Orthotopic liver transplantation for alagille syndrome
| Autor: | Giuliano Torre, Paola Stroppa, Domenico Pinelli, Elena Torri, Marco Spada, Michele Colledan, Alessandro Lucianetti, Daniele Alberti, M. Zambelli, M Guizzetti, A Bertani, M. Giovanelli, V. Corno, G. Maldini, B. Gridelli |
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| Přispěvatelé: | Maldini, G, Torri, E, Lucianetti, A, Guizzetti, M, Pinelli, D, Bertani, A, Corno, V, Giovanelli, M, Zambelli, M, Stroppa, P, Alberti, D, Torre, G, Spada, M, Gridelli, B, Colledan, M |
| Rok vydání: | 2005 |
| Předmět: |
treatment planning
Cardiac Catheterization Time Factors hepatic artery bile duct obstruction graft survival Gastroenterology Orthotopic Liver Transplantation Alagille Syndrome Postoperative Complications Actuarial Analysis Alagille syndrome anastomosis dehiscence postoperative complication surgical drainage Child conference paper intestine necrosi hemoperitoneum Pediatric clinical article Incidence (epidemiology) abdominal bleeding Whole liver cholestasi lymph node metastasi artery thrombosi pulmonary artery stenosi aorta reconstruction ascite female priority journal survival rate medicine.medical_specialty organ donor Orthotopic liver transplantation pleura effusion surgical infection reoperation blood vessel occlusion percutaneous transluminal angioplasty Actuarial survival aorta coarctation acute graft rejection male liver graft Internal medicine death heart ventricle septum defect liver vein thrombosi medicine Humans cadaver donor human portal vein thrombosi postoperative thrombosi treatment failure Retrospective Studies Transplantation business.industry recipient disease association medicine.disease infant Survival Analysis thorax surgery Surgery Liver Transplantation Hepatic artery thrombosis adolescent Split liver transplantation bile duct fistula incidence surgeon stent business Cadaveric spasm radiologist liver graft rejection Follow-Up Studies |
| Zdroj: | ResearcherID Web of Science |
| ISSN: | 0041-1345 |
| Popis: | Alagille syndrome (AS) is a dominantly inherited, multisystem disorder involving the liver, heart, eyes, face, and skeleton. From October 1997 through July 2004, 260 pediatric orthotopic liver transplantations (OLTx) were performed in 231 patients. This report describes 21 patients of median age 1.95 years (range, 0.7-16.7) who had alagille syndrome. We present the technical features of the OLTx, incidence and type of complications, medical conditions related to the syndrome, need for retransplantation, as well as patient and graft survival rates. A split liver technique was used in 16 patients (76%) who received a left lateral segment (LLS) graft whereas 7 patients (33%) received a whole liver. Only cadaveric donors were used. The major surgical complications requiring reintervention in 11 patients (52%) included biliary problems (19%) and vascular complications (17%). One case of hepatic artery thrombosis required retransplantation. Three recipients (14%) died. All other patients are alive with an actuarial survival rate of 90% at 1 year and 80% at 5 years. The actuarial graft survival rate is 85% at 1 year and 75% at 5 years. Patients with AS, despite the associated cardiovascular anomalies, can be treated successfully by a combined approach between cardiologist, radiologist, cardiothoracic, and liver transplant surgeons. With careful planning and operative management, the results are comparable with those obtained with other more common cholestatic diseases. |
| Databáze: | OpenAIRE |
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