All are indicated in a 30-year-old patient with increased serum cystine and multiple renal stones except:
The clinical diagnosis of profile given in the question is CYSTINURIA . Cystinuria- AR,defect in re-absorptive transpo of cystine and the dibasic amino acids ornithine, arginine, and lysine from the luminal fluid of the renal proximal tubule and small intestine. Urinalysis- hexagonal shaped crystals: pathognomonic of cystinuria. The foundation of cystine stone prevention is adequate hydration and urinary alkalinization. When this conservative therapy fails, the addition of drugs, such as D-penicillamine and captopril. -The clinical profile should not be confused with Cystinosis which is treated with Cysteamine.
Cysteamine
Increase fluid intake
Alkalinization of urine
Penicillamine
Kidney Stones & Renal Tubular Acidosis