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Safety Issues Common With Dietary Management of Glycogen Storage Diseases

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Patients with hepatic glycogen storage diseases (GSDs) lack an enzyme critical to the breakdown, synthesis, or transport of glycogen. The disease is typically managed with a combination of dietary measures (ie, frequent consumption of cornstarch and meals and continuous nocturnal gastric drip feeding). A multinational team of investigators surveyed 249 patients with GSD (median age, 14.8 years) or their caregivers about complications of inadequate dietary management. Approximately half of the patients had at least 1 acute complication, and a total of 425 complications were reported. The most common were hypoglycemia, hospital admission, and drowsiness. Some patients experienced seizures or comas. Complications more often occurred at night. The authors concluded better guidance and more resources for patients were needed.

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Steunenberg TAH, Peeks F, Hoogeveen IJ, et al. Safety issues associated with dietary management in patients with hepatic glycogen storage disease. Mol Genet Metab. 2018 Jul 18. pii: S1096-7192(18)30240-3. doi: 10.1016/j.ymgme.2018.07.004.

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