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Consortium

BESTCILIA unifies distinguished clinicians and researchers in the field of PCD who are dedicated to establishing better diagnostic tools and treatments for patients affected by PCD. Several work partners within BESTCILIA have cooperatively published a number of high-impact research papers on PCD, thus demonstrating a successful history of collaboration. This continued collaboration will feature a well-balanced and complementary set of work skills to achieve the objectives of the work packages.

Westfälische Wilhelms-Universität Muenster (WWU)

The Westfälische Wilhelms Universität Muenster (WWU) is the leading university in the state of Nordrhein-Westfalen. Several Max-Planck institutes focusing on molecular biology and medicine as well as a national Centre for clinical Trials strengthen the local research environment. The University Hospital in Muenster is a large institution with strong emphasis on research, education and patient care, and the Department of Pediatrics is distinguished as a leading reference centre for PCD patients in Germany. The Department is currently the only clinical centre worldwide that offers all PCD diagnostic tools (nNO, HVM, TEM, IFM, genetics) recommended by the PCD European Respiratory Society Task Force1. Electron microscopy and high-resolution immunofluorescence microscopy is performed in a modern well-equipped molecular and cellular biological laboratory. The Department maintains the largest cohort of PCD patients in Germany and its clinical staff has an exceptional history of PCD patient care.

Heymut Omran is Full Professor and Chair of the Department of Pediatrics at the University Hospital Muenster as well as Adjunct Professor for Pediatrics at the University of North Carolina. He has been appointed to become member of the Leopoldina Academy which is the National Academy of Sciences in Germany. He has been/is principal investigator in several grant projects financed by both the Deutsche Forschungsgemeinschaft (DFG Om 6/4 1-5) and the EU (e.g., EUCILIA, SYSCILIA). He is a member of the review panel for grants in the area of Genetics, Medicine and Developmental Biology for the German government. He has served as Scientific and Clinical advisor of the German Primary Ciliary Dyskinesia patient support group for over 10 years and specialized in Pediatric Pulmonology and clinical care for PCD patients.

Claudius Werner, an experienced Pediatric Pulmonologist involved in establishing the German PCD registry.

Simone Helms is a MTA in pediatric pulmonology training will become involved in the clinical studies.

Julia Wallmeier MD

Johanna Raidt: Resident physician in pediatric pulmonology training. Main focus is PCD outpatient care. Involved in establishment of PCD registry (WP2), PCD diagnostics (WP3) and Azithromycin trial (WP5).

University of Bern (UNIBE)

The University of Bern is rated as one of the world wide top 200 Universities, receiving over 164.8M€ in research grants in 2010, including over 6.8M€ from the European Commission. UNIBE is involved in over 80 European (FP7) grants. The Institute for Social and Preventive Medicine is currently involved in 4 FP7 projects, the children’s clinic is a partner of UBIOPRED, an IMI project of the European Union. UNIBE has 13,000 undergraduate and 2,140 postgraduate students, 13% are international students from over 110 countries. UNIBE has a dedicated FP7 office providing finance, contractual and research administration support. The Institute of Social and Preventive Medicine (ISPM; www.ispm.ch) carries out research in a range of disciplines relevant to public health, facilitating an interdisciplinary approach in the fields of social & behavioral health, clinical epidemiology, biostatistics, and international health. For the last 6 years, it was ranked number one in the research assessment of the faculty of medicine, based on number and quality of publications and grant income. The research group in child and adolescent health, headed by Prof. Kuehni, hosts two large birth cohort studies on respiratory disease involving >10’000 children (www.leicestercohorts.org) and three nationwide population-based disease registries with detailed clinical data and long-term follow-up, including the Swiss Childhood Cancer Registry (www.childhoodcancerregistry.ch. A large clinical trial unit is associated with the ISPM.

Claudia Kuehni has a longstanding experience with management and analysis of registries and cohort studies. As co- chair of the ERS taskforce on PCD in children (2007-9) she conducted an international survey of PCD in Europe, with participation of 223 centres in 26 countries and data from >1100 patients, the largest number ever studied. She can thus rely on a successful international network of centres treating PCD.

