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[Glycogenosis type I]

C V Reis1, F J Penna, M C Oliveira

  • 1Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.

Jornal De Pediatria
|December 20, 2003
PubMed
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Glycogen storage disease type I (GSD-type I) is a rare metabolic disorder causing hypoglycemia. Early diagnosis and dietary management, like uncooked cornstarch, significantly improve prognosis and alter the clinical course in infants.

Area of Science:

  • Biochemistry
  • Pediatrics
  • Metabolic Disorders

Background:

  • Glycogen storage disease type I (GSD-type I) results from glucose-6-phosphatase deficiency.
  • This leads to glycogen accumulation and metabolic disturbances, primarily hypoglycemia.

Purpose of the Study:

  • To update knowledge on GSD-type I for pediatricians.
  • To guide precocious diagnosis and management of this metabolic disturbance.

Main Methods:

  • Systematic review of significant articles from the last 20 years via Medline.
  • Focus on dietary treatment strategies for GSD-type I.

Main Results:

  • Discusses glycogen metabolism and GSD-type I consequences, including hypoglycemia, hyperuricemia, and hyperlipidemia.

Related Experiment Videos

  • Highlights uncooked cornstarch and enteral carbohydrate infusion for normoglycemia.
  • Addresses treatment of associated disorders, infections, and hepatic transplant indications.

    • Conclusions:

    • GSD-type I is a rare but significant cause of hepatomegaly and hypoglycemia in infants.
    • Dietary treatment has dramatically improved the clinical course and prognosis.
    • Pediatricians must be familiar with diagnosis and rigorous dietary control for optimal outcomes.