GLP Analogs for Diabetes in Wolfram Syndrome Patients
Status:
Unknown status
Trial end date:
1969-12-31
Target enrollment:
Participant gender:
Summary
Wolfram syndrome, also referred to as DIDMOAD (diabetes insipidus, diabetes mellitus, optic
atrophy and deafness) is a genetic syndrome characterized by beta-cell dysfunction and
apoptosis leading to diabetes, neurodegeneration and psychiatric illness. Accumulating
evidence indicates that beta-cell failure and neuronal cell dysfunction in Wolfram's syndrome
results from a high level of ER stress in affected cells. The current treatment of Wolfram
syndrome is insulin, which fails to prevent the progression of beta-cell failure.
Several studies showed that GLP-1 analogs are very effective in protecting beta-cells from ER
stress. Herein, the investigators suggest studying the impact of GLP-1 analogs in the
treatment of patients with Wolfram syndrome.
The investigators will Study the effects of GLP-1 analog (Exanatide) on beta-cell function
and glycemic control of patients with Wolfram syndrome. Evaluation of beta cell function will
be done by performing meal test and IVGTT test before starting GLP-1 therapy, and after 3
month of treatment.