Pulmonary function in children and young adults with ataxia telangiectasia

Sharon A. McGrath-Morrow, Howard M. Lederman, Angela D. Aherrera, Maureen A. Lefton-Greif, Thomas O. Crawford, Timothy Ryan, Jennifer Wright, Joseph M. Collaco

Research output: Contribution to journalArticlepeer-review

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. 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.

Original languageEnglish (US)
Pages (from-to)84-990
Number of pages907
JournalPediatric pulmonology
Volume49
Issue number1
DOIs
StatePublished - Jan 2014

Keywords

  • ataxia telangiectasia
  • lung
  • pulmonary function

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pulmonary and Respiratory Medicine

Fingerprint

Dive into the research topics of 'Pulmonary function in children and young adults with ataxia telangiectasia'. Together they form a unique fingerprint.

Cite this