Trunk muscle strength and limitations in functioning in children and youth with spinal muscular atrophy

ISRCTN ISRCTN63278972
DOI https://doi.org/10.1186/ISRCTN63278972
Secondary identifying numbers 1/2017
Submission date
04/04/2017
Registration date
11/04/2017
Last edited
07/06/2023
Recruitment status
No longer recruiting
Overall study status
Completed
Condition category
Musculoskeletal Diseases
Prospectively registered
Protocol
Statistical analysis plan
Results
Individual participant data

Plain English summary of protocol

Background and study aims
Spinal muscular atrophy (SMA) is a rare inherited condition that causes muscle weakness and progressive (worsening over time) loss of movement. It is caused by deterioration in the nerve cells connecting the brain and spinal cord to the body’s muscles. As the link between the nerves and muscles breaks down, patients gradually lose their ability to move and their muscles waste away (atrophy). The aim of this study is to assess the strength of muscles in the trunk (torso) for rotational activity (twisting) and the level of independent functioning in children and youth with SMA.

Who can participate?
Patients aged 5-18 years who have been diagnosed with SMA and helathy children and youths of the same age.

What does the study involve?
All participants attend a single study visit at which the strength of their trunk (torso) is measured using a handheld measurement device while they are lying on their side. This is done at six points on the body. Following this, participants are asked to complete questionnaires about their daily activities and any limitations they may be facing as well as background information.

What are the possible benefits and risks of participating?
There are no direct benefits or risks involved with participating.

Where is the study run from?
Center of Functional Rehabilitation ORTHOS (Poland)

When is the study starting and how long is it expected to run for?
December 2016 to December 2017

Who is funding the study?
Józef Piłsudski University of Physical Education (Poland)

Who is the main contact?
Dr Agnieszka Stępień

Contact information

Dr Agnieszka Stępień
Scientific

Józef Piłsudski University of Physical Education
Marymoncka 34
Warsaw
00-968
Poland

ORCiD logoORCID ID 0000-0003-3905-8229

Study information

Study designSingle-centre case-control study
Primary study designObservational
Secondary study designCase-control study
Study setting(s)Other
Study typePrevention
Participant information sheet No participant information sheet available
Scientific titleStrength of trunk rotational muscles and limitations in everyday functioning in children and youth with spinal muscular atrophy
Study acronymSMA
Study objectives1. Strength of trunk muscles responsible for rotating trunk or pelvis in SMA children and youth is lower than in healthy peers
2. There is a correlation between the strength values of these muscles and limitations in everyday functioning
Ethics approval(s)The Senate Research Ethics Committee at Józef Piłsudski University of Physical Education in Warsaw, Poland, 07/02/2017, ref: SKE 01-03/2017
Health condition(s) or problem(s) studiedSpinal muscular atrophy
InterventionAll participants will have a one-time measurement of muscle strength taken in a side-lying position using a handheld MICROFET measurement system. The device will be placed consecutively in three points on the front side of the body (i.e. forehead; trunk – in the shoulder joint area; trunk – in the anterior superior iliac spine area) and later in three points at the back of the body (i.e. posterior middle part of the skull; posterior part of the trunk in the shoulder joint area; pelvis – below iliac crest). 5-10 seconds will be devoted to one measurement during maximal isometric tension. Two series of measurements on each side will be performed in each of the aforementioned points. A higher value will be included in the analysis.

Participants will be also asked to complete a questionnaire including questions regarding limitations during everyday activities. Additional information will be collected on age, gender, accompanying illnesses.
Intervention typeOther
Primary outcome measure1. Strength of muscles responsible for trunk rotation is assessed using a handheld MICROFET, in which the value of strength expressed in pounds or Newtons in a pre-set time unit, on the study visit
2. Functioning limitations are measured using a questionnaire designed for the purpose of this study, including questions regarding limitations during everyday activities prepared on the basis of ICF on the study visit
Secondary outcome measuresNo secondary outcome measures
Overall study start date01/12/2016
Completion date31/12/2017

Eligibility

Participant type(s)Mixed
Age groupChild
Lower age limit5 Years
Upper age limit18 Years
SexBoth
Target number of participantsminimum 30
Total final enrolment56
Key inclusion criteriaPatients:
1. Spinal muscular atrophy (SMA) diagnosed through clinical and genetic examinations
2. Aged 5-18 years

Controls:
1. Healthy
2. Aged 5-18 years
3. Do not practice sport
Key exclusion criteriaPatients:
1. No confirmation of SMA in genetic tests
2. Children under 5
3. Children and youth not able to cooperate during an examination

Controls:
1. Age under 5 or over 18
2. Children not able to cooperate during an examination.
Date of first enrolment15/04/2017
Date of final enrolment30/09/2017

Locations

Countries of recruitment

  • Poland

Study participating centre

Center of Functional Rehabilitation ORTHOS
Modzelewskiego 37
Warsaw
02-679
Poland

Sponsor information

Józef Piłsudski University of Physical Education
University/education

Marymoncka 34
Warsaw
00-968
Poland

ROR logo "ROR" https://ror.org/043k6re07

Funders

Funder type

University/education

Józef Piłsudski University of Physical Education

No information available

Results and Publications

Intention to publish date31/12/2018
Individual participant data (IPD) Intention to shareYes
IPD sharing plan summaryAvailable on request
Publication and dissemination planThe results will be published in an article in a neurologic, physical therapy or rehabilitation journal.
IPD sharing planThe datasets generated during and/or analysed during the current study are/will be available upon request from Agnieszka Stępień (orthosas@wp.pl)

Study outputs

Output type Details Date created Date added Peer reviewed? Patient-facing?
Results article 30/04/2021 07/06/2023 Yes No

Editorial Notes

07/06/2023: Publication reference and total final enrolment added.