title: [Liver disease in homozygous alpha1-antitrypsin deficiency (author's transl)].
abstract: Among twelve patients with homozygous alpha1-antitrypsin deficiency (Pi-type Z), five cases of infantile liver disease were diagnosed. The course of the disease was extremely variable; only one patient died of liver cirrhosis at the age of fourteen. In four cases the clinical, biochemical and histological (2 cases) findings became normal over a follow-up period of one to fifteen years. The results of these observations demonstrate that in alpha1-antitrypsin deficiency even when associated with proven liver disease the prognosis need not be unfavorable.
mesh_terms: D000293:Adolescent; D001706:Biopsy; D001796:Blood Protein Disorders / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006720:Homozygote; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000209:etiology; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D008297:Male; D011379:Prognosis; D019896:alpha 1-Antitrypsin Deficiency
vernacular_title: Hepatopathie bei homozygotem alpha1-Antitrypsin-Mangel.