Review
Copyright ©The Author(s) 2016.
World J Transplant. Jun 24, 2016; 6(2): 278-290
Published online Jun 24, 2016. doi: 10.5500/wjt.v6.i2.278
Table 2 Review of documented liver transplantation outcomes for progressive familial intrahepatic cholestasis patients (≥ 3 patients)
Ref.PFIC typeAge at transplant (years old)Previous managementGraft survivalPatient survivalNotes
Soubrane et al[45]14 “byler disease” PFIC type unspecified6.5 (0.4-13)NR93.3%92.8%Consanguineous to the 2nd degree in 8 cases
Emond et al[91]11 PFIC unspecified type4.6 ± 3.42 had previous partial biliary diversion procedures76.9%73%LT performed on those with advanced cirrhosis (6 received diversion procedures only)
Ismail et al[80]8 PFIC of unspecified typeUnknown1 patient PEBD, all received cholestyramine, phenobarbital, rifampicin, UDCA100%85.7%6 cadaver livers, 2 living donors
Kondo et al[63]4 PFIC of unspecified type2-7NR75%75%
Bassas et al[56]5 PFIC3 8 “low GGT PFIC” PFIC1/210-40 moNR84.6%84.6%Parents of 12 out of 13 were 1st cousins
Cutillo et al[57]6 PFIC1/2 1 PFIC34-53 moNR100%75%
Englert et al[44]33 patients PFIC2 and 3UnknownUDCA 10 of 33 received biliary diversion then LT100% with prior diversion 89% without prior diversion100%
Aydogdu et al[52]10 PFIC1/2 2 PFIC343.2 ± 27 moUDCA69.2%75%Surviving patients show good quality of life, exacerbation of diarrhea as the exception, mix of LDLT and cadaveric
Hori et al[50,51]11 PFIC10.6-18.2 years oldTotal external biliary diversion performed at time of re-transplantation in one PFIC1 patient82.4% total graft survival (14/17)PFIC1 - 90.9% at 5 yr, 72.7% at 10 yr, 54.5% at 15 yr; PFIC2 - 100% at 5 yrDigestive symptoms in 10 out of 11 PFIC1; 8 out of 11 PFIC1 recipients exhibited steatosis; 9 out of 11 PFIC1 recipients exhibited fibrosis
Miyagawa-Hayashino et al[54], Egawa et al[53]3 PFIC2
Kaur et al[58]2 PFIC3 2 PFIC1/22, 2.5, 6 and 9 years old malesUDCA, phenobarbital and ondansetron100%75%