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Early recurrence of oxalate deposition after renal transplantation in a patient with primary hyperoxaluria type I

1999; Elsevier BV; Volume: 31; Issue: 8 Linguagem: Inglês

10.1016/s0041-1345(99)00699-5

1873-2623

N Bi̇lgi̇n, M.B. Tirnaksiz, Gökhan Moray, H. Karakayalı, Sedat Yıldırım, B. Demirhan, Mehmet Haberal,

Biomedical Research and Pathophysiology

RIMARY hyperoxaluria type I (PHI) is a rare inherited (autosomal recessive) metabolic disorder caused by the missing liver enzyme alanine glyoxalate (AGT). The disease is characterised by an increased excretion of glycolate and oxalate in the urine. The excess oxalic acid may lead to calcium oxalate nephrolithiasis, nephrocalcinosis, and gradual reduction in renal function with end-stage renal failure. 1 This report concerns a 27-year-old patient with PHI who developed recurrence of nephrocalcinosis shortly after renal transplantation. CASE REPORT A 27-year-old male was diagnosed with chronic renal failure secondary to nephrolithiasis 1 year ago. His renal failure progressed rapidly and the patient required chronic intermittent hemodialysis. There was no family history of renal disease. Eight months later he received a cadaver kidney transplant from a 45-year-old male who had died due to cerebral hemorrhage. During the same operation, the patient also underwent left native nephrectomy due to left hydroureteronephrosis. The histologic examination of the native kidney showed chronic tubulointerstitial nephritis due to oxalate deposition. The diagnosis of primary hyperoxaluria type I was confirmed by the finding of significantly increased amounts of oxalic acid being excreted. In the immediate postoperative period, serum creatinine levels gradually fell. Despite the absence of histologic evidence of acute rejection, the patient was given a total of 3000 mg of methylprednisolone. High diuresis, exceeding 4 L/d, was maintained using furosemide associated with high fluid infusion. The patient also received oral pyridoxine therapy. Renal function slowly improved and the patient was discharged with a normal serum creatinine level. The outcome was favourable until day 18 posttransplantation, when acute deterioration of renal function was encountered. A graft kidney biopsy revealed oxalate deposition and acute rejection. Urinary oxalic acid levels were still significantly high. The patient received a total of 6000 mg of methylprednisolone. Intensive hemodialysis was used to maintain plasma oxalate levels near the normal range. Unfortunately, antirejection therapy was unsuccessful, and the patient required graft nephrectomy. Chronic intermittent hemodialysis was restarted. At day 35 posttransplantation, the patient underwent right native nephrectomy.