Cystic FibrosisNCT04242420
Connexin Genotypes in Cystic Fibrosis
A study testing Genotyping, Lung function and Microbiology in people with Cystic Fibrosis and Inflammation, run by University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn) at 3 sites in 2 countries.
Trial summary
Background: There is wide variety in lung disease phenotype for the delta F508 (homozygous) genotype. A leukocyte driven inflammation is most important for the pathogenesis of pulmonary disease in CF. Blood cytokines correlate negatively with pulmonary function in delta F508 homozygous patients. Read more about this study
- Who is it for?
- Ages 6 years and older
- Conditions the study is for
- Cystic Fibrosis, Inflammation
- What is being tested?
- Genotyping, Lung function and Microbiology
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 300 participants
- Where
- 3 sitesin 2 countries
- Study dates
- Apr 2002to 31 Dec 2024
- Phase
- Interventional
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 6 years and older
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
Who may be able to join
- Homozygosity for delta F 508
Who cannot join
- treatment with systemic steroids 14 days preceding this trial, participation in another study within the past 30 days, treatment with Orkambi or status after lung transplantation (for assessment of all parameters except survival).
Trial locations and contacts
3 sites in 2 countries.
3 sites in 2 countries
Germany2 sites, 2 recruiting
- Recruiting
University Hospital
Bonn, Nordrhine-Westphalia, 53113
Sabina Schmitt-Grohe, MD, 004915254568942, s.schmitt-grohe@ukbonn.de
- Recruiting
University Hospital
Frankfurt, Hessia, 60590
Christina Smaczny, MD, 0049-69-6301 ext. 4232, smaczny@em.uni-frankfurt.de
Netherlands1 site, 1 recruiting
- Recruiting
University Hospital
Amsterdam
Anne Neerinx-vanStuvyenberg, PhD, a.vanstuyvenbergneerincx@amc.uva.nl
About this study
Official title: Impact of Connexin 37, Connexin 43 on Clinical Disease Phenotype in Delta F508 Homozygous Patients With Cystic Fibrosis (CF) (CF-Modifier)
Background: There is wide variety in lung disease phenotype for the delta F508 (homozygous) genotype. A leukocyte driven inflammation is most important for the pathogenesis of pulmonary disease in CF. Blood cytokines correlate negatively with pulmonary function in delta F508 homozygous patients. Gap junction proteins might be of importance for the influx of blood cells into the lung and may influence the course of pulmonary inflammation. A primary analysis (Horn et al. 2020) has shown that GJA4 variants (rs41266431) are linked to more severe disease in CF. This is very similar to variants of MBL.
Aims: To assess the relationship between gap junction proteins alpha 1 (GJA1/Connexin 43) and alpha 4 (GJA4/connexin 37) genotypes and clinical disease phenotype. Moreover are GJA4 variants in terms of clinical phenotype independent of MBL variants.
Methods:Patients homozygous for delta F508 get recruited from the CF centres of Bonn, Frankfurt and Amsterdam. Sequence analysis is performed for connexin 43 and 37 and MBL genotypes. Clinical disease is assessed longitudinally over 3 years by pulmonary function tests (FEV1 (forced expiratory volume in one second), FVC (=(forced vital capacity), FEF75 % (Forced expiratory flow at 75% of the pulmonary volume) pred), BMI (percentiles), P. aeruginosa colonization, diabetes mellitus and survival to end-stage CF lung disease (death or lung transplantation).
Read the detailed description
Progressive pulmonary destruction is the major cause of morbidity and mortality in human subjects with cystic fibrosis. Many studies could not find an association between delta F 508 and severity of pulmonary disease . The most important factor in CF lung disease is an inflammation driven by leukocytes and cytokines. The investigators have provided evidence in former studies that cytokines (Interleukin-8 (IL-8), tumour necrosis factor (TNF) alpha, Lipopolysaccahride binding protein (LBP), transforming growth factor (TGF) ß)measured in blood correlate negatively with lung function in delta 508 homozygous patients.
The question arises, what other factors influence recruitment of proinflammatory leukocytes from blood capillaries into the lung .
