Prevention of complications in glycogen storage disease type Ia with optimization of metabolic control

Pediatr Diabetes. 2017 Aug;18(5):327-331. doi: 10.1111/pedi.12540. Epub 2017 Jun 1.

Abstract

Prior to 1971, type Ia glycogen storage disease was marked by life-threatening hypoglycemia, lactic acidosis, severe failure to thrive, and developmental delay. With the introduction of continuous feeds in the 1970s and cornstarch in the 1980s, the prognosis improved, but complications almost universally developed. Changes in the management of type Ia glycogen storage disease have resulted in improved metabolic control, and this manuscript reviews the increasing evidence that complications can be delayed or prevented with optimal metabolic control as previously was seen in diabetes.

Keywords: GSD; adenomas; hypoglycemia; metabolic control.

Publication types

  • Review

MeSH terms

  • Adenoma / complications
  • Adenoma / prevention & control
  • Adult
  • Child
  • Combined Modality Therapy
  • Glycogen Storage Disease Type I / blood
  • Glycogen Storage Disease Type I / complications
  • Glycogen Storage Disease Type I / diagnosis
  • Glycogen Storage Disease Type I / therapy*
  • Humans
  • Hypoglycemia / complications
  • Hypoglycemia / prevention & control
  • Liver Neoplasms / complications
  • Liver Neoplasms / prevention & control
  • Nephrocalcinosis / complications
  • Nephrocalcinosis / prevention & control
  • Nephrolithiasis / complications
  • Nephrolithiasis / prevention & control
  • Osteoporosis / complications
  • Osteoporosis / prevention & control
  • Precision Medicine*
  • Prognosis
  • Renal Insufficiency / complications
  • Renal Insufficiency / prevention & control*

Supplementary concepts

  • Hepatorenal form of glycogen storage disease