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Literature DB >> 17452236

Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma.

A O Scheimann¹, S S Strautnieks, A S Knisely, J A Byrne, R J Thompson, M J Finegold.

Abstract

Fatal peripheral cholangiocarcinoma developed in 2 girls with progressive familial intrahepatic cholestasis, ABCB11 mutations, and absent bile salt export pump (BSEP) expression. BSEP deficiency may cause cholangiocarcinoma through bile-composition shifts or bile-acid damage within cells capable of hepatocytic/cholangiocytic differentiation. This observation suggests the need for hepatobiliary-malignancy surveillance and early consideration for liver transplantation.

Entities: Chemical Disease Gene Species

Mesh：

Substances：

Year: 2007 PMID： 17452236 DOI： 10.1016/j.jpeds.2007.02.030

Source DB: PubMed Journal: J Pediatr ISSN： 0022-3476 Impact factor: 4.406

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Cited

34 in total

1. Morphologic findings in progressive familial intrahepatic cholestasis 2 (PFIC2): correlation with genetic and immunohistochemical studies.

Authors: Kimberley Evason; Kevin E Bove; Milton J Finegold; A S Knisely; Sue Rhee; Philip Rosenthal; Alexander G Miethke; Saul J Karpen; Linda D Ferrell; Grace E Kim
Journal: Am J Surg Pathol Date: 2011-05 Impact factor: 6.394

Review 2. Liver transplantation and the management of progressive familial intrahepatic cholestasis in children.

Authors: Ashley Mehl; Humberto Bohorquez; Maria-Stella Serrano; Gretchen Galliano; Trevor W Reichman
Journal: World J Transplant Date: 2016-06-24

3. Bile acids via FXR initiate the expression of major transporters involved in the enterohepatic circulation of bile acids in newborn mice.

Authors: Julia Yue Cui; Lauren M Aleksunes; Yuji Tanaka; Zidong Donna Fu; Ying Guo; Grace Liejun Guo; Hong Lu; Xiao-Bo Zhong; Curtis D Klaassen
Journal: Am J Physiol Gastrointest Liver Physiol Date: 2012-01-19 Impact factor: 4.052

Review 4. Progressive familial intrahepatic cholestasis.

Authors: Anshu Srivastava
Journal: J Clin Exp Hepatol Date: 2013-11-23

Review 5. Genetic and environmental determinants of risk for cholangiocarcinoma in Thailand.

Authors: Masanao Miwa; Satoshi Honjo; Gyokukou You; Masakazu Tanaka; Kazuhiko Uchida; Petcharin Srivatanakul; Thiravud Khuhaprema; Watcharin Loilome; Anchalee Techasen; Chaisiri Wongkham; Temduang Limpaiboon; Puangrat Yongvanit; Sopit Wongkham
Journal: World J Gastrointest Pathophysiol Date: 2014-11-15

Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma.

1. Morphologic findings in progressive familial intrahepatic cholestasis 2 (PFIC2): correlation with genetic and immunohistochemical studies.

Review 2. Liver transplantation and the management of progressive familial intrahepatic cholestasis in children.

3. Bile acids via FXR initiate the expression of major transporters involved in the enterohepatic circulation of bile acids in newborn mice.

Review 4. Progressive familial intrahepatic cholestasis.

Review 5. Genetic and environmental determinants of risk for cholangiocarcinoma in Thailand.

6. Analysis of gene mutations in children with cholestasis of undefined etiology.

7. Hydrophilic bile acids prevent liver damage caused by lack of biliary phospholipid in Mdr2^-/- mice.

8. Bile Acid Receptors and Liver Cancer.

9. Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis.

10. Bile acids as endogenous etiologic agents in gastrointestinal cancer.