Crigler–Najjar syndrome
Rare inherited disorder affecting the metabolism of bilirubin / From Wikipedia, the free encyclopedia
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Crigler–Najjar syndrome is a rare inherited disorder affecting the metabolism of bilirubin, a chemical formed from the breakdown of the heme in red blood cells. The disorder results in a form of nonhemolytic jaundice, which results in high levels of unconjugated bilirubin and often leads to brain damage in infants. The disorder is inherited in an autosomal recessive manner. The annual incidence is estimated at 1 in 1,000,000.[1]
This article needs more reliable medical references for verification or relies too heavily on primary sources. (March 2022) |
Crigler–Najjar syndrome | |
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Other names | CNS |
Bilirubin | |
Specialty | Pediatrics, hepatology |
This syndrome is divided into types I and II, with the latter sometimes called Arias syndrome. These two types, along with Gilbert's syndrome, Dubin–Johnson syndrome, and Rotor syndrome, make up the five known hereditary defects in bilirubin metabolism. Unlike Gilbert's syndrome, only a few cases of Crigler–Najjar syndrome are known.[citation needed]