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Clearance in Primary Ciliary Dyskinesia (CLIP)

9. juli 2026 opdateret af: University of North Carolina, Chapel Hill

Evaluating Non-ciliary Mechanisms of Airway Clearance in Muco-obstructive Lung Disease

Understanding mechanisms of mucus clearance from the airways may lead to treatments in muco-obstructive lung conditions. Mucociliary clearance (MCC) scans are a way to measure the rate at which a person's lungs can clear inhaled particles. Through a single-arm pilot study in participants with primary ciliary dyskinesia (PCD), the investigators will evaluate the effect of exercise as a way to increase clearance through a mechanism called gas liquid transport (GLT) where the faster flow of air through the lungs during exercise is able to clear more particles. The investigators will repeat MCC measures with exercise after administering hypertonic saline (HS) to evaluate the effects of mucus hydration on both GLT and cough clearance (CC).

Aim 1. To quantify non-ciliary, non-cough mechanisms of mucus clearance (i.e., GLT) in PCD through an MCC protocol that introduces a standardized exercise intervention after isotope delivery.

Aim 2. To evaluate whether exercise-induced GLT and/or CC are responsive to hydrator intervention, using a short acting bronchodilator with 7% HS prior to isotope delivery followed by MCC with exercise intervention.

Studieoversigt

Status

Ikke rekrutterer endnu

Betingelser

Intervention / Behandling

Detaljeret beskrivelse

The investigators will perform an open label, single-arm interventional pilot study with all participants undergoing the interventions (exercise, and exercise with HS pretreatment) and serving as their own control to evaluate the effects of exercise on non-ciliary mucus clearance mechanisms in PCD to better identify role of GLT and CC. The investigators will also evaluate the effect of pretreatment with HS on GLT (if present) and CC, to determine if these mechanisms of mucus clearance are modifiable by future therapies in PCD and other muco-obstructive lung diseases.

Patients will be asked to attend a total of 3 study visits: screening visit 1 (V1), baseline exercise MCC (visit 2 (V2) or visit 3 (V3)), and HS pretreatment MCC (V2 or V3). Patients will be randomized to perform their baseline exercise MCC scan first or their HS pretreatment MCC scan first to minimize introduction of bias. The time between V1 and V2 will be 3 days to 28 days to allow for flexibility of scheduling, but participants already prescribed HS will be required to undergo a washout period of at least 3 days. V3 will take place at least 3 days after V2 to allow for remaining MCC isotope washout. At V1, tolerability and change in airway caliber in response to HS will be assessed by symptom assessment and spirometry, along with the performance of an exercise assessment to inform the exercise protocol in V2 and V3. From the baseline exercise MCC visit, the primary outcome of Whole Lung Total GLT will be measured. Through comparing the data from the exercise MCC visit and the HS pretreatment MCC visit, change in Whole Lung Total GLT and CC will be determined.

Undersøgelsestype

Interventionel

Tilmelding (Anslået)

8

Fase

  • Ikke anvendelig

Kontakter og lokationer

Dette afsnit indeholder kontaktoplysninger for dem, der udfører undersøgelsen, og oplysninger om, hvor denne undersøgelse udføres.

Studiekontakt

Undersøgelse Kontakt Backup

Studiesteder

Deltagelseskriterier

Forskere leder efter personer, der passer til en bestemt beskrivelse, kaldet berettigelseskriterier. Nogle eksempler på disse kriterier er en persons generelle helbredstilstand eller tidligere behandlinger.

Berettigelseskriterier

Aldre berettiget til at studere

  • Voksen
  • Ældre voksen

Tager imod sunde frivillige

Ingen

Beskrivelse

Inclusion Criteria:

  • 18 years old or older
  • Diagnosis of PCD with supporting genetics
  • Lung function with forced expiratory volume in 1 second (FEV1) percent predicted >30%
  • Ability to exercise for 20 minutes (Exercise Assessment performed at screening visit)

Exclusion Criteria:

  • Intolerance to 7% HS
  • Failure of HS tolerance test (HSTT) at visit 1 (V1), defined as a decline of 10% in FEV1 from baseline pre-bronchodilator (BD) FEV1 after 7% HS
  • Intolerance to levalbuterol
  • Exercise-induced asthma
  • Complex congenital heart disease or other exercise limitation
  • Lung transplantation or listed for lung transplantation
  • History of lung resection of the lung analyzed by MCC (depending on situs status)
  • Pregnancy or unwillingness to use approved birth control during study period
  • Recent hemoptysis (>60 ml in the last 3 months)
  • Pulmonary exacerbation in preceding 4 weeks
  • Change in pulmonary therapies within preceding 4 weeks
  • Resting or exertional O2 requirement (nocturnal acceptable)
  • Tobacco / smoking history (any within last year, and >10 pack year history in past)
  • More than 2 chest computed tomography (CT) scans in the past year or a combination of procedures believed to have exposed the lungs to >150 millisieverts (mSv), including from other research studies
  • Participation in another interventional trial within 30 days

Studieplan

Dette afsnit indeholder detaljer om studieplanen, herunder hvordan undersøgelsen er designet, og hvad undersøgelsen måler.

Hvordan er undersøgelsen tilrettelagt?

