Further Outpatient Care

Close nutritional and biochemical genetic follow-up is critical, especially during initial and pubertal growth periods.

Patients should be seen by subspecialists at least every 6 months.

Further Inpatient Care

Conditions that reduce oral (PO) intake, such as in glycogen-storage disease type I (GSD I) require intravenous (IV) glucose to maintain blood sugar and to avoid complications of severe hypoglycemia.

Inpatient & Outpatient Medications

No medications are required for glycogen-storage disease type Ia.

Patients with glycogen-storage disease type Ib require granulocyte colony-stimulating factor (GCSF) on a weekly basis.

Transfer

Consider transferring any patient who is admitted for any reason other than routine IV fluid administration for blood glucose support.

Complications

See the list below:

Severe hypoglycemia, cerebral edema, coma, death
Hepatic adenoma, adenocarcinoma, or both
Glomerular hyperfiltration and glomerulosclerosis
Brain damage
Severe anemia
Growth failure

Prognosis

Patients receiving proper treatment should have a reasonable life span.

Patient Education

Teach parents of infants how to insert a nasogastric (NG) feeding tube.

Teach family members how to test blood glucose levels.

Teach family members and older children how to recognize signs of impending hypoglycemia.

Provide intensive nutritional education to patients so they can assist in their own dietary control as early as possible.

For excellent patient education resources, visit eMedicineHealth's Ear, Nose, and Throat Center. Also, see eMedicineHealth's patient education article Nosebleeds.

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Media Gallery

Microsome is shown in relation to the substrate, glucose-6-phosphate, which has been released from cytosolic glycogen. This substrate is transferred across the microsomal membrane by the protein translocase, where by glucose-6-phosphatase acts on it to release free glucose and inorganic phosphate. Patients with glycogen-storage disease type Ia are genetically deficient in glucose-6-phosphate activity, while those affected with glycogen-storage disease type Ib lack translocase.

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