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Sample records for bronchiolitis obliterans syndrome

  1. Bronchiolitis Obliterans

    Science.gov (United States)

    ... flavor in many foods and commonly used in e-cigarette flavoring, has been shown to cause bronchiolitis obliterans ... to avoid: Acetaldehyde Acetaldehyde is in cannabis and e-cigarette smoke. It irritates the skin, eyes, mucous membranes, ...

  2. Blood Gene Expression Predicts Bronchiolitis Obliterans Syndrome

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    Richard Danger

    2018-01-01

    Full Text Available Bronchiolitis obliterans syndrome (BOS, the main manifestation of chronic lung allograft dysfunction, leads to poor long-term survival after lung transplantation. Identifying predictors of BOS is essential to prevent the progression of dysfunction before irreversible damage occurs. By using a large set of 107 samples from lung recipients, we performed microarray gene expression profiling of whole blood to identify early biomarkers of BOS, including samples from 49 patients with stable function for at least 3 years, 32 samples collected at least 6 months before BOS diagnosis (prediction group, and 26 samples at or after BOS diagnosis (diagnosis group. An independent set from 25 lung recipients was used for validation by quantitative PCR (13 stables, 11 in the prediction group, and 8 in the diagnosis group. We identified 50 transcripts differentially expressed between stable and BOS recipients. Three genes, namely POU class 2 associating factor 1 (POU2AF1, T-cell leukemia/lymphoma protein 1A (TCL1A, and B cell lymphocyte kinase, were validated as predictive biomarkers of BOS more than 6 months before diagnosis, with areas under the curve of 0.83, 0.77, and 0.78 respectively. These genes allow stratification based on BOS risk (log-rank test p < 0.01 and are not associated with time posttransplantation. This is the first published large-scale gene expression analysis of blood after lung transplantation. The three-gene blood signature could provide clinicians with new tools to improve follow-up and adapt treatment of patients likely to develop BOS.

  3. Bronchiolitis obliterans in children with Stevens-Johnson syndrome: follow-up with high resolution CT

    International Nuclear Information System (INIS)

    Kim, M.J.; Lee, K.Y.

    1996-01-01

    About one third of children with Stevens-Johnson syndrome have pulmonary involvement. As a consequence of airway epithelial injury, bronchiolitis obliterans can occur in these patients. Two cases of Stevens-Johnson syndrome-associated bronchiolitis obliterans in children were diagnosed and followed by high resolution CT without open lung biopsy. Serial changes of high resolution CT features of bronchiolitis obliterans are discussed and the literature is reviewed. (orig.)

  4. Metalloproteinase Profiling in Lung Transplant Recipients With Good Outcome and Bronchiolitis Obliterans Syndrome

    NARCIS (Netherlands)

    Heijink, Irene H.; Rozeveld, Dennie; van der Heide, Sicco; Bij, van der Wim; Bischoff, Rainer; Oosterhout, van Antoon J,; van der Toorn, Marco

    Background. Bronchiolitis obliterans syndrome (BOS), the major cause of death on lung transplantation, is characterized by bronchiolar inflammation and tissue remodeling. Matrix metalloproteinases (MMPs) have been implicated in these processes, although it is still unclear whether MMP activity and

  5. Parametric Response Mapping as an Indicator of Bronchiolitis Obliterans Syndrome after Hematopoietic Stem Cell Transplantation

    NARCIS (Netherlands)

    Galban, Craig J.; Boes, Jennifer L.; Bule, Maria; Kitko, Carrie L.; Couriel, Daniel R.; Johnson, Timothy D.; Lama, Vihba; Telenga, Eef D.; van den Berge, Maarten; Rehemtulla, Alnawaz; Kazerooni, Ella A.; Ponkowski, Michael J.; Ross, Brian D.; Yanik, Gregory A.

    2014-01-01

    The management of bronchiolitis obliterans syndrome (BOS) after hematopoietic cell transplantation presents many challenges, both diagnostically and therapeutically. We developed a computed tomography (CT) voxel-wise methodology termed parametric response mapping (PRM) that quantifies normal

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

    Science.gov (United States)

    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.

  7. Lung transplantation for bronchiolitis obliterans syndrome after allo-SCT.

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    Holm, A M; Riise, G C; Hansson, L; Brinch, L; Bjørtuft, O; Iversen, M; Simonsen, S; Fløisand, Y

    2013-05-01

    Chronic GVHD (cGVHD) associated bronchiolitis obliterans syndrome (BOS) is a serious complication after allo-SCT, and lung transplantation (LTx) may be the ultimate treatment option. To evaluate this treatment, data on all patients with LTx after allo-SCT ever performed in Sweden, Norway, Denmark 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 cGVHD, with median interval from allo-SCT to LTx of 8.2 (0.7-16) years. Median age at LTx was 34 (16-55) years, and the median postoperative observation time was 4.2 (0.1-15) years. Two patients died, one due to septicemia, the other of relapsing leukemia, after 2 and 14 months, respectively. Four developed BOS, one of these was retransplanted. The survival did not significantly differ from the survival in matched LTx controls, being 90% 1 year and 75% 5 years after LTx compared with 85% and 68% in the controls. We therefore suggest that LTx may be considered in carefully selected patients with BOS due to cGVHD after allo-SCT.

  8. Biomarkers for the prediction of the bronchiolitis obliterans syndrome after lung transplantation

    NARCIS (Netherlands)

    Paantjens, A.W.M.

    2011-01-01

    The main limitation for overall survival after lung transplantation (LTx) is the development of chronic rejection, which is represented by the bronchiolitis obliterans syndrome (BOS). The diagnosis BOS is based on lung function testing, however, it is a surrogate marker. And because BOS is an

  9. Bronchiolitis obliterans syndrome after lung transplantation and health-related quality of life

    NARCIS (Netherlands)

    van den Berg, JWK; van der Bij, W; Koeter, GH; Postma, DS; ten Vergert, EM

    The present study was undertaken to assess the relationship between health-related quality of life (HRQOL) and bronchiolitis obliterans syndrome (BOS), as both represent important parameters of outcome after lung transplantation. HRQOL was measured both cross-sectionally and longitudinally by

  10. Bronchiolitis obliterans syndrome: thin-section CT diagnosis of obstructive changes in infants and young children after lung transplantation.

    Science.gov (United States)

    Lau, D M; Siegel, M J; Hildebolt, C F; Cohen, A H

    1998-09-01

    To characterize the thin-section computed tomographic (CT) appearance of bronchiolitis fibrosa obliterans syndrome in infants and young children after lung transplantation. Thin-section CT studies in six patients with bronchiolitis obliterans syndrome (age range, 2 months to 5 1/2 years) and in 15 control patients without obstructive airway disease (age range, 2 months to 7 years) who underwent bilateral lung transplantation were retrospectively reviewed. The thin-section CT scans were obtained during quiet sleep at a median of 24 months (range, 6-36 months) after transplantation. The CT studies were evaluated for mosaic perfusion, bronchial dilatation, bronchial wall thickening, and mucous plugging Final diagnoses in all patients were based pulmonary function test results. Thin-section CT findings in the six patients with clinically proved bronchiolitis obliterans syndrome were mosaic perfusion in five (83%) bronchial dilation in three (50%), and bronchial wall thickening in one (17%). Of the 15 control patients with normal pulmonary function test results, six (40%) had mosaic perfusion; none had bronchial dilatation or bronchial wall thickening. Mucous plugging was not seen in either group. Only the association of bronchial dilatation with bronchiolitis obliterans syndrome was significant (P = .02). Infants and young children with bronchiolitis obliterans syndrome after lung transplantation are more likely to have CT abnormalities than those with normal pulmonary function test results.

  11. Gastroesophageal reflux disease and its association with bronchiolitis obliterans syndrome in allogeneic hematopoietic stem cell transplant recipients.

    Science.gov (United States)

    Khalid, Mohammed; Aljurf, Mahmoud; Saleemi, Sarfraz; Khan, Mohammed Qaseem; Khan, Basha; Ahmed, Shad; Ibrahim, Khalid El Tayeb; Mobeireek, Abdullah; Al Mohareb, Fahad; Chaudhri, Naeem

    2013-06-01

    Bronchiolitis obliterans syndrome is a significant postallogeneic hematopoietic stem cell transplant problem. Recent data in lung transplant patients suggest an association with gastroesophageal reflux disease and bronchiolitis obliterans syndrome. We studied posthematopoietic stem cell transplant patients with bronchiolitis obliterans syndrome for gastroesophageal reflux disease and its response to a proton pump inhibitor. Seven postallogeneic hematopoietic stem cell transplant patients with bronchiolitis obliterans syndrome were studied. Gastroesophageal reflux disease was assessed by 24-hour pH monitoring with a Bravo catheter-free radio pH capsule. Patients with positive gastroesophageal reflux disease were started on omeprazole. Pretreatment and posttreatment pulmonary function tests were done at 3-month intervals. Of 7 patients, 5 had positive results for gastroesophageal reflux disease (71%). Omeprazole had a disease-stabilizing effect on the patients' pulmonary function tests. Our study shows a significant association between bronchiolitis obliterans syndrome and gastroesophageal reflux disease in postallogeneic hematopoietic stem cell transplant patients. Use of omeprazole may have a disease-stabilizing effect in short-term follow-up.

  12. Bronchiolitis obliterans organising pneumonia associated with anticonvulsant hypersensitivity syndrome induced by lamotrigine.

    Science.gov (United States)

    Ghandourah, Hasan; Bhandal, Samarjeet; Brundler, Marie-Anne; Noseworthy, Mary

    2016-01-29

    A 14-year-old girl who was known to have a seizure disorder and on lamotrigine treatment was admitted to the hospital, with a history of rash, fever and cough. Her condition deteriorated with clinical features suggestive of anticonvulsant hypersensitivity syndrome (ACHS) complicated with bronchiolitis obliterans organising pneumonia (BOOP). Her chest CT showed multifocal parenchymal opacities and lung biopsy was typical for BOOP. Initially, the lamotrigine was discontinued since the onset of the rash, then she was treated for pneumonia with antibiotics, which may have delayed the diagnosis. Eventually, BOOP was considered and she was treated with a high dose of corticosteroid. She improved clinically and her repeated chest CT showed a marked resolution of the lesions. This case illustrates the possible occurrence of BOOP as a complication of ACHS secondary to lamotrigine treatment. 2016 BMJ Publishing Group Ltd.

  13. 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...

  14. Bronchiolitis Obliterans with Organizing Pneumonia (BOOP)

    Science.gov (United States)

    ... What can you tell me about cryptogenic organizing pneumonia? Answers from Teng Moua, M.D. Previously called bronchiolitis obliterans with organizing pneumonia, cryptogenic organizing pneumonia (COP) is a rare lung ...

  15. Clinical investigation of predictors of radiation-induced bronchiolitis obliterans organizing pneumonia syndrome after breast-conserving therapy

    International Nuclear Information System (INIS)

    Matsuyama, Tomohiko; Furusawa, Mitsuhiro; Yasunaga, Tadamasa; Nishimura, Reiki; Ohya, Natsuo

    2011-01-01

    We investigated 710 patients with breast cancer who received radiotherapy after breast-conserving surgery at our institution to evaluate the incidence of radiation-induced bronchiolitis obliterans organizing pneumonia (BOOP) syndrome focusing on the interval from irradiation to onset and the clinical presentation. The predictive value of age (≤50 or >50), chemotherapy and hormone therapy was statistically analyzed to determine whether these are risk factors for BOOP syndrome. Radiation-induced BOOP syndrome was seen in 1.3% (9/710). In most cases, the symptoms were mild and none of the patients required hospitalization. Eight patients (88.9%) responded well to steroid administration, but 5 of these patients relapsed after or during tapering of steroids. Although we could not detect significant risk factors for BOOP syndrome, a higher patient age was associated with a higher incidence of radiation-induced BOOP syndrome after breast-conserving therapy. (author)

  16. 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

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

    Science.gov (United States)

    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

  18. Bortezomib-Induced Bronchiolitis Obliterans Organizing Pneumonia

    Directory of Open Access Journals (Sweden)

    E. Vandeix

    2012-01-01

    Full Text Available Introduction. Bortezomib is a proteasome inhibitor indicated for the treatment of multiple myeloma patients. The most frequent side effects are gastrointestinal and neurological. Serious pulmonary complications have been described rarely. Observation. This case involves a 74-year-old man suffering from IgG Kappa myeloma treated with bortezomib, melphalan, and dexamethasone. After administering chemotherapy, the patient developed an acute respiratory distress syndrome (ARDS. A surgical pulmonary biopsy proved the existence of bronchiolitis obliterans organizing pneumonia (BOOP lesions. Systemic corticotherapy led to a rapid improvement in the patient’s condition. Conclusion. This is the first reported histologically confirmed case of bortezomid-induced BOOP. Faced with severe respiratory symptoms in the absence of other etiologies, complications due to bortezomid treatment should be evoked and corticotherapy considered.

  19. Performance of long-term CT monitoring in diagnosing bronchiolitis obliterans after lung transplantation

    Energy Technology Data Exchange (ETDEWEB)

    Berstad, Audun E. [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway)]. E-mail: a.e.berstad@medisin.uio.no; Aalokken, Trond Mogens [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway); Kolbenstvedt, Alf [Department of Radiology, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway); Bjortuft, Oystein [Department of Thoracic Medicine, Rikshospitalet University Hospital, Sognsvannsveien 20, N-0027 Oslo (Norway)

    2006-04-15

    Aim: The purpose of the study was to evaluate the ability of CT, including expiratory scans with minimum intensity projection in predicting the development of bronchiolitis obliterans syndrome after lung transplantation. Materials and methods: Forty consecutive patients, 29 bilateral and 11 single lung transplanted, were followed-up with regular scans for a median of 36 months. Air trapping was evaluated on expiratory scans constructed from two short spiral scans with minimum intensity projection-technique, one at the level of the carina and the other midway between the right diaphragm and the carina. Air trapping was scored on a 16-point scale. Bronchiolitis obliterans syndrome was diagnosed according to established clinical criteria and quantified spirometrically. Results: Bronchiolitis obliterans syndrome developed in 17 patients (43%) after a median of 12 months. Air trapping and bronchiectasis was seen before the diagnosis of bronchiolitis obliterans syndrome in only two and one patient, respectively. Interobserver agreement for air trapping score was good (kappa = 0.65). Air trapping scores performed significantly better than that achieved by chance alone in determining the presence of bronchiolitis obliterans syndrome (P = 0.0025). An air trapping score of 4 or more provided the best results with regard to sensitivity and specificity in diagnosing bronchiolitis obliterans syndrome. The sensitivity, specificity, positive and negative predictive values of an air trapping of 4 or more in the diagnosis of bronchiolitis obliterans syndrome were 77, 74, 68 and 81%, respectively. Conclusion: Expiratory CT scans with minimum intensity projection-reconstruction did not predict the development of bronchiolitis obliterans syndrome in most patients. The findings seriously limit the clinical usefulness of long-term CT monitoring for diagnosing bronchiolitis obliterans syndrome after lung transplantation.

