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一种伴有吲哚菁绿不耐受的体质性非结合胆红素血症:一种新的功能性疾病?

A constitutional unconjugated hyperbilirubinemia combined with indocyanine green intolerance: a new functional disorder?

作者信息

Ohkubo H, Okuda K, Iida S

出版信息

Hepatology. 1981 Jul-Aug;1(4):319-24. doi: 10.1002/hep.1840010407.

DOI:10.1002/hep.1840010407
PMID:7286909
Abstract

The kinetics of plasma clearance of indocyanine green and bromosulfophthalein were studied in 49 consecutive patients with chronic unconjugated hyperbilirubinemia. Forty-four patients had Gilbert's syndrome whereas five patients had impaired hepatic uptake of indocyanine green and virtually normal hepatic bromosulfophthalein uptake. There was no difference in bilirubin metabolism between the two groups. A family study of the patients with impaired indocyanine green uptake revealed that the defect appears to be transmitted as an autosomal dominant trait associated with inheritance of impaired bilirubin metabolism. These patients manifest a noval disorder of organic anion transport which, like Gilbert's syndrome, is also characterized by chronic milk unconjugated hyperbilirubinemia.

摘要

对49例连续性慢性非结合胆红素血症患者进行了吲哚菁绿和溴磺酞钠血浆清除动力学研究。44例患者患有吉尔伯特综合征,而5例患者吲哚菁绿肝脏摄取受损,肝脏溴磺酞钠摄取基本正常。两组之间胆红素代谢无差异。对吲哚菁绿摄取受损患者的家族研究显示,该缺陷似乎作为常染色体显性性状传递,与胆红素代谢受损的遗传相关。这些患者表现出一种新的有机阴离子转运障碍,与吉尔伯特综合征一样,其特征也是慢性轻度非结合胆红素血症。

相似文献

1
A constitutional unconjugated hyperbilirubinemia combined with indocyanine green intolerance: a new functional disorder?一种伴有吲哚菁绿不耐受的体质性非结合胆红素血症:一种新的功能性疾病?
Hepatology. 1981 Jul-Aug;1(4):319-24. doi: 10.1002/hep.1840010407.
2
Gilbert's syndrome and drug metabolism.吉尔伯特综合征与药物代谢。
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Two cases of constitutional unconjugated hyperbilirubinemia with marked retention of indocyanine green.两例伴有吲哚菁绿显著潴留的体质性非结合性高胆红素血症
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Abnormal hepatic transport of indocyanine green in Gilbert's syndrome.吉尔伯特综合征中吲哚菁绿的肝脏转运异常。
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Hepatic transport and metabolism of various organic anions in patients with congenital non-hemolytic hyperbilirubinemia, including constitutional indocyanine green excretory defect.先天性非溶血性高胆红素血症患者中各种有机阴离子的肝脏转运和代谢,包括体质性吲哚菁绿排泄缺陷。
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Effect of cimetidine on the metabolism of cholephilic dyes in Gilbert's syndrome.西咪替丁对吉尔伯特综合征中亲胆色素代谢的影响。
Eur J Clin Pharmacol. 1984;27(2):227-32. doi: 10.1007/BF00544050.
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[Recent advances in pathogenesis and diagnosis of constitutional hepatic dysfunction (Gilbert's syndrome) (author's transl)].体质性肝功能不全(吉尔伯特综合征)发病机制与诊断的最新进展(作者译)
Ther Umsch. 1978 Sep;35(9):742-6.
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Effect of clofibrate on the metabolism of bilirubin, bromosulphophthalein and indocyanine green and on the biliary lipid composition in Gilbert's syndrome.氯贝丁酯对吉尔伯特综合征患者胆红素、溴磺酞钠和吲哚菁绿代谢及胆汁脂质成分的影响。
Clin Sci (Lond). 1984 Apr;66(4):389-97. doi: 10.1042/cs0660389.

引用本文的文献

1
Effect of cimetidine on the metabolism of cholephilic dyes in Gilbert's syndrome.西咪替丁对吉尔伯特综合征中亲胆色素代谢的影响。
Eur J Clin Pharmacol. 1984;27(2):227-32. doi: 10.1007/BF00544050.
2
N-acetylation and debrisoquine hydroxylation polymorphisms in patients with Gilbert's syndrome.吉尔伯特综合征患者的N-乙酰化和异喹胍羟基化多态性
Br J Clin Pharmacol. 1991 Oct;32(4):467-72. doi: 10.1111/j.1365-2125.1991.tb03932.x.