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Malnutrition and Sarcopenia Among Primary Ciliary Dyskinesia (Malnutrition)

tiistai 28. heinäkuuta 2026 päivittänyt: 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.

Tutkimuksen yleiskatsaus

Yksityiskohtainen kuvaus

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.

Opintotyyppi

Havainnollistava

Ilmoittautuminen (Arvioitu)

15

Yhteystiedot ja paikat

Tässä osiossa on tutkimuksen suorittajien yhteystiedot ja tiedot siitä, missä tämä tutkimus suoritetaan.

Opiskeluyhteys

Tutki yhteystietojen varmuuskopiointi

Opiskelupaikat

Osallistumiskriteerit

Tutkijat etsivät ihmisiä, jotka sopivat tiettyyn kuvaukseen, jota kutsutaan kelpoisuuskriteereiksi. Joitakin esimerkkejä näistä kriteereistä ovat henkilön yleinen terveydentila tai aiemmat hoidot.

Kelpoisuusvaatimukset

Opintokelpoiset iät

  • Lapsi
  • Aikuinen

Hyväksyy terveitä vapaaehtoisia

Ei

Näytteenottomenetelmä

Ei-todennäköisyysnäyte

Tutkimusväestö

The study will include children and adolescents aged 6-18 years with Primary Ciliary Dyskinesia (PCD).

Kuvaus

Inclusion Criteria:

Inclusion criteria for children and adolescents with PCD

  1. 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.
  2. Cooperative individuals,
  3. Individuals who have never smoked
  4. 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:

  1. Individuals with any acute, chronic, or systemic illness other than PCD
  2. Individuals who smoke or are quitting smoking
  3. Individuals who are not willing to participate in the study
  4. Individuals who are uncooperative

Opintosuunnitelma

Tässä osiossa on tietoja tutkimussuunnitelmasta, mukaan lukien kuinka tutkimus on suunniteltu ja mitä tutkimuksella mitataan.

Miten tutkimus on suunniteltu?

Suunnittelun yksityiskohdat

Kohortit ja interventiot

Ryhmä/Kohortti
Interventio / Hoito
Patient of Primary Ciliary Dyskinesia
The study will include children and adolescents with PCD aged 6 to 18 (n=15).
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.

Mitä tutkimuksessa mitataan?

Ensisijaiset tulostoimenpiteet

Tulosmittaus
Toimenpiteen kuvaus
Aikaikkuna
Handgrip Muscle Strength in Participants
Aikaikkuna: From enrollment to the end of treatment at 1 year
Bilateral grip strength in children and adolescents with PCD will be measured in kgF using a hand dynamometer.
From enrollment to the end of treatment at 1 year

Toissijaiset tulostoimenpiteet

Tulosmittaus
Toimenpiteen kuvaus
Aikaikkuna
Malnutrition risk score in Participants
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year
Body Weight
Aikaikkuna: From enrollment to the end of treatment at 1 year
Body weight in children and adolescents with PCD will be measured using a pediatric scale.
From enrollment to the end of treatment at 1 year
Height
Aikaikkuna: From enrollment to the end of treatment at 1 year
In children and adolescents with PCD, height will be measured using a stadiometer.
From enrollment to the end of treatment at 1 year
Body Mass Index
Aikaikkuna: From enrollment to the end of treatment at 1 year
For children and adolescents with PCD, the body mass index will be calculated by dividing body weight by the square of height in meters.
From enrollment to the end of treatment at 1 year
Waist circumference
Aikaikkuna: From enrollment to the end of treatment at 1 year
Waist circumference in children and adolescents with PCD will be measured using a tape measure
From enrollment to the end of treatment at 1 year
Hip circumference
Aikaikkuna: From enrollment to the end of treatment at 1 year
Hip circumference in children and adolescents with PCD will be measured using a tape measure
From enrollment to the end of treatment at 1 year
Waist-to-hip ratio
Aikaikkuna: From enrollment to the end of treatment at 1 year
In children and adolescents with PCD, the waist-to-hip ratio will be calculated by dividing the waist circumference by the hip circumference.
From enrollment to the end of treatment at 1 year
Waist-to-Height ratio
Aikaikkuna: From enrollment to the end of treatment at 1 year
In children and adolescents with PCD, the waist-to-height ratio will be calculated by dividing the waist circumference by the height.
From enrollment to the end of treatment at 1 year
Upper-middle arm circumference
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year
Triceps skinfold thickness
Aikaikkuna: From enrollment to the end of treatment at 1 year
In children and adolescents with PCD, triceps skinfold thickness will be measured using a skinfold caliper.
From enrollment to the end of treatment at 1 year
Body fat percentage
Aikaikkuna: From enrollment to the end of treatment at 1 year
Body fat percentage in children and adolescents with PCD will be measured using bioelectrical impedance analysis.
From enrollment to the end of treatment at 1 year
Height by Age
Aikaikkuna: From enrollment to the end of treatment at 1 year
The percentile values for height by age in children and adolescents with PCD will be determined using data from the CDC's website.
From enrollment to the end of treatment at 1 year
Weight by Height
Aikaikkuna: From enrollment to the end of treatment at 1 year
The percentile values for Weight by Height in children and adolescents with PCD will be determined using data from the CDC's website.
From enrollment to the end of treatment at 1 year
Weight by Age
Aikaikkuna: From enrollment to the end of treatment at 1 year
The percentile values for Weight by Age in children and adolescents with PCD will be determined using data from the CDC's website.
From enrollment to the end of treatment at 1 year
Body Mass Index by Age
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year
Forced Expiratory Volume at One Second
Aikaikkuna: From enrollment to the end of treatment at 1 year
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).
From enrollment to the end of treatment at 1 year
Forced Vital Capacity
Aikaikkuna: From enrollment to the end of treatment at 1 year
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).
From enrollment to the end of treatment at 1 year
Forced Expiratory Volume at One Secondto Forced Vital Capacity Ratio
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year
Peak Expiratory Flow
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year
Forced Expiratory Flow at 25-75% of Forced Vital Capacity
Aikaikkuna: From enrollment to the end of treatment at 1 year
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.
From enrollment to the end of treatment at 1 year

Yhteistyökumppanit ja tutkijat

Täältä löydät tähän tutkimukseen osallistuvat ihmiset ja organisaatiot.

Tutkijat

  • Päätutkija: Gülşah BARĞI, Assoc. Dr., Izmir Democracy University
  • Opintojohtaja: Kerim K Göküstün, Dr. Lecturer, Izmir Democracy University
  • Päätutkija: Aybüke Sena DEMİR, MSc., Izmir Democracy University
  • Päätutkija: Ece OCAK, Dr., University of Health science

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Opintojen ennätyspäivät

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