Associations Between the Status of Malnutrition and Sarcopenia in Children and Adolescents With Primary Ciliary Dyskinesia
试验速览
- 阶段
- 不适用
- 状态
- 尚未招募
- 发起方
- 入组人数
- 15
- 试验地点
- 1
- 主要终点
- Handgrip Muscle Strength in Participants
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Cross Sectional
入排标准
- 年龄范围
- 6 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •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
结局指标
主要结局
Handgrip Muscle Strength in Participants
时间窗: 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.
次要结局
- Malnutrition risk score in Participants(From enrollment to the end of treatment at 1 year)
- Body Weight(From enrollment to the end of treatment at 1 year)
- Height(From enrollment to the end of treatment at 1 year)
- Body Mass Index(From enrollment to the end of treatment at 1 year)
- Waist circumference(From enrollment to the end of treatment at 1 year)
- Hip circumference(From enrollment to the end of treatment at 1 year)
- Waist-to-hip ratio(From enrollment to the end of treatment at 1 year)
- Waist-to-Height ratio(From enrollment to the end of treatment at 1 year)
- Upper-middle arm circumference(From enrollment to the end of treatment at 1 year)
- Triceps skinfold thickness(From enrollment to the end of treatment at 1 year)
- Body fat percentage(From enrollment to the end of treatment at 1 year)
- Height by Age(From enrollment to the end of treatment at 1 year)
- Weight by Height(From enrollment to the end of treatment at 1 year)
- Weight by Age(From enrollment to the end of treatment at 1 year)
- Body Mass Index by Age(From enrollment to the end of treatment at 1 year)
- Forced Expiratory Volume at One Second(From enrollment to the end of treatment at 1 year)
- Forced Vital Capacity(From enrollment to the end of treatment at 1 year)
- Forced Expiratory Volume at One Secondto Forced Vital Capacity Ratio(From enrollment to the end of treatment at 1 year)
- Peak Expiratory Flow(From enrollment to the end of treatment at 1 year)
- Forced Expiratory Flow at 25-75% of Forced Vital Capacity(From enrollment to the end of treatment at 1 year)
研究者
Kerim Kaan Göküstün
Principal Investigator
Izmir Democracy University