Glycogen-Storage Disease Type 0 (GSD-0) (Glycogen Synthetase Deficiency) Follow-up

Updated: May 03, 2017
  • Author: Reem Saadeh-Haddad, MD; Chief Editor: Luis O Rohena, MD, MS, FAAP, FACMG  more...
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Follow-up

Further Outpatient Care

Conduct a follow-up evaluation to assess for adequate physical growth, developmental maturation, and avoidance of hypoglycemic episodes, with adjustments in dietary management as needed.

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Deterrence/Prevention

Avoid prolonged fasting of greater than 5-7 hours. Some patients cannot tolerate even a shorter fasting period of less than 5 hours. During an acute illness with decreased oral intake, maintain normoglycemia with intravenous infusion of glucose-containing solution.

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Prognosis

The prognosis is good for normal growth and intellectual development when the condition is diagnosed early and when episodes of hypoglycemia are prevented with good dietary management.

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Patient Education

Educate the patient and parents about proper diet management and avoidance of fasting. Educate the parents and primary physician about the administration of intravenous glucose solutions during acute illness with decreased oral intake. Dietary teaching is suggested for children as soon as they are developmentally ready. Educate the patient and the family about the autosomal-recessive inheritance of this condition and recurrent risk within the same family, as well as extended relatives.

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