Malnutrition and Sarcopenia Among Primary Ciliary Dyskinesia (Malnutrition)
28. juli 2026 oppdatert av: 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.
Studieoversikt
Status
Har ikke rekruttert ennå
Forhold
Intervensjon / Behandling
Detaljert beskrivelse
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.
Studietype
Observasjonsmessig
Registrering (Antatt)
15
Kontakter og plasseringer
Denne delen inneholder kontaktinformasjon for de som utfører studien, og informasjon om hvor denne studien blir utført.
Studiekontakt
- Navn: Kerim Kaan K GÖKÜSTÜN, Dr. Lecturer
- Telefonnummer: +90 232 299 07 39
- E-post: kerimkaangokustun@hotmail.com
Studer Kontakt Backup
- Navn: Gülşah BARĞI, Assoc. Dr.
- Telefonnummer: +90 232 299 07 39
- E-post: gulsahbargi35@gmail.com
Studiesteder
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Izmir, Tyrkia (Türkiye), 35140
- Izmir Democracy University
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Ta kontakt med:
- Kerim K GÖKÜSTÜN, Dr. Lecturer
- Telefonnummer: 05058699032
- E-post: kerimkaangokustun@hotmail.com
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Ta kontakt med:
- Gülşah BARĞI, Assoc. Dr.
- E-post: gulsahbargi35@gmail.com
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Deltakelseskriterier
Forskere ser etter personer som passer til en bestemt beskrivelse, kalt kvalifikasjonskriterier. Noen eksempler på disse kriteriene er en persons generelle helsetilstand eller tidligere behandlinger.
Kvalifikasjonskriterier
Alder som er kvalifisert for studier
- Barn
- Voksen
Tar imot friske frivillige
Nei
Prøvetakingsmetode
Ikke-sannsynlighetsprøve
Studiepopulasjon
The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).
Beskrivelse
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
Studieplan
Denne delen gir detaljer om studieplanen, inkludert hvordan studien er utformet og hva studien måler.
Hvordan er studiet utformet?
Designdetaljer
Kohorter og intervensjoner
Gruppe / Kohort |
Intervensjon / Behandling |
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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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Hva måler studien?
Primære resultatmål
Resultatmål |
Tiltaksbeskrivelse |
Tidsramme |
|---|---|---|
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Handgrip Muscle Strength in Participants
Tidsramme: 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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Sekundære resultatmål
Resultatmål |
Tiltaksbeskrivelse |
Tidsramme |
|---|---|---|
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Malnutrition risk score in Participants
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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
Tidsramme: 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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Samarbeidspartnere og etterforskere
Det er her du vil finne personer og organisasjoner som er involvert i denne studien.
Sponsor
Etterforskere
- Hovedetterforsker: Gülşah BARĞI, Assoc. Dr., Izmir Democracy University
- Studieleder: Kerim K Göküstün, Dr. Lecturer, Izmir Democracy University
- Hovedetterforsker: Aybüke Sena DEMİR, MSc., Izmir Democracy University
- Hovedetterforsker: Ece OCAK, Dr., University of Health science
Publikasjoner og nyttige lenker
Den som er ansvarlig for å legge inn informasjon om studien leverer frivillig disse publikasjonene. Disse kan handle om alt relatert til studiet.
Generelle publikasjoner
- 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.
Studierekorddatoer
Disse datoene sporer fremdriften for innsending av studieposter og sammendragsresultater til ClinicalTrials.gov. Studieposter og rapporterte resultater gjennomgås av National Library of Medicine (NLM) for å sikre at de oppfyller spesifikke kvalitetskontrollstandarder før de legges ut på det offentlige nettstedet.
Studer hoveddatoer
Studiestart (Antatt)
3. august 2026
Primær fullføring (Antatt)
1. juli 2027
Studiet fullført (Antatt)
1. juli 2028
Datoer for studieregistrering
Først innsendt
23. juli 2026
Først innsendt som oppfylte QC-kriteriene
28. juli 2026
Først lagt ut (Faktiske)
31. juli 2026
Oppdateringer av studieposter
Sist oppdatering lagt ut (Faktiske)
31. juli 2026
Siste oppdatering sendt inn som oppfylte QC-kriteriene
28. juli 2026
Sist bekreftet
1. juli 2026
Mer informasjon
Begreper knyttet til denne studien
Ytterligere relevante MeSH-vilkår
- Ciliopatier
- Nevrologiske manifestasjoner
- Sykdommer i nervesystemet
- Nevromuskulære manifestasjoner
- Ernæringsforstyrrelser
- Patologiske tilstander, anatomiske
- Genetiske sykdommer, medfødte
- Sykdommer i luftveiene
- Medfødte abnormiteter
- Otorhinolaryngologiske sykdommer
- Abnormiteter, flere
- Muskelatrofi
- Atrofi
- Medfødte, arvelige og neonatale sykdommer og abnormiteter
- Patologiske tilstander, tegn og symptomer
- Ernæringsmessige og metabolske sykdommer
- Tegn og symptomer
- Underernæring
- Ciliære motilitetsforstyrrelser
- Sarkopeni
Andre studie-ID-numre
- Primary Ciliary Dyskinesia
Plan for individuelle deltakerdata (IPD)
Planlegger du å dele individuelle deltakerdata (IPD)?
NEI
Legemiddel- og utstyrsinformasjon, studiedokumenter
Studerer et amerikansk FDA-regulert medikamentprodukt
Nei
Studerer et amerikansk FDA-regulert enhetsprodukt
Nei
Denne informasjonen ble hentet direkte fra nettstedet clinicaltrials.gov uten noen endringer. Hvis du har noen forespørsler om å endre, fjerne eller oppdatere studiedetaljene dine, vennligst kontakt register@clinicaltrials.gov. Så snart en endring er implementert på clinicaltrials.gov, vil denne også bli oppdatert automatisk på nettstedet vårt. .