Related Experiment Videos
Impaired glucose effectiveness in chronic progressive external ophthalmoplegia
Regine Becker1, Heiner Laube, Heiner Laube
1Department of Internal Medicine, University of Giessen, Giessen, Germany.
Metabolism: Clinical and Experimental
|May 31, 2002
Summary
Mitochondrial disease patients with chronic progressive external ophthalmoplegia (CPEO) have a higher incidence of diabetes. Impaired glucose effectiveness is a key factor in developing diabetes in these patients.
Area of Science:
- Endocrinology
- Metabolic Disorders
- Mitochondrial Medicine
Background:
- Mitochondrial gene mutations are linked to diabetes mellitus.
- Patients with mitochondrial diseases like chronic progressive external ophthalmoplegia (CPEO) exhibit a higher prevalence of diabetes.
Purpose of the Study:
- To investigate insulin sensitivity (SI), insulin secretion (AIR(Glucose)), and glucose effectiveness (Sg) in CPEO patients.
- To understand the role of metabolic dysfunction in the pathogenesis of diabetes in CPEO.
Main Methods:
- Utilized a modified intravenous glucose tolerance test (IVGTT) with Bergman's minimal model.
- Studied six unrelated patients with CPEO and six matched healthy controls.
Main Results:
- Three CPEO patients showed impaired glucose tolerance (IGT); one had diabetes mellitus.
- No significant differences in SI or AIR(Glucose) between CPEO patients and controls.
- CPEO patients demonstrated significantly impaired glucose effectiveness (Sg) (P =.016).
Conclusions:
- CPEO is associated with a high incidence of IGT and diabetes mellitus.
- Impaired glucose effectiveness is a significant factor in the early development of diabetes in CPEO.
- Overt diabetes in CPEO results from insulin resistance, impaired insulin secretion, and reduced glucose effectiveness.