العلاج المحافظ الأولي للسيستينوريا

DOI: 10.1016/j.kint.2020.06.035

• Fluid intake should guarantee a urine output large enough to maintain a cystine concentration of <250 mg/l (1 mmol/l) during 24 hours.
• In most adults, at least 3 l of urine output per 24 hours are required.
• To increase cystine solubility, alkalinization should be a cornerstone of treatment for all patients.
• All patients should self-monitor urinary pH to maintain values between 7.5 and 8.