title: Liver pathology in alpha1-antitrypsin deficiency. A review.
abstract: Alpha1-antitrypsin deficiency (AATD), in addition to its association with chronic pulmonary disease, is reported with liver disease. Twenty per cent of Pi-type ZZ AATD infants present with a cholestatic type of neonatal hepatitis and develop a slowly progressive cirrhosis, and most die before adult life. Ten per cent of Pi-type ZZ adults develop cirrhosis. They have an increased frequency of primary liver carcinoma. In Z homozygotes and heterozygotes specific globules, due to accumulation of a type of alpha1-antitrypsin, are seen in liver cells. They are thought not to be hepatotoxic but to render the liver cell more susceptible to damage by an additional factor.
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D006528:Carcinoma, Hepatocellular / Q000473:pathology; D002779:Cholestasis / Q000473:pathology; D003594:Cytoplasmic Granules / Q000032:analysis; D006651:Histocytochemistry; D006720:Homozygote; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000473:pathology; D008099:Liver / Q000032:analysis / Q000378:metabolism / Q000473:pathology*; D008103:Liver Cirrhosis / Q000473:pathology; D008107:Liver Diseases / Q000175:diagnosis / Q000209:etiology; D008113:Liver Neoplasms / Q000473:pathology; D008875:Middle Aged; D011379:Prognosis; D000515:alpha 1-Antitrypsin / Q000032:analysis / Q000378:metabolism; D019896:alpha 1-Antitrypsin Deficiency
chemical_list: D000515:alpha 1-Antitrypsin