淤胆型肝损伤的临床与病理

刘杜先; 刘兰侠; 杨永峰

doi:10.3969/j.issn.1001-5256.2018.11.004

淤胆型肝损伤的临床与病理

DOI: 10.3969/j.issn.1001-5256.2018.11.004

1. 南京中医药大学附属南京医院南京市第二医院病理科
2. 南京中医药大学附属南京医院南京市第二医院肝病科

详细信息

中图分类号: R575
计量
- 文章访问数: 1913
- HTML全文浏览量: 41
- PDF下载量: 479
- 被引次数: 0
出版历程
- 收稿日期: 2018-09-11
- 出版日期: 2018-11-20

Clinical and pathological features of cholestatic liver injury

Department of Pathology, Nanjing Hospital Affiliated to Nanjing University of Chinese Medicine& The Second Hospital of Nanjing

摘要

摘要: 胆汁淤积可因胆汁代谢异常、胆汁流动及回流不畅、胆管损伤及梗阻等原因引起。胆汁代谢异常往往由遗传基因异常引起,而胆汁流通不畅往往由胆管炎所致的损伤或胆道阻塞引发。胆汁代谢异常以进行性家族性胆汁淤积(PFIC)最为常见,而胆管损伤性疾病以自身免疫性胆管炎为主要原因。先天性/自身免疫性胆管损伤以及继发性/获得性胆管反应的病理组织学改变,均具有相对特异性,疾病的最终确诊常需要结合临床、病理学、影像学、免疫学、遗传学、基因检测等综合分析。重点阐述了自身免疫性胆管损伤和PFIC病理形态学方面的研究进展,引申介绍了其他类似病变,以期对胆管损伤性、胆汁淤积性肝病的临床诊断提供参考。
- 肝疾病 /
- 胆汁淤积 /
- 疾病特征 /
- 病理学
Abstract: Major causes of cholestasis include abnormal bile metabolism, obstructed bile flow and regurgitation, and bile duct injury and obstruction. Abnormal bile metabolism is mainly caused by gene abnormality, while obstructed bile flow is often caused by bile duct injury or occlusion due to cholangitis. Abnormal bile metabolism is the most common cause of progressive familial intrahepatic cholestasis ( PFIC) , while autoimmune cholangitis is the major cause of bile duct injury. Both congenital/autoimmune bile duct injury and secondary/acquired ductular reaction had relatively specific histopathological changes, and a confirmed diagnosis needs a comprehensive analysis based on clinical, pathological, imaging, immunological, and genetic examinations. This article elaborates on the research advances in the pathomorphology of autoimmune bile duct injury and PFIC and introduces the similar lesions, in order to provide a reference for the clinical diagnosis of bile duct injury and cholestatic liver diseases.
- liver diseases /
- cholestasis /
- disease attributes /
- pathology

HTML全文

参考文献(30)

[1]PORTMANN B, ZEN Y.Inflammatory disease of the bile ductscholangiopathies:Liver biopsy challenge and clinicopathological correlation[J].Histopathology, 2012, 60 (2) :236-248.

[2]KAPLAN M, GERSHWIN M.Primary biliary cirrhosis[J].N Engl J Med, 2005, 353 (12) :1261-1273.

[3]LINDOR KD, GERSHWIN ME, POUPON R, et al.Primary biliary cirrhosis[J].Hepatology, 2009, 50 (1) :291-308.

[4]SCHEUER P.Primary biliary cirrhosis[J].Proc R Soc Med, 1967, 60 (12) :1257-1260.

[5]BROOMU, OLSSON R, LF L, et al.Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis[J].Gut, 1996, 38 (4) :610-615.

[6]LINDOR KD, KOWDLEY KV, HARRISON ME.ACG Clinical Guideline:Primary sclerosing cholangitis[J].Am J Gastroenterol, 2015, 110 (5) :646-659.

[7]ZEN Y, KAWAKAMI H, KIM JH.Ig G4-related sclerosing cholangitis:All we need to know[J].J Gastroenterol, 2016, 51 (4) :295-312.

[8]ZEN Y.The pathology of Ig G4-related disease in the bile duct and pancreas[J].Semin Liver Dis, 2016, 36 (3) :242-256.

[9]GHAZALE A, CHARI ST, ZHANG L, et al.Immunoglobulin G4-associated cholangitis:Clinical profile and response to therapy[J].Gastroenterology, 2008, 134 (3) :706-715.

