Orthotopic liver transplantation for Byler's disease
| Autor: | Marco Spada, M Guizzetti, G. Maldini, E. Doffria, Giuliano Torre, Domenico Pinelli, V. Corno, Bruno Gridelli, M. Zambelli, A Bertani, M. Giovanelli, M.L. Melzi, Michele Colledan, Daniele Alberti, Elena Torri, Alessandro Lucianetti |
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| Přispěvatelé: | Torri, E, Lucianetti, A, Pinelli, D, Corno, V, Guizzetti, M, Maldini, G, Zambelli, M, Bertani, A, Melzi, M, Alberti, D, Doffria, E, Giovanelli, M, Torre, G, Spada, M, Gridelli, B, Colledan, M |
| Rok vydání: | 2005 |
| Předmět: |
Liver Cirrhosis
Male surgical mortality medicine.medical_treatment Fistula hepatic artery treatment indication bile duct obstruction graft survival Liver transplantation blood culture Gastroenterology postoperative complication Child conference paper clinical article Liver Cirrhosis Biliary Incidence (epidemiology) disease course Biliary Progressive familial intrahepatic cholestasis postoperative infection Thrombosis artery thrombosi Treatment Outcome priority journal Child Preschool Female survival rate medicine.medical_specialty Adolescent reoperation Anastomosis acute graft rejection body weight Internal medicine liver vein thrombosi medicine fistula follow up Humans human Preschool Retrospective Studies Transplantation Follow-Up Studies Infant Liver Transplantation business.industry Retrospective cohort study intrahepatic cholestasi medicine.disease Surgery clinical feature Stenosis incidence business |
| Zdroj: | Transplantation proceedings. 37(2) |
| ISSN: | 0041-1345 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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