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Case ReportOpen Access
Cystinuria : Cause of Recurrent Renal Stones in a 4-Year-Old Girl
Tangnararatchakit K 
,
Ariyaprakai W ,
Tapaneya-Olarn W ,
Shotelersuk V ,
Petchthong T
,
Ariyaprakai W ,
Tapaneya-Olarn W ,
Shotelersuk V ,
Petchthong T 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.
Key word : Recurrent Renal Stones, Cystinuria, D-Penicillamine, Alkalinization
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.
Key word : Recurrent Renal Stones, Cystinuria, D-Penicillamine, Alkalinization
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