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Hepatol Commun. 2018 Mar 30;2(5):515-528. doi: 10.1002/hep4.1168. eCollection 2018 May.

Outcomes of surgical management of familial intrahepatic cholestasis 1 and bile salt export protein deficiencies.

Hepatology communications

Laura N Bull, Ludmila Pawlikowska, Sandra Strautnieks, Irena Jankowska, Piotr Czubkowski, Jennifer L Dodge, Karan Emerick, Catherine Wanty, Sami Wali, Samra Blanchard, Florence Lacaille, Jane A Byrne, Albertien M van Eerde, Kaija-Leena Kolho, Roderick Houwen, Steven Lobritto, Vera Hupertz, Patricia McClean, Giorgina Mieli-Vergani, Etienne Sokal, Philip Rosenthal, Peter F Whitington, Joanna Pawlowska, Richard J Thompson

Affiliations

  1. Liver Center Laboratory, Department of Medicine University of California San Francisco San Francisco CA.
  2. Institute for Human Genetics University of California San Francisco San Francisco CA.
  3. Department of Anesthesia and Perioperative Care University of California San Francisco San Francisco CA.
  4. Institute of Liver Studies King's College London London United Kingdom.
  5. Department of Gastroenterology, Hepatology, Eating Disorders, and Pediatrics Children's Memorial Health Institute Warsaw Poland.
  6. Department of Surgery University of California San Francisco San Francisco CA.
  7. Department of Pediatrics University of Connecticut Hartford CT.
  8. Université Catholique de Louvain Cliniques Saint Luc, Department of Pediatric Gastroenterology and Hepatology Brussels Belgium.
  9. Department of Pediatrics Riyadh Armed Forces Hospital Riyadh Saudi Arabia.
  10. Department of Pediatric Gastroenterology University of Maryland College Park MD.
  11. Department of Pediatrics Hôpital Necker-Enfants Malades Paris France.
  12. Department of Genetics University Medical Center Utrecht Utrecht the Netherlands.
  13. Children's Hospital University of Helsinki Helsinki Finland.
  14. Tampere University Tampere Finland.
  15. Department of Pediatric Gastroenterology University Medical Center Utrecht Utrecht the Netherlands.
  16. Center for Liver Disease and Transplantation Columbia University New York NY.
  17. Department of Pediatric Gastroenterology, Hepatology, and Nutrition Cleveland Clinic Foundation Cleveland OH.
  18. Children's Liver and Gastroenterology Unit Leeds Children's Hospital Leeds United Kingdom.
  19. Department of Pediatrics University of California San Francisco San Francisco CA.
  20. Department of Pediatrics, Northwestern University Feinberg School of Medicine Ann and Robert H. Lurie Children's Hospital of Chicago Chicago IL.

PMID: 29761168 PMCID: PMC5944593 DOI: 10.1002/hep4.1168

Abstract

Progressive familial intrahepatic cholestasis (PFIC) with normal circulating gamma-glutamyl transpeptidase levels can result from mutations in the

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