  20. 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.

  1. Conservation of small-airway function by tacrolimus/cyclosporine conversion in the management of bronchiolitis obliterans following lung transplantation.

    Science.gov (United States)

    Revell, M P; Lewis, M E; Llewellyn-Jones, C G; Wilson, I C; Bonser, R S

    2000-12-01

    We studied serial lung function in 11 patients with bronchiolitis obliterans syndrome who were treated with tacrolimus conversion following lung or heart-lung transplantation. Our results show that tacrolimus conversion slows the decline of lung function in bronchiolitis obliterans syndrome. The attenuation continues for at least 1 year following conversion.

  2. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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

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

    Science.gov (United States)

    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.

  7. Clinical and radiological features of bronchiolitis obliterans in children

    International Nuclear Information System (INIS)

    Peng Yun; Sun Guoqiang; Zeng Jinjin; Ma Daqing

    2006-01-01

    Objective: To study the value of chest radiograph and thin-section computed tomography (CT) in diagnosis bronchiolitis obliterans in children, and to determine clinical view of obliterative bronchiolitis in children. Methods: We identified 12 infants, 10 boys, and 2 girls (age range, 5 month to 11 years) with clinical confirmation of bronchiolitis obliterans. Three cases were after Steven-Johnson syndrome, 8 were post-infection (2 adenovirus, 2 measles and 1 Pseudomonas aeruginosa infection, 3 cases were unknown etiology infection); The symptoms lasted for at least 6 weeks. One case had lung ventilation nuclear scan. We evaluated individual bronchoscopy, pulmonary function test, chest radiograph and thin- section CT features and their characteristic appearance. Results: All cases had typical clinical characteristics and pulmonary function testing results that were consistent with nonreversible small airways obstruction. One case had lung ventilation nuclear scan illustrated absent and reduced ventilation of the right lower lobe. Nine cases who underwent bronchoscopy were chronic endobronchial inflammation. Three children had transbronchial biopsy and 1 patient who underwent open pulmonary biopsies were uncertain of histological diagnosis. Chest radiography showed hyperinflation in 8 cases; peribronchial thickening in 6 cases; consolidation/atelectasia in 6 cases; unilateral hyperlucency of a small/normal-sized lung in 4 cases. Thin-section CT/HRCT features included: mosaic perfusion pattern, decreased lung attenuation in 11 cases, pulmonary vascular attenuation in 10 cases; bronchial dilatation in 7 cases; bronchial wall thickening in 9 cases; unilateral hyperlucency of a small/normal-sized lung in 5 cases; consolidation in 6 cases; nodular in 3 cases; mucoid impaction in 5 cases. Conclusions: In our study, correct diagnoses of bronchiolitis obliterans in children were made more special with thin-section CT than with chest radiographs. The diagnosis of BO in

  8. Bronchiolitis obliterans organising pneumonia in patients taking acebutolol or amiodarone.

    Science.gov (United States)

    Camus, P; Lombard, J N; Perrichon, M; Piard, F; Guérin, J C; Thivolet, F B; Jeannin, L

    1989-01-01

    Two patients, treated with acebutolol and amiodarone respectively, developed a disease clinically, radiologically, and pathologically indistinguishable from bronchiolitis obliterans organising pneumonia. In one case recovery followed discontinuation of acebutolol; in the other case cessation of amiodarone had no effect, and corticosteroids were required. In addition to these patients, several cases of bronchiolitis obliterans organising pneumonia have been reported during treatment with gold salts, amiodarone, and miscellaneous other drugs. Taken together, this information supports the view that bronchiolitis obliterans organising pneumonia may be a form of response by the lungs to insult by drugs. Images PMID:2588206

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

    Science.gov (United States)

    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

  10. Nitrofurantoin-Associated Bronchiolitis Obliterans Organizing Pneumonia: Report of a Case

    Directory of Open Access Journals (Sweden)

    Mark E Fenton

    2008-01-01

    Full Text Available Bronchiolitis obliterans organizing pneumonia due to nitrofurantoin has rarely been reported and is associated with poor outcomes. A case of nitrofurantoin-associated bronchiolitis obliterans organizing pneumonia responsive to drug withdrawal and corticosteroids is presented.

  11. 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.

    Science.gov (United States)

    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.

  12. Quinine-induced bronchiolitis obliterans and organizing pneumonia

    Directory of Open Access Journals (Sweden)

    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.

  13. Bronchiolitis obliterans organising pneumonia: a consequence of breast radiotherapy.

    Science.gov (United States)

    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.

  14. 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.

  15. Oleic acid-associated bronchiolitis obliterans-organizing pneumonia in beagle dogs.

    Science.gov (United States)

    Li, X; Botts, S; Morton, D; Knickerbocker, M J; Adler, R

    2006-03-01

    Accidental intra-airway exposure of dogs with pure oleic acid produced bronchiolitis obliterans and bronchopneumonia. Pulmonary changes included multifocal to coalescing necrosis of bronchioles and adjacent alveoli, hemorrhage, inflammation, and exudation of fibrin. Hyperplasia of bronchiolar and alveolar epithelial cells and proliferation of loose fibrovascular connective tissue formed polyps or plugs of variable size and shape. Polyps in the airways primarily consisted of fibroblasts with loose or myxoid stroma and were variably covered with attenuated epithelial cells. Some polyps had prominent vasculature, mixed inflammatory cell infiltration, and/or necrosis. Polyps or plugs variably effaced bronchioles and adjacent alveoli. The changes closely resembled human bronchiolitis obliterans-organizing pneumonia (BOOP). Controlled intra-airway delivery of oleic acid in dogs may be a potential animal model of obstructive pulmonary diseases such as BOOP or bronchiolitis obliterans.

  16. Bronchiolitis obliterans organizing pneumonia: experience at three hospitals in Riyadh

    International Nuclear Information System (INIS)

    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)

  17. Bronchiolitis obliterans after allogenic bone marrow transplantation: HRCT findings

    International Nuclear Information System (INIS)

    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

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  19. 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

  20. 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.

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    DEFF Research Database (Denmark)

    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....

  3. 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.)

  4. 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)

  5. 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.)

  6. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2004-11-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    International Nuclear Information System (INIS)

    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)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  13. Bronchiolitis obliterans organizing pneumonia–Experience of a pulmonology ward

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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

  15. 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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  17. 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...

  18. 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

    Energy Technology Data Exchange (ETDEWEB)

    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)

  19. 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)

  20. 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...

  1. 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 ...

  2. 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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  5. 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.

  6. 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.)

  7. 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.

  8. 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

  9. Bronchiolitis from nitrous fumes

    Energy Technology Data Exchange (ETDEWEB)

    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.

  10. 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

  11. 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

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

    DEFF Research Database (Denmark)

    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...

  13. 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.)

  14. 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.

  15. 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 ...

  16. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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 ...

  19. 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

  20. 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

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

    Science.gov (United States)

    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.

  2. 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.)

  3. [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.

  4. 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.

  5. 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.

  6. 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

  7. 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.)

  8. 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.)

  9. 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- ...

  10. 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

  11. 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.

  12. 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.

  13. 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

  14. 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

  15. 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

  16. 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.

  17. 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)

  18. 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)

  19. 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.

  20. 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.

  1. Oxidative and antioxidative status of children with acute bronchiolitis

    Directory of Open Access Journals (Sweden)

    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.

  2. 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...

  3. 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

  4. 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.

  5. 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.

  6. [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.

  7. Obliterative bronchiolitis due to Mycoplasma pneumoniae infection in a child

    Energy Technology Data Exchange (ETDEWEB)

    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.

  8. 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...

  9. 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...

  10. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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.

  13. Oxidative and antioxidative status of children with acute bronchiolitis

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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.

  15. 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.

  16. Granulomatous Bronchiolitis with Necrobiotic Pulmonary Nodules in Chrohn's Disease

    Directory of Open Access Journals (Sweden)

    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.

  17. Herpes simplex virus bronchiolitis in a cannabis user

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

  19. 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.

  20. 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.

  1. 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)

  2. CT findings of respiratory bronchiolitis caused by cigarette smoking

    Energy Technology Data Exchange (ETDEWEB)

    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)

  3. 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)

  4. 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

  5. C Reactive protein and disease severity in bronchiolitis

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

  7. 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.

  8. 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

  9. 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

  10. Prevention and treatment of respiratory syncytial virus bronchiolitis and postbronchiolitic wheezing

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    OpenAIRE

    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...

  14. 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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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

  19. 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

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

    Directory of Open Access Journals (Sweden)

    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

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

    International Nuclear Information System (INIS)

    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)

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

    Science.gov (United States)

    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.

  3. 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.

  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...

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

    Science.gov (United States)

    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

  6. 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

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

    Science.gov (United States)

    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.

  8. 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.

  9. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  12. 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.

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

    Science.gov (United States)

    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

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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

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

    Directory of Open Access Journals (Sweden)

    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

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

    Science.gov (United States)

    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

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

    International Nuclear Information System (INIS)

    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)

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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)

  1. 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

  2. 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.)

  3. 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.

  4. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  7. 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.

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

    Science.gov (United States)

    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.

  9. 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

  10. 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.

  11. 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

  12. 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...

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

    Science.gov (United States)

    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.

  14. 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

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

    Science.gov (United States)

    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.

  16. 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.

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

    Science.gov (United States)

    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.

  18. Rib fractures after chest physiotherapy for bronchiolitis or pneumonia in infants

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  19. 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.)

  20. 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.

  1. 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)

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  7. 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...

  8. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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

  11. 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...

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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...

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

    Directory of Open Access Journals (Sweden)

    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

  1. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  5. 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)

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  15. 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

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

    International Nuclear Information System (INIS)

    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

  17. 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.

  18. 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.

  19. 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®.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

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

    International Nuclear Information System (INIS)

    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)

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

  3. 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....

  4. 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

  5. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  7. 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 ...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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.

  13. Beals Syndrome

    Science.gov (United States)

    ... 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. ...

  14. Pulmonary manifestations of Sjögren's syndrome

    Directory of Open Access Journals (Sweden)

    Thomas Flament

    2016-06-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

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

    Science.gov (United States)

    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.

  19. 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 ...

  20. 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 -- ...

  1. 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.

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

    Directory of Open Access Journals (Sweden)

    Parashari Umesh

    2010-01-01

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

  3. 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 ...

  4. 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), ...

  5. 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...

  6. 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

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

    Directory of Open Access Journals (Sweden)

    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

  8. Bronchiolitis (For Parents)

    Science.gov (United States)

    ... flu) , human metapneumovirus, adenovirus , coronavirus, and parainfluenza. Childcare attendance and exposure to cigarette smoke also can increase ... View more About Us Contact Us Partners Editorial Policy Permissions Guidelines Privacy Policy & Terms of Use Notice ...

  9. 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 ...

  10. 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 ...

  11. 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 ...

  12. 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.

  13. 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. ...

  14. 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

    Directory of Open Access Journals (Sweden)

    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.

  15. Hepatorenal syndrome

    Science.gov (United States)

    ... 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, ...

  16. Belatacept for Maintenance Immunosuppression in Lung Transplantation

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  18. 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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

  20. Cushing's Syndrome

    Science.gov (United States)

    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 ...

  1. Usher Syndrome

    Science.gov (United States)

    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 ...

  2. Metabolic Syndrome

    Science.gov (United States)

    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 ...

  3. Reye Syndrome

    Science.gov (United States)

    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, ...

  4. Rett Syndrome

    Science.gov (United States)

    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. ...

  5. Caplan syndrome

    Science.gov (United States)

    ... 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 ...

  6. Turner Syndrome

    Science.gov (United States)

    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 ...

  7. 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

  8. Sotos Syndrome

    Science.gov (United States)

    ... 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 ...

  9. Felty syndrome

    Science.gov (United States)

    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 ...

  10. Bartter syndrome

    Science.gov (United States)

    ... 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 ...

  11. Pendred Syndrome

    Science.gov (United States)

    ... 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 ...

  12. Dravet Syndrome

    Science.gov (United States)

    ... 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 ...

  13. 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

    Directory of Open Access Journals (Sweden)

    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

  14. 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 ...

  15. Rowell syndrome

    Directory of Open Access Journals (Sweden)

    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.

  16. 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 ...

  17. 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)

  18. 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)

  19. 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

  20. 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...

  1. 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...

  2. Urofacial syndrome

    Directory of Open Access Journals (Sweden)

    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.

  3. 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...

  4. Revesz syndrome

    Directory of Open Access Journals (Sweden)

    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.

  5. Reye's Syndrome

    Science.gov (United States)

    ... that contain aspirin. Some hospitals and medical facilities conduct newborn screenings for fatty acid oxidation disorders to determine which children are at greater risk of developing Reye's syndrome. ...

  6. Marfan Syndrome (For Teens)

    Science.gov (United States)

    ... 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 ...

  7. 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 ...

  8. 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 ...

  9. 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 ...

  10. 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...

  11. Burnout Syndrome

    OpenAIRE

    Panova, Gordana; Panov, Nenad; Stojanov, H; Sumanov, Gorgi; Panova, Blagica; Stojanovski, Angel; Nikolovska, Lence; Jovevska, Svetlana; Trajanovski, D; Asanova, D

    2013-01-01

    Introduction: Increasing work responsibilities, allocation of duties, loss of energy and motivation in everyday activities, emotional exhaustion, lack of time for themselves, insuffi cient time for rest and recreation, dissatisfaction in private life. All these symptoms can be cause of Burnout Syndrome. Aim: To see the importance of this syndrome, the consequences of job dissatisfaction, the environment, family and expression in drastic chan...

  12. Tourette Syndrome

    Science.gov (United States)

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics are throat- ... spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. It ...

  13. Fahr's Syndrome

    Science.gov (United States)

    ... or 50s, although it can occur at any time in childhood or adolescence. × Definition Fahr's Syndrome is a rare, genetically dominant, inherited ... or 50s, although it can occur at any time in childhood or adolescence. View Full Definition Treatment There is no cure for Fahr's Syndrome, ...

  14. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine; Bødtger, Uffe; Heltberg, Ole

    2014-01-01

    Lemierre's syndrome is an often un-diagnosed disease seen in previously healthy young subjects, presenting with symptoms of pharyngitis, fever and elevated markers of inflammation. The syndrome is characterised by infectious thrombosis of the jugular vein due to infection with Fusobacteria, causing...

