Crigler-Najjar syndrome

Crigler-Najjar syndrome (CNS) is a hereditary disorder of bilirubin metabolism characterized by unconjugated hyperbilirubinemia due to a hepatic deficit of bilirubin glucuronosyltransferase (GT) activity. Two types have been described, CNS types 1 and 2 (see these terms). CNS1 is characterized by a complete deficit of the enzyme and is unaffected by phenobarbital induction therapy, whereas the enzymatic deficit is partial and responds to phenobarbital in CNS2.

Hemolytic anemia

A type of anemia caused by premature destruction of red blood cells (hemolysis).


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(per page)
PMID (PMCID)
1962749
MALE Adult
[Case report of a patient with pseudohypoparathyroidism associated with slight increase in serum level of unconjugated bilirubin].
Kamitani A, Morimoto S, Takamoto S, Nabata T, Miyashita Y, Kitano S, Ogihara T.
Nihon Naibunpi Gakkai Zasshi. 1990;66(11):1148-57.
The cause of the increases in serum bilirubin levels was not clear; however, hemolytic anemia, hereditary unconjugated hyperbilirubinemia or ineffective erythropoiesis were ruled out as causes for the increase, since 1) his serum level of haptoglobin was normal, 2) increase in serum level of indirect bilirubin 120 minutes after the infusion of 50mg nicotinic acid was within the normal range, and 3) severe anemia was not observed.