Lisbon Conference 2013 - Speakers Presentations
Please click on the links (where available) to view the presentations given at the Lisbon 2013 Conference
| Wednesday 12 June 2013 | |
| 17:00-18:00 | ECFS Annual General Meeting |
| 18:30-20:00 | Opening Plenary Dr. Marques Pinto [1]- John Dodge (UK) ECFS Award Lecture [2] - Kevin Webb (UK) |
| 20:00-21:30 | Welcome Reception |
| Thursday 13 June 2013 | |
| 07:15-08:15 | Round Tables |
| 07:00-08:15 | Johns Hopkins University School of Medicine Satellite Symposium |
| 08:30-10:00 | Symposium 1 - CFTR modulators Moderators: I. Fajac (FR) / T. Lee (UK) |
| Correcting and Potentiating CFTR - Michael Boyle (US) | |
| ENaC blockers - Henry Danahay (UK) | |
| New Correctors and Potentiators from high throughput Screening - Luis Galietta (IT) | |
| CF drug discovery and personalized medicine - Fred Van Goor (US) | |
| 08:30-10:00 | Symposium 2 - Upper Airways Infection Moderators: S. Molin (DK) / L. Dupont (BE) |
| Upper and lower respiratory tract microbiome in CF and non CF - Michael Cox (UK) | |
| Host response to sinus infection -AT Peters (US) | |
| Medical interventions for sinus infection in CF - Kasper Aanaes (DK) | |
| Importance of sinus disease in patients undergoing lung transplantation [3] - C. Knoop (BE) | |
| 08:30-10:00 | Symposium 3 - Newborn Screening Moderators: L. Vilarinho (PT) / P. Farrell (US) |
| Neonatal screening in Europe: common goals, many protocols! [4] - Kevin Southern (UK) | |
| Incorporating DNA analysis into neonatal screening protocols: Pluses and minuses [5]- Philip Farrell (US)/Richard Parad (US) | |
| The arguments for and against a safety net strategy in the NBS protocol - Olaf Sommerburg (DE) | |
|
Newborn screening in Australia, investigating alternative strategies such as PAP - Enzo Ranieri (AU) |
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| 08:30-10:00 | Symposium 4 - Beyond Epidemiology: Expanding the Role of CF Registries Moderators: E. McKone (IE) / L . Pereira (PT) |
| Registry meets Regulators: Using registries for Pharmacovigilence - Irmgard Eichler (EU) | |
| Nature v. Nurture: Interpreting differences in outcomes in and across EU countries [6] - Ed McKone (IE) | |
| Standardising CT Scans [7] - Harm Tiddens (NL) | |
| Registry meets Politics: EuroCareCF 2007-2013 [8] - Anil Mehta (UK) | |
| 08:30-10:00 | Symposium 5 - Coping and resilience Moderators: J. Abbott (UK) / G. Latchford (UK) |
| An overview of coping and resilience in CF [9]- Janice Abbott (UK) | |
| Illness representations, chronic illness and coping [10] - John Weinman (UK) | |
| Illness representations and quality of life in CF [11]- Gregory Sawicki (US) | |
| Illness representations, coping and adherence - Rob Horne (UK) | |
| 08:30-10:00 | Symposium 6 - Outcome Determinants in CF Moderators: M. Schechter (US) / M. do Céu Machado (PT) |
| Socioeconomic - Michael Schechter (US) | |
| Sex [12] - Nicholas Simmonds (UK) | |
| Climate/Environment [13]- JM. Collaco (US) | |
| Adherence [14] - Alexandra Quittner (US) | |
| 10:00-10:30 | Coffee Break |
| 10:30-12:00 | Symposium 7 - The organisation of the CF Unit Moderators: M. Boyle (US) / H. Heijerman (NL) |
| Starting from Scratch: Building a New CF Unit [15] - Charles Gallagher (IE) | |
| Managing the rapid growth of the centre [16] - Andrew Jones (UK) | |
| Training and motivating CF teams - Isabelle Sermet (FR) | |
| Quality Control: the role of peer review [17]- Michael Boyle (US) | |
| 10:30-12:00 | Symposium 8 - Translating what we have learned from Bacterial Genomics to CF Care Moderators: J. Foweraker (UK) / I. Sá Correia (PT) |
| Introduction to the technology - what we can learn from the next generation - Eshwar Mahenthiralingam (UK) | |
| Pseudomonas aeruginosa - Lutz Wiehlmann (DE) | |
| Staphylococcus aureus [18] - Barbara Kahl (DE) | |
| Achromobacter and other gram negatives [19] - John Lipuma (US) | |
| 10:30-12:00 | Symposium 9 - What to do with a positive screening for metabolic complications? Moderators: A. Munck (FR) / A.M. Boot (NL) |
| Glucose intolerance: When to start insulin [20] - David Zangen (IL) | |
| Delayed puberty; is it still a problem? Should we screen and treat [21]- Marie Bournez (FR) | |