Connexins are a family of transmembrane proteins, which oligomerize into hexameric structures to form a hemichannel (connexon) and ultimately pair with a partner hemichannel in an adjacent cell to form gap junction intercellular communication channels (GJIC) . There is evidence of expression of connexin 37 (=gap junction protein A4 (GJA4)) on macrophages in humans. Moreover there is evidence of expression of connexin 37 on vascular endothelia in humans . Connexin 37 is expressed on human neutrophils . Pulmonary disease in CF is dominated by a leukocyte driven inflammation. GAP junction proteins might be of importance for the influx of blood cells into the lung. In this regard, the hypothesis was that Cx37 or Cx43 genotypes have an impact on clinical disease phenotype in CF patients homozygous for delta F508. The first analysis (Horn et al 2020) has shown a clinical phenotype linked to the GJA4 genotype is very similar to MBL variants. In this regard the question arises whether there is a link between the MBL variant alleles and the GJA4 variants. Moreover some TGFbeta genotypes are linked to certain pulmonary phenotypes. So we are looking for interactions between Cx37 and TGFbeta genotypes and their impact on the clinical phenotype as well.
- Conditions
- Cystic Fibrosis, Inflammation
- Being tested
- Genotyping, Lung function, Microbiology, Blood sample, Induced Sputum
- Keywords
- connexin 37+43, MBL, TGF beta, Lung function, Interleukin 8
- Medical subject headings
- Cystic Fibrosis, Inflammation, Camurati-Engelmann Syndrome
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
GenotypingDiagnostic test
Genotyping for single nucleotide polymorphisms for Connexin 37\&43, Mannose binding lectin (MBL) and transforming growth factor beta (TGF beta) genotypes
Lung functionDiagnostic test
Spirometry,
MicrobiologyDiagnostic test
Bacterial culture from Sputum or swab
Blood sampleDiagnostic test
Interleukin-8 assay (via chemiluminescence) blood cell count
Induced SputumDiagnostic test
Interleukin-8 assay (via chemiluminescence) blood cell count
| Study group | Role | What they receive |
|---|---|---|
| Connexin genotype | Other | Genotyping Receives: Diagnostic Test: Genotyping; Diagnostic Test: Lung function; Diagnostic Test: Microbiology; Diagnostic Test: Blood sample; Diagnostic Test: Induced Sputum |
What is being measured
The primary outcome is the main question the study is designed to answer.
Survival to end stage lung disease
End stage lung disease: Death or lung transplantation
Measured: through study completion, on average 27 years
Lung function parameter: FEV1
Best FEV1 value in % predicted of the year according to German registry according to Global lung initiative (GLI)
Measured: 3 years
Lung function parameters: FVC
Best FVC value in % predicted of the year according to German registry according to Global lung initiative (GLI)
Measured: 3 years
Lung function parameters: FEF75
Best FEF75 value in % predicted of the year according to German registry according to Global lung initiative (GLI)
Measured: 3 years
Secondary outcomes (4)
Interleukin-8 in blood
single spot value (cross-sectional)
Measured: through study completion, an average of 3 year
Interleukin-8 in sputum
single spot value (cross-sectional)
Measured: through study completion, an average of 3 year
White blood cell count
Leukocytes (single spot value (cross-sectional))
Measured: through study completion, an average of 3 year
Inflammatory markers in sputum
Leukocyte count in sputum (single spot value (cross-sectional))
Measured: through study completion, an average of 3 year
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted27 Jan 2020
- Study startApr 2002
- Primary completion31 Dec 2023 (estimated)
- Study completion31 Dec 2024 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (14)Papers the sponsor has linked to this study.
- Other trials for Cystic FibrosisStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Model description
- Evaluation of clinical parameters by genotype
- Masking
- None (open label)
- Enrolment
- 300 participants (estimated)
Sponsor and investigators
- Lead sponsor
- University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn)
- Sponsor type
- University, hospital or other
- Collaborators
- Goethe University, Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
- Responsible party
- Principal investigator, Sabina Schmitt-Grohe, University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn)
- Investigators
- Sabina Schmitt-Grohe, MD, Principal investigator, University Hospital, Bonn
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT04242420
- Sponsor's protocol ID
- BN 178/01
- Status last verified
- Aug 2021
- First submitted
- 20 Dec 2019
- Last updated
- 16 Aug 2021
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F, Zariwala M, Fargo D, Xu A, Dunn JM, Darrah RJ, Dorfman R, Sandford AJ, Corey M, Zielenski J, Durie P, Goddard K, Yankaskas JR, Wright FA, Knowles MR; Gene Modifier Study Group. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med. 2005 Oct 6;353(14):1443-53. doi: 10.1056/NEJMoa051469.
