Genetics of Glycogen-Storage Disease Type III Medication

Updated: Feb 18, 2019
  • Author: David H Tegay, DO, FACMG; Chief Editor: Maria Descartes, MD  more...
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Medication

Medication Summary

Glucose—oral, enteral, and intravenous forms—is used to manage hypoglycemic episodes. Glucagon administration may have value for managing hypoglycemic episodes. For patients with a concurrent illness, pay particular attention to ensuring an adequate intake of glucose and glucose precursors.

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Monosaccharides

Class Summary

Dextrose is a metabolic substrate and simple sugar.

Dextrose (D-Glucose)

Absorbed rapidly from the small intestine and then distributed to other tissues. Administer parenterally injected dextrose to patients who cannot maintain adequate PO intake or to patients with hypoglycemia who require rapidly increased blood glucose levels. Concentrated dextrose infusions provide large amounts of glucose in a small volume. Paradoxically, rebound hypoglycemia can be produced if hyperinsulinemia is induced by excessively raising serum glucose levels.

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Glucogen-stimulating agent

Class Summary

Pancreatic alpha cells of the islets of Langerhans produce glucagon, a polypeptide hormone, which exerts opposite effects of insulin on blood glucose. Glucagon elevates blood glucose levels by inhibiting glycogen synthesis and by enhancing glucose formation from noncarbohydrate sources such as proteins and fats (ie, gluconeogenesis). The most important role of glucagon in treating GSD III is to stimulate glycogenolysis in the liver.

Glucagon

Polypeptide (single chain) with 29 amino acid residues and a molecular weight of 3483. Acts only on liver glycogen to release glucose via a complex series of reactions involving cAMP, epinephrine, phosphorylase, and phosphorylase kinase. May be useful when IV access is problematic and dextrose cannot be administered.

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