Primary Ciliary Dyskinesia (PCD)
Conditions
Keywords
primary ciliary dyskinesia (PCD), sarcopenia, respiratory function test, malnutrion
Brief summary
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.
Detailed description
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.
Interventions
OTHERPhysical Evaluations in Children with Primary Ciliary Dyskinesia
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.
Sponsors
Izmir Democracy University
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
6 Years to 18 Years
Healthy volunteers
Yes
Inclusion criteria
for Children and Adolescents with Primary Ciliary Dyskinesia: * To be between the ages of 6-18 * To have been diagnosed with primary ciliary dyskinesia * To be clinically stable for at least 3 weeks * To have the necessary cooperation for the measurements * To volunteer to participate in the study Inclusion Criteria for Healthy Children and Adolescents: * To be between the ages of 6-18 * To be in a similar average and ratio with the group of children and adolescents with primary ciliary dyskinesia in terms of age and gender * To have the necessary cooperation for the measurements * To volunteer to participate in the study
Exclusion criteria
for Children and Adolescents with Primary Ciliary Dyskinesia: * Having any orthopedic, neurological, psychological or cardiovascular problem that may prevent the measurements from being performed in the last 6 months * Smoking or quitting smoking
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Hand Grip Strength | through study completion, an average of 1 year | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Vertical Jump Power | through study completion, an average of 1 year | 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. |
| Core muscle strength | through study completion, an average of 1 year | 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). |
| Waist circumference measurement | through study completion, an average of 1 year | 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. |
| Malnutrition Risk Score of participants | through study completion, an average of 1 year | 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. |
| Body Fat Percentage | through study completion, an average of 1 year | 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. |
| Forced Vital Capacity | through study completion, an average of 1 year | Forced Vital Capacity will be measured via spirometry. |
| Forced Expiratory Volume in 1 second | through study completion, an average of 1 year | Forced Expiratory Volume in 1 second will be measured using spirometry. |
| Forced Expiratory Volume in 1 second Forced Vital Capacity Ratio | through study completion, an average of 1 year | 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. |
| Peak Expiratory Flow | through study completion, an average of 1 year | This value will be measured using spirometry. |
| Forced Expiratory Flow at 25-75% | through study completion, an average of 1 year | This value will be measured using spirometry. |
| Hip circumference measurement | through study completion, an average of 1 year | 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. |
| upper middle arm circumference measurement | through study completion, an average of 1 year | 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. |
| Triceps skinfold thickness | through study completion, an average of 1 year | 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. |
Countries
Turkey (Türkiye)
Contacts
CONTACTGülşah Barğı, Assoc Prof
CONTACTAybüke S Demir, PT, MSc
STUDY_DIRECTORGülşah Barğı, Assoc Prof
Izmir Democracy University
PRINCIPAL_INVESTIGATORAybüke S Demir, PT, MSc
Izmir Democracy University
PRINCIPAL_INVESTIGATORKerim K Göküstün, Asst Prof
Izmir Democracy University
PRINCIPAL_INVESTIGATOREce Ocak, Dr
Izmir City Hospital
Outcome results
None listed