Homozygous alpha 1-antitrypsin deficiency. Lung changes in children and adolescents
Paul, K.; Niggemann, B.
Monatsschrift Kinderheilkunde Organ der Deutschen Gesellschaft für Kinderheilkunde 141(5): 395-400
1993
ISSN/ISBN: 0026-9298 PMID: 8326958 Document Number: 415935
Homozygous alpha 1-antitrypsin deficiency can lead to pan-lobular emphysema early in adulthood. Based on own studies and a review of the literature the article dates up the knowledge about risk factors (especially smoking), the variable clinical pattern (relationship to bronchial hyperreactivity) and pulmonary function (30% pulmonary hyperinflation) of children and adolescents with homozygous alpha 1-antitrypsin deficiency (PIZZ). It discusses diagnostic (selective screening, regular lung function tests) and therapeutic possibilities (substitution) as well as preventive aspects (passive and active smoking cessation).