Idiopathic neonatal hepatitis: a long term follow-up study

Abstract

SUMMARY
Background: Idiopathic neonatal hepatitis (INH) is the term
given to any hepatitis that occurs from birth up to the 4th
month of life and remains etiologically unclassified after
clinical and laboratory evaluation. Our purpose was to find
symptoms, natural history or factors associated with hepatobiliary
disease in infancy and long term prognosis of nonfamilial
type of INH. Methods: We followed up 33 cases of
INH over a period of 12 years. Features of INH included: a)
presence of conjugated hyperbilirubinaemia ?3mg% lasting
for more than 2 weeks during the first 4 months of life,
b) biochemical and histological features of hepatocellular
damage and c) exclusion of all other causes of conjugated
hyperbilirubinaemia. Results: Jaundice was developed in all
but one child. The liver was palpable in all patients and
spleen was present in nineteen patients. Twelve patients
with severe anemia (Hb<6gr%) required a blood transfusion.
Associated features were history of prematurity (14),
perinatal asphyxia (7), septicemia (9), respiratory distress
syndrome (4), and hypoglycemia (5). All patients had abnormal
liver function tests indicative of cholestasis while
percutaneous liver biopsy revealed histological changes of
neonatal hepatitis in all patients with changes of giant cell
hepatitis in 9 neonates and non-specific changes (extramedullary
haemopoiesis) in 4 neonates. According to the outcome,
10 patients had a moderate to severe outcome regarding
liver disease. Two of them died, one from septicemia
and the other from fulminant hepatic failure at the age of 6
and 9 weeks respectively. The remaining 8 patients are still being followed up because they developed features of possible
chronic liver disease. A second liver biopsy in these
children revealed mild fibrosis in three and findings of nonspecific
hepatitis in five. Twenty-three infants made a
regular recovery from liver disease. Conclusion: We consider
that the outcome of INH is good despite the serious problems
that may present early in the course of the disease
and the overall mortality seems to be low. Associated problems
such as developmental delay should be recognized and
addressed promptly.
Key words: Cholestasis, jaundice neonatal hepatitis, idiopathic
neonatal hepatitis.