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Acute Nutritional Ketosis in VLCAD Deficiency

NCT03531554 · Status: COMPLETED · Phase: NA · Type: INTERVENTIONAL · Enrollment: 5

Last updated 2018-05-21

No results posted yet for this study

Summary

To test if a ketone-ester based drink can boost muscle mitochondrial function in vivo in patients with VLCADD in order to establish a rational basis for therapeutic use in this disorder.

Conditions

  • VLCAD Deficiency
  • Fatty 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

Sponsors & Collaborators

  • UMC Utrecht

    collaborator OTHER

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

    collaborator OTHER

  • University of Oxford

    collaborator OTHER

  • ESN (Erfelijke Stofwisselingsziekten Nederland)

    collaborator UNKNOWN

  • University Medical Center Groningen

    lead OTHER

Principal Investigators

  • Jeroen AL Jeneson, PhD · Dept of Neuroscience/ Neuroimaging Center Groningen

Study Design

Allocation
RANDOMIZED
Purpose
OTHER
Masking
DOUBLE
Model
CROSSOVER

Eligibility

Min Age
16 Years
Max Age
65 Years
Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2016-04-01
Primary Completion
2017-03-31
Completion
2017-04-01

Countries

  • Netherlands

Study Locations

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Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT03531554 on ClinicalTrials.gov