PubMed 16207846Cystic Fibrosis Genotype-Phenotype Consortium. Correlation between genotype and phenotype in patients with cystic fibrosis. N Engl J Med. 1993 Oct 28;329(18):1308-13. doi: 10.1056/NEJM199310283291804.
PubMed 8166795McKone EF, Goss CH, Aitken ML. CFTR genotype as a predictor of prognosis in cystic fibrosis. Chest. 2006 Nov;130(5):1441-7. doi: 10.1378/chest.130.5.1441.
PubMed 17099022Schmitt-Grohe S, Naujoks C, Bargon J, Wagner TO, Schubert R, Hippe V, Zielen S. Interleukin-8 in whole blood and clinical status in cystic fibrosis. Cytokine. 2005 Jan 7;29(1):18-23. doi: 10.1016/j.cyto.2004.09.004.
PubMed 15579374Schmitt-Grohe S, Hippe V, Igel M, von Bergmann K, Posselt HG, Krahl A, Smaczny C, Wagner TO, Nikolazik W, Schubert R, Lentze MJ, Zielen S. Lipopolysaccharide binding protein, cytokine production in whole blood, and lipoproteins in cystic fibrosis. Pediatr Res. 2005 Nov;58(5):903-7. doi: 10.1203/01.PDR.0000182598.98167.24. Epub 2005 Sep 23.
PubMed 16183806Schmitt-Grohe S, Stuber F, Book M, Bargon J, Wagner TO, Naujoks C, Schubert R, Lentze MJ, Zielen S. TNF-alpha promoter polymorphism in relation to TNF-alpha production and clinical status in cystic fibrosis. Lung. 2006 Mar-Apr;184(2):99-104. doi: 10.1007/s00408-005-2568-x.
PubMed 16622779Eickmeier O, Boom Lv, Schreiner F, Lentze MJ, NGampolo D, Schubert R, Zielen S, Schmitt-Grohe S. Transforming growth factor beta1 genotypes in relation to TGFbeta1, interleukin-8, and tumor necrosis factor alpha in induced sputum and blood in cystic fibrosis. Mediators Inflamm. 2013;2013:913135. doi: 10.1155/2013/913135. Epub 2013 Aug 26.
PubMed 24062613Dempsie Y, Martin P, Upton PD. Connexin-mediated regulation of the pulmonary vasculature. Biochem Soc Trans. 2015 Jun;43(3):524-9. doi: 10.1042/BST20150030.
PubMed 26009202Chanson M, Derouette JP, Roth I, Foglia B, Scerri I, Dudez T, Kwak BR. Gap junctional communication in tissue inflammation and repair. Biochim Biophys Acta. 2005 Jun 10;1711(2):197-207. doi: 10.1016/j.bbamem.2004.10.005. Epub 2004 Oct 30.
PubMed 15955304Kwak BR, Mulhaupt F, Veillard N, Gros DB, Mach F. Altered pattern of vascular connexin expression in atherosclerotic plaques. Arterioscler Thromb Vasc Biol. 2002 Feb 1;22(2):225-30. doi: 10.1161/hq0102.104125.
PubMed 11834520Zahler S, Hoffmann A, Gloe T, Pohl U. Gap-junctional coupling between neutrophils and endothelial cells: a novel modulator of transendothelial migration. J Leukoc Biol. 2003 Jan;73(1):118-26. doi: 10.1189/jlb.0402184.
PubMed 12525569Saez PJ, Shoji KF, Aguirre A, Saez JC. Regulation of hemichannels and gap junction channels by cytokines in antigen-presenting cells. Mediators Inflamm. 2014;2014:742734. doi: 10.1155/2014/742734. Epub 2014 Sep 9.
