The prevalence and causes of malnutrition and sarcopenia in children and adolescents with Primary ciliary dyskinesia (PCD) have not been sufficiently elucidated. The aim of this study is to investigate the prevalence of malnutrition and sarcopenia in children and adolescents with PCD.
Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder characterized by abnormal ciliary motility and impaired mucociliary clearance. It develops as a result of mutations in genes responsible for ciliary movement. Structural or functional abnormalities of cilia lead to chronic upper and lower respiratory tract infections, fertility problems, and organ laterality defects. The prevalence of PCD ranges from 1 in 2,000 to 1 in 40,000, varying among different ethnic groups. The disease begins to negatively affect lung function from the preschool period onward. In children with PCD, respiratory function is generally characterized by mild to moderate airway obstruction. As the disease progresses, the severity of airway obstruction increases. Therefore, regular monitoring of pulmonary function is of great importance in the management of PCD. The prevalence and causes of malnutrition and sarcopenia in children and adolescents with PCD have not been sufficiently elucidated. The aim of this study is to investigate the prevalence of malnutrition and sarcopenia in children and adolescents with PCD.
Study Type
OBSERVATIONAL
Enrollment
15
Participants will be evaluated in terms of nutritional status, anthropometric measurements (waist circumference, hip circumference, waist-to-height ratio, skinfold measurements) and body composition, pulmonary functions, anaerobic capacity, muscle strength.
Izmir Democracy University
Izmir, Turkey (Türkiye)
Handgrip Muscle Strength in Participants
Bilateral grip strength in children and adolescents with PCD will be measured in kgF using a hand dynamometer.
Time frame: From enrollment to the end of treatment at 1 year
Malnutrition risk score in Participants
The Nutritional Status and Growth Risk Screening Tool will be used to assess the risk of growth failure. The total score on this scale ranges from 0 to 5; a score of 0 indicates a low risk of malnutrition, a score of 1-3 indicates a moderate risk of malnutrition, and a score of 4-5 indicates a high risk of malnutrition.
Time frame: From enrollment to the end of treatment at 1 year
Body Weight
Body weight in children and adolescents with PCD will be measured using a pediatric scale.
Time frame: From enrollment to the end of treatment at 1 year
Height
In children and adolescents with PCD, height will be measured using a stadiometer.
Time frame: From enrollment to the end of treatment at 1 year
Body Mass Index
For children and adolescents with PCD, the body mass index will be calculated by dividing body weight by the square of height in meters.
Time frame: From enrollment to the end of treatment at 1 year
Waist circumference
Waist circumference in children and adolescents with PCD will be measured using a tape measure
Time frame: From enrollment to the end of treatment at 1 year
Hip circumference
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
Hip circumference in children and adolescents with PCD will be measured using a tape measure
Time frame: From enrollment to the end of treatment at 1 year
Waist-to-hip ratio
In children and adolescents with PCD, the waist-to-hip ratio will be calculated by dividing the waist circumference by the hip circumference.
Time frame: From enrollment to the end of treatment at 1 year
Waist-to-Height ratio
In children and adolescents with PCD, the waist-to-height ratio will be calculated by dividing the waist circumference by the height.
Time frame: From enrollment to the end of treatment at 1 year
Upper-middle arm circumference
In children and adolescents with PCD, upper arm circumference will be measured using a non-stretchable tape measure, without tightening it or leaving any slack.
Time frame: From enrollment to the end of treatment at 1 year
Triceps skinfold thickness
In children and adolescents with PCD, triceps skinfold thickness will be measured using a skinfold caliper.
Time frame: From enrollment to the end of treatment at 1 year
Body fat percentage
Body fat percentage in children and adolescents with PCD will be measured using bioelectrical impedance analysis.
Time frame: From enrollment to the end of treatment at 1 year
Height by Age
The percentile values for height by age in children and adolescents with PCD will be determined using data from the CDC's website.
Time frame: From enrollment to the end of treatment at 1 year
Weight by Height
The percentile values for Weight by Height in children and adolescents with PCD will be determined using data from the CDC's website.
Time frame: From enrollment to the end of treatment at 1 year
Weight by Age
The percentile values for Weight by Age in children and adolescents with PCD will be determined using data from the CDC's website.
Time frame: From enrollment to the end of treatment at 1 year
Body Mass Index by Age
Body Mass Index (BMI) is an anthropometric index calculated by dividing body weight in kilograms by the square of height in meters. Body Mass Index by Age (BMI-for-Age) is the body mass index (BMI) adjusted for age and sex and interpreted using age- and sex-specific growth reference charts.The percentile values for Body Mass Index by Age in children and adolescents with PCD will be determined using data from the CDC's website.
Time frame: From enrollment to the end of treatment at 1 year
Forced Expiratory Volume at One Second
Forced Expiratory Volume at One Second is the volume of air that an individual can forcibly exhale during the first second of a maximal forced expiration following a full inspiration. It is typically expressed in liters or as a percentage of the predicted value (% predicted).
Time frame: From enrollment to the end of treatment at 1 year
Forced Vital Capacity
Forced Vital Capacity is the total volume of air that an individual can forcibly exhale after taking a maximal inspiration. It is typically expressed in liters (L) or as a percentage of the predicted value (% predicted).
Time frame: From enrollment to the end of treatment at 1 year
Forced Expiratory Volume at One Secondto Forced Vital Capacity Ratio
Forced Expiratory Volume at One Second to Forced Vital Capacity Ratio is the proportion of the forced vital capacity that is exhaled during the first second of a forced expiration following a maximal inspiration. It is expressed as a percentage.
Time frame: From enrollment to the end of treatment at 1 year
Peak Expiratory Flow
Peak Expiratory Flow is the maximum expiratory flow rate achieved during a forced expiration following a maximal inspiration. It is typically expressed in liters per minute.
Time frame: From enrollment to the end of treatment at 1 year
Forced Expiratory Flow at 25-75% of Forced Vital Capacity
Forced Expiratory Flow at 25-75% of Forced Vital Capacity is the average forced expiratory flow measured during the middle 50% of the forced vital capacity maneuver, specifically between 25% and 75% of the exhaled volume. It is typically expressed in liters per second.
Time frame: From enrollment to the end of treatment at 1 year