márcia; Figueiredo, Viviane Rossi

2012-01-01

To evaluate the diagnostic accuracy of bronchoscopy in patients with clinical or radiological suspicion of tuberculosis who were unable to produce sputum or with negative sputum smear microscopy results. A prospective cross-sectional study involving 286 patients under clinical or radiological suspicion of having pulmonary tuberculosis and submitted to bronchoscopy-BAL and transbronchial biopsy (TBB). The BAL specimens were submitted to direct testing and culture for AFB and fungi, whereas the TBB specimens were submitted to histopathological examination. Of the 286 patients studied, 225 (79%) were diagnosed on the basis of bronchoscopic findings, as follows: pulmonary tuberculosis, in 127 (44%); nonspecific chronic inflammation, in 51 (18%); pneumocystis, fungal infections, or nocardiosis, in 20 (7%); bronchiolitis obliterans organizing pneumonia, alveolites, or pneumoconiosis, in 14 (5%); lung or metastatic neoplasms, in 7 (2%); and nontuberculous mycobacterium infections, in 6 (2%). For the diagnosis of tuberculosis, BAL showed a sensitivity and a specificity of 60% and 100%, respectively. Adding the TBB findings significantly increased this sensitivity (to 84%), as did adding the post-bronchoscopy sputum smear microscopy results (total sensitivity, 94%). Minor post-procedure complications occurred in 5.6% of the cases. Bronchoscopy is a reliable method for the diagnosis of pulmonary tuberculosis, with low complication rates. The combination of TBB and BAL increases the sensitivity of the method and facilitates the differential diagnosis with other diseases.

[Lower lymphocyte response in severe cases of acute bronchiolitis due to respiratory syncytial virus].

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Ramos-Fernández, José Miguel; Moreno-Pérez, David; Antúnez-Fernández, Cristina; Milano-Manso, Guillermo; Cordón-Martínez, Ana María; Urda-Cardona, Antonio

2017-08-14

Acute bronchiolitis (AB) of the infant has a serious outcome in 6-16% of the hospital admitted cases. Its pathogenesis and evolution is related to the response of the T lymphocytes. The objective of the present study is to determine if the lower systemic lymphocytic response is related to a worse outcome of AB in hospitalised infants. Retrospective observational-analytical study of cases-controls nested in a cohort of patients admitted due to RSV-AB between the period from October 2010 to March 2015. Those with a full blood count in the first 48hours of respiratory distress were included. Infants with underlying disease, bacterial superinfection, and premature infants <32 weeks of gestation were excluded. The main dichotomous variable was PICU admission. Other variables were: gender, age, post-menstrual age, gestational and post-natal tobacco exposure, admission month, type of lactation, and days of onset of respiratory distress. Lymphocyte counts were categorised by quartiles. Bivariate analysis was performed with the main variable and then by logistic regression to analyse confounding factors. The study included 252 infants, of whom 6.6% (17) required PICU admission. The difference in mean±SD of lymphocytes for patients admitted to and not admitted to PICU was 4,044±1755 and 5,035±1786, respectively (Student-t test, P<.05). An association was found between PICU admission and lymphocyte count <3700/ml (Chi-squared, P=.019; OR: 3.2) and it was found to be maintained in the logistic regression, regardless of age and all other studied factors (Wald 4.191 P=.041, OR: 3.8). A relationship was found between lymphocytosis <3700/ml in the first days of respiratory distress and a worse outcome in previously healthy infants <12 months and gestational age greater than 32 weeks with RSV-AB. Copyright © 2017. Publicado por Elsevier España, S.L.U.

Increased Risk of RSV Infection in Children with Down's Syndrome: Clinical Implementation of Prophylaxis in the European Union

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Dianne van Beek

2013-01-01

Full Text Available Prospective cohort studies show that Down’s syndrome (DS is an independent risk factor for hospitalization for RSV bronchiolitis. It is unknown whether this observation has been translated into specific management for DS children. The primary goal was to assess the knowledge of healthcare providers in the European Union about RSV infection in DS children and to determine whether it influenced the implementation of prophylaxis. DS caregivers were surveyed using a standardized questionnaire, and country-specific guidelines were obtained. Fifty-three caregivers participated. Thirty-nine (86.7% had knowledge of the increased risk of severe RSV infection in DS children, and 30 (71.4% graded that it was important to have a statement on the use of RSV prophylaxis in existing guidelines. Twenty-eight participants had a local DS guideline; hard copies of twelve unique guidelines were obtained. Only one (8.3% contained a statement on RSV prophylaxis for DS, and five considered such a statement for the next version. Conclusion. Most pediatricians had knowledge that DS children have an increased risk of severe RSV infection. Despite the lack of a specific RSV prophylaxis trial in DS, they felt that a statement on RSV prophylaxis in DS guidelines was important, but this was rarely present in current guidelines.

Increased risk of RSV infection in children with Down's syndrome: clinical implementation of prophylaxis in the European Union.

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van Beek, Dianne; Paes, Bosco; Bont, Louis

2013-01-01

Prospective cohort studies show that Down's syndrome (DS) is an independent risk factor for hospitalization for RSV bronchiolitis. It is unknown whether this observation has been translated into specific management for DS children. The primary goal was to assess the knowledge of healthcare providers in the European Union about RSV infection in DS children and to determine whether it influenced the implementation of prophylaxis. DS caregivers were surveyed using a standardized questionnaire, and country-specific guidelines were obtained. Fifty-three caregivers participated. Thirty-nine (86.7%) had knowledge of the increased risk of severe RSV infection in DS children, and 30 (71.4%) graded that it was important to have a statement on the use of RSV prophylaxis in existing guidelines. Twenty-eight participants had a local DS guideline; hard copies of twelve unique guidelines were obtained. Only one (8.3%) contained a statement on RSV prophylaxis for DS, and five considered such a statement for the next version. Conclusion. Most pediatricians had knowledge that DS children have an increased risk of severe RSV infection. Despite the lack of a specific RSV prophylaxis trial in DS, they felt that a statement on RSV prophylaxis in DS guidelines was important, but this was rarely present in current guidelines.

CD64 on monocytes and granulocytes in severe acute bronchiolitis: Pilot study on its usefulness as a bacterial infection biomarker.

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García-Salido, Alberto; Serrano-González, Ana; Casado-Flores, Juan; Sierra-Colomina, Montserrat; de Azagra-Garde, Amelia Martínez; García-Teresa, María Ángeles; Melen, Gustavo J; Ramírez-Orellana, Manuel

2018-02-27

The CD64 receptor has been described as a biomarker of bacterial infection. We speculated that CD64 surface expression on monocytes and granulocytes of children with severe acute bronchiolitis (SAB) could be altered in cases of probable bacterial infection (PBI) determined using classical biomarkers (procalcitonin and C-reactive protein, leukocyte count, and radiographic findings). A prospective observational pilot study was conducted from October 2015 to February 2016 in children admitted for pediatric critical care. A blood sample was taken in the first 24 hours of admission, and CD64 was measured by flow cytometry. The values obtained were analyzed and correlated with traditional biomarkers of PBI. Thirty-two children were included; a correlation was found between CD64 expression and the PBI criteria. CD64 surface expression was higher in children with PBI (area under the receiver operating characteristic curve of 0.73; P = 0.042) and the percentage of CD64 + granulocytes was higher in children with PBI. This is the first study to describe CD64 surface expression on monocytes and granulocytes in SAB, finding CD64 values to be higher in children with PBI. Larger clinical studies are needed to elucidate the real accuracy of CD64 as a biomarker of bacterial infection. ©2018 Society for Leukocyte Biology.

Understanding Bartter syndrome and Gitelman syndrome.

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Fremont, Oliver T; Chan, James C M

2012-02-01

We aim to review the clinical features of two renal tubular disorders characterized by sodium and potassium wasting: Bartter syndrome and Gitelman syndrome. Selected key references concerning these syndromes were analyzed, together with a PubMed search of the literature from 2000 to 2011. The clinical features common to both conditions and those which are distinct to each syndrome were presented. The new findings on the genetics of the five types of Bartter syndrome and the discrete mutations in Gitelman syndrome were reviewed, together with the diagnostic workup and treatment for each condition. Patients with Bartter syndrome types 1, 2 and 4 present at a younger age than classic Bartter syndrome type 3. They present with symptoms, often quite severe in the neonatal period. Patients with classic Bartter syndrome type 3 present later in life and may be sporadically asymptomatic or mildly symptomatic. The severe, steady-state hypokalemia in Bartter syndrome and Gitelman syndrome may abruptly become life-threatening under certain aggravating conditions. Clinicians need to be cognizant of such renal tubular disorders, and promptly treat at-risk patients.

HRCT of the lung in collagen vascular diseases

International Nuclear Information System (INIS)

Diederich, S.; Roos, N.; Schmitz-Linneweber, B.; Gaubitz, M.; Peters, P.E.

1996-01-01

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.) [de

[Lung transplantation in sporadic lymphangioleiomyomatosis: study of 7 cases].

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Ansótegui Barrera, Emilio; Mancheño Franch, Nuria; Peñalver Cuesta, Juan Carlos; Vera-Sempere, Francisco; Padilla Alarcón, José

2013-10-19

Sporadic lymphangioleiomyomatosis (S-LAM) is a rare disease that affects only women. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells) that grow in an aberrant manner in the airway, parenchymal lung lymph and blood vessels, determining the onset of pulmonary cystic lesions. The disease has no treatment, progressing to respiratory failure, and lung transplantation (LT) may be a treatment option at this stage. Our goal was to study 7 patients undergoing LT for S-LAM. We studied a series of clinical and demographic characteristics, diagnostic modality and post-transplant outcomes. We performed a descriptive analysis of the series. The Kaplan-Meier method was used to estimate survival. The mean age of onset of symptoms was 35 years, the diagnosis of 37 years and that of LT 38 years. The most common symptom was dyspnea. Four patients had a history of pneumothorax and pleural effusion. The mean forced expiratory volume in one second was 32.7% and the diffusing capacity for carbon monoxide was 29%. All patients were subjected to LT and survival was 100, 85.7 and 57.1% at one, 3 and 5 years, respectively. Three died of bronchiolitis obliterans and 2 necropsies did not show evidence of disease recurrence. LT is a therapeutic option in patients with S-LAM with an advanced respiratory functional impairment. Copyright © 2013 Elsevier España, S.L. All rights reserved.

Ground-glass opacity at high resolution CT: an approach for differential diagnosis

International Nuclear Information System (INIS)

Spina, Juan C.; Rogondino, Jose; Vidales, Valeria; Rolnik, Maria C.; Montanari, Mariano; Salazar, Santiago N.

2004-01-01

Purpose: To evaluate the Ground-Glass Opacity in high resolution computed tomography (HRCT) with its underlying abnormality and anatomic distribution and its correlation with different etiologies. Methods: A 38 patients series, (32 men, 16 women, mean age 54,6 years, range 20-28) was retrospectively analyzed. They were evaluated with high resolution computed tomography, 2 mm thick sections and 10 mm of interval. Contrast intravenous iodinated contrast (no-ionic) was injected in 11 patients. The final diagnosis was made with sputum analysis, bronchioalveolar lavage, trans bronchial biopsy and open lung biopsy. Results: The differential diagnosis of ground glass opacity is based on analyzing their anatomic resolution and the underlying pathology in the lung parenchyma. Centrilobular distribution indicated early air-spaces pathology produced in our series by 21 infections, 4 pulmonary hemorrhages, 1 hypersensitivity pneumonitis and 1 descamative interstitial pneumonitis. Panlobular distribution, alveolar proteinosis (1 case) sarcoidosis (1 case) drug toxicity 1 case and one case of pneumocystis carinii. Peripherical distribution typical of early idiopathic fibrosis (1). Bronchiolitis obliterans with organizing pneumonia (1). Structural alterations of the lung parenchyma with bronchiectasias was seen in 16 cases, cystic lesions in 3 cases, sub pleural linear opacities 4 cases, peribronchovascular interstitial thickening or nodularity and emphysema in 10 cases. Conclusion: HRCT is useful to evaluate ground glass opacities pattern with the anatomic distribution and the underlying structural pathology. These findings under some clinical circumstances can suggest a specific diagnosis in most cases, indicating a potentially treatable disease. (author)

Sulfur mustard and respiratory diseases.

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Tang, Feng Ru; Loke, Weng Keong

2012-09-01

Victims exposed to sulfur mustard (HD) in World War I and Iran-Iraq war, and those suffered occupational or accidental exposure have endured discomfort in the respiratory system at early stages after exposure, and marked general physical deterioration at late stages due to pulmonary fibrosis, bronchiolitis obliterans or lung cancer. At molecule levels, significant changes of cytokines and chemokines in bronchoalveolar lavage and serum, and of selectins (in particular sE-selectin) and soluble Fas ligand in the serum have been reported in recent studies of patients exposed to HD in Iran-Iraq war, suggesting that these molecules may be associated with the pathophysiological development of pulmonary diseases. Experimental studies in rodents have revealed that reactive oxygen and nitrogen species, their product peroxynitrite (ONOO(-)), nitric oxide synthase, glutathione, poly (adenosine diphosphate-ribose) polymerase, activating protein-1 signaling pathway are promising drug targets for preventing HD-induced toxicity, whereas N-acetyl cysteine, tocopherols, melatonin, aprotinin and many other molecules have been proved to be effective in prevention of HD-induced damage to the respiratory system in different animal models. In this paper, we will systemically review clinical and pathophysiological changes of respiratory system in victims exposed to HD in the last century, update clinicians and researchers on the mechanism of HD-induced acute and chronic lung damages, and on the relevant drug targets for future development of antidotes for HD. Further research directions will also be proposed.

Epidemiology of organising pneumonia in Iceland

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Gudmundsson, G; Sveinsson, O; Isaksson, H J; Jonsson, S; Frodadottir, H; Aspelund, T

2006-01-01

Background Cryptogenic organising pneumonia (COP) has also been called idiopathic bronchiolitis obliterans organising pneumonia. In secondary organising pneumonia (SOP) the causes can be identified or it occurs in a characteristic clinical context. The aim of this study was to determine the incidence and epidemiological features of COP and SOP nationwide in Iceland over an extended period. Methods A retrospective study of organising pneumonia (OP) in Iceland over 20 years was conducted and the epidemiology and survival were studied. All pathological reports of patients diagnosed with or suspected of having COP or SOP in the period 1984–2003 were identified and the pathology samples were re‐evaluated using strict diagnostic criteria. Results After re‐evaluation, 104 patients fulfilled the diagnostic criteria for OP (58 COP and 46 SOP). The mean annual incidence of OP was 1.97/100 000 population (1.10/100 000 for COP and 0.87/100 000 for SOP). The mean age at diagnosis was 67 years with a wide age range. The most common causes of death were lung diseases other than OP, and only one patient died from OP. Patients with OP had a lower rate of survival than the general population, but there was no statistical difference between COP and SOP. Conclusions The incidence of OP is higher than previously reported, suggesting that OP needs to be considered as a diagnosis more often than has been done in the past. PMID:16809413

[Lung transplantation in pulmonary fibrosis and other interstitial lung diseases].

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Berastegui, Cristina; Monforte, Victor; Bravo, Carlos; Sole, Joan; Gavalda, Joan; Tenório, Luis; Villar, Ana; Rochera, M Isabel; Canela, Mercè; Morell, Ferran; Roman, Antonio

2014-09-15

Interstitial lung disease (ILD) is the second indication for lung transplantation (LT) after emphysema. The aim of this study is to review the results of LT for ILD in Hospital Vall d'Hebron (Barcelona, Spain). We retrospectively studied 150 patients, 87 (58%) men, mean age 48 (r: 20-67) years between August 1990 and January 2010. One hundred and four (69%) were single lung transplants (SLT) and 46 (31%) bilateral-lung transplants (BLT). The postoperative diagnoses were: 94 (63%) usual interstitial pneumonia, 23 (15%) nonspecific interstitial pneumonia, 11 (7%) unclassifiable interstitial pneumonia and 15% miscellaneous. We describe the functional results, complications and survival. The actuarial survival was 87, 70 and 53% at one, 3 and 5 years respectively. The most frequent causes of death included early graft dysfunction and development of chronic rejection in the form of bronchiolitis obliterans (BOS). The mean postoperative increase in forced vital capacity and forced expiratory volume in the first second (FEV1) was similar in SLT and BLT. The best FEV1 was reached after 10 (r: 1-36) months. Sixteen percent of patients returned to work. At some point during the evolution, proven acute rejection was diagnosed histologically in 53 (35%) patients. The prevalence of BOS among survivors was 20% per year, 45% at 3 years and 63% at 5 years. LT is the best treatment option currently available for ILD, in which medical treatment has failed. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

Utilization of the organ care system for bilateral lung transplantation: preliminary results of a comparative study.

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Zeriouh, Mohamed; Sabashnikov, Anton; Mohite, Prashant N; Zych, Bartlomiej; Patil, Nikhil P; García-Sáez, Diana; Koch, Achim; Weymann, Alexander; Soresi, Simona; Wippermann, Jens; Wahlers, Thorsten; De Robertis, Fabio; Popov, Aron-Frederik; Simon, André R

2016-09-01

Lung transplantation (LTx) remains the gold standard for patients with end-stage lung disease. However, due to donor organ shortage and brain stem death-related lung injury, only a small proportion of lungs are used increasing the mortality rate on the waiting list. A portable normothermic continuous ex vivo perfusion using the organ care system (OCS) represents one of the tools to increase the pool of donor organs and to improve the function of marginal lungs. We sought to assess mid-term outcomes after LTx using OCS and to compare outcomes including overall survival and freedom from bronchiolitis obliterans syndrome (BOS) with those after conventional preservation. Included were 322 consecutive LTx performed at Harefield Hospital between January 2007 and December 2014. Recipients were divided into two groups depending on the organ storage strategy: the majority of patients (n = 308) were transplanted using lungs after cold storage (cold storage group), whereas 14 organs were preserved using OCS (OCS group). The primary end-points were overall survival and freedom from BOS after LTx. The secondary end-points were perioperative clinical characteristics and adverse events. There were no statistically significant differences in terms of most baseline donor and recipient characteristics. The percentage of heavy smokers among donors [8 (2.9%) vs 6 (42.9%), P < 0.001] and the median number of pack-years smoked by donors [14 (7;24) vs 25 (24;30), P = 0.026] were statistically higher in the OCS group. Patients from the OCS group had significantly better postoperative FEV1 at 3 [69 (54;86) vs 93 (87;89), P < 0.001] and 6 [77 (60;90) vs 94 (84;100), P = 0.006] months. There were no statistically significant differences in terms of cumulative survival and freedom from BOS between the two groups. Results after LTx using OCS are acceptable with excellent survival, and superior early outcome in terms of postoperative lung function despite higher risk in the OCS group. Further

Mir-22-3p Inhibits Arterial Smooth Muscle Cell Proliferation and Migration and Neointimal Hyperplasia by Targeting HMGB1 in Arteriosclerosis Obliterans

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Shui-chuan Huang

2017-08-01

Full Text Available Background: Aberrant vascular smooth muscle cell (VSMC proliferation and migration contribute to the development of vascular pathologies, such as atherosclerosis and post-angioplasty restenosis. The aim of this study was to determine whether miR-22-3p plays a role in regulating human artery vascular smooth muscle cell (HASMC function and neointima formation. Methods: Quantitative real-time PCR (qRT-PCR and fluorescence in situ hybridization (FISH were used to detect miR-22-3p expression in human arteries. Cell Counting Kit-8 (CCK-8 and EdU assays were performed to assess cell proliferation, and transwell and wound closure assays were performed to assess cell migration. Moreover, luciferase reporter assays were performed to identify the target genes of miR-22-3p. Finally, a rat carotid artery balloon-injury model was used to determine the role of miR-22-3p in neointima formation. Results: MiR-22-3p expression was downregulated in arteriosclerosis obliterans (ASO arteries compared with normal arteries, as well as in platelet-derived growth factor-BB (PDGF-BB-stimulated HASMCs compared with control cells. MiR-22-3p overexpression had anti-proliferative and anti-migratory effects and dual-luciferase assay showed that high mobility group box-1 (HMGB1 is a direct target of miR-22-3p in HASMCs. Furthermore, miR-22-3p expression was negatively correlated with HMGB1 expression in ASO tissue specimens. Finally, LV-miR-22-3p-mediated miR-22-3p upregulation significantly suppressed neointimal hyperplasia specifically by reducing HMGB1 expression in vivo. Conclusions: Our results indicate that miR-22-3p is a key molecule in regulating HASMC proliferation and migration by targeting HMGB1 and that miR-22-3p and HMGB1 may be therapeutic targets in the treatment of human ASO.

[Maxillofacial and dental abnormalities in some multiple abnormality syndromes. "Cri du chat" syndrome, Wilms' tumor-aniridia syndrome; Sotos syndrome; Goldenhar syndrome].

