Liver retransplantation with external biliary diversion for progressive familial intrahepatic cholestasis type 1: A case report

Masanobu Usui, Shuji Isaji, Bidhan C. Das, Motoyuki Kobayashi, Ichiro Osawa, Taku Iida, Hiroyuki Sakurai, Masami Tabata, Toru Yorifuji, Hiroto Egawa, Shinji Uemoto

Research output: Contribution to journalArticlepeer-review

15 Citations (Scopus)

Abstract

PFIC1, originally described as "Byler disease," is characterized by cholestatic feature and chronic diarrhea. Many patients require LT for the cure, but intractable diarrhea and prolonged growth retardation after LT are serious complications limiting the ultimate outcome of LT for this disease. EBD has recently been shown to be a promising and effective treatment. Recently, we successfully treated a five-yr-old boy with PFIC1 employing EBD after re-transplantation. The patient received LDLT at the age of one yr. Six months after initial transplantation, he developed repeated attacks and diarrhea followed by the development of liver dysfunction and ascites. Liver biopsy at three yr after LDLT revealed the features of chronic graft rejection. With a diagnosis of chronic graft rejection with liver failure, we performed a repeat LDLT with EBD in which the jejunal loop used for hepaticojejunostomy was taken out of the body surface through the abdominal wall. Ten months after surgery, he is doing well, having no attack of diarrhea.

Original languageEnglish
Pages (from-to)611-614
Number of pages4
JournalPediatric Transplantation
Volume13
Issue number5
DOIs
Publication statusPublished - 08-2009
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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