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The primary hyperoxalurias (PHs) are rare inherited metabolic disorders that mainly affect the kidney. It results from abnormal glyoxylate metabolism causing increased excretion of urinary oxalate leading to recurrent renal stone formation, nephrocalcinosis, and ultimately renal impairment. Primary hyperoxaluria is rarely reported from Bangladesh. We here by discuss a case of primary hyperoxaluria in a 10-year-old boy who presented with convulsion and unconsciousness.