| Effectiveness of nutritional interventions: What conclusions from the literature? Roderick Houwen (NL) | |
| Bone mineral density and Vitamin D status in adolescents – what needs to be done? [22] - A.M. Boot (NL) | |
| 10:30-12:00 | Symposium 10 - Learning more about CFTR Moderators: M. Amaral (PT) / L. Galietta (IT) |
| How does deltaF508 CFTR travel to the cell surface? - Carlos Farinha (PT) | |
| Opportunities for pharmacological modulation of CFTR at the cell surface - David Sheppard (UK) | |
| Novel opportunities for drug development using primary CF organoids - Florijn Dekkers (NL) | |
| Functional interaction of CFTR with SLC26 anion transporters and anoctamins [23]- Karl Kunzelmann (DE) | |
| 10:30-12:00 | Symposium 11 - Airway Clearance Challenges in Reactive Airways Disease Moderators: I. Maguire (IE) / L. Lannefors (DK) |
| Pathophysiology and medical management of Reactive Airways Disease in CF - Eitan Kerem (IL) | |
| Preparing the airways for Airway Clearance: optimising inhalation therapy [24]- Brenda Button (AU) | |
| Do's and Dont's of Airway Clearance in reactive airways disease [25] - Maggie McIlwaine (CA) | |
| Exercise and reactive airways disease [26] - Mark Elkins (AU) | |
| 10:30-12:00 | Symposium 12 - Innate Immunity: Is it important in the CF Lung? Moderators: D. Hartl (DE) / A. Gaggar (US) |
| Pathogen-recognition in CF airways [27] - Catherine Greene (IE) | |
| Proteolytic cleavage of SP-D in CF Lung Disease [28] - A. Gaggar (US) | |
| Biometals in CF inflammation - David Reid (AU) | |
| Assessing early inflammation based on biomarkers – the AREST study [29] - Peter Sly (AU) | |
| 12:00-15:00 | Lunch |
| 12:30-14:00 | Gilead Satellite Symposium |
| 12:30-14:00 | Pharmaxis Satellite Symposium |
| 14:00-15:00 | EPoster Sessions: Nursing/Psychosocial Issues | Lung Disease Guided Poster Tours: Screening/Diagnosis |Microbiology |Gastrointestinal/Liver Disease/Metabolic complications/Nutrition |
| 15:00-16:30 | Workshop 1 - Infection in the clinic Moderators: H. Krogh Johansen (DK) / K. Van der Ent (NL) |
| Abstract WS1.1: Evolutionary pathways in two dominant clinical Pseudomonas aeruginosa clones - Helle Krogh Johansen (DK) | |
| Abstract WS1.2: Three clinically distinct chronic pediatric airway infections share a common core microbiota [30] - Lucas Hoffman (US) | |
| Abstract WS1.3: Respiratory microbiota dynamics in newborns with cystic fibrosis and healthy controls: a longitudinal study - Sabine Prevaes (NL) | |
| Abstract WS1.4: Significant bacterial infection missed using cough swabs compared to bronchoalveolar lavage in 1-year old newborn screened CF infants [31] - Lena Thia (UK) | |
| Abstract WS1.5: First evidence of Mycobacterium abscessus biofilm in the lungs of chronically infected CF patients - Tavs Qvist (DK) | |
| Abstract WS1.6: Successful Treatment of Mycobacterium abscessus Infection in Cystic Fibrosis Patients using a Novel Regimen [32] - Andrew Jones (UK) | |
| 15:00-16:30 | Workshop 2 - The experience of parents Moderators: J. Noordhoek (NL) / M. Jessup (AU) |
| Introduction [33] by Teresa Oliveira (PT) and Hélio Ferreira (PT) | |
| Abstract WS2.1: “Feeling my way”: Information needs for parents whose child has been diagnosed with CF following newborn screening [34] - Melanie Jessup (AU) | |
| Abstract WS2.2: What do parents experience and how do they cope with the AREST-CF Early Surveillance Program for infants and children with cystic fibrosis? - Cindy Branch-Smith (AU) | |
| Abstract WS2.3: Parents’ roles and involvement in young adults’ clinic appointments - Lucy Stuttard (UK) | |
| Abstract WS2.4: Use of non-prescribed medicines, supplements and therapies by children with cystic fibrosis, other chronic illnesses and children with an acute injury - A comparative mixed-methods study [35] - Matthew Hurley (UK) | |
| Abstract WS2.5: The development, implementation and evaluation of a therapeutic group for non-affected siblings of children and young people with Cystic Fibrosis attending a Regional Paediatric CF centre [36]- Clare Dixon (UK) | |
| 15:00-16:30 | Workshop 3 - What's up in the gut Moderators: B. Plant (IE) / A. Isabel Lopes (PT) |
| Abstract WS3.1: Gastric aspiration into the CF lung; relationship with reflux symptoms and lung function [37]- Gemma Crossfield (UK) | |
