Moderate Intensity Exercise and Phenylketonuria

NCT01904708 | Terminated

• 1 other identifier: 9202
• Study Type: interventional
• Target: 2
• Locations: 1 country
• Sites: 1

Brief Summary

Phenylketonuria (PKU), an inherited genetic disorder, can cause irreversible brain damage, declined executive function, and autistic tendencies unless a phenylalanine (Phe) restricted diet is consistently maintained throughout life. Promoting anabolism, the uptake of free amino acids from the extracellular space, is a key component to maintaining plasma phenylalanine concentrations within treatment range among patients with PKU. Exercise promotes muscle protein synthesis and anabolism, but the effect on blood phenylalanine concentrations in patients with PKU has not been reported. Our objective is to assess the impact of an acute bout of moderate intensity exercise on protein oxidation and plasma amino acid concentrations, as a potential adjunctive therapy for patients with PKU. The investigators hypothesize that moderate intensity exercise decreases amino acid oxidation, increases muscle protein synthesis, and promotes tissue uptake of essential amino acids, thereby lowering plasma phenylalanine concentrations in patients with Phenylketonuria.

Conditions

Phenylketonuria; PKU

Keywords

Phenylketonuria; PKU; Exercise; Muscle Protein Synthesis; Plasma Phenylalanine concentration

Outcome Measures

Primary Outcomes (1)

• Compare plasma phenylalanine concentration of final blood draw during exercise versus sedentary visits.

  Compare the final blood sample for phenylalanine concentration from each arm of the study.

  up to 8 hours

Secondary Outcomes (1)

• Breath Enrichment of C13-Lysine during sedentary and exercise

  Hours 1-8 of the study day from each arm.

Study Arms (2)

Sedentary Visit

NO INTERVENTION

Subjects will do quiet, sedentary activities for 8 hours. Hourly blood draws and breath sampling will be collected. Bolus C13-Lysine will be given at hour 4.

Exercise Visit

ACTIVE COMPARATOR

Subjects will do quiet, sedentary activities until hour 5. Blood and breath samples will be collected. At hour 4, subject will consume a bolus of C13-Lysine and immediately walk on a treadmill at a moderate intensity (exercise at 75% of max heart rate) for 45 minutes.

Other: Acute Moderate Intensity Exercise

Interventions

Acute Moderate Intensity Exercise OTHER

Moderate intensity exercise by walking on a treadmill at 75% of max heart rate.

Exercise Visit

Eligibility Criteria

• Age: 14 Years - 17 Years
• Sex: male
• Healthy Volunteers: No
• Age Groups: Child (0-17)

You may qualify if:

• plasma phenylalanine concentration of \>1200 micromols at diagnosis
• treated by dietary phenylalanine restriction from early infancy
• willing to participate in this study
• stable BMI of 20-25 for the past 3 months
• currently in average to good physical fitness condition
• physically active 3-4 days per week

You may not qualify if:

• previously or are currently taking approved or experimental pharmacologic treatments (i.e.: Biopterin (BH4), sapropterin dihydrochloride (Kuvan), Large Neutral Amino Acids (LNAA), pegylated phenylalanine ammonia lyase (PEG-PAL))
• recent history of weight loss
• endocrine disorder
• pregnant
• anemic
• involved in any other study or research protocol
• participation in a current strength training or performance training regimen

Sponsors & Collaborators

• Oregon Health and Science University: lead

Study Sites (1)

Oregon Health and Science University

Portland, Oregon, 97239, United States

Location

MeSH Terms

Conditions

Phenylketonurias; Motor Activity

Condition Hierarchy (Ancestors)

Brain Diseases, Metabolic, Inborn; Brain Diseases, Metabolic; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Amino Acid Metabolism, Inborn Errors; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Metabolic Diseases; Nutritional and Metabolic Diseases; Behavior

Study Officials

• Melanie Gillingham, PhD, RD

  Oregon Health and Science University

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: RANDOMIZED
• Masking: NONE
• Purpose: BASIC SCIENCE
• Intervention Model: CROSSOVER
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Assistant Professor

Study Record Dates

• First Submitted: May 31, 2013
• First Posted: July 22, 2013
• Study Start: May 1, 2013
• Primary Completion: June 1, 2014
• Study Completion: June 1, 2014
• Last Updated: October 16, 2014

Record last verified: 2014-10

Locations

US (1)