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Berio, A; Trucchi, R; Meliota, M

1992-05-01

The paper describes the maxillo-facial and dental anomalies observed in some chromosome and non-chromosome poly-malformative syndromes ("Cri du chat" syndrome; Wilms' tumour; Sotos' syndrome; Goldenhar's syndrome). The Authors emphasise the possibility of diagnosing these multiple deformity syndromes from maxillo-facial alterations in early infancy; anomalous tooth position and structure cal also be successfully treated immediately after the first appearance of teeth. This is a particularly promising field of pediatrics and preventive pediatric medicine.

Effects of vitamin D in the prevention of acute viral bronchiolitis: systematic review = Efeitos da vitamina D na prevenção da bronquiolite viral aguda: revisão sistemática

Directory of Open Access Journals (Sweden)

Rueda, Angélica Maria Barba

2015-01-01

Full Text Available Objetivos: Evidências publicadas sugerem que a suplementação da vitamina D pode ter efeito protetor nas infecções do trato respiratório inferior. O objetivo desta revisão foi avaliar os efeitos da ingestão de vitamina D na prevenção da bronquiolite viral aguda em crianças. Métodos: Foram feitas buscas nas bases de dados Medline, EMBASE, Web of Science, LILACS e Cochrane Central Register of Controlled Trials, até dezembro de 2014, usando os descritores "Vitamin D" ou cholecalciferol ou ergocalciferol e "bronchiolitis, viral" ou "viral bronchiolitis" ou "bronchiolitides, viral" ou "viral bronchiolitides". Foram incluídos estudos que avaliaram o efeito da ingesta da vitamina D na prevenção da bronquiolite viral aguda em crianças. Estudos com intervenção menor que duas semanas e artigos de revisão foram excluídos. Resultados: A busca identificou 241 artigos, entre os quais 20 artigos foram selecionados para leitura na íntegra e dois artigos foram incluídos na revisão sistemática, incluindo 296 crianças. Nenhum estudo mediu os níveis séricos de vitamina D. Um dos estudos incluídos foi um ensaio clinico, no qual o número de episódios de bronquiolite foi significativamente menor nas crianças suplementadas com vitamina D (Grupo I: média 0, 6±0, 7 Grupo II: média 1, 4 ±0, 9; P =0, 001. No outro, um estudo de casos e controles, não se encontrou relação significativa entre casos de bronquiolite viral aguda e ingesta de vitamina D (odds ratio 1, 7 – intervalo de confiança 95% 0, 7 - 4, 0. Conclusões: As evidências científicas atuais são insuficientes para comprovar os benefícios clínicos da vitamina D na prevenção da bronquiolite viral aguda

Marfan syndrome masked by Down syndrome?

NARCIS (Netherlands)

Vis, J.C.; Engelen, K. van; Timmermans, J.; Hamel, B.C.J.; Mulder, B.J.

2009-01-01

Down syndrome is the most common chromosomal abnormality. A simultaneous occurrence with Marfan syndrome is extremely rare. We present a case of a 28-year-old female with Down syndrome and a mutation in the fibrillin-1 gene. The patient showed strikingly few manifestations of Marfan syndrome.

The developmental trajectory of disruptive behavior in Down syndrome, fragile X syndrome, Prader-Willi syndrome and Williams syndrome.

Science.gov (United States)

Rice, Lauren J; Gray, Kylie M; Howlin, Patricia; Taffe, John; Tonge, Bruce J; Einfeld, Stewart L

2015-06-01

The aim of this study was to investigate the developmental trajectories of verbal aggression, physical aggression, and temper tantrums in four genetic syndrome groups. Participants were part of the Australian Child to Adult Development Study (ACAD), which collected information from a cohort of individuals with an intellectual disability at five time points over 18 years. Data were examined from a total of 248 people with one of the four following syndromes: Down syndrome, Fragile X syndrome, Prader-Willi syndrome, or Williams syndrome. Changes in behaviors were measured using validated items from the Developmental Behavior Checklist (DBC). The results indicate that, while verbal aggression shows no evidence of diminishing with age, physical aggression, and temper tantrums decline with age before 19 years for people with Down syndrome, Fragile X syndrome, and William syndrome; and after 19 years for people with Prader-Willi syndrome. These findings offer a somewhat more optimistic outlook for people with an intellectual disability than has previously been suggested. Research is needed to investigate the mechanisms predisposing people with PWS to persistence of temper tantrums and physical aggression into adulthood. © 2015 Wiley Periodicals, Inc.

Hamartomatous polyposis syndromes

DEFF Research Database (Denmark)

Jelsig, Anne Marie; Qvist, Niels; Brusgaard, Klaus

2014-01-01

Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes such as ......Hamartomatous Polyposis Syndromes (HPS) are genetic syndromes, which include Peutz-Jeghers syndrome, Juvenile polyposis syndrome, PTEN hamartoma tumour syndrome (Cowden Syndrom, Bannayan-Riley-Ruvalcaba and Proteus Syndrome) as well as hereditary mixed polyposis syndrome. Other syndromes...

Pulmonary autoimmunity as a feature of autoimmune polyendocrine syndrome type 1 and identification of KCNRG as a bronchial autoantigen.

Science.gov (United States)

Alimohammadi, Mohammad; Dubois, Noémie; Sköldberg, Filip; Hallgren, Asa; Tardivel, Isabelle; Hedstrand, Håkan; Haavik, Jan; Husebye, Eystein S; Gustafsson, Jan; Rorsman, Fredrik; Meloni, Antonella; Janson, Christer; Vialettes, Bernard; Kajosaari, Merja; Egner, William; Sargur, Ravishankar; Pontén, Fredrik; Amoura, Zahir; Grimfeld, Alain; De Luca, Filippo; Betterle, Corrado; Perheentupa, Jaakko; Kämpe, Olle; Carel, Jean-Claude

2009-03-17

Patients with autoimmune polyendocrine syndrome type 1 (APS-1) suffer from multiple organ-specific autoimmunity with autoantibodies against target tissue-specific autoantigens. Endocrine and nonendocrine organs such as skin, hair follicles, and liver are targeted by the immune system. Despite sporadic observations of pulmonary symptoms among APS-1 patients, an autoimmune mechanism for pulmonary involvement has not been elucidated. We report here on a subset of APS-1 patients with respiratory symptoms. Eight patients with pulmonary involvement were identified. Severe airway obstruction was found in 4 patients, leading to death in 2. Immunoscreening of a cDNA library using serum samples from a patient with APS-1 and obstructive respiratory symptoms identified a putative potassium channel regulator (KCNRG) as a pulmonary autoantigen. Reactivity to recombinant KCNRG was assessed in 110 APS-1 patients by using immunoprecipitation. Autoantibodies to KCNRG were present in 7 of the 8 patients with respiratory symptoms, but in only 1 of 102 APS-1 patients without respiratory symptoms. Expression of KCNRG messenger RNA and protein was found to be predominantly restricted to the epithelial cells of terminal bronchioles. Autoantibodies to KCNRG, a protein mainly expressed in bronchial epithelium, are strongly associated with pulmonary involvement in APS-1. These findings may facilitate the recognition, diagnosis, characterization, and understanding of the pulmonary manifestations of APS-1.

Seckel syndrome: an overdiagnosed syndrome.

OpenAIRE

Thompson, E; Pembrey, M

1985-01-01

Five children in whom a diagnosis of Seckel syndrome had previously been made were re-examined in the genetic unit. One child had classical Seckel syndrome, a sib pair had the features of the syndrome with less severe short stature, and in two children the diagnosis was not confirmed. Seckel syndrome is only one of a group of low birth weight microcephalic dwarfism and careful attention should be paid to fulfillment of the major criteria defined by Seckel before the diagnosis is made. There r...

Nanosized zinc oxide particles do not promote DHPN-induced lung carcinogenesis but cause reversible epithelial hyperplasia of terminal bronchioles.

Science.gov (United States)

Xu, Jiegou; Futakuchi, Mitsuru; Alexander, David B; Fukamachi, Katsumi; Numano, Takamasa; Suzui, Masumi; Shimizu, Hideo; Omori, Toyonori; Kanno, Jun; Hirose, Akihiko; Tsuda, Hiroyuki

2014-01-01

Zinc oxide (ZnO) is known to induce lung toxicity, including terminal bronchiolar epithelial hyperplasia, which gives rise to concerns that nanosized ZnO (nZnO) might lead to lung carcinogenesis. We studied the tumor promoting activity of nZnO by an initiation-promotion protocol using human c-Ha-ras proto-oncogene transgenic rats (Hras128 rats). The rats were given 0.2 % N-nitrosobis(2-hydroxypropyl)amine (DHPN) in the drinking water for 2 weeks and then treated with 0.5 ml of 250 or 500 μg/ml nZnO suspension by intra-pulmonary spraying once every 2 weeks for a total of 7 times. Treatment with nZnO particles did not promote DHPN-induced lung carcinogenesis. However, nZnO dose-dependently caused epithelial hyperplasia of terminal bronchioles (EHTB) and fibrosis-associated interstitial pneumonitis (FAIP) that were independent of DHPN treatment. Tracing the fate of EHTB lesions in wild-type rats indicated that the hyperplastic lesions almost completely disappeared within 12 weeks after the last nZnO treatment. Since nZnO particles were not found in the lung and ZnCl2 solution induced similar lung lesions and gene expression profiles, the observed lesions were most likely caused by dissolved Zn(2+). In summary, nZnO did not promote carcinogenesis in the lung and induced EHTB and FAIP lesions that regressed rapidly, probably due to clearance of surplus Zn(2+) from the lung.

Superior Mesenteric Artery Syndrome or Wilkie Syndrome

International Nuclear Information System (INIS)

Castano Llano, Rodrigo; Chams Anturi, Abraham; Arango Vargas, Paula

2009-01-01

We described three cases of superior mesenteric artery (SMA) syndrome, also known as Wilkie's syndrome, chronic duodenal ileus, or cast syndrome. This syndrome occurs when the third portion of the duodenum is compressed between the SMA and the aorta. The major risk factors for development of SMA syndrome are rapid weight loss and surgical correction of spinal deformities. The clinical presentation of SMA syndrome is variable and nonspecific, including nausea, vomiting, abdominal pain, and weight loss. The diagnosis is based on endoscopic, radiographic and tomographic findings of duodenal compression by the SMA. The treatment of SMA syndrome is aimed at the precipitating factor, which usually is related to weight loss. Therefore, conservative therapy with nutritional supplementation is the initial approach, and surgery is reserved for those who do not respond to nutritional therapy.

Beals Syndrome

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... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of the ... bone) and aortic enlargement problems as people with Marfan syndrome, and treatments for these problems are the same. ...

Serotonin syndrome

Science.gov (United States)

Hyperserotonemia; Serotonergic syndrome; Serotonin toxicity; SSRI - serotonin syndrome; MAO - serotonin syndrome ... brain area. For example, you can develop this syndrome if you take migraine medicines called triptans together ...

[Association Budd Chiari syndrome, antiphospholipid syndrome and Grave's disease].

Science.gov (United States)

Mouelhi, Leila; Chaieb, Mouna; Debbeche, Radhouane; Salem, Mohamed; Sfar, Imene; Trabelsi, Sinda; Gorgi, Yosr; Najjar, Taoufik

2009-02-01

Antiphospholipid syndrome is revealed by Budd Chiari syndrome in 5% of the cases. Antiphospholipid syndrome is characterized by venous or arterial thrombosis, foetal loss and positivity of antiphospholipid antibodies, namely lupus anticoagulant, anticardiolipin antibodies and anti-beta2-glycoprotein I. Anticardiolipin antibodies was reported in auto-immune thyroid disorders, particularly in Grave's disease. Antiphospholipid syndrome associated to Grave's disease was reported in only three cases. To describe a case report of association of Grave's disease and antiphospholipid syndrome. We report the first case of Grave's disease associated with antiphospholipid syndrome, revealed by Budd Chiari syndrome. Our observation is particular by the fact that it is about a patient presenting a Grave's disease associated with antiphospholipid syndrome revealed by Budd Chiari syndrome. This triple association has never been reported in literature. Although association between antiphospholipid syndrome and Grave's disease was previously described, further studies evaluating the coexistence of these two affections in the same patient would be useful.

Duane Syndrome

Science.gov (United States)

... Frequently Asked Questions Español Condiciones Chinese Conditions Duane Syndrome En Español Read in Chinese What is Duane Syndrome? Duane syndrome, also called Duane retraction syndrome (DRS), ...

Wells syndrome and its relationship to Churg-Strauss syndrome.

Science.gov (United States)

Ratzinger, Gudrun; Zankl, Julia; Zelger, Bernhard

2013-08-01

  Wells syndrome has been described as an inflammatory disorder based on typical clinical appearance combined with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Churg-Strauss syndrome, on the other hand, is primarily a diffuse, necrotizing vasculitis but is also typically displaying eosinophils and flame figures. Despite several parallels, the present understanding of these two diseases excludes any pathogenetic relationship.   We describe the clinical course and histopathological appearance of three patients who had initially been diagnosed with Wells syndrome that developed into Churg-Strauss syndrome during the course of their disease.   The clinical presentation of all three patients led to the diagnosis of Wells syndrome by independent specialists. Histopathology showed an eosinophilic infiltrate and flame figures next to features of leukocytoclastic vasculitis. Detailed examination revealed asthma bronchiale and additional symptoms indicating Churg-Strauss syndrome. The initial diagnosis of Wells syndrome had to be revised to Churg-Strauss syndrome.   We conclude that Wells syndrome could be the starting point of a pathogenetic process that might reach its maximum in Churg-Strauss syndrome. As a clinical consequence, patients with Wells syndrome should be evaluated and followed for Churg-Strauss syndrome. © 2013 The International Society of Dermatology.

Goldenhar Syndrome in Association with Duane Syndrome

Directory of Open Access Journals (Sweden)

U D Shrestha

2012-03-01

Full Text Available Goldenhar syndrome (GHS is also known as Oculo-Auriculo-Vertebral (OAV syndrome or Branchial arch syndrome. Duane retraction syndrome (DRS is a congenital disorder of ocular motility characterized by limited abduction, adduction or both. It is unilateral in 80% of cases. The important and interesting part of this eight months old child is presence of GHS with DRS. She has bilateral invol-vement, which is seen in only 5-8% of GHS, as compared to high incidence of unilateral involve-ment. This child also had refractive error of + 6.00/ - 1.5 * 180. At four year of age her vision with glass was 6/9. Children with GHS and DRS should have early eye examination done to treat the problem of refractive error. Keywords: Duane retraction syndrome; goldenhar syndrome, refractive error.

Interstitial lung diseases with fibrosis - the pattern at high resolution

International Nuclear Information System (INIS)

Jarzemska, A.; Lasek, W.; Nawrocka, E.; Meder, G.; Zapala, M.

2003-01-01

Surgical lung biopsy, either open thoracotomy or video-assisted thoracoscopy is recommended in the diagnosis of interstitial lung diseases (ILD). In some cases, however, the repetitive pattern of radiological features in high-resolution computed tomography is often sufficient to confirm the diagnosis in a non-invasive manner. The purpose of the study was to determine whether patients with ILD can be selected on the basis of the HRCT pattern. Thin-section CT scans were performed in 40 patients with histologically proven idiopathic interstitial pneumonia (26 patients with usual interstitial pneumonia UIP, 2 patients with desquamative interstitial pneumonia DIP, 2 patients with bronchiolitis obliterans organizing pneumonia BOOP, 2 patients with non-specific interstitial pneumonia NSIP, 11 patients with hypersensitivity pneumonitis, and 3 patients with pulmonary histiocytosis X). The location and the intensity of lesions were taken into consideration. Clinical and histopathological findings were compared. HRCT features of interstitial lung diseases such as nodules and cystic spaces in hypersensitivity pneumonitis and pulmonary histiocytosis, and ground-glass opacities in idiopathic interstitial pneumonias (IIP) were statistically significant for differential diagnosis in ILD cases. Combination of honeycombing and ground-glass opacities found in UIP and nodules found in DIP were also statistically significant features in IIP subtypes diagnosis. In some cases, HRCT patterns of hypersensitivity pneumonitis, pulmonary histiocytosis X and IPF combined with clinical findings allowed for the accurate diagnosis without resorting to lung biopsy. Within a group of idiopathic interstitial pneumonia only in usual interstitial pneumonia characteristic pattern in thin-section CT can be defined. In other subgroups some typical features can imply a diagnosis. (author)

Therapeutic activity of multiple common γ-chain cytokine inhibition in acute and chronic GVHD.

Science.gov (United States)

Hechinger, Anne-Kathrin; Smith, Benjamin A H; Flynn, Ryan; Hanke, Kathrin; McDonald-Hyman, Cameron; Taylor, Patricia A; Pfeifer, Dietmar; Hackanson, Björn; Leonhardt, Franziska; Prinz, Gabriele; Dierbach, Heide; Schmitt-Graeff, Annette; Kovarik, Jiri; Blazar, Bruce R; Zeiser, Robert

2015-01-15

The common γ chain (CD132) is a subunit of the interleukin (IL) receptors for IL-2, IL-4, IL-7, IL-9, IL-15, and IL-21. Because levels of several of these cytokines were shown to be increased in the serum of patients developing acute and chronic graft-versus-host disease (GVHD), we reasoned that inhibition of CD132 could have a profound effect on GVHD. We observed that anti-CD132 monoclonal antibody (mAb) reduced acute GVHD potently with respect to survival, production of tumor necrosis factor, interferon-γ, and IL-6, and GVHD histopathology. Anti-CD132 mAb afforded protection from GVHD partly via inhibition of granzyme B production in CD8 T cells, whereas exposure of CD8 T cells to IL-2, IL-7, IL-15, and IL-21 increased granzyme B production. Also, T cells exposed to anti-CD132 mAb displayed a more naive phenotype in microarray-based analyses and showed reduced Janus kinase 3 (JAK3) phosphorylation upon activation. Consistent with a role of JAK3 in GVHD, Jak3(-/-) T cells caused less severe GVHD. Additionally, anti-CD132 mAb treatment of established chronic GVHD reversed liver and lung fibrosis, and pulmonary dysfunction characteristic of bronchiolitis obliterans. We conclude that acute GVHD and chronic GVHD, caused by T cells activated by common γ-chain cytokines, each represent therapeutic targets for anti-CD132 mAb immunomodulation. © 2015 by The American Society of Hematology.

[Organising Pneumonia - a review and results from Icelandic studies].

Science.gov (United States)

Sveinsson, Olafur A; Isaksson, Helgi J; Gudmundsson, Gunnar

2008-01-01

Organising pneumonia (OP) is a relatively rare interstitial lung disease. It s definition is based on a characteristic histological pattern in the presence of certain clinical and radiological features. Organising pneumonia represents also what has been called Bronchiolitis Obliterans Organising Pneumonia (BOOP). Recently it has been recommended to call OP cryptogenic organising pneumonia (COP) when no definite cause or characteristic clinical context is found and secondary organising pneumonia (SOP) when causes can be identified such as infection or it occurs in a characteristic clinical context such as connective tissue disorder. The most common clinical symptoms are dyspnea, cough, fever and general malaise. It is common that symptoms have been present for some weeks before the diagnosis is made. Patients commonly have lowered PO2 and a mildly restrictive spirometry. Radiographic features are most often patchy bilateral airspace opacities but an interstitial pattern or focal opacities can also be seen. Most of patients respond well to steroids but relapses are quite common. The aim of this paper is to present an overview of the disease and the main results from studies on OP in Iceland. The mean annual incidence for OP in Iceland was 1.97/100,000 inhabitants. Annual incidence for COP was 1.10/100,000 and 0.87/100,000 for SOP. This is higher than in most other studies. In Iceland patients with OP had a higher standardized mortality ratio than the general population despite good clinical responses. No clinical symptoms could separate between SOP and COP.

