Long-term results of combined liver-kidney transplantation for Primary Hyperoxaluria Type 1: The French experience.

[primary hyperoxaluria type 1]

Primary hyperoxaluria type 1 (PH 1 ) is a hepatic metabolic defect leading to end stage renal failure (ESRF ). Post-transplant recurrence of kidney disease incites to propose combined liver -kidney transplantation (LKT ). However , the risk of LKT procedure is theoretically far higher than kidney alone transplantation (KAT ). An unselected consecutive series of 54 patients with PH 1 was analyzed according to the type of transplantation initially performed from May 1979 to June 2010 in 10 French centers . Duration of dialysis , extra -renal lesions , age and follow-up were similar between groups . Post-operative morbi-mortality did not differ between groups . Ten -year patient 's survival was similar between LKT (n =33 ) and KAT (n =21 ) groups (78 % vs . 70 %). Kidney graft survival at 10 years was better after LKT (87 % vs . 13 %; p <0 .0001 ). Four patients (12 .1 %) lost their first kidney graft in LKT group vs . 19 (90 %) in KAT group (p <0 .0001 ). Recurrence of oxalosis occurred in 11 renal grafts (52 %) of the KAT group vs . none in LKT group (p < 0 .0001 ). ESRF due to rejection was also higher in the KAT group (19 % vs . 9 %; p <0 .0001 ). A second kidney transplantation was performed in 15 patients (71 %) in the KAT group versus 4 patients (12 %) in the LKT group (P < 0 .001 ). LKT for PH 1 provides better kidney graft survival , lesser rejection , a similar long -term patient survival and is not associated with an increased short -term mortality risk . LKT must be the first -line treatment for PH 1 patients with end stage renal disease . Liver Transpl , 2014 . © 2014 AASLD .