| Abstract WS3.2: Higher levels of fecal calprotectin levels are associated with SIBO in cystic fibrosis after lung transplantation [38] - Emina Halilbasic (AT) | |
| Abstract WS3.3: Molecular characterization of fecal microbiota and metabolic profiles association in cystic fibrosis patients [39] - Valerio Iebba (IT) | |
| Abstract WS3.4: Clostridium difficile, but not Giardia lambliais, is a frequent finding in the stool of CF patients [40] - Lutz Naehrlich (DE) | |
| Abstract WS3.5: Clostridium difficilie in adult cystic fibrosis (CF): prevalence, ribotyping and toxigenic capability. A prospective study [41] - Michael Harrison (IE) | |
| Abstract WS3.6: Probiotic intake improves the gastrointestinal health of cystic fibrosis patients - Ana de Blas Zapata (ES) | |
| 15:00-16:30 | Workshop 4 - New Insights in CFTR biology Moderators: D. Sheppard (UK) / A. Grangeia (PT) |
| Abstract WS4.1: New molecular models of CFTR based on ABC transporter structures - Brice Hoffmann (FR) | |
| Abstract WS4.2: Exploration of the ATP binding site in CFTR-NBD1 for enhanced ligand binding [42]- Merav Fichman (IL) | |
| Abstract WS4.3: Rescue of F508del-CFTR without the regulatory insertion (?RI) and regulatory extension (?RE) in combination with genetic revertants and small molecules [43] - Inna Uliyakina (PT) | |
| Abstract WS4.4: Restoration of F508?-CFTR trafficking and function by liposome-mediated delivery of antibodies against cytokeratin 8 - Benoît Chatin (FR) | |
| Abstract WS4.5: LMTK2 Regulates CFTR Endocytosis by Phosphorylation at CFTR SER-737 - Simao Luz (PT) | |
| Abstract WS4.6: MicroRNA-30s negatively regulate Planar Cell Polarity genes in Human Cystic Fibrosis Bronchial Epithelial cells [44] - Sabrina Noel (BE) | |
| 15:00-16:30 | Workshop 5 - Inflammatory Connections Moderators: D. Hartl (DE) / L. Touqui (FR) |
| Abstract WS5.1: High degree of variation in virulence of clonal Burkholderia cenocepacia ST32 isolates from cystic fibrosis patients in zebra fish embryos [45]- Klara Dedeckova (CZ) | |
| Abstract WS5.2: Toll-like receptor 9 deficiency protects mice against Pseudomonas aeruginosa pulmonary infection [46]- Fatima Ben Mohamed (FR) | |
| Abstract WS5.3: GSNOR Inhibitors as Potential, Novel Anti-inflammatory Therapy in Cystic Fibrosis - Steven Shoemaker (US) | |
| Abstract WS5.4: Increased production of IL-17A in circulating Th17 and dysfunctional Tregs in adults with cystic fibrosis-related diabetes [47] - Sophie Ziai (CA) | |
| Abstract WS5.5: Impact of Antibiotic Treatment on Concentration of Pro-inflammatory Cytokines in Nasal Lavages of CF Patients [48] - Julia Hentschel (DE) | |
| Abstract WS5.6: Regulatory T cells in cystic fibrosis patients infected with Pseudomonas aeruginosa [49] - A. Hector (DE) | |
| 15:00-16:30 | Workshop 6 - Delivering and monitoring care Moderators: S. Madge (UK) / K. De Rijcke (BE) |
| Abstract WS6.1: A cross-sectional evaluation of factors influencing adherence in children with cystic fibrosis [50] - Nicola Goodfellow (UK) | |
| Abstract WS6.2: The Impact of Transition to Adult Services on Weight and BMI for Patients with Cystic Fibrosis [51]- Caroline Cousins (UK) | |
| Abstract WS6.3: Airway clearance and physical activity - Results from a UK Survey [52]- Kate Ferguson (UK) | |
| Abstract WS6.4: Benchmarking in Germany: an expanding systematic quality improvement project [53] - Nadja Niemann (DE) | |
| Abstract WS6.5: Experience and satisfaction of patients and parents with CF services – a nationwide survey in Germany [54] - Gratiana Steinkamp (DE) | |
| Abstract WS6.6: A prospective multi centre study to examine the impact of home intravenous antibiotic treatment (H-IVAT) on carer well being [55] - Holly Hope (UK) | |
| Abstract WS6.7: Patient responses to the introduction of telemedicine and EHRs [56]- John Wilson (AU) | |
| Abstract WS6.8: Intensive Care Unit Outcomes in Cystic Fibrosis Patients Admitted with Hemoptysis vs All Other Indications - Boaz Markewitz (US) | |
| Abstract WS6.9: Urinary Kidney Injury Molecule – 1 is superior to creatinine and cystatin-C based formulas at predicting chronic kidney disease in patients with cystic fibrosis [57] - Andrew Prayle (UK) | |
| 16:30-17:00 | Coffee Break |
