Inborn Errors Of Metabolism That Cause Hyperbilirubinemia

Hereditary or congenital metabolic disorders cause unconjugated or conjugated hyperbilirubinemia.

See also liver structure and function and assessment of patients with liver disease.) Hereditary or congenital metabolic disorders cause unconjugated or conjugated hyperbilirubinemia. Unconjugated hyperbilirubinemia: Gilbert syndrome, Crigler-Najjar syndrome and primary shunt hyperbilirubinemia Conjugated Hyperbilirubinemia: Dubin-Johnson syndrome and Rotor syndrome Primary shunt hyperbilirubinemia This rare familial, benign suffering is by an overproduction of “early-labeled in “bilirubin (bilirubin, which arises from ineffective erythropoiesis, and non-heme hemoglobin instead of the normal erythrocyte maturation). Dubin-Johnson syndrome and Rotor syndrome Dubin-Johnson syndrome and Rotor syndrome cause conjugated hyperbilirubinemia without cholestasis, which apart from a jaundice has no other symptoms. In contrast to unconjugated hyperbilirubinemia in Gilbert syndrome (which also does not cause any other symptoms) can appear in urine bilirubin. The transaminases and alkaline phosphatase are usually normal. Treatment is not necessary. Dubin-Johnson syndrome This rare autosomal recessive syndrome characterized by a reduced excretion of Bilirubinglucuronid. It is usually diagnosed by a liver biopsy: the liver cells contain dark pigment as a result of deposition of intracellular melanin-like substance in otherwise unremarkable histological Befund.Rotor syndrome This also rare syndrome resembles clinically the Dubin-Johnson syndrome, but the liver is not pigmented and there are other metabolic differences.

Health Life Media Team

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