中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Issue 11
Nov.  2018
Turn off MathJax
Article Contents

Clinical and pathological features of cholestatic liver injury

DOI: 10.3969/j.issn.1001-5256.2018.11.004
  • Received Date: 2018-09-11
  • Published Date: 2018-11-20
  • 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.

     

  • loading
  • [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.
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Article Metrics

    Article views (1923) PDF downloads(480) Cited by()
    Proportional views
    Related

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return