Oral Ketones and Exercise Among Patients With Long-chain Fatty Acid Oxidation Disorders

Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency Clinical Trial

Official title:

Safety and Tolerability of Oral Ketones and Exercise Among Patients With Long-chain Fatty Acid Oxidation Disorders

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT05411835
• Other study ID #: 23859
• Status: Completed
• Phase: Early Phase 1
• Start date: August 1, 2022
• Est. completion date: January 22, 2024

Study information

• Verified date: October 2022
• Source: Oregon Health and Science University
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

The purpose of the study is to determine if an oral ketone beverage is safe and well-tolerated during moderate intensity exercise in participants with long-chain fatty acid oxidation disorders and if it will raise blood ketones to levels similar to that reported among normal healthy subjects.

Description:

Purpose: Subjects with long-chain fatty acid oxidation disorders (LC-FAOD) do not make ketones during fasting or with exercise. Ketones are an important alternative energy substrate during moderate exercise, sparing the oxidation of glucose and providing a source of ATP to the central nervous system and exercising muscle. Fatty acid oxidation in the liver is required to make ketones. Subjects with a LC-FAOD cannot generate ketones because of their block in fatty acid oxidation during exercise. Providing ketones in an oral ketone beverage may increase blood ketones with exercise to levels normally observed in humans. Aim: To determine the safety and tolerability of an oral ketone beverage during moderate intensity exercise among subjects with a LC-FAOD compared to an isocaloric maltodextrin beverage, and to determine blood ketone concentrations. Hypothesis: Oral consumption of a ketone beverage before moderate intensity exercise will be safe and well-tolerated, and will raise blood ketones among subjects with a LC-FAOD to concentrations similar to that reported in the literature among normal healthy subjects.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 5
• Est. completion date: January 22, 2024
• Est. primary completion date: January 31, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion Criteria:

• confirmed diagnosis of VLCAD, LCHAD/TFP or CPT2 deficiency
• speak English
• willing to complete 2 moderate intensity exercise treadmills

Exclusion Criteria:

• subjects actively participating in another research study that prohibits their participation
• pregnant females
• subjects with diabetes or taking medications to treat diabetes

Related Conditions & MeSH terms

• Long-chain 3-hydroxyacyl-CoA Dehydrogenase Deficiency
• Protein Deficiency
• Trifunctional Protein Deficiency
• Very Long Chain Acyl Coa Dehydrogenase Deficiency

Intervention

Dietary Supplement:
• Nutritional Ketone Supplement
Mix of sodium, calcium, and magnesium salts of D-beta-hydroxybutyrate with nicotinamide riboside chloride, flavors and stevia sweetener
• Isocaloric Placebo Supplement
Maltodextrin with flavors and stevia sweetener

Locations

Country	Name	City	State
United States	Oregon Health & Science University	Portland	Oregon

Sponsors (1)

Lead Sponsor	Collaborator
Oregon Health and Science University

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Adverse Events- incidence	Number of Adverse events	2 hours after product intake
Primary	Adverse Events- type	Description of Adverse events: categorical	2 hours after product intake
Primary	Adverse Events- severity	Severity of adverse event: grade 1-5	2 hours after product intake
Primary	Adverse Events- causal relationship	Yes/No related to product intake	2 hours after product intake
Primary	Abdominal discomfort	visual analogue scale (VAS) 0-10	2 hours after product intake
Primary	Decreased appetite	visual analogue scale (VAS) 0-10	2 hours after product intake
Primary	Gastric reflux	visual analogue scale (VAS) 0-10	2 hours after product intake
Primary	Nausea	visual analogue scale (VAS) 0-10	2 hours after product intake
Primary	Diarrhea	visual analogue scale (VAS) 0-10	2 hours after product intake
Primary	Headache	visual analogue scale (VAS) 0-10	2 hours after product intake
Secondary	Blood Ketones	plasma beta-hydroxybutyrate concentration	20 minutes after moderate intensity exercise
Secondary	Blood Creatine Kinase	plasma CK concentration	20 minutes after moderate intensity exercise
Secondary	Blood Glucose	serum glucose	20 minutes after moderate intensity exercise
Secondary	Blood Lactate	serum lactate concentration	20 minutes after moderate intensity exercise
Secondary	Blood Pressure	diastolic BP	peak 40 minute exercise
Secondary	Blood Pressure	systolic BP	peak 40 minute exercise
Secondary	Perceived exertion	Borg scale 1-20	peak 40 minute exercise
Secondary	Respiratory Exchange Ratio	VCO2/VO2 0.7 - 1.0	peak 40 minute exercise
Secondary	Heart Rate	beats per minute	peak 40 minute exercise