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Article
February 1964

Congenital Nonhemolytic Icterus: Report of a Case

Author Affiliations

OWEN BLACK; COLUMBIA. MO
Harold P. Martin, MD, University Hospital, Department of Pediatrics, Columbia, Mo.; Resident (Dr. Martin); Senior Medical Student (Mr. Black); Instructor (Dr. VanLeeuwen).

Am J Dis Child. 1964;107(2):195-197. doi:10.1001/archpedi.1964.02080060197017
Abstract

Introduction  In 1952, Crigler and Najjar1 reported the first seven patients with familial nonhemolytic icterus, six of whom had neurological damage. At a later date they2 reported an eighth patient who had no demonstrable neurological damage. Rosenthal 3 reported a patient with late onset of neurological involvement. Whitington4 reported the first Negro infant with this syndrome but was unable to demonstrate familial incidence or consanguinity in his patient.The salient manifestations are well summarized by the designation—"congenital familial nonhemolytic jaundice with kernicterus"—which is the descriptive name originally given this syndrome by Crigler and Najjar.1 Elevated unconjugated serum bilirubin is the striking feature with absence of evidence for hemolysis, liver disease, or infection. Kernicterus usually occurs in the first year. The syndrome is a manifestation of a recessive gene. Histologic changes in the liver have been conspicuously absent. This report describes an infant with unconjugated bilirubinemia and

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