PubMed 25301274Horn T, Ludwig M, Eickmeier O, Neerinex AH, Maitland-van der Zee AH, Smaczny C, Wagner TOF, Schubert R, Zielen S, Majoor C, Bos LD, Schmitt-Grohe S. Impact of a Gap Junction Protein Alpha 4 Variant on Clinical Disease Phenotype in F508del Homozygous Patients With Cystic Fibrosis. Front Genet. 2020 Oct 28;11:570403. doi: 10.3389/fgene.2020.570403. eCollection 2020.
PubMed 33193670Laubach JP, Ludwig M, Horn T, Eickmeier O, Smaczny C, Schubert R, Zielen S, Majoor C, Aydin M, Schnell A, Schmitt-Grohe S. Transforming Growth Factor ss1 and Gap Junction Protein Alpha 4 Gene Heterogeneity in Relation to the Severity of Clinical Disease in Cystic Fibrosis. Front Biosci (Landmark Ed). 2023 Jul 19;28(7):138. doi: 10.31083/j.fbl2807138.
PubMed 37525914
Study at a glance
From the registry record, last updated by the sponsor on 16 Aug 2021. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Impact of Connexin 37, Connexin 43 on Clinical Disease Phenotype in Delta F508 Homozygous Patients With Cystic Fibrosis (CF) (CF-Modifier)
- ClinicalTrials.gov ID
- NCT04242420
- Acronym / sponsor ID
- BN 178/01
- Conditions
- Cystic Fibrosis, Inflammation
- Keywords
- connexin 37+43, MBL, TGF beta, Lung function, Interleukin 8
- Study type
- Interventional
- Recruitment status
- Unknown status
- Enrolment
- 300 participants (estimated)
- Lead sponsor
- University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn)University, hospital or other
- Collaborators
- Goethe University, Academisch Medisch Centrum - Universiteit van Amsterdam (AMC-UvA)
- Study start
- Apr 2002 (actual)
- Primary completion
- 31 Dec 2023 (estimated)
- Study completion
- 31 Dec 2024 (estimated)
- First posted
- 27 Jan 2020 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Background: There is wide variety in lung disease phenotype for the delta F508 (homozygous) genotype. A leukocyte driven inflammation is most important for the pathogenesis of pulmonary disease in CF. Blood cytokines correlate negatively with pulmonary function in delta F508 homozygous patients. Gap junction proteins might be of importance for the influx of blood cells into the lung and may influence the course of pulmonary inflammation. A primary analysis (Horn et al. 2020) has shown that GJA4 variants (rs41266431) are linked to more severe disease in CF. This is very similar to variants of MBL.
Aims: To assess the relationship between gap junction proteins alpha 1 (GJA1/Connexin 43) and alpha 4 (GJA4/connexin 37) genotypes and clinical disease phenotype. Moreover are GJA4 variants in terms of clinical phenotype independent of MBL variants.
Methods:Patients homozygous for delta F508 get recruited from the CF centres of Bonn, Frankfurt and Amsterdam. Sequence analysis is performed for connexin 43 and 37 and MBL genotypes. Clinical disease is assessed longitudinally over 3 years by pulmonary function tests (FEV1 (forced expiratory volume in one second), FVC (=(forced vital capacity), FEF75 % (Forced expiratory flow at 75% of the pulmonary volume) pred), BMI (percentiles), P. aeruginosa colonization, diabetes mellitus and survival to end-stage CF lung disease (death or lung transplantation).
Detailed description
Progressive pulmonary destruction is the major cause of morbidity and mortality in human subjects with cystic fibrosis. Many studies could not find an association between delta F 508 and severity of pulmonary disease . The most important factor in CF lung disease is an inflammation driven by leukocytes and cytokines. The investigators have provided evidence in former studies that cytokines (Interleukin-8 (IL-8), tumour necrosis factor (TNF) alpha, Lipopolysaccahride binding protein (LBP), transforming growth factor (TGF) ß)measured in blood correlate negatively with lung function in delta 508 homozygous patients.
The question arises, what other factors influence recruitment of proinflammatory leukocytes from blood capillaries into the lung .
