Comparison of Individuals With Primary Ciliary Dyskinesia and Healthy Controls

Официальное название

Comparison of Nutritional Status and Sarcopenia Between Children and Adolescents With Primary Ciliary Dyskinesia and Healthy Children

Обзор

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.

Подробное описание

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.

Вмешательства

• Другое Physical 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.
• Другое Physical Evaluations in Healthy Children
  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.

Первичные конечные точки

• Hand Grip Strength [Срок оценки: through study completion, an average of 1 year]

Вторичные конечные точки (12)

• Vertical Jump Power [Срок оценки: through study completion, an average of 1 year]
• Core muscle strength [Срок оценки: through study completion, an average of 1 year]
• Waist circumference measurement [Срок оценки: through study completion, an average of 1 year]
• Malnutrition Risk Score of participants [Срок оценки: through study completion, an average of 1 year]
• Body Fat Percentage [Срок оценки: through study completion, an average of 1 year]
• Forced Vital Capacity [Срок оценки: through study completion, an average of 1 year]
• Forced Expiratory Volume in 1 second [Срок оценки: through study completion, an average of 1 year]
• Forced Expiratory Volume in 1 second Forced Vital Capacity Ratio [Срок оценки: through study completion, an average of 1 year]
• Peak Expiratory Flow [Срок оценки: through study completion, an average of 1 year]
• Forced Expiratory Flow at 25-75% [Срок оценки: through study completion, an average of 1 year]
• Hip circumference measurement [Срок оценки: through study completion, an average of 1 year]
• upper middle arm circumference measurement [Срок оценки: through study completion, an average of 1 year]

Критерии участия

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

Exclusion Criteria for Healthy Children and Adolescents:

• Having any orthopedic, neurological, psychological or cardiovascular problem that may prevent the measurements from being performed in the last 6 months
• Having any chronic disease
• Smoking or quitting smoking

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Да

Дизайн исследования

Модель наблюдения

Случай-контроль

Центры проведения

Turkey (Türkiye) · 1 центр

• Izmir Democracy University — Izmir

Публикации

• Baptista, F., Zymbal, V., & Janz, K. F. (2022). Predictive validity of handgrip strength, vertical jump power, and plank time in the identification of pediatric sarcopenia. Measurement in Physical Education and Exercise Science, 26(4), 361-370. https://doi.org/10.1080/1091367X.2021.1987242
• Goutaki M, Halbeisen FS, Spycher BD, Maurer E, Belle F, Amirav I, Behan L, Boon M, Carr S, Casaulta C, Clement A, Crowley S, Dell S, Ferkol T, Haarman EG, Karadag B, Knowles M, Koerner-Rettberg C, Leigh MW, Loebinger MR, Mazurek H, Morgan L, Nielsen KG, Phillipsen M, Sagel SD, Santamaria F, Schwerk N, Yiallouros P, Lucas JS, Kuehni CE; PCD Israeli Consortium; Swiss PCD Group; French Reference Cent PMID 29269581
• Denizoglu Kulli H, Gurses HN, Zeren M, Ucgun H, Cakir E. Do pulmonary and extrapulmonary features differ among cystic fibrosis, primary ciliary dyskinesia, and healthy children? Pediatr Pulmonol. 2020 Nov;55(11):3067-3073. doi: 10.1002/ppul.25052. Epub 2020 Sep 11. PMID 32877003
• Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Tana-Aslan A. Respiratory muscle strength, exercise capacity and physical activity in patients with primary ciliary dyskinesia: A cross-sectional study. Respir Med. 2022 Jan;191:106719. doi: 10.1016/j.rmed.2021.106719. Epub 2021 Dec 17. PMID 34952415

Идентификаторы

NCT: NCT07740551 · Sarcopenia in PCD

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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