The case of a child with persistent severe jaundice, beginning in the neonatal period, and progressive neurologic symptoms, with onset at the age of 3 years is reported. This patient is considered to be another example of the disease described originally by Crigler and Najjar. The severe jaundice is the result of reduced capacity of the liver to excrete bilirubin, although the pathophysiologic mechanism is obscure. The neurologic syndrome is believed to be the result of bilirubin encephalopathy. If this be the case, the course of this patient indicates that development of bilirubin encephalopathy is not necessarily confined to infancy. It can apparently occur in young children, and probably in older children and adults, if the concentrations of indirect-reacting bilirubin in the serum are sufficiently high for a prolonged period.

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