Crigler-Najjar syndrome

Crigler-Najjar syndrome is a rare inherited disorder, passed on in an autosomal recessive manner, that affects the metabolism of bilirubin, the pigment formed when heme in red blood cells breaks down. It produces a form of nonhemolytic jaundice with high levels of unconjugated bilirubin, which often leads to brain damage in infants. The estimated annual incidence is 0.6 to 1 in 1,000,000. It is divided into types I and II, the latter sometimes called Arias syndrome. With Gilbert syndrome, Dubin-Johnson syndrome and Rotor syndrome it makes up the five known hereditary defects of bilirubin metabolism.