Matthias Egger is an international expert for the methodology of systematic reviews and meta-analyses, including IPD meta-analysis and network meta-analysis. He founded the “Strengthening the Reporting of Observational studies in Epidemiology (STROBE)” initiative, an international collaborative effort published by many leading biomedical journals.

Cyprus University of Technology (CUT)

The Cyprus University of Technology even though it is a new university has already managed to be awarded a significant number of National Research Projects (more than 100) funded by the Cyprus Research Promotion Foundation and to be involved in more than 35 European Research Projects funded under the European Research Framework Programmes (FP6 and FP7), the LIFE Programme, the European Territorial Cooperation Programmes such as Interreg IIIB Archimed, Interreg IVC, MED and other. CUT has a dedicated Research Office providing finance, contractual and research administration support in the implementation of Research Projects including the FP7 Grants.

The Cyprus International Institute for Environmental and Public Health (CII) is an international research, education, and technology institution at CUT, established in 2005 in association with the Harvard School of Public Health, aiming to address environmental and public health issues in Cyprus and the region. In the area of public health, CII has set up a remarkable record of conducting large population studies examining the epidemiology and pathogenesis of respiratory diseases in relation to environmental and lifestyle exposures.

Panayiotis Yiallouros, Visiting Lecturer at CUT, in the last 7 years has received competitive grant awards of more than €800,000 and conducted several studies on asthma and cystic fibrosis in Cyprus. He is also the head of the Paediatric Pulmonology Unit of Arch. Makarios III Hospital in Nicosia, which is the single tertiary paediatric referral centre on the island. Since 1997, he runs a centralized PCD clinic that now cares for 40 paediatric and adult patients. The PCD Clinic functions as the national referral centre for PCD diagnostics and management in Cyprus

Nicos Middleton, biostatistician/epidemiologist, CUT, will give advice on data analysis for evaluation of the role of nNO measurement with the cheaper electrochemical method in screening for PCD.

Dr. Kyriacos Kyriacou, biologist - TEM scientist, CING, will provide the processing of HVM and TEM of cilia samples from Cyprus in WP3.

University of North Carolina at Chapel Hill (UNC)

The University of North Carolina is one of the top research Universities in the US receiving over $788 million in research grants and contracts in 2010/11, including over $570 million from the National Institutes of Health (NIH) and 0.6 million from European Commission. UNC has a strong track record of working in multi-center research efforts.

Margaret Leigh, Professor/ Partner in WP 1, 2, 3, 4, 6. Dr. Leigh will oversee standardization of nasal nitric oxide methodology and training in nasal nitric oxide measurement for WP3. Dr. Leigh will provide clinical data from PCD patients evaluated at UNC (after obtaining appropriate consents) for WP2. Dr. Leigh will provide anonymized data from our PCD database for the observational trials for WP1. Dr. Leigh will work with Dr. Quittner to perform patient interviews for generation of questions for QOL tool and will then test that tool in PCD patients after obtaining appropriate consent for WP4.

Michael Knowles, Professor/Partner in WP 6. Dr. Knowles provides extensive experience in multi-center study of PCD and will function as advisor in scientific coordination of studies.

Region Hovedstaden (RegionH)

Region Hovedstaden (RegionH), The Capital Region of Denmark, is one of five administrative units in DK. The region provides healthcare and research for approx. 30% of the population. Besides district hospitals the region has one specialised hospital, Copenhagen University Hospital, Rigshospitalet, providing healthcare services and research on international, national and regional level; includes Danish PCD & chILD Centre, CF Centre Copenhagen & Paediatric Pulmonary Service Department of Paediatrics and Adolescent Medicine.

Kim G Nielsen has participated in several clinical studies in different paediatric and adolescent pulmonary diseases including PCD, CF, alveolitis, and asthma. Several publications within the field of PCD diagnostics, nNO, pulmonary radioaerosol mucociliary clearance, genetics, and PCD lung function studies.

Jann Mortensen, will participate in and supervise the development and implementation of new outcome measures.

