Fourteen Years of Experience of Liver Transplantation for Wilson's Disease; a Report on 107 Cases from Shiraz, Iran.

BACKGROUND AND AIM: Liver transplantation is a potential cure for liver damage from Wilson's disease but the course of neuropsychiatric manifestations after transplantation remains undetermined. MATERIAL AND METHODS: In this study, data on all patients who'd received a liver transplant for Wilson's disease at the Shiraz Organ Transplantation Center between December 2000 and March 2014 were reviewed and compared to data on a control group who'd received a liver transplant over the same period but due to other causes. RESULTS: Out of 2198 patients who'd received a liver transplant in the period; 107 patients were diagnosed with Wilson's disease (21 with fulminant hepatic failure); age of patient ranged from 5 to 59 years; 56.07% of patients in this series had some type of neuropsychiatric manifestation before transplantation, of which 66.67% showed improvement after the procedure. 18 patients had aggravation of neuropsychiatric symptoms after transplantation. These neuropsychiatric symptoms were mostly for anxiety, tremor and depression but there were four cases of new onset dysarthria, rigidity and ataxia in various combinations. Survival rates of 1-month, 1-year, and 5-years for patients with Wilson's disease were 88%, 86%, 82%, respectively, evaluations were not statistically different from that of the control group. CONCLUSIONS: Liver transplantation showed good long-term results in patients with Wilson's disease, even in those presenting fulminant hepatic failure. Neuropsychiatric manifestations normally show improvement after transplantation but in some cases new onset of manifestations occurred after successful liver transplantation.

Effects of surgical technique on postoperative renal function after orthotopic liver transplant.

OBJECTIVES: The classic technique for orthotopic liver transplant consists of the total excision of the retrohepatic inferior vena cava during native hepatectomy. Controversy about the effects of the classic technique on postoperative renal function continues. The aim of this study was to evaluate the effects of the chosen hepatectomy technique on postoperative renal function. MATERIALS AND METHODS: Of 253 patients who received an orthotopic liver transplant between June 2006 and July 2008 in the Shiraz transplant unit, only 15 underwent operation with the classic technique. Patient demographics and factors including cold ischemic time, warm ischemic time, operative time, transfusions, blood loss, and early postoperative renal function were assessed retrospectively. The criteria for acute renal failure were a serum creatinine level of > 133 micromol/L (1.5 mg/dL), an increase in the baseline serum creatinine level by 50%, or oliguria requiring renal replacement therapy. RESULTS: All patients received a liver from a deceased donor, and none required venovenous bypass during the operation. The minimum mean arterial blood pressure value of the patients during clamping was 65 -/+ 19 mm Hg. The mean preoperative plasma creatinine level was 87.51 -/+ 39.78 micromol (0.99 -/+ 0.45 mg/dL). During the first week after transplant, 7 patients (46.6%) experienced acute renal failure, and 3 of those 7 required renal replacement therapy. By the sixth postsurgical month, 4 of those 7 patients had died (1 from adult respiratory distress syndrome, 2 from sepsis, and 1 from recurrent cholangiocarcinoma). In all other patients, the plasma creatinine level had returned to the normal range by the third postsurgical week 3 or during short-term follow-up. CONCLUSIONS: Use of the classic technique for orthotopic liver transplant may increase the rate of postoperative renal failure, but that complication usually resolves during short-term follow-up.