  15. Ambras syndrome

    Directory of Open Access Journals (Sweden)

    Sudhir Malwade

    2015-01-01

    Full Text Available Ambras syndrome, a form of congenital hypertrichosis lanuginosa, is extremely rare in neonates. It is characterized by typical pattern of hair distribution, dysmorphic facial features and a familial pattern of inheritance. We report a case of Ambras syndrome in a preterm neonate with history of consanguinity and positive family history.

  16. Antiphospholipid syndrome

    DEFF Research Database (Denmark)

    Cervera, Ricard; Piette, Jean-Charles; Font, Josep

    2002-01-01

    To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression.......To analyze the clinical and immunologic manifestations of antiphospholipid syndrome (APS) in a large cohort of patients and to define patterns of disease expression....

  17. Noonan syndrome

    OpenAIRE

    Roberts, Amy E; Allanson, Judith E; Tartaglia, Marco; Gelb, Bruce D

    2013-01-01

    Noonan syndrome is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, lymphatic malformations, and bleeding difficulties. Mutations that cause Noonan syndrome alter genes encoding proteins with roles in the RAS–MAPK pathway, leading to pathway dysregulation. Management guidelines have been developed. Several clinically relevant genotype–phenotype correlations aid ris...

  18. 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.

  19. Goldenhar syndrome

    Directory of Open Access Journals (Sweden)

    Neeraj Sharma

    2013-01-01

    Full Text Available Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area.

  20. Cowden syndrome

    Directory of Open Access Journals (Sweden)

    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.

  1. Costello syndrome

    Directory of Open Access Journals (Sweden)

    Madhukara J

    2007-01-01

    Full Text Available Costello syndrome is a rare, distinctive, multiple congenital anomaly syndrome, characterized by soft, loose skin with deep palmar and plantar creases, loose joints, distinctive coarse facial features and skeletal and cardiac abnormalities. The affected patients have a predisposition to develop malignancy, developmental delays and mental retardation. Recently, a 7-year-old male child born to normal nonconsanguineous parents presented to us with abnormal facial features, arrhythmia, mitral valve dysfunction and growth retardation. His cutaneous examination revealed lax and pigmented skin over hands and feet with deep creases, acanthosis nigricans and short curly hairs. Its differentiation from other syndromes with similar clinical features is discussed in this article.

  2. Increased protein-energy intake promotes anabolism in critically ill infants with viral bronchiolitis: a double‑blind randomised controlled trial

    Science.gov (United States)

    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

  3. 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 ...

  4. 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 ...

  5. Reye Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now What Is Reye’s Syndrome? ...

  6. Alagille Syndrome

    Science.gov (United States)

    ... Legacy Society Make Gifts of Stock Donate Your Car Personal Fundraising Partnership & Support Share Your Story Spread the Word Give While You Shop Contact Us Donate Now Alagille Syndrome Back Alagille ...

  7. Turner Syndrome

    Science.gov (United States)

    ... Failure to begin sexual changes expected during puberty Sexual development that "stalls" during teenage years Early end to menstrual cycles not due to pregnancy For most women with Turner syndrome, inability to ...

  8. [Refeeding syndrome].

    Science.gov (United States)

    Ševela, Stanislav; Novák, František; Kazda, Antonín; Brodská, Helena

    Despite being known more than 60 years, refeeding syndrome (RS) still bears many uncertainties. For example, its definition is not clear and definite, and the attitude to it varies from the complete neglect to over-prevention.The term "refeeding syndrome" refers to electrolyte and metabolic changes occurring in malnourished patients after the readministration of nutrition. These changes concern especially to phosphates and ions. Potassium, magnesium, naturism and fluids balance are involved. The changes lead to cell energetic metabolism and electric potential disturbances, with related clinical symptoms.Fully developed refeeding syndrome is quite rare; nevertheless it can be fatal for the patient. However, even its development can lead to many complications increasing the patient's morbidity and the length of stay in the hospital. Yet the refeeding syndrome is more or less predictable and if kept in mind also preventable.The aim of this article is to get the reader to know more about this metabolic phenomenon and possible attitudes towards it.

  9. Cockayne syndrome

    DEFF Research Database (Denmark)

    Karikkineth, Ajoy C; Scheibye-Knudsen, Morten; Fivenson, Elayne

    2017-01-01

    Cockayne syndrome (CS) is a disorder characterized by a variety of clinical features including cachectic dwarfism, severe neurological manifestations including microcephaly and cognitive deficits, pigmentary retinopathy, cataracts, sensorineural deafness, and ambulatory and feeding difficulties...

  10. Alagille Syndrome

    Science.gov (United States)

    ... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

  11. Reye Syndrome

    Science.gov (United States)

    ... Liver Function Tests Clinical Trials Liver Transplant FAQs Medical Terminology Diseases of the Liver Alagille Syndrome Alcohol-Related ... the Liver The Progression of Liver Disease FAQs Medical Terminology HOW YOU CAN HELP Sponsorship Ways to Give ...

  12. Turner Syndrome

    Science.gov (United States)

    ... crowding, and osteoporosis (brittle bones). Because of their physical conditions, health concerns, and infertility, some girls and women with TS may have low self- esteem, anxiety, or depression. How is Turner syndrome diagnosed? Physical features may ...

  13. Cushing's Syndrome

    Science.gov (United States)

    ... person cured of Cushing’s syndrome might have some memory loss and slight mental decline. But the change is ... Categories: Family Health, Infants and Toddlers, Kids and Teens, Men, Seniors, WomenTags: acth, adenomas, hormone, sickness September ...

  14. Levator Syndrome

    Science.gov (United States)

    ... Abscess Anorectal Fistula Foreign Objects in the Rectum Hemorrhoids Levator Syndrome Pilonidal Disease Proctitis Rectal Prolapse (See ... out other painful rectal conditions (such as thrombosed hemorrhoids , fissures , or abscesses ). The physical examination is often ...

  15. Alport Syndrome

    Science.gov (United States)

    ... signs and symptoms may differ, based on age, gender and inherited type of Alport syndrome. For example, ... prevention and treatment of kidney disease. The Better Business Bureau Wise Giving Alliance Charity Seal provides the ...

  16. Gilbert's Syndrome

    Science.gov (United States)

    ... not know you have the condition until it's discovered by accident, such as when a blood test ... chemotherapy drug Some protease inhibitors used to treat HIV If you have Gilbert's syndrome, talk to your ...

  17. Potter syndrome

    Science.gov (United States)

    Potter phenotype ... In Potter syndrome, the primary problem is kidney failure. The kidneys fail to develop properly as the baby is ... kidneys normally produce the amniotic fluid (as urine). Potter phenotype refers to a typical facial appearance that ...

  18. Moebius Syndrome

    Science.gov (United States)

    ... delays; high or cleft palate; hearing problems and speech difficulties. Children with Moebius syndrome are unable to move their eyes back and forth. Decreased numbers of muscle fibers have been reported. Deformities of the tongue, jaw, and limbs, such ...

  19. Fraser syndrome

    DEFF Research Database (Denmark)

    Barisic, Ingeborg; Odak, Ljubica; Loane, Maria

    2013-01-01

    Fraser syndrome is a rare autosomal recessive disorder characterized by cryptophthalmos, cutaneous syndactyly, laryngeal, and urogenital malformations. We present a population-based epidemiological study using data provided by the European Surveillance of Congenital Anomalies (EUROCAT) network of...

  20. Angelman Syndrome

    Science.gov (United States)

    ... therapy for seizures is usually necessary. Physical and occupational therapies, communication therapy, and behavioral therapies are important in allowing individuals with Angelman syndrome to reach their maximum developmental potential. × Treatment There ...

  1. Joubert Syndrome

    Science.gov (United States)

    ... CEP290 . View Full Definition Treatment Treatment for Joubert syndrome is symptomatic and supportive. Infant stimulation and physical, occupational, and speech therapy may benefit some children. Infants with abnormal breathing ...

  2. Zellweger Syndrome

    Science.gov (United States)

    ... swallow. Some babies will be born with glaucoma, retinal degeneration, and impaired hearing. Jaundice and gastrointestinal bleeding also may occur. Treatment There is no cure for Zellweger syndrome, nor ...

  3. Nephrotic Syndrome

    Science.gov (United States)

    ... your blood — typically with an artificial kidney machine (dialyzer). Chronic kidney disease. Nephrotic syndrome may cause your ... opportunities Reprint Permissions A single copy of these materials may be reprinted for noncommercial personal use only. " ...

  4. Ohtahara Syndrome

    Science.gov (United States)

    ... are more often affected than girls. View Full Definition Treatment Antiepileptic drugs are used to control seizures, but are unfortunately ... Other therapies are symptomatic and supportive. × ... Definition Ohtahara syndrome is a neurological disorder characterized by ...

  5. Usher Syndrome

    Science.gov (United States)

    ... to abnormal development of the vestibular hair cells, sensory cells that detect gravity and head movement. RP ... 3 Ben-Rebeh, I., et al. (2016). Genetic analysis of Tunisian families with Usher syndrome type 1: ...

  6. Eagle's Syndrome

    OpenAIRE

    Pinheiro,Thaís Gonçalves; Soares,Vítor Yamashiro Rocha; Ferreira,Denise Bastos Lage; Raymundo,Igor Teixeira; Nascimento,Luiz Augusto; Oliveira,Carlos Augusto Costa Pires de

    2013-01-01

    Summary Introduction:?Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is diffic...

  7. Barth Syndrome

    DEFF Research Database (Denmark)

    Saric, Ana; Andreau, Karine; Armand, Anne-Sophie

    2016-01-01

    Mutations in the gene encoding the enzyme tafazzin, TAZ, cause Barth syndrome (BTHS). Individuals with this X-linked multisystem disorder present cardiomyopathy (CM) (often dilated), skeletal muscle weakness, neutropenia, growth retardation, and 3-methylglutaconic aciduria. Biopsies of the heart......, liver and skeletal muscle of patients have revealed mitochondrial malformations and dysfunctions. It is the purpose of this review to summarize recent results of studies on various animal or cell models of Barth syndrome, which have characterized biochemically the strong cellular defects associated...

  8. Pendred's syndrome

    International Nuclear Information System (INIS)

    Hashmi, M.I.; Cheema, I.A.; Qasim, G.

    2003-01-01

    This report describes Pendred's syndrome in three siblings of a consanguineous marriage, belonging to Rahimyar Khan. The children presented with deafmutism and goiters. The investigations included scintigram, perchlorate discharge test and audiometery. The perchlorate discharge was positive in index case. Bilateral sensorineural hearing defect was detected on Pure Tone Average (PTA) audiometry. Meticulous clinical and laboratory evaluation is mandatory for the detection of rare disorders like Pendred's syndrome. (author)

  9. [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.

  10. 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 ...

  11. 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 ...

  12. 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 ...

  13. 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 ...

  14. Turner Syndrome: Other FAQs

    Science.gov (United States)

    ... 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 ...

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

    International Nuclear Information System (INIS)

    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

  16. 3He-MRI in follow-up of lung transplant recipients

    International Nuclear Information System (INIS)

    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.)

  17. Pfeiffer syndrome

    Directory of Open Access Journals (Sweden)

    Fryns Jean-Pierre

    2006-06-01

    Full Text Available Abstract Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.

  18. 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 ...

  19. Nutcracker syndrome

    International Nuclear Information System (INIS)

    Jolley, Ingrid

    2014-01-01

    Purpose: The purpose of this case study is to highlight the symptoms of the Nutcracker Syndrome (NCS), the methods of clinical investigations and the importance of differential diagnosis. Introduction: The NCS refers to left renal vein entrapment caused by abnormal branching patterns of the superior mesenteric artery from the aorta. 1,2 Clinical case presentation: A 27 years old female presented to the emergency department with complaints of abdominal discomfort, bloating, loose bowel motions and irregular micro-haematuria. The radiologist's report indicated the findings from computed tomography examination to be consistent with anterior NCS. Discussion: In most of the NCS cases the clinical symptoms are non-specific. 3 The syndrome is caused by a vascular disorder, but its clinical manifestation can relate to a wide range of abdominal, urological, endovascular or gynaecological pathologies. 4 Conclusion: Nutcracker Syndrome is a relatively rare disease and underdiagnosed may lead to left renal vein thrombosis

  20. Compartment syndromes

    Science.gov (United States)

    Mubarak, S. J.; Pedowitz, R. A.; Hargens, A. R.

    1989-01-01

    The compartment syndrome is defined as a condition in which high pressure within a closed fascial space (muscle compartment) reduces capillary blood perfusion below the level necessary for tissue viability'. This condition occurs in acute and chronic (exertional) forms, and may be secondary to a variety of causes. The end-result of an extended period of elevated intramuscular pressure may be the development of irreversible tissue injury and Volkmann's contracture. The goal of treatment of the compartment syndrome is the reduction of intracompartmental pressure thus facilitating reperfusion of ischaemic tissue and this goal may be achieved by decompressive fasciotomy. Controversy exists regarding the critical pressure-time thresholds for surgical decompression and the optimal diagnostic methods of measuring intracompartmental pressures. This paper will update and review some current knowledge regarding the pathophysiology, aetiology, diagnosis, and treatment of the acute compartment syndrome.

  1. Usher Syndrome

    Directory of Open Access Journals (Sweden)

    Ana Fakin

    2012-06-01

    Full Text Available Usher syndrome is an autosomal recessive disease with prevalence of 3–6/100.000 and is the most common syndrome that affects vision and hearing. Three subtypes are distinguished on the basis of different degree of hearing loss. All patients develop retinitis pigmentosa with night vision difficulties and constriction of visual field, and ultimately a decline in visual acuity and color vision. Future holds promise for gene therapy. We present a patient with typical clinical picture of Usher syndrome, who started noticing night vision problems at age 13. At age 25 he was operated on for posterior cortical cataracts. At age 34 he has only 5–10° of visual field remaining with 1.0 visual acuity in both eyes. Fundus autofluorescence imaging revealed a typical hyperautofluorescent ring on the border between normal and affected retina.

