Upper Extremity Exercise Capacity and Muscle Oxygenation in Patients With Primary Ciliary Dyskinesia.

Background And Objectives: Patients with primary ciliary dyskinesia (PCD) have a reduction in exercise capacity from the early stages. Although there are studies investigating these patients' lower extremity exercise capacity using various methodologies, there needs to be more research on the functional exercise capacity of the upper extremities. This study aimed to compare pulmonary function, upper extremity functional exercise capacity, muscle oxygenation, muscle strength, and physical activity level in children with PCD and controls.

Methods: Twenty-seven children with PCD and 26 controls were included. Pulmonary function (spirometry), upper extremity functional exercise capacity (6-min pegboard and ring test (6PBRT), muscle oxygenation (Moxy device), deltoid muscle strength (dynamometer), and physical activity level (metabolic holter) were assessed.

Results: Patients' FVC%, FEV%, FEV/FVC, PEF%, FEF, deltoid muscle strength (%) were statistically decreased compared with controls (p < 0.05). The 6PBRT ring count, deltoid muscle oxygen saturations, and total hemoglobin levels were similar in both groups (p > 0.05).

Discussion: Pulmonary function deteriorates and upper extremity muscle strength decreases in children with PCD. The upper extremity exercise capacity of these patients remains preserved. However, upper extremity resting muscle oxygen saturation is lower in children compared to healthy children. Most patients and healthy children are inactive. The upper extremity exercise capacity possibly declines due to deteriorating pulmonary function as the patients get older, which needs to be investigated routinely. Both upper-lower extremity aerobic exercise training applied consistently and physical activity counseling as part of their pulmonary rehabilitation program should be considered in children with PCD.