J Med Assoc Thai 2003; 86 (6):573

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Cystine Urinary Lithiasis in Thailand : A Report of Five Cases
Chaimuangraj S Mail, Jamavan K , Mokkhavesa C , Timvipak C , Junvimalaung N , Stitchantrakul W , Kochakarn W , gojaseni P

KAMOLKANT JAMA VAN, BEd**,
CHUCHART TIMVIPAK, BSc**,
WAS ANA STITCHANTRAKUL, MSc***,
PHAITUN GOJASENI, MD*
Cystine urinary stone is an autosomal recessive hereditary disease, frequently recurring and
resisting fragmentation by Shockwave lithotripsy. As cases have never been reported before in Thai-
land, five cases of renal cystine stones at Ramathibodi Hospital were reported. Two were in the same
family. In all cases the stones were removed by open surgery or percutaneous nephrolithotomy. Post-
operatively, all the stones were analyzed by infrared spectroscopy for cystine. In two cases, cystine
stones were also identified by scanning electron microscopy. Urine was analyzed for cystine by sodium
cyanide-nitroprusside test, its concentration by spectrophotometry and cystine crystals were identified
by the new crystal induction technique under light microscopy. By high-performance liquid chromato-
graphy (HPLC) test, urinary dibasic amino acids (ornithine, lysine, arginine) in these cases were also
found to be significantly elevated. Clinical findings, diagnosis, treatment and prevention of cystine
stones are reviewed.
Key word : Cystine Stone, Crystal, Cystinuria, Infrared Spectroscopy, Sodium Cyanide Niroprusside,
Spectrophotometry, Scanning Electronmicroscopy, Urinary Dibasic Amino Acids, Litho-
triptor

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