To date, the literature has not yet elucidated the status of malnutrition and sarcopenia in children and adolescents with PCD compared to healthy controls. Therefore, the aim of this study is to compare the nutritional and sarcopenia status of children and adolescents with primary ciliary dyskinesia to that of healthy controls.
Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder characterized by abnormal ciliary motility and impaired mucociliary clearance, caused by mutations in the genes responsible for ciliary movement. Structural or functional defects of the cilia lead to chronic upper and lower respiratory tract infections, fertility issues, and organ asymmetry. Activities of daily living predominantly consist of movements requiring short-term and repetitive anaerobic performance, and in children particularly, physical activities generally involve brief, low-to-moderate intensity exertion. However, in children with PCD, factors such as pulmonary dysfunction, malnutrition, and decreased physical activity levels can adversely affect anaerobic exercise capacity.
Study Type
OBSERVATIONAL
Enrollment
30
In this study, malnutrition and sarcopenia status in children and adolescents diagnosed with primary ciliary dyskinesia will be evaluated. All data pertaining to these assessments will be acquired via face-to-face interviews with a duration of 45 to 60 minutes.
In this study, malnutrition and sarcopenia status in children and adolescents diagnosed with primary ciliary dyskinesia will be evaluated. All data pertaining to these assessments will be acquired via face-to-face interviews with a duration of 45 to 60 minutes.
Izmir Democracy University
Izmir, Turkey (Türkiye)
Hand Grip Strength
Handgrip strength (kg) will be assessed twice in each hand using a dynamometer (Jamar) with a maximal isometric contraction lasting approximately 2 seconds. The best result (kg) will be used for data analysis.
Time frame: through study completion, an average of 1 year
Vertical Jump Power
Vertical jump test, participants will assume a jumping position with their hands resting at the waist and their feet spaced pelvis-width apart. Participants will perform one practice jump and three test jumps. The highest of the three test jumps will be used for data analysis.
Time frame: through study completion, an average of 1 year
Core muscle strength
Core muscle strength was assessed using the plank test (Plank, s) in which the body weight was supported by the toes and forearms. The test was terminated when participants were unable to maintain posture or their pelvis moved up or down five or more cm, measured using a ruler. The test was also ended if participants remained in the plank position for 3 minutes (highest score).
Time frame: through study completion, an average of 1 year
Waist circumference measurement
Waist circumference measurement is the assessment of abdominal fat by measuring the distance around the narrowest part of the torso, usually just above the hip bones. It is a simple indicator used to evaluate central obesity and the risk of metabolic and cardiovascular diseases.
Time frame: through study completion, an average of 1 year
Malnutrition Risk Score of participants
Screening Tool for Risk on Nutritional Status and Growth will used to evaluate malnutrition risk score. Total score of this tool ranges from 0 to 5; 0 shows low risk score while 5 shows high risk score.
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Time frame: through study completion, an average of 1 year
Body Fat Percentage
Body fat percentage is the proportion of fat mass compared to total body weight. It is an important indicator of overall health and fitness, reflecting the balance between fat and lean body tissues.
Time frame: through study completion, an average of 1 year
Forced Vital Capacity
Forced Vital Capacity will be measured via spirometry.
Time frame: through study completion, an average of 1 year
Forced Expiratory Volume in 1 second
Forced Expiratory Volume in 1 second will be measured using spirometry.
Time frame: through study completion, an average of 1 year
Forced Expiratory Volume in 1 second Forced Vital Capacity Ratio
This value will be measured using spirometry. This ratio compares the amount of air exhaled in the first second to the total exhaled air. It is used to differentiate between obstructive and restrictive lung disorders.
Time frame: through study completion, an average of 1 year
Peak Expiratory Flow
This value will be measured using spirometry.
Time frame: through study completion, an average of 1 year
Forced Expiratory Flow at 25-75%
This value will be measured using spirometry.
Time frame: through study completion, an average of 1 year
Hip circumference measurement
Hip circumference measurement is the process of measuring the distance around the widest part of the hips and buttocks using a measuring tape. It is commonly used to assess body fat distribution and calculate ratios like the waist-to-hip ratio.
Time frame: through study completion, an average of 1 year
upper middle arm circumference measurement
Upper middle arm circumference (MUAC) measurement is the process of measuring the circumference of the upper arm at the midpoint between the shoulder and the elbow using a measuring tape. It is commonly used to assess nutritional status, especially in children and adults in clinical or field settings.
Time frame: through study completion, an average of 1 year
Triceps skinfold thickness
Triceps skinfold thickness is the measurement of the thickness of a fold of skin and subcutaneous fat at the back of the upper arm using calipers. It is used to estimate body fat percentage and assess nutritional status.
Time frame: through study completion, an average of 1 year