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

Neurofibromatosis Type 1 Clinical Trial

Official title:

Effects of Physical Training on Bone and Muscle Quality, Muscle Strength, and Motor Coordination in Children With Neurofibromatosis Type 1

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01058330
• Other study ID #: SLC00038711
• Status: Completed
• Phase: N/A
• Start date: February 2010
• Est. completion date: February 2014

Study information

• Verified date: March 2021
• Source: Shriners Hospitals for Children
• Contact: n/a
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial Summary

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.

Description:

Disorders of the Ras pathway have significant phenotypic overlap and include Noonan syndrome, Cardiofaciocutaneous 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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 36
• Est. completion date: February 2014
• Est. primary completion date: February 2014
• Accepts healthy volunteers: No
• Gender: All
• Age group: 4 Years to 19 Years

Eligibility

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

Related Conditions & MeSH terms

• Neurofibroma
• Neurofibromatoses
• Neurofibromatosis 1
• Neurofibromatosis Type 1

Intervention

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 five lower extremity exercises and five upper extremity exercises

Locations

Country	Name	City	State
United States	Shriners Hospitals for Children, Salt Lake City	Salt Lake City	Utah
United States	University of Utah	Salt Lake City	Utah

Sponsors (3)

Lead Sponsor	Collaborator
Shriners Hospitals for Children	Thrasher Research Fund, University of Utah

Country where clinical trial is conducted

United States, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Bone & muscle quality, DXA, pQCT, & bone ultrasound.		1 year
Secondary	Motor proficiency BOT-2. Muscle strength force plate & dynamometer. Quality of life questionnaires.		1 year