VU University Medical Center (VUMC)

The VU University medical center (VUMC) with over 750 beds and 5.600 employees is one of the largest care and knowledge institutions in the Netherlands, located in the Amsterdam metropolitan area close to Schiphol airport. As one of the eight Dutch University Medical Centers, it is closely affiliated with the VU University Amsterdam. The three major tasks of VUMC: academic teaching, scientific research and top referral patient care, including cystic fibrosis and PCD.

Eric Gerardus Haarman, is a pediatric pulmonologist, VU University Medical Center and will help to plan and lead the randomized controlled trial, be local PI and supervise local clinical assessment.

University of Southampton (SOTON)

The University of Southampton is one of the UK’s top ten research-intensive Universities receiving over £93.6M in research grants and contracts in 2010/11, including over £11.3M from the European Commission. SOTON has more than 17,000 full time undergraduate students and 7,000 postgraduates from over 130 different countries. Jane Lucas leads a national centre providing comprehensive diagnostic and management for patients with PCD, based in Southampton UK. Her research includes translational, epidemiological, basic science and qualitative methods.

Dr Lucas’ laboratories house state-of-the-art equipment for studying ciliated respiratory epithelium including high speed video microscopy, TEM and SEM, with close access to laser scanning confocal microscopes. Her team routinely culture primary epithelial cells on an air liquid interface (ALI), re-differentiating the cilia. All patients seen in Southampton for PCD diagnostics are invited to participate in research providing a rich source of samples and linked clinical data from patients with PCD and other suppurative lung disease. Modifications of ALI-culture model are being used to investigate how the ciliated respiratory epithelium interacts with bacterial infections in health and disease with an aim to understand the role of cilia in respiratory tract infection. Dr Lucas is currently investigating the interaction of Pseudomonas lectins on ciliary function. She is also investigating the effect of ciliary function on bacterial biofilm formation.

The robust phenotypic data on PCD patients has led to successful collaboration with groups focussing on identification and characterisation of novel genes and mutations. With the other two national PCD centres in Leicester and London, Dr Lucas manages a national database with longitudinal data on all PCD patients. The database forms the basis of an observational trial for answering pertinent questions on clinical phenotype of PCD, severity, prognosis and effect of treatments on outcomes. Jane Lucas has published widely using qualitative and quantitative methods to study quality of life in allergic patients. She is using this expertise to lead a work package within BESTCILIA, developing and validating health related quality of life instruments for patients with PCD.

Jane Lucas is an academic paediatrician specialising in PCD at University of Southampton, UK. She is leading the BESTCILIA work package to develop and validate quality of life questionnaires for children, teens and adults with PCD. Dr Lucas is also supervising the research of patients from the South of England to participate in the BESTCILIA observational study and registry, as well as the clinical trial of azithromycin in patients with PCD.

University of Miami (UM)

The University of Miami (UM) is a private, non-profit university in the United States that attracts a diverse and international student body and faculty. UM continues to be the top-ranked school in Florida and recently achieved No. 38 among all US colleges according to U.S. News and World Reports, 2011. UM is dedicated to basic research, especially at the Miller School of Medicine (nearly $339 million in annual expenditures).

Alexandra L. Quittner is a Full Professor of Psychology and Pediatrics at the University of Miami and has published over 120 peer-reviewed articles. She will assist with the development of HRQoL tools for PCD and is in the process of developing these instruments in the US. Dr. Quittner has developed the “gold standard” HRQoL measures for CF; these instruments have been used in over 120 published studies in the US and Europe. These measures have been recognized by both the FDA and EMA regulatory bodies as reliable, well-validated patient-reported outcomes.

International Institute of Molecular and Cell Biology (IIMCB)

IIMCB is the Polish modern research institute in molecular biomedicine. Research topics cover: cancer biology, neurobiology, protein structural biology, intracellular communication, dendritic tree formation, bioinformatics/computer modeling, enzyme engineering and mitochondrial biogenesis etc. There are 9 research groups, ca. 100 researchers incl. 54 PhD students. In 2000-2010 IIMCB researchers published 426 publications in the top referenced journals.

Michal Witt, Scientific Director of the International Institute of Molecular and Cell Biology, Warsaw, Poland. Will be responsible for organization and supervision of educational workshops and training organised in Poland. Will also coordinate flow of information and competence in the area of genetic testing with Eurogentest.

