| Cystinurua in Siblings. |
|
Sung Ik Cho, Min Yong Oum, Jae Ock Park, Dong Hwan Lee, Sang Jhoo Lee |
|
Department of Pediatrics, College of Medicine, Soon Chun Hyang University, Seoul, Korea |
| 남매에서 발생한 Cystinuria |
|
조성익, 엄민용, 박재옥, 이동환, 이상주 |
|
순천향대학 의학부 소아과학교실 |
|
Received: 22 June 1989 • Accepted: 19 September 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. |
| Key Words:
Cystinuria, Amino acid analysis |
|
PDF Links
Download Citation
