The aim of the study is to investigate insulin sensitivity in patients with an inborn mitochondrial disorder due to a defect in the oxidative phosphorylation (OXPHOS).
ID
Source
Brief title
Condition
- Glucose metabolism disorders (incl diabetes mellitus)
- Inborn errors of metabolism
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The insulin sensitivity index (Mi-value) is used to kwantify insulin
sensitivity. A difference of more than 30% between the patients and the control
group is considered clinically relevant.
Secondary outcome
not applicable
Background summary
Mitochondrial dysfunction is associated with insulin resistance and diabetes
mellitus type 2. If patients with an inborn mitochondrial disorder are at risk
for developing insulin resistance is unknown.
Study objective
The aim of the study is to investigate insulin sensitivity in patients with an
inborn mitochondrial disorder due to a defect in the oxidative phosphorylation
(OXPHOS).
Study design
a case-control study.
Study burden and risks
The duration of the study is 4-5 hours. The patient needs to be sober before
the start of the study. The protocol includes a short questionnaire. Then, two
intravenous accesses are needed for the clamp study for frequent blood analysis
and administration of insulin and glucose. In theory, hypoglycemia can occur.
This risk is minimized since glucose levels are checked very frequently.
Because lactate levels can increase during the clamp study, these levels are
monitored.
postbus 9101
6500 HB Nijmegen
Nederland
postbus 9101
6500 HB Nijmegen
Nederland
Listed location countries
Age
Inclusion criteria
Adult patients with an inborn mitochondrial disorder due to a defect in the oxidative phosphorylation (OXPHOS).
Exclusion criteria
diabetes mellitus, mental retardation or the use of drugs that influence glucose metabolism.
Design
Recruitment
Medical products/devices used
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL29209.091.09 |