Leucine-enriched Essential Amino Acid Intake to Optimize Protein Anabolism in Children With Cystic Fibrosis
Information source: Texas A&M University
ClinicalTrials.gov processed this data on August 23, 2015
Link to the current ClinicalTrials.gov record.
Condition(s) targeted: Cystic Fibrosis
Intervention: Essential amino acid intake + Leucine vs total AA supplement (Dietary Supplement)
Sponsored by: Texas A&M University
Official(s) and/or principal investigator(s):
Nicolaas EP Deutz, MD, PhD, Principal Investigator, Affiliation: University of Arkansas
Malnutrition, including muscle wasting commonly occurs in children with cystic fibrosis
(CF), negatively influencing their quality of life and survival. At the time of a diagnosis
of CF, severe protein deficits can already be present. It is important to get CF children
fed adequately to prevent that their condition becomes worse or that recovery takes longer.
Oral supplementation trials showed that gains in lean body mass are difficult to achieve in
CF unless specific metabolic abnormalities are targeted. However, the specific needs for
certain food components are not clear yet in children that are ill. Therefore, more
information is necessary on the need for protein and certain amino acids in children with
CF. Previous studies support the concept of essential amino acids (EAA) as an anabolic
stimulus in the young and elderly and in insulin resistant states. Until yet no information
is present on the anabolic effects of EAA in CF.
It is therefore our hypothesis that a high-leucine essential amino acids mixture
specifically designed to stimulate protein anabolism will target the metabolic alterations
of pediatric subjects with CF. In the present proposal, the acute metabolic effects of this
high leucine essential amino acids mixture will be examined in pediatric subjects with CF
and compared to that of a regular balanced total mixture of essential and non-essential
amino acids. The principal endpoints will be the extent of stimulation of whole body protein
synthesis as this is the principal mechanism by which either amino acid or protein intake
causes muscle anabolism, and the reduction in endogenous protein breakdown. Both endpoints
will be assessed by isotope methodology which is thought to be the reference method.
Official title: Leucine-enriched Essential Amino Acid Intake to Optimize Protein Anabolism in Children With Cystic Fibrosis
Study design: Allocation: Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Single Group Assignment, Masking: Double Blind (Subject, Investigator)
Primary outcome: Net whole body protein synthesis rate
Whole body collagen breakdown rate
Urea turnover rate
Arginine turnover rate
Liver protein synthesis rate
Resting Energy expenditure
Amino acid kinetics
In this study, we will test the following hypothesis: A high-leucine essential amino acid
mixture (dose of 6. 7 g) will stimulate protein anabolism to a greater extent than a standard
balanced mixture of total (essential and non-essential) amino acids in CF pediatric
subjects. The principal endpoints will be the extent of stimulation of protein synthesis
rate and the reduction in endogenous protein breakdown. The current project will provide
information that will enable us to better understand the underlying metabolic mechanisms
that regulate protein metabolism in pediatric subjects with CF.
Minimum age: 10 Years.
Maximum age: 21 Years.
1. Subjects who already have a diagnosis of CF based on universal diagnostic criteria.
2. Age 14 to 21 years at the time of enrollment
3. Under routine medical control at the CF center of ACH
4. Admitted to the ACH for treatment of pulmonary exacerbation of CF disease.
5. Improvement in lung function (FEV1) at the time of enrollment back to baseline values
(as determined in the clinically stable pre-hospital period)
6. Central or peripheral venous line in place
7. No planned major changes or interventions in the treatment and care of the pediatric
subject on Day - 2 and -1 before discharge from the hospital.
1. Established diagnosis of Diabetes Mellitus
2. Presence of fever within the last 3 days
3. Unstable metabolic diseases including liver (cirrhosis) or renal disease
4. Chronic respiratory failure with cor pulmonale
5. Use of long-term oral corticosteroids or short course of oral corticosteroids in the
preceding month before enrollment
6. Any other condition according to the principle investigator or study physician would
interfere with collecting study samples
7. Failure to give assent / informed consent
Locations and Contacts
University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205, United States
Starting date: July 2008
Last updated: August 7, 2015