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Lung function in adolescents with alpha 1-antitrypsin deficiency

T Sveger1, E Piitulainen, M Arborelius

  • 1Department of Paediatrics, University of Lund, Malmö General Hosptial, Sweden.

Acta Paediatrica (Oslo, Norway : 1992)
|November 1, 1994
PubMed
Summary

Children with alpha 1-antitrypsin deficiency show normal lung development by age 16. This study found a favorable prognosis for these individuals, with anti-smoking advice proving effective in preventing smoking initiation.

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Area of Science:

  • Pediatrics
  • Pulmonology
  • Genetics

Background:

  • Alpha 1-antitrypsin deficiency (AATD) is a genetic condition that can lead to lung disease.
  • Early screening and prospective follow-up are crucial for understanding AATD's long-term effects in children.

Purpose of the Study:

  • To assess lung function and overall health in adolescents with AATD at 16 years of age.
  • To compare lung function and respiratory health between AATD subtypes (PiZ, PiSZ) and healthy controls.

Main Methods:

  • Prospective follow-up of children screened at birth for AATD.
  • Lung function tests (FVC, FEV1, TLC, RV, FRC) and standardized questionnaires administered to 150 adolescents with AATD and 50 controls.
  • Comparison of respiratory symptoms, smoking habits, and asthma prevalence.

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Main Results:

  • No significant differences in lung function, respiratory symptoms, or smoking habits were observed between AATD subtypes and controls at 16 years.
  • Asthma prevalence was similar across groups (10.7% PiZ, 6.5% PiSZ, 4% controls).
  • Anti-smoking advice was effective, with only 3% of participants initiating smoking.

Conclusions:

  • Children with alpha 1-antitrypsin deficiency have a favorable prognosis with normal lung development up to 16 years of age.
  • Genetic factors of AATD did not significantly impact lung function variables in this cohort.
  • Public health interventions, such as smoking cessation advice, are effective in this population.