title: [Clinical aspects, diagnostic lung function and biochemical parameters in children with homozygous alpha 1-antitrypsin deficiency].
abstract: Both the clinical course of the homozygous alpha 1-PI deficiency and also the pulmonary function and measured clinical parameters in these children revealed very marked inter-individual fluctuations. In one child, the lung function revealed certain signs of incipient pulmonary emphysema. The biochemical parameters of two children revealed TIC/alpha 1-PI- and PEIC/alpha 1-PI ratios greater than 1. This indicates that, in addition to alpha 1-PI, further inhibitors may develop a compensatory effect.
authors.affiliation: Thoraxklinik, Heidelberg-Rohrbach.
authors.forename: B
authors.initials: B
authors.lastname: Heck
authors.forename: W
authors.initials: W
authors.lastname: Ebert
authors.forename: V
authors.initials: V
authors.lastname: Schulz
authors.forename: G
authors.initials: G
authors.lastname: Trefz
authors.forename: K
authors.initials: K
authors.lastname: Paul
mesh_terms: D002648:Child; D006720:Homozygote; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000175:diagnosis / Q000235:genetics*; D008176:Lung Volume Measurements; D010993:Plethysmography, Whole Body; D012307:Risk Factors; D013147:Spirometry; D019896:alpha 1-Antitrypsin Deficiency
vernacular_title: Klinik, lungenfunktionsdiagnostische und biochemische Parameter bei Kindern mit homozygotem alpha 1-PI-Mangel.