[10]DESHPANDE V, ZEN Y, CHAN JK, et al.Consensus statement on the pathology of Ig G4-related disease[J].Mod Pathol, 2012, 25 (9) :1181-1192.

[11]KLOMP LW, VARGAS JC, van MIL SW, et al.Characterization of mutations in ATP8B1 associated with hereditary cholestasis[J].Hepatology, 2004, 40 (1) :27-38.

[12]van MIL SWC, KLOMP LW, BULL LN, et al.FIC1 disease baspectrum of intrahepatic cholestic diseases[J].Semin Liver Dis, 2003, 21 (4) :535-543.

[13]EPPENS EF, van MIL SWC, de VREE JML, et al.FIC1 the protein a infected in two form of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte[J].J Hepatol, 2001, 35 (2) :436-443.

[14]CLAYTON RJ, IBER FL, RUEBNER BH, et al.Byler disease.Fatal familial intrahepatic cholestasis in an Amish kindred[J].Am J Dis Child, 1969, 117 (1) :112-124.

[15]LINARELLI LG, WILLIAMS CN, PHILLIPS MJ.Byler's disease:Fatal intrahepatic cholestasis[J].J Pediatr, 1972, 81 (3) :484-492.

[16]KEITEL V, BURDELSKI M, VOJNISEK Z, et al.De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation:A novel mechanism of cholestasis[J].Hepatology, 2009, 50 (2) :510-517.

[17]MIYAGAWA-HAYASHINO A, EGAWA H, YORIFUJI T, et al.Allograft steatohepatitis in progressive familial intrahepatic cholestasis type 1 after living donor liver transplantation[J].Liver Transpl, 2009, 15 (6) :610-618.

[18]DEMEILLIERS C, JACQUEMIN E, BARBU V, et al.Altered hepatobiliary gene expressions in PFIC1:ATP8B1 gene defect is associated with CFTR downregulation[J].Hepatology, 2006, 43 (5) :1125-1134.

[19]ALONSO EM, SNOVER DC, MONTAG A, et al.Histologic pathology of the liver in progressive familial intrahepatic cholestasis[J].J Pediatr Gastroenterol Nutr, 1994, 18 (2) :128-133.

[20]STRAUTNIEKS SS, BYRNE JA, PAWLIKOWSKA L, et al.Severe bile salt export pump deficiency:82 different ABCB11 mutations in 109 families[J].Gastroenterology, 2008, 134 (4) :1203-1214.

[21]KNISELY AS, STRAUTNIEKS SS, MEIER Y, et al.Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency[J].Hepatology, 2006, 44 (2) :478-486.

[22]SCHEIMANN AO, STRAUTNIEKS SS, KNISELY AS, et al.Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma[J].J Pediatr, 2007, 150 (5) :556-559.

[23]BASS LM, PATIL D, RAO MS, et al.Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis[J].BMC Gastroenterol, 2010, 10:30.

[24]ARNELL H, BERGDAHL S, PAPADOGIANNAKIS N, et al.Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis[J].J Pediatr Surg, 2008, 43 (7) :1312-1320.

[25]JARA P, HIERRO L, MARTINEZ-FERNANDEZ P, et al.Recurrence of bile salt export pump deficiency after liver transplantation[J].N Engl J Med, 2009, 361 (14) :1359-1367.

[26]SIEBOLD L, DICK AA, THOMPSON R, et al.Recurrent low gamma-glutamyl transpeptidase cholestasis following liver transplantation for bile salt export pump (BSEP) disease (posttransplant recurrent BSEP disease) [J].Liver Transpl, 2010, 16 (7) :856-863.

[27]JACQUEMIN E, de VREE JM, CRESTEIL D, et al.The wide spectrum of multidrug resistance 3 dficiency:From neonatal cholestasis to cirrhosis of adulthood[J].Gastroenterology, 2001, 120 (6) :1448-1458.

[28]JACQUEMIN E.Role of multidrug resistance 3 deficiency in pediatric and adult liver disease:One gene for three diseases[J].Semin Liver Dis, 2001, 21 (4) :551-562.

[29]NAKANISHI Y, SAXENA R.Pathophysiology and diseases of the proximal pathways of the biliary system[J].Arch Pathol Lab Med, 2015, 139 (7) :858-866.

[30]QUAGLIA A, DUARTE R, PATCH D, et al.Histopathology of graft versus host disease of the liver[J].Histopathology, 2007, 50 (6) :727-738.

施引文献

资源附件(0)

访问统计