A Case of Cystine Stone in a Child.
- Author:
Chan Hong CHO
1
;
Kong Sik HAHM
;
Joo Kyun PARK
;
Kyu Hwan KIM
Author Information
1. Department of Urology, Han-il Hospital, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
cystine stone;
childhood;
cystinuria
- MeSH:
Adult;
Amino Acids;
Arginine;
Calculi;
Child*;
Cystine*;
Cystinuria;
Homozygote;
Humans;
Hydrogen-Ion Concentration;
Lysine;
Male;
Ornithine;
Penicillamine;
Urinary Tract
- From:Korean Journal of Urology
1986;27(6):933-938
- CountryRepublic of Korea
- Language:Korean
-
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.