Effects of Physical Training on Bone and Muscle Quality, Muscle Strength, and Motor Coordination in Children With NF1

This study is ongoing, but not recruiting participants.
Sponsor:
Collaborators:
Thrasher Research Fund
University of Utah
Information provided by (Responsible Party):
David Stevenson, MD, Shriners Hospitals for Children
ClinicalTrials.gov Identifier:
NCT01058330
First received: January 26, 2010
Last updated: February 25, 2013
Last verified: February 2013
  Purpose

A physical training program will improve quality of life, participation in physical activity, motor coordination, muscle strength, and bone and muscle strength in children with neurofibromatosis type 1.


Condition Intervention
Neurofibromatosis Type 1
Other: Plyometric training program

Study Type: Interventional
Study Design: Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Open Label
Primary Purpose: Prevention
Official Title: Effects of Physical Training on Bone and Muscle Quality, Muscle Strength, and Motor Coordination in Children With Neurofibromatosis Type 1

Resource links provided by NLM:


Further study details as provided by Shriners Hospitals for Children:

Primary Outcome Measures:
  • Bone & muscle quality, DXA, pQCT, & bone ultrasound. [ Time Frame: 1 year ] [ Designated as safety issue: No ]

Secondary Outcome Measures:
  • Motor proficiency BOT-2. Muscle strength force plate & dynamometer. Quality of life questionnaires. [ Time Frame: 1 year ] [ Designated as safety issue: No ]

Estimated Enrollment: 100
Study Start Date: February 2010
Estimated Study Completion Date: February 2014
Estimated Primary Completion Date: February 2014 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Active Comparator: Plyometric physical training
Individualized plyometric training program to increase strength, coordination, and bone density.
Other: Plyometric training program
The intervention is a year long individualized plyometric exercise training program. Examples of plyometric activities include jumping, hopping, running, and throwing. The number of plyometric exercises will increase gradually to prevent over training to a total of 5 lower extremity exercises and 5 upper extremity exercises
No Intervention: Control Group
This group will have no intervention

Detailed Description:

Disorders of the Ras pathway have significant phenotypic overlap and include Noonan syndrome, CFC syndrome, Legius syndrome, Costello syndrome and neurofibromatosis type 1 (NF1). NF1 is one of the most common genetic disorders presenting in childhood with an incidence of 1/3000. NF1 is associated with skeletal abnormalities such as short stature, scoliosis, and long bone fracture with non-union. We recently reported that children with NF1 have abnormalities of bone and muscle architecture as evidenced by decreased bone mineral density, decreased bone strength, and low muscle mass, all of which may predispose them to fractures and scoliosis (Stevenson et al., 2005, 2007, 2009). Our preliminary data show that children with NF1 have poor motor coordination and muscle strength, potentially secondary to abnormal neuromotor learning. We hypothesize that poor motor coordination and decreased muscle strength contribute to the osteopenia in NF1. Our objective is to identify effective and non-invasive strategies to improve motor coordination, muscle strength, and bone and muscle architecture in children with disorders of the Ras pathway, in hopes of decreasing fractures and improving physical activity levels. Plyometric physical training consists of quick, high-intensity, weight-bearing movements, and is an encouraging intervention for use in these children.

  Eligibility

Ages Eligible for Study:   4 Years to 19 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • Fulfill NIH clinical diagnostic criteria

Exclusion Criteria:

  • Visual impairment
  • Participation in a simultaneous medical intervention trial
  • Orthopedic procedure within the last 6 months.
  • Pregnancy
  • Home location greater than 3-4 hours drive time from Shriners Hospital
  • Tibial pseudarthrosis
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01058330

Locations
United States, Utah
Shriners Hospitals for Children, Salt Lake City
Salt Lake City, Utah, United States, 84103
University of Utah
Salt Lake City, Utah, United States, 84132
Sponsors and Collaborators
Shriners Hospitals for Children
Thrasher Research Fund
University of Utah
Investigators
Principal Investigator: David Stevenson, MD Shriners Hospitals for Children
  More Information

No publications provided

Responsible Party: David Stevenson, MD, Assistant Professor, Division of Medical Genetics, University of Utah, Shriners Hospitals for Children
ClinicalTrials.gov Identifier: NCT01058330     History of Changes
Other Study ID Numbers: SLC00038711
Study First Received: January 26, 2010
Last Updated: February 25, 2013
Health Authority: United States: Institutional Review Board

Keywords provided by Shriners Hospitals for Children:
Neurofibromatosis type 1
NF1

Additional relevant MeSH terms:
Neurofibromatoses
Neurofibromatosis 1
Genetic Diseases, Inborn
Heredodegenerative Disorders, Nervous System
Neoplasms
Neoplasms by Histologic Type
Neoplasms, Nerve Tissue
Neoplastic Syndromes, Hereditary
Nerve Sheath Neoplasms
Nervous System Diseases
Neurocutaneous Syndromes
Neurodegenerative Diseases
Neurofibroma
Neuromuscular Diseases
Peripheral Nervous System Diseases

ClinicalTrials.gov processed this record on October 30, 2014