Endurance testing assesses functional capacity Endurance tests measure the efficiency of performing a task and provide assessments of Functional status of the cardiac respiratory and musculoskeletal systems ID: 538490
Download Presentation The PPT/PDF document "Morquio A: Endurance" is the property of its rightful owner. Permission is granted to download and print the materials on this web site for personal, non-commercial use only, and to display it on your personal computer provided you do not modify the materials and that you retain all copyright notices contained in the materials. By downloading content from our website, you accept the terms of this agreement.
Slide1
Morquio A: EnduranceSlide2
Endurance testing assesses functional capacity
Endurance tests measure the efficiency of performing a task and provide assessments of:
Functional status of the cardiac, respiratory and musculoskeletal systems
Overall disease progression
Quality of lifeResponse to treatments
McDonald et al,
J
Pediatr
Rehabil
Med
, 2010;
Hendriksz
et al,
J Inherit
Metab
Dis
, 2012Slide3
Image courtesy of Elizabeth Wright
Recommended endurance tests for assessing submaximal functional capacity in
Morquio
A patients include:
6 minute walk test (6MWT)measures how far a person can walk on a hard, flat surface in 6 minutes a standardized test (American Thoracic Society, 2002)used to assess endurance in patients with MPS VI (Harmatz et al, 2005) and Morquio A (Harmatz et al, 2013)
3 minute stair climb (3MSCT)
measures how many steps a person can ascend in 3 minutes, using rails and resting as needed
used to assess endurance in patients with MPS VI (Harmatz et al, 2005) and Morquio A (Harmatz et al, 2013)
Harmatz et al, Mol Genet Metab, 2013; Harmatz et al, Pediatrics, 2005; McDonald et al, J Pediatr Rehabil Med, 2010; Hendriksz et al, J Inherit Metab Dis, 2012
Submaximal intensity tests are sensitive indicators of endurance for MPS patientsSlide4
Endurance is impaired in Morquio A
Mean 6MWT = 212.6 ±152.2 m (n = 316 subjects)
Mean 3MSCT = 30.0 ±24.0 stairs (n= 274 subjects)
Harmatz et al,
Mol Genet Metab, 2013MorCAP baseline data
Mean 6MWT
Mean
3MSCT
n=248n=68n=316
n=215
n=59
n=274Slide5
Assessment
At diagnosis
Annually
As clinically indicated
Evaluation by physiotherapist
X
X
X
6 MWT (if physically and developmentally able)
X
X
X
3 MSCT
(if physically and developmentally able)
X
X
X
Regular assessments are recommended
for optimal patient quality of life
Hendriksz
et al,
J Inherit
Metab
Dis
, 2012