Genetics of Glycogen Storage Disease Type VI (Hers Disease) Treatment & Management

Updated: Jun 13, 2018
  • Author: Anna V Blenda, PhD; Chief Editor: Maria Descartes, MD  more...
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Treatment

Consultations

Patients with glycogen storage disease type VI (GSD VI), also known as Hers disease, should be referred to a dietitian experienced with glycogen storage diseases (GSDs) and the management of disorders associated with an increased risk of hypoglycemic episodes.

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Diet

Dietary management is the only form of treatment necessary for this rather mild glycogen storage disease. Avoiding prolonged fasting and eating frequent meals are recommended. [16] A high-protein diet is recommended for patients with GSD VI who exhibit fasting hypoglycemia. [4, 17]

A small 2015 study reported the use of extended-release cornstarch to maintain normal glucose levels during overnight fasting in patients with different types of GSDs, including GSD VI. It was found to be beneficial in some patients. [18]

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Activity

Activity should not be restricted unless significant hepatomegaly is present; recommend that patients with significant hepatomegaly avoid contact sports and activities.

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Prevention

Instruct patients who exhibit episodes of fasting hypoglycemia to avoid prolonged fasts (in excess of 5-7 hours). During an acute illness with decreased oral intake, maintain normoglycemia with intravenous infusion of glucose-containing solution.

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Long-Term Monitoring

Perform follow-up evaluation to assess physical growth and to prevent hypoglycemic episodes by adjusting diet, as needed.

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