CT of chronic infiltrative lung disease: Prevalence of mediastinal lymphadenopathy

Energy Technology Data Exchange (ETDEWEB)

Niimi, Hiroshi; Kang, Eun-Young; Kwong, S. [Univ. of British Columbia and Vancouver Hospital and Health Sciences Centre (Canada)] [and others

1996-03-01

Our goal was to determine the prevalence of mediastinal lymph node enlargement at CT in patients with diffuse infiltrative lung disease. The study was retrospective and included 175 consecutive patients with diffuse infiltrative lung diseases. Diagnoses included idiopathic pulmonary fibrosis (IPF) (n = 61), usual interstitial pneumonia associated with collagen vascular disease (CVD) (n = 20), idiopathic bronchiolitis obliterans organizing pneumonia (BOOP) (n = 22), extrinsic allergic alveolitis (EAA) (n = 17), and sarcoidosis (n = 55). Fifty-eight age-matched patients with CT of the chest performed for unrelated conditions served as controls. The presence, number, and sites of enlarged nodes (short axis {ge}10 mm in diameter) were recorded. Enlarged mediastinal nodes were present in 118 of 175 patients (67%) with infiltrative lung disease and 3 of 58 controls (5%) (p < 0.001). The prevalence of enlarged nodes was 84% (46 of 55) in sarcoidosis, 67% (41 of 61) in IPF, 70% (14 of 20) in CVD, 53% (9 of 17) in EAA, and 36% (8 of 22) in BOOP. The mean number of enlarged nodes was higher in sarcoidosis (mean 3.2) than in the other infiltrative diseases (mean 1.2) (p < 0.001). Enlarged nodes were most commonly present in station 10R, followed by 7, 4R, and 5. Patients with infiltrative lung disease frequently have enlarged mediastinal lymph nodes. However, in diseases other than sarcoid, usually only one or two nodes are enlarged and their maximal short axis diameter is <15 mm. 11 refs., 2 figs., 1 tab.

Amiodarone pulmonary toxicity: Case report

Directory of Open Access Journals (Sweden)

Vasić Nada

2014-01-01

Full Text Available Introduction. Amiodarone, an antiarrhythmic drug, which contains iodine compound, has a tendency to accumulate in some organs including the lungs. This is age, drug dosage and therapy duration dependent. Case Outline. We present a case of a 73-year-old man, a smoker, who was admitted as emergency case due to severe dyspnea, tachypnea with signs of cyanosis and respiratory insufficiency. Chest x-ray revealed bilateral diffuse pulmonary shadows in the middle and upper parts of the lungs, similar to those in tuberculosis. His illness history showed chronic obstructive pulmonary disease, arterial hypertension, and atrial fibrillation which has been treated with amiodarone for six years. Sputum smears were negative for mycobacteria, and by the diagnostic elimination method for specific, non-specific and malignant disease the diagnosis of amiodarone pulmonary toxicity was made. Fiberoptic bronchoscopy and pathohistological findings of bronchiolitis obliterans organizing pneumonia confirmed the diagnosis. As the first therapeutic approach, amiodarone therapy was stopped. Then, systemic therapy with methylprednisolone 21 (sodium succinate 40 mg i.v. daily during the first two weeks was initiated and continued with daily dose of methylprednisolone 30 mg orally during the next three months. The patient showed a marked subjective improvement during the first week, which was followed by the improvement of respiratory function and withdrawal of pulmonary changes with complete radiographic and CT resolution after eight months. Conclusion. Amiodarone pulmonary toxicity should be taken into consideration, especially in elderly patients with respiratory symptoms and pulmonary changes, even if only a low dose of amiodarone is administred over a longer time period.

Percutaneous Irreversible Electroporation Lung Ablation: Preliminary Results in a Porcine Model

International Nuclear Information System (INIS)

Deodhar, Ajita; Monette, Sébastien; Single, Gordon W.; Hamilton, William C.; Thornton, Raymond H.; Sofocleous, Constantinos T.; Maybody, Majid; Solomon, Stephen B.

2011-01-01

Objective: Irreversible electroporation (IRE) uses direct electrical pulses to create permanent “pores” in cell membranes to cause cell death. In contrast to conventional modalities, IRE has a nonthermal mechanism of action. Our objective was to study the histopathological and imaging features of IRE in normal swine lung. Materials and Methods: Eleven female swine were studied for hyperacute (8 h), acute (24 h), subacute (96 h), and chronic (3 week) effects of IRE ablation in lung. Paired unipolar IRE applicators were placed under computed tomography (CT) guidance. Some applicators were deliberately positioned near bronchovascular structures. IRE pulse delivery was synchronized with the cardiac rhythm only when ablation was performed within 2 cm of the heart. Contrast-enhanced CT scan was performed immediately before and after IRE and at 1 and 3 weeks after IRE ablation. Representative tissue was stained with hematoxylin and eosin for histopathology. Results: Twenty-five ablations were created: ten hyperacute, four acute, and three subacute ablations showed alveolar edema and necrosis with necrosis of bronchial, bronchiolar, and vascular epithelium. Bronchovascular architecture was maintained. Chronic ablations showed bronchiolitis obliterans and alveolar interstitial fibrosis. Immediate post-procedure CT images showed linear or patchy density along the applicator tract. At 1 week, there was consolidation that resolved partially or completely by 3 weeks. Pneumothorax requiring chest tube developed in two animals; no significant cardiac arrhythmias were noted. Conclusion: Our preliminary porcine study demonstrates the nonthermal and extracellular matrix sparing mechanism of action of IRE. IRE is a potential alternative to thermal ablative modalities.

Fanconi syndrome

Science.gov (United States)

De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome in ...

Bronchiolitis

Science.gov (United States)

... Kids and Teens Pregnancy and Childbirth Women Men Seniors Your Health Resources Healthcare Management End-of-Life Issues Insurance & Bills Self Care Working With Your Doctor Drugs, Procedures & Devices Over-the- ...

OCULO-CEREBRO-RENAL SYNDROME (LOWE'S SYNDROME)

Institute of Scientific and Technical Information of China (English)

无

1991-01-01

Oculo-cerebro-renal syndrome (Lowe's syndrome) is characterized by mental and motor retardation, cataract, glaucoma and renal abnormalities. It is an X-linked recessive metabolic disease. Two brothers suffering from Lowe's syndrome are reported. Their mother with lenticular opacities and peculiar facial appearance is in concordance with the obligate carrier. The ocular changes and heridity are discussed.

Neurofibromatosis-Noonan syndrome or LEOPARD Syndrome? A clinical dilemma.

Directory of Open Access Journals (Sweden)

Tullu M

2000-04-01

Full Text Available Neurofibromatosis (NF, Noonan syndrome (NS, and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the ′Neurofibromatosis-Noonan syndrome′ (NF-NS. The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported.

LEOPARD syndrome is not linked to the Marfan syndrome and the Watson syndrome loci

Energy Technology Data Exchange (ETDEWEB)

Rass-Rothchild, A.: Abeliovitch, D.; Kornstein, A. [Tel Aviv Univ. (Israel)]|[Hebrew Univ., Jerusalem (Israel)

1994-09-01

The acronym LEOPARD stands for a syndromic association of Lentigines, Eletrocardiographic changes, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, Retardation of growth and sensorineural Deafness. Inheritance is autosomal dominant with high penetrance and variable expressivity. In 1990 Torok et al. reported on the association of LEOPARD and Marfan syndrome. In addition a clinical similarity (cardiac and cutaneous involvement) exists with the Watson syndrome (neurofibromatosis and pulmonic stenosis) which is linked to the marker D17S33 on chromosome 17. We studied possible linkage of LEOPARD syndrome to the Marfan syndrome locus on chromosome 15 (D15S1, MF13, and (TAAAA)n repeats) and to the NF-1 locus on chromosome 17 in a family with 9 cases of LEOPARD syndrome. Close linkage between LEOPARD syndrome and both the Marfan locus on chromosome 15 and the NF-1 locus on chromosome 17 was excluded (lod score <-2.0 through {theta} = 0.1).

Investigating the Role of the Post-transcriptional Gene Regulator MiR-24-3p in the Proliferation, Migration and Apoptosis of Human Arterial Smooth Muscle Cells in Arteriosclerosis Obliterans

Directory of Open Access Journals (Sweden)

Xiao-feng Zhu

2015-07-01

Full Text Available Aims: To explore the expression of miR-24-3p in human arteries with arteriosclerosis obliterans (ASO as well as the role of miR-24-3p in the pathogenesis of ASO. Methods: We used quantitative real-time PCR (qRT-PCR and in situ hybridization to monitor miR-24-3p expression in human arteries. To investigate the effect of miR-24-3p on human arterial smooth muscle cells (HASMCs, we applied cell counting and EdU assays to monitor proliferation and transwell and wound healing assays to investigate migration and flow cytometry to investigate apoptosis. Furthermore, we applied 3'-untranslated region (3'-UTR luciferase assays to investigate the role of miR-24-3p in targeting platelet-derived growth factor receptor B (PDGFRB and c-Myc. Results: MiR-24-3p was mainly located in the media of arteries and was downregulated in ASO arteries compared with normal arteries. Platelet-derived growth factor BB (PDGF-BB treatment reduced the expression of miR-24-3p in primary cultured HASMCs. MiR-24-3p mimic oligos inhibited the proliferation and migration, and promotes apoptosis of HASMCs. Our 3'-UTR luciferase assays confirmed that PDGFRB and c-Myc were targets of miR-24-3p. Conclusion: The results suggest that miR-24-3p regulates the proliferation and migration of HASMCs by targeting PDGFRB and c-Myc. The PDGF/miR-24-3p/PDGFRB and PDGF/miR-24-3p/c-Myc pathways may play critical roles in the pathogenesis of ASO. These findings highlight the potential for new therapeutic targets for ASO.

Assessment of pathological changes associated with chronic allograft rejection and tolerance in two experimental models of rat lung transplantation.

Science.gov (United States)

Matsumura, Y; Marchevsky, A; Zuo, X J; Kass, R M; Matloff, J M; Jordan, S C

1995-06-15

Lung transplantation is now routinely performed for a wide range of end-stage cardiopulmonary disorders. Despite overcoming the problems associated with early acute rejection, chronic rejection (CR) in the form of obliterative bronchiolitis has emerged as the primary cause of late graft loss. The mechanisms involved in the development of CR of lung allografts are poorly understood, and no effective therapy is currently available. To better understand the pathological events associated with CR and tolerance, we examined two models of lung allograft rejection established in our laboratory. First, we exchanged left lung allografts between moderately histoincompatible inbred rat strains (WKY-->F344: n = 42 and F344-->WKY: n = 40). The WKY-->F344 model was previously shown to develop spontaneous tolerance, while the converse model (F344-->WKY) showed persistent acute rejection. The purpose of this investigation was to assess histopathological changes associated with long-term grafts left in place up to 140 days after transplant. To confirm that tolerance had developed, skin-grafting experiments were performed. Five skin grafts from each strain were placed on lung allograft recipients on day 35 after transplant and skin allograft survival was assessed and compared with controls. Acute rejection (AR) was graded histologically (stage O-IV) and the pathologic intensity of inflammation and CR were graded (0-4: 0 = 0%, 1 = 1-25%, 2 = 26-50%, 3 = 51-75%, and 4 = 76-100%) on percentage of involvement with the following categories being examined: (a) lymphocytic infiltration (perivascular, peribronchial, and peribronchiolar) and (b) vasculitis, edema, hemorrhage, and necrosis. Finally, chronic rejection was diagnosed by the presence of intimal hyperplasia, interstitial fibrosis, peribronchiolar fibrosis, bronchiolitis obliterans, and bronchiectasis. The WKY-->F344 animals showed progressive AR (stage III, day 21). Thereafter, the AR subsided spontaneously and was stage 0 on day

Metabolic syndrome and polycystic ovary syndrome: an intriguing overlapping.

Science.gov (United States)

Caserta, Donatella; Adducchio, Gloria; Picchia, Simona; Ralli, Eleonora; Matteucci, Eleonora; Moscarini, Massimo

2014-06-01

Metabolic syndrome is an increasing pathology in adults and in children, due to a parallel rise of obesity. Sedentary lifestyle, food habits, cultural influences and also a genetic predisposition can cause dyslipidemia, hypertension, abdominal obesity and insulin resistance which are the two main features of metabolic syndrome. Polycystic ovary syndrome (PCOS) is a condition directly associated with obesity, insulin resistance (HOMA index) and metabolic syndrome, and it is very interesting for its relationship and overlap with the metabolic syndrome. The relationship between the two syndromes is mutual: PCOS women have a higher prevalence of metabolic syndrome and also women with metabolic syndrome commonly present the reproductive/endocrine trait of PCOS. Prevention and treatment of metabolic syndrome and PCOS are similar for various aspects. It is necessary to treat excess adiposity and insulin resistance, with the overall goals of preventing cardiovascular disease and type 2 diabetes and improving reproductive failure in young women with PCOS. First of all, lifestyle changes, then pharmacological therapy, bariatric surgery and laparoscopic ovarian surgery represent the pillars for PCOS treatment.

Barber-Say syndrome and Ablepharon-Macrostomia syndrome: An overview

NARCIS (Netherlands)

de Maria, Beatrice; Mazzanti, Laura; Roche, Nathalie; Hennekam, Raoul C.

2016-01-01

Barber-Say syndrome (BSS) and Ablepharon-Macrostomia syndrome (AMS) are congenital malformation syndromes caused by heterozygous mutations in TWIST2. Here we provide a critical review of all patients published with these syndromes. We excluded several earlier reports due to misdiagnosis or

Autosomal dominant syndrome resembling Coffin-Siris syndrome.

Science.gov (United States)

Flynn, Maureen A; Milunsky, Jeff M

2006-06-15

Coffin-Siris syndrome is a multiple congenital anomaly/mental retardation syndrome with phenotypic variability [OMIM 135900]. The diagnosis is based solely on clinical findings, as there is currently no molecular, biochemical, or cytogenetic analysis available to confirm a diagnosis. Although typically described as an autosomal recessive disorder, autosomal dominant inheritance has also been infrequently reported. We describe a mother and her two daughters who all have features that resemble Coffin-Siris syndrome. However, this is not a completely convincing diagnosis given that hypertelorism is not a feature of Coffin-Siris syndrome and the family is relatively mildly affected. Yet, this family provides further evidence of an autosomal dominant mode of inheritance for a likely variant of Coffin-Siris syndrome (at least in some families). In addition, Sibling 1 had premature thelarche. She is the second reported individual within the spectrum of Coffin-Siris syndrome to have premature thelarche, indicating that it may be a rare clinical feature. Copyright 2006 Wiley-Liss, Inc.

West syndrome in a patient with Schinzel-Giedion syndrome.

Science.gov (United States)

Miyake, Fuyu; Kuroda, Yukiko; Naruto, Takuya; Ohashi, Ikuko; Takano, Kyoko; Kurosawa, Kenji

2015-06-01

Schinzel-Giedion syndrome is a rare recognizable malformation syndrome defined by characteristic facial features, profound developmental delay, severe growth failure, and multiple congenital anomalies. The causative gene of Schinzel-Giedion syndrome, SETBP1, has been identified, but limited cases have been confirmed by molecular analysis. We present a 9-month-old girl affected by West syndrome with Schinzel-Giedion syndrome. Congenital severe hydronephrosis, typical facial features, and multiple anomalies suggested a clinical diagnosis of Schinzel-Giedion syndrome. Hypsarrhythmia occurred at 7 months of age and was temporarily controlled by adrenocorticotropic hormone (ACTH) therapy during 5 weeks. SETBP1 mutational analysis showed the presence of a recurrent mutation, p.Ile871Thr. The implications in management of Schinzel-Giedion syndrome are discussed. © The Author(s) 2014.

Costo-efectividad de la radiografía de tórax en lactantes con sospecha clínica de bronquiolitis viral en Colombia Cost-effectiveness of chest x-rays in infants with clinically suspected viral bronchiolitis in Colombia

Directory of Open Access Journals (Sweden)

Carlos E. Rodríguez Martínez

2011-03-01

pacientes que tengan predictores de anormalidades radiológicas.OBJECTIVE: Estimate the cost-effectiveness of not taking chest x-rays of any infant with clinically suspected viral bronchiolitis versus routinely taking them of all such patients, the most common practice today in Colombia. METHODS: A cost-effectiveness study was conducted, comparing strategies of taking chest x-rays of all infants with clinically suspected viral bronchiolitis and not x-raying any of these infants. The principal outcome was the proportion of correct diagnoses. The time horizon was the clinical course of the bronchiolitis. The perspective was that of the third-party payer, and the costs were obtained from the rates in effect in a clinic in Bogotá. Deterministic and probabilistic sensitivity analyses were performed. RESULTS: The strategy of not taking a chest x-ray of any patient prevailed over that of routinely taking one in all cases, with an average cost of US$ 111.00 and a correct diagnosis rate of 0.8020, versus the respective values of US$ 129.00 and 0.7873 for the strategy of routinely x-raying all of these patients. The most influential variable was pneumonia-related hospital costs. In the probabilistic sensitivity analysis, the strategy of not x-raying any infant prevailed in 61.1% of the simulations. CONCLUSIONS: The results suggest that not taking routine chest x-rays of infants with clinically suspected viral bronchiolitis is a cost-effective strategy compared with the common practice of taking them in all cases, since the former yields a greater proportion of correct diagnoses at a lower average cost per patient. Nevertheless, new studies will be needed that have more representative samples from all of the health facilities and include the strategy of taking chest x-rays only of patients with predictors of radiologic abnormalities.

Cushing syndrome

Science.gov (United States)

Hypercortisolism; Cortisol excess; Glucocorticoid excess - Cushing syndrome ... The most common cause of Cushing syndrome is taking too much ... Cushing syndrome . Prednisone, dexamethasone, and prednisolone ...

Russell-Silver syndrome

Science.gov (United States)

Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome ... One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11. Most of the time, it ...

[Cockett's syndrome, May-Thurner syndrome, or iliac vein compression syndrome].

Science.gov (United States)

Gil Martín, A R; Carreras Aja, M; Arrieta Ardieta, I; Labayen Azparren, I

2014-01-01

Iliac vein compression syndrome (also known as May-Thurner syndrome or Cockett's syndrome) is a rare clinical entity in which the left common iliac vein is compressed when it passes between the right common iliac artery and the spine. The sustained compression and trauma caused by the pulsatile force of the artery on the vein damage the intima and lead to the formation of membranes or bands in the vascular lumen that hinder or obstruct the flow of blood in the vein, favoring thrombus formation. The current treatment strategy of choice is endovascular vein patch angioplasty and stenting with the aim of improving the caliber of the lumen and enabling normal venous drainage. We present two cases of May-Thurner syndrome and review the clinical and CT findings. Copyright © 2011 SERAM. Published by Elsevier Espana. All rights reserved.

LEOPARD syndrome

Science.gov (United States)

Multiple lentigines syndrome; Noonan syndrome with multiple lentigines ... Genetics Home Reference -- ghr.nlm.nih.gov/condition/noonan-syndrome-with-multiple-lentigines National Organization for Rare Disorders -- ...

Dravets syndrom

DEFF Research Database (Denmark)

Hansen, Lars Kjaersgård; Rasmussen, Niels Henrik; Ousager, Lilian Bomme

2010-01-01

Dravet syndrome is an epileptic syndrome of infancy and early childhood. Most cases of Dravet syndrome seem to be due to a genetic defect causing the sodium channel to malfunction. We describe the main features of the syndrome. This epilepsy is medically intractable, but we call attention...... to the fact that some medications are of benefit and some could exacerbate the condition. Early recognition of the syndrome including by genetic testing could possibly improve outcome and reduce the need for other specialized investigations. Udgivelsesdato: 2010-Feb-22...

A Chinese patient with pusher syndrome and unilateral spatial neglect syndrome.

Science.gov (United States)

Chen, Xiao-Wei; Lin, Cheng-He; Zheng, Hua; Lin, Zhen-Lan

2014-07-01

To observe clinical manifestations, behavioral characteristics, and effects of rehabilitation on a patient with pusher syndrome and unilateral spatial neglect caused by right thalamic hemorrhage. Assessment of pusher syndrome was made by the Scale for Contraversive pushing (SCP), and unilateral spatial neglect syndrome was diagnosed using line cancellation, letter and star cancellation, line bisection tests and copy and continuation of graphic sequence test. Behavioral therapy, occupational therapy, reading training and traditional Chinese medicine methods were adopted for treatment of pusher syndrome and unilateral spatial neglect. The patient showed typical pusher syndrome and unilateral spatial neglect symptoms. The pusher syndrome and unilateral spatial neglect symptoms were significantly improved following rehabilitation treatments. Pusher syndrome and unilateral spatial neglect syndrome occurred simultaneously after right thalamic hemorrhage. Early rehabilitation therapy can reduce the symptoms of pusher syndrome and unilateral spatial neglect syndrome and improve motor function.

Aarskog syndrome

Science.gov (United States)

Aarskog disease; Aarskog-Scott syndrome; AAS; Faciodigitogenital syndrome; Gaciogenital dysplasia ... Aarskog syndrome is a genetic disorder that is linked to the X chromosome. It affects mainly males, but females ...

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome).

Science.gov (United States)

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Ectrodactyly-ectodermal dysplasia clefting syndrome (EEC syndrome)

OpenAIRE

Koul, Monika; Dwivedi, Rahul; Upadhyay, Vinod

2014-01-01

Ectrodactyly-ectodermal dysplasia- clefting syndrome (also k/a. split hand- split foot malformation /split hand-split foot ectodermal dysplasia- cleft syndrome/ectodermal dysplasia cleft lip/cleft palate syndrome) a rare form of ectodermal dysplasia, is an autosomal dominant disorder inherited as a genetic trait and characterized by a triad of (i) ectrodactyly, (ii) ectodermal dysplasia and, (iii) & facial clefts.

