Liver Transplantation Outcome in Iranian Patients with Primary Hyperoxaluria; Risks and Perspectives

Abstract

Background: Primary hyperoxaluria (PH) is an autosomal genetic disorder characterized by abnormal glycosylate metabolism. Objectives: The aim of the present study was to assess post-transplant complications and survival of patients with PH who underwent either liver transplantation (LT) or simultaneous liver-kidney transplantation. Methods: 18 patients with established PH diagnosis who underwent LT or simultaneous liver-kidney transplantation in the Transplantation Center of Shiraz, Iran, were included. Demographic and clinical data were collected by reviewing clinical documents and interviews by the patients. The patients were prospectively followed up for the occurrence of the intended outcomes. The data was analyzed by SPSS 18 software. Results: 12 patients (66.7%) were male, and six patients (33.3%) were female with the age range of 3 to 32 years (mean age, 18.89 ± 7.42 years). The patients’ weight ranged from 13 to 73 kg (mean weight, 47.39 ± 17.18 kg). Polyuria was the most common clinical presentation (11/18), and end-stage renal disease and hemodialysis were noted in 13 and 12 patients pre-transplantation, respectively. Hepatic arterial thrombosis, biliary complications, infections, and graft rejection comprised the most frequent post-transplant complications. Of 18 patients, seven patients (38.9%) died due to various complications during one year after transplant. Based on the Kaplan-Meier analysis, the survival rate was 61.1% at the end of the study. The mean survival time was 46.25 ± 18.6 months. The patients succumbed to the disease died within 3 to 320 days (mean, 61.57 days) post-surgery. Conclusions: LT seems an effective intervention in prevention of kidney failure in patients with PH.