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Pulmonary function in children and young adults with ataxia telangiectasia
Author(s) -
McGrathMorrow Sharon A.,
Lederman Howard M.,
Aherrera Angela D.,
LeftonGreif Maureen A.,
Crawford Thomas O.,
Ryan Timothy,
Wright Jennifer,
Collaco Joseph M.
Publication year - 2014
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.22760
Subject(s) - medicine , pulmonary function testing , vital capacity , pediatrics , population , young adult , lung , ataxia telangiectasia , lung function , diffusing capacity , dna , genetics , biology , dna damage , environmental health
Background Pulmonary disease contributes to significant morbidity and mortality in people with ataxia telangiectasia (A‐T). To determine the association between age and lung function in children and young adults with A‐T and to identify factors associated with decreased lung function, pulmonary function tests were performed in 100 consecutive people with A‐T. Methods Children and adults ranging from 6 to 29 years of age and with the diagnosis of A‐T were recruited, and underwent pulmonary function tests. Results The mean forced vital capacity % predicted (FVC %) in the population was 56.6 ± 20.0. Males and females between 6 and 10 years of age had similar pulmonary function. Older females were found to have significantly lower FVCs % than both older males ( P < 0.02) and younger females ( P < 0.001). The use of supplemental gamma globulin was associated with significantly lower FVC %. A modest correlation was found between higher radiation‐induced chromosomal breakage and lower FVC % in males. No significant change in FVC % was found in a subset of subjects (n = 25) who underwent pulmonary function testing on two or more occasions over an average of 2 years. Conclusion In children and young adults with A‐T, older females and people who required supplemental gamma globulin had significantly lower lung function by cross‐sectional analysis. Stable lung function is possible over a 2‐year period. Recognition of groups who are at higher risk for lower pulmonary function may help direct care and improve clinical outcomes in people with A‐T. Pediatr Pulmonol. 2014; 49:84–990. © 2013 Wiley Periodicals, Inc.