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Pulmonary function in children and young adults with ataxia telangiectasia

Authors

  • Sharon A. McGrath-Morrow MD,

    Corresponding author
    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
    • Correspondence to: Eudowood Division of Pediatric Respiratory Sciences, David M. Rubenstein Building, Suite 3075B, 200 North Wolfe Street, Baltimore, MD 21287-2533. E-mail: smcgrath@jhmi.edu

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  • Howard M. Lederman MD, PhD,

    1. Division of Allergy and Immunology, Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Angela D. Aherrera BA,

    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Maureen A. Lefton-Greif PhD,

    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Thomas O. Crawford MD,

    1. Department of Neurology and Adjunct in Department of Pediatrics, The Johns Hopkins Medical Institutions, Baltimore, Maryland
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  • Timothy Ryan,

    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Jennifer Wright RN,

    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
    2. Division of Allergy and Immunology, Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Joseph M. Collaco MD

    1. Eudowood Division of Pediatric Respiratory Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland
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  • Disclosures: All authors disclose that they have no financial interests in the subject of this manuscript.

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. © 2013 Wiley Periodicals, Inc.

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