Combined liver-
kidney transplantation is a therapeutic option for children affected by type 1
primary hyperoxaluria. Persistently high plasma
oxalate levels may lead to kidney graft failure. It is debated whether pre-emptive
liver transplantation, followed by
kidney transplantation, might be a better strategy to reduce kidney graft loss. Our experience of 6 pediatric combined liver-kidney transplants for
primary hyperoxaluria type 1 in pediatric recipients was retrospectively analyzed. Plasma
oxalate levels were monitored before and after
transplantation. All the recipients were on
hemodialysis at
transplantation. Median [IQR] recipient's age at
transplantation was 11 [1-14] years; in all cases, a compatible graft from a pediatric
brain-dead donor aged 8 [2-16] years was used. In a median follow-up of 7 [2-19] years after combined liver-
kidney transplantation, no child died and no liver graft failure was observed; three kidney grafts were lost, due to chronic rejection, primary non-function, and early renal
oxalate accumulation. Liver and kidney graft survival remained stable at 1, 3, and 5 years, at 100% and 85%, respectively. Kidney graft loss was the major complication in our series. Risk is higher with very young, low-weight donors. The impact of treatment with glyoxalate pathway
enzyme inhibitors treatment in children with advanced disease as well as of donor kidney preservation by ex vivo machine perfusion needs to be evaluated. At present, a case-by-case discussion is needed to establish an optimal treatment strategy.