PREEMPTIVE LIVER-TRANSPLANTATION IN PRIMARY HYPEROXALURIA TYPE-1 - TIMING AND PRELIMINARY-RESULTS

Preemptive isolated liver transplantation (PLTX) can cure the metaboli c defect in primary hyperoxaluria type 1 (PH1) but there are no unifor mally accepted recommendations concerning the timing of this transplan tation procedure. We have performed PLTX successfully in 4 children (a ge 3-9 years) with PH1 with no mortality or morbidity due to the trans plantation procedure. Plasma and urinary oxalate levels normalised rap idly and renal function remained stable including one patient with adv anced chronic renal failure who showed a stable course for more than 2 4 months. Although treatment must be individualised in this severe met abolic disorder and PLTX has to be viewed as invasive procedure, we fe el PLTX should be offered and discussed not too late in the treatment of PHI to prevent or at least delay the progression to end stage renal disease and systemic oxalosis.