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Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation

Philipp Ellinger1, Jan Stindt1, Carola Dröge1

  • 1Philipp Ellinger, Jan Stindt, Sander H J Smits, Lutz Schmitt, Institute of Biochemistry, Heinrich-Heine-University Düsseldorf, 40225 Düsseldorf, Germany.

World Journal of Gastroenterology
|August 26, 2017
PubMed
Summary

Residual bile salt export pump (BSEP) function and substrate specificity impact the effectiveness of partial external biliary diversion (PEBD) for progressive familial intrahepatic cholestasis type 2 (PFIC-2). This highlights the importance of BSEP mutations in treatment outcomes.

Keywords:
ATP binding cassette transporterBile salt export pumpFamilial intrahepatic cholestasis type 2Intrahepatic cholestasisPartial external biliary diversion

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