  2. Metabolic Syndrome

    Directory of Open Access Journals (Sweden)

    Sevil Ikinci

    2010-10-01

    Full Text Available Metabolic Syndrome is a combination of risk factors including common etiopathogenesis. These risk factors play different roles in occurence of atherosclerotic diseases, type 2 diabetes, and cancers. Although a compromise can not be achieved on differential diagnosis for MS, the existence of any three criterias enable to diagnose MS. These are abdominal obesity, dislipidemia (hypertrigliceridemia, hypercholesterolemia, and reduced high density lipoprotein hypertension, and elevated fasting blood glucose. According to the results of Metabolic Syndrome Research (METSAR, the overall prevalence of MS in Turkey is 34%; in females 40%, and in males it is 28%. As a result of “Western” diet, and increased frequency of obesity, MS is observed in children and in adolescents both in the world and in Turkey. Resulting in chronic diseases, it is thought that the syndrome can be prevented by healthy lifestyle behaviours. [TAF Prev Med Bull 2010; 9(5.000: 535-540

  3. Eagle's Syndrome

    Science.gov (United States)

    Pinheiro, Thaís Gonçalves; Soares, Vítor Yamashiro Rocha; Ferreira, Denise Bastos Lage; Raymundo, Igor Teixeira; Nascimento, Luiz Augusto; Oliveira, Carlos Augusto Costa Pires de

    2013-01-01

    Summary Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT) of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical. PMID:25992033

  4. Eagle's Syndrome

    Directory of Open Access Journals (Sweden)

    Pinheiro, Thaís Gonçalves

    2014-01-01

    Full Text Available Introduction: Eagle's syndrome is characterized by cervicopharyngeal signs and symptoms associated with elongation of the styloid apophysis. This elongation may occur through ossification of the stylohyoid ligament, or through growth of the apophysis due to osteogenesis triggered by a factor such as trauma. Elongation of the styloid apophysis may give rise to intense facial pain, headache, dysphagia, otalgia, buzzing sensations, and trismus. Precise diagnosis of the syndrome is difficult, and it is generally confounded by other manifestations of cervicopharyngeal pain. Objective: To describe a case of Eagle's syndrome. Case Report: A 53-year-old man reported lateral pain in his neck that had been present for 30 years. Computed tomography (CT of the neck showed elongation and ossification of the styloid processes of the temporal bone, which was compatible with Eagle's syndrome. Surgery was performed for bilateral resection of the stylohyoid ligament by using a transoral and endoscopic access route. The patient continued to present pain laterally in the neck, predominantly on his left side. CT was performed again, which showed elongation of the styloid processes. The patient then underwent lateral cervicotomy with resection of the stylohyoid process, which partially resolved his painful condition. Final Comments: Patients with Eagle's syndrome generally have a history of chronic pain. Appropriate knowledge of this disease is necessary for adequate treatment to be provided. The importance of diagnosing this uncommon and often unsuspected disease should be emphasized, given that correct clinical-surgical treatment is frequently delayed. The diagnosis of Eagle's syndrome is clinical and radiographic, and the definitive treatment in cases of difficult-to-control pain is surgical.

  5. Rapunzel syndrome

    International Nuclear Information System (INIS)

    Al-Wadan, Ali H.; Al-Saai, Azan S.; Abdoulgafour, Mohamed; Al-Absi, Mohamed

    2006-01-01

    An 18-year-old single female patient, presented with non specific gastrointestinal symptoms of anorexia, abdominal pain, and change in bowel habit. Clinically she was anemic, cachectic, and depressed. Abdominal examination revealed mobile epigastric mass. The scalp alopecia and endoscopy coupled by computed tomography scan, confirmed the diagnoses of trichobezoar, but it was not diagnosed as Rapunzel syndrome except after laparotomy, gastrotomy, and enterotomy. There are less than 16 cases of Rapunzel syndrome described worldwide, and this is the first case to be described in the middle east. (author)

  6. Waardenburg syndrome

    Directory of Open Access Journals (Sweden)

    Tagra Sunita

    2006-01-01

    Full Text Available Waardenburg syndrome is a rare inherited and genetically heterogenous disorder of neural crest cell development. Four distinct subtypes showing marked interfamilial and intrafamilial variability have been described. We report a girl showing constellation of congenital hearing impairment with 110 dB and 105 dB loss in right and left ear respectively, hypoplastic blue iridis, white forelock, dystopia canthorum and broad nasal root. Other affected relatives of the family, with variable features of the syndrome, have been depicted in the pedigree.

  7. Olmsted syndrome

    Directory of Open Access Journals (Sweden)

    Kumar Pramod

    2008-01-01

    Full Text Available Olmsted syndrome is a rare disorder characterized by the combination of periorificial, keratotic plaques and bilateral palmoplantar keratoderma. New associated features are being reported. Olmsted syndrome is particularly rare in a female patient, and we report such a case in a six year-old Indian girl, who presented with keratoderma of her soles since birth and on her palms since the age of two years along with perioral and perinasal hyperkeratosis. She had sparse, light brown, thin hair. Although the psychomotor development of the child was normal until 18 months of age, the keratoderma plaques had restricted the child′s mobility after that stage.

  8. Eagle syndrome

    International Nuclear Information System (INIS)

    Raina, Deepika; Gothi, Rajesh; Rajan, Sriram

    2009-01-01

    Eagle syndrome occurs due to elongation of the styloid process or calcification of the stylohyoid ligament, which then may produce a pain sensation due the pressure exerted on various structures in the head and neck. When suspected, imaging helps in identifying the abnormally elongated styloid process or the calcified ligament. In recent years, three-dimensional CT (3DCT) has proved to be valuable in these cases. We report the case of a 62-year-old man with this syndrome in whom imaging with 3DCT conclusively established the diagnosis

  9. Turner Syndrome

    Directory of Open Access Journals (Sweden)

    Ramachandran Sudarshan

    2012-08-01

    Full Text Available Turner syndrome is a genetic disorder that affects mostly females. Affected females have characteristic features such as short stature, premature ovarian failure, and several other features. Oral manifestations of this condition are not much discussed in the literature. But reported literature includes teeth, palate, periodontal and salivary changes. So the aim of this review is to illustrate the general manifestations, and especially the oral manifestations of Turner syndrome and evaluate their possible management. [Archives Medical Review Journal 2012; 21(4.000: 246-252

  10. Fenton's syndrome

    International Nuclear Information System (INIS)

    Rimondi, E.; Albasini, V.

    1989-01-01

    The authors report two recent cases of Fenton's syndrome, a very rare carpal fracture-dislocation. After some anatomophysiopathological considerations and a review of the literature, a wider nosographic frame is proposed in which the entity of the dislocation of the head of capitate bone is not essential. According to both the literature and personal findings, the authors remark that this syndrome is always found in the presence of two morphological variants of the distal radioulnar joint. Finally, the authors stress the importance of a corect diagnosis of this lesion to avoid unnecessary attempts of reduction

  11. Reiter's Syndrome.

    Science.gov (United States)

    Savant, S S; Fernandez, J C; Dhurandhar, M W; Fernandez, R J

    1979-01-01

    A case of Reiter's syndrome occurring in a young mate aged 20 years having extensive skin lesions of keratoderina blenoffhagica is presented along with a review of literature. Although urethritis was absent, other clinical and histopathological features of the cutaneous lesions led us to the diagnosis. The-possible relationship of postural psoriasis to Reiter's syndrome is discussed. Failure of the patient to respond satisfactorily to steroids, antibiotics etc, prompted the use of rnethotrexate in the case. The result was dramatic, as the patient completely recovered within ten days of starting treatment.

  12. Larsen syndrome

    Directory of Open Access Journals (Sweden)

    Mohammed Mahbubul Islam

    2016-08-01

    Full Text Available Larsen syndrome is a rare inherited disorder characterized by congenital dislocation of multiple joints along with other anomalies of heart, face, hands and bones. Larsen syndrome was first described in 1950 by Larsen, Schottstaedt and Bost. In the present report, we describe a 10 year old girl who presented with mid facial hypoplasia with depressed nasal bridge, high arched palate, bilateral talipes equinovarus and high arched feet. On examination, she had short stature (HAZ -3.5 SD with hyperextension of knee joint, fixed flexion of elbow joint. Awareness of this condition and associated complications may help in management and follow up of these patients. 

  13. Joubert syndrome

    International Nuclear Information System (INIS)

    Villanua, J.A.; Lopez, J.M.; Recondo, J.A.; Garcia, J.M.; Gaztanaga, R.

    1998-01-01

    Joubert syndrome is a rare malformation of the posterior fossa, mainly affecting the cerebellar vermis, which generally appears as a dysplastic lesion. Other structures of the cervico medullary junction may be involved, with accompanying brainstem hypoplasia according to neuroimaging studies. The diagnosis is usually reached during, childhood, based on a constellation of changes in the child's neurological development that are supported by the results of imaging studied. Respiratory problems are the most common signs in newborns,leading to the suspicion of the presence of this syndrome. (Author) 11 refs

  14. Lemierre's syndrome.

    LENUS (Irish Health Repository)

    O'Dwyer, D N

    2012-02-01

    Lemierre\\'s syndrome is a rare disease that results in an oropharyngeal infection, which precipitates an internal jugular vein thrombosis and metastatic infection. Fusobacterium necrophorum is an anaerobic Gram-negative bacillus and has been identified as the causative agent. We describe the case of a young girl whose presentation and diagnosis were confounded by a history of valvular heart disease. Infection of heart valves can produce many of the signs and symptoms associated with Lemierre\\'s syndrome. We describe the diagnosis, investigation and optimal management of this rare disorder.

  15. Meigs' Syndrome

    International Nuclear Information System (INIS)

    Baloch, S.; Khaskheli, M.; Farooq, S.

    2006-01-01

    Meigs' syndrome is a rare clinical condition commonly considered to be associated with malignant ovarian tumour. A case of unmarried female is presented who came with a slowly increasing abdominal mass. Clinical and ultrasonic investigations revealed a mobile, solid right adenexal tumour in the lower abdomen, along with ascites and pleural effusion of the right lung. The level of CA 125 was also raised. Diagnosis of Meigs' syndrome was confirmed after surgical intervention. The tumour was successfully removed and pleural effusion disappeared 15 days after the intervention. Cytomorphologic study of both the tumour and ascitic fluid was negative for malignancy. (author)

  16. [Elsberg syndrome].

    Science.gov (United States)

    Nielsen, Kristine Esbjerg; Knudsen, Troels Bygum

    2013-12-16

    A syndrome involving acute urinary retention in combination with sacral radiculitis and cerebrospinal fluid pleocytosis was first described by the American neurosurgeon Charles Elsberg in 1931. In many instances the aetiology is herpes simplex virus type 2 (HSV-2) reactivation from sensory neurons. In this case report we present a 34-year-old pregnant woman with previous undiagnosed sensory lumbosacral symptoms. She was hospitalized with HSV-2 meningitis and lumbosacral radiculitis but no genital rash. A week after the onset of symptoms she developed acute urinary retention, thus indicating Elsberg syndrome.

  17. 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.

  18. Lemierre's syndrome

    DEFF Research Database (Denmark)

    Johannesen, Katrine M; Bodtger, Uffe

    2016-01-01

    This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and/or ...... LS in this day and age appears to be low, however the syndrome is difficult to recognize, and still requires the full attention of the clinician.......This is a systematic review of cases with Lemierre's syndrome (LS) in the past 5 years. LS is characterized by sepsis often evolving after a sore throat or tonsillitis and then complicated by various septic emboli and thrombosis of the internal jugular vein. Symptoms include sepsis, pain, and....../or swelling in the throat or neck, as well as respiratory symptoms. Laboratory findings show elevated infectious parameters and radiological findings show thrombosis of the internal jugular vein and emboli in the lungs or other organs. The syndrome is often associated with an infection with Fusobacterium...

  19. Sjogren syndrome

    NARCIS (Netherlands)

    Brito-Zeron, Pilar; Baldini, Chiara; Bootsma, Hendrika; Bowman, Simon J.; Jonsson, Roland; Mariette, Xavier; Sivils, Kathy; Theander, Elke; Tzioufas, Athanasios; Ramos-Casals, Manuel

    2016-01-01

    Sjogren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be

  20. Rett Syndrome

    Science.gov (United States)

    ... loss of interest in normal play Delayed speech development or loss of previously acquired speech abilities Problem behavior or marked mood swings Any clear loss of previously gained milestones in gross motor or fine motor skills Causes Rett syndrome is a rare genetic disorder. ...

  1. Nodding Syndrome

    Centers for Disease Control (CDC) Podcasts

    2013-12-19

    Dr. Scott Dowell, a CDC director, discusses the rare illness, nodding syndrome, in children in Africa.  Created: 12/19/2013 by National Center for Emerging and Zoonotic Infectious Diseases (NCEZID).   Date Released: 1/27/2014.

  2. Piriformis Syndrome

    Science.gov (United States)

    ... can usually resume their normal activities. In some cases, exercise regimens may need to be modified in order to reduce the likelihood of recurrence or worsening. Clinical Trials Throughout the U.S. ... Definition Piriformis syndrome is a rare neuromuscular disorder that ...

  3. Hellp syndrome

    International Nuclear Information System (INIS)

    Chaudhry, A.A.

    2002-01-01

    A 24 years old female presented with hypertension, haemolysis, elevated liver enzymes and thrombocytopenia in an unconscious state after undergoing an emergency caesarian section. A diagnosis of HELLP syndrome was made on the above findings. Patient made an uneventful recovery with conservative management. A brief review of the literature is included along with the case report. (author)

  4. Kartagener's Syndrome.

    Science.gov (United States)

    Dhar, D K; Ganguly, K C; Alam, S; Hossain, A; Sarker, U K; Das, B K; Haque, M J

    2009-01-01

    Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.

  5. Carraro syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Wendler, H.; Schwarz, R.

    1980-07-01

    The report concerns a girl aged 9 1/2 years who was deaf and dumb and had marked shortening of the calves with deformities of the feet and bilateral, congenital hypoplasia of the tibiae. This syndrome was first described by Carraro in 1931, but there have been no further reports since then.

  6. Rett Syndrome.

    Science.gov (United States)

    Culbert, Linda A.

    This pamphlet reviews the historical process involved in initially recognizing Rett Syndrome as a specific disorder in girls. Its etiology is unknown, but studies have considered factors as hyperammonemia, a two-step mutation, a fragile X chromosome, metabolic disorder, environmental causation, dopamine deficiency, and an inactive X chromosome.…

  7. Alagille Syndrome

    Science.gov (United States)

    ... 3] Kamath BM, Loomes KM, Piccoli DA. Medical management of Alagille syndrome. Journal of Pediatric Gastroenterology and Nutrition. 2010;50(6): ... 30 a.m. to 5 p.m. eastern time, M-F Follow Us NIH… Turning Discovery Into ... Disease Urologic Diseases Endocrine Diseases Diet & Nutrition ...

  8. Kounis syndrome

    African Journals Online (AJOL)

    neoplastic agents), exposure to radiological contrast media, poison ivy, bee stings, shellfish and coronary stents. In addition to coronary arterial involvement, Kounis syndrome com prises other arterial systems with similar physiologies, such as mesenteric and cerebral circulation resulting in ischaemia/infarction of the vital ...