| 17:00-18:30 | Workshop 7 - Novel Therapies Moderators: E. Kerem (IL) / I. Fajac (FR) |
| Abstract WS7.1: Multi-target corrective effect of vardenafil on F508del-CFTR function and localization [58]- Barbara Dhooghe (BE) | |
| Abstract WS7.2: Ex vivo effect of CFTR modulators VX770, VX809 and PTC124 on CFTR-mediated chloride secretion in rectal biopsies from CF patients [59] - Sheila Scheinert (DE) | |
| Abstract WS7.3: VX-661, an investigational CFTR corrector, in combination with ivacaftor, a CFTR potentiator, in patients with CF and homozygous for the F508Del-CFTR mutation: interim analysis [60]- Scott Donaldson (US) | |
| Abstract WS7.4: Lumacaftor, an investigational CFTR corrector, in combination with ivacaftor, a CFTR potentiator, in CF patients with the F508Del-CFTR mutation: Phase 2 interim analysis - Michael Boyle (US) | |
| Abstract WS7.5: Interim results of the Phase 3 open-label study of ataluren in nonsense mutation cystic fibrosis (nmCF) - Eitan Kerem (IL) | |
| Abstract WS7.6: UK and Ireland review of Ivacaftor in severe CF: impact on lung function and weight - Peter Barry (UK) | |
| 17:00-18:30 | Workshop 8 - Microbial Communities: relationships and disease Moderators: E. Mahenthiralingham (UK) / B. Kahl (DE) |
| Abstract WS8.1: The role of second-generation sequencing in describing the fungal microbiota in the adult cystic fibrosis (CF) airway and its correlation with clinical phenotype [61] - Michael Harrison (IE) | |
| Abstract WS8.2: Effect of Staphylococcus aureus on the outcome of pulmonary Pseudomonas aeruginosa infection in a murine model - Avril Monahan (UK) | |
| Abstract WS8.3: Cystic fibrosis transmembrane conductance regulator (CFTR) allelic variants relate to shifts in fecal microbiota of cystic fibrosis patients [62] - Valerio Iebba (IT) | |
| Abstract WS8.4: Temporal bacterial community dynamics of cystic fibrosis lung infections - Leah Cuthbertson (UK) | |
| Abstract WS8.5: Airway bacterial community structure and correlation during health and disease [63]- Gisli Einarsson (UK) | |
| Abstract WS8.6: Interaction of microorganisms modulating the Cystic Fibrosis clinical severity [64]- Fernando de Lima Marson (BR) | |
| 17:00-18:30 | Workshop 9 - Clearing the airways Moderators: M. McIlwaine (CA) / M. Donadio (BR) |
| Abstract WS9.1: Effectiveness of airway clearance techniques when patients chose their airway clearance technique [65] - Maggie McIlwaine (CA) | |
| Abstract WS9.2: Patient and physiotherapist reported adherence to airway clearance therapy, inhalational therapy and exercise in cystic fibrosis [66]- Brenda Button (AU) | |
| Abstract WS9.3: Bronchial hyperreactivity related to 4 commonly used inhalation drugs in cystic fibrosis [67]- Hanneke Eyns (BE) | |
| Abstract WS9.4: Non invasive ventilation as airway clearance technique compared to PEP in adult patients with cystic fibrosis [68]- Maria Cecilia Rodriguez (SE) | |
| Abstract WS9.5: Saline at lower tonicity in cystic fibrosis (SALTI-CF) trial – A randomised, controlled trial comparing 0.9% v 3% v 6% nebulised saline [69] - Mark Elkins (AU) | |
| Abstract WS9.6: Cystic fibrosis-related postural and baropodometric changes: a comparison with healthy individuals [70]- Marcio Donadio (BR) | |
| 17:00-18:30 | Workshop 10 - Screening, Genetics & Biomarkers Moderators: H. Cuppens (BE) / C. Bombieri (IT) |
| Abstract WS10.1: The performance of the UK national newborn screening (NBS) programme for CF; results from a UK Regional Paediatric Network - Julia Harrington (UK) | |
| Abstract WS10.2: Complete sequencing of 2,000 cystic fibrosis and CFTR-Related Disease high risk alleles - Joanna Brock (UK) | |
| Abstract WS10.3: What can next-generation sequencing do for CF? [71] - Jessica Varilh (FR) | |
| Abstract WS10.4: A New Molecular Strategy using CFTR2 Data for Improving IRT/DNA Screening [72] - Philip Farrell (US) | |
| Abstract WS10.5: Impact of CFTR mutations affecting mRNA processing and looking for therapeutic compounds - Anabela Ramalho (PT) | |
| Abstract WS10.6: Organ-specific CFTR dysfunction: comparison of biomarkers for CF diagnosis and CFTR modulator effects [73] - Lea Pinders (DE) | |
| 17:00-18:30 | Workshop 11 - Adult Care - Partnership with care teams Moderators: N. Simmonds (UK) / D. Hubert (FR) |
