Kernikterus not associated with haemolytic disease.

Kernikterus (bile staining of brain nuclei at necropsy or jaundice causing neurological signs during life) not associated with haemolytic disease has been reported in the literature during the last few years. Black-Schaffer, Kambe, Furuta and Moloney (1954) described 13 cases which had died with kernikterus in Hiroshima during the neonatal period. Eleven of the 13 cases were premature infants. Aidin, Corner and Tovey (1950) likewise described 24 cases of kernikterus of which 23 were premature infants. Crigler and Najjar (1952) described a case of kernikterus in a family with an inherited bilirubin excretion defect in the liver, and Ehrlich and Ratner (1955) described a case of kernikterus in an infant with ' neonatal hepatitis'. Most of the published cases dealt with premature infants, but in the following series it will be shown that kernikterus in full-term infants not associated with iso-immunization is quite common in Singapore. During the period from March, 1955, to December, 1955, 96 cases of jaundice during the first year of life were admitted to the Paediatric Unit of the General Hospital. In all these cases, the blood of the infant was tested for Rh and ABO antibodies and the Coombs antiglobulin test was done, but only two cases were found to be caused by isoimmunization (one due to Rh incompatibility and one due to ABO incompatibility). There were, however, 26 cases showing the symptoms, signs or pathological lesions of kernikterus. These 26 infants were all full term, under the age of 15 days on admission, and showed no evidence of iso-immunization. The criteria for diagnosis of these cases were a high serum bilirubin level with or without severe jaundice, with neurological signs, and evidence of bile-staining in the brain nuclei at necropsy in those who died. In more than half of the 26 cases the serum bilirubin was estimated after the manner of Hsia and his colleagues' modification of Evelyn and Malloy's method. The one-minute value corresponds to the 'direct-bilirubin', the normal for which is 0-0 2 mg. % for all ages, and the 30-minute value is the 'indirect-bitirubin', the normal for which one week after birth is less than 0 8 mg. %.