SUN-331 COMBINED LIVER KIDNEY TRANSPLANTATION FOR PRIMARY HYPEROXALURIA: SINGLE CENTRE EXPERIENCE IN SOUTH INDIA

Abstract

Type 1 primary hyperoxaluria is a rare inherited autosomal recessive disorder, characterized by deficiency of the hepatic enzyme alanine glyoxylate aminotransferase, leading to increased oxalate production, nephrocalcinosis, urolithiasis, and systemic oxalosis. Early diagnosis and management is pivotal as medical management and definitive treatment in the form of combined or sequential liver-kidney transplantation, isolated liver or kidney transplantation have been studied to transform disease outcomes. Here, we review the experience from our institution of combined liver-kidney transplantation among patients with primary hyperoxaluria from February, 2014 to October, 2019. Among three children with primary hyperoxaluria, two underwent deceased donor liver-kidney transplantation, and the third live related donor sequential liver-kidney transplantation. Case 1 was that of a 17 year old boy, who had been on intensive daily dialysis for two years prior to deceased donor sequential liver-kidney transplantation, with a history of bladder calculus in childhood. He had a relatively uneventful post -transplant period and is doing well on follow up five years following transplantation. Case 2 was a 4 year old boy, diagnosed to have oxalosis two years prior to transplant, on dialysis five days a week through tunnelled catheter, prior to deceased donor liver-kidney transplantation, with a history of tonic-clonic seizures. Post-operatively, he had a stormy course with cytomegalovirus infection of the hepatic allograft, and hepatic artery thrombosis, and expired a month following transplantation. Case 3 was that of a 13 year old girl, diagnosed at 10 years of age with primary hyperoxaluria, who underwent live related donor sequential liver-kidney transplantation. She developed obstructive jaundice following liver transplantation, owing to biliary stricture, and underwent ERCP and biliary stenting. She also had an upper gastro -intestinal bleed, and profound anaemia, and later developed sepsis and cholangitis, which were conservatively managed. Subsequently, she underwent renal transplantation and later developed gram negative septicemia, and recurrent proximal bile duct stricture, which was subjected to biliary stenting. Presently, she has stable graft function, and is clinically stable. The experience from our institution re-emphasises the significance of early diagnosis, and pre-transplant intensive dialysis in reducing oxalate load if planning for sequential liver-kidney transplantation to improve outcomes in primary hyperoxaluria. Isolated liver or kidney transplantation have not been adopted in our centre. In all patients, Scheinman protocol was followed in the immediate post-transplant period. Renal biopsies in the recipient showed only mild oxalate deposits which did not affect graft survival.