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.
調査の概要
詳細な説明
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.
研究の種類
観察的
入学 (推定)
15
連絡先と場所
このセクションには、調査を実施する担当者の連絡先の詳細と、この調査が実施されている場所に関する情報が記載されています。
研究連絡先
- 名前：Kerim Kaan K GÖKÜSTÜN, Dr. Lecturer
- 電話番号：+90 232 299 07 39
- メール：kerimkaangokustun@hotmail.com
研究連絡先のバックアップ
- 名前：Gülşah BARĞI, Assoc. Dr.
- 電話番号：+90 232 299 07 39
- メール：gulsahbargi35@gmail.com
研究場所
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Izmir、トルコ（Türkiye）、35140
- Izmir Democracy University
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コンタクト：
- Kerim K GÖKÜSTÜN, Dr. Lecturer
- 電話番号：05058699032
- メール：kerimkaangokustun@hotmail.com
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コンタクト：
- Gülşah BARĞI, Assoc. Dr.
- メール：gulsahbargi35@gmail.com
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-
参加基準
研究者は、適格基準と呼ばれる特定の説明に適合する人を探します。これらの基準のいくつかの例は、人の一般的な健康状態または以前の治療です。
適格基準
就学可能な年齢
- 子
- 大人
健康ボランティアの受け入れ
いいえ
サンプリング方法
非確率サンプル
調査対象母集団
The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).
説明
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
研究計画
このセクションでは、研究がどのように設計され、研究が何を測定しているかなど、研究計画の詳細を提供します。
研究はどのように設計されていますか？
デザインの詳細
コホートと介入
グループ/コホート |
介入・治療 |
|---|---|
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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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この研究は何を測定していますか？
主要な結果の測定
結果測定 |
メジャーの説明 |
時間枠 |
|---|---|---|
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Handgrip Muscle Strength in Participants
時間枠：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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二次結果の測定
結果測定 |
メジャーの説明 |
時間枠 |
|---|---|---|
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Malnutrition risk score in Participants
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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
時間枠：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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協力者と研究者
ここでは、この調査に関係する人々や組織を見つけることができます。
捜査官
- 主任研究者：Gülşah BARĞI, Assoc. Dr.、Izmir Democracy University
- スタディディレクター：Kerim K Göküstün, Dr. Lecturer、Izmir Democracy University
- 主任研究者：Aybüke Sena DEMİR, MSc.、Izmir Democracy University
- 主任研究者：Ece OCAK, Dr.、University of Health science
出版物と役立つリンク
研究に関する情報を入力する責任者は、自発的にこれらの出版物を提供します。これらは、研究に関連するあらゆるものに関するものである可能性があります。
一般刊行物
- 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.
研究記録日
これらの日付は、ClinicalTrials.gov への研究記録と要約結果の提出の進捗状況を追跡します。研究記録と報告された結果は、国立医学図書館 (NLM) によって審査され、公開 Web サイトに掲載される前に、特定の品質管理基準を満たしていることが確認されます。
主要日程の研究
研究開始 (推定)
2026年8月3日
一次修了 (推定)
2027年7月1日
研究の完了 (推定)
2028年7月1日
試験登録日
最初に提出
2026年7月23日
QC基準を満たした最初の提出物
2026年7月28日
最初の投稿 (実際)
2026年7月31日
学習記録の更新
投稿された最後の更新 (実際)
2026年7月31日
QC基準を満たした最後の更新が送信されました
2026年7月28日
最終確認日
2026年7月1日
詳しくは
本研究に関する用語
キーワード
追加の関連 MeSH 用語
その他の研究ID番号
- Primary Ciliary Dyskinesia
個々の参加者データ (IPD) の計画
個々の参加者データ (IPD) を共有する予定はありますか?
いいえ
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米国FDA規制医薬品の研究
いいえ
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いいえ
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