All are indicated in a patient with cystinuria with multiple renal stones except
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
Cysteamine
Increase fluid intake
Alkalinization of urine
Penicillamine