treatment
Last reviewed 01/2018
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