  9. Proteus syndrome

    Directory of Open Access Journals (Sweden)

    Debi Basanti

    2005-01-01

    Full Text Available Proteus syndrome is a variable and complex disorder characterized by multifocal overgrowths affecting any tissue or structure of the body. We present a girl aged 3 years and 8 months with an epidermal nevus, port-wine stain, macrodactyly with gigantism of the feet, lymphohemagiomas and multiple lipomas.

  10. Crest syndrome

    International Nuclear Information System (INIS)

    Koch, B.; Roedl, W.

    1988-01-01

    If a patient has peri- and intra-articular calcinosis, as well as acro-osteolysis and esophageal hypomotility, and rheumatic symptoms, Crest syndrome should be considered as a manifestation of progressive systemic sclerosis. In connection with relevant symptoms on the skin and visceral involvement, radiological studies offer the possibility of classifying progressive systemic sclerosis more accurately. (orig.) [de

  11. Gitelman syndrome.

    NARCIS (Netherlands)

    Knoers, N.V.A.M.; Levtchenko, E.N.

    2008-01-01

    Gitelman syndrome (GS), also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence

  12. Marfan Syndrome

    Science.gov (United States)

    ... can treat many of its symptoms. Thanks to new research and treatments, people with Marfan syndrome who are diagnosed early ... This helps doctors stay on top of any new problems. Doctors might also ... or kids with amblyopia or strabismus will probably need to wear glasses. ...

  13. Kartagener's Syndrome

    African Journals Online (AJOL)

    GB

    presenting with recurrent upper and lower respiratory tract infections, sinusitis or bronchiectasis. Inability to diagnose this condition may subject the patient to unnecessary and repeated hospital admissions, investigations and treatment failure. KEY WORDS: Kartagener's syndrome, primary cilliary dyskinesia, situs inversus, ...

  14. Dangerous drug interactions leading to hemolytic uremic syndrome following lung transplantation

    Directory of Open Access Journals (Sweden)

    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.

  15. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Ebru Yilmaz

    2014-06-01

    Full Text Available Hepatorenal syndrome (HRS is functional renal failure that occurs with advanced liver failure. HRS is considered the most severe complication of cirrhosis. Type 1 HRS develops due to severe reduction of effective circulating volume results in hemodynamic dysfunction. Type 1 HRS is characterized by acute renal failure and rapid deterioration in the function of other organs. It can ocur spontaneously or in the setting of a precipitating event. Type 2 hepatorenal syndrome (HRS, which is characterized by slowly progressive renal failure and refractory ascites. Liver transplantation is the only definitive treatment for both type. The most suitable and ldquo;bridge treatments and rdquo; or treatment for patients ineligible for a liver transplant include terlipressin plus albumin. [J Contemp Med 2014; 4(2.000: 106-113

  16. Dravet syndrome

    Directory of Open Access Journals (Sweden)

    Incorpora Gemma

    2009-09-01

    Full Text Available Abstract "Dravet syndrome" (DS previously named severe myoclonic epilepsy of infancy (SMEI, or epilepsy with polymorphic seizures, is a rare disorder characterized by an early, severe, generalized, epileptic encephalopathy. DS is characterized by febrile and afebrile seizures beginning in the 1st year of life followed by different types of seizures (either focal or generalized, which are typically resistant to antiepileptic drugs. A developmental delay from the 2nd to 3rd year of life becomes evident, together with motor disturbances and personality disorders. Beside the classic syndrome, there are milder cases which have been called severe myoclonic epilepsy borderline (SMEB. DS is caused by a mutation in the neuronal sodium channel gene, SCN1A , that is also mutated in generalized epilepsy with FS+ (GEFS+.

  17. Apert syndrome

    Directory of Open Access Journals (Sweden)

    Premalatha

    2010-01-01

    Full Text Available Apert syndrome (acrocephalosyndactyly is a rare developmental malformation characterized by craniosynostosis, mid-face hypoplasia, symmetrical syndactyly of hands and feet. The prodromal characteristics for the typical cranio-facial appearance are early craniosynostosis of the coronal suture, cranial base and agenesis of the sagittal suture. The purpose of this paper is to report a case of Apert syndrome with emphasis on craniofacial and oral features in an eighteen-month-old male child. The patient presented with several craniofacial deformities, including brachycephaly, midface hypoplasia, flat face, hypertelorism, ocular proptosis, downslanting palpebral fissures. Syndactylies with osseous fusion of the hands and feet were also observed. Intraoral findings included delayed eruption of teeth, high arched palate with pseudo cleft in the posterior one third.

  18. Paraneoplastic syndromes

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms, or syndromes which can mimic other disease conditions encountered in veterinary medicine. Recognition of PNS is valuable for several reasons: the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor; they may allow assessment of premalignant states; they may aid in the search metastases; they may help quantify and monitor response to therapy; and, they may provide insight into the study of malignant transformation and oncogene expression. This review will concentrate on the pathophysiology, diagnosis, and treatment of some of the common PNS encountered in veterinary medicine.

  19. Paraneoplastiske syndromer

    OpenAIRE

    Røsbekk, Stein Helge

    2007-01-01

    During the last 50 years it has become clear that malignant tumours can induce symptoms unrelated to the mechanical effects of the primary tumour itself or its metastasis. Today, the name Paraneoplastic syndrome is given to those symptom complexes that may affect the blood cells, electrolytes, coagulation system, muscle, skin, nerve and the endocrine system. Endocrine symptoms were first recognised, and different hormones were isolated from the tumour tissue. However, tumour derived hormones ...

  20. Caroli's syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Numan, F; Cokyueksel, O; Camuscu, S; Demir, K; Dueren, M

    1986-07-01

    In 1958 Caroli described the syndrome of congenital, either segmental or involving the entire bile duct system, saccular extensions of the intrahepatic bile ducts. He differentiated between two types of this disease pattern. The first form concerns pure cystic dilatations of the intrahepatic bile ducts, whereas the second one is combined with hepatic fibrosis and portal hypertension. Both types are characterised by cystic dilatations in the kidneys and in the extrahepatic bile ducts, pancreas and spleen.

  1. Griscelli syndrome

    Directory of Open Access Journals (Sweden)

    Kumar T

    2006-01-01

    Full Text Available Partial albinism with immunodeficiency is a rare and fatal immunologic disorder characterized by pigmentary dilution and variable cellular immunodeficiency. It was initially described in 1978. Primary abnormalities included silvery grayish sheen to the hair, large pigment agglomerations in hair shafts and an abundance of mature melanosomes in melanocytes, with reduced pigmentation of adjacent keratinocytes. We describe a child with Griscelli syndrome who presented with hepatitis, pancytopenia and silvery hair. The diagnosis was confirmed by microscopic skin and hair examination.

  2. Waardenburg syndrome

    OpenAIRE

    Mehta, Manish; Kavadu, Paresh; Chougule, Sachin

    2004-01-01

    We report a case of Waardenburg syndrome in a female child aged 2yrs. Petrus Johannes Waardenburg(1) , a Dutch Ophthalmologist in 1951 described individuals with retinal pigmentary differences who had varying degrees of hearing loss and dystopia canthorum (i.e., latral displacement of inner canthi of eyes). The disease runs in families with a dominant inheritance pattern with varying degree of clinical presentation. Patient usually present with heterochromic iris, pigmentary abnormalities of ...

  3. [PHACES syndrome].

    Science.gov (United States)

    Morcillo Azcárate, J; Bernabeu-Wittel, J; Fernández-Pineda, I; Conejo-Mir, M D; Tuduri Limousin, I; Aspiazu Salinas, D A; de Agustín Asensio, J C

    2010-04-01

    PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. Retrospective study of the cases seen at our unit in the last year. We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.

  4. Anserine syndrome.

    Science.gov (United States)

    Helfenstein, Milton; Kuromoto, Jorge

    2010-01-01

    Knee pain is a common complaint in clinical practice, and pes anserinus tendino-bursitis syndrome (PATB) has been frequently diagnosed based only on clinical features that may cause equivocal interpretations. Patients complain of characteristic spontaneous medial knee pain with tenderness in the inferomedial aspect of the joint. Studies with different imaging modalities have been undertaken during the last years to identify whether these patients suffer from bursitis, tendinitis, or both. Nevertheless, little is known regarding the structural defect responsible for this disturbance. Due to these problems and some controversies, we suggest the term "anserine syndrome" for this condition. Diabetes Mellitus is a known predisposing factor for this syndrome. Overweight and osteoarthritis seem to represent additional risk factors; however, their role in the pathophysiology of the disease is not yet understood. Treatment includes non-steroidal anti-inflammatory drugs, physiotherapy, and injections of corticosteroid, with highly variable responses, from 10 days to 36 months to achieve recovery. The lack of knowledge about its epidemiological, etiological, and pathophysiological aspects requires future studies for this common and intriguing disorder.

  5. Neonatal respiratory distress syndrome

    Science.gov (United States)

    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 ...

  6. 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- ...

  7. 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 ...

  8. What Causes Cushing's Syndrome?

    Science.gov (United States)

    ... Share Facebook Twitter Pinterest Email Print What causes Cushing syndrome? Cushing syndrome can develop for two reasons: Medication ... uhs ), thyroid, or thymus How Tumors Can Cause Cushing Syndrome Normally, the pituitary gland in the brain controls ...

  9. Genetics Home Reference: antiphospholipid syndrome

    Science.gov (United States)

    ... 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 ...

  10. Genetics Home Reference: Costello syndrome

    Science.gov (United States)

    ... 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. ...

  11. Acute nephritic syndrome

    Science.gov (United States)

    Glomerulonephritis - acute; Acute glomerulonephritis; Nephritis syndrome - acute ... Acute nephritic syndrome is often caused by an immune response triggered by an infection or other disease. Common causes in children ...

  12. Morvan Syndrome

    Science.gov (United States)

    Maskery, Mark; Chhetri, Suresh K.; Dayanandan, Rejith; Gall, Claire

    2016-01-01

    A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management. PMID:26740856

  13. Jacobsen syndrome

    Directory of Open Access Journals (Sweden)

    Grossfeld Paul

    2009-03-01

    Full Text Available Abstract Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears. Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from ~7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be

  14. Robinow syndrome

    Directory of Open Access Journals (Sweden)

    Suresh S

    2008-01-01

    Full Text Available Robinow syndrome is a rare autosomal recessive mesomelic dwarfism with just more than 100 cases reported in the literature so far. The lower extremity is spared with skeletal deformity usually confined to the forearm, hand, and the dorsal spine. Diagnosis is made easily in the early childhood by the typical "fetal facies" appearance, which disappears to a certain extent as the patient grows. The author reports two cases of this entity with vertebral segmentation defects, rib fusion, and typical severe brachymelia and facial features.

  15. Trichorhinophalangeal syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Tuzovic, S.; Fiebach, B.J.O.; Magnus, L.; Sauerbrei, H.U.

    1982-11-01

    This article reports on 14 cases of a trichorhinophalangeal syndrome in five successive generations. Besides the well-known characteristics of the TRPS the following symptoms observed in this family are new: Teething was considerably delayed, intelligence was reduced, and there were skin manifestations resembling eczema. Besides, struma colli and colitis ulcerosa were also observed. Subsequent observations have to clarify whether these symptoms are a facultative part of the TRPS pattern. The constant appearance of carriers of these characteristics during five generation points to dominant heredity.

  16. Olmsted Syndrome

    Directory of Open Access Journals (Sweden)

    Sirka C

    1999-01-01

    Full Text Available A 20-year-old Sikh man had palmoplantar keratoderma, flexion deformity of digits, universal alopecia, keratotic plaques at the angles of mouth, gluteal cleft, knees and dorsal aspects of the metacarpophalangeal joints of the hand; features of Olmsted syndrome. He had normal nails, teeth, oral mucosa and normal joint movements. Treatment with acitretin, 25mg/day for three and a half months, followed by 25mg once daily alternating with 50mg once daily for 3 months resulted in significant improvement.

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

    Institute of Scientific and Technical Information of China (English)

    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.

  18. 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

  19. Cardiorenal syndrome

    Directory of Open Access Journals (Sweden)

    Sabry Omar

    2013-01-01

    Full Text Available Cardiovascular disease is the leading cause of death in patients with chronic kidney disease.  Heart failure may lead to acute kidney injury and vice versa. Chronic kidney disease may affect the clinical outcomes in patients with cardiovascular disorders. Renal impairment with any degree of albuminuria has been increasingly recognized as an independent risk factor for cardiovascular events and heart failure hospitalizations, while chronic heart failure may cause chronic kidney disease. The bidirectional nature of these disorders contributes to the complexity and the composite definitions of cardiorenal syndromes. However, the most important clinical trials in heart failure tend to exclude patients with significant renal dysfunction. The mechanisms whereby renal insufficiency worsens the outcome in heart failure are not known, and several pathways could contribute to the ‘‘vicious heart/kidney circle.’’ Traditionally, renal impairment has been attributed to the renal hypoperfusion due to reduced cardiac output and decreased systemic pressure. The hypovolemia leads to sympathetic activity, increased renin-angiotensin aldosterone pathway, and arginine-vasopressin release. These mechanisms cause fluid and sodium retention, peripheral vasoconstriction, and volume overload. Therapy to improve renal dysfunction, reduce neurohormonal activation and ameliorate renal blood flow could lead to a reduction in mortality and hospitalization in patients with cardiorenal syndrome.

  20. Lowe syndrome

    Directory of Open Access Journals (Sweden)

    Loi Mario

    2006-05-01

    Full Text Available Abstract Lowe syndrome (the oculocerebrorenal syndrome of Lowe, OCRL is a multisystem disorder characterised by anomalies affecting the eye, the nervous system and the kidney. It is a uncommon, panethnic, X-linked disease, with estimated prevalence in the general population of approximately 1 in 500,000. Bilateral cataract and severe hypotonia are present at birth. In the subsequent weeks or months, a proximal renal tubulopathy (Fanconi-type becomes evident and the ocular picture may be complicated by glaucoma and cheloids. Psychomotor retardation is evident in childhood, while behavioural problems prevail and renal complications arise in adolescence. The mutation of the gene OCRL1 localized at Xq26.1, coding for the enzyme phosphatidylinositol (4,5 bisphosphate 5 phosphatase, PtdIns (4,5P2, in the trans-Golgi network is responsible for the disease. Both enzymatic and molecular testing are available for confirmation of the diagnosis and for prenatal detection of the disease. The treatment includes: cataract extraction, glaucoma control, physical and speech therapy, use of drugs to address behavioural problems, and correction of the tubular acidosis and the bone disease with the use of bicarbonate, phosphate, potassium and water. Life span rarely exceeds 40 years.

