Malnutrition and Sarcopenia Among Primary Ciliary Dyskinesia (Malnutrition)
28. července 2026 aktualizováno: 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.
Přehled studie
Postavení
Zatím nenabíráme
Podmínky
Intervence / Léčba
Detailní popis
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.
Typ studie
Pozorovací
Zápis (Odhadovaný)
15
Kontakty a umístění
Tato část poskytuje kontaktní údaje pro ty, kteří studii provádějí, a informace o tom, kde se tato studie provádí.
Studijní kontakt
- Jméno: Kerim Kaan K GÖKÜSTÜN, Dr. Lecturer
- Telefonní číslo: +90 232 299 07 39
- E-mail: kerimkaangokustun@hotmail.com
Studijní záloha kontaktů
- Jméno: Gülşah BARĞI, Assoc. Dr.
- Telefonní číslo: +90 232 299 07 39
- E-mail: gulsahbargi35@gmail.com
Studijní místa
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Izmir, Turecko (Türkiye), 35140
- Izmir Democracy University
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Kontakt:
- Kerim K GÖKÜSTÜN, Dr. Lecturer
- Telefonní číslo: 05058699032
- E-mail: kerimkaangokustun@hotmail.com
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Kontakt:
- Gülşah BARĞI, Assoc. Dr.
- E-mail: gulsahbargi35@gmail.com
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Kritéria účasti
Výzkumníci hledají lidi, kteří odpovídají určitému popisu, kterému se říká kritéria způsobilosti. Některé příklady těchto kritérií jsou celkový zdravotní stav osoby nebo předchozí léčba.
Kritéria způsobilosti
Věk způsobilý ke studiu
- Dítě
- Dospělý
Přijímá zdravé dobrovolníky
Ne
Metoda odběru vzorků
Vzorek nepravděpodobnosti
Studijní populace
The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).
Popis
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
Studijní plán
Tato část poskytuje podrobnosti o studijním plánu, včetně toho, jak je studie navržena a co studie měří.
Jak je studie koncipována?
Detaily designu
Kohorty a intervence
Skupina / kohorta |
Intervence / Léčba |
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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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Co je měření studie?
Primární výstupní opatření
Měření výsledku |
Popis opatření |
Časové okno |
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Handgrip Muscle Strength in Participants
Časové okno: 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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Sekundární výstupní opatření
Měření výsledku |
Popis opatření |
Časové okno |
|---|---|---|
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Malnutrition risk score in Participants
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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
Časové okno: 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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Spolupracovníci a vyšetřovatelé
Zde najdete lidi a organizace zapojené do této studie.
Sponzor
Vyšetřovatelé
- Vrchní vyšetřovatel: Gülşah BARĞI, Assoc. Dr., Izmir Democracy University
- Ředitel studie: Kerim K Göküstün, Dr. Lecturer, Izmir Democracy University
- Vrchní vyšetřovatel: Aybüke Sena DEMİR, MSc., Izmir Democracy University
- Vrchní vyšetřovatel: Ece OCAK, Dr., University of Health science
Publikace a užitečné odkazy
Osoba odpovědná za zadávání informací o studiu tyto publikace poskytuje dobrovolně. Mohou se týkat čehokoli, co souvisí se studiem.
Obecné publikace
- 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.
Termíny studijních záznamů
Tato data sledují průběh záznamů studie a předkládání souhrnných výsledků na ClinicalTrials.gov. Záznamy ze studií a hlášené výsledky jsou před zveřejněním na veřejné webové stránce přezkoumány Národní lékařskou knihovnou (NLM), aby se ujistily, že splňují specifické standardy kontroly kvality.
Hlavní termíny studia
Začátek studia (Odhadovaný)
3. srpna 2026
Primární dokončení (Odhadovaný)
1. července 2027
Dokončení studie (Odhadovaný)
1. července 2028
Termíny zápisu do studia
První předloženo
23. července 2026
První předloženo, které splnilo kritéria kontroly kvality
28. července 2026
První zveřejněno (Aktuální)
31. července 2026
Aktualizace studijních záznamů
Poslední zveřejněná aktualizace (Aktuální)
31. července 2026
Odeslaná poslední aktualizace, která splnila kritéria kontroly kvality
28. července 2026
Naposledy ověřeno
1. července 2026
Více informací
Termíny související s touto studií
Další relevantní podmínky MeSH
- Ciliopatie
- Neurologické projevy
- Nemoci nervového systému
- Neuromuskulární projevy
- Poruchy výživy
- Patologické stavy, anatomické
- Genetické choroby, vrozené
- Nemoci dýchacích cest
- Vrozené vady
- Otorinolaryngologická onemocnění
- Abnormality, vícenásobné
- Svalová atrofie
- Atrofie
- Vrozené, dědičné a neonatální nemoci a abnormality
- Patologické stavy, příznaky a symptomy
- Nutriční a metabolické nemoci
- Příznaky a symptomy
- Podvýživa
- Poruchy ciliární motility
- Sarkopenie
Další identifikační čísla studie
- Primary Ciliary Dyskinesia
Plán pro data jednotlivých účastníků (IPD)
Plánujete sdílet data jednotlivých účastníků (IPD)?
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Informace o lécích a zařízeních, studijní dokumenty
Studuje lékový produkt regulovaný americkým FDA
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Studuje produkt zařízení regulovaný americkým úřadem FDA
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Tyto informace byly beze změn načteny přímo z webu clinicaltrials.gov. Máte-li jakékoli požadavky na změnu, odstranění nebo aktualizaci podrobností studie, kontaktujte prosím register@clinicaltrials.gov. Jakmile bude změna implementována na clinicaltrials.gov, bude automaticky aktualizována i na našem webu .