Acute Nutritional Ketosis in VLCAD Deficiency

NACompletedINTERVENTIONAL
Enrollment

5

Participants

Timeline

Start Date

April 1, 2016

Primary Completion Date

March 31, 2017

Study Completion Date

April 1, 2017

Conditions
VLCAD DeficiencyFatty Acid Oxidation Defects
Interventions
DIETARY_SUPPLEMENT

ketone ester drink

395 mg of ketone ester/kg

BEHAVIORAL

exercise

35 min cycling test on an upright bicycle, followed by 10 minutes of supine cycling inside a MR scanner.

PROCEDURE

muscle biopsy

biopsy from the quadriceps muscle prior to and immediately after upright bicycling

DIAGNOSTIC_TEST

Magnetic Resonance Imaging

1H MR images and 31P MR spectra were acquired from the upper leg prior to-, during and after exercise

Trial Locations (2)

1105 AZ

Academic Medical Center, Amsterdam

9700RB

Dept of Neuroscience/ Neuroimaging Center, Groningen

All Listed Sponsors
collaborator

UMC Utrecht

OTHER

collaborator

Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)

OTHER

collaborator

University of Oxford

OTHER

collaborator

ESN (Erfelijke Stofwisselingsziekten Nederland)

UNKNOWN

lead

University Medical Center Groningen

OTHER

NCT03531554 - Acute Nutritional Ketosis in VLCAD Deficiency | Biotech Hunter | Biotech Hunter