Cushing's Syndrome

OpenAIRE

宗, 友厚; 伊藤, 勇; 諏訪, 哲也; 武田, 純; MUNE, Tomoatsu

2003-01-01

Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature.

Polycystic ovary syndrome and metabolic syndrome.

Science.gov (United States)

Ali, Aus Tariq

2015-08-01

Polycystic ovary syndrome (PCOS) is a heterogeneous disorder, where the main clinical features include menstrual irregularities, sub-fertility, hyperandrogenism, and hirsutism. The prevalence of PCOS depends on ethnicity, environmental and genetic factors, as well as the criteria used to define it. On the other hand, metabolic syndrome is a constellation of metabolic disorders which include mainly abdominal obesity, insulin resistance, impaired glucose metabolism, hypertension and dyslipidaemia. These associated disorders directly increase the risk of Type 2 diabetes mellitus (DMT2), coronary heart disease (CHD), cardiovascular diseases (CVD) and endometrial cancer. Many patients with PCOS have features of metabolic syndrome such as visceral obesity, hyperinsulinaemia and insulin resistance. These place patients with PCOS under high risk of developing cardiovascular disease (CVD), Type 2 diabetes (DMT2) and gynecological cancer, in particular, endometrial cancer. Metabolic syndrome is also increased in infertile women with PCOS. The aim of this review is to provide clear and up to date information about PCOS and its relationship with metabolic syndrome, and the possible interaction between different metabolic disorders.

Tourette syndrome

Science.gov (United States)

Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. ...

Metabolic syndrome in acute coronary syndrome

International Nuclear Information System (INIS)

Bhalli, M.A.; Aamir, M.; Mustafa, G.

2011-01-01

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Metabolic syndrome in acute coronary syndrome

Energy Technology Data Exchange (ETDEWEB)

Bhalli, M A; Aamir, M; Mustafa, G [Combined Military Hospital, Abbottabad (Pakistan)

2011-06-15

Objective: To determine the frequency of metabolic syndrome in male patients presenting with acute coronary syndrome Study design: A Descriptive study Place and duration of study: Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from October 2007 to September 2008 Patients and Methods: Male patients with acute coronary syndrome (ACS) were included. Patients having angioplasty (PCI), coronary artery bypass surgery in the past and other co-morbid diseases were excluded. All patients were assessed for the presence of five components of metabolic syndrome including hypertension, HDL-Cholesterol and triglycerides, glucose intolerance and abdominal obesity. Systolic, diastolic blood pressures, waist circumference (WC) and body mass index (BMI) were measured. ECG, cardiac enzymes, fasting glucose and lipid profile were also done. Results: A total of 135 male patients of ACS were studied with a mean age of 54.26 +- 11 years. Metabolic syndrome (MS) was present in 55 (40.7%) patients. MS with all five components was documented in 4 (7.27%) while MS with four and three components was seen in 23 (41.81%) and 28 (50.90%) patients respectively. Only 24 (43.63%) patients with MS had diabetes mellitus, remaining 31(56.36%) were non diabetic. Frequencies of diabetes, hypertension and family history of CAD were significantly higher (p<0.05) in patients with metabolic syndrome as compared to patients with normal metabolic status. Conclusion: Metabolic syndrome is fairly common and important risk factor in patients of IHD. Other risk factors like smoking, dyslipidemia, hypertension and diabetes were also frequently found. Public awareness to control the risk factors can reduce the prevalence of CAD in our country. (author)

Exogenous Cushing syndrome

Science.gov (United States)

Cushing syndrome - corticosteroid induced; Corticosteroid-induced Cushing syndrome; Iatrogenic Cushing syndrome ... Cushing syndrome is a disorder that occurs when your body has a higher than normal level of the hormone ...

Targets to treat metabolic syndrome in polycystic ovary syndrome.

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2015-01-01

Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics.

Targets to treat metabolic syndrome in polycystic ovary syndrome

Science.gov (United States)

Mahalingaiah, Shruthi; Diamanti-Kandarakis, Evanthia

2016-01-01

Introduction Metabolic syndrome is comprised of a combination of the following states: increased insulin resistance, dyslipidemia, cardiovascular disease, and increased abdominal obesity. Women with polycystic ovary syndrome (PCOS) have an increased risk of developing metabolic syndrome over the course of their lives. Metabolic syndrome increases risk of major cardiovascular events, morbidity, quality of life, and overall health care costs. Though metabolic syndrome in women with PCOS is an area of great concern, there is no effective individual medical therapeutic to adequately treat this issue. Areas Covered This article will review key aspects of metabolic syndrome in PCOS. We will discuss classic and novel therapeutics to address metabolic syndrome in women with PCOS. We will conclude with the importance of developing strategic interventions to increase the compliance to lifestyle and dietary modification, in addition to appreciation of the emerging pharmaceutical therapeutics available. Expert Opinion Innovation in lifestyle modification, including diet, exercise, with and without dedicated stress reduction techniques is the future in treatment of metabolic syndrome in PCOS. Application of novel interventions, such as group medical care, may improve future adherence to lifestyle modification recommendations, in addition to or in combination with pharmaceutical therapeutics. PMID:26488852

Concurrent Van der Woude syndrome and Turner syndrome: A case report.

Science.gov (United States)

Los, Evan; Baines, Hayley; Guttmann-Bauman, Ines

2017-01-01

Most cases of Van der Woude syndrome are caused by a mutation to interferon regulatory factor 6 on chromosome 1. Turner syndrome is caused by complete or partial absence of the second sex chromosome in girls. We describe a unique case of the two syndromes occurring concurrently though apparently independently in a girl with Van der Woude syndrome diagnosed at birth and Turner syndrome at 14 years 9 months. Short stature was initially misattributed to Van der Woude syndrome and pituitary insufficiency associated with clefts before correctly diagnosing Turner syndrome. We discuss the prevalence of delayed diagnosis of Turner syndrome, the rarity of reports of concurrent autosomal chromosome mutation and sex chromosome deletion, as well as the need to consider the diagnosis of Turner syndrome in all girls with short stature regardless of prior medical history.

Milk-alkali syndrome

Science.gov (United States)

Calcium-alkali syndrome; Cope syndrome; Burnett syndrome; Hypercalcemia; Calcium metabolism disorder ... Milk-alkali syndrome is almost always caused by taking too many calcium supplements, usually in the form of calcium carbonate. Calcium ...

Marfan Syndrome

Science.gov (United States)

Marfan syndrome is a disorder that affects connective tissue. Connective tissues are proteins that support skin, bones, blood vessels, ... A problem with the fibrillin gene causes Marfan syndrome. Marfan syndrome can be mild to severe, and ...

Brief Report: Repetitive Behaviour Profiles in Williams Syndrome: Cross Syndrome Comparisons with Prader-Willi and Down Syndromes

Science.gov (United States)

Royston, R.; Oliver, C.; Moss, J.; Adams, D.; Berg, K.; Burbidge, C.; Howlin, P.; Nelson, L.; Stinton, C.; Waite, J.

2018-01-01

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were…

Loeys-Dietz Syndrome

Science.gov (United States)

... to the signs and symptoms of Loeys-Dietz syndrome. Marfan syndrome is different from Loeys-Dietz syndrome in that the gene mutation which causes Marfan syndrome is in fibrillin-1 (FBN-1), a protein ...

Lipid raft facilitated ligation of K-{alpha}1-tubulin by specific antibodies on epithelial cells: Role in pathogenesis of chronic rejection following human lung transplantation

Energy Technology Data Exchange (ETDEWEB)

Tiriveedhi, Venkataswarup; Angaswamy, Nataraju [Department of Surgery, Pathology and Immunology, Washington University School of Medicine, St. Louis, MO (United States); Weber, Joseph [Department of Medicine, Washington University School of Medicine, St. Louis, MO (United States); Mohanakumar, T., E-mail: kumart@wustl.edu [Department of Surgery, Pathology and Immunology, Washington University School of Medicine, St. Louis, MO (United States)

2010-08-20

Research highlights: {yields} Addition of KAT Abs (+) sera to NHBE culture causes upregulation of growth factors. {yields} Cholesterol depletion causes down regulation of growth factor expression. {yields} Cholesterol depletion is accompanied by loss of membrane bound caveolin. {yields} Thus, we demonstrate lipid raft are critical for efficient ligation of the KAT Abs. -- Abstract: Long term function of human lung allografts is hindered by development of chronic rejection manifested as Bronchiolitis Obliterans Syndrome (BOS). We have previously identified the development of antibodies (Abs) following lung transplantation to K-{alpha}1-tubulin (KAT), an epithelial surface gap junction cytoskeletal protein, in patients who develop BOS. However, the biochemical and molecular basis of the interactions and signaling cascades mediated by KAT Abs are yet to be defined. In this report, we investigated the biophysical basis of the epithelial cell membrane surface interaction between KAT and its specific Abs. Towards this, we analyzed the role of the lipid raft-domains in the membrane interactions which lead to cell signaling and ultimately increased growth factor expression. Normal human bronchial epithelial (NHBE) cells, upon specific ligation with Abs to KAT obtained either from the serum of BOS(+) patients or monoclonal KAT Abs, resulted in upregulation of growth factors VEGF, PDGF, and bFGF (6.4 {+-} 1.1-, 3.2 {+-} 0.9-, and 3.4 {+-} 1.1-fold increase, respectively) all of which are important in the pathogenesis of BOS. To define the role for lipid raft in augmenting surface interactions, we analyzed the changes in the growth factor expression pattern upon depletion and enrichment with lipid raft following the ligation of the epithelial cell membranes with Abs specific for KAT. NHBE cells cultured in the presence of {beta}-methyl cyclodextran ({beta}MCD) had significantly reduced growth factor expression (1.3 {+-} 0.3, vs {beta}MCD untreated being 6.4 {+-} 1.1-fold

Lipid raft facilitated ligation of K-α1-tubulin by specific antibodies on epithelial cells: Role in pathogenesis of chronic rejection following human lung transplantation

International Nuclear Information System (INIS)

Tiriveedhi, Venkataswarup; Angaswamy, Nataraju; Weber, Joseph; Mohanakumar, T.

2010-01-01

Research highlights: → Addition of KAT Abs (+) sera to NHBE culture causes upregulation of growth factors. → Cholesterol depletion causes down regulation of growth factor expression. → Cholesterol depletion is accompanied by loss of membrane bound caveolin. → Thus, we demonstrate lipid raft are critical for efficient ligation of the KAT Abs. -- Abstract: Long term function of human lung allografts is hindered by development of chronic rejection manifested as Bronchiolitis Obliterans Syndrome (BOS). We have previously identified the development of antibodies (Abs) following lung transplantation to K-α1-tubulin (KAT), an epithelial surface gap junction cytoskeletal protein, in patients who develop BOS. However, the biochemical and molecular basis of the interactions and signaling cascades mediated by KAT Abs are yet to be defined. In this report, we investigated the biophysical basis of the epithelial cell membrane surface interaction between KAT and its specific Abs. Towards this, we analyzed the role of the lipid raft-domains in the membrane interactions which lead to cell signaling and ultimately increased growth factor expression. Normal human bronchial epithelial (NHBE) cells, upon specific ligation with Abs to KAT obtained either from the serum of BOS(+) patients or monoclonal KAT Abs, resulted in upregulation of growth factors VEGF, PDGF, and bFGF (6.4 ± 1.1-, 3.2 ± 0.9-, and 3.4 ± 1.1-fold increase, respectively) all of which are important in the pathogenesis of BOS. To define the role for lipid raft in augmenting surface interactions, we analyzed the changes in the growth factor expression pattern upon depletion and enrichment with lipid raft following the ligation of the epithelial cell membranes with Abs specific for KAT. NHBE cells cultured in the presence of β-methyl cyclodextran (βMCD) had significantly reduced growth factor expression (1.3 ± 0.3, vs βMCD untreated being 6.4 ± 1.1-fold increase) upon stimulation with KAT Abs. Depletion

Efficacy of total lymphoid irradiation for chronic allograft rejection following bilateral lung transplantation

International Nuclear Information System (INIS)

Diamond, David A.; Michalski, Jeff M.; Lynch, John P.; Trulock, Elbert P.

1998-01-01

Purpose: To assess the safety and efficacy of total lymphoid irradiation (TLI) in patients experiencing chronic rejection following bilateral lung transplantation (BLT). Patients and Materials: Eleven patients received TLI for chronic allograft rejection (bronchiolitis obliterans syndrome) refractory to conventional treatment modalities. Radiation therapy (RT) was prescribed as 8 Gy delivered in 10 0.8-Gy fractions, 2 fractions/week, via mantle, paraaortic, and inverted-Y fields. Serial pre- and post-RT pulmonary function values, complete blood counts, and immunosuppressive augmentation requirements [use of methylprednisolone, murine anti-human mature T-cell monoclonal antibody (OKT3), polyclonal antithymocyte globulin (ATG), and tacrolimus] were monitored. Results: In the 3 months preceding TLI, the average decrease in forced expiratory volume in 1 s (FEV 1 ) was 34% (range 0-75%) and the median number of immunosuppression augmentations was 3 (range 0-5). Only 4 of 11 patients completed all 10 TLI treatment fractions. Reasons for discontinuation included progressive pulmonary decline (four patients), worsening pulmonary infection (two patients), and persistent thrombocytopenia (one patient). Seven of the 11 patients failed within 8 weeks of treatment cessation. One patient had unabated rejection and received bilateral living related-donor transplants; he is alive and well. Six patients died. Two of these deaths were due to pulmonary infection from organisms isolated prior to the start of RT; the other four deaths were from progressive pulmonary decline. The four remaining patients had durable positive responses to TLI (mean follow-up of 47 weeks; range 24-72). Comparing the 3 months preceding RT to the 3 months following treatment, these four patients had improvements in average FEV 1 (40% decline vs. 1% improvement) and fewer median number of immunosuppressive augmentations (3.5 vs. 0). None of these patients has developed lymphoproliferative disease or has died

Pre-Menstrual Syndrome in Women with Down Syndrome

Science.gov (United States)

Mason, Linda; Cunningham, Cliff

2009-01-01

Background: Prevalence of pre-menstrual syndrome (PMS) may be higher in women with Down syndrome due to syndrome specific characteristics in biochemistry, psychopathology and lifestyle. Recognition of PMS may be difficult for women with intellectual disabilities and their carers. Method: A daily diary, used to diagnose PMS with typical women, was…

Williams syndrome

Science.gov (United States)

Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. It may be passed down in families. ... history of the condition. However, people with Williams syndrome have a 50% chance of passing the disorder ...

Lung disease with chronic obstruction in opium smokers in Singapore

Science.gov (United States)

Da Costa, J. L.; Tock, E. P. C.; Boey, H. K.

1971-01-01

Fifty-four opium smokers with chronic obstructive lung disease were studied for two-and-a-half years. Forty-eight patients had a cough for at least two years before the onset of inappropriate exertional dyspnoea. Fine, bubbling adventitious sounds suggesting small airway disease were heard on auscultation over the middle and lower lobes in 38 patients. The prevalence of inflammatory lung disease and chronic respiratory failure in this series is suggested as the main cause for the frequent finding of right ventricular hypertrophy and congestive heart failure. Physiological studies revealed moderate to severe airways obstruction with gross over-inflation and, in 32 patients, an additional restrictive defect probably due to peribronchiolar fibrosis. Radiological evidence of chronic bronchitis and bronchiolitis was observed in 45 patients, `pure' chronic bronchiolitis in six patients, and `widespread' emphysema in 25 patients respectively. Necropsy examinations in nine patients, however, showed destructive emphysema of variable severity in all. Chronic bronchiolitis often associated with striking bronchiolectasis was present in six cases. More severe bronchiolar rather than bronchial inflammation was noted. The heavy opium smokers had characteristic nodular shadows on chest radiography, sometimes associated with a striking reticular pattern not seen in `pure' cigarette smokers. This was due to gross pigmented dust (presumably carbon) deposition in relation to blood vessels, lymphatics, and bronchioles, and also within the alveoli. It is speculated that the initial lesion is an acquired bronchiolitis. Opium smoking induces an irritative bronchopathy favouring repeated attacks of acute bronchiolitis and eventually resulting in obliterative bronchiolitis, peribronchiolar fibrosis, chronic bronchitis, and destructive emphysema. Images PMID:5134057

Coexistence of Reverse Capgras Syndrome, Subjective Double and Cotard Syndrome

Directory of Open Access Journals (Sweden)

Azadeh Mashayekhi

2016-01-01

Full Text Available Misidentification syndrome is a condition in which the person thinks that familiar persons have been replaced with other one. Coexistence of some types of this syndrome has been reported with other psychiatric syndromes. In this report, we present a 47-year-old married man with coexistence of reverse Capgras and subjective double syndromes with Cotard syndrome. There is no previous report of coexistence of these three forms of delusions in a single case.

Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome).

Science.gov (United States)

Bresler, Scott C; Padwa, Bonnie L; Granter, Scott R

2016-06-01

Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

Aicardi Syndrome

Science.gov (United States)

... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) × Definition Aicardi syndrome is a rare genetic ... from Aicardi-Goutieres syndrome, which is an inherited encephalopathy that affects newborn infants.) View Full Definition Treatment There is no ...

Fournier gangrene associated with hyper IgE syndrome (Job syndrome).

Science.gov (United States)

Hori, Junichi; Yamaguchi, Satoshi; Watanabe, Masaki; Osanai, Hiroaki; Hori, Masako

2008-04-01

We report a case of a 32-year-old man with hyper IgE syndrome (Job syndrome) who developed Fournier gangrene due to infectious multiple atheromas of the scrotal skin that progressed to the right groin and thigh. The patient required surgical debridement and subsequent skin grafting. This is a rare case of Fournier gangrene associated with hyper IgE syndrome (Job syndrome). When a patient without diabetes mellitus has repeated infections and atopic-like dermatitis, Job syndrome should be considered.

Down Syndrome

Science.gov (United States)

... Down syndrome increases as a woman gets older. Down syndrome cannot be cured. Early treatment programs can help improve skills. They may include ... occupational, and/or educational therapy. With support and treatment, many ... Down syndrome live happy, productive lives. NIH: National Institute of ...

TAFRO Syndrome.

Science.gov (United States)

Igawa, Takuro; Sato, Yasuharu

2018-02-01

TAFRO syndrome is a newly recognized variant of idiopathic multicentric Castleman disease (iMCD) that involves a constellation of syndromes: thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O). Thrombocytopenia and severe anasarca accompanied by relatively low serum immunoglobulin levels are characteristic clinical findings of TAFRO syndrome that are not present in iMCD-not otherwise specified (iMCD-NOS). Lymph node biopsy is recommended to exclude other diseases and to diagnose TAFRO syndrome, which reveals characteristic histopathological findings similar to hyaline vascular-type CD. TAFRO syndrome follows a more aggressive course, compared with iMCD-NOS, and there is no standard treatment. Copyright © 2017 Elsevier Inc. All rights reserved.

Development and characteristics of children with Usher syndrome and CHARGE syndrome.

Science.gov (United States)

Dammeyer, Jesper

2012-09-01

Individuals with Usher syndrome or CHARGE syndrome are faced with a number of difficulties concerning hearing, vision, balance, and language development. The aim of the study is to describe the developmental characteristics of children with Usher syndrome and CHARGE syndrome, respectively. Data about the developmental characteristics of 26 children with Usher syndrome and 17 children with CHARGE syndrome was obtained. Associations between deafblindness (dual sensory loss), motor development (age of walking), language abilities, and intellectual outcome of these children were explored for each group independently. Both groups of children face a number of difficulties associated with vision, hearing, language, balance and intellectual outcome. Intellectual disability and/or language delay was found among 42% of the children with Usher syndrome and among 82% of the children with CHARGE syndrome. Intellectual disability was associated with language delay and age of walking for both groups. Even though Usher and CHARGE are two different genetic syndromes, both groups are challenged with a number of similar developmental delays. Clinicians need to be aware of several developmental issues in order to offer adequate support to children with Usher or CHARGE syndrome. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

Pregnancy outcome in joint hypermobility syndrome and Ehlers-Danlos syndrome.

