Primary Ciliary Dyskinesia (PCD)
Conditions
Keywords
Malnutrition, Muscle strength, Sarcopenia, Respiratory function test, Primary Ciliary Dyskinesia
Brief summary
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.
Detailed description
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.
Interventions
OTHERPhysical assessments of individuals
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.
Sponsors
Izmir Democracy University
Study design
Observational model
CASE_ONLY
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
No
Inclusion criteria
Inclusion criteria for children and adolescents with PCD 1. Unexplained neonatal respiratory distress, lateralization defect, productive cough, bronchiectasis, daily nasal congestion, and pansinusitis, as well as laboratory tests such as high-speed video microscopy, transmission electron microscopy, or genetic testing according to the European Respiratory Society diagnostic guidelines. 2. Cooperative individuals, 3. Individuals who have never smoked 4. Willingness to participate in the study Inclusion criteria for healthy children and adolescents: 1\. Voluntary participation in the study
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Handgrip Muscle Strength in Participants | From enrollment to the end of treatment at 1 year | Bilateral grip strength in children and adolescents with PCD will be measured in kgF using a hand dynamometer. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Malnutrition risk score in Participants | From enrollment to the end of treatment at 1 year | 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. |
| Body Weight | From enrollment to the end of treatment at 1 year | Body weight in children and adolescents with PCD will be measured using a pediatric scale. |
| Height | From enrollment to the end of treatment at 1 year | In children and adolescents with PCD, height will be measured using a stadiometer. |
| Body Mass Index | From enrollment to the end of treatment at 1 year | For children and adolescents with PCD, the body mass index will be calculated by dividing body weight by the square of height in meters. |
| Waist circumference | From enrollment to the end of treatment at 1 year | Waist circumference in children and adolescents with PCD will be measured using a tape measure |
| Hip circumference | From enrollment to the end of treatment at 1 year | Hip circumference in children and adolescents with PCD will be measured using a tape measure |
| Waist-to-hip ratio | From enrollment to the end of treatment at 1 year | In children and adolescents with PCD, the waist-to-hip ratio will be calculated by dividing the waist circumference by the hip circumference. |
| Waist-to-Height ratio | From enrollment to the end of treatment at 1 year | In children and adolescents with PCD, the waist-to-height ratio will be calculated by dividing the waist circumference by the height. |
| Upper-middle arm circumference | From enrollment to the end of treatment at 1 year | 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. |
| Triceps skinfold thickness | From enrollment to the end of treatment at 1 year | In children and adolescents with PCD, triceps skinfold thickness will be measured using a skinfold caliper. |
| Body fat percentage | From enrollment to the end of treatment at 1 year | Body fat percentage in children and adolescents with PCD will be measured using bioelectrical impedance analysis. |
| Height by Age | From enrollment to the end of treatment at 1 year | The percentile values for height by age in children and adolescents with PCD will be determined using data from the CDC's website. |
| Weight by Height | From enrollment to the end of treatment at 1 year | The percentile values for Weight by Height in children and adolescents with PCD will be determined using data from the CDC's website. |
| Weight by Age | From enrollment to the end of treatment at 1 year | The percentile values for Weight by Age in children and adolescents with PCD will be determined using data from the CDC's website. |
| Body Mass Index by Age | From enrollment to the end of treatment at 1 year | 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. |
| Forced Expiratory Volume at One Second | From enrollment to the end of treatment at 1 year | 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). |
| Forced Vital Capacity | From enrollment to the end of treatment at 1 year | 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). |
| Forced Expiratory Volume at One Secondto Forced Vital Capacity Ratio | From enrollment to the end of treatment at 1 year | 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. |
| Peak Expiratory Flow | From enrollment to the end of treatment at 1 year | 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. |
| Forced Expiratory Flow at 25-75% of Forced Vital Capacity | From enrollment to the end of treatment at 1 year | 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. |
Countries
Turkey (Türkiye)
Contacts
CONTACTKerim Kaan K GÖKÜSTÜN, Dr. Lecturer
CONTACTGülşah BARĞI, Assoc. Dr.
STUDY_DIRECTORKerim K Göküstün, Dr. Lecturer
Izmir Democracy University
PRINCIPAL_INVESTIGATORGülşah BARĞI, Assoc. Dr.
Izmir Democracy University
PRINCIPAL_INVESTIGATORAybüke Sena DEMİR, MSc.
Izmir Democracy University
PRINCIPAL_INVESTIGATOREce OCAK, Dr.
University of Health science
Outcome results
None listed