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The feasibility and validity of forced spirometry in ataxia telangiectasia
Author(s) -
Vilozni Daphna,
Berkun Yakov,
Levi Yonit,
Weiss Batya,
Jacobson Jeffrey M.,
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.21291
Subject(s) - medicine , spirometry , vital capacity , expiration , airway obstruction , physical therapy , asthma , pulmonary function testing , cardiology , pediatrics , airway , respiratory system , anesthesia , diffusing capacity , lung function , lung
Objectives To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A‐T). Study design Twenty‐eight patients (aged 3.7–19.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness. Results Start of test criteria for correct expiratory effort was significantly prolonged (183 ± 115 ms; P < 0.001). The rise‐time to peak flow in children free of respiratory symptoms (Group‐FRS; n = 8) increased by 16.2 ± 12.5 ms/year above recommended and in children having recurrent infections (n = 8) 30.4 ± 16.1 ms/year, P < 0.01. Expiration‐time was significantly shorter than requested (1.21 ± 0.47 sec) and was ended abruptly in 57% of the patients. FEV 1 could not be established by 8/20 patients. The intra‐subject reproducibility met criteria (4.4 ± 2.7%, 5.2 ± 2.8%, 2.9 ± 3.2%, 6.3 ± 5.3%, for FVC, FEV 0.5 , PEF, FEF 25–75 , respectively). Group‐FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper‐reactive airways (Group‐HRA; n = 12) had a deterioration rate of 3.6%/year. FEV 0.5 deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group‐HRA were significantly lower than those of Group‐FRS ( P = 0.029) in similar young ages, indicating airway obstruction at early ages in Group‐HRA. FEV 0.5 values deterioration also correlated with body mass index ( P < 0.017). Conclusion Forced spirometry in A‐T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A‐T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results. Pediatr Pulmonol. 2010; 45:1030–1036. © 2010 Wiley‐Liss, Inc.