Glycogen storage disease type I medical therapy

Glycogen storage disease type I Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Glycogen storage disease type I from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X Ray
CT
MRI
Ultrasound
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Glycogen storage disease type I medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Glycogen storage disease type I medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Glycogen storage disease type I medical therapy
CDC on Glycogen storage disease type I medical therapy
Glycogen storage disease type I medical therapy in the news
Blogs on Glycogen storage disease type I medical therapy
Directions to Hospitals Treating Glycogen storage disease type I
Risk calculators and risk factors for Glycogen storage disease type I medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Medical Therapy

The medical management of GSD is divided into nutritional therapy and medical management of systemic complications.

• The primary concern in infants and young children with GSD type 1 is hypoglycemia.
• So, the first line treatment for GSD type 1 is the prevention of hypoglycemia.
• Small frequent feeds high in complex carbohydrates (preferably those high in fiber) are distributed evenly throughout 24 hours for the prevention of hypoglycemia.
• A metabolic dietician should be consulted once a case of GSD type 1 is diagnosed.
• Distribution of calories:
  • Calories from carbohydrate: 60-70%
  • Calories from protein: 10-15%
  • Calories from fats: Remaining calories (<30% for children older than 2 years)
• Sucrose (fructose and glucose) and lactose (galactose and glucose) may be limited or avoided.

Nutritional Therapy

• 1. Infants
  • 1.1 Formula and enteral feedings
    • 1.1.1 Infant sleep <3-4 hours
      • Preferred treatment (1): Soy-based formula, fed on demand q2h – q3h
      • Preferred treatment (2): Sugar-free formula, fed on demand q2h – q3h
      • Preferred treatment (3): A formula that is free of sucrose, fructose, and lactose; fed on demand q2h – q3h
    • 1.1.2 Infant sleep >3-4 hours
      • Preferred treatment (1): Overnight gastric feedings (OGFs)
      • Preferred treatment (2): Wake up infant q3h - q4h; monitor blood glucose and offer feeding

      Note (1): As hypoglycemia in GSD type 1 can be life-threatening and may cause seizures, permanent brain damage and even death, training of the parents (and/or child, when older) in inserting a nasogastric (NG) tube or that a G-tube be surgically placed so that there is always access to treat for hypoglycemia, especially during times of illness or refusal to eat is recommended.

      Note (2): A G-tube may not be a good option in patients with GSD type 1b and neutropenia as it increases the risk of recurrent infections at the surgical site. Granulocyte colony-stimulating factor (G-CSF) (Neupogen) is should be administered before placing a G-tube if the child has neutropenia.