Arch Dis Child 1985 May;60(5):447-51

Long term prognosis for babies with neonatal liver disease

Deutsch J, Smith AL, Danks DM, Campbell PE

Abstract
A total of 123 patients with neonatal liver disease without extrahepatic bile
duct obstruction or arteriohepatic dysplasia have been studied for six to 18
years. Idiopathic neonatal hepatitis, present in 73 babies, carried a high
mortality due to liver failure (18%), septicaemia (6%), and associated defects
(14%), especially in the first year of life (25%). Progression to chronic liver
disease in non-familial idiopathic cases occurred in three of 40 reviewed
patients. Only 12 of these children were completely healthy, the remainder
having other permanent disabilities (57%). Four of nine familial cases of
idiopathic neonatal hepatitis died in the first 12 months of life as did two of
the four reviewed survivors. Progression to chronic liver disease or to death
was a continuous process without any interval of recovery in all but one of
these patients. Among patients with a presumed infective cause, cytomegalovirus
infection caused a particularly benign form of neonatal hepatitis but was a
frequent cause of brain damage or other disabilities. Babies who survived other
infective liver diseases showed complete healing of the liver damage. Neonatal
liver disease associated with alpha 1 antitrypsin deficiency progressed to death
or chronic liver disease in three of nine patients and was not associated with a
paucity of interlobular bile ducts