Rotor syndrome

Transport to intestine affected in Dubin-Johnson (black pigmentation in liver) Rotor syndrome... 

Defective excretion of conjugated bilirubin into the bile. This occurs in the Ehibin-Johnson and Rotor syndromes. The resultant hyperbilirubinemia is mainly of the conjugated type as is the hyperbilirubinemia of extiahepatic obstruction of the biliaiy ducts (as by tumor or gallstones). 

Congenital defects include the Dubin-Johnson syndrome and Rotor syndrome, (s. tab. 12.4) Both of these diseases present a genetically determined disorder in the secretion of bilirubin. 

The Rotor syndrome was first described by A. B. Rotor et at in 1948 (63), after G. Canali had already reported on this form of jaundice (albeit not histologically investigated) in 1945. 

Hepatic storage disease (J. Golan et at, 1985) is deemed to be a variant of Rotor syndrome. This dysfunction is witnessed during the first years of life. Bilirubin values are only moderately elevated (2—4 mg/dl). About half of the bilirubin is conjugated. The pathogenetic mechanisms are presumably the same as in Rotor syndrome. Treatment is likewise not required for this benign disorder. 

Zimniak, R Dubin-Johnson and Rotor syndromes molecular basis and pathogenesis. Semin. Liver Dis. 1993 13 248 -260... 

Rotor syndrome is another form of conjugated hyperbilirubinemia similar to Dubin-Johnson syndrome but without pigment in the liver. The gallbladder is seen on intravenous cholecystography. Total urinary coproporphyrins are elevated, with about two thirds being coproporphyrin I. The prognosis is excellent. 

Rotor syndrome, a distinct inheritable pathophysiologic entity. Am J Med 1976 60 173-9. 

In Gilbert s disease and in the Crigler-Najjar syndrome the disposal of BSP from plasma is usually normal (B20, MIO), but in the Rotor syndrome, there is BSP retention (R24). In the Dubin-Johnson syndrome, BSP retention is usually increased (B61, D9, Dll, S16). 

Gilbert s syndrome Type 1 Crigler-Najjar syndrome Type II Crigler-Najjar syndrome Dubin-Johnson syndrome Rotor s syndrome... 

Shimizu, Y., Naruto, H., Ida, S., Kohakura, M. Urinary coproporphyrin isomers in Rotor s syndrome. A study in eight families. Hepatology 1981 1 173-178... 

H., Shorey, J., Combes, B. Viral hepatitis complicating the Dubin-Johnson syndrome. Gastroenterolcw 1972 63 331-339 Wolkoff, A.W., Wolpert, E., Pascasio, F.N., Arias, I.M. Rotor s syndrome. A distinct inheritable pathophysiologic entity. Amer. J. Med. 1976 60 173-179... 

Inherited disorders of bilirubm metabolism include Gilbert s, Crigler-Najjar (Type I), Crigler-Najjar (Type II), Lucey-DriscoU, Dubin-Johnson, and Rotor s syndromes. 

Dubin-Johnson and Rotor s syndromes Drugs (estradiol)... 

In the Dubin-Johnson syndrome there is increased urinary excretion of coproporphyrin-I and a reduced excretion of coproporphyrin-III. In Rotor s syndrome, urinary excretion of coproporphyrin-I is increased with normal coproporphyrin-IIl excretion,and in Gilbert s disease there is increased urinary excretion of both isomers. 

Familial diseases include Dubin-Johnson syndrome, Rotor s syndrome, and benign familial recurrent cholestasis. Serum bilirubin values in Dubin-Johnson syndrome are the same as those found in Rotor s syndrome (Table 29-2). Very little is known about benign familial recurrent cholestasis. All three disorders are uncommon or rare, and all are benign. The liver in Dubin-Johnson... 

In Rotor s syndrome, patients lack the hepatic pigment but have urinary coproporphyrin levels 2.5-5 times greater than normal. Coproporphyrin type I is increased relative to the type III isomer but not as much as in Dubin-Johnson syndrome. Intrahepatic storage of sulfobromophthalein is... 

Conjugated hyperbilimbinemia is rare during the neonatal period. It can result from impaired hepatocellular function or extrahepatic obstmction. Hepatocellular defects can be caused by bacterial, viral, or parasitic infections, cystic fibrosis, a -antitrypsin deficiency, Dubin-Johnson and Rotor s syndromes, and other genetic disease. Extrahepatic obstruction can be congenital (biliary atresia) or acquired. 

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