  1. Cotard Syndrome.

    Science.gov (United States)

    Dieguez, Sebastian

    2018-01-01

    Cotard's syndrome is often described as the delusional belief that one is dead or non-existent. However, Jules Cotard's initial description (1880) of the "delusion of negations" was much richer and also involved delusions and claims of immortality and enormity, feelings of damnation, and illusions of bodily dissolution and transformation. Alternatively conceived as an extreme case of depression, hypochondria, or psychosis, the condition is considered rare and remains poorly understood. Cotard himself provided a taxonomy and several explanations for the condition, focusing on its distinction from classical persecutory delusions and suggesting that it could be a kind of reversed grandiosity. He proposed a psychosensory basis in the dissolution of mental imagery, which he then extended to a more general psychomotor impairment of volition. Other early authors highlighted a disorder of the bodily self, and more recent theories postulated an impairment of right hemispheric functions, leading to perceptual and somatosensory feelings of unreality, which coupled with reasoning impairments and an internalized attributional style led in turn to beliefs of non-existence. However, despite its striking presentation and its relevance to our understanding of self-awareness, Cotard's syndrome remains an elusive condition, rarely reported and poorly researched. © 2018 S. Karger AG, Basel.

  2. KBG syndrome

    Directory of Open Access Journals (Sweden)

    Brancati Francesco

    2006-12-01

    Full Text Available Abstract KBG syndrome is a rare condition characterised by a typical facial dysmorphism, macrodontia of the upper central incisors, skeletal (mainly costovertebral anomalies and developmental delay. To date, KBG syndrome has been reported in 45 patients. Clinical features observed in more than half of patients that may support the diagnosis are short stature, electroencephalogram (EEG anomalies (with or without seizures and abnormal hair implantation. Cutaneous syndactyly, webbed short neck, cryptorchidism, hearing loss, palatal defects, strabismus and congenital heart defects are less common findings. Autosomal dominant transmission has been observed in some families, and it is predominantly the mother, often showing a milder clinical picture, that transmits the disease. The diagnosis is currently based solely on clinical findings as the aetiology is unknown. The final diagnosis is generally achieved after the eruption of upper permanent central incisors at 7–8 years of age when the management of possible congenital anomalies should have been already planned. A full developmental assessment should be done at diagnosis and, if delays are noted, an infant stimulation program should be initiated. Subsequent management and follow-up should include an EEG, complete orthodontic evaluation, skeletal investigation with particular regard to spine curvatures and limb asymmetry, hearing testing and ophthalmologic assessment.

  3. Elsberg syndrome

    Science.gov (United States)

    Savoldi, Filippo; Kaufmann, Timothy J.; Flanagan, Eoin P.; Toledano, Michel

    2017-01-01

    Objective: Elsberg syndrome (ES) is an established but often unrecognized cause of acute lumbosacral radiculitis with myelitis related to recent herpes virus infection. We defined ES, determined its frequency in patients with cauda equina syndrome (CES) with myelitis, and evaluated its clinical, radiologic, and microbiologic features and outcomes. Methods: We searched the Mayo Clinic medical records for ES and subsequently for combinations of index terms to identify patients with suspected CES and myelitis. Results: Our search yielded 30 patients, 2 diagnosed with ES and an additional 28 with clinical or radiologic evidence of CES retrospectively suspected of having ES. We classified patients in 5 groups according to diagnostic certainty. MRI and EMG confirmed that 2 had only myelitis, 5 only radiculitis, and 16 both. Two had preceding sacral herpes infection and 1 oral herpes simplex. Spinal cord lesions were commonly multiple, discontinuous, not expansile, and centrally or ventrally positioned. Lesions generally spared the distal conus. Nerve root enhancement was occasionally prominent and was smooth rather than nodular. Lymphocytic CSF pleocytosis was common. Thirteen patients (43%) had viral isolation studies, which were commonly delayed; the delay may have accounted for the low rate of viral detection. Acyclovir was administered to 6 patients. Most patients recovered with sequelae; 1 patient experienced encephalomyelitis and died. Conclusion: ES is a definable condition likely responsible for 10% of patients with combined CES and myelitis. Radiologic findings are not entirely specific but may help in differentiating ES from some competing diagnostic considerations. We propose criteria to facilitate diagnosis. PMID:28534040

  4. Sotos syndrome

    Directory of Open Access Journals (Sweden)

    Cormier-Daire Valérie

    2007-09-01

    Full Text Available Abstract Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC, advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (

  5. Marfan Syndrome (For Parents)

    Science.gov (United States)

    ... 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 ...

  6. 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

  7. Turner Syndrome (For Teens)

    Science.gov (United States)

    ... 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 ...

  8. Understanding Bartter syndrome and Gitelman syndrome.

    Science.gov (United States)

    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.

  9. 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.

  10. [Syndrome X vs metabolic syndrome].

    Science.gov (United States)

    Morales Villegas, Enrique

    2006-01-01

    Himsworth in 1939 postulated that Diabetes Mellitus type 2 (DM2) was not only an insulin deficiency state but also a cellular insulin insensitivity disease. Thirty years later, DeFronzo and Reaven demonstrated that insulin resistance (IR) preceded and predisposed for DM2 and atherosclerotic-cardiovascular-disease (ACVD). Reaven was the first to point out the relationship between IR and with hyperglycemia, dyslipidosis, and hypertension as mediators for ACVD, creating the concept of Syndrome X (SX) in 1988. WHO and, thereafter, other medical societies and medical groups, mainly ATP-III, in 2002, based on the difficulty of diagnosing IR in a simple, reliable, and inexpensive way, proposed and published the Metabolic Syndrome (MS) concept, as a group of five variables, i.e., obesity, hyperglycemia, hypertriglyceridemia, low HDL, and hypertension, as an easy clinical approximation to suspect and treat an increased cardiometabolic risk. Nowadays, there are deep and extensive controversies on this issue; however, these controversies do not really exist since all discordant points of view are rather quantitative and not qualitative in nature. This article is aimed at differentiating and harmonizing the complementary concepts of SX and MS, at analyzing why MS is a good "clinical window" to look for IR and its underlying manifestations, and finally to accept that the MS concept complements, but does not substitute or antagonize, traditional scales used to asses cardiovascular risk, such as the Framingham scale.

  11. Metabolic Syndrome: Polycystic Ovary Syndrome.

    Science.gov (United States)

    Mortada, Rami; Williams, Tracy

    2015-08-01

    Polycystic ovary syndrome (PCOS) is a heterogeneous condition characterized by androgen excess, ovulatory dysfunction, and polycystic ovaries. It is the most common endocrinopathy among women of reproductive age, affecting between 6.5% and 8% of women, and is the most common cause of infertility. Insulin resistance is almost always present in women with PCOS, regardless of weight, and they often develop diabetes and metabolic syndrome. The Rotterdam criteria are widely used for diagnosis. These criteria require that patients have at least two of the following conditions: hyperandrogenism, ovulatory dysfunction, and polycystic ovaries. The diagnosis of PCOS also requires exclusion of other potential etiologies of hyperandrogenism and ovulatory dysfunction. The approach to PCOS management differs according to the presenting symptoms and treatment goals, particularly the patient's desire for pregnancy. Weight loss through dietary modifications and exercise is recommended for patients with PCOS who are overweight. Oral contraceptives are the first-line treatment for regulating menstrual cycles and reducing manifestations of hyperandrogenism, such as acne and hirsutism. Clomiphene is the first-line drug for management of anovulatory infertility. Metformin is recommended for metabolic abnormalities such as prediabetes, and a statin should be prescribed for cardioprotection if the patient meets standard criteria for statin therapy. Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.

  12. 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.

  13. Gorlin's syndrome.

    Science.gov (United States)

    Ramsden, R T; Barrett, A

    1975-06-01

    The uncommon familial syndrome of multiple odontogenic keratocysts, basal cell naevi and skeletal anomalies is reviewed, and seven cases are described, including one patient who developed squamous cell carcinoma in a previous odontogenic keratocyst of the maxilla. We wish to thank Consultants from the Royal National Throat, Nose and Ear Hospital, The Middlesex Hospital and the Eastman Dental Hospital, who allowed us access to their patients; Mr. D. Garfield Davies, Dr. M. F. Spittle, Mr. D. Winstock, Mr. H. P. Cook, Professor H. C. Killey and Mr. L. W. Kay. We are grateful to Professor L. Michaels and Mr. D. J. Connolly for preparation of the illustrations and to Mrs. A. Matthews for the typescript.

  14. HEPATORENAL SYNDROME

    Directory of Open Access Journals (Sweden)

    Matjaž Hafner

    2001-12-01

    Full Text Available Background. Hepatorenal syndrome (HRS is acommon complication of advanced hepatic disease characterizedby marked abnormalities in arterial circulation and byrenal failure. An extreme arteriolar vasodilatation located inthe splanchnic circulation results in a reduction of total systemicvascular resistence and arterial hypotension. Vasoconstrictionoccurs in the renal circulation as in all other extrasplanchnicvascular territories. In the kidney, marked renalvasoconstriction results in a low glomerular filtration rate.Conclusions. The diagnosis of HRS is currently based on exclusionof other causes of renal failure. Prognosis of patientswith HRS is very poor. Liver transplantation is the best therapeuticoption, but it is seldom applicable due to the short survivalexpectancy of most patients with HRS, particularly thosewith the rapidly progressive type of HRS. New therapies developedduring the last few years, such as the use of systemicvasoconstrictors or transjugular intrahepatic portosystemicshunts (TIPS appear promising. Such treatments are of interestnot only as a bridge to liver transplantation but also as atherapy for patients who are not candidates for transplantation.

  15. Noonan syndrome

    Directory of Open Access Journals (Sweden)

    van der Burgt Ineke

    2007-01-01

    Full Text Available Abstract Noonan Syndrome (NS is characterised by short stature, typical facial dysmorphology and congenital heart defects. The incidence of NS is estimated to be between 1:1000 and 1:2500 live births. The main facial features of NS are hypertelorism with down-slanting palpebral fissures, ptosis and low-set posteriorly rotated ears with a thickened helix. The cardiovascular defects most commonly associated with this condition are pulmonary stenosis and hypertrophic cardiomyopathy. Other associated features are webbed neck, chest deformity, mild intellectual deficit, cryptorchidism, poor feeding in infancy, bleeding tendency and lymphatic dysplasias. The syndrome is transmitted as an autosomal dominant trait. In approximately 50% of cases, the disease is caused by missense mutations in the PTPN11 gene on chromosome 12, resulting in a gain of function of the non-receptor protein tyrosine phosphatase SHP-2 protein. Recently, mutations in the KRAS gene have been identified in a small proportion of patients with NS. A DNA test for mutation analysis can be carried out on blood, chorionic villi and amniotic fluid samples. NS should be considered in all foetuses with polyhydramnion, pleural effusions, oedema and increased nuchal fluid with a normal karyotype. With special care and counselling, the majority of children with NS will grow up and function normally in the adult world. Management should address feeding problems in early childhood, evaluation of cardiac function and assessment of growth and motor development. Physiotherapy and/or speech therapy should be offered if indicated. A complete eye examination and hearing evaluation should be performed during the first few years of schooling. Preoperative coagulation studies are indicated. Signs and symptoms lessen with age and most adults with NS do not require special medical care.

  16. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

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

    International Nuclear Information System (INIS)

    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.)

  19. Gitelman syndrome

    Directory of Open Access Journals (Sweden)

    Levtchenko Elena N

    2008-07-01

    Full Text Available Abstract Gitelman syndrome (GS, also referred to as familial hypokalemia-hypomagnesemia, is characterized by hypokalemic metabolic alkalosis in combination with significant hypomagnesemia and low urinary calcium excretion. The prevalence is estimated at approximately 1:40,000 and accordingly, the prevalence of heterozygotes is approximately 1% in Caucasian populations, making it one of the most frequent inherited renal tubular disorders. In the majority of cases, symptoms do not appear before the age of six years and the disease is usually diagnosed during adolescence or adulthood. Transient periods of muscle weakness and tetany, sometimes accompanied by abdominal pain, vomiting and fever are often seen in GS patients. Paresthesias, especially in the face, frequently occur. Remarkably, some patients are completely asymptomatic except for the appearance at adult age of chondrocalcinosis that causes swelling, local heat, and tenderness over the affected joints. Blood pressure is lower than that in the general population. Sudden cardiac arrest has been reported occasionally. In general, growth is normal but can be delayed in those GS patients with severe hypokalemia and hypomagnesemia. GS is transmitted as an autosomal recessive trait. Mutations in the solute carrier family12, member 3 gene, SLC12A3, which encodes the thiazide-sensitive NaCl cotransporter (NCC, are found in the majority of GS patients. At present, more than 140 different NCC mutations throughout the whole protein have been identified. In a small minority of GS patients, mutations in the CLCNKB gene, encoding the chloride channel ClC-Kb have been identified. Diagnosis is based on the clinical symptoms and biochemical abnormalities (hypokalemia, metabolic alkalosis, hypomagnesemia and hypocalciuria. Bartter syndrome (especially type III is the most important genetic disorder to consider in the differential diagnosis of GS. Genetic counseling is important. Antenatal diagnosis for GS

  20. Burning Mouth Syndrome and "Burning Mouth Syndrome".

    Science.gov (United States)

    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.

  1. Hepatorenal Syndrome

    Directory of Open Access Journals (Sweden)

    Pınar Zeyneloğlu

    2012-04-01

    Full Text Available Renal failure is a common major complication in patients with advanced cirrhosis and generally indicates a poor prognosis when combined with liver failure. Hepatorenal syndrome (HRS is characterised by a combination of disturbances in circulatory and kidney function. Arterial pressure is decreased in the systemic circulation due to reduced total systemic vascular resistance. Kidney dysfunction is caused by reduction in renal blood flow. The diagnosis of HRS is based on exclusion of other disorders that cause acute kidney injury in cirrhosis as there are no specific tests. There are two types of HRS with different characteristics and prognostics. Liver transplantation is the treatment of choice for all patients without contraindication. The best approach to the pharmacologic management is the administration vasoconstrictor drugs based on the pathogenesis. Many vasoconstrictors including vasopressin analogues (terlipressin, ornipressin and vasopressin, somatostatin analogues (octreotide and alpha-adrenergic analogues (midodrine and norepinephrine have been studied. In most of the studies intravenous albumin therapy was coadministered with vasoconstrictor drugs and suggested that albumin should be considered as the component of pharmacologic intervention in patients with HRS. Renal replacement therapy in the form of hemodialysis or continuous venovenous hemofiltration has been used in the management of HRS patients awaiting transplantation or in those with acute potentially reversible conditions. The artificial hepatic support systems require further investigation. (Journal of the Turkish Society Intensive Care 2012; 10: 37-44

  2. Pseudohypopituitary syndromes.

    Science.gov (United States)

    Heinze, E; Holl, R W

    1992-07-01

    In a child with short stature, the finding of normal or elevated GH levels in the presence of low concentrations of IGF-I raises the following possibilities. (1) A modification of the GH molecule, which is still detected by RIA, but inactive biologically. Therefore, an RRA or bioassay for hGH should result in considerably lower GH measurements compared with RIA determinations in the same sample. As both bioassays as well as RRAs are not widely available and are hampered by several difficulties, few children with this presumptive diagnosis have been described. So far, it has not been possible to define a specific molecular defect in one of these patients. (2) Abnormalities of the GH receptor or postreceptor mechanisms lead to a GH insensitivity syndrome. Laron-type dwarfism is usually due to a deletion in the gene for hepatic GH receptors: the serum binding protein for GH is absent. In three additional populations, the Pygmies of Zaire, the little women of Loja in Ecuador and the Mountain Ok people in Papua New Guinea, alterations of GH receptor function have been described. Finally, some reports describe patients with normal or elevated serum levels of both growth hormone and IGF-I in whom resistance to IGF has been implied in the pathogenesis of small stature.

