Rotor Syndrome

A benign form of mixed jaundice with conjugated (50%) and unconjugated (50%) bilirubin. Normal hepatic function and histology probably related to a defective binding and storage protein for bilirubin in liver cells.

Hyperbilirubinemia Rotor type.

Autosomal recessive.

Benign, familial, unconjugated, nonhemolytic hyperbilirubinemia. Jaundice caused by impaired excretion or storage of conjugated bilirubin.

Routine blood tests reveal conjugated hyperbilirubinemia. Albumin, transaminases, alkaline phosphatase, and prothrombin time are normal. Gross and microscopic examination of the liver are normal. Bromosulphthalein transport is reduced. Elevated urinary coproporphyrin is present.

Benign hyperbilirubinemia of no clinical importance. No identified precipitating factors. No specific treatment.

Avoid halothane and drugs that can displace bilirubin from albumin (e.g., cephalosporin).

The hereditary hyperbilirubinemias include firstly those predominantly unconjugated hyperbilirubinemia, i.e., Gilbert or Arias Syndrome, Crigler-Najjar Syndrome type I, and Crigler-Najjar Syndrome type II, and secondly those resulting in predominantly conjugated hyperbilirubinemia: Dubin-Johnson Syndrome, Rotor Syndrome, and several forms of Intrahepatic Cholestasis.

Gilbert Syndrome: Characterized by normal liver function tests of the usual type, normal liver histology, delayed clearance of bilirubin from the blood, and mild jaundice that tends to fluctuate in severity, particularly after fasting. This disorder is difficult to distinguish from prolonged posthepatic hyperbilirubinemia. Gilbert Syndrome is distinguished by the lack of morbidity in patients and by a lower total serum bilirubin level, ranging from 1 to 6 mg/dL.

Crigler-Najjar Syndrome presents in two forms: Type I and Type II. Type I is characterized by a deficiency in hepatic glucuronyltransferase activity. Hyperbilirubinemia is severe, with total serum bilirubin levels ranging from 20 to 45 mg/dL most often associated with kernicterus. Type I patients do not respond to phenobarbital treatment and only traces of bilirubin glucuronides can be found in their bile. It is inherited by autosomal recessive transmission. In type II, total serum bilirubin ranges from 6 to 20 mg/dL and phenobarbital treatment lowers serum bilirubin levels by more than 30%. Bilirubin glucuronides are present in bile. Type II is less severe than type I.

Dubin-Johnson Syndrome: Characterized by hyperbilirubinemia, deposition of melanin-like pigment leading to hepatomegaly and abdominal pain, prolonged retention of sulfobromophthalein (which may show a higher concentration at 60 to 90 minutes than at 45 minutes), and otherwise normal liver function. The incidence is reported for the Iranian Jews to 1 per 1,300. Age at onset for the jaundice varied from 10 weeks to 56 years. Penetrance is reduced in females. Sixty-four of all cases reported were Iranian Jews. The inheritance is consistent with autosomal recessive with reduced penetrance. Urinary coproporphyrin I is a good indicator of the homozygote and heterozygote states in the Dubin-Johnson Syndrome. Normal individuals excreted 24.8% of urinary coproporphyrin as coproporphyrin I, whereas homozygotes and heterozygotes excreted 88.9 and 31.6%, respectively. Factor VII deficiency has been suggested in Japanese cases that were diagnosed in the neonatal ...

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