All issues > Volume 33(3); 1990

Original Article

J Korean Pediatr Soc. 1990;33(3):351-359. Published online March 31, 1990.

Cystinurua in Siblings.

Sung Ik Cho¹, Min Yong Oum¹, Jae Ock Park¹, Dong Hwan Lee¹, Sang Jhoo Lee¹

¹Department of Pediatrics, College of Medicine, Soon Chun Hyang University, Seoul, Korea

Received: June 22, 1989;  Accepted: September 19, 1989.

Abstract

We report cystinuria in siblings. Renal colic, hematuria, dysuria and stone passage were developed in younger brother (4 year 6 month old boy). But the elder sister (6 year old girl) had no specific symptoms nor signs. The identification of the disease was proved by cyanide nitroprusside test and amino acid analysis of urine. In our patients the chromatographic amino acid patterns of urine showed remarkably increased excretion of cystine, ornithine, lysine, and arginine. They are managed by adequate hydration with Shohl solution for rendering the urine more alkaline, and alpha-mercaptopropionylglycine (Thiola®). A review of literatures was also attempted briefly.

Keywords :Cystinuria, Amino acid analysis