Energy Metabolism, Insulin Resistance, Metabolic Syndrome
Conditions
Keywords
Organic acidemias, Mitochondria, Oxidative stress, Fatty liver, Dyslipidemia
Brief summary
Energy metabolism and insulin sensitivity were assessed in a case-control study in patients with branched-chain organic acidemias.
Detailed description
Children and adults with branched-chain organic acidemias and matched healthy humans underwent deep metabolic phenotyping using non-invasive magnetic resonance spectroscopy, oral glucose tolerance tests and measurements of leucocyte respirometry in order to assess changes in energy metabolism and insulin sensitivity.
Interventions
OTHERNo intervention
No intervention, observational study with metabolic characterisation
Sponsors
University Children's Hospital, Düsseldorf
German Diabetes Center
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
5 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* known branched-chain organic acidemia diagnosed by enzymatic or genetic test * age\>5 years
Exclusion criteria
* acute inflammatory conditions with fever, cold symptoms, gastro-intestinal infection within the last 24 hours * contraindications for an oral glucose tolerance test such as fasting blood glucose \>126 mg/dl, severe chronic gastrointestinal conditions, allergy against black currant * contraindications for magnetic resonance tomography (MRT) such as claustrophobia, metallic implants which are not MRT compatible
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Hepatic and skeletal muscle energy metabolism | At baseline | Hepatic and skeletal muscle energy metabolism assessed by magnetic resonance spectroscopy |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Insulin sensitivity | At baseline | Insulin sensitivity measured from oral glucose tolerance test |
Countries
Germany
Outcome results
None listed