Design detaljer

  • Primært formål: Behandling
  • Tildeling: N/A
  • Interventionel model: Enkelt gruppeopgave
  • Maskning: Ingen (Åben etiket)

Våben og indgreb

Deltagergruppe / Arm
Intervention / Behandling
Eksperimentel: Exercise with and without HS pretreatment
All participants will undergo interventions of exercise alone, and exercise with HS pretreatment, in two separate study visits according to randomization to intervention sequence.
Adfærdsmæssigt: Exercise
At either V2 or V3, after 16 minutes of baseline MCC data collection, participants will perform 20 minutes of moderate-intensity exercise based on the Borg Rating of Perceived Exertion (RPE) scale, on a cycle ergometer. The goal through this moderate intensity exercise is to increase respiratory rate and airflow velocity, thereby potentially inducing GLT. Participants will then resume MCC scan data collection post-exercise to continue to measure the effects induced by exercise on clearance from the lungs.
Andet: HS pretreatment with Exercise
At either V2 or V3, prior to the start of baseline MCC scanning, participants will undergo pretreatment with 4 puffs of inhaled levalbuterol (90 mcg per actuation, via spacer) followed by 4 ml of 7% HS via nebulizer. Participants will then start MCC scan data collection, and after 16 minutes of baseline MCC collection they will then complete 20 minutes of moderate intensity exercise according to the Borg RPE on a cycle ergometer. After 20 minutes, MCC scan will resume to continue to measure rates of clearance following HS pretreatment and exercise.

Hvad måler undersøgelsen?

Primære resultatmål

Resultatmål
Foranstaltningsbeskrivelse
Tidsramme
Whole Lung Total GLT
Tidsramme: Either Day 1 (Visit 2) or up to Day 28 (Visit 3) depending on randomization to sequence intervention
Through MCC scan with built in exercise protocol, the investigators will measure the effects of exercise on rates of clearance of radiolabeled isotopes from the lungs, thought to represent GLT. The investigators will measure the difference between retained particles from immediately before (after collection of 16 minutes of baseline MCC data through eight 2-minute images) and immediately after 20 minutes of moderate intensity exercise on a cycle ergometer (40 minutes after start of MCC data collection). This will represent Active GLT. The investigators will then resume measurement of MCC clearance rate by serial 2-minute image collection of the whole lung from 40 minutes to 70 minutes. This will represent Sustained GLT. The sum of Active and Sustained GLT will represent Whole Lung GLT. This measurement will occur at either Visit 2 or Visit 3, depending on visit sequence randomization.
Either Day 1 (Visit 2) or up to Day 28 (Visit 3) depending on randomization to sequence intervention
Change in Whole Lung Total GLT after HS pretreatment
Tidsramme: Day 3 to 28, depending on timing of Visit 3
After pretreatment with levalbuterol and 7% HS, participants will undergo an MCC scan with built in exercise protocol. The investigators will measure the effects of exercise on rates of clearance of radiolabeled isotopes from the lungs, thought to represent GLT. The investigators will measure Active GLT and Sustained GLT after HS pretreatment, which taken together will represent Whole Lung GLT after HS pretreatment. This measurement will occur at either Visit 2 or Visit 3 depending on visit sequence randomization. The difference between Whole Lung GLT from exercise alone will be compared with the HS pretreatment Whole Lung GLT.
Day 3 to 28, depending on timing of Visit 3
Change in Cough Clearance (CC) after HS Pretreatment
Tidsramme: Day 3 to 28, depending on timing of Visit 3
The investigators will measure the effects of exercise and HS pretreatment on rates of clearance of radiolabeled isotopes from the lungs during a period of controlled coughs at regular intervals. During Visit 2 and Visit 3 MCC scans, from minutes 70 through 100 of MCC data collection, participants will be instructed to perform 30 voluntary huff-cough maneuvers while serial 2-minute images are obtained. The change in clearance during this time will represent CC. At one of these visits (either Visit 2 or Visit 3 depending on randomization to intervention sequence), participants will undergo pretreatment with levalbuterol and 7% HS prior to the MCC scan with exercise protocol. The difference between CC after exercise alone and CC after exercise with HS pretreatment will be measured.
Day 3 to 28, depending on timing of Visit 3

Samarbejdspartnere og efterforskere

Det er her, du vil finde personer og organisationer, der er involveret i denne undersøgelse.

Sponsor

University of North Carolina, Chapel Hill

Samarbejdspartnere

Cystic Fibrosis Foundation

Efterforskere

  • Ledende efterforsker: Katherine A Despotes, MD, University of North Carolina, Chapel Hill

Datoer for undersøgelser

Disse datoer sporer fremskridtene for indsendelser af undersøgelsesrekord og resumeresultater til ClinicalTrials.gov. Studieregistreringer og rapporterede resultater gennemgås af National Library of Medicine (NLM) for at sikre, at de opfylder specifikke kvalitetskontrolstandarder, før de offentliggøres på den offentlige hjemmeside.

Studer store datoer

Studiestart (Anslået)

30. juli 2026

Primær færdiggørelse (Anslået)

1. januar 2027

Studieafslutning (Anslået)

1. januar 2027

Datoer for studieregistrering

Først indsendt

29. juni 2026

Først indsendt, der opfyldte QC-kriterier

9. juli 2026

Først opslået (Faktiske)

13. juli 2026

Opdateringer af undersøgelsesjournaler

Sidste opdatering sendt (Faktiske)

13. juli 2026

Sidste opdatering indsendt, der opfyldte kvalitetskontrolkriterier

9. juli 2026

Sidst verificeret

1. juni 2026

Mere information

Begreber relateret til denne undersøgelse

Nøgleord

Yderligere relevante MeSH-vilkår

Andre undersøgelses-id-numre

  • 25-1650

Plan for individuelle deltagerdata (IPD)

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INGEN

IPD-planbeskrivelse

The investigators will not share individual participant data (IPD) with other researchers to protect patient confidentiality.

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