| Abstract WS11.1: Characterising extreme survival in cystic fibrosis: a case series of over-50 year olds [74] - Panayiotis Constantinou (UK) | |
| Abstract WS11.2: Change pathways: The power of engaging in partnerships to change and enhance care for adults with cystic fibrosis [75] - Hugh Greville (AU) | |
| Abstract WS11.3: Improving communication between adults with cystic fibrosis and their CF physician [76] - Dominique Hubert (FR) | |
| Abstract WS11.4: Education and employment: a qualitative exploration of the beliefs, aspirations and experiences of young people with CF [77] - Gary Latchford (UK) | |
| Abstract WS11.5: The effect of social deprivation, disease severity, and time in hospital on employment chances in the UK cystic fibrosis population: A longitudinal study - David Taylor-Robinson (UK) | |
| Abstract WS11.6: Unmet needs in end of life care for the adult cystic fibrosis patient: a review of expectations, challenges, and current practice - Bridget Quinn (US) | |
| 17:00-18:30 | Workshop 12 - Assessment of disease severity Moderators: D. VanDevanter (US) / T. Pressler (DK) |
| Abstract WS12.1: Evidence of Diminished FEV1 in CF 6-year-olds followed in the European CF Registry, 2007 – 2009 [78] - Donald VanDevanter (US) | |
| Abstract WS12.2: The impact of switching to the new Global Lung Function Initiative equations on spirometry results in the UK CF Registry - Paul Aurora (UK) | |
| Abstract WS12.3: Prognostic value of the 6 minute walk test in adults with cystic fibrosis - Pierre-Régis Burgel (FR) | |
| Abstract WS12.4: Determinants of 6 minute walking distance and desaturation during the 6 minute walk test in adults with cystic fibrosis - Pierre-Régis Burgel (FR) | |
| Abstract WS12.5: Lung clearance index and exercise capacity among children with mild CF- and non-CF bronchiectasis - Elpida Hatziagorou (GR) | |
| Abstract WS12.6: Comparison of two methods to identify bronchiectasis on chest computed tomography scans in two cohorts of young patients with cystic fibrosis [79] - Wieying Kuo (NL) | |
| Pulmonary emphysema in cystic fibrosis detected by densitometry on chest multidetector computed tomography (MDCT) [80] - Mark Wielpütz (DE) | |
| 19:00-20:30 | Vertex Satellite Symposium |
| Friday 14 June 2013 | |
| 07:15-08:15 | Round Tables |
| 07:15-08:15 | Abbott Satellite Symposium |
| 08:30-10:00 | Symposium 13 - New Inhaled Therapies Moderators: C. Barreto (PT) / K. Van der Ent (NL) |
| Inhaled antibiotics [81]– P. Flume (US) | |
| Anticholinergics [82] – Felix Ratjen (CA) | |
| Inhaled nitric oxide – Christopher Miller (CA) | |
| Delivering drugs to the airways: DPI or nebulizer? [83] – D. Geller (US) | |
| 08:30-10:00 | Symposium 14 - Fungi in CF Moderators: R. Moss (US) / G. McElvaney (IE) |
| Fungal detection in the real world? [84] - Jean-Philippe Bouchara (FR) | |
| Metagenome: fungal and bacterial interactions - Laurence Delhaes (FR) | |
| Aspergillus in CF - what can we learn from other lung diseases? [85] - Richard Moss (US) | |
| To treat or not to treat? - Shawn Aaron (CA) | |
| 08:30-10:00 | Symposium 15 - Planning your Research – The next Steps Moderators: R. Dentice (AU) / A. Morton (UK) |
| Turning ideas into specific aims [86] - Mark Elkins (AU) | |
| Tips for writing a successful grant application [87] - Alexandra Quittner (US) | |
| Formulating a budget - Judy Bradley (UK) | |
| How a grant review panel works [88] - Susan Madge (UK) | |
| 08:30-10:00 | Symposium 16 - Ion transport regulation in normal and CF organ physiology Moderators: K. Kunzelmann (DE) / H. De Jonge (NL) |
| Primary Ion transport defects in the CF lung - Marcus Mall (DE) | |
| Role of anion transport in the intestine - Lane Clarke (US) | |
| New insights into the role of bicarbonate secretion in the pancreas - Michael Gray (UK) | |
| Luminal pH regulation and epithelial barrier function [89] - Ursula Seidler (DE) | |
| 08:30-10:00 | Symposium 17 - News from the PERT front?! Moderators: G. Connett (UK) / D. Borowitz (US) |
| Sense and non-sense in PERT practice [90]-Frank Bodewes (NL) | |
| Pancreatic function in the first year of life - Brian O'Sullivan (US) | |
| Effects of CFTR potentiators on the gastrointestinal tract [91]- Drucy Borowitz (US) | |
