Malnutrition and Sarcopenia Among Primary Ciliary Dyskinesia (Malnutrition)
2026年7月28日 更新者：Kerim Kaan Göküstün、Izmir Democracy University
Associations Between the Status of Malnutrition and Sarcopenia in Children and Adolescents With Primary Ciliary Dyskinesia
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.
研究概览
详细说明
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.
研究类型
观察性的
注册 (估计的)
15
联系人和位置
本节提供了进行研究的人员的详细联系信息，以及有关进行该研究的地点的信息。
学习联系方式
- 姓名：Kerim Kaan K GÖKÜSTÜN, Dr. Lecturer
- 电话号码：+90 232 299 07 39
- 邮箱：kerimkaangokustun@hotmail.com
研究联系人备份
- 姓名：Gülşah BARĞI, Assoc. Dr.
- 电话号码：+90 232 299 07 39
- 邮箱：gulsahbargi35@gmail.com
学习地点
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Izmir、土耳其（türkiye）、35140
- Izmir Democracy University
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接触：
- Kerim K GÖKÜSTÜN, Dr. Lecturer
- 电话号码：05058699032
- 邮箱：kerimkaangokustun@hotmail.com
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接触：
- Gülşah BARĞI, Assoc. Dr.
- 邮箱：gulsahbargi35@gmail.com
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参与标准
研究人员寻找符合特定描述的人，称为资格标准。这些标准的一些例子是一个人的一般健康状况或先前的治疗。
资格标准
适合学习的年龄
- 孩子
- 成人
接受健康志愿者
不
取样方法
非概率样本
研究人群
The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).
描述
Inclusion Criteria:
Inclusion criteria for children and adolescents with PCD
- 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.
- Cooperative individuals,
- Individuals who have never smoked
- Willingness to participate in the study
Inclusion criteria for healthy children and adolescents:
1. Voluntary participation in the study
Exclusion Criteria:
Exclusion criteria for children and adolescents with PCD:
- Individuals with any acute, chronic, or systemic illness other than PCD
- Individuals who smoke or are quitting smoking
- Individuals who are not willing to participate in the study
- Individuals who are uncooperative
学习计划
本节提供研究计划的详细信息，包括研究的设计方式和研究的衡量标准。
研究是如何设计的？
设计细节
队列和干预
团体/队列 |
干预/治疗 |
|---|---|
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Patient of Primary Ciliary Dyskinesia
The study will include children and adolescents with PCD aged 6 to 18 (n=15).
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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.
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研究衡量的是什么？
主要结果指标
结果测量 |
措施说明 |
大体时间 |
|---|---|---|
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Handgrip Muscle Strength in Participants
大体时间：From enrollment to the end of treatment at 1 year
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Bilateral grip strength in children and adolescents with PCD will be measured in kgF using a hand dynamometer.
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From enrollment to the end of treatment at 1 year
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次要结果测量
结果测量 |
措施说明 |
大体时间 |
|---|---|---|
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Malnutrition risk score in Participants
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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Body Weight
大体时间：From enrollment to the end of treatment at 1 year
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Body weight in children and adolescents with PCD will be measured using a pediatric scale.
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From enrollment to the end of treatment at 1 year
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Height
大体时间：From enrollment to the end of treatment at 1 year
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In children and adolescents with PCD, height will be measured using a stadiometer.
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From enrollment to the end of treatment at 1 year
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Body Mass Index
大体时间：From enrollment to the end of treatment at 1 year
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For children and adolescents with PCD, the body mass index will be calculated by dividing body weight by the square of height in meters.
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From enrollment to the end of treatment at 1 year
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Waist circumference
大体时间：From enrollment to the end of treatment at 1 year
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Waist circumference in children and adolescents with PCD will be measured using a tape measure
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From enrollment to the end of treatment at 1 year
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Hip circumference
大体时间：From enrollment to the end of treatment at 1 year
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Hip circumference in children and adolescents with PCD will be measured using a tape measure
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From enrollment to the end of treatment at 1 year
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Waist-to-hip ratio
大体时间：From enrollment to the end of treatment at 1 year
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In children and adolescents with PCD, the waist-to-hip ratio will be calculated by dividing the waist circumference by the hip circumference.
