Fat and Glucose Metabolism in Fed and Fasted State in Patients With Low Skeletal Muscle Mass

NCT03970135 | Completed

• 1 other identifier: H-16049377
• Study Type: interventional
• Target: 13
• Locations: 1 country
• Sites: 1

Brief Summary

In a study from 2003 the investigators showed that adult patients with very low skeletal muscle mass (spinal muscular atrophy (SMA) type II, Duchenne muscular dystrophy, congenital muscular dystrophy) are prone to develop hypoglycemia during prolonged fasting. Since then case reports have described the same phenomenon with hypoglycemia and metabolic crises in children with low skeletal muscle mass provoked by infection, fasting and surgery. Pathophysiological mechanisms of metabolism have never been investigated in adults or children with SMA II. Thus the investigators studied fat and glucose metabolism during prolonged fasting in patients with SMA II and LAMA 2 and compared results to those found in healthy controls.

Conditions

Spinal Muscular Atrophy; Merosin Deficient Congenital Muscular Dystrophy

Outcome Measures

Primary Outcomes (2)

• Change in fat metabolisms from fed to fasted state

  Using indirect calorimetri and stable isotope technique: of \[U-13C\]-palmitate (0.0026 mg kg-1 min-1, primed by a 0.085 mg kg-1 NaH13CO3 bolus) fat metabolism was measured at fed state and during 24 hours of fasting

  24 hours

• Change in carbohydrates metabolisms from fed to fasted state

  Using indirect calorimetri and stable isotope technique: of \[D2\]-glucose (0.0728 mg kg-1 min-1, primed by a 3.203 mg kg-1 D2- glucose bolus) glucose metabolism was measured at fed state and during 24 hours of fasting

  24 hours

Secondary Outcomes (11)

• Change in insulin, glucagon, epinephrine and norepinephrine and the metabolites palmitate, free fatty acids (FFA), glycerol, glucose, pyruvate, β-hydroxybuturate, acetoacetate from fed to fasted state.

  24 hours

• Change in glucagon from fed to fasted state.

  24 hours

• Change in epinephrine from fed to fasted state.

  24 hours

• Change in norepinephrine from fed to fasted state.

  24 hours

• Change in palmitate from fed to fasted state.

  24 hours

Study Arms (1)

Fasting

EXPERIMENTAL

Other: Fasting

Interventions

Fasting OTHER

Fasting for 24 hours or until hypoglycemia (blood glucose \< 3mmol/L) or symptoms of hypoglycemia

Fasting

Eligibility Criteria

• Age: 1 Year - 80 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Patients with low skeletal muscle mass

You may not qualify if:

• Competing disorders interfering with interpretation of results
• Medication that will interfere with results
• Compliance problems
• Participation in other clinical trials that will interfere with interpretation of results
• Pregnancy or breastfeeding

Sponsors & Collaborators

• Rigshospitalet, Denmark: lead

Study Sites (1)

Copenhagen Neuromuscular Center

Copenhagen, 2200, Denmark

Location

MeSH Terms

Conditions

Muscular Atrophy, Spinal; Muscular dystrophy congenital, merosin negative

Interventions

Angptl4 protein, mouse

Condition Hierarchy (Ancestors)

Spinal Cord Diseases; Central Nervous System Diseases; Nervous System Diseases; Motor Neuron Disease; Neurodegenerative Diseases; Neuromuscular Diseases

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: PI

Study Record Dates

• First Submitted: May 21, 2019
• First Posted: May 31, 2019
• Study Start: May 29, 2017
• Primary Completion: November 30, 2017
• Study Completion: November 30, 2017
• Last Updated: May 31, 2019

Record last verified: 2019-05

Data Sharing

• IPD Sharing: Will not share

Locations

DK (1)