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Familial Nonhemolytic JaundiceCongenital, with Kernicterus
PAUL SUGAR, M.D.
Arch Intern Med. 1961;108(1):121-127.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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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
. . . [Full Text PDF of this Article]
Author Affiliations
NEGEV, ISRAEL
Workers' Sick-Fund of Israel Negev Branch.
Footnotes
Submitted for publication June 29, 1960.
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