| Reversible airway obstruction in children with ataxia telangiectasia. | |
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MedLine Citation:
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PMID: 20146367 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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INTRODUCTION: Lung disease is a significant cause of the short life span of ataxia telangiectasia (A-T) patients. Objective lung function measurements are difficult to achieve in A-T. AIM: To assess lung function by spirometry in relation to the clinical characteristics of A-T patients followed up at the Israeli Ataxia Telangiectasia National Clinic. PATIENTS AND METHODS: Medical and spirometry data were collected from 27 A-T patients during 2004-2007. Laboratory, nutritional condition, mode of treatment, pulmonary status, and malignancies were assessed. The spirometry values FVC, FEV(1), FEV(0.5), FEF(25-75), PEF and time rise to peak flow were analyzed individually and values were compared to those of healthy age-matched children. RESULTS: Eleven patients (40.7%) were found to suffer from asthma according to clinical symptoms and response to bronchodilators. We found significant reduction in FEV(1) and FEV(0.5) (z-scores: -0.84 + or - 0.7 SD, -0.7 + or - 0.6 SD; P = 0.0014 and P = 0.003, respectively), in relation to healthy predicted values. FEF(25-75) was significantly lower than that in healthy children in 5 of 11 asthmatic patients. All 27 patients showed higher than healthy FEV(1)/FVC and FEV(0.5)/FVC ratios (z-scores 0.68 + or - 0.99 SD, P < 0.0015, and 2.12 + or - 1.50 SD, P < 0.0015, respectively). The rise time to peak flow was three-fold longer than that of healthy children. CONCLUSION: Obstructive lung disease is common among A-T patients. Maximal peak flow reduction and prolonged rise time to peak flow may be the first signs of pulmonary involvement in these patients. Early treatment with anti-asthma therapy, bronchodilators, and steroids, may prevent further pulmonary deterioration and improve the prognosis of A-T patients. |
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Authors:
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Yackov Berkun; Daphna Vilozni; Yonit Levi; Sheron Borik; Dalia Waldman; Raz Somech; Andreea Nissenkorn; Ori Efrati |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric pulmonology Volume: 45 ISSN: 1099-0496 ISO Abbreviation: Pediatr. Pulmonol. Publication Date: 2010 Mar |
Date Detail:
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Created Date: 2010-02-26 Completed Date: 2010-06-16 Revised Date: 2010-09-30 |
Medline Journal Info:
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Nlm Unique ID: 8510590 Medline TA: Pediatr Pulmonol Country: United States |
Other Details:
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Languages: eng Pagination: 230-5 Citation Subset: IM |
Affiliation:
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Ataxia Telangiectasia National Clinic, Chaim Sheba Medical Center, Tel Hashomer, affiliated with the Sackler Medical School, Tel-Aviv University, Israel. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Administration, Inhalation Adolescent Anti-Asthmatic Agents / administration & dosage Asthma / complications, drug therapy Ataxia Telangiectasia / complications* Bronchodilator Agents / administration & dosage* Child Child, Preschool Female Forced Expiratory Volume Humans Male Pulmonary Disease, Chronic Obstructive / drug therapy*, etiology*, physiopathology Spirometry Vital Capacity Young Adult |
| Chemical | |
Reg. No./Substance:
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0/Anti-Asthmatic Agents; 0/Bronchodilator Agents |
| Comments/Corrections | |
Comment In:
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Pediatr Pulmonol. 2010 Oct;45(10):1043-4; author reply 1045
[PMID:
20597085
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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