Failure of isolated kidney transplantation in a pediatric patient with primary hyperoxaluria type 2

Abstract

PH type 2 is caused by decreased activity of GRHPR enzyme that eventually leads to ESRD and systemic oxalosis. Here, we describe an Iranian pediatric patient with PH2 and early ESRD development who received recommended treatment by undergoing isolated kidney transplantation. Diagnosis criteria included a history of reoccurring calcium oxalate renal stones and elevated oxalate levels combined with liver biopsy and decreased enzymatic activity at age five. ESRD prompted transplantation and was performed at age nine. On Day 12 post-op, his serum creatinine level increased. A graft biopsy showed calcium oxalate crystal deposits in renal tubes with no evidence of acute rejection, which resolved with intensive hydration and administration of a potassium citrate solution. Subsequent biopsies confirmed results found in first biopsy. Despite the immunosuppressive therapy, his serum creatinine level increased again after 11 months. Renal tubular obstruction then led to graft nephrectomy. Pathological analysis of tissue confirmed findings of past biopsies. This was a very rare case of early ESRD in PH2 resulting in a failed isolated kidney transplant. As the GRHPR enzyme is predominantly expressed in liver, we suggest a combined liver-kidney transplant may be beneficial in patients with PH2.

Combined Liver–Kidney Transplantation for Primary Hyperoxaluria Type 2: A Case Report

“…After transplant, hemodialysis is often required in cases of delayed graft function to keep the plasma oxalate level below supersaturation threshold until renal allograft function improves. Typically, kidney transplant alone is the preferred option for PH2 patients who develop ESRD 1, 10 although loss of renal allograft due to recurrence of oxalate nephropathy, as occurred in our patient, has been reported 11 .…”

Combined liver–kidney transplantation for hyperoxaluria type II?

“…Naderi et al. presented an interesting case of rapid graft loss in a patient with PH type II receiving conventional hemodialysis thrice weekly. Considering the circumstances, it is understandable that adjusting dialysis to maintain plasma oxalate levels below 30 μ m post‐hemodialysis has not been feasible.…”

“…In this edition of Pediatric Transplantation , Naderi et al. published an unusual case of end‐stage renal disease (ESRD) in a relatively young patient with primary hyperoxaluria (PH) type II. Although the patient was considered for an isolated kidney transplant, the standard of care for this condition, evidence suggesting immediate recurrence and subsequent graft loss was found .…”

Tubular and genetic disorders associated with kidney stones

“…Conservative treatment is similar to PH2, except for pyridoxine administration (unresponsive). The current treatment of choice is isolated kidney transplantation; however, the less severe course of PH2 means that only a few patients have required transplantation, although immediate recurrence with subsequent rapid graft loss has been reported in one patient [65,64,61,66].…”