Liver pathology in alpha1-antitrypsin deficiency. A review.
S Afr Med J, 1975/5/17;49(21):849-52.
PMID: 166455
Impact factor: 2.162
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
Adult; Aged; Biopsy; Carcinoma, Hepatocellular; Cholestasis; Cytoplasmic Granules; Histocytochemistry; Homozygote; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Liver; Liver Cirrhosis; Liver Diseases; Liver Neoplasms; Middle Aged; Prognosis; alpha 1-Antitrypsin; alpha 1-Antitrypsin Deficiency
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