Upper Extremity Exercise Capacity, Muscle Oxygenation, and Balance in Pediatric CF and PCD

Cystic fibrosis (CF) is an autosomal recessive, monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In individuals with CF, infections, inflammation, mucus plugging, nutritional deficiencies, pulmonary exacerbations, and respiratory tract diseases contribute to a decline in pulmonary function. Increased airway resistance, air trapping, and reduced airflow rates lead to a decrease in lung capacities. Primary ciliary dyskinesia (PCD) is a genetically heterogeneous disorder caused by the absence or dysfunction of motile cilia. In PCD, pulmonary function is affected from early in life and remains below reference values across all age groups and both sexes. Decreased muscle strength has been demonstrated in both CF and PCD patients. In chronic obstructive pulmonary disease (COPD), pulmonary function and limb muscle strength are important determinants of exercise capacity. There is a limited number of studies in the literature assessing pulmonary function and upper extremity functional exercise capacity in patients with CF and PCD. Furthermore, to our knowledge, no studies have compared upper extremity functional exercise capacity and upper extremity muscle oxygenation between patients with CF and PCD. The aim of this study is to compare pulmonary function, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance among children with CF, children with PCD, and healthy controls. The study was planned as cross-sectional and retrospective. The patient group will include individuals diagnosed with CF and PCD, aged between 6 and 18 years, receiving standard medical treatment. The healthy group will include individuals aged between 6 and 18 years without any known chronic disease. Individuals' respiratory functions, upper extremity functional exercise capacity, peripheral muscle strength, muscle oxygenation, and balance will be evaluated. Upper extremity functional exercise capacity was evaluated using the 6-minute pegboard and ring test, pulmonary function using spirometry, peripheral muscle strength using a hand-held dynamometer, muscle oxygenation using the Moxy monitor device (Moxy, Fortiori Design LLC, Minnesota, ABD), static balance using the "Biosway Portable Balance System", and dynamic balance assessment using the Y balance test. The assessments will be completed in two days.