Rare Diseases Symptoms Automatic Extraction

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

[primary hyperoxaluria type 1]

Primary hyperoxaluria type 1 (PH1) 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 PH1 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 PH1 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 PH1 patients with end stage renal disease. Liver Transpl , 2014. © 2014 AASLD.

Diseases presenting "unselected consecutive series" symptom

  • primary hyperoxaluria type 1

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