Living related liver transplantation in 13 cases of progressive familial intrahepatic cholestasis.
Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of disorders with various etiologies. Recent molecular and genetic studies have categorized the spectrum of types. Liver transplantation is a curative modality of treatment in this disease. We report our experience with 13 patients with PFIC who underwent living related liver transplantation. The follow-up periods ranged from 12 to 50 months. Two children died at 1 and 2 years posttransplantation, leading to a decrease in survival rate from 100% in the first year to 84.6%.
Bassas
A
A
Department of Surgery, Riyadh Armed Forces Hospital, Riyadh 11159, Kingdom of Saudi Arabia.
Chehab
M
M
Hebby
H
H
Al Shahed
M
M
Al Husseini
H
H
Al Zahrani
A
A
Wali
S
S
D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000235:genetics* / Q000401:mortality / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000706:statistics & numerical data*; D019520:Living Donors; D008297:Male; D011183:Postoperative Complications / Q000145:classification / Q000453:epidemiology; D016896:Treatment Outcome