Abstract
BackgroundPrimary hyperoxaluria (PH) is an inherited disease lacking of hepatic oxalic acid metabolic enzymes which could lead to irreverisible renal damage. Currently, liver–kidney transplantation is a curative but highly invasive therapy used to treat patients with PH. However, limited studies have focused on combined liver–kidney transplantation (CLKT) and sequential liver and kidney transplantation (SLKT) in patients with PH.MethodsThe present study included 201 patients with PH who received both liver and kidney transplants and who were listed on the Scientific Registry of Transplant Recipients from 1987 to 2018. According to the liver–kidney transplant procedure, patients were separated into a CLKT group and a SLKT group. Patient demographics and transplant outcomes were assessed in each group.ResultsCompared with the SLKT group, The CLKT group got a worse pretransplant dialysis condition in both the proportion of patients under pretransplant dialysis (p = 0.048) and the duration of the pretransplant dialysis (p < 0.001). The SLKT group got higher human leukocyte antigen mismatch score of kidney donor (p < 0.001) and liver donor (p = 0.003). The CLKT group utilized higher proportion (98.9%) of organs from a single deceased donor, while the SLKT group utilized 75.0% of organs from deceased liver donors and only 35.0% of organs from deceased kidney donors (p < 0.001). Kidney function measured by serum creatinine concentration before liver transplantation (LT) or CLKT was similar (p = 0.305) between groups. Patient survival was not significantly different between the two groups (p = 0.717) and liver (p = 0.685) and kidney (p = 0.464) graft outcomes were comparable between the two groups.ConclusionsSLKT seems to be an alternative option with strict condition for CLKT, further exploration about the SLKT is still required.
Highlights
Primary hyperoxaluria (PH) is an inherited disease lacking of hepatic oxalic acid metabolic enzymes which could lead to irreverisible renal damage
The proportion of patients under dialysis was higher in the combined liver–kidney transplantation (CLKT) group compared with the sequential liver and kidney transplantation (SLKT) group (87.9% vs. 64.3%, p = 0.048), and the pretransplant dialysis duration was found longer in the CLKT group than the SLKT group (17.0 months (IQR 7.0, 22.0) vs. 4.0 months (IQR 0.0, 12.3), p < 0.001)
As for liver transplantation (LT) procedure type, the proportion of patients receiving whole liver graft was higher in the CLKT group compared with the SLKT group (80.1% vs. 60.0%; P = 0.025)
Summary
Primary hyperoxaluria (PH) is an inherited disease lacking of hepatic oxalic acid metabolic enzymes which could lead to irreverisible renal damage. Liver–kidney transplantation is a curative but highly invasive therapy used to treat patients with PH. Primary hyperoxaluria (PH) is a kind of congenital disorder that results from abnormal glyoxylate metabolism owing to deficiency of hepatic enzymes [1]. Symptoms of PH are usually observed in the urinary tract, and are related to continuous oxalate deposition and irreversible renal damage resulting in end-stage kidney disease (ESKD). Since simple renal therapies such as dialysis or kidney transplant [1] are not able to solve oxalate accumulation, more effective strategies are under exploration
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