Cystinuria: cause of recurrent renal stones in a 4-year-old girl.
This paper presents the case report of a 4-year and 6-month old girl with cystinuria. She clinically presented with recurrent radiopaque renal stones since the age of 3 years. She received 2 subsequent operations of pyelolithotomy combined with ureterolithotomy at the age of 3 years 6 months, and pyelolithotomy alone at the age of 5 years. She was initially diagnosed as having cystinuria by the presence of hexagonal plate crystals in her acidified urine and positive for the urinary cyanide-nitroprusside test. The diagnosis was confirmed by urinary amino acid analysis using quantitative ion-exchange chromatography which revealed increased amounts of cystine and dibasic amino acids of lysine and ornithine. In spite of maintaining a high fluid intake and alkalinizing urine by giving potassium citrate after the first operation, recurrent renal stones were found. Therefore, after the second operation, D-penicillamine was additionally introduced. During the 18-month follow-up, although there were recurrent renal stones, the rate of stone formation was slower. To the authors' knowledge, this is the first case report in Thailand.
Tangnararatchakit
Kanchana
K
Nephrology Unit, Department of Pediatrics, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
Ariyaprakai
Watcharin
W
Tapaneya-Olarn
Wiwat
W
Shotelersuk
Vorasuk
V
Petchthong
Thanom
T
D002675:Child, Preschool; D003555:Cystinuria / Q000150:complications*; D005260:Female; D006801:Humans; D007669:Kidney Calculi / Q000209:etiology*; D012008:Recurrence