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[良性慢性结合胆红素血症:罗特综合征还是肝储存功能受损?]

[Benign chronic conjugated hyperbilirubinaemia: rotor syndrome or hepatic storage impairment?].

作者信息

Knoflach P, Horak W, Kerstan E, Thaler H

出版信息

Z Gastroenterol. 1979 Jan;17(1):32-7.

PMID:419795
Abstract

In a 52-year-old female who was jaundiced since early childhood, marked conjugated hyperbilirubinaemia and a delay of bromsulfophthalein and indocyanine green plasma disappearance rates were observed. Liver histology, plasma bile acids and plasma clearance of 14-C-cholic acid were normal. There was a pronounced impairment of the relative storage capacity of bromsulfophthalein (S = 2,4 mg/mg%) and a moderate decrease of BSP-transport maximum.

摘要

在一名自幼儿期起就出现黄疸的52岁女性患者中,观察到明显的结合胆红素血症以及溴磺酞钠和吲哚菁绿血浆消失率延迟。肝脏组织学、血浆胆汁酸和14-C-胆酸的血浆清除率均正常。溴磺酞钠的相对储存能力明显受损(S = 2.4 mg/mg%),且溴磺酞钠转运最大值有中度降低。

相似文献

1
[Benign chronic conjugated hyperbilirubinaemia: rotor syndrome or hepatic storage impairment?].[良性慢性结合胆红素血症:罗特综合征还是肝储存功能受损?]
Z Gastroenterol. 1979 Jan;17(1):32-7.
2
Dye clearance studies in Rotor's syndrome.罗特综合征的染料清除研究。
Am J Gastroenterol. 1979 Apr;71(4):380-8.
3
[Diagnostic significance of serum bile acids].[血清胆汁酸的诊断意义]
Schweiz Med Wochenschr. 1975 Apr 26;105(17):533-5.
4
Chronic hyperbilirubinemia associated with hepatic uptake and storage impairment. A new syndrome resembling that of the mutant Southdown sheep.
Gastroenterology. 1975 Oct;69(4):988-93.
5
Inheritable disorders manifested by conjugated hyperbilirubinemia.
Semin Liver Dis. 1983 Feb;3(1):65-72. doi: 10.1055/s-2008-1040672.
6
Presently recognized forms of inherited jaundice in infancy. A summary and review.
Clin Pediatr (Phila). 1973 Jun;12(6):326-32.
7
[A case with Rotor syndrome in hyperbilirubinemic family].
Korean J Gastroenterol. 2007 Apr;49(4):251-5.
8
Nine cases with marked retention of indocyanine green test and normal sulfobromophthalein test without abnormal liver histology: constitutional indocyanine green excretory defect.9例吲哚菁绿试验明显潴留且磺溴酞钠试验正常,肝脏组织学无异常:体质性吲哚菁绿排泄缺陷。
Hepatogastroenterology. 1981 Feb;28(1):6-12.
9
Bromsulfophthalein clearance and aminopyrine test in patients with Gilbert's syndrome.吉尔伯特综合征患者的磺溴酞钠清除率及氨基比林试验
Isr J Med Sci. 1986 May;22(5):376-9.
10
[Hyperbilirubinemia].[高胆红素血症]
Schweiz Rundsch Med Prax. 1992 Feb 18;81(8):230-3.

引用本文的文献

1
The hepatic excretion of 131I-rose bengal and 99mTc-IDA derivatives in Rotor's syndrome.131I-玫瑰红和99mTc-IDA衍生物在罗托综合征中的肝脏排泄情况。
Eur J Nucl Med. 1982;7(7):311-7. doi: 10.1007/BF00253427.
2
Partial defect in hepatic glutathione S-transferase activity in a case of Rotor's syndrome.罗特综合征一例中肝脏谷胱甘肽S-转移酶活性部分缺陷
Gastroenterol Jpn. 1987 Feb;22(1):34-8. doi: 10.1007/BF02806332.