Familial nonhemolytic jaundice with late onset of neurological damage

A patient with familial nonhemolytic jaundice is described. The child had been normal, apart from his jaundice, for all of his life. But, at 16 years of age he developed neurological disabilities which progressed to his death 6 months later. The signs were similar to those of kernicterus seen in inf...

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Bibliographic Details
Published in:Pediatrics (Evanston) 1968-11, Vol.42 (5), p.786-792
Main Authors: Blumenschein, S D, Kallen, R J, Storey, B, Natzschka, J C, Odell, G B, Childs, B
Format: Article
Language:English
Subjects:
Adolescent
Bilirubin - blood
Central Nervous System Diseases - etiology
Cholestyramine Resin - therapeutic use
Consanguinity
Electroencephalography
Humans
Hyperbilirubinemia, Hereditary - complications
Hyperbilirubinemia, Hereditary - drug therapy
Intelligence Tests
Male
Peritoneal Dialysis
Tromethamine - therapeutic use
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Description
Summary:A patient with familial nonhemolytic jaundice is described. The child had been normal, apart from his jaundice, for all of his life. But, at 16 years of age he developed neurological disabilities which progressed to his death 6 months later. The signs were similar to those of kernicterus seen in infants. Attempts to reduce the bilirubin level of the blood and so to reverse the nervous system damage were unsuccessful.
ISSN:0031-4005
1098-4275
DOI:10.1542/peds.42.5.786