Living Donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Two Reported Cases

Oya, Yuki; Sugawara, Yasuhiko; Honda, Masaki; Yoshii, Daiki; Isono, Kaori; Hayashida, Shintaro; Yamamoto, Haruyasu; Inomata, Yukihiro

doi:10.1016/j.transproceed.2017.03.035

Cited by 10 publications

(12 citation statements)

References 11 publications

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“…Alternatively, total biliary diversion is an option for progressive graft inflammation and refractory diarrhea post-LT. Despite reports of satisfactory outcome of external biliary diversion during or post-LT [ 21 , 31 ] added burden of the necessary stoma was met by parental rejection in all of our cases. TIBD is another possible technique that has been described in two cases after LT allowing for biliary diversion without the need for an external stoma [ 25 , 26 ].…”

Section: Discussionmentioning

confidence: 72%

“…Partial diversion is done by rerouting bile from an existing gale bladder or bile duct either to the body surface (partial external biliary diversion; PEBD) or to the colon (partial internal biliary diversion; PIBD) while preserving bile flow in the common bile duct [ 30 ]. This can be achieved with drain placements or via enteric conduits [ 21 , 31 ]. Total diversion involves complete rerouting of produced bile either externally or internally (TIBD).…”

Section: Discussionmentioning

confidence: 99%

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Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

et al. 2022

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Background: Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the enterohepatic circulation, e.g., surgical biliary diversion (SBD) can slow down development of liver cirrhosis. Eventually, end stage liver disease necessitates liver transplantation (LT). PFIC1 patients might develop diarrhea, graft steatosis and inflammation after LT. SBD after LT was shown to be effective in the alleviation of liver steatosis and graft injury. Case report: Three PFIC1 patients received LT at the ages of two, two and a half and five years. Shortly after LT diarrhea and graft steatosis was recognized, SBD to the terminal ileum was opted to prevent risk for ascending cholangitis. After SBD, inflammation and steatosis was found to be reduced to resolved, as seen by liver biochemistry and ultrasounds. Diarrhea was reported unchanged. Conclusion: We present three PFIC1 cases for whom SBD to the terminal ileum successfully helped to resolve graft inflammation and steatosis.

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

et al. 2022

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“…Bile absorptive resin is shown to be capable of reducing diarrhea and steatosis after LT, but the effect is considered genotype dependent [ 7 ]. LT combined with biliary diversion may be of help to revert steatohepatitis after LT, with liver–intestine co-transplantation is also proposed [ 19 , 21 , 22 , 23 , 24 ]. In our study, one PFIC type I patient who received LDLT reported diarrhea 6 months after LT and began to intake cholestyramine, which resulted in normal fecal output.…”

Section: Discussionmentioning

confidence: 99%

Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis

Hang

Jin

Luo

et al. 2022

JCM

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We analyzed the long-term survival rate and development of progressive familial intrahepatic cholestasis (PFIC) patients after liver transplantation (LT). From October 2007 to May 2019, 41 patients were diagnosed as PFIC (type I-III) and received LT in Ren Ji Hospital due to end-stage liver diseases. The median age at LT was 2.93 years, with 75.6% of patients receiving living donor liver transplantation (LDLT). The 5- and 10-year patient survival rates after LT were 92.7% and 92.7%, respectively, and no difference was found among the three subtypes of PFIC. Two PFIC type II patients received re-transplantation due to vascular complications. Liver function and bile acid metabolism returned to normal levels in all living recipients. Catch-up growth was recorded as the height and weight Z scores increased from −2.53 and −1.54 to −0.55 and −0.27 with a median follow-up time of 5.55 years. Improved psychomotor ability and age-appropriate study ability was also observed. A total of 72.4% of school-aged recipients exhibited average academic performance. Diarrhea was reported in all PFIC type I recipients but resolved after resin absorptive treatment. However, allograft steatosis occurred in one PFIC type I patient and exhibited a “remission–relapse circle” under the treatment of cholestyramine. In conclusion, LT is an effective treatment for end-stage PFIC patients with encouraging long-term survival rate and development. However, allograft steatosis should be closely monitored in PFIC type I patients even if diarrhea has been well treated.

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“…The cause of death in the latter group was AMR in 2 patients and the other was multiple organ failure related to complications of the primary disease (progressive familial intrahepatic cholestasis type 1 [PFIC1]) including bleeding from the ileostomy, severe malnutrition, and chronic renal failure. 18 …”

Section: Resultsmentioning

confidence: 99%

Long-term Outcomes of ABO-incompatible Pediatric Living Donor Liver Transplantation

et al. 2018

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BackgroundABO-incompatible (ABOi) living donor liver transplantation (LDLT) has been performed to compensate for donor shortage. To date, few studies have reported detailed B-cell desensitization protocols and long-term outcomes of ABOi pediatric LDLT.MethodsTwenty-nine pediatric ABOi LDLT recipients were retrospectively analyzed. We compared the clinical outcomes between ABOi (n = 29) and non-ABOi (n = 131) pediatric LDLT recipients. Furthermore, we evaluated the safety and efficacy of our rituximab-based regimen for ABOi pediatric LDLT (2 ≤ age < 18; n = 10).ResultsThere were no significant differences in the incidence of infection, vascular complications, biliary complications, and acute cellular rejection between ABOi and non-ABOi groups. The cumulative graft survival rate at 1, 3, and 5 years for non-ABOi group were 92.1%, 87.0%, and 86.1%, and those for ABOi group were 82.8%, 82.8%, and 78.2%, respectively. Rituximab-based desensitization protocol could be performed safely, and reduced CD19+ lymphocyte counts effectively. Although rituximab-treated ABOi group showed comparable clinical outcomes and graft survival rate, 2 patients developed antibody-mediated rejection.ConclusionsABOi LDLT is a feasible option for pediatric end-stage liver disease patients. However, it should be noted that current desensitization protocol does not completely prevent the onset of antibody-mediated rejection in several cases.

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Living Donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Two Reported Cases

Cited by 10 publications

References 11 publications

Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

Internal Ileal Diversion as Treatment for Progressive Familial Intrahepatic Cholestasis Type 1-Associated Graft Inflammation and Steatosis after Liver Transplantation

Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis

Long-term Outcomes of ABO-incompatible Pediatric Living Donor Liver Transplantation

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