Malnutrition and Sarcopenia Among Primary Ciliary Dyskinesia (Malnutrition)
28 juli 2026 uppdaterad 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.
Studieöversikt
Status
Har inte rekryterat ännu
Betingelser
Intervention / Behandling
Detaljerad beskrivning
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.
Studietyp
Observationell
Inskrivning (Beräknad)
15
Kontakter och platser
Det här avsnittet innehåller kontaktuppgifter för dem som genomför studien och information om var denna studie genomförs.
Studiekontakt
- Namn: Kerim Kaan K GÖKÜSTÜN, Dr. Lecturer
- Telefonnummer: +90 232 299 07 39
- E-post: kerimkaangokustun@hotmail.com
Studera Kontakt Backup
- Namn: Gülşah BARĞI, Assoc. Dr.
- Telefonnummer: +90 232 299 07 39
- E-post: gulsahbargi35@gmail.com
Studieorter
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Izmir, Turkiet (Türkiye), 35140
- Izmir Democracy University
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Kontakt:
- Kerim K GÖKÜSTÜN, Dr. Lecturer
- Telefonnummer: 05058699032
- E-post: kerimkaangokustun@hotmail.com
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Kontakt:
- Gülşah BARĞI, Assoc. Dr.
- E-post: gulsahbargi35@gmail.com
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Deltagandekriterier
Forskare letar efter personer som passar en viss beskrivning, så kallade behörighetskriterier. Några exempel på dessa kriterier är en persons allmänna hälsotillstånd eller tidigare behandlingar.
Urvalskriterier
Åldrar som är berättigade till studier
- Barn
- Vuxen
Tar emot friska volontärer
Nej
Testmetod
Icke-sannolikhetsprov
Studera befolkning
The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).
Beskrivning
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
Det här avsnittet ger detaljer om studieplanen, inklusive hur studien är utformad och vad studien mäter.
Hur är studien utformad?
Designdetaljer
Kohorter och interventioner
Grupp / Kohort |
Intervention / 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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Vad mäter studien?
Primära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
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Handgrip Muscle Strength in Participants
Tidsram: 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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Sekundära resultatmått
Resultatmått |
Åtgärdsbeskrivning |
Tidsram |
|---|---|---|
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Malnutrition risk score in Participants
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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
Tidsram: 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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Samarbetspartners och utredare
Det är här du hittar personer och organisationer som är involverade i denna studie.
Sponsor
Utredare
- Huvudutredare: Gülşah BARĞI, Assoc. Dr., Izmir Democracy University
- Studierektor: Kerim K Göküstün, Dr. Lecturer, Izmir Democracy University
- Huvudutredare: Aybüke Sena DEMİR, MSc., Izmir Democracy University
- Huvudutredare: Ece OCAK, Dr., University of Health science
Publikationer och användbara länkar
Den som ansvarar för att lägga in information om studien tillhandahåller frivilligt dessa publikationer. Dessa kan handla om allt som har med studien att göra.
Allmänna publikationer
- 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.
Studieavstämningsdatum
Dessa datum spårar framstegen för inlämningar av studieposter och sammanfattande resultat till ClinicalTrials.gov. Studieposter och rapporterade resultat granskas av National Library of Medicine (NLM) för att säkerställa att de uppfyller specifika kvalitetskontrollstandarder innan de publiceras på den offentliga webbplatsen.
Studera stora datum
Studiestart (Beräknad)
3 augusti 2026
Primärt slutförande (Beräknad)
1 juli 2027
Avslutad studie (Beräknad)
1 juli 2028
Studieregistreringsdatum
Först inskickad
23 juli 2026
Först inskickad som uppfyllde QC-kriterierna
28 juli 2026
Första postat (Faktisk)
31 juli 2026
Uppdateringar av studier
Senaste uppdatering publicerad (Faktisk)
31 juli 2026
Senaste inskickade uppdateringen som uppfyllde QC-kriterierna
28 juli 2026
Senast verifierad
1 juli 2026
Mer information
Termer relaterade till denna studie
Ytterligare relevanta MeSH-villkor
- Ciliopatier
- Neurologiska manifestationer
- Sjukdomar i nervsystemet
- Neuromuskulära manifestationer
- Näringsstörningar
- Patologiska tillstånd, anatomiska
- Genetiska sjukdomar, medfödda
- Luftvägssjukdomar
- Medfödda abnormiteter
- Otorhinolaryngologiska sjukdomar
- Avvikelser, flera
- Muskelatrofi
- Atrofi
- Medfödda, ärftliga och neonatala sjukdomar och abnormiteter
- Patologiska tillstånd, tecken och symtom
- Närings- och metabola sjukdomar
- Tecken och symtom
- Undernäring
- Ciliär motilitetsstörning
- Sarkopeni
Andra studie-ID-nummer
- Primary Ciliary Dyskinesia
Plan för individuella deltagardata (IPD)
Planerar du att dela individuella deltagardata (IPD)?
NEJ
Läkemedels- och apparatinformation, studiedokument
Studerar en amerikansk FDA-reglerad läkemedelsprodukt
Nej
Studerar en amerikansk FDA-reglerad produktprodukt
Nej
Denna information hämtades direkt från webbplatsen clinicaltrials.gov utan några ändringar. Om du har några önskemål om att ändra, ta bort eller uppdatera dina studieuppgifter, vänligen kontakta register@clinicaltrials.gov. Så snart en ändring har implementerats på clinicaltrials.gov, kommer denna att uppdateras automatiskt även på vår webbplats .