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Sundelin, Heléne E K; Stephansson, Olof; Johansson, Kari; Ludvigsson, Jonas F

2017-01-01

An increased risk of preterm birth in women with joint hypermobility syndrome or Ehlers-Danlos syndrome is suspected. In this nationwide cohort study from 1997 through 2011, women with either joint hypermobility syndrome or Ehlers-Danlos syndrome or both disorders were identified through the Swedish Patient Register, and linked to the Medical Birth Register. Thereby, 314 singleton births to women with joint hypermobility syndrome/Ehlers-Danlos syndrome before delivery were identified. These births were compared with 1 247 864 singleton births to women without a diagnosis of joint hypermobility syndrome/Ehlers-Danlos syndrome. We used logistic regression, adjusted for maternal age, smoking, parity, and year of birth, to calculate adjusted odds ratios for adverse pregnancy outcomes. Maternal joint hypermobility syndrome/Ehlers-Danlos syndrome was not associated with any of our outcomes: preterm birth (adjusted odds ratio = 0.6, 95% confidence interval 0.3-1.2), preterm premature rupture of membranes (adjusted odds ratio = 0.8; 95% confidence interval 0.3-2.2), cesarean section (adjusted odds ratio = 0.9, 95% confidence interval 0.7-1.2), stillbirth (adjusted odds ratio = 1.1, 95% confidence interval 0.2-7.9), low Apgar score (adjusted odds ratio = 1.6, 95% confidence interval 0.7-3.6), small for gestational age (adjusted odds ratio = 0.9, 95% confidence interval 0.4-1.8) or large for gestational age (adjusted odds ratio = 1.2, 95% confidence interval 0.6-2.1). Examining only women with Ehlers-Danlos syndrome (n = 62), we found a higher risk of induction of labor (adjusted odds ratio = 2.6; 95% confidence interval 1.4-4.6) and amniotomy (adjusted odds ratio = 3.8; 95% confidence interval 2.0-7.1). No excess risks for adverse pregnancy outcome were seen in joint hypermobility syndrome. Women with joint hypermobility syndrome/Ehlers-Danlos syndrome do not seem to be at increased risk of adverse pregnancy outcome. © 2016 Nordic Federation of

Wolf-Hirschhorn (4p-) syndrome with West syndrome.

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Motoi, Hirotaka; Okanishi, Tohru; Kanai, Sotaro; Yokota, Takuya; Yamazoe, Tomohiro; Nishimura, Mitsuyo; Fujimoto, Ayataka; Yamamoto, Takamichi; Enoki, Hideo

2016-01-01

Wolf-Hirschhorn syndrome (WHS) is a chromosome disorder (4p-syndrome) which is characterized by craniofacial features and epileptic seizures. Here, we report a case of WHS with West syndrome, in whom the seizures were refractory to several antiepileptic drugs but were responsive to the addition of lamotrigine. The patient had epileptic spasms at age seven months. The interictal electroencephalogram was hypsarrhythmic. After adding lamotrigine, seizures decreased remarkably, and spasms disappeared. We have identified and described the very rare case of a girl with WHS who also developed West syndrome. In this case, adding lamotrigine to her medications effectively treated the spasms.

Rowell syndrome

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Ramesh Y Bhat

2014-01-01

Full Text Available Rowell syndrome is a rare disease consisting of erythema multiforme-like lesions associated with lupus erythematosus. The syndrome occurs mostly in middle-aged women. The authors describe the syndrome in a 15-year-old boy who responded well to systemic steroids and hydroxychloroquine.

[Poland's syndrome].

Science.gov (United States)

Slezak, R; Sasiadek, M

2000-08-01

Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

[Gardner syndrome--parent alienation syndrome (PAS). Diagnosis or family reality?].

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Namysłowska, Irena; Heitzman, Janusz; Siewierska, Anna

2009-01-01

The authors present characteristics of Parental Alienation Syndrome (PAS) proposed by Gardner as well as data, which may help to differentiate that syndrome with real psychological, physical and sexual abuse. The consequences of Gardner Syndrome for legal decisions in the court cases of child custody and the critique of this syndrome in forensic and psychiatric literature are also discussed, and several questions posed. Authors propose to treat Gardner Syndrome not as as a child disorder but as a specific, dynamic family situation, which occurs sometimes, during divorce and fight about child custody.

Burning Mouth Syndrome and "Burning Mouth Syndrome".

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Rifkind, Jacob Bernard

2016-03-01

Burning mouth syndrome is distressing to both the patient and practitioner unable to determine the cause of the patient's symptoms. Burning mouth syndrome is a diagnosis of exclusion, which is used only after nutritional deficiencies, mucosal disease, fungal infections, hormonal disturbances and contact stomatitis have been ruled out. This article will explore the many causes and treatment of patients who present with a chief complaint of "my mouth burns," including symptomatic treatment for those with burning mouth syndrome.

Hyperlucent lung

International Nuclear Information System (INIS)

Jimenez-Gutierrez, Florana; Soto-Quiros, Manuel E.

2007-01-01

Unilateral hyperlucent lung is also known as Swyer-James Syndrome, Macleod Syndrome or lobular or unilateral emphysema. It is an uncommon disease characterized by lung or unilateral lobe hiperlucency associated to an air trapping upon expiration. As regards to etiology, this syndrome is considered to be an acquired disease that appears secondary to respiratory infections during the early years of life, probably bronchiolitis and/ or viral pneumonia. The clinical presentation varies among patients. Some of them are asymptomatic, others present a history of recurrent episodes of pulmonary infections from early years of life or present effort dyspnea. The diagnosis is usually made accidentally by a chest radiograph in a child with history of respiratory infections or in an adult during a routine chest x- ray in an asymptomatic person. It is important to differentiate this syndrome from other causes of unilateral pulmonary hiperlucency on conventional chest x-rays. Few cases of Swyer-James Syndrome in children have been reported, it is presented the clinical case of a patient who had a parainfluenza 3 bronchopneumonia when he was a month and eighteen days of age. The differential diagnosis of this syndrome should be done with other thoracic entities that diminish the radiological pulmonary unilateral density. A case of a child who is the bearer of hyperlucent lung is described. (author) [es

Marfan Syndrome (For Teens)

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... genetic disorder called Marfan syndrome. What Is Marfan Syndrome? Marfan syndrome is named after Antoine Marfan, the French ... immediately. What's Life Like for Teens With Marfan Syndrome? Marfan syndrome affects people differently, so life is not ...

Learning about Marfan Syndrome

Science.gov (United States)

... Additional Resources for Marfan Syndrome What is Marfan syndrome? Marfan syndrome is one of the most common inherited ... FAQ Top of page Additional Resources For Marfan Syndrome Marfan syndrome [nlm.nih.gov] From Medline Plus Marfan ...

Divorce in families of children with Down Syndrome or Rett Syndrome.

Science.gov (United States)

Lederman, Vivian Renne Gerber; Alves, Bianca dos Santos; Negrão, Juliana; Maria, Juliana Negrão; Schwartzman, José Salomão; D'Antino, Maria Eloisa Famá; Brunoni, Decio

2015-05-01

This study evaluates the impact in the stability and management of the marriage of parents of a child with Down or Rett Syndrome. Morbidity of the syndromes and the marital status of the couples before and after the birth of the affected children were considered variables. The divorce rate in families with Down syndrome was 10%, similar to the Brazilian rate population. In Rett Syndrome, the divorce rate was significantly higher, 23.5%. The higher morbidity of Rett Syndrome, and the moment of diagnosis could be relevant factors for the increased divorce rate related to this syndrome.

The sick-building syndrome; Das Sick-Building-Syndrom

Energy Technology Data Exchange (ETDEWEB)

Henne, A.; Neumann, H.F.; Winneke, G.

1992-12-31

The sick-building syndrome is characterized by the presence of general, non-specific symptoms (e.g., headache, tiredness, respiratory problems, eye trouble, vertigo, nausea, unspecific hypersensitivity) in association with a particular indoor ambience. It is clearly distinguishable from `building-related illness`, referring to a well-defined clinical syndrome due to staying in a building and for which a cause can, in general, be established. Disorders in the case of the sick-building syndrome are manifold and confirmed objectifiable results are hardly available so far. Yet there are some organ-related methods for the confirmation of findings concerning, for instance, the eyes, the skin and the area of the nose. The causes of the incidence of sick-building syndrome are more or less unclear. It is a multifactorial phenomenon involving physical, biological, chemical, individual-specific and psychological factors. Buildings where sick-building syndrome occurs typically exhibit certain properties. The European Community has already made proposals for the investigation of incriminated buildings. A systematic survey by questionnaire together with individual interviews plays an import part towards clarifying the syndrome. (orig./UWA) [Deutsch] Das Sick-Building-Syndrom beschreibt das Vorhandensein von allgemeinen, nicht spezifischen Symptomen (z.B. Kopfschmerzen, Muedigkeit, Atembeschwerden, Augenreizungen, Schwindelgefuehl, Uebelkeit, unspezifische Ueberempfindlichkeit), assoziiert mit einer besonderen Innenraumumgebung. Deutlich hiervon abzugrenzen ist die ``Building related illness``, bei der ein klinisch definiertes Krankheitsbild vorliegt, das durch den Aufenthalt im Gebaeude verursacht wird und fuer das im allgemeinen eine Ursache ermittelt werden kann. Das Beschwerdebild beim Sick-Building-Syndrom ist vielfaeltig, und gesicherte, objektivierbare Befunde liegen hierzu bisher kaum vor. Dennoch gibt es einige organbezogenen Methoden zur Befundabsicherung, z.B. fuer das

Hypokalaemia: Bartter's syndrome or pseudo-Bartter's syndrome?

OpenAIRE

Robb, J D; Delargy, M A; Nolan, M; Tomkin, G H

1984-01-01

The difficulties in the diagnosis of hypokalaemia are often considerable. This paper reports three patients who presented with hypokalaemia. Investigations are described which may help to distinguish Bartter's syndrome from pseudo-Bartter's syndrome.

Syndromes with supernumerary teeth.

Science.gov (United States)

Lubinsky, Mark; Kantaputra, Piranit Nik

2016-10-01

While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

What Is Usher Syndrome?

Science.gov (United States)

... Action You are here Home › Retinal Diseases Listen Usher Syndrome What is Usher syndrome? How is Usher syndrome ... available? Are there any related diseases? What is Usher Syndrome? Usher syndrome is an inherited condition characterized by ...

Epidemiology of adenovirus respiratory infections among hospitalized children in Seremban, Malaysia.

Science.gov (United States)

Foong Ng, Khuen; Kee Tan, Kah; Hong Ng, Boon; Nair, Pritiss; Ying Gan, Wan

2015-07-01

There is scarcity of data regarding epidemiology and clinical aspects of human adenovirus acute respiratory infection (ARI) among children in developing countries. Retrospective data on demographics, clinical presentation, outcomes and laboratory findings of 116 children admitted into Tuanku Jaafar Hospital in Seremban, Malaysia from 2012 to 2013 with documented diagnosis of community-acquired adenovirus ARI were collected and analyzed. Male to female ratio was 1.70. Median age was 14 (1-107) months. The commonest symptoms were fever (94.8%; 110/116), cough (82.8%, 96), rhinorrhea (63.8%; 74), interrupted feeding (66.4%; 77), diarrhea (33.6%; 39) and conjunctivitis (21.6%; 25). Mean temperature on admission was 38.4°C±0.9°C. Among all 116 subjects, 20.7% (24) needed oxygen supplementation, 57.8% (67) required intravenous hydration, 11.2% (13) were admitted into the pediatric intensive care unit and 6.9% (8) required mechanical ventilation. Only 1% (1/87) had positive blood culture (Streptococcus pneumoniae) among 87 who received antibiotic treatment. Case fatality rate was 2.6% (3/116) and 1.7% (2/116) developed bronchiolitis obliterans. Median length of hospital stay was 4 (1-50) days. Adenovirus ARI caused significant morbidity and substantial resource utilization among hospitalized Malaysian children. It should be considered in the differential diagnosis of infants below two years presenting with ARI associated with high fever. Antibiotics should not be prescribed as secondary bacterial infections are uncommon. © The Author 2015. Published by Oxford University Press on behalf of Royal Society of Tropical Medicine and Hygiene. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

The Role of Oxidative Stress in Severity of Obstructive Pulmonary Complications in Sputum of Sulfur Mustard-Injured Patients

Directory of Open Access Journals (Sweden)

Javad Heydari

2017-09-01

Full Text Available Background: Sulfur mustard (SM is a strong bifunctional alkylating agent that causes delayed complications in organs such as lung. Oxidative stress plays a pivotal role in the pathogenesis and progression of many pulmonary diseases. The aim of this study was to investigate the oxidative stress in sputum of SM exposed patients with mild, moderate and severe pulmonary dysfunction and assessing their relationship with pulmonary function. Methods: In this cross–sectional study, oxidative stress biomarkers in sputum were examined on 26 patients with SM-induced bronchiolitis obliterans (9 mild, 14 moderate and 3 severe and 12 matched healthy controls referred to Baqiyatallah Hospital, Tehran between October 2015 and April 2016. Results: Sputum superoxide dismutase, catalase and glutathione S-transferase activities and malondialdehyde level in moderate and severe groups were significantly higher than in the control group (P=0.002, P=0.004, P=0.014 and P=0.009, respectively. Glutathione (GSH level in moderate (22.29%, P=0.025 and severe (45.07%, P=0.004 groups were significantly lower than the control. A decreased in GSH level in severe (41.7% groups was observed as compared with the mild group. Pearson analysis revealed strong correlations between disease severity and oxidative stress biomarkers in sputum of patients with moderate and severe injuries. Conclusions: Oxidative stress is involved in the pathogenesis of patients with moderate and severe pulmonary dysfunction following SM exposure. The presence of enhanced oxidative stress relates to the decline lung function and the progression of the disease. Sputum induction in SM-injured patients can be used to the assessment of the antioxidant status of bronchial secretions.

Late-onset Radiologic Findings of Respiratory System Following Sulfur Mustard Exposure

Directory of Open Access Journals (Sweden)

Mahnaz Amini

2013-06-01

Full Text Available Background: Sulfur mustard (SM as a chemical warfare agent, increases permeability of bronchial vessels and damages airway epithelium. SM exposure causes debilitating respiratory complications. This study was designed to evaluate clinical respiratory manifestations, and to compare chest X ray (CXR and high resolution computed tomography (HRCT scan of chest in SM exposed patients with respiratory complaints. Methods:All patients with history of SM exposure who visited Imam Reza Specialized Clinic of Respiratory Diseases from September 2001 to March 2011 were included. Patients with other comorbidities which affect respiratory system were excluded. CXR and chest HRCT scan were performed on the same day and were repeated after 5 years. Clinical and radiologic findings were collected and were compared with each other. Results: In total, 62 male patients with mean age of 53 (6.9, 41-65 were studied. Dyspnea (61 cases; 100%, dry cough (40 cases; 66%, hemoptysis (21 cases; 35% and productive cough (20 cases; 33% were the most common respiratory manifestations. Pulmonary infiltration (51; 83%, pleural thickening (25; 40% and emphysema (16; 26% were the most common findings on CXR. According to HRCT scan, pulmonary infiltration (53; 85%, bronchiolitis obliterans (38; 61% and pleural thickening (36; 58% were the most common findings (Table 2. Repeated radiologic assessments after 5 years showed a few additional findings in HRCT scan, while in about one fifth of CXRs, new pathologic findings were found. Conclusion: Patients with SM exposure experience debilitating respiratory disorders in long term. Repeating CXR in patients who present with subjective symptoms may show new findings; however, repeating HRCT scan is probably not necessary.

Effects of Curcuminoids-Piperine Combination on Systemic Oxidative Stress, Clinical Symptoms and Quality of Life in Subjects with Chronic Pulmonary Complications Due to Sulfur Mustard: A Randomized Controlled Trial.

Science.gov (United States)

Panahi, Yunes; Ghanei, Mostafa; Hajhashemi, Ali; Sahebkar, Amirhossein

2016-01-01

Oxidative stress plays a key role in the development of chronic pulmonary complications of sulfur mustard (SM). Curcuminoids are polyphenols with documented safety and antioxidant activity. The present study aimed to investigate the efficacy of short-term supplementation with curcuminoids (co-administered with piperine to enhance the bioavailability of curcuminoids) in alleviating systemic oxidative stress and clinical symptoms, and improvement of health-related quality of life (HRQoL) in subjects suffering from chronic pulmonary complications due to SM exposure who are receiving standard respiratory treatments. Eighty-nine subjects were recruited to this randomized double-blind placebo-controlled trial, being randomly allocated to either curcuminoids (1500 mg/day) + piperine (15 mg/day) combination (n = 45) or placebo (n = 44) for a period of 4 weeks. High-resolution computed tomography suggested the diagnosis of bronchiolitis obliterans in all subjects. Efficacy measures were changes in serum levels of reduced glutathione (GSH) and malonedialdehyde (MDA). The severity and frequency of respiratory symptoms and HRQoL were also assessed using St. George respiratory Questionnaire (SGRQ) and COPD Assessment Test (CAT) indices. Serum levels of GSH were increased whilst those of MDA decreased by the end of trial in both groups. Likewise, there were significant improvements in the total as well as subscale (symptoms, activity and impact) SGRQ and CAT scores in both groups. However, comparison of magnitude of changes revealed a greater effect of curcuminoids-piperine combination compared to placebo in elevating GSH, reducing MDA and improving CAT and SGRQ (total and subscale) scores (p stress, clinical symptoms and HRQoL, these phytochemicals may be used as safe adjuvants in patients suffering from chronic SM-induced pulmonary complications who are receiving standard treatments.

Autoimmune/inflammatory syndrome induced by adjuvants (Shoenfeld's syndrome) - An update.

Science.gov (United States)

Watad, A; Quaresma, M; Brown, S; Cohen Tervaert, J W; Rodríguez-Pint, I; Cervera, R; Perricone, C; Shoenfeld, Y

2017-06-01

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been widely described in many studies conducted thus far. The syndrome incorporates five immune-mediated conditions, all associated with previous exposure to various agents such as vaccines, silicone implants and several others. The emergence of ASIA syndrome is associated with individual genetic predisposition, for instance those carrying HLA-DRB1*01 or HLA-DRB4 and results from exposure to external or endogenous factors triggering autoimmunity. Such factors have been demonstrated as able to induce autoimmunity in both animal models and humans via a variety of proposed mechanisms. In recent years, physicians have become more aware of the existence of ASIA syndrome and the relationship between adjuvants exposure and autoimmunity and more cases are being reported. Accordingly, we have created a registry that includes at present more than 300 ASIA syndrome cases that have been reported by different physicians worldwide, describing various autoimmune conditions induced by diverse adjuvants. In this review, we have summarized the updated literature on ASIA syndrome and the knowledge accumulated since 2013 in order to elucidate the association between the exposure to various adjuvant agents and its possible clinical manifestations. Furthermore, we especially referred to the relationship between ASIA syndrome and systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS).

Toxic shock syndrome

Science.gov (United States)

Staphylococcal toxic shock syndrome; Toxic shock-like syndrome; TSLS ... Toxic shock syndrome is caused by a toxin produced by some types of staphylococcus bacteria. A similar problem, called toxic shock- ...

Eosinophilic leukocytoclastic vasculitis - a spectrum ranging from Wells' syndrome to Churg-Strauss syndrome?

Science.gov (United States)

Ratzinger, Gudrun; Zankl, Julia; Eisendle, Klaus; Zelger, Bernhard

2014-01-01

Wells' syndrome is defined as an inflammatory disorder with the histopathological presence of eosinophilic infiltrates and flame figures in the absence of vasculitis. Eosinophilic leukocytoclastic vasculitis shows eosinophilic infiltrates in combination with vasculitic changes. And Churg Strauss Syndrome comprises all three characteristics - eosinophilic infiltrates, vasculitis and flame figures. To determine whether these three diseases are distinct entities or different manifestations of a similar clinicopathologic process. Histopathological samples and clinical courses of 17 patients with eosinophilic infiltrates, flame figures and clinical features of Wells' syndrome were re-evaluated. Histopathologically, we focused on the presence or absence of vasculitic features. Clinically, we included only patients who were diagnosed with Wells' syndrome at least once in the course of their disease. 4 patients were finally diagnosed with Wells' syndrome, 5 with eosinophilic leukocytoclastic vasculitis and 6 with Churg Strauss syndrome. Further, we had one case of an overlap between Wells' syndrome and eosinophilic vasculitis and one case of Wegener granulomatosis. Vasculitic features were found in the samples of all patients. Histologically, we find vasculitic features in typical presentations of Wells' syndrome. Clinically, we find typical features of Wells' syndrome in patients finally diagnosed with eosinophilic leukocytoclastic vasculitis or Churg Strauss syndrome. Furthermore, we have observed and formerly reported 3 patients with progression from Wells' syndrome to Churg Strauss syndrome. Thus, we assume that eosinophilic leukocytoclastic vasculitis might form a bridge between Wells' syndrome and Churg Strauss syndrome.

Management of moyamoya syndrome in patients with Noonan syndrome.

Science.gov (United States)

Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

2016-06-01

A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. Copyright © 2015 Elsevier Ltd. All rights reserved.

Obstructive sleep apnoea/hypopnoea syndrome in adults with Down syndrome

OpenAIRE

Hill, Elizabeth A.

