1 -Antitrypsin deficiency and neonatal hepatitis

Abstract

Five out of 28 infants investigated in a regional survey of neonatal hepatitis were found to have genetically-determined deficiency of alpha(1)-antitrypsin (ZZ phenotype). The clinical course and pathological changes varied considerably. All five infants had an acute hepatitis-like illness, and although this subsided cirrhosis later developed in three cases. The remaining two infants had minimal abnormalities of the liver function tests at 12 and 18 months of age, and one had increased hepatic fibrosis. Australia antigen was found in the serum of three infants, and Australia antigen or antibody in one or both parents of these and of one further case whose serum was negative. It is suggested that the association of neonatal hepatitis with alpha(1)-antitrypsin deficiency may be commoner than previously realized and that Australia antigen acts as a trigger factor in these cases.