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A family with Rotor's syndrome: with a special emphasis on excretion capacity of the liver
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- MIYATA Yasuji
- The Third Department of Internal Medicine, Faculty of Medicine, Kyushu University
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- KOGA Shunichi
- The Third Department of Internal Medicine, Faculty of Medicine, Kyushu University
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- YAMAMOTO Kyosuke
- The Third Department of Internal Medicine, Faculty of Medicine, Kyushu University
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- IBAYASHI Hiroshi
- The Third Department of Internal Medicine, Faculty of Medicine, Kyushu University
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- MAKINO Isao
- The Third Department of Internal Medicine, Faculty of Medicine, Hirosaki University
Bibliographic Information
- Other Title
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- 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
- 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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Description
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.
Journal
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- Kanzo
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Kanzo 23 (5), 540-545, 1982
The Japan Society of Hepatology
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Keywords
Details 詳細情報について
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- CRID
- 1390282679768386688
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- NII Article ID
- 130000878418
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- NII Book ID
- AN00047770
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- ISSN
- 18813593
- 04514203
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- NDL BIB ID
- 2489886
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL Search
- Crossref
- CiNii Articles
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- Abstract License Flag
- Disallowed