engleski

Primary hyperoxaluria - Croatian experience

Primary hyperoxaluria type 1 is a rare, inborn glyoxalate error of an autosomal recessive trait. We present 3 patients with this disease drom different families, who are consistent with inheritance. Laboratory data including urine oxalate excretion confirmed the diagnosis. Computer programe EQUIL2 was used to calculate urine calcium oxalate saturation. The treatment consisted of administration od piridoxine (300-600 mg/m2) with alkali citrate (0.15 g/kg). All of three patients had a reduced spontaneous passage of urinary stones, one of them without urolithiasis for the last of 2 years, after 4 years of follow-up, and with normal renal function. The patient with transplanted kidney without liver transplantation now shows clear ultrasound signs of nephrocalcinosis.

primary hyperoxaluria ; Croatia

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