| Can we maintain/restore pancreatic sufficiency: lessons from other diseases [92](Swachman) - Peter Durie (CA) | |
| 08:30-10:00 | Symposium 18 - Grand Rounds in Pulmonary Disease Expert Panel: K. De Boeck (BE) / H. Heijerman (NL) / A. Smyth (UK) / E. McKone (IE) / C. Barbara (PT) |
| Severe persistent arthritis ceased after discontinuation of omalizumab therapy used for treatment of allergic bronchopulmonary aspergillosis (ABPA) - Cordula Koerner (DE) | |
| Toxic megacolon as fatal complication of antibiotic treatment in cystic fibrosis. Possibilities of ECMO treatment [93] - Ferenc Karpati (SE) | |
| It's a family affair -a diagnostic dilemma in a family with cystic fibrosis [94] - Imogen Rose (UK) | |
| A case of accelerated clinical decline in association with Exophiala dermatitidis colonisation in cystic fibrosis [95]- Imogen Felton (UK) | |
| First description of arxula adeninivorans causing an invasive pulmonary mycosis and sepsis in a 34-year-old patient with cystic fibrosis - Jobst Röhmel (DE) | |
| Man´s best friend? [96] - William Flight (UK) | |
| 10:00-10:30 | Coffee Break |
| 10:30-12:00 | Symposium 19 - Cost of CF Care Moderators C. Goss (US) / S. Elborn (UK) |
| Funding of CF Care around the World [97] - Christopher Goss (US) | |
| Pharmacoecomonics of new therapies - Kees van Gool (AU) | |
| Personalised medicine in CF is unaffordable Pro: Ian Balfour-Lynn (UK) Con [98]: Kris De Boeck (BE) |
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| 10:30-12:00 | Symposium 20 - The pulmonary and intestinal microbiome in CF Moderators: J. Leitao (PT) / J. Lipuma (US) |
| Interactions between organisms in the microbiome of the lung [99] - Jean Marc Rolain (FR) | |
| The gut microbiome in CF [100] - R. DeLisle (US) | |
| Direct sampling of CF lungs indicates that DNA-based analyses of upper airway specimens can misrepresent lung microbiota [101] - Pradeep Singh (US) | |
| Impact of intestinal microbiome in the development of the CF respiratory microbiome in infants [102]- Devin Koestler (US) | |
| 10:30-12:00 | Symposium 21 - Genetics and Diagnosis Moderators: J. Lavinha (PT) / G. Cutting (US) |
| Update on CF modifiers [103]- Harriet Corvol (FR) | |
| Role of next generation sequencing in CF - Harry Cuppens (BE) | |
| Diagnosis of CF and related disorders in the newborn infant [104]- Carlo Castellani (IT) | |
| Diagnosis of CF and related disorders in the older child and adult [105] - Cristina Bombieri (IT) | |
| 10:30-12:00 | Symposium 22 - Altered host defense function in CF Moderators: M. Chanson (CH) / L. Clarke (PT) |
| Remodelling in CF - Bob Scholte (NL) | |
| Further lessons from the CF pig - Paul McCray (US) | |
| Altered epithelial host cell function [106] - Clifford Taggart (UK) | |
| Altered immune cell function - Emer Reeves (IE) | |
| 10:30-12:00 | Symposium 23 - CF in the shadow of co-morbidities Moderators: T. Goldschmidt (PT) / M. Bryon (UK) |
| Child developmental and behavioural co-morbidities [107]- Mandy Bryon (UK) | |
| CF related diabetes [108] - Sylvia Ockhorst (NL) | |
| Pain in CF: assessment and self-management [109]- Trudy Havermans (BE) | |
| Physical co-morbidities of CF - Lena Hjelte (SE) | |
| 10:30-12:00 | Symposium 24 - Investigator-led trials Moderators: G. Rault (FR) / S. Gartner (ES) |
| European MRSA eradication protocol: Feasibility [110]- Damian Downey (UK) | |
| Oestrogens and Pseudomonas: how to translate into a clinical trial- the OCP - Gerry McElvaney (IE) | |
| Physiotherapy: studying it is impossible? [111] - Maggie McIlwaine (CA) | |
| Outome measures in preschool children and infants [112]- Paul Aurora (UK) | |
| 12:00-15:00 | Lunch |
| 12:30-14:00 | Novartis Satellite Symposium |
| 14:00-15:00 | ePoster Sessions: CFTR/Genetics | Microbiology Guided Poster Tours Immunology/Pulmonology | Physiotherapy | Delivery of Care/Epidemiology |
| 15:00-16:30 | Special Symposium - ECFS/CFF Guidelines for the Management of Non Tuberculous Mycobacteria in CF Moderators: C. Haworth (UK) / B. Marshall (US) |
| The microbiology of nontuberculous mycobacteria - Jean Louis Herrmann (FR) | |
| Epidemiology and risk factors for NTM – Andres Floto (UK) | |
| Diagnosing NTM in CF – Charles Haworth (UK) | |
