Premium
α–Antitrypsin Deficiency
Author(s) -
Alagille Daniel
Publication year - 1984
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.1840040705
Subject(s) - cirrhosis , medicine , cholestasis , gastroenterology , portal hypertension , neonatal cholestasis , jaundice , liver function , congenital hepatic fibrosis , fibrosis , pediatrics , biliary atresia , transplantation , liver transplantation
Liver disease related to a–1–antitrypsin deficiency occurs only in Pi ZZ homozygous children. Eleven per cent of Pi ZZ infants present with prolonged neonatal cholestasis. In our group, 25 of 45 Pi ZZ infants with prolonged neonatal cholestasis presented with later cirrhosis. Persistence of jaundice beyond the sixth month of age, early development of splenomegaly, persistence of hard hepatomegaly and liver function abnormalities, and early portal fibrosis have a poor prognostic significance. The most severe course occurs in infants with an early histologic pattern of paucity of interlobular bile ducts. Portal hypertension was present in 19 of 25 children presenting with cirrhosis; 8 of 25 Pi ZZ children with cirrhosis died during childhood. Long‐term protein‐restricted diet and portal systemic shunts were helpful in treatment of four Pi ZZ children with cirrhosis; however, the long‐term course in Pi ZZ children with cirrhosis is unpredictable.