Article

Congenital Nonhemolytic Jaundice (Crigler-Najjar Syndrome): Report of a Case

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Abstract

CONGENITAL nonhemolytic jaundice was first reported in 1952 by Crigler and Najjar.1 The seven infants they described formed part of a large kindred in western Maryland which was closely inbred. Six of the infants died with kernicterus in the first year of life; while the survivor, at five years of age, was reported by Childs and Najjar2 in 1956 as being healthy despite icterus. A case of late involvement of the central nervous system was reported by Rosenthal.3 The syndrome in a Negro child was described by Whitington4 in 1960.

The present case is being reported because of the rarity of the condition, the absence of consanguinity, and the unusual renal findings.

Report of a Case

A four-week-old male was first examined at the Cleveland Clinic on June 15, 1962, because of persistent jaundice. His mother was gravida 2, para 2, and Rh positive. She is of Spanish descent, and has resided in the United States for six years. The father is ethnically Anglo-Saxon, and American for several generations. They stated that there was no possibility of consanguinity. The sibling, a female, aged 19 months, was in good health. The maternal grandmother was said to have been jaundiced most of her life and to have died in her eighth decade.

The mother was not a diabetic, and she received no steroids or estrogens during pregnancy. Apart from an illness described as “flu” during the second trimester, the gestation period of 36 weeks was uneventful. After the spontaneous onset of labor . . .


 

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