| Treatment of Mycobacterium [113] – Ken Olivier (US) | |
| 15:00-16:30 |
Workshop 13 - Imaging and physiology for assessment of infection |
| Abstract WS13.1: Lung clearance index predicts time to pulmonary exacerbation in children with CF [114] - Francois Vermeulen (BE) | |
| Abstract WS13.2: Lung Clearance Index (LCI) and airway infection in CF - Katherine O'Neill (UK) | |
| Abstract WS13.3: Lung Clearance Index: a tool to assess the response to intravenous treatment among children with Cystic Fibrosis - Elpida Hatziagorou (GR) | |
| Abstract WS13.4: Chest HRCT ( cCT) score predicts later Pseudomonas infection in young children with cystic fibrosis [115] - Sonia Volpi (IT) | |
| Abstract WS13.5: Structural correlation of multiple breath washout indices derived from alveolar slope analysis in adult cystic fibrosis [116] - Katharine Hurt (UK) | |
| Abstract WS13.6: Evidence of short-term acinar response following intravenous antibiotics therapy in adults with Cystic Fibrosis [117] - Eef Vanderhelst (BE) | |
| 15:00-16:30 | Workshop 14 - Exercise for Everyone Moderators: B. Arets (NL) / B. O'Neill (UK) |
| Abstract WS14.1: Evaluation of physical activity using the Habitual Activity Estimation Scale (HAES) questionnaire in a multicenter study [118] - Nancy Alarie (CA) | |
| Abstract WS14.2: Assessing components of fitness in cystic fibrosis: The practicality and value of using a battery of exercise tests in CF clinical practice [119] - Adrian Morris (UK) | |
| Abstract WS14.3: Chronic inflammation and infection affect exercise training response in adolescents with cystic fibrosis - Hubertus Arets (NL) | |
| Abstract WS14.4: Effects of individualised aerobic exercise training in adults with cystc fibrosis: A 4 year controlled trial [120] - Milos Petrovic (AT) | |
| Abstract WS14.5: Interval Exercise Training in Cystic Fibrosis – Effects on Maximal and Submaximal Exercise Capacity in Severely Affected Adults - Wolfgang Gruber (DE) | |
| Abstract WS14.6: Physical activity, energy expenditure and quality of life in CF adults receiving intravenous antibiotics at home and in hospital [121]- Heena Khiroya (UK) | |
| 15:00-16:30 | Workshop 15 - Exploring & developing novel therapeutic strategies Moderators: M. Mall (DE) / U. Seidler (DE) |
| Abstract WS15.1: Development of a human model of airway epithelial cells invalidated for CFTR [122]- Jessica Bellec (FR) | |
| Abstract WS15.2: Derivation of normal and cystic fibrosis human induced pluripotent stem cells (iPSCs) from airway epithelium [123] - Roberto Loi (IT) | |
| Abstract WS15.3: Human Amniotic Mesenchymal Stem Cells Modify the Function and Cytokine Production of F508del Airway Epithelial Cells Upon Coculture - Massimo Conese (IT) | |
| Abstract WS15.4: Purinergic signalling regulates pancreatic epithelial transport and pancreatic stellate cells - Ivana Novak (DK) | |
| Abstract WS15.5: Effect of treatment with oligosaccharide nanomedicine OligoG on the rheology of cystic fibrosis sputum [124] - Manon Pritchard (UK) | |
| Abstract WS15.6: Development of nanomedicines for the treatment of pulmonary biofilm infections: insights from advanced fluorescence microscopy studies - Katrien Forier (BE) | |
| 15:00-16:30 | Workshop 16 - Nutrition matters Moderators: S. Bell (AU) / A. Munck (FR) |
| Abstract WS16.1: Treatment of early diagnosed CFRD with oral drugs versus insulin: An open prospective randomized study - Manfred Ballmann (DE) | |
| Abstract WS16.2: Glucose handling in CF – Implications for the diagnosis and screening of CFRD - Dilip Nazareth (UK) | |
| Abstract WS16.3: Vitamin D Supplementation in Patients with Cystic Fibrosis: A Pilot Randomized Controlled Trial - Terezia Pincikova (SE) | |
| Abstract WS16.4: Treatment Algorithm for Vitamin D Deficiency- A Review A Year On [125] - Sejal Pandya (UK) | |
| Abstract WS16.5: Comparison of cystic fibrosis adolescents with normal and low bone mineral density [126]- Monika Mielus (PL) | |
| Abstract WS16.6: Newborn Screening and Early Life Weight Recovery are Associated with Better Adolescent Growth in Children with CF [127] - HuiChuan Lai (US) | |
| Abstract WS16.7: Growth, nutritional care and biological assessment in newborn screened CF infants - Anne Munck (FR) | |