Ewa Zietkiewicz, independent researcher at the Institute of Human Genetics, Poznan, Poland. Will be involved in program preparations of meetings of medical professionals and mainly will participate in production of educational publications for professionals and for lay public.

Andrzej Pogorzelski, clinician at the Institute of Tuberculosis and Lung Disaeses, Rabka Poland with vast experience in PCD and CF. His main involvement will focus on contacts with PCD families and their education on theoretical issues and mainly on practical issues of advanced care of patients and application of modern diagnostic methods.

National and Kapodistrian University of Athens (UA)

The 3rd Department of Paediatrics of the National and Kapodistrian University of Athens, Greece, is located in the newly built ‘‘Attikon’’ University Hospital, the largest and most technologically advanced in Athens. One of the developed Unites is the Respiratory one, with strong clinical and research activity.

Konstantinos Priftis, Assistant Professor in Paediatrics – Paediatric Pulmonology, 3rd Department of Paediatrics, National and Kapodistrian University of Athens, School of Medicine, ‘‘Attikon’’ University Hospital, Athens, Greece. This period Dr Priftis is serving as the secretary of Bronchology Group of Paediatric Assembly of European Respiratory Society and he is also co-chairing the ERS Task Force for the European Reference Database on Respiratory Sounds.

Konstantinos Douros, Consultant paediatrician, paediatric pulmonologist, 3rd Department of Paediatrics, ‘‘Attikon’’ University Hospital, Athens. Dr Douros works in our Respiratory Unit (now in Sheffield, UK, as consultant Paediatrician, back in September 2012) and he will be responsible for nNO and HVM measurements.

Marios Papadopoulos, Consultant paediatrician, paediatric pulmonologist, 3rd Department of Paediatrics, ‘‘Attikon’’ University Hospital, Athens. Dr Papadopoulos is going to be the responsible person for the national network and patients’ recruitment. We have already contacted most of the University Respiratory Units in the country.

European Research Services GmbH (ERS)

The European Research Services GmbH is a public-private-partnership, founded in 2007.
ERS is a specialised consultant company. It supports researchers in planning and managing EC-funded projects. ERS provides general technical and administrative support, and adapted project-management fitting the size and complexity of the project. In addition, ERS provides expert advice to set up an efficient communication and decision structure, and how to handle emerging management issues (defaulting partners, bankruptcy, new partners, changes in a partner’s legal status, etc.). ERS uses self-designed tools to streamline documentation and reporting towards the needs of the European Union. At present ERS is partner is thirteen running projects.

Oliver Panzer is private stockholder and managing director of ERS. He holds a Diploma in physics from the WWU Münster. He worked 5 years as a postgraduate researcher in the area of nanoanalytics.
Next to experience in industry and in managing the office of a national network of excellence, he spend 2,5 years at the European Commission as a Seconded National Expert, before founding ERS. In this function he was responsible for the negotiation and monitoring of projects, and for the organisation of proposal reviews (evaluations). As Project Technical Advisor, contracted by the EU, he reviews EU projects and offers advice for management issues.

Hildegard Luhmann is working as project assistant with ERS. She studied business economics at the University of Applied Science Münster and has a wide experience of the administration in EU projects under the 6th and 7th Framework Programme. She is versed in preparing and handling websites and other media related to projects and is the expert for organisation-support at ERS.

Royal Brompton Hospital [RBH] and Imperial College London

Royal Brompton Hospital is situated within Imperial College University. RBH and Imperial at RBH have a multi-million pound research income annually. In 2008/09 funding of over £10 million was obtained to establish Biomedical Research Units, which are now fully operational. RBH has a long history of collaborative research both nationally and internationally and has been involved in many FP7 grants and other multi-centre trials. RBH hosts the busiest diagnostic service for Primary Ciliary Dyskinesia in the UK, and has the largest PCD specialist clinic in Europe with over 300 patients in our pediatric and adult clinics.

Claire Hogg is the principal investigator for these work packages at RBH. She will directly supervise recruitment of patients into the studies and oversee the transfer of data for the PCD database.

Andrew Bush is Advisor on this application and a co-investigator.