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...

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Veröffentlicht in:	Transplantation proceedings 2003-12, Vol.35 (8), p.3003-3005
Hauptverfasser:	Bassas, A, Chehab, M, Hebby, H, Al Shahed, M, Al Husseini, H, Al Zahrani, A, Wali, S
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological and medical sciences Child, Preschool Cholestasis, Intrahepatic - genetics Cholestasis, Intrahepatic - mortality Cholestasis, Intrahepatic - surgery Female Gastroenterology. Liver. Pancreas. Abdomen Humans Infant Liver Function Tests Liver Transplantation - statistics & numerical data Liver, biliary tract, pancreas, portal circulation, spleen Liver. Biliary tract. Portal circulation. Exocrine pancreas Living Donors Male Medical sciences Other diseases. Semiology Postoperative Complications - classification Postoperative Complications - epidemiology Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Surgery of the digestive system Treatment Outcome
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Zusammenfassung:	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%.
ISSN:	0041-1345 1873-2623
DOI:	10.1016/j.transproceed.2003.10.047