Abstract

SUMMARY Cystinuria is a hereditary disorder of cystine and dibasic amino acids (lysine, arginine, ornithine) transport across the luminal membrane of renal tubules and intestine, resulting in recurrent nephrolithiasis. Predominant clinical finding of this disease is the occurrence of recurrent cystine stones. Cystine stones frequently occur in the second or third decade of life with an occasional occurrence in infancy. Atypical symptoms of renal stones as anuria can become apparent in infancy. Bilateral cystine stones causing acute anuria in infants are extremely rare. We report the first case of bilateral pyelolitotomy performed for cystine stone and impacted bilateral double-J (DJ) stents in an infant presenting with anuria. In our patients DJ stents were removed, and a stone-free state was successfully achieved.

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