In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient and graft survivals. BD was the indication in 12 patients of median age 1.32 years and median weight 10 kg. Median follow-up was 670 days. Major surgical complications requiring reintervention occurred in three patients. No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in four patients. One patient had a biliary anastomotic stenosis successfully treated by percutaneous techniques. Four patients had episodes of acute rejection treated with steroids. Two patients were retransplanted, both of whom died in the early postoperative period due to hepatic vein thrombosis and venoenteric fistula. The actuarial patient and graft survival was 83% at 1 year and 83% at 5 years. Split-liver grafts represent an excellent organ supply for these patients, achieving good results with no mortality on the waiting list.

Torri, E., Lucianetti, A., Pinelli, D., Corno, V., Guizzetti, M., Maldini, G., et al. (2005). Orthotopic liver transplantation for Byler's disease. TRANSPLANTATION PROCEEDINGS, 37(2), 1149-1150 [10.1016/j.transproceed.2005.01.041].

Orthotopic liver transplantation for Byler's disease

Colledan M
2005

Abstract

In this study we analyzed the features of 12 patients who underwent liver transplantation for progressive familial intrahepatic cholestasis (Byler's disease [BD]) in view of the technical features of the OLTx, incidence and type of complications, need for retransplantation, as well as patient and graft survivals. BD was the indication in 12 patients of median age 1.32 years and median weight 10 kg. Median follow-up was 670 days. Major surgical complications requiring reintervention occurred in three patients. No thrombosis of the hepatic artery was observed. Infections with positive blood cultures were diagnosed in four patients. One patient had a biliary anastomotic stenosis successfully treated by percutaneous techniques. Four patients had episodes of acute rejection treated with steroids. Two patients were retransplanted, both of whom died in the early postoperative period due to hepatic vein thrombosis and venoenteric fistula. The actuarial patient and graft survival was 83% at 1 year and 83% at 5 years. Split-liver grafts represent an excellent organ supply for these patients, achieving good results with no mortality on the waiting list.
Articolo in rivista - Articolo scientifico
acute graft rejection; adolescent; artery thrombosis; bile duct obstruction; blood culture; body weight; child; clinical article; clinical feature; conference paper; disease course; female; fistula; follow up; graft survival; hepatic artery; human; incidence; infant; intrahepatic cholestasis; liver transplantation; liver vein thrombosis; male; postoperative complication; postoperative infection; priority journal; reoperation; surgical mortality; survival rate; treatment indication; treatment outcome;
English
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Torri, E., Lucianetti, A., Pinelli, D., Corno, V., Guizzetti, M., Maldini, G., et al. (2005). Orthotopic liver transplantation for Byler's disease. TRANSPLANTATION PROCEEDINGS, 37(2), 1149-1150 [10.1016/j.transproceed.2005.01.041].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/365664
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