Genetics of von Gierke Disease (Glycogen-Storage Disease Type 1) Follow-up

Updated: Aug 10, 2017
  • Author: Karl S Roth, MD; Chief Editor: Maria Descartes, MD  more...
  • Print
Follow-up

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.

Next:

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.

Previous
Next:

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.

Previous
Next:

Transfer

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

Previous
Next:

Complications

See the list below:

  • Severe hypoglycemia, cerebral edema, coma, death

  • Hepatic adenoma, adenocarcinoma, or both

  • Glomerular hyperfiltration and glomerulosclerosis

  • Brain damage

  • Severe anemia

Previous
Next:

Prognosis

Patients receiving proper treatment should have a reasonable life span.

Previous
Next:

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.

Previous