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Reversible airway obstruction in children with ataxia telangiectasia
Author(s) -
Berkun Yackov,
Vilozni Daphna,
Levi Yonit,
Borik Sheron,
Waldman Dalia,
Somech Raz,
Nissenkorn Andreea,
Efrati Ori
Publication year - 2010
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.21095
Subject(s) - medicine , spirometry , asthma , airway obstruction , ataxia telangiectasia , cardiology , pulmonary function testing , obstructive lung disease , pediatrics , airway , surgery , copd , dna , genetics , biology , dna damage
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 ± 0.7 SD, −0.7 ± 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 ± 0.99 SD, P < 0.0015, and 2.12 ± 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. Pediatr Pulmonol. 2010; 45:230–235. © 2010 Wiley‐Liss, Inc.