Cystinuria
ICD-10 E72.0 · ICD-11 5C60.2

Treatment of Cystinuria in Pregnancy

Pregnancy introduces a distinct risk profile in cystinuria. Both anatomical changes associated with gestation and altered urinary physiology elevate the likelihood of stone formation, requiring a carefully adapted management strategy.

Patients with cystinuria who are pregnant face an increased risk of stone formation driven by anatomical factors and hypercalciuria. Close monitoring and the use of only pregnancy-compatible interventions are essential throughout gestation.
Management is restricted to conservative measures. Certain pharmacological agents routinely used in cystinuria outside of pregnancy are contraindicated in this setting, so the regimen relies on dietary modifications and fluid strategies — the complete protocol details the full set of interventions, monitoring parameters, and adjustment criteria.
Maintain urinary cystine concentration below 250 mg/l and urinary pH within the target therapeutic range, with patient self-monitoring throughout pregnancy.

References

DOI: 10.1016/j.kint.2020.06.035

There is an increased risk of stone formation in general during pregnancy because of anatomical factors and hypercalciuria.

Cystine-binding thiols are contraindicated.

Fluid intake should guarantee a urine output large enough to maintain a cystine concentration of <250 mg/l (1 mmol/l) during 24 hours.

All patients should self-monitor urinary pH to maintain values between 7.5 and 8.

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