title: [Homozygous alpha 1-antitrypsin deficiency. Lung changes in children and adolescents].
abstract: 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).
authors.affiliation: Universitäts-Kinderklinik Heidelberg.
authors.forename: K
authors.initials: K
authors.lastname: Paul
authors.forename: B
authors.initials: B
authors.lastname: Niggemann
mesh_terms: D000293:Adolescent; D016535:Bronchial Hyperreactivity / Q000235:genetics*; D002648:Child; D005820:Genetic Testing; D006720:Homozygote; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000235:genetics*; D008176:Lung Volume Measurements; D010641:Phenotype; D014028:Tobacco Smoke Pollution / Q000009:adverse effects; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D014028:Tobacco Smoke Pollution; D000515:alpha 1-Antitrypsin
vernacular_title: Homozygotemer alpha 1-Antitrypsinmangel. Lungenveränderugen bei Kindern und Jugendlichen.