Comparison of respiratory functions, muscle strength, and physical activity among children with primary ciliary dyskinesia with and without Kartagener's syndrome and healthy controls.

Abstract

Introduction: Kartagener's syndrome (KS), consisting of bronchiectasis, situs inversus totalis, and sinusitis, is a subtype of primary ciliary dyskinesia (PCD). The presence of KS may affect respiratory and physical functions.

Purpose: This study aimed to compare respiratory functions, exercise capacity, muscle strength, and physical activity levels among children with PCD with/without KS and healthy peers.

Methods: Fifteen patients with KS, 23 with PCD without KS, and 27 controls were compared. Pulmonary function, functional exercise capacity (6-minute walk test - 6MWT), maximal inspiratory, expiratory (MIP, MEP), and skeletal muscle strength, inspiratory muscle endurance (IME), and physical activity level were evaluated.

Results: The forced expiratory volume in one second (FEV₁) % (p = .009), forced expiratory flow from 25%-75% (FEF_25-75%) % (p = .001), MIP (p = .034), MEP (p = .003), 6MWT distance (p = .001), and daily steps (p = .034) were significantly different among the groups. Quadriceps femoris (QF) muscle strength and IME were similar in groups (p ˃ .05). FEV₁% (p = .002), FEF_25-75% % (p = .001), MIP (p = .027), MEP (p = .001), and 6MWT distance (p = .003) in patients with KS; 6MWT distance (p = .003) in patients with PCD without KS was significantly lower than controls.

Conclusion: The presence of KS affects pulmonary function, respiratory muscle strength, and physical activity more. Exercise capacity and physical activity levels are decreased, inspiratory muscle endurance and QF muscle strength are preserved in patients with KS and PCD without KS. Kartagener's syndrome further impairs pulmonary and extrapulmonary outcomes; the reasons should be investigated, and the necessity of rehabilitation approaches that will prevent deterioration come to the fore.