Rotor病11家系15例の検討--特にその病態についての考察

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The study on eleven families of fifteen patients with fulfilling criteria for Rotor's hyperbilirubinemia, in whom thirteen patients on ten families were observed in the suburbs of Naha City, revealed familial occurrence in at least thirteen cases. The detailed pedigrees were investigated on two families and it was suggested that Rotor's hyperbilirubinemia is inherited as an autosomal incomplete dominant trait.<BR>Serum bilirubin levels and plasma disappearance rate (K) and plasma retention rate (R) of indocyanine green (ICG) and sulfobromophthalein (BSP) were determined. The results were as follows; Serum bilirubin levels: total 5.6±2.6mg/dl (mean±SD), direct 3.5±1.3mg/dl. The direct bilirubin was dominant in thirteen cases, whereas the indirect bilirubin was dominant in two cases. KICG: 0.022±0.009 (n=14), R15ICG (retention rate at 15 minutes after intravenous injection of 0.5mg/kg BW of ICG): 63.9±13.6% (n=13), K.BSP: 0.028±0.006 (n=4), R30BSP (retention rate at 30 minutes after intravenous injection of 5.0mg/kg BW of BSP): 62.0±23.9% (n=5), R45BSP: 40.8±6.1% (n=7). The plasma disappearance rates were markedly low and the plasma retention rates were high in both ICG and BSP .<BR>Oral cholecystography was well visualized in 14 cases in whom it was performed. Biopsies of the liver were performed on 7 cases and the histologic appearance was almost normal.<BR>We advocate that Rotor's hyperbilirubinemia should be called Rotor's disease even in the case without familial occurrence, because this is characteristic in differential diagnosis of the constitutional hyperbilirubinemias.

Rotor病11家系15例の検討--特にその病態についての考察

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