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[一名44岁肝功能酶升高且有胆石症家族史的女性]

[44-year-old woman with elevated liver enzymes and a family history for cholelithiasis].

作者信息

Hopf C, Beuers U, Bikker H, Denk G U, Rust C

机构信息

Medizinische Klinik und Poliklinik II, LMU Klinikum der Universität München - Campus Großhadern, Marchioninistraße 15, 81377, München, Deutschland.

出版信息

Internist (Berl). 2011 Oct;52(10):1234-7. doi: 10.1007/s00108-010-2775-2.

DOI:10.1007/s00108-010-2775-2
PMID:21161147
Abstract

"Low phospholipid associated cholelithiasis" (LPAC) syndrome is an important differential diagnosis in younger patients with biliary symptoms after cholecystectomy and concomitant elevated serum liver tests. Typical symptoms include recurrence of biliary colics after cholecystectomy, echogenic material in the intrahepatic bile ducts, intrahepatic cholestasis of pregnancy or cholestasis under hormonal contraception and a family history of gallstone disease. Patients with LPAC syndrome can be successfully treated with ursodeoxycholic acid.

摘要

“低磷脂相关胆石症”(LPAC)综合征是胆囊切除术后出现胆道症状且血清肝功能检查结果同时升高的年轻患者的重要鉴别诊断。典型症状包括胆囊切除术后胆绞痛复发、肝内胆管出现回声物质、妊娠期肝内胆汁淤积或激素避孕下的胆汁淤积以及胆结石疾病家族史。LPAC综合征患者可用熊去氧胆酸成功治疗。

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本文引用的文献

1
Combined features of low phospholipid-associated cholelithiasis and progressive familial intrahepatic cholestasis 3.低磷脂结合性胆石症与进行性家族性肝内胆汁淤积症 3 型的联合特征。
Liver Int. 2010 Feb;30(2):327-31. doi: 10.1111/j.1478-3231.2009.02148.x. Epub 2009 Oct 19.
2
ABCB4 sequence variations in young adults with cholesterol gallstone disease.患有胆固醇结石病的年轻成年人中的ABCB4序列变异
Liver Int. 2009 May;29(5):743-7. doi: 10.1111/j.1478-3231.2008.01914.x. Epub 2008 Oct 24.
3
Low phospholipid associated cholelithiasis: association with mutation in the MDR3/ABCB4 gene.
低磷脂相关胆石症:与MDR3/ABCB4基因突变的关联。
Orphanet J Rare Dis. 2007 Jun 11;2:29. doi: 10.1186/1750-1172-2-29.
4
ABCB4 gene mutation-associated cholelithiasis in adults.成人ABCB4基因突变相关的胆石症
Gastroenterology. 2003 Aug;125(2):452-9. doi: 10.1016/s0016-5085(03)00898-9.
5
A multidrug resistance 3 gene mutation causing cholelithiasis, cholestasis of pregnancy, and adulthood biliary cirrhosis.一种导致胆结石、妊娠期胆汁淤积症和成人期胆汁性肝硬化的多药耐药3基因突变。
Gastroenterology. 2003 Apr;124(4):1037-42. doi: 10.1053/gast.2003.50144.
6
MDR3 gene defect in adults with symptomatic intrahepatic and gallbladder cholesterol cholelithiasis.有症状的成人肝内和胆囊胆固醇结石患者的MDR3基因缺陷
Gastroenterology. 2001 May;120(6):1459-67. doi: 10.1053/gast.2001.23947.
7
The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood.多药耐药3缺乏症的广泛谱系:从新生儿胆汁淤积到成人肝硬化
Gastroenterology. 2001 May;120(6):1448-58. doi: 10.1053/gast.2001.23984.