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Treatment

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Treatment aims to increase cystine solubility by:

  • high fluid intake:
    • at normal urine pH and with typical urinary cystine excretion of 4 mmol per day, an adequate fluid intake would be more than 4 litres per day to keep urine cystine concentration less than 1 mmol per litre

  • urinary alkalinisation:
    • taking bicarbonates to keep urine pH above 8 at which cystine solubility is increased

  • D-penicillamine:
    • acts as a chelating agent but may need 1-2 g daily which may be poorly tolerated because of side effects

  • lysine supplementation:
    • this is the only essential aminoacid which is excessively excreted in cystinuria

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