2016-01-01

Key points Adults with Down syndrome are predisposed to obstructive sleep apnoea/hypopnoea syndrome (OSAHS) due to overlap between the Down syndrome phenotype and OSAHS risk factors. The prevalence of OSAHS in adults with Down syndrome is estimated at 35?42%. This is up to ten-times higher than in the general adult population. Symptoms of OSAHS, including behavioural and emotional disturbances as well as standard symptoms such as sleepiness, should be monitored as part of regular health surve...

What is Metabolic Syndrome?

Science.gov (United States)

... Intramural Research Home / Metabolic Syndrome Metabolic Syndrome Also known as What Is Metabolic syndrome ... metabolic risk factors to be diagnosed with metabolic syndrome. Metabolic Risk Factors A Large Waistline Having a large ...

Prune belly syndrome

Science.gov (United States)

Eagle-Barrett syndrome; Triad syndrome ... The exact causes of prune belly syndrome are unknown. The condition affects mostly boys. While in the womb, the developing baby's abdomen swells with fluid. Often, the cause is ...

Medline Plus

Full Text Available Air first enters the body through the mouth or nose, quickly moves to the pharynx (throat), passes ... called bronchioles. The smallest bronchioles end in tiny air sacs, called alveoli, which inflate during inhalation, and ...

Gorlin-goltz syndrome

Directory of Open Access Journals (Sweden)

B V Shobha

2011-01-01

Full Text Available Gorlin-Goltz syndrome also known as nevoid basal cell carcinoma syndrome (NBCCS is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by keratocystic odontogenic tumors (KCOT in the jaw, multiple basal cell carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic examination in the first decade of life, as KCOTs are usually one of the first manifestations of the NBCCS syndrome. This article reports the case of a 12-year-old girl with Gorlin-Goltz syndrome, emphasizing its clinical and radiographic manifestation. This study highlights the importance of health professionals in the early diagnosis of this syndrome and a multidisciplinary approach to provide a better diagnosis and prognosis.

Refeeding syndrome

OpenAIRE

Tripathy, Swagata; Mishra, Padmini; Dash, S. C.

2008-01-01

Refeeding syndrome is a potentially fatal medical condition that may affect malnourished patients in response to an inappropriately rapid overfeeding. This commonly occurs following the institution of nutritional support, especially parenteral or enteral nutrition. The most characteristic pathophysiology of refeeding syndrome relates to the rapid consumption of phosphate after glucose intake and subsequent hypophosphatemia. Refeeding syndrome can manifest as either metabolic changes （hypokala...

Nevoid basal cell carcinoma syndrome

Science.gov (United States)

NBCC syndrome; Gorlin-Goltz syndrome; Basal cell nevus syndrome; BCNS; Basal cell cancer - nevoid basal cell carcinoma syndrome ... Nevoid basal cell carcinoma nevus syndrome is a rare genetic ... syndrome is known as PTCH ("patched"). The gene is passed down ...

The wellness syndrome

DEFF Research Database (Denmark)

Mik-Meyer, Nanna

2015-01-01

Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.).......Klumme. Wellness er blevet et syndrom, og dets symptomer er angst, selvbebrejdelser og skyldfølelse. Kommentar med udgangspunkt i: Carl Cederström & Andre Spicer, "The Wellness Syndrome" (Polity Books, 2015. 200 p.)....

Structural and functional localization of airway effects from episodic exposure of infant monkeys to allergen and/or ozone

International Nuclear Information System (INIS)

Joad, Jesse P.; Kott, Kayleen S.; Bric, John M.; Peake, Janice L.; Plopper, Charles G.; Schelegle, Edward S.; Gershwin, Laurel J.; Pinkerton, Kent E.

2006-01-01

Both allergen and ozone exposure increase asthma symptoms and airway responsiveness in children. Little is known about how these inhalants may differentially modify airway responsiveness in large proximal as compared to small distal airways. We evaluated whether bronchi and respiratory bronchioles from infant monkeys exposed episodically to allergen and/or ozone differentially develop intrinsic hyperresponsiveness to methacholine and whether eosinophils and/or pulmonary neuroendocrine cells play a role. Infant monkeys were exposed episodically for 5 months to: (1) filtered air, (2) aerosolized house dust mite allergen, (3) ozone 0.5 ppm, or (4) house dust mite allergen + ozone. Studying the function/structure relationship of the same lung slices, we evaluated methacholine airway responsiveness and histology of bronchi and respiratory bronchioles. In bronchi, intrinsic responsiveness was increased by allergen exposure, an effect reduced by bombesin antagonist. In respiratory bronchioles, intrinsic airway responsiveness was increased by allergen + ozone exposure. Eosinophils were increased by allergen and allergen + ozone exposure in bronchi and by allergen exposure in respiratory bronchioles. In both airways, exposure to allergen + ozone resulted in fewer tissue eosinophils than did allergen exposure alone. In bronchi, but not in respiratory bronchioles, the number of eosinophils and neuroendocrine cells correlated with airway responsiveness. We conclude that episodically exposing infant monkeys to house dust mite allergen with or without ozone increased intrinsic airway responsiveness to methacholine in bronchi differently than in respiratory bronchioles. In bronchi, eosinophils and neuroendocrine cells may play a role in the development of airway hyperresponsiveness

Arterialização do arco venoso do pé para tratamento da tromboangeíte obliterante Arterialization of the venous arch of the foot for the treatment of thromboangiitis obliterans

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Cesar Roberto Busato

2008-09-01

Full Text Available Em isquemia crítica sem leito arterial distal, um dos modos de irrigar o membro isquêmico é derivar o fluxo de maneira retrógrada através do sistema venoso. As primeiras tentativas de fístulas arteriovenosas terapêuticas datam do início do século passado. Realizadas na parte proximal dos membros inferiores, não obtiveram resultados favoráveis. A partir da década de 70, com os trabalhos pioneiros de Lengua, as fístulas passaram a ser estendidas até o pé, e os bons resultados apareceram em várias publicações. Os autores relatam a evolução de um caso de tromboangeíte obliterante submetida ao procedimento. Essa é uma cirurgia de indicação precisa, que requer estudo pré-operatório arterial e venoso e observância a detalhes de técnica operatória.In critical ischemia without arterial run-off, it is possible to irrigate the ischemic limb by turning the course of the flow reversely through the venous system. The first experiments with therapeutic arteriovenous fistulas date from the beginning of the last century. They were performed in the proximal area of the lower limbs, but showed unfavorable results. Since the 1970's, with the pioneer studies of Lengua, fistulas started being extended to the foot and several publications have reported good outcomes. The authors report the evolution of a case of thromboangiitis obliterans which was submitted to the procedure. This is an accurate surgical procedure which requires arterial and venous preoperative study and the observance of technical operative details.

Turner Syndrome: Other FAQs

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... Other FAQs Share Facebook Twitter Pinterest Email Print Turner Syndrome: Other FAQs Basic information for topics, such as " ... been diagnosed with Turner syndrome. Now what? Is Turner syndrome inherited? Turner syndrome is usually not inherited, but ...

Bardet-Biedl syndrome and Usher syndrome.

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Koenig, Rainer

2003-01-01

Bardet-Biedl syndrome (BBS) and Usher syndrome (USH) are the most prevalent syndromic forms of retinitis pigmentosa (RP), together they make up almost a quarter of the patients with RP. BBS is defined by the association of retinopathy, obesity, hypogonadism, renal dysfunction, postaxial polydactyly and mental retardation. This clinically complex syndrome is genetically heterogeneous with linkage to more than 6 loci, and 4 genes have been cloned so far. Recent molecular data present evidence that, in some instances, the clinical manifestation of BBS requires recessive mutations in 1 of the 6 BBS loci plus one or two additional mutations in a second BBS locus (tri- or tetra-allelic inheritance). USH is characterized by the combination of congenital or early-onset sensorineural deafness, RP, and variable degrees of vestibular dysfunction. Each of the three clinical types is genetically heterogeneous: 7 loci have been mapped for type 1, three loci for type 2, and two loci for type 3. Currently, 6 USH genes (MYO7A, USH1C, CDH23, PCDH15, USH2A, USH3) have been identified. Pathogenetically, mutations of the USH1 genes seem to result in defects of auditory and retinal sensory cells, the USH 2 phenotype is caused by defects of extracellular matrix or cell surface receptor proteins, and USH3 may be due to synaptic disturbances. The considerable contribution of syndromic forms of RP requires interdisciplinary approaches to the clinical and diagnostic management of RP patients.

Abdominal compartment syndrome with acute reperfusion syndrome

International Nuclear Information System (INIS)

Maleeva, A.

2017-01-01

Abdominal compartment syndrome was recognized clinically in the 19th century when Marey and Burt observed its association with declines in respiratory function. Abdominal compartment syndrome is first used as a medical terminology from Fietsman in a case of ruptured abdominal aortic aneurysm. A condition caused by abnormally increased pressure within the abdomen. Causes of abdominal compartment syndrome include trauma, surgery, or infection. Common symptoms: abdominal distension, fast heart rate, insufficient urine production, or low blood pressure Medical procedure: nasogastric intubation Surgery: laparotomy Specialists: radiologist, primary care provider (PCP), surgeon, and emergency medicine doctor [6, 10]. Keywords: Stomach. Gastroparesis . Diabetes Mellitus [bg

Munchausen syndrome and Munchausen syndrome by proxy in dermatology.

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Boyd, Alan S; Ritchie, Coleman; Likhari, Sunaina

2014-08-01

Patients with Munchausen syndrome purposefully injure themselves, often with the injection of foreign materials, to gain hospital admission and the attention associated with having a difficult-to-identify condition. Munchausen syndrome by proxy occurs when a child's caregiver, typically the mother, injures the child for the same reasons. Cases of Munchausen syndrome and Munchausen syndrome by proxy with primary cutaneous involvement appear to be rarely described in the literature suggesting either that diagnosis is not made readily or that it is, in fact, an uncommon disorder. At the center of both conditions is significant psychological pathology and treatment is difficult as many patients with Munchausen syndrome when confronted with these diagnostic possibilities simply leave the hospital. Little is known about the long-term outcome or prognosis of these patients. Copyright © 2014 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

Roberts-SC syndrome, a rare syndrome and cleft palate repair

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Murthy Jyotsna

2008-01-01

Full Text Available Roberts SC syndrome is a rare syndrome with only 17 previously recognized patients reported in medical literature. The syndrome is characterized by multiple malformations, particularly, symmetrical limb reduction, craniofacial anomalies such as bilateral cleft lip and palate, micrognathia, and severe growth and mental retardation. Our patient, a young child of five years having Roberts-SC, was successfully operated for cleft palate under general anesthesia. The main features of the syndrome and the technical problems of anesthesia and surgery are discussed in this report.

Sjogren-Larsson Syndrome

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... Or In Memory Of Obituaries Contact Us Donate Sjogren-Larsson Syndrome What causes SLS? SLS is caused by mutations ... methods of diagnosing SLS. Other Clinical Names for Sjogren-Larsson Syndrome Other clinical names of Sjogren-Larsson Syndrome include: ...

Redefining syndromic surveillance

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Rebecca Katz

2011-12-01

Full Text Available With growing concerns about international spread of disease and expanding use of early disease detection surveillance methods, the field of syndromic surveillance has received increased attention over the last decade. The purpose of this article is to clarify the various meanings that have been assigned to the term syndromic surveillance and to propose a refined categorization of the characteristics of these systems. Existing literature and conference proceedings were examined on syndromic surveillance from 1998 to 2010, focusing on low- and middle-income settings. Based on the 36 unique definitions of syndromic surveillance found in the literature, five commonly accepted principles of syndromic surveillance systems were identified, as well as two fundamental categories: specific and non-specific disease detection. Ultimately, the proposed categorization of syndromic surveillance distinguishes between systems that focus on detecting defined syndromes or outcomes of interest and those that aim to uncover non-specific trends that suggest an outbreak may be occurring. By providing an accurate and comprehensive picture of this field’s capabilities, and differentiating among system types, a unified understanding of the syndromic surveillance field can be developed, encouraging the adoption, investment in, and implementation of these systems in settings that need bolstered surveillance capacity, particularly low- and middle-income countries.

Increased protein-energy intake promotes anabolism in critically ill infants with viral bronchiolitis: a double‑blind randomised controlled trial

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de Betue, Carlijn T; van Waardenburg, Dick A; Deutz, Nicolaas E; van Eijk, Hans M; van Goudoever, Johannes B; Luiking, Yvette C; Zimmermann, Luc J; Joosten, Koen F

2011-01-01

Objective The preservation of nutritional status and growth is an important aim in critically ill infants, but difficult to achieve due to the metabolic stress response and inadequate nutritional intake, leading to negative protein balance. This study investigated whether increasing protein and energy intakes can promote anabolism. The primary outcome was whole body protein balance, and the secondary outcome was first pass splanchnic phenylalanine extraction (SPEPhe). Design This was a double-blind randomised controlled trial. Infants (n=18) admitted to the paediatric intensive care unit with respiratory failure due to viral bronchiolitis were randomised to continuous enteral feeding with protein and energy enriched formula (PE-formula) (n=8; 3.1±0.3 g protein/kg/24 h, 119±25 kcal/kg/24 h) or standard formula (S-formula) (n=10; 1.7±0.2 g protein/kg/24 h, 84±15 kcal/kg/24 h; equivalent to recommended intakes for healthy infants <6 months). A combined intravenous-enteral phenylalanine stable isotope protocol was used on day 5 after admission to determine whole body protein metabolism and SPEPhe. Results Protein balance was significantly higher with PE-formula than with S-formula (PE-formula: 0.73±0.5 vs S-formula: 0.02±0.6 g/kg/24 h) resulting from significantly increased protein synthesis (PE-formula: 9.6±4.4, S-formula: 5.2±2.3 g/kg/24 h), despite significantly increased protein breakdown (PE-formula: 8.9±4.3, S-formula: 5.2±2.6 g/kg/24 h). SPEPhe was not statistically different between the two groups (PE-formula: 39.8±18.3%, S-formula: 52.4±13.6%). Conclusions Increasing protein and energy intakes promotes protein anabolism in critically ill infants in the first days after admission. Since this is an important target of nutritional support, increased protein and energy intakes should be preferred above standard intakes in these infants. Dutch Trial Register number: NTR 515. PMID:21673183

Gorlin-Goltz Syndrome

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Padma Pandeshwar

2012-01-01

Full Text Available The Gorlin-Goltz syndrome (GGS (the nevoid basal cell carcinoma syndrome—NBCCS is a rare autosomal dominant syndrome caused due to mutations in the PTCH (patched gene found on chromosome arm 9q. The syndrome, characterized by increased predisposition to develop basal cell carcinoma and associated multiorgan anomalies, has a high level of penetrance and variable expressiveness. GGS is a multidisciplinary problem, early diagnosis of which allows introduction of secondary prophylaxis and following an appropriate treatment to delay the progress of the syndrome. The following report emphasizes the need for awareness of the diagnostic criteria of this syndrome in cases with no typical skin lesions.

Sjögren syndrome

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Xerostomia - Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome ... The cause of Sjögren syndrome is unknown. It is an autoimmune disorder. This means the body attacks healthy tissue by mistake. The syndrome occurs most ...

Marfan Syndrome (For Parents)

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... en español Síndrome de Marfan What Is Marfan Syndrome? Marfan syndrome is a genetic disorder of the body's ... bones , blood vessels, and organs. What Causes Marfan Syndrome? Marfan syndrome happens because of an abnormality in one ...

A randomized trial of montelukast in respiratory syncytial virus postbronchiolitis

DEFF Research Database (Denmark)

Bisgaard, Hans

2003-01-01

Infants often develop reactive airway disease after respiratory syncytial virus (RSV) bronchiolitis. Cysteinyl-leukotrienes (cys-LT) are released during RSV infection and may contribute to the inflammation. We hypothesized that a cys-LT receptor antagonist would ameliorate reactive airway disease...... subsequent to RSV bronchiolitis. One hundred and thirty infants who were 3 to 36 months old, hospitalized with acute RSV bronchiolitis, were randomized into a double-blind, parallel comparison of 5-mg montelukast chewable tablets or matching placebo given for 28 days starting within 7 days of symptom debut...

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome).

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Kiran, N K; Tilak Raj, T N; Mukunda, K S; Rajashekar Reddy, V

2012-10-01

The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS), is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome

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N K Kiran

2012-01-01

Full Text Available The Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome (NBCCS, is an infrequent multisystemic disease inherited in a dominant autosomal way, which shows a high level of penetrance and variable expressiveness. It is characterized by odontogenic keratocysts in the jaw, multiple basal cell nevi carcinomas and skeletal abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the odontogenic keratocysts are usually one of the first manifestations of the syndrome. This case report presents a patient diagnosed as NBCCS by clinical, radiographic and histological findings in a 13-year-old boy. This paper highlights the importance of early diagnosis of NBCCS which can help in preventive multidisciplinary approach to provide a better prognosis for the patient.

``Battered child`` syndrome; Das ``Battered-Child``-Syndrom

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Elsner, K.; Merk, J.; Sokiranski, R. [Ulm Univ. (Germany). Abt. Diagnostische Radiologie

1997-10-01

Synonyms for the `battered child` syndrome (BCS) are terms describing the physical and body aspects of the process, such as `child abuse`, or `non-accidental injury`. These are to be distinguished from the psychic aspects and abuse, emotional and bodily neglect, and sexual abuse. Most cases are one or another combination of these aspects. Radiology is the essential method for giving proof of such abuses, identifying the signs of maltreatment in a medical record, or for disproving suspected abuse. (orig./AJ) [Deutsch] Als Synonym fuer das `Battered-Child`-Syndrom (BCS) stehen die Begriffe der koerperlichen-/physikalischen-Kindesmisshandlung, im angelsaechsischen Sprachraum die Begriffe `Child Abuse` und `Nonaccidental Injury`. Vom Syndrom abzugrenzen sind die seelische Misshandlung, die seelische und koerperliche Vernachlaessigung, und der sexuelle Missbrauch. Kombinationsformen sind nicht selten. Bei der Diagnostik des Syndroms spielt die Radiologie eine entscheidende Rolle. So hilft der Einsatz adaequater Untersuchungsmethoden, den Tatbestand der Misshandlung zu identifizieren und zu dokumentieren, aber auch einen Verdacht zu widerlegen. (orig./AJ)

Hypercholermic metabolic alcalsosis as a presentation of cystic fibrosis: presentation of two cases = Alcalosis metabólica hipoclorémica como presentación de la fibrosis quística. Informe de dos casos

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Olga Lucía Morales Múnera

2013-07-01

Full Text Available Introduction: We describe two cases of patients with hyperchloremic metabolic acidosis as an initial presentation of cystic fibrosis (CF or as part of a second CF exacerbation. Clinical Cases: Two patients, 6 and 9 months old, consulted for cough, fever, and dyspnea. The first had syndrome of recurrent bronchial obstruction, without a diagnosis of CF on admission. Both presented with difficulty breathing, dehydration, and malnutrition. Arterial blood gases showed metabolic acidosis, hypokalemia, and severe hypochloremia. Treatment with sodium chloride and potassium improved their electrolyte balance and acid-base status. They did not present with renal or gastrointestinal losses of chloride. CF and pseudo-Barter’s Syndrome were diagnosed. Conclusion: Metabolic alkalosis can present as an initial manifestation of CF in infants with recurrent bronchiolitis and short stature suspected of having CF: equally it can be an acute exacerbation in patients with known CF. Your recognition and treatment are an opportunity to decrease morbidity.

Hepatorenal syndrome

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... 2016:chap 153. Nevah MI, Fallon MB. Hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and other systemic complications of liver disease. In: Feldman M, Friedman LS, Brandt LJ, ...

Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

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Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

2015-03-01

Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

Turner Syndrome (For Teens)

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... Staying Safe Videos for Educators Search English Español Turner Syndrome KidsHealth / For Teens / Turner Syndrome What's in this ... en español El síndrome de Turner What Is Turner Syndrome? Turner syndrome (TS) is a genetic condition found ...

DIDMOAD (Wolfram Syndrome

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Masoud Nashibi

2016-07-01

Full Text Available Wolfram syndrome was first described by physician D J Wolfram and Wagener in 1938. This autosomal recessive syndrome is also referred to as DIDMOAD syndrome which stands for Diabetes Insipidus, Insulin Dependent Diabetes Mellitus, Optic Atrophy and Deafness

[The Capgras syndrome].

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Anikina, M A; Levin, O S

2013-01-01

The Capgras syndrome is one of delusional-like misidentification syndrome in which a person holds a delusion that one or several his/her friends or relatives have been replaced by an identical-looking impostor. As any other delusional disorder, the Capgras syndrome is characterized by stability despite the indisputable arguments against fault views. Initially, this syndrome was considered as a presentation of schizophrenia but later it has been described in brain organic disorders, primarily in elderly patients with dementia.