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From enrollment to the end of treatment at 1 year
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Waist-to-Height ratio
大体时间：From enrollment to the end of treatment at 1 year
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In children and adolescents with PCD, the waist-to-height ratio will be calculated by dividing the waist circumference by the height.
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From enrollment to the end of treatment at 1 year
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Upper-middle arm circumference
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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Triceps skinfold thickness
大体时间：From enrollment to the end of treatment at 1 year
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In children and adolescents with PCD, triceps skinfold thickness will be measured using a skinfold caliper.
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From enrollment to the end of treatment at 1 year
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Body fat percentage
大体时间：From enrollment to the end of treatment at 1 year
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Body fat percentage in children and adolescents with PCD will be measured using bioelectrical impedance analysis.
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From enrollment to the end of treatment at 1 year
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Height by Age
大体时间：From enrollment to the end of treatment at 1 year
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The percentile values for height by age in children and adolescents with PCD will be determined using data from the CDC's website.
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From enrollment to the end of treatment at 1 year
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Weight by Height
大体时间：From enrollment to the end of treatment at 1 year
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The percentile values for Weight by Height in children and adolescents with PCD will be determined using data from the CDC's website.
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From enrollment to the end of treatment at 1 year
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Weight by Age
大体时间：From enrollment to the end of treatment at 1 year
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The percentile values for Weight by Age in children and adolescents with PCD will be determined using data from the CDC's website.
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From enrollment to the end of treatment at 1 year
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Body Mass Index by Age
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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Forced Expiratory Volume at One Second
大体时间：From enrollment to the end of treatment at 1 year
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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).
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From enrollment to the end of treatment at 1 year
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Forced Vital Capacity
大体时间：From enrollment to the end of treatment at 1 year
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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).
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From enrollment to the end of treatment at 1 year
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Forced Expiratory Volume at One Secondto Forced Vital Capacity Ratio
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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Peak Expiratory Flow
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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Forced Expiratory Flow at 25-75% of Forced Vital Capacity
大体时间：From enrollment to the end of treatment at 1 year
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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.
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From enrollment to the end of treatment at 1 year
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合作者和调查者
在这里您可以找到参与这项研究的人员和组织。
调查人员
- 首席研究员：Gülşah BARĞI, Assoc. Dr.、Izmir Democracy University
- 研究主任：Kerim K Göküstün, Dr. Lecturer、Izmir Democracy University
- 首席研究员：Aybüke Sena DEMİR, MSc.、Izmir Democracy University
- 首席研究员：Ece OCAK, Dr.、University of Health science
出版物和有用的链接
负责输入研究信息的人员自愿提供这些出版物。这些可能与研究有关。
一般刊物
- Despotes KA, Zariwala MA, Davis SD, Ferkol TW. Primary Ciliary Dyskinesia: A Clinical Review. Cells. 2024 Jun 4;13(11):974. doi: 10.3390/cells13110974.
- McCoy J, Gatt D, Shaw M, Solomon M, Kritzinger F, Ratjen F. The Impact of Nutritional Status on Lung Function Trajectories in Pediatric Patients With Primary Ciliary Dyskinesia. Pediatr Pulmonol. 2026 Apr;61(4):e71634. doi: 10.1002/ppul.71634.
- Karavasiloglou N, Gkatzou V, Fernandez-Rodriguez A, Schwartz V, Goutaki M. Nutrition and growth of primary ciliary dyskinesia patients: a systematic review. Eur Respir Rev. 2026 May 27;35(180):260024. doi: 10.1183/16000617.0024-2026. Print 2026 Apr.
研究记录日期
这些日期跟踪向 ClinicalTrials.gov 提交研究记录和摘要结果的进度。研究记录和报告的结果由国家医学图书馆 (NLM) 审查，以确保它们在发布到公共网站之前符合特定的质量控制标准。
研究主要日期
学习开始 (估计的)
2026年8月3日
初级完成 (估计的)
2027年7月1日
研究完成 (估计的)
2028年7月1日
研究注册日期
首次提交
2026年7月23日
首先提交符合 QC 标准的
2026年7月28日
首次发布 (实际的)
2026年7月31日
研究记录更新
最后更新发布 (实际的)
2026年7月31日
上次提交的符合 QC 标准的更新
2026年7月28日
最后验证
2026年7月1日
更多信息
与本研究相关的术语
其他相关的 MeSH 术语
其他研究编号
- Primary Ciliary Dyskinesia
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