소아에 발생한 Cystine Stone 1례
A Case of Cystine Stone in a Child
소속 상세정보
조찬홍/Cho CH
함공식/박주견/김규환/Hahm KS/Park JK/Kim KH
KMID : 0358319860270060933
Abstract
Cystinuria is an autosomal recessive inherited defect in renal tubular reabsorption of four amino acids, cystine, ornithine, lysine and arginine. Homozygotes were identified by the formation of urinary tract calculi composed of cystine and by gross hyperexcretion of above mentioned four amino acids.
Urinary tract calculi composed primarily of cystine are rare in adults and children. These are usually pure and found exclusively in patients with cystinuria.
Herein we report on a 4 and 3/12 years old male child with a right renal stone composed primarily of cystine which was confirmed by chemical analysis method postoperatively. After discharge he has been treated with D-penicillamine, large fluid intake and conversion of urine pH.
키워드
결석; 소아; 시스틴결석; cystine stone; childhood; cystinuria
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