Energy Metabolism in Branched-chain Organic Acidemias

April 15, 2019 updated by: German Diabetes Center

Non-invasive Measurement of Energy Metabolism in Patients With Primary and Secondary Mitochondrial Diseases

Energy metabolism and insulin sensitivity were assessed in a case-control study in patients with branched-chain organic acidemias.

Study Overview

Status

Completed

Conditions

Intervention / Treatment

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.

Study Type

Observational

Enrollment (Actual)

57

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • Germany
    • NRW
      • Düsseldorf, NRW, Germany
        • Department of General Pediatrics, Neonatology and Pediatric Cardiology of the University Children's Hospital Düsseldorf
      • Düsseldorf, NRW, Germany
        • Institute for Clinical Diabetology, German Diabetes Center

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

5 years and older (Child, Adult, Older Adult)

Accepts Healthy Volunteers

Yes

Genders Eligible for Study

All

Sampling Method

Probability Sample

Study Population

Patients with known propionic acidemia, methylmalonic acidemia or isovaleric acidemia were enrolled as well as healthy humans of comparable age, sex and BMI

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
Patients
Patients with propionic acidemia, isovaleric acidemia, methylmalonic acidemia
Other: No intervention
No intervention, observational study with metabolic characterisation
Controls
Healthy humans
Other: No intervention
No intervention, observational study with metabolic characterisation

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Hepatic and skeletal muscle energy metabolism
Time Frame: At baseline
Hepatic and skeletal muscle energy metabolism assessed by magnetic resonance spectroscopy
At baseline

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Insulin sensitivity
Time Frame: At baseline
Insulin sensitivity measured from oral glucose tolerance test
At baseline

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

German Diabetes Center

Collaborators

University Children's Hospital, Düsseldorf

Investigators

  • Principal Investigator: Michael Roden, Professeor, German Diabetes Center
  • Principal Investigator: Regina Ensenauer, Professor, Children's Hospital Düsseldorf

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

March 1, 2012

Primary Completion (Actual)

November 1, 2017

Study Completion (Actual)

March 1, 2018

Study Registration Dates

First Submitted

April 9, 2019

First Submitted That Met QC Criteria

April 15, 2019

First Posted (Actual)

April 16, 2019

Study Record Updates

Last Update Posted (Actual)

April 16, 2019

Last Update Submitted That Met QC Criteria

April 15, 2019

Last Verified

April 1, 2019

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • MITO-MRS

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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