patients with familial hypobetalipoproteinemia and matched controls
Conditions
Interventions
Subjects will be studied on 2 occasions:
- after an overnight fast glucose production, disposal and oxidation will be measured using [6,6-2H2]glucose and indirect calorimetry. Lipolysis will be meas
Sponsors
-
Eligibility
Inclusion criteria
Inclusion criteria: 1. Subjects with documented FHBL, who have liver steatosis (FHBL group). Healthy subjects (control group), exactly matched for age, sex, body mass index, waist circumference and physical activity 2. Male subjects 3. Age > 18 years 4. Body Mass Index 20-35 kg/m2 5. No participation in other medical intervention studies in the last three months 6. Able to communicate well with the investigator and to comply with the requirements of the study 7. Written informed consent.
Exclusion criteria
Exclusion criteria: 1. Known any somatic illness, including neoplasm, metabolic or endocrine disorder, neurologic disorder, active infection, or recent surgical procedures within 3 months of study initiation. 2. Use of medication, which can influence glucose or FFA metabolism (insuline, anabolic steroids, growth hormone, testosteron, DHEA, statines, ACE-inhibitors, AII-antagonists, aspirin) 3. Presence of FHBL linked to chromosome 3p21 (since they have no liver steatosis) 4. History of recreational drug use within the last 30 days, or regular consumption of greater than three units of alcohol per day 5. Diabetes mellitus 6. Seropositive for HbsAg, HbcAg, HCV, HAV or HIV 7. Having a pacemaker or other metal device in the body 8. Claustrophobia 9. Regular exercise above sedentary level.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| To determine in detail: 1) Quantity of liver- and muscle triglycerides 2) Body fat distribution 3) hepatic insulin sensitivity 4) peripheral insulin sensitivity 5) intramyocellular differences regarding fatty acid handling of drug-free subjects with FHBL as compared to healthy controls matched for age, sex, body mass index, waist circumference and physical activity. | — |
Secondary
| Measure | Time frame |
|---|---|
| Adiponectin levels and levels of different adiponectin forms in plasma. | — |
Contacts
Public ContactN.M. Lammers
Academic Medical Centre (AMC) Department of Endocrinology & Metabolism F5-162
Outcome results
None listed