PEDIATRICS Vol. 43 No. 3 March 1969, pp. 365-376
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FAMILIAL NONHEMOLYTIC JAUNDICE: BILIRUBINOSIS AND ENCEPHALOPATHY

William A. Gardner Jr. M.D.1 and Bruce W. Konigsmark M.D.1

1 Department of Pathology and Division of Laryngology and Otology, The Johns Hopkins University School of Medicine, Baltimore

The unique pathological findings of a case of congenital familial hyperbilirubinemia are presented. The patient (Case 3 of Crigler and Najjar's original report), although severely jaundiced, had developed normally without evidence of neurological disease until 15frac12 years of age. He then developed a progressive neurological deterioration which was clinically similar to infantile kernicterus.

At autopsy most of his organs showed extensive intra- and extracellular deposition of bile pigment, particularly the renal papillae, atrial endocardium, intestinal mucosa, Kupffer cells of the liver, and the perivascular adventitia.

Although no pigment was found in the central nervous system, there was striking neuronal loss and gliosis of the thalamus. Moderate neuronal loss was found in the putamen, caudate nucleus, dentate nucleus, and red nuclei. No histopathological changes were found in the hippocampus or cerebral cortex.

It is suggested that the patient suffered from a late onset of "kernicterus" with involvement, in this older patient, of regions of the nervous system somewhat different from those in infantile kernicterus.

Submitted on April 4, 1968
Accepted on October 25, 1968




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M. I. Shevell, A. Majnemer, and D. Schiff
Neurologic Perspectives of Crigler-Najjar Syndrome Type I
J Child Neurol, June 1, 1998; 13(6): 265 - 269.
[Abstract] [PDF]