title: Pulmonary function in children and young adults with ataxia telangiectasia.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.
authors: Sharon A
authors: SA
authors: McGrath-Morrow
authors: Howard M
authors: HM
authors: Lederman
authors: Angela D
authors: AD
authors: Aherrera
authors: Maureen A
authors: MA
authors: Lefton-Greif
authors: Thomas O
authors: TO
authors: Crawford
authors: Timothy
authors: T
authors: Ryan
authors: Jennifer
authors: J
authors: Wright
authors: Joseph M
authors: JM
authors: Collaco
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001260:Ataxia Telangiectasia / Q000235:genetics / Q000503:physiopathology*; D002648:Child; D019457:Chromosome Breakage; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D012129:Respiratory Function Tests; D012737:Sex Factors; D013147:Spirometry; D014797:Vital Capacity; D005719:gamma-Globulins / Q000009:adverse effects
chemical_list: D005719:gamma-Globulins
keywords: ataxia telangiectasia; lung; pulmonary function
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