Respiratory muscle strength, exercise capacity and physical activity in patients with primary ciliary dyskinesia: A cross-sectional study

Abstract

Background and objectivesThe pathophysiological mechanisms of primary ciliary dyskinesia (PCD) may affect many functions, including respiratory, physical, and health status. This study aimed to compare respiratory muscle strength, inspiratory muscle endurance, muscle strength, exercise capacity, physical activity levels, and quality of life in PCD patients and controls. MethodsTwenty-seven patients and 28 controls were included. Respiratory muscle strength (maximal inspiratory (MIP) and maximal expiratory (MEP) pressures), inspiratory muscle endurance (incremental threshold loading test), muscle strength (quadriceps femoris, shoulder abductor, elbow flexor, handgrip), exercise capacity (6-min walk test (6MWT)), physical activity and quality of life (QOL-PCD) were evaluated. ResultsMIP, inspiratory muscle endurance, quadriceps femoris, and handgrip muscle strength, 6MWT distance, total energy expenditure; childrens' (6–12 years) and their parents' physical function, upper, lower respiratory, and hearing symptoms and treatment burden QOL-PCD subscales scores were significantly lower in patients compared with controls (p < 0.05). The 66.7% of patients did not meet the optimal number of steps. MEP, shoulder abductor, and elbow flexor muscle strength, active energy expenditure, physical activity duration, average metabolic equivalents, number of steps, lying time, and sleep duration; childrens' and their parents' other subscales and adolescents’ all QOL-PCD subscales scores were similar between groups (p > 0.05). ConclusionInspiratory muscle strength and endurance, lower extremity and total muscle strength, exercise capacity, total energy expenditure, and childrens’ quality of life are impaired compared to healthy controls. Decreased physical activity level is prevalent in these patients. Effects of pulmonary rehabilitation on these impaired outcomes for PCD patients should be investigated. Trial registrationClinicaltrials.gov: NCT03370029; December 12, 2017.