[Alpha 1 antitrypsin deficiency in childhood].

The author deals with the clinical importance of the alpha-1-antitrypsin deficiency in form of a survey with tabular representation of own cases. Alpha-1-antitrypsin as an inactivator of proteolytically effective enzymes essentially participates in the localised effect of proteinases. A genetically determined decreased alpha-1-antitrypsin serum level (= alpha-1-antitrypsin deficiency) by this causally participates in the pathogenesis of certain hepatopathies and in the pulmonary emphysema appearing already at the carly adult age. Within differential-diagnostic considerations an alpha-1-antitrypsin deficiency should be excluded in: 1. etiologically unclear cholestasis in infancy, 2. etiologically unclear hepatopathy in childhood, and in 3. early emphysema.