Revesz syndrome

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Dayane Cristine Issaho

2015-04-01

Full Text Available Revesz syndrome is a rare variant of dyskeratosis congenita and is characterized by bilateral exudative retinopathy, alterations in the anterior ocular segment, intrauterine growth retardation, fine sparse hair, reticulate skin pigmentation, bone marrow failure, cerebral calcification, cerebellar hypoplasia and psychomotor retardation. Few patients with this syndrome have been reported, and significant clinical variations exist among patients. This report describes the first Brazilian case of Revesz syndrome and its ocular and clinical features.

Cardiorenal Syndrome in Acute Heart Failure Syndromes

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Mohammad Sarraf

2011-01-01

Full Text Available Impaired cardiac function leads to activation of the neurohumoral axis, sodium and water retention, congestion and ultimately impaired kidney function. This sequence of events has been termed the Cardiorenal Syndrome. This is different from the increase in cardiovascular complications which occur with primary kidney disease, that is, the so-called Renocardiac Syndrome. The present review discusses the pathogenesis of the Cardiorenal Syndrome followed by the benefits and potential deleterious effects of pharmacological agents that have been used in this setting. The agents discussed are diuretics, aquaretics, natriuretic peptides, vasodilators, inotropes and adenosine α1 receptor antagonists. The potential role of ultrafiltration is also briefly discussed.

Interstitial Cystitis/Painful Bladder Syndrome and Associated Medical Conditions With an Emphasis on Irritable Bowel Syndrome, Fibromyalgia and Chronic Fatigue Syndrome

DEFF Research Database (Denmark)

Nickel, J.C.; Tripp, D.A.; Pontari, M.

2010-01-01

of associated conditions increased (ie localized, regional, systemic), pain, stress, depression and sleep disturbance increased while social support, sexual functioning and quality of life deteriorated. Anxiety and catastrophizing remained increased in all groups. Symptom duration was associated......Purpose: We characterized and compared the impact of clinical phenotypic associations between interstitial cystitis/painful bladder syndrome and controls in relation to potentially related conditions, particularly irritable bowel syndrome, fibromyalgia and chronic fatigue syndrome. Materials...... cystitis/painful bladder syndrome vs controls was irritable bowel syndrome 38.6% vs 5.2%, fibromyalgia 17.7% vs 2.6% and chronic fatigue syndrome 9.5% vs 1.7% (all p

Plummer-Vinson syndrome

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Novacek Gottfried

2006-09-01

Full Text Available Abstract Plummer-Vinson or Paterson-Kelly syndrome presents as a classical triad of dysphagia, iron-deficiency anemia and esophageal webs. Exact data about epidemiology of the syndrome are not available; the syndrome is extremely rare. Most of the patients are white middle-aged women, in the fourth to seventh decade of life but the syndrome has also been described in children and adolescents. The dysphagia is usually painless and intermittent or progressive over years, limited to solids and sometimes associated with weight loss. Symptoms resulting from anemia (weakness, pallor, fatigue, tachycardia may dominate the clinical picture. Additional features are glossitis, angular cheilitis and koilonychia. Enlargement of the spleen and thyroid may also be observed. One of the most important clinical aspects of Plummer-Vinson syndrome is the association with upper alimentary tract cancers. Etiopathogenesis of Plummer-Vinson syndrome is unknown. The most important possible etiological factor is iron deficiency. Other possible factors include malnutrition, genetic predisposition or autoimmune processes. Plummer-Vinson syndrome can be treated effectively with iron supplementation and mechanical dilation. In case of significant obstruction of the esophageal lumen by esophageal web and persistent dysphagia despite iron supplementation, rupture and dilation of the web are necessary. Since Plummer-Vinson syndrome is associated with an increased risk of squamous cell carcinoma of the pharynx and the esophagus, the patients should be followed closely.

Genetics Home Reference: antiphospholipid syndrome

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... Share: Email Facebook Twitter Home Health Conditions Antiphospholipid syndrome Antiphospholipid syndrome Printable PDF Open All Close All Enable ... area? Other Names for This Condition anti-phospholipid syndrome antiphospholipid antibody syndrome Hughes syndrome Related Information How are ...

Genetics Home Reference: Costello syndrome

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... other genetic conditions, cardiofaciocutaneous syndrome (CFC syndrome) and Noonan syndrome . In affected infants, it can be difficult to ... These individuals may actually have CFC syndrome or Noonan syndrome , which are caused by mutations in related genes. ...

Metabolic syndrome and menopause

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Jouyandeh Zahra

2013-01-01

Full Text Available Abstract Background The metabolic syndrome is defined as an assemblage of risk factors for cardiovascular diseases, and menopause is associated with an increase in metabolic syndrome prevalence. The aim of this study was to assess the prevalence of metabolic syndrome and its components among postmenopausal women in Tehran, Iran. Methods In this cross-sectional study in menopause clinic in Tehran, 118 postmenopausal women were investigated. We used the adult treatment panel 3 (ATP3 criteria to classify subjects as having metabolic syndrome. Results Total prevalence of metabolic syndrome among our subjects was 30.1%. Waist circumference, HDL-cholesterol, fasting blood glucose, diastolic blood pressure ,Systolic blood pressure, and triglyceride were significantly higher among women with metabolic syndrome (P-value Conclusions Our study shows that postmenopausal status is associated with an increased risk of metabolic syndrome. Therefore, to prevent cardiovascular disease there is a need to evaluate metabolic syndrome and its components from the time of the menopause.

Wolfram syndrome 1 and Wolfram syndrome 2.

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Rigoli, Luciana; Di Bella, Chiara

2012-08-01

Wolfram syndrome 1 (WS1) is an autosomal recessive disorder characterized by diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DI DM OA D syndrome) associated with other variable clinical manifestations. The causative gene for WS1 (WFS1) encoding wolframin maps to chromosome 4p16.1. Wolframin has an important function in maintaining the homeostasis of the endoplasmic reticulum (ER) in pancreatic β cells. Recently, another causative gene, CISD2, has been identified in patients with a type of Wolfram syndrome (WS2) resulting in early optic atrophy, diabetes mellitus, deafness, decreased lifespan, but not diabetes insipidus. The CISD2-encoded protein ERIS (endoplasmic reticulum intermembrane small protein) also localizes to ER, but does not interact directly with wolframin. ERIS maps to chromosome 4q22. Numerous studies have shown an interesting similarity between WFS1 and CISD2 genes. Experimental studies demonstrated that the Cisd2 knockout (Cisd2) mouse shows premature aging and typical symptoms of Wolfram syndrome. These researches provide interesting insight into the relation of neurodegenerative diseases, mitochondrial disorders, and autophagy and are useful for the pathophysiological understanding of both Wolfram syndrome and mitochondrial-mediated premature aging. The knowledge of WS1 and WS2 pathogenesis, and of the interactions between WFS1 and CISD2 genes, is useful for accurate diagnostic classification and for diagnosis of presymptomatic individuals.

Interaction Between Syndromic and Non-Syndromic Factors Affecting Speech and Language Development in Treacher-Collins Syndrome

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Marziyeh Poorjavad

2011-09-01

Full Text Available Background: Treacher-Collins syndrome is a congenital craniofacial disorder with multiple anomalies. This syndrome affects the maxilla, mandible, eyes, middle and outer ears, and soft palate. Conductive hearing loss due to the deformities of the middle and external ears is prevalent. The characteristics of this syndrome include multiple and serious threats to normal communication development in children. In this study, speech and language features of a Persian speaking child with this syndrome are presented.Case: The case was an 8-year old girl with Treacher-Collins syndrome and bilateral moderate conductive hearing loss due to atretic canal. In language and speech assessments, moderate hypernasality, numerous compensatory errors and morphosyntactic deficits were observed. There were 13 phonemes that were incorrectly produced at least in one position. Besides, she used 22 types of phonological processes that were abnormal and disappear before the age of three in normal Persian speaking children.Conclusion: Moderate hearing loss, velopharyngeal incompetency, malocclusion and dental anomalies, attention deficit/hyperactivity disorder (ADHD and environmental factors resulted in severe speech and language disorders in this case. These disorders affected her academic performance as well. Moderate hypernasality, numerous compensatory errors, and excessive and abnormal use of phonological processes were not presented as prevalent characteristics of Treacher-Collins syndrome in other resources.

Urofacial syndrome

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Kamal F Akl

2012-01-01

Full Text Available The urofacial syndrome is characterized by functional obstructive uropathy asso-ciated with an inverted smile. The importance of the subject is that it sheds light, not only on the muscles of facial expression, but also on the inheritance of voiding disorders and lower urinary tract malformations. We report a 10-year-old-male patient who had the urofacial syndrome. Early diagnosis of the urofacial syndrome is important to avoid upper urinary tract damage and renal failure.

Felty syndrome

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Seropositive rheumatoid arthritis (RA); Felty's syndrome ... The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis (RA) for a long time. People with ...

Pendred Syndrome

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... other possible long-term consequences of the syndrome. Children with Pendred syndrome should start early treatment to gain communication skills, such as learning sign language or cued speech or learning to ...

Unusual headache syndromes.

Science.gov (United States)

Queiroz, Luiz P

2013-01-01

Some headache syndromes have few cases reported in the literature. Their clinical characteristics, pathogenesis, and treatment may have not been completely defined. They may not actually be uncommon but rather under-recognized and/or underreported. A literature review of unusual headache syndromes, searching PubMed and ISI Web of Knowledge, was performed. After deciding which disorders to study, relevant publications in scientific journals, including original articles, reviews, meeting abstracts, and letters or correspondences to the editors were searched. This paper reviewed the clinical characteristics, the pathogenesis, the diagnosis, and the treatment of five interesting and unusual headache syndromes: exploding head syndrome, red ear syndrome, neck-tongue syndrome, nummular headache, and cardiac cephalgia. Recognizing some unusual headaches, either primary or secondary, may be a challenge for many non-headache specialist physicians. It is important to study them because the correct diagnosis may result in specific treatments that may improve the quality of life of these patients, and this can even be life saving. © 2013 American Headache Society.

Burnout Syndrome of Teachers

OpenAIRE

Semrádová, Michaela

2013-01-01

The bachelor's thesis covers burnout syndrome of teachers. Defines burnout syndrome, describes its causes and symptoms. Describes teaching as helping profession and focousing on stressful situations at school. In the last chapter described different prevention strategies burnout syndrome. Key words: burnout syndrome, teaching, teacher, helping professions, beginning teacher, stress

Role of viral coinfections in asthma development.

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Maria Luz Garcia-Garcia

Full Text Available Viral respiratory infections, especially acute bronchiolitis, play a key role in the development of asthma in childhood. However, most studies have focused on respiratory syncytial virus or rhinovirus infections and none of them have compared the long-term evolution of single versus double or multiple viral infections.Our aim was to compare the frequency of asthma development at 6-8 years in children with previous admission for bronchiolitis associated with single versus double or multiple viral infection.A cross-sectional study was performed in 244 children currently aged 6-8 years, previously admitted due to bronchiolitis between September 2008 and December 2011. A structured clinical interview and the ISAAC questionnaire for asthma symptoms for 6-7-year-old children, were answered by parents by telephone. Specimens of nasopharyngeal aspirate for virological study (polymerase chain reaction and clinical data were prospectively taken during admission for bronchiolitis.Median current age at follow-up was 7.3 years (IQR: 6.7-8.1. The rate of recurrent wheezing was 82.7% in the coinfection group and 69.7% in the single-infection group, p = 0.06. The number of wheezing-related admissions was twice as high in coinfections than in single infections, p = 0.004. Regarding the ISAAC questionnaire, 30.8% of coinfections versus 15% of single infections, p = 0.01, presented "wheezing in the last 12 months", data that strongly correlate with current prevalence of asthma. "Dry cough at night" was also reported more frequently in coinfections than in single infections, p = 0.02. The strongest independent risk factors for asthma at 6-8 years of age were: age > 9 months at admission for bronchiolitis (OR: 3.484; CI95%: 1.459-8.317, p:0.005, allergic rhinitis (OR: 5.910; 95%CI: 2.622-13.318, p<0.001, and viral coinfection-bronchiolitis (OR: 3.374; CI95%: 1.542-7.386, p:0.01.Asthma at 6-8 years is more frequent and severe in those children previously hospitalized

Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

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Ana Cláudia Giaxa Prosdócimo

2015-03-01

Full Text Available Background: Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS. Objective: To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods: Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI, which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE, emotional distancing (EmD, dehumanization (De and professional fulfillment (PF. The Lipp’s Stress Symptoms Inventory for Adults (LSSI was applied to evaluate global stress. Results: Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion: We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases

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Rohan R Mahale

2015-01-01

Full Text Available "Eight-and-a-half" syndrome is a rare condition involving the ipsilateral abducens nucleus or paramedian pontine reticular formation (PPRF, the ipsilateral medial longitudinal fasciculus (MLF, and the adjacent facial colliculus/facial nerve fascicle. The condition is often caused by a lesion (vascular or demyelinating in the dorsal tegmentum of the caudal pons. There are new variants of this syndrome caused by extension of lesion to involve new adjacent structures in pontine tegmentum. We report two patients with different etiology presenting with clinical features suggestive of eight-and-a-half syndrome associated with hemiataxia representing "nine" syndrome (8– + – = 9 adding new dimension to "eight-and-a-half" syndrome.

Mobius syndrome redefined: a syndrome of rhombencephalic maldevelopment.

NARCIS (Netherlands)

Verzijl, H.T.F.M.; Zwaag, A. van der; Cruysberg, J.R.M.; Padberg, G.W.A.M.

2003-01-01

OBJECTIVE: To investigate the variable clinical picture of Mobius syndrome (MIM no. 157900) and to further understand the pathogenesis of the disorder. METHODS: A standardized questionnaire was submitted to 37 Dutch patients with Mobius syndrome. All underwent standardized neurologic examination

Bartter syndrome

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... this page: //medlineplus.gov/ency/article/000308.htm Bartter syndrome To use the sharing features on this page, please enable JavaScript. Bartter syndrome is a group of rare conditions that affect ...

Dravet Syndrome

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... and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome. Study of the genetic defects responsible for Dravet syndrome and related ... Publications Definition Dravet ...

Genetics Home Reference: Marfan syndrome

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... Share: Email Facebook Twitter Home Health Conditions Marfan syndrome Marfan syndrome Printable PDF Open All Close All Enable Javascript ... Marfan syndrome KidsHealth from Nemours Foundation MalaCards: marfan syndrome Orphanet: Marfan syndrome Your Genes Your Health from Cold Spring ...

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

Science.gov (United States)

Choi, Intae

2017-01-01

The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea's metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report

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Shukla Umesh

2010-11-01

Full Text Available Abstract Introduction We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. Case presentation A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. Conclusion In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome: a case report.

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Goyal, Deepak; Yadav, Dinesh K; Shukla, Umesh; Sethi, Sidharth K

2010-11-08

We report the case of an unusual association of Coffin-Siris syndrome with Mayer-Rokitansky-Küster-Hauser syndrome. This association has never previously been reported in the medical literature. A nine-year-old Indian girl was referred to our hospital for growth retardation, mental retardation, lax joints, generalized hypertrichosis, and hypoplastic fifth fingernails and toenails. A thorough medical examination and evaluation revealed she had phenotypic features of Coffin-Siris syndrome, with Mayer-Rokitansky-Küster-Hauser syndrome on radiological evaluation. The karyotype of our patient was normal. In an unexplained case of mental retardation with facies suggestive of Coffin-Siris syndrome, association with Mayer-Rokitansky-Küster-Hauser syndrome should be considered and the patient should be evaluated for the same. Both of these syndromes may have a common pathogenesis, as yet unknown. This case report has broad implications, as similar cases in future may give insights into the pathogenesis of both these syndromes.

Bronchiole alveolar carcinoma: biological profile, clinicopathologic presentation, evaluation and treatment. Literature review and national series of 26 cases

International Nuclear Information System (INIS)

Vázquez, A.; Terzieff, V.

2004-01-01

B. Objective: To study the biological profile, clinic pathologic presentation of BAC, characterized by a peculiar evolutionary behavior, study and treatment. P Methodology: Literature review national and international communication based on 26 cases clinical studied and treated in our midst. Results: The BAC behaves as a well-differentiated adenocarcinoma of origin peripheral, typically linked to the Airborne spread to bilateral intrapulmonary cell its three forms: a clear cells and mucous secreting alveolar type II. Present day not considered characteristic of BAC nodal dissemination or hematologic level in the absence of type IV collagenase secretion by tumor cells. It is of no destroying, in one or more cell layers, the bronchiole alveolar septa. Its origin is discussed single or multicenter, while some authors establish etiological factors from benign diseases that can evolve into this neoplasm. It may or may not be linked smoking, presenting clinically in three forms: uni nodular, multi nodular and sclerosing, is studied by CT, FBC generally negative cytological aspiration, puncture or biopsy. It is treated by curative treatment with surgery alone, Rt, Qt and this study the use of tyrosine kinase inhibitors with apparent antitumor activity in BAC Conclusions Some authors present it as a different adenocarcinoma to other CBPNPC, especially in its advanced form multi nodular. Biological tests set for it that his understanding is confirmed. In its evolution can indiferenciarse to infiltrantres forms characteristic of other adenocarcinomas, particularly in the form sclerosing. this fact biological can explain the emergence of mixed tumors with extensive BAC component together another adenocarcinoma. We believe that there is insufficient evidence at present to say that this is a different from the rest of the CBPNCP adenocarcinoma, although studies biological-genetic of this point to gather evidence for this difference. characteristically FBC is usually negative

Rett Syndrome

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Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

Caplan syndrome

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... enable JavaScript. Rheumatoid pneumoconiosis (RP; also known as Caplan syndrome) is swelling (inflammation) and scarring of the ... avoid exposure to inorganic dust. Alternative Names RP; Caplan syndrome; Pneumoconiosis - rheumatoid; Silicosis - rheumatoid pneumoconiosis; Coal worker's ...

Sotos Syndrome

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... Clinical Trials Organizations Publications Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ... have also been reported. × Definition Sotos syndrome (cerebral gigantism) is a rare genetic disorder caused by mutation ...

Chronic exertional compartment syndrome with medial tibial stress syndrome in twins.

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Banerjee, Purnajyoti; McLean, Christopher

2011-06-14

Chronic exertional compartment syndrome and medial tibial stress syndrome are uncommon conditions that affect long-distance runners or players involved in team sports that require extensive running. We report 2 cases of bilateral chronic exertional compartment syndrome, with medial tibial stress syndrome in identical twins diagnosed with the use of a Kodiag monitor (B. Braun Medical, Sheffield, United Kingdom) fulfilling the modified diagnostic criteria for chronic exertional compartment syndrome as described by Pedowitz et al, which includes: (1) pre-exercise compartment pressure level >15 mm Hg; (2) 1 minute post-exercise pressure >30 mm Hg; and (3) 5 minutes post-exercise pressure >20 mm Hg in the presence of clinical features. Both patients were treated with bilateral anterior fasciotomies through minimal incision and deep posterior fasciotomies with tibial periosteal stripping performed through longer anteromedial incisions under direct vision followed by intensive physiotherapy resulting in complete symptomatic recovery. The etiology of chronic exertional compartment syndrome is not fully understood, but it is postulated abnormal increases in intramuscular pressure during exercise impair local perfusion, causing ischemic muscle pain. No familial predisposition has been reported to date. However, some authors have found that no significant difference exists in the relative perfusion, in patients, diagnosed with chronic exertional compartment syndrome. Magnetic resonance images of affected compartments have indicated that the pain is not due to ischemia, but rather from a disproportionate oxygen supply versus demand. We believe this is the first report of chronic exertional compartment syndrome with medial tibial stress syndrome in twins, raising the question of whether there is a genetic predisposition to the causation of these conditions. Copyright 2011, SLACK Incorporated.

Moyamoya syndrome in a patient with Noonan-like syndrome with loose anagen hair.

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Choi, Jin-Ho; Oh, Moon-Yeon; Yum, Mi-Sun; Lee, Beom Hee; Kim, Gu-Hwan; Yoo, Han-Wook

2015-03-01

Noonan-like syndrome with loose anagen hair is one of the RASopathies characterized by Noonan syndrome-like features with unique ectodermal abnormalities. This syndrome is caused by mutations in the SHOC2 gene. We encountered a patient with moyamoya syndrome associated with Noonan-like syndrome with loose anagen hair presenting with transient ischemic attacks. A 6-year-old girl was diagnosed with Noonan-like syndrome with loose anagen hair because of profound short stature and ectodermal anomalies such as sparse and easily pluckable hair. A heterozygous mutation of c.4A>G (p.S2G) in the SHOC2 gene was identified, and recombinant human growth hormone therapy was initiated at 8 years of age. At age 10, she manifested recurrent left hemiplegia. Moreover, cerebrovascular imaging revealed occlusion or narrowing of both internal carotid arteries and both middle cerebral arteries with distal moyamoya-like vessels. She is treated with aspirin and calcium channel blocker. We describe the first case of Noonan-like syndrome with loose anagen hair associated with moyamoya syndrome, although it has been reported to be associated with a few cases of other RASopathies, including Noonan, cardiofaciocutaneous, and Costello syndromes. This report emphasizes the associations between cerebrovascular anomalies and Noonan-like syndrome with loose anagen hair. Copyright © 2015 Elsevier Inc. All rights reserved.