  3. Hepatorenal syndrome.

    Science.gov (United States)

    Papper, S

    1980-01-01

    Renal failure without apparent cause (the hepatorenal syndrome) may develop in the course of cirrhosis of the liver. While the development of renal failure bears a poor prognosis, spontaneous recovery can occur. The data suggest that for the most part patients die in rather than of renal failure. The latter seems to be only part of a broader more fundamental disturbance. The pathogenesis of HRS is unknown, but the evidence supports an impairment of effective renal perfusion. The two major hypotheses concerning the nature of the impaired perfusion are that it is a physiologic response to alterations in the extrarenal circulation, and that there is an unidentified humoral agent(s) produced by or inadequately inactivated by or bypassing the diseased liver and causing circulatory changes in the kidney as well as in other organs. It is possible that both mechanisms are operative. Treatment is unsatisfactory and emphasis is presently best placed upon searching for more treatable causes of renal functional impairment in individual patients.

  4. Terlipressin for hepatorenal syndrome

    DEFF Research Database (Denmark)

    Gluud, Lise Lotte; Christensen, Kurt; Christensen, Erik

    2012-01-01

    Clinical trials suggest that terlipressin improves renal function in hepatorenal syndrome, but the evidence concerning mortality is equivocal.......Clinical trials suggest that terlipressin improves renal function in hepatorenal syndrome, but the evidence concerning mortality is equivocal....

  5. Chinese restaurant syndrome

    Science.gov (United States)

    Chinese restaurant syndrome is a set of symptoms that some people have after eating Chinese food. A food additive ... Chinese restaurant syndrome is most often diagnosed based on the symptoms. The health care provider may ask the following ...

  6. Obesity hypoventilation syndrome (OHS)

    Science.gov (United States)

    ... this page: //medlineplus.gov/ency/article/000085.htm Obesity hypoventilation syndrome (OHS) To use the sharing features on this page, please enable JavaScript. Obesity hypoventilation syndrome (OHS) is a condition in some ...

  7. Hermansky-Pudlak syndrome

    Directory of Open Access Journals (Sweden)

    Preena A

    2017-04-01

    Full Text Available Hermansky-Pudlak syndrome is a rare autosomal recessive multisystem disease, with oculocutneous albinism, pulmonary fibrosis and bleeding diathesis. Here we report a case of Hermansky-Pudlak syndrome who presented with dyspnea, oculocutaneous albinism and nystagmus.

  8. Marfan syndrome (image)

    Science.gov (United States)

    Marfan syndrome is a disorder of connective tissue which causes skeletal defects typically recognized in a tall, lanky person. A person with Marfan syndrome may exhibit long limbs and spider-like fingers, ...

  9. Acute respiratory distress syndrome

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/000103.htm Acute respiratory distress syndrome To use the sharing features on this page, please enable JavaScript. Acute respiratory distress syndrome (ARDS) is a life-threatening lung ...

  10. Oculoauriculovertebral dysplasia (Goldenhar's syndrome).

    Science.gov (United States)

    Nkrumah, F K

    1971-03-01

    A case of Goldenhar's Syndrome or Oculoauriculovertebral dysplasia in a Ghanaian infant is described. Significant were the additional findings of congenital esophageal atresia and arthrogryposis which have so far not been reported in association with the syndrome.

  11. Guillain-Barre Syndrome

    Science.gov (United States)

    Guillain-Barre syndrome is a rare disorder that causes your immune system to attack your peripheral nervous system (PNS). The PNS ... your brain. No one knows what causes the syndrome. Sometimes it is triggered by an infection, surgery, ...

  12. Carpal Tunnel Syndrome

    Science.gov (United States)

    ... a passing cramp? It could be carpal tunnel syndrome. The carpal tunnel is a narrow passageway of ... three times more likely to have carpal tunnel syndrome than men. Early diagnosis and treatment are important ...

  13. Polycystic Ovary Syndrome

    Science.gov (United States)

    Polycystic ovary syndrome (PCOS) happens when a woman's ovaries or adrenal glands produce more male hormones than normal. PCOS causes cysts ( ... PCOS are at higher risk of diabetes, metabolic syndrome, heart disease, and high blood pressure. PCOS is ...

  14. Hyperimmunoglobulin E syndrome

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/001311.htm Hyperimmunoglobulin E syndrome To use the sharing features on this page, please enable JavaScript. Hyperimmunoglobulin E syndrome is a rare, inherited disease. It causes ...

  15. Holmes-Adie Syndrome

    Science.gov (United States)

    ... other diseases of the nervous system, such as Sjogren’s syndrome or migraine. It is most often seen in ... other diseases of the nervous system, such as Sjogren’s syndrome or migraine. It is most often seen in ...

  16. The obstetric antiphospholipid syndrome

    NARCIS (Netherlands)

    Derksen, R. H. W. M.; de Grootb, Ph. G.

    The association of persistent presence of circulating antiphospholipid antibodies and thromboembolic events, (recurrent) pregnancy loss or both is termed antiphospholipid syndrome. Pregnancies in women with the syndrome should be regarded as at high-risk for complications. Optimal management

  17. Tics and Tourette Syndrome

    Science.gov (United States)

    ... for Nausea and Vomiting Home Diseases and Conditions Tics and Tourette Syndrome Condition Tics and Tourette Syndrome Share Print Table of Contents1. ... little or no control over. These are called tics. Several different tics can happen at the same ...

  18. Down Syndrome (For Kids)

    Science.gov (United States)

    ... Changed What's Life Like for Kids With Down Syndrome? Print en español El síndrome de Down You have probably seen people who have Down syndrome. They have certain physical features, such as a ...

  19. Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... to Yosemite FAQ: Non-U.S. Visitors to Yosemite History of HPS Related Links Prevent Rodent Infestations Cleaning Up After Rodents Diseases From Rodent Hantavirus Pulmonary Syndrome (HPS) Recommend on Facebook Tweet Share Compartir Hantavirus Pulmonary Syndrome (HPS) is ...

  20. Ramsay Hunt syndrome

    Science.gov (United States)

    Hunt syndrome; Herpes zoster oticus; Geniculate ganglion zoster; Geniculate herpes; Herpetic geniculate ganglionitis ... The varicella-zoster virus that causes Ramsay Hunt syndrome is the same virus that causes chickenpox and ...

  1. Moebius Syndrome Foundation

    Science.gov (United States)

    ... craniofacial/neurological disorder. Individuals with Moebius syndrome cannot smile or frown, and do not have lateral eye ... the organization to ensure that they are in line with the mission of the Moebius Syndrome Foundation. ...

  2. Burning Mouth Syndrome

    Science.gov (United States)

    ... Care Home Health Info Health Topics Burning Mouth Burning Mouth Syndrome (BMS) is a painful, complex condition often described ... or other symptoms. Read More Publications Cover image Burning Mouth Syndrome Publication files Download Language English PDF — Number of ...

  3. Neuroleptic Malignant Syndrome

    Science.gov (United States)

    ... such as neuroleptic malignant syndrome. Much of this research focuses on finding ways to prevent and treat the disorder. Show More Show Less Search Disorders SEARCH SEARCH Definition Treatment Prognosis Clinical Trials Organizations Publications Definition Neuroleptic malignant syndrome is ...

  4. Skin Peeling Syndrome

    Directory of Open Access Journals (Sweden)

    Sharma Rajeev

    2000-01-01

    Full Text Available Peeling of the skin is an uncommonly encountered disorder. Occurrence of vesicles and bullae in peeling skin syndrome is very rare. We report a case of idiopathic peeling skin syndrome with vesicular lesions.

  5. [The Capgras syndrome].

    Science.gov (United States)

    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.

  6. 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.)....

  7. PRES syndrome

    International Nuclear Information System (INIS)

    Georgiev, R.; Novakova, M.; Balev, B.; Baleva, D.; Nedelchev, K.

    2010-01-01

    Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity characterized by headache, confusion, visual disturbances, seizures and posterior transient changes on neuroimaging. PRES has been described in several conditions including hypertensive encephalopathy, preeclampsia, eclampsia, infections, electrolyte imbalance, hypercalcaemia and use of several drugs. It occurs due to elevated blood pressure which exceeds the autoregulatory capacity of brain vasculature. The posterior circulation supplied by vertibro-basilar system has poor sympathetic innervation and, therefore, is frequently involved. The role of neuroimaging is to establish the initial diagnosis and to exclude other causes of neurological symptoms and signs. NCCT is sufficient to make the diagnosis in a proper clinical setting. MRI features are characteristic and has diagnostic and prognostic value. Diffusion weighted imaging (DWI) can differentiate this condition from ischemia/cytotoxic edema. Differential diagnosis of PRES includes PCA territory infarcts, venous thrombosis, demyelinating disorders, vasculitis and encephalitis. The diagnosis has important implications because the reversibility of the clinico-radiological abnormalities is contingent on the prompt control of blood pressure and/or withdrawing of the offending drug. We describe here a case of PRES in a 12 years old girl with acute lymphoblasts leukaemia, treated with cytostatics-vincristine, pharmorubycin and methotrexate. After 39 days from the beginning of the treatment there are good results in the myelogram and the flowcytometric examination, but the patient made two tonic-clonic seizures. CT and MRI were made and signs of leucoencephalopathy were diagnosed. Several control MRI examinations after cessation of the therapy and disappearance of the neurologic symptoms were made. The normal findings and the clinical course were the reasons for the PRES diagnosis

  8. Postthrombotic syndrome.

    Science.gov (United States)

    Pesavento, Raffaele; Bernardi, Enrico; Concolato, Alessia; Dalla Valle, Fabio; Pagnan, Antonio; Prandoni, Paolo

    2006-10-01

    Despite considerable progress in the diagnosis and treatment of deep vein thrombosis (DVT) of the lower extremities, one of every three patients will develop postthrombotic sequelae within 2 years; these sequelae are severe in approximately 20% of cases and produce considerable socioeconomic consequences. Among factors potentially related to the development of the postthrombotic syndrome (PTS) are older age, obesity, insufficient oral anticoagulant therapy, and recurrent ipsilateral thrombosis. Whether the extent and location of the initial thrombosis are associated with the development of PTS is controversial. Based on recent findings, the lack of vein recanalization within the first 6 months appears to be an important predictor of PTS, whereas the development of transpopliteal venous reflux is not. The diagnosis of PTS can be made on clinical grounds for patients with a history of DVT. The combination of a standardized clinical evaluation with the results of compression ultrasonography and Doppler ultrasound helps diagnose or exclude a previous proximal vein thrombosis. According to the results of recent clinical studies, the prompt administration of adequate compression elastic stockings in patients with symptomatic DVT has the potential to reduce the frequency of late PTS development by half. The management of this condition is demanding and often frustrating. However, when carefully supervised and instructed to wear proper elastic stockings, more than 50% of patients will either remain stable or improve during long-term follow-up. Clinical presentation helps predict the prognosis; the outcome of patients who refer with initially severe manifestations is more favorable than that of patients whose symptoms deteriorate progressively over time.

  9. Cardio-renal syndrome

    OpenAIRE

    Gnanaraj, Joseph; Radhakrishnan, Jai

    2016-01-01

    Cardio-renal syndrome is a commonly encountered problem in clinical practice. Its pathogenesis is not fully understood. The purpose of this article is to highlight the interaction between the cardiovascular system and the renal system and how their interaction results in the complex syndrome of cardio-renal dysfunction. Additionally, we outline the available therapeutic strategies to manage this complex syndrome.

  10. Facts about Down Syndrome

    Science.gov (United States)

    ... monitor children with Down syndrome for these conditions. Treatments Down syndrome is a lifelong condition. Services early in life ... of these services focus on helping children with Down syndrome develop to their ... therapy, and they are typically offered through early intervention ...

  11. Gorlin-goltz syndrome

    International Nuclear Information System (INIS)

    Ahmed, N.; Salman, M.; Mansoor, M.A.

    2007-01-01

    Multiple jaw cysts are a characteristic manifestation of basal cell nevus (Gorlin) syndrome. Gorlin-Goltz syndrome is characterized by symptoms primarily involving the skin, central nervous system, and skeletal system. In 90% of the patients, nevoid basal cell carcinoma syndrome is associated with recurring odontogenic keratocysts. This patient showed recurrent jaw and maxillary cysts, for which he was followed for 2 years. (author)

  12. Sjogren-Larsson Syndrome

    Science.gov (United States)

    ... 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: ...

  13. Cushing's syndrome during pregnancy

    NARCIS (Netherlands)

    Mulder, W. J.; Berghout, A.; Wiersinga, W. M.

    1990-01-01

    Two cases of Cushing's syndrome during pregnancy are reported, both due to an adrenal adenoma. The association of pregnancy and Cushing's syndrome has up to now been described in 48 patients (including our two cases); Cushing's syndrome was ACTH-independent in 59%, ACTH-dependent in 33%, and of

  14. Polycystic Ovary Syndrome FAQ

    Science.gov (United States)

    ... Ovary Syndrome (PCOS) • What are common signs and symptoms of polycystic ovary syndrome (PCOS)? • What causes PCOS? • What is insulin resistance? • ... with PCOS? •Glossary What are common signs and symptoms of polycystic ovary syndrome (PCOS)? Common PCOS signs and symptoms include the ...

  15. Diagnostik af Dravet 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. We describe the features of two cases with genetically verified SCNA1 mutations. The diagnosis was established rather late in one case. The epilepsies were medically intractable and the symptoms characteristic of Dravet syndrome. The children...

  16. The acute radiation syndrome

    International Nuclear Information System (INIS)

    Souhami Filho, L.

