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July 1961

Familial Nonhemolytic Jaundice: Congenital, with Kernicterus

Author Affiliations


Workers' Sick-Fund of Israel Negev Branch.

Arch Intern Med. 1961;108(1):121-127. doi:10.1001/archinte.1961.03620070123016

During the last decade a few well-defined clinical entities have been separated from the group of nonhemolytic intermittent hyperbilirubinemias which are characterized by a positive indirect van den Bergh reaction and continue for several years or even for life.

The most frequently encountered is Gilbert's disease—icterus intermittens juvenilis1,2 or congenital hepatic dysfunction.3-5 This illness consists of a mild intermittent icterus with few clinical complaints and has as a rule an excellent prognosis. It is considered due to a metabolic error in the liver cells, that is, lack of the enzyme glucuronyl-transferase, leading to failure of the liver cells to excrete bilirubin as bilirubin diglucuronide.

Following viral hepatitis one frequently finds a residual isolated disturbance of pigment metabolism, which may last for prolonged periods of time and has a good prognosis. If the hepatitis is mild or subclinical, the residual pigment disturbance, as pointed out by Hult,6 is

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