Ｒｏｔｏｒ症候群の１家系　　とくに色素排せつ能に関する検討を中心に

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タイトル別名
  • A family with Rotor's syndrome: with a special emphasis on excretion capacity of the liver
  • Rotor ショウコウグン ノ 1 カケイ トクニ シキソ ハイセツノウ ニ

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抄録

Plasma clearance of unconjugated bilirubin, sulfobromophthalein sodium (BSP), indocyanine green (ICG) and ursodeoxycholic acid (UDCA) were studied in father and son cases with Rotor's syndrome. Plasma disappearance rates of unconjugated bilirubin, BSP and ICG were markedly reduced. The transfer rate analysis showed that the uptake of BSP by the liver was markedly reduced whereas the uptake of unconjugated bilirubin was unaffected. A decrease in the transfer rate from liver to bile and an increased rate of transfer from liver to plasma were observed with both substances. The plasma disappearance rates of orally administrated UDCA were not affected.<BR>The family study revealed that the father and his wife were cousinship each other and two of five their children were manifest Rotor's syndrome. BSP clearance studies in other two children without jaundice were performed and found some defects in the dye clearance in those family members. The form of hereditary transmission of this family with Rotor's syndrome was compatible with incomplete autosomal dominant.

収録刊行物

  • 肝臓

    肝臓 23 (5), 540-545, 1982

    一般社団法人　日本肝臓学会

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