Oxalosis with nephrocalcinosis.

Primary hyperoxaluria type I is a rare autosomal show reflux. Cystoscopy with retrograde urogram showed no evidence of obstruction. A diagnosis of recessive metabolic disorder characterized by absence of liver specific peroxisomal alanine glyoxylate aminoend-stage renal disease (ESRD) of unknown aetiology was made. Live related donor kidney transplant was transferase (AGT ) leading to elevated urinary excretion of glycolate and oxalate [1]. Calcium oxalate has performed. The graft function was excellent and creatinine reached 0.9 mg/dl on third post-transplant day. a very low solubility leading to nephrocalcinosis, urolithiasis and progressive renal insufficiency [2]. Due to From the tenth day, there was gradual increase in serum creatinine. When creatinine rose to 1.4 mg/dl the rarity of the disease, these specific diagnostic tests are not performed routinely unless the diagnosis is on 25th day, a kidney biopsy was performed which showed tubules filled with oxalate crystals (Figure 2). strongly suspected. We report a case of primary hyperoxaluria in which the diagnosis was not entertained Over the next few days there was progressive decline in graft function due to recurrence of primary disease. because there was no past history of urinary calculi and no evidence of nephrocalcinosis on plain X-ray A repeat abdominal X-ray (Figure 3) after the patient restarted dialysis, revealed dense nephrocalcinosis in and ultrasonography. The disease was detected and diagnosed only after kidney transplantation. the native kidneys. If this nephrocalcinosis had been obvious at the initial diagnosis of ESRD, renal transA 19-year-old female presented in September 1993 with uraemic symptoms, severe anaemia and azotaemia plantation would have been avoided. This case highlights the fact that in young patients (serum creatinine, 13.6 mg/dl ). Routine urinalysis was unremarkable. Ultrasound of kidneys showed right with ESRD of unknown origin with any evidence of renal calculi, oxalosis must be excluded. kidney of 7.1×3.1 cm size and left kidney of 10.7×5.5 cm. Both kidneys showed increased echogenicity. The pelvicalyceal system of left kidney appeared References mildly dilated and a plain abdominal X-ray (Figure 1) revealed a radio-opaque density at upper pole of the 1. Gruessner RW. Pre-emptive liver transplantation from a living left kidney. A micturating cystourethrogram did not related donor for primary hyperoxaluria type I. N Eng J Med 1998; 338: 1924 2. Shah B, Antoine C, Mercier F, Julia P, Duboust A, Glotz D. Correspondence and offprint requests to: Dr Bharat V. Shah, MD, Successful kidney retransplantation after combined liver/kidney DNB, Head, Department of Nephrology, P.D. Hinduja National transplantation in primary hyperoxaluria type I. Nephrol Dial Hospital and Medical Research Centre, Veer Savarkar Marg, Transplant 1998; 13: 1568–1570 Mahim, Mumbai-400 016, India.