    1985-01-01

    Symptoms and signs from medical aspects resulting from whole body exposure, or in the main part, to ionizing radiation are described. The dose-response relationship is studied and the exposure is divided in three parts: central nervous system syndrome, gastrointestinal syndrome and hematopoietic syndrome. Brief comments about the treatment are reported. (M.A.C.) [pt

  17. DIDMOAD (Wolfram Syndrome

    Directory of Open Access Journals (Sweden)

    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

  18. 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.

  19. Gorlin-Goltz Syndrome

    Directory of Open Access Journals (Sweden)

    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.

  20. Barth syndrome

    Directory of Open Access Journals (Sweden)

    Clarke Sarah LN

    2013-02-01

    Full Text Available Abstract First described in 1983, Barth syndrome (BTHS is widely regarded as a rare X-linked genetic disease characterised by cardiomyopathy (CM, skeletal myopathy, growth delay, neutropenia and increased urinary excretion of 3-methylglutaconic acid (3-MGCA. Fewer than 200 living males are known worldwide, but evidence is accumulating that the disorder is substantially under-diagnosed. Clinical features include variable combinations of the following wide spectrum: dilated cardiomyopathy (DCM, hypertrophic cardiomyopathy (HCM, endocardial fibroelastosis (EFE, left ventricular non-compaction (LVNC, ventricular arrhythmia, sudden cardiac death, prolonged QTc interval, delayed motor milestones, proximal myopathy, lethargy and fatigue, neutropenia (absent to severe; persistent, intermittent or perfectly cyclical, compensatory monocytosis, recurrent bacterial infection, hypoglycaemia, lactic acidosis, growth and pubertal delay, feeding problems, failure to thrive, episodic diarrhoea, characteristic facies, and X-linked family history. Historically regarded as a cardiac disease, BTHS is now considered a multi-system disorder which may be first seen by many different specialists or generalists. Phenotypic breadth and variability present a major challenge to the diagnostician: some children with BTHS have never been neutropenic, whereas others lack increased 3-MGCA and a minority has occult or absent CM. Furthermore, BTHS was first described in 2010 as an unrecognised cause of fetal death. Disabling mutations or deletions of the tafazzin (TAZ gene, located at Xq28, cause the disorder by reducing remodeling of cardiolipin, a principal phospholipid of the inner mitochondrial membrane. A definitive biochemical test, based on detecting abnormal ratios of different cardiolipin species, was first described in 2008. Key areas of differential diagnosis include metabolic and viral cardiomyopathies, mitochondrial diseases, and many causes of neutropenia and

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

    Science.gov (United States)

    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.

  2. 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

  3. Cardiorenal Syndrome in Acute Heart Failure Syndromes

    Directory of Open Access Journals (Sweden)

    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.

  4. Genetics Home Reference: Marfan syndrome

    Science.gov (United States)

    ... 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 ...

  5. Fibrosing organising pneumonia.

    Science.gov (United States)

    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.

  6. 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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2003-06-01

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

  8. 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.

  9. Mobius syndrome: MRI features

    International Nuclear Information System (INIS)

    Markarian, Maria F.; Villarroel, Gonzalo M.; Nagel, Jorge R.

    2003-01-01

    Purpose: Mobius Syndrome or congenital facial diplegia is associated with paralysis of the lateral gaze movements. This syndrome may include other cranial nerve palsies and be associated to musculoskeletal anomalies. Our objective is to show the MRI findings in Mobius Syndrome. Material and methods: MRI study was performed in 3 patients with clinic diagnosis of Mobius Syndrome. RMI (1.5T); exams included axial FSE (T1 and T2), FLAIR, SE/EPI, GRE/20, sagittal FSE T2 , coronal T1, diffusion, angio MRI and Spectroscopy sequences. Results: The common features of this syndrome found in MRI were: depression or straightening of the floor of the fourth ventricle, brainstem anteroposterior diameter diminution, morphologic alteration of the pons and medulla oblongata and of the hypoglossal nuclei as well as severe micrognathia. Conclusion: The morphologic alterations of Mobius Syndrome can be clearly identified by MRI; this method has proved to be a useful diagnostic examination. (author)

  10. [Menopause and metabolic syndrome].

    Science.gov (United States)

    Meirelles, Ricardo M R

    2014-03-01

    The incidence of cardiovascular disease increases considerably after the menopause. One reason for the increased cardiovascular risk seems to be determined by metabolic syndrome, in which all components (visceral obesity, dyslipidemia, hypertension, and glucose metabolism disorder) are associated with higher incidence of coronary artery disease. After menopause, metabolic syndrome is more prevalent than in premenopausal women, and may plays an important role in the occurrence of myocardial infarction and other atherosclerotic and cardiovascular morbidities. Obesity, an essential component of the metabolic syndrome, is also associated with increased incidence of breast, endometrial, bowel, esophagus, and kidney cancer. The treatment of metabolic syndrome is based on the change in lifestyle and, when necessary, the use of medication directed to its components. In the presence of symptoms of the climacteric syndrome, hormonal therapy, when indicated, will also contribute to the improvement of the metabolic syndrome.

  11. Orofacial syndromes: A review

    Directory of Open Access Journals (Sweden)

    N Shyam Sunder

    2011-01-01

    Full Text Available A syndrome is a set of signs and symptoms that tend to occur together and reflect the presence of a particular disease or an increased chance of developing to a particular disease. There are numerous orofacial syndromes and a thorough knowledge of their manifestations and implications is pertinent in good oral health care delivery. The aim of this review is to describe collective esoteric knowledge, about various malformations and syndromes associated with orofacial region.

  12. Steele Richardson Olszewski syndrome

    Directory of Open Access Journals (Sweden)

    Vijayashree S Gokhale

    2013-01-01

    Full Text Available Parkinson′s disease and its plus syndromes are an important cause of morbidity in the geriatric age group. Its plus syndromes show a myriad of clinical features characterized by progressive symptoms. Here we present a 65-year-old woman with progressive "Parkinsonian-like features," i.e., mask-like face, slowness of all movements and tendency to fall, and difficulty in eye movements, leading to the diagnosis of Steele Richardson Olszewski Syndrome or progressive supranuclear palsy.

  13. Metabolic syndrome and menopause

    OpenAIRE

    Jouyandeh, Zahra; Nayebzadeh, Farnaz; Qorbani, Mostafa; Asadi, Mojgan

    2013-01-01

    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 t...

  14. Post cardiac injury syndrome

    DEFF Research Database (Denmark)

    Nielsen, S L; Nielsen, F E

    1991-01-01

    The post-pericardiotomy syndrome is a symptom complex which is similar in many respects to the post-myocardial infarction syndrome and these are summarized under the diagnosis of the Post Cardiac Injury Syndrome (PCIS). This condition, which is observed most frequently after open heart surgery, i...... on the coronary vessels, with cardiac tamponade and chronic pericardial exudate. In the lighter cases, PCIS may be treated with NSAID and, in the more severe cases, with systemic glucocorticoid which has a prompt effect....

  15. A seizuring alagille syndrome

    Directory of Open Access Journals (Sweden)

    Jomon Mathew John

    2017-01-01

    Full Text Available Alagille syndrome is a rare autosomal dominant inherited disorder with incidence of one in 100,000 live births. This syndrome with seizure as a presentation has been rarely reported in Indian studies. We present a 3-month-old infant who presented to us with seizures was found to have a dysmorphic face, jaundice, hepatomegaly, and soft systolic murmur. Infant was stabilized and remained seizure free. A detailed clinical evaluation of a common presentation may reveal a rare syndrome.

  16. Genetics Home Reference: Waardenburg syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Waardenburg syndrome Waardenburg syndrome Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Waardenburg syndrome is a group of genetic conditions that can ...

  17. What Is Antiphospholipid Antibody Syndrome?

    Science.gov (United States)

    ... Back To Health Topics / Antiphospholipid Antibody Syndrome Antiphospholipid Antibody Syndrome Also known as What Is Antiphospholipid (AN-te-fos-fo-LIP-id) antibody syndrome (APS) is an autoimmune disorder. Autoimmune disorders ...

  18. What Is Respiratory Distress Syndrome?

    Science.gov (United States)

    ... Home / Respiratory Distress Syndrome Respiratory Distress Syndrome Also known as What Is Respiratory ... This condition is called apnea (AP-ne-ah). Respiratory Distress Syndrome Complications Depending on the severity of ...

  19. Genetics Home Reference: Turner syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Turner syndrome Turner syndrome Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Turner syndrome is a chromosomal condition that affects development in ...

  20. Guide to Understanding Pfeiffer Syndrome

    Science.gov (United States)

    ... syndrome occurs more often in children with older fathers. if I have pfeiffer syndrome what are the odds of passing it to my children? p feiffer syndrome is a rare, autosomal dominant disorder, meaning it requires only one parent to ...

  1. Genetics Home Reference: Cockayne syndrome

    Science.gov (United States)

    ... Cockayne syndrome type II is also known as cerebro-oculo-facio-skeletal (COFS) syndrome, and while some ... link) National Institute of Neurological Disorders and Stroke: Cerebro-Oculo-Facio-Skeletal Syndrome Educational Resources (8 links) ...

  2. Genetics Home Reference: MEGDEL syndrome

    Science.gov (United States)

    ... Leigh-like syndrome 3-methylglutaconic aciduria with deafness, encephalopathy, and Leigh-like syndrome MEGDHEL syndrome SERAC1 ... Topic: Newborn Screening Genetic and Rare Diseases Information Center (1 ...

  3. Genetics Home Reference: Usher syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Usher syndrome Usher syndrome Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Usher syndrome is a condition characterized by partial or total ...

  4. Genetics Home Reference: Bartter syndrome

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Bartter syndrome Bartter syndrome Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Bartter syndrome is a group of very similar kidney disorders ...

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

    Directory of Open Access Journals (Sweden)

    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.

  6. 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.

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

    Science.gov (United States)

    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.

  8. Lesch-Nyhan Syndrome

    Science.gov (United States)

    ... National Organization for Rare Disorders (NORD) Purine Research Society See all related organizations Publications Order NINDS Publications Definition Lesch-Nyhan syndrome (LNS) is a rare, inherited ...

  9. SNEDDON’S SYNDROME

    Directory of Open Access Journals (Sweden)

    Valentin Valtchev

    2008-10-01

    Full Text Available Sneddon’s syndrome is usually characterized by the association of an ischemic cerebrovascular disease and a widespread livedo reticularis. The incidence of Sneddon syndrome is 4/1000 000. We present 42-year-old woman with livedo reticularis, recurrence ischaemic cerebrovascular accidents, two repetitive miscarriages and positive anti-2GPi antibodies. Skin biopsy specimens reveal inflammatory changes of small- to medium-sized arteries and subendothelial proliferation and fibrosis. The diagnosis Sneddon syndrome is confirmed by skin biopsy, and MR evidence. We suggest that anti-2GPi antibodies may be pathophysiologically related to the clinical manifestation observed in some patients with Sneddon syndrome.

  10. Fragile X syndrome

    Science.gov (United States)

    ... problems, or intellectual disability may not be present. Symptoms Behavior problems associated with fragile X syndrome include: Autism spectrum disorder Delay in crawling, walking, or twisting Hand flapping ...

  11. [Neurobiology of Tourette Syndrome].

    Science.gov (United States)

    Ünal, Dilek; Akdemir, Devrim

    2016-01-01

    Tourette Syndrome (TS) is a neurodevelopmental disorder characterized by chronic motor and vocal tics. Although it is a common disorder in childhood, the etiology of Tourette Syndrome has not been fully elucidated yet. Studies, -conducted so far- have revealed differences in neurobiological structures of individuals who suffer from Tourette Syndrome. The objective of this review is to assess etiological and pathophysiological studies in the Tourette Syndrome literature. An electronical search was conducted in PubMed database using the keywords tic disorders, Tourette Syndrome, neurobiology, genetics, neuroimaging and animal models. Research and review studies published between 1985 and 2015, with a selection preference towards recent publications, were reviewed. According to the studies, genetic predisposition hypothesis is considered as a priority. However, a precise genetic disorder associated with Tourette Syndrome has not been found. The evidence from postmortem and neuroimaging studies in heterogenous patient groups and animal studies supports the pathological involvement of cortico-striato-thalamo-cortical (CSTC) circuits in Tourette Syndrome. Consequently, the most emphasized hypothesis in the pathophysiology is the dopaminergic dysfunction in these circuits. Furthermore, these findings of the animal, postmortem and neuroimaging studies have confirmed the neurodevelopmental hypothesis of Tourette Syndrome. In conclusion, more studies are needed to understand the etiology of the disorder. The data obtained from neurobiological studies of the disorder will not only shed light on the way of Tourette Syndrome, but also guide studies on its treatment options.

  12. Cushing's syndrome in pregnancy.

    Science.gov (United States)

    Nassi, Rossella; Ladu, Cristina; Vezzosi, Chiara; Mannelli, Massimo

    2015-02-01

    Cushing's syndrome is a rare condition in the general population and is even less common during pregnancy with only a few cases reported in literature. The diagnosis of Cushing's syndrome may be difficult during pregnancy because the typical features of the disorder and pregnancy may overlap. However, Cushing's syndrome results in increased fetal and maternal complications, and diagnosis and treatment are critical. This report describes a case of 26-year-old female at the 19th week of pregnancy with symptoms and signs of hypercortisolism, where ACTH-independent Cushing's syndrome was diagnosed and treated by robotic laparoscopic adrenalectomy at the 21th week of gestation.

  13. Central Pain Syndrome

    Science.gov (United States)

    ... such as neurontin (gabapentin) can be useful. Lowering stress levels appears to reduce pain. View Full Treatment Information Definition Central pain syndrome is a neurological condition caused ...

  14. 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)

  15. 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)

  16. 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.

  17. Wolfram syndrome 1 and Wolfram syndrome 2.

    Science.gov (United States)

    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.

  18. Bardet-Biedl syndrome and Usher syndrome.

    Science.gov (United States)

    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.

  19. Alport Syndrome Diagnosis

    Science.gov (United States)

    ... 4 months later, my father walked me down the aisle. We danced to a Beatles song & then to j… Connect on Instagram Email: info@alportsyndrome.org Phone: (480) 800-3510 Mailing Address: Alport syndrome Foundation P.O. Box 4130 ... 5, 2017 Cecil Alport: Naming the Syndrome November 26, 2016 The Renal Diet: Potassium ...

  20. Adult onset Leigh syndrome

    Directory of Open Access Journals (Sweden)

    Pandit Lekha

    2007-01-01

    Full Text Available Leigh syndrome is a rare progressive mitochondrial disorder of oxidative metabolism. Though it has been reported in infancy and childhood, it is rarely described in adults. The authors describe a patient who had clinical and magnetic resonance imaging features diagnostic of Leigh syndrome, with supportive biochemical and muscle histochemistry evidence.