Connexins are a family of transmembrane proteins, which oligomerize into hexameric structures to form a hemichannel (connexon) and ultimately pair with a partner hemichannel in an adjacent cell to form gap junction intercellular communication channels (GJIC) . There is evidence of expression of connexin 37 (=gap junction protein A4 (GJA4)) on macrophages in humans. Moreover there is evidence of expression of connexin 37 on vascular endothelia in humans . Connexin 37 is expressed on human neutrophils . Pulmonary disease in CF is dominated by a leukocyte driven inflammation. GAP junction proteins might be of importance for the influx of blood cells into the lung. In this regard, the hypothesis was that Cx37 or Cx43 genotypes have an impact on clinical disease phenotype in CF patients homozygous for delta F508. The first analysis (Horn et al 2020) has shown a clinical phenotype linked to the GJA4 genotype is very similar to MBL variants. In this regard the question arises whether there is a link between the MBL variant alleles and the GJA4 variants. Moreover some TGFbeta genotypes are linked to certain pulmonary phenotypes. So we are looking for interactions between Cx37 and TGFbeta genotypes and their impact on the clinical phenotype as well.
Study design
- Primary purpose
- Diagnosis
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
- Model description
- Evaluation of clinical parameters by genotype
| Arm | Type | Receives |
|---|---|---|
| Connexin genotype | Other | Diagnostic Test: Genotyping, Diagnostic Test: Lung function, Diagnostic Test: Microbiology, Diagnostic Test: Blood sample, Diagnostic Test: Induced SputumGenotyping |
Who may qualify
- Ages
- 6 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
Key inclusion criteria (1 listed)
- Homozygosity for delta F 508
Key exclusion criteria (1 listed)
- treatment with systemic steroids 14 days preceding this trial, participation in another study within the past 30 days, treatment with Orkambi or status after lung transplantation (for assessment of all parameters except survival).
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| GenotypingGenotyping for single nucleotide polymorphisms for Connexin 37\&43, Mannose binding lectin (MBL) and transforming growth factor beta (TGF beta) genotypes | Diagnostic test | Connexin genotype |
| Lung functionSpirometry, | Diagnostic test | Connexin genotype |
| MicrobiologyBacterial culture from Sputum or swab | Diagnostic test | Connexin genotype |
| Blood sampleInterleukin-8 assay (via chemiluminescence) blood cell count | Diagnostic test | Connexin genotype |
| Induced SputumInterleukin-8 assay (via chemiluminescence) blood cell count | Diagnostic test | Connexin genotype |
Primary outcome
Survival to end stage lung disease
Time frame: through study completion, on average 27 years
End stage lung disease: Death or lung transplantation
Lung function parameter: FEV1
Time frame: 3 years
Best FEV1 value in % predicted of the year according to German registry according to Global lung initiative (GLI)
Lung function parameters: FVC
Time frame: 3 years
Best FVC value in % predicted of the year according to German registry according to Global lung initiative (GLI)
Lung function parameters: FEF75
Time frame: 3 years
Best FEF75 value in % predicted of the year according to German registry according to Global lung initiative (GLI)
4 secondary outcomes
Interleukin-8 in blood
Time frame: through study completion, an average of 3 year
Interleukin-8 in sputum
Time frame: through study completion, an average of 3 year
White blood cell count
Time frame: through study completion, an average of 3 year
Inflammatory markers in sputum
Time frame: through study completion, an average of 3 year
Results and safety
Results are not available in the source record.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 16 Aug 2021.
- Background: PubMed 16207846Drumm ML, Konstan MW, Schluchter MD, Handler A, Pace R, Zou F, Zariwala M, Fargo D, Xu A, Dunn JM, Darrah RJ, Dorfman R, Sandford AJ, Corey M, Zielenski J, Durie P, Goddard K, Yankaskas JR, Wright FA, Knowles MR; Gene Modifier Study Group. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med. 2005 Oct 6;353(14):1443-53. doi: 10.1056/NEJMoa051469.
- Results publication: PubMed 8166795Cystic Fibrosis Genotype-Phenotype Consortium. Correlation between genotype and phenotype in patients with cystic fibrosis. N Engl J Med. 1993 Oct 28;329(18):1308-13. doi: 10.1056/NEJM199310283291804.
- Results publication: PubMed 17099022McKone EF, Goss CH, Aitken ML. CFTR genotype as a predictor of prognosis in cystic fibrosis. Chest. 2006 Nov;130(5):1441-7. doi: 10.1378/chest.130.5.1441.
