Search results for "maximal voluntary ventilation"
showing 4 items of 4 documents
Longitudinal analysis of endurance and respiratory function from a natural history study of Morquio A syndrome
2015
Objectives: Baseline data from the Morquio A Clinical Assessment Program (MorCAP) revealed that individuals with Morquio A syndrome show substantial impairment in multiple domains including endurance and respiratory function (Harmatz et al., Mol Genet Metab, 2013). Here, 1- and 2-year longitudinal endurance and respiratory function data are presented.Methods: Endurance was assessed using the 6-minute walk test (6MWT) and the 3-minute stair climb test (3MSCT). Respiratory function was evaluated by measuring forced vital capacity (PVC) and maximum voluntary ventilation (MW). Data were analyzed using repeated measures ANCOVA models. Annualized estimates of change were determined using model es…
Multi-domain impact of elosufase alfa in Morquio A syndrome in the pivotal phase III trial
2015
Objective: To report and discuss the multi-domain impact of elosulfase alfa, with focus on tertiary and composite endpoints, in the 24-week, randomized, double-blind, placebo-controlled phase 3 trial in patients with Morquio A syndrome (mucopolysaccharidosis IVA). Methods: Patients with Morquio A syndrome >= 5 aged years were randomized 1:1:1 to elosulfase alfa 2.0 mg/kg/week (qw; N = 58), elosulfase alfa 2.0 mg/kg/every other week (qow; N = 59), or placebo (N = 59) for 24 weeks. Primary and secondary efficacy measures were 6-minute walk test (6MWT; primary), 3-minute stair climb test (3-MSCT) and urinary keratan sulfate (KS). Safety was also evaluated. Tertiary efficacy measures included r…
Physiotherapy Intervention for Preventing the Respiratory Muscle Deterioration in Institutionalized Older Women With Functional Impairment
2013
Abstract Introduction In elderly seniors (>80 years), respiratory function may be compromised when, in addition to the presence of comorbidity and loss of mobility, there is also reduced respiratory muscle (RM) strength. The literature has shown that RM training could be an effective method to improve RM function and prevent clinical deterioration, particularly in population with RM weakness. The main purpose of this paper was to assess the effectiveness of RM training on the respiratory muscle strength and endurance of institutionalized elderly women with functional impairment. Method Fifty-four residents (mean=85 years, SD=6.7) were randomly assigned to either a control (n=27) or training…
Relationship Between Patient-Reported Outcomes and Clinical Outcomes in Patients With Morquio A Syndrome
2019
Abstract This cross-sectional analysis assessed the correlation between patient-reported outcomes (PROs) and clinical outcomes in 24 German patients with Morquio A. Clinical outcomes included 6-minute walk test (6MWT), 3-minute stair climb (3MSC) test, and joint range of motion as measures for endurance/mobility, forced vital capacity (FVC) and maximum voluntary ventilation (MVV) as measures for respiratory function, and height as an important manifestation. The PROs included the EuroQoL (EQ) 5D-5L (EQ5D-5L), to measure health-related QoL (HRQoL), and patients’ rating of their ability to walk, climb, or breathe. In adults, endurance and pulmonary function measures and height showed strong a…