علاج داء تخزين الجليكوجين النوع الأول مع نقص سيترات البول وحصيات الكلى أو تكلس الكلى

References

DOI: 10.1038/gim.2014.128

• With hypocitraturia, citrate supplementation should be considered, especially if there is concomitant hypercalciuria or a history of nephrolithiasis or nephrocalcinosis.
• Oral citrate supplementation will augment citrate excretion, favorably altering the urinary milieu to decrease the chances of urinary calcium precipitation and, as a result, is likely very beneficial in GSD I patients with low urinary citrate levels.
• In individuals with normal renal function, potassium citrate is preferred over sodium citrate because higher sodium intake is linked to greater urinary calcium excretion.
• With citrate supplementation, the aim is to achieve at least 300 mg/g creatinine on spot urine ratios.