Abstract

Three premature infants are reported who developed obstructive jaundice in the first 3 months of life, with a chemical and histological picture consistent with neonatal hepatitis. The disease was complicated by gross generalized aminoaciduria, rickets, and anaemia. Serum tyrosine levels were raised to 0·28-1·64 μmole/ml in 2 infants, and did not fall in response to ascorbic acid given orally. The infants recovered over the next 3 to 5 months without specific therapy. Many features of the illness in the 3 infants are suggestive of hereditary tyrosinosis, but the transient nature of the illness excluded this diagnosis.

It is suggested that the infants had neonatal hepatitis and that the aminoaciduria and rickets were complications of the hepatitis and the prematurity. The recognition of this syndrome of neonatal hepatitis simulating tyrosinosis is important, if unnecessary and possibly dangerous therapy directed at hereditary tyrosinosis is to be avoided.