- Results publication: PubMed 15579374Schmitt-Grohe S, Naujoks C, Bargon J, Wagner TO, Schubert R, Hippe V, Zielen S. Interleukin-8 in whole blood and clinical status in cystic fibrosis. Cytokine. 2005 Jan 7;29(1):18-23. doi: 10.1016/j.cyto.2004.09.004.
- Results publication: PubMed 16183806Schmitt-Grohe S, Hippe V, Igel M, von Bergmann K, Posselt HG, Krahl A, Smaczny C, Wagner TO, Nikolazik W, Schubert R, Lentze MJ, Zielen S. Lipopolysaccharide binding protein, cytokine production in whole blood, and lipoproteins in cystic fibrosis. Pediatr Res. 2005 Nov;58(5):903-7. doi: 10.1203/01.PDR.0000182598.98167.24. Epub 2005 Sep 23.
- Results publication: PubMed 16622779Schmitt-Grohe S, Stuber F, Book M, Bargon J, Wagner TO, Naujoks C, Schubert R, Lentze MJ, Zielen S. TNF-alpha promoter polymorphism in relation to TNF-alpha production and clinical status in cystic fibrosis. Lung. 2006 Mar-Apr;184(2):99-104. doi: 10.1007/s00408-005-2568-x.
- Results publication: PubMed 24062613Eickmeier O, Boom Lv, Schreiner F, Lentze MJ, NGampolo D, Schubert R, Zielen S, Schmitt-Grohe S. Transforming growth factor beta1 genotypes in relation to TGFbeta1, interleukin-8, and tumor necrosis factor alpha in induced sputum and blood in cystic fibrosis. Mediators Inflamm. 2013;2013:913135. doi: 10.1155/2013/913135. Epub 2013 Aug 26.
- Results publication: PubMed 26009202Dempsie Y, Martin P, Upton PD. Connexin-mediated regulation of the pulmonary vasculature. Biochem Soc Trans. 2015 Jun;43(3):524-9. doi: 10.1042/BST20150030.
- Results publication: PubMed 15955304Chanson M, Derouette JP, Roth I, Foglia B, Scerri I, Dudez T, Kwak BR. Gap junctional communication in tissue inflammation and repair. Biochim Biophys Acta. 2005 Jun 10;1711(2):197-207. doi: 10.1016/j.bbamem.2004.10.005. Epub 2004 Oct 30.
- Results publication: PubMed 11834520Kwak BR, Mulhaupt F, Veillard N, Gros DB, Mach F. Altered pattern of vascular connexin expression in atherosclerotic plaques. Arterioscler Thromb Vasc Biol. 2002 Feb 1;22(2):225-30. doi: 10.1161/hq0102.104125.
- Results publication: PubMed 12525569Zahler S, Hoffmann A, Gloe T, Pohl U. Gap-junctional coupling between neutrophils and endothelial cells: a novel modulator of transendothelial migration. J Leukoc Biol. 2003 Jan;73(1):118-26. doi: 10.1189/jlb.0402184.
- Results publication: PubMed 25301274Saez PJ, Shoji KF, Aguirre A, Saez JC. Regulation of hemichannels and gap junction channels by cytokines in antigen-presenting cells. Mediators Inflamm. 2014;2014:742734. doi: 10.1155/2014/742734. Epub 2014 Sep 9.
- Individual participant dataSharing planned: No
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Sabina Schmitt-Grohe, MD principal investigator
University Hospital, Bonn
3 sites in 2 countries: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Genotyping, Lung function and Microbiology in people with Cystic Fibrosis and Inflammation, run by University Childrens' Hospital (Zentrum für Kinderheilkunde des Universitätsklinikum Bonn) at 3 sites in 2 countries.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 1 inclusion and 1 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: status not confirmed, as of the sponsor's last update on 16 Aug 2021. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 3 sites in Germany, Netherlands. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
Results are not available in the source record.
How can a doctor refer a patient or contact the research team?
Contact Sabina Schmitt-Grohe, MD (004915254568942, s.schmitt-grohe@ukbonn.de), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Survival to end stage lung disease (time frame: through study completion, on average 27 years). The study lists 4 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT04242420, which also lists 14 linked publications.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 16 Aug 2021.