Abdominal vascular syndromes: characteristic imaging findings

International Nuclear Information System (INIS)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D'Ippolito, Giuseppe

2016-01-01

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Abdominal vascular syndromes: characteristic imaging findings

Energy Technology Data Exchange (ETDEWEB)

Cardarelli-Leite, Leandro; Velloni, Fernanda Garozzo; Salvadori, Priscila Silveira; Lemos, Marcelo Delboni; D' Ippolito, Giuseppe, E-mail: leandrocleite@gmail.com [Universidade Federal de Sao Paulo (EPM/UNIFESP), Sao Paulo, SP (Brazil). Escola Paulista de Mediciana. Departmento de Diagnostico por Imagem

2016-07-15

Abdominal vascular syndromes are rare diseases. Although such syndromes vary widely in terms of symptoms and etiologies, certain imaging findings are characteristic. Depending on their etiology, they can be categorized as congenital - including blue rubber bleb nevus syndrome, Klippel-Trenaunay syndrome, and hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome) - compressive - including 'nutcracker' syndrome, median arcuate ligament syndrome, Cockett syndrome (also known as May-Thurner syndrome), and superior mesenteric artery syndrome. In this article, we aimed to illustrate imaging findings that are characteristic of these syndromes, through studies conducted at our institution, as well as to perform a brief review of the literature on this topic. (author)

Cushing's Syndrome

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Cushing's syndrome is a hormonal disorder. The cause is long-term exposure to too much cortisol, a hormone that ... your body to make too much cortisol. Cushing's syndrome is rare. Some symptoms are Upper body obesity ...

Usher Syndrome

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Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes ... and vision. There are three types of Usher syndrome: People with type I are deaf from birth ...

Metabolic Syndrome

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Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These conditions ... agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

Reye Syndrome

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Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently ... a viral illness, seek medical attention immediately. Reye syndrome can lead to a coma and brain death, ...

Turner Syndrome

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Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or incomplete ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of skin ...

Apert syndrome (acrocephalosyndactyly

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Milovanović J.

2014-01-01

Full Text Available Apert syndrome is named for the French physician, Eugen Apert who was, in 1906. described anomalous shape of the skull with coronary suture synostosis and hypoplasia sphenoethmoidmaxillary part of the face and fingers syndactyly of hands and feet. Apert syndrome accounts for about 4,5% of all craniosynostosis. With the prevalence of 1:160 000-200 000, inherited in an autosomal domi­nant, and in 25% of cases are fresh mutations in the gene. This syndrome has no predilection by gender and race, varies in severity form in witch it is manifested. Anomality of internal organs are very rare, but half of the patients with this syndrome have mental retardation. Apert syndrome has no cure, but surgery can help to correct some of the problems.

Coexistent Brugada Syndrome and Wolff-Parkinson-White Syndrome: What is the Optimal Management?

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Abhishek Jaiswal, MBBS

2013-09-01

Full Text Available Coexistent Brugada syndrome and Wolff-Parkinson-White (WPW syndrome is rare, and as such poses management challenges. The overlap of symptoms attributable to each condition, the timing of ventricular stimulation after accessory pathway ablation and the predictive value of programmed stimulation in Brugada syndrome are controversial. We describe a case of coexistent Brugada syndrome and WPW syndrome in a symptomatic young adult. We discuss our treatment approach and the existing literature along with the challenges in management of such cases.

Cowden syndrome

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Ravi Prakash S

2010-01-01

Full Text Available Cowden syndrome or multiple hamartoma syndrome is an autosomal dominant condition with variable expressions that result mainly from mutation in the PTEN gene on arm 10q. It is characterized by multiple hamartomatous neoplasms of the skin, oral mucosa, gastrointestinal tract, bones, CNS, eyes, and genitourinary tract. Mucocutaneous features include trichilemmomas, oral mucosal papillomatosis, acral keratosis, and palmoplantar keratosis. Here we present a case of Cowden syndrome in a 14-year-old female patient with the chief complaint of multiple oral papillomatous lesions.

Metabolic syndrome and the risk of adverse cardiovascular events after an acute coronary syndrome.

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Cavallari, Ilaria; Cannon, Christopher P; Braunwald, Eugene; Goodrich, Erica L; Im, KyungAh; Lukas, Mary Ann; O'Donoghue, Michelle L

2018-05-01

Background The incremental prognostic value of assessing the metabolic syndrome has been disputed. Little is known regarding its prognostic value in patients after an acute coronary syndrome. Design and methods The presence of metabolic syndrome (2005 International Diabetes Federation) was assessed at baseline in SOLID-TIMI 52, a trial of patients within 30 days of acute coronary syndrome (median follow-up 2.5 years). The primary endpoint was major coronary events (coronary heart disease death, myocardial infarction or urgent coronary revascularization). Results At baseline, 61.6% ( n = 7537) of patients met the definition of metabolic syndrome, 34.7% (n = 4247) had diabetes and 29.3% had both ( n = 3584). The presence of metabolic syndrome was associated with increased risk of major coronary events (adjusted hazard ratio (adjHR) 1.29, p metabolic syndrome was numerically but not significantly associated with the risk of major coronary events (adjHR 1.13, p = 0.06). Conversely, diabetes was a strong independent predictor of major coronary events in the absence of metabolic syndrome (adjHR 1.57, p metabolic syndrome identified patients at highest risk of adverse outcomes but the incremental value of metabolic syndrome was not significant relative to diabetes alone (adjHR 1.07, p = 0.54). Conclusions After acute coronary syndrome, diabetes is a strong and independent predictor of adverse outcomes. Assessment of the metabolic syndrome provides only marginal incremental value once the presence or absence of diabetes is established.

Cases of Trichohepatoenteric Syndrome (Syndromic Diarrhea with Underlying Crohn’s Disease

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Е. А. Roslavtseva

2015-01-01

Full Text Available Tricho-hepato-enteric syndrome (syndromic, phenotypic diarrhea, SD/THES is a rare inborn disease, which affects bowels. It is caused by the mutation of genes SKIV2L or TTC37. Manifestations include intrauterine hypotrophy, severe chronic diarrhea, which starts in infancy, characteristic facial features and hair growth abnormalities, immune disorders. There are data on two patients dealing with tricho-hepato-enteric syndrome with underlying Crohn’s disease. This is the first description of cases of aggravated tricho-hepatoenteric syndrome ever found in Russian medical literature. 

A new familial intrauterine growth retardation syndrome the "3-M syndrome".

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Spranger, J; Opitz, J M; Nourmand, A

1976-09-01

Two pairs of siblings are described with proportionate dwarfism due to skeletal hypoplasia of prenatal onset. The head size was normal for age and disproportionately large for height. The patients had a characteristic face different from that seen in the Silver-Russell syndrome. The family data are in accordance with autosomal recessive inheritance. In spite of some similarities, the bulk of clinical and genetic evidence suggests that the described intrauterine growth retardation syndrome is different from the Silver-Russell syndrome and presents an apparently "new" entity which has been designated 3-M syndrome.

Are ECG abnormalities in Noonan syndrome characteristic for the syndrome?

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Raaijmakers, R; Noordam, C; Noonan, J A; Croonen, E A; van der Burgt, C J A M; Draaisma, J M T

2008-12-01

Of all patients with Noonan syndrome, 50-90% have one or more congenital heart defects. The most frequent occurring are pulmonary stenosis (PS) and hypertrophic cardiomyopathy. The electrocardiogram (ECG) of a patient with Noonan syndrome often shows a characteristic pattern, with a left axis deviation, abnormal R/S ratio over the left precordium, and an abnormal Q wave. The objective of this study was to determine if these ECG characteristics are an independent feature of the Noonan syndrome or if they are related to the congenital heart defect. A cohort study was performed with 118 patients from two university hospitals in the United States and in The Netherlands. All patients were diagnosed with definite Noonan syndrome and had had an ECG and echocardiography. Sixty-nine patients (58%) had characteristic abnormalities of the ECG. In the patient group without a cardiac defect (n = 21), ten patients had a characteristic ECG abnormality. There was no statistical relationship between the presence of a characteristic ECG abnormality and the presence of a cardiac defect (p = 0.33). Patients with hypertrophic cardiomyopathy had more ECG abnormalities in total (p = 0.05), without correlation with a specific ECG abnormality. We conclude that the ECG features in patients with Noonan syndrome are characteristic for the syndrome and are not related to a specific cardiac defect. An ECG is very useful in the diagnosis of Noonan syndrome; every child with a Noonan phenotype should have an ECG and echocardiogram for evaluation.

Brain-hepato-renal syndrome (Zellweger syndrome). Report of two cases and a review of the syndrome

International Nuclear Information System (INIS)

Ruiz, T.; Caparros, C.; Blanco, A.; Lopez, A.M.

1997-01-01

Cerebro-hepato-renal syndrome is a rare disorder that is transmitted by autosomal recessive inheritance. Children with this syndrome present mongoloid facies and severe muscle hypotonic at birth. Scimitar-like knee calcifications are considered a pathognomonic feature of this disorder. We present two patients with Zellweger syndrome, according to the diagnosis suggested by our Radiodiagnostic Service. Our objective is to stress the importance of the radiological findings, which in many cases are decisive in establishing the definitive diagnosis. (Author) 10 refs

Does wastewater discharge have relations with increase of Turner syndrome and Down syndrome?

Directory of Open Access Journals (Sweden)

Intae Choi

2017-08-01

Full Text Available The purpose of this study is to examine whether water and air pollutants have a relationship with an increase in the genetic disorders Turner syndrome and Down syndrome, which are caused by congenital chromosomal abnormalities, and to generate a hypothesis about the genetic health effects of environmental pollutants. A panel regression based on random effect was conducted on Korea’s metropolitan councils from 2012 to 2014. The dependent variable was the number of Turner syndrome and Down syndrome cases, and the main independent variables were those regarding the water and air pollution. Air pollutants did not have a significant impact on the number of Turner syndrome and Down syndrome cases; however, the increase in number of wastewater discharge companies did have a significant relationship with the number of cases. The more the number of wastewater discharge companies, the more the number Turner syndrome and Down syndrome cases were observed. Therefore, scientific investigation on water and air pollutants in relation with genetic health effects needs to be performed.

Skin symptoms in four ectodermal dysplasia syndromes including two case reports of Rapp-Hodgkin-Syndrome.

Science.gov (United States)

Knaudt, Björn; Volz, Thomas; Krug, Markus; Burgdorf, Walter; Röcken, Martin; Berneburg, Mark

2012-01-01

The skin, hair and nail changes in four distinct ectodermal dysplasia syndromes are compared and reviewed. These syndromes comprise Christ-Siemens-Touraine syndrome; ectrodactyly, ectodermal dysplasia and cleft lip/palate syndrome; ankyloblepharon-ectodermal defects-cleft lip/palate syndrome and Rapp-Hodgkin syndrome. A comprehensive overview of the dermatological signs and symptoms in these syndromes was generated from the database of the Ectodermal Dysplasia Network Germany, the clinical findings in the patients seen in our department and an extensive review of the literature. The findings included abnormalities of skin, sweating, hair and nails. These clinical findings are discussed in relation to the underlying molecular defects known to play a role in these four ectodermal dysplasia syndromes.

Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome.

Science.gov (United States)

Roma, Maria; Marden, Colleen L; De Wandele, Inge; Francomano, Clair A; Rowe, Peter C

2018-03-05

To review the association between orthostatic intolerance syndromes and both joint hypermobility and Ehlers-Danlos syndrome, and to propose reasons for identifying hereditary connective tissue disorders in those with orthostatic intolerance in the context of both clinical care and research. We searched the published peer-reviewed medical literature for papers reporting an association between joint hypermobility or Ehlers-Danlos syndrome and orthostatic intolerance. We identified 10 relevant papers. Although methodological variability between studies introduces some limitations, the published literature consistently identifies a significantly higher prevalence of orthostatic intolerance symptoms in patients with joint hypermobility or Ehlers-Danlos syndrome than in healthy controls, and a significantly higher prevalence of cardiovascular and autonomic abnormalities both at rest and during orthostatic challenge. Postural tachycardia syndrome is the most commonly recognized circulatory disorder. The severity of orthostatic symptoms in those with EDS correlates with impairments in quality of life. There is a strong association between several forms of cardiovascular dysfunction, most notably postural tachycardia syndrome, and joint hypermobility or Ehlers-Danlos syndrome. We propose that recognition of joint hypermobility and Ehlers-Danlos syndrome among those with orthostatic intolerance syndromes has the potential to improve clinical care and the validity of research findings. Copyright © 2018 Elsevier B.V. All rights reserved.

Gardner's syndrome

International Nuclear Information System (INIS)

Sobrado Junior, C.W.; Bresser, A.; Cerri, G.G.; Habr-Gama, A.; Pinotti, H.W.; Magalhaes, A.

1988-01-01

A case of familiar poliposis of colon related to a right mandibular osteoma is reported (this association is usually called Gardner's syndrome). Radiologic pictures ae shown and some commentaries about this syndrome concerning the treatment are made. (author) [pt

Confirmation that RIPK4 mutations cause not only Bartsocas-Papas syndrome but also CHAND syndrome.

Science.gov (United States)

Busa, Tiffany; Jeraiby, Mohammed; Clémenson, Alix; Manouvrier, Sylvie; Granados, Viviana; Philip, Nicole; Touraine, Renaud

2017-11-01

CHAND syndrome is an autosomal recessive disorder characterized by curly hair, ankyloblepharon, and nail dysplasia. Only few patients were reported to date. A homozygous RIPK4 mutation was recently identified by homozygosity mapping and whole exome sequencing in three patients from an expanded consanguineous kindred with a clinical diagnosis of CHAND syndrome. RIPK4 was previously known to be implicated in Bartsocas-Papas syndrome, the autosomal recessive form of popliteal pterygium syndrome. We report here two cases of RIPK4 homozygous mutations in a fetus with severe Bartsocas-Papas syndrome and a patient with CHAND syndrome. The patient with CHAND syndrome harbored the same mutation as the one identified in the family previously reported. We thus confirm the implication of RIPK4 gene in CHAND syndrome in addition to Bartsocas-Papas syndrome and discuss genotype/phenotype correlations. © 2017 Wiley Periodicals, Inc.

Drug treatment of metabolic syndrome.

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Altabas, Velimir

2013-08-01

The metabolic syndrome is a constellation of risk factors for cardiovascular diseases including: abdominal obesity, a decreased ability to metabolize glucose (increased blood glucose levels and/or presence of insulin resistance), dyslipidemia, and hypertension. Patients who have developed this syndrome have been shown to be at an increased risk of developing cardiovascular disease and/or type 2 diabetes. Genetic factors and the environment both are important in the development of the metabolic syndrome, influencing all single components of this syndrome. The goals of therapy are to treat the underlying cause of the syndrome, to reduce morbidity, and to prevent complications, including premature death. Lifestyle modification is the preferred first-step treatment of the metabolic syndrome. There is no single effective drug treatment affecting all components of the syndrome equally known yet. However, each component of metabolic syndrome has independent goals to be achieved, so miscellaneous types of drugs are used in the treatment of this syndrome, including weight losing drugs, antidiabetics, antihypertensives, antilipemic and anticlothing drugs etc. This article provides a brief insight into contemporary drug treatment of components the metabolic syndrome.

Numerical Magnitude Processing Impairments in Genetic Syndromes: A Cross-Syndrome Comparison of Turner and 22Q11.2 Deletion Syndromes

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Brankaer, Carmen; Ghesquière, Pol; De Wel, Anke; Swillen, Ann; De Smedt, Bert

2017-01-01

Cross-syndrome comparisons offer an important window onto understanding heterogeneity in mathematical learning disabilities or dyscalculia. The present study therefore investigated symbolic numerical magnitude processing in two genetic syndromes that are both characterized by mathematical learning disabilities: Turner syndrome and 22q11.2 deletion…

Neonatal bartter syndrome

International Nuclear Information System (INIS)

Parkash, J.; Salat, S. M.; Khan, I.A.

2006-01-01

A pre-term baby girl was born following a pregnancy complicated by severe polyhydramnios at a gestational age of 36 weeks. She was initially suffering from respiratory distress consistent with idiopathic respiratory distress syndrome, and altered electrolyte imbalance with hyponatremia, hypokalemia and hypochloremic metabolic alkalosis. However, during the third week of life when she had dehydration along with significant electrolyte imbalance, Bartter's syndrome was considered which was supported by findings of high renin and aldosterone levels. Treatment was done by correction of electrolytes and dehydration along with indomethacin. The drug was well tolerated. The infant showed correction of electrolyte imbalance. The features of this case suggest an extreme form of Bartter's syndrome presenting from the early days of life. The syndrome is reported because of it's rarity and alerts pediatricians to the antenatal and neonatal variant of Bartter's syndrome. (author)

Kallmann syndrome and ichthyosis: a case of contiguous gene deletion syndrome

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Irene Berges-Raso

2017-09-01

Full Text Available Kallmann syndrome is a genetically heterogeneous form of hypogonadotropic hypogonadism caused by gonadotropin-releasing hormone deficiency and characterized by anosmia or hyposmia due to hypoplasia of the olfactory bulbs; osteoporosis and metabolic syndrome can develop due to longstanding untreated hypogonadism. Kallmann syndrome affects 1 in 10 000 men and 1 in 50 000 women. Defects in 17 genes, including KAL1, have been implicated. Kallmann syndrome can be associated with X-linked ichthyosis, a skin disorder characterized by early onset dark, dry, irregular scales affecting the limb and trunk, caused by a defect of the steroid sulfatase gene (STS. Both KAL1 and STS are located in the Xp22.3 region; therefore, deletions in this region cause a contiguous gene syndrome. We report the case of a 32-year-old man with ichthyosis referred for evaluation of excessive height (2.07 m and weight (BMI: 29.6 kg/m2, microgenitalia and absence of secondary sex characteristics. We diagnosed Kallmann syndrome with ichthyosis due to a deletion in Xp22.3, a rare phenomenon.

Neonatal respiratory distress syndrome

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Hyaline membrane disease (HMD); Infant respiratory distress syndrome; Respiratory distress syndrome in infants; RDS - infants ... improves slowly after that. Some infants with severe respiratory distress syndrome will die. This most often occurs ...

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

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Prabhas Prasun Giri

2011-01-01

Full Text Available Drug rash with eosinophilia and systemic symptoms (DRESS syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS and spontaneous air leak syndrome (pnemothorax and pneumomediastinum. She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.

MOMO Syndrome with Holoprosencephaly and Cryptorchidism: Expanding the Spectrum of the New Obesity Syndrome

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Sheetal Sharda

2011-01-01

Full Text Available There are multiple genetic disorders with known or unknown etiology grouped under obesity syndromes. Inspite of having multisystem involvement and often having a characteristic presentation, the understanding of the genetic causes in the majority of these syndromes is still lacking. The common obesity syndromes are Bardet-Biedl, Prader-Willi, Alstrom, Albright's hereditary osteodystrophy, Carpenter, Rubinstein-Taybi, Fragile X, and Börjeson-Forssman-Lehman syndrome. The list is ever increasing as new syndromes are being added to it. One of the recent additions is MOMO syndrome, with about five such cases being reported in literature. Expanding the spectrum of clinical features, we report the first case of MOMO syndrome from India with lobar variant of holoprosencephaly and cryptorchidism, which have not been reported previously.

Smoking-related interstitial lung diseases: histopathological and imaging perspectives

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Desai, S.R.; Ryan, S.M.; Colby, T.V

2003-04-01

The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed.

Smoking-related interstitial lung diseases: histopathological and imaging perspectives

International Nuclear Information System (INIS)

Desai, S.R.; Ryan, S.M.; Colby, T.V.

2003-01-01

The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed

Duane retraction syndrome type 1 with Usher syndrome type 2: an unreported association.

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Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

2015-05-07

Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. Copyright 2015, SLACK Incorporated.

Rare case of nephrotic syndrome: Schimke syndrome.

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Pedrosa, Anna Kelly Krislane de Vasconcelos; Torres, Luiz Fernando Oliveira; Silva, Ana Corina Brainer Amorim da; Dantas, Adrianna Barros Leal; Zuntini, Káthia Liliane da Cunha Ribeiro; Aguiar, Lia Cordeiro Bastos

2016-01-01

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case. Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Gorlin-goltz syndrome