SINGLE CENTER TRANSPLANT EXPERIENCE WITH PRIMARY HYPEROXALURIA

Abstract

607 The unique management challenges posed by transplantation (tp) in primary hyperoxaluria greatly influence outcomes. Familiarity with the pathophysiologic properties of oxalate, individualized pt selection protocols, and rigorous post-transplant care that minimizes kidney allograft loss from recurrent oxalosis result in improved graft survival. We report single center experience for combined liver/kidney(L/K) and kidney(K) alone transplantation in primary hyperoxaluria types I (PHI) and II (PHII). Of 25 PHI pts seen between 1948 and 1998, 13 (52%) developed ESRD (mean age 29.9 yrs). 9 pts underwent K tp (4 CAD, 5 LRD) and 6 L/K tp. Mean duration of ESRD prior to K tp was 6.0 months (range 0-24, median 4.5) and 30 months for L/K tp (range 4-68, median 29.5). One patient received a solitary liver tp after 2 previous renal tps elsewhere. One PHII pt received a CAD K tp 4 months after presenting with ESRD. 4 PHI patients had extrarenal (primarily bone marrow) calcium oxalate deposition prior to transplantation. Post tp urine (mmol/SA/d) and plasma (umol/L) oxalate values were: (Table)TableTotal rejection episodes were 7 (6 renal, 1 hepatic). 5 acute renal rejection episodes occurred in L/K tps (range 1 mo.-9yrs post tp). 7 pts had functioning allografts at last follow-up. (Table)TableAdvances in immunosuppression, the established benefit of treatment (pyridoxine, orthophosphate, high urine volumes), the use of specific oxalosis tp protocols, and careful long-term F/U of PH patients continue to improve outcomes in experienced centers.