| Abstract WS16.8: Validation of bioelectrical impedance for routine monitoring of nutritional status in Cystic Fibrosis patients [128]- Perrine Dusser (FR) | |
| 15:00-16:30 |
Workshop 17 - Novel anti-microbial therapies |
| Abstract WS17.1: Biofilm disruption and potentiating antimicrobial effects of a novel alginate oligomer on Pseudomonas aeruginosa in a murine lung infection model [129] - Wang Hengzhuang (DK) | |
| Abstract WS17.2: ALX-109 potentiates the effect of inhaled antibiotics at killing Pseudomonas aeruginosa biofilms on human airway cells - Sophie Moreau-Marquis (US) | |
| Abstract WS17.3: Allicin revisited: antimicrobial activity against the Burkholderia cepacia complex and interaction with a peroxidase target - Daynea Wallock (UK) | |
| Abstract WS17.4: Anti-Pseudomonal bacteriophage cocktail reduces inflammatory responses in the murine lung [130] - Rishi Pabary (UK) | |
| Abstract WS17.5: Evaluation of antibiofilm activity of new homoserine lactones (HSL) analogs of Pseudomonas aeruginosa [131] - Aurelie Furiga-Chusseau (FR) | |
| Abstract WS17.6: Phase 3 Trial of Inhaled Levofloxacin (Aeroquin ™, MP-376, APT-1026) vs. Tobramycin Inhalation Solution (TIS) in Intensively Treated CF Patients Over 6 Months [132]- S. Elborn (UK) | |
| 16:30-17:00 | Coffee Break |
| 17:00-18:30 | Workshop 18 - The Pregnancy "Epidemic" Moderators: Guy Thorpe-Beeston (UK) / Isabelle Durieu (FR) |
| Overview [133] - Damian Downey (UK) | |
| Abstract WS18.1: Evolution of pregnancies in the French CF Registry: 1992-2011 [134] - Lydie Lemonnier (FR) | |
| Abstract WS18.2: Impact of Pregnancy in Women with Cystic Fibrosis (CF) - A Retrospective Single Centre Study [135] - Audrey Tierney (AU) | |
| Abstract WS18.3: Pregnancy outcomes in cystic fibrosis: a 10-year experience from a UK centre [136] - Mary Renton (UK) | |
| Abstract WS18.4: Does the presence of diabetes affect lung function outcomes in a pregnant individual with Cystic Fibrosis? - Elyssa Williams (AU) | |
|
Abstract WS18.5: When women with CF become mothers: A qualitative study on psychosocial impact and adjustment [137] - Sophie Cammidge (UK) |
|
| 17:00-18:30 | Workshop 19 - Clinical Perspectives Moderators: C. Goss (US) / S. Bell (AU) |
| Abstract WS19.1: Mediastinal lymphadenopathy: a prognostic tool in adult patients with cystic fibrosis [138]- Marco Mantero (IT) | |
| Abstract WS19.2: Sinus pathogens in children with cystic fibrosis: do they relate to lower respiratory tract pathogens and is eradication successful? [139] - Paul Wilson (UK) | |
| Abstract WS19.3: The value of deep oropharyngeal suction specimens in identifying lower airway bacteria in young children with cystic fibrosis [140] - Michael Doumit (AU) | |
| Abstract WS19.4: A randomised controlled trial of the effect of hypertonic saline (HS) inhalation on exacerbation resolution, hospital length of stay and time to relapse in adults with Cystic Fibrosis [141]- Ruth Dentice (AU) | |
| Abstract WS19.5: Characterization of fungal colonization phenotypes in pediatric cystic fibrosis lung disease [142] - Ute Graepler-Mainka (DE) | |
| Abstract WS19.6: Changes in the weather and the respiratory health of adults with cystic fibrosis [143]- William Flight (UK) | |
| Abstract WS19.7: Assessment of habitual physical activity in adults with Cystic Fibrosis - Daniela Savi (IT) | |
| Abstract WS19.8: Use of Palivizumab to prevent Respiratory Syncytial Virus (RSV) infection in Cystic Fibrosis. A 10 year retrospective review pre and post introduction of palivizumab prophylaxis in Northern Ireland - Helen Groves (UK) | |
| Abstract WS19.9: A Prospective Multicenter Study To Dissect Staphylococcus aureus-Colonization From Infection In Cystic Fibrosis Patients - Barbara Kahl (DE) | |
| 17:00-18:30 | Workshop 20 - CFTR Gene Modifiers Moderators: G. Cutting (US) / H. Corvol (FR) |
| Abstract WS20.1: Role of transcription factors and microRNAs in CFTR gene expression [144] - Jennifer Bonini (FR) | |
| Abstract WS20.2: Over-expression of miR-494 and miR-145 correlates with CFTR and SMAD3 down-regulation in Cystic Fibrosis patients - Francesca Megiorni (IT) | |
| Abstract WS20.3: Transcriptomics of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies [145]- Luka Clarke (PT) | |
| