Primary prevention
If gene defects are inherited there is no way to prevent GSD I. Genetic screening should be considered in high-risk populations (e.g., prenatal carrier detection in partners of a known carrier of a GSD I mutation and in some Ashkenazi Jewish populations where carriage rates of GSD Ia are high).[11]
Secondary prevention
Intensive glucose therapy may prevent or postpone many of the metabolic sequelae of the disease.
Distal renal tubular dysfunction is almost universal in adolescents and adults and is associated with acidification defects, hypercalciuria, and hypocitraturia.[38][52] The combination of low urinary citrate and high urinary calcium concentrations predisposes patients to nephrocalcinosis and nephrolithiasis, and citrate supplementation may prevent these complications.[38]
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