All are indicated in a patient with cystinuria with multiple renal stones except
Correct Answer: Cysteamine
Description: Ans. a. Cysteamine Patient with cystinuria with multiple renal stones should be treated with increase urine volume (high fluid intake), alkalinization of urine Penicillamine and tiopronin. Cystinuria (AR) Inheritance: Autosomal recessive Molecular defect: Shared dibasic cystine transpoer SLC3A1, SLC7A9Q Tissue manifesting transpo defect: Proximal renal tubule, jejunal mucosaQ Individual substrate: COLA (Cystine, Ornithine, Lysine, Arginine)Q Clinical features: Cystine nephrolithiasisQ Treatment: Increase urine volumeQ (high fluid intake) Alkalinization of urineQ PenicillamineQ and tiopronin undergoes sulfhydryl-disulphide exchange with cystine to form mixed disulphides, since these disulphides are much soluble than cystine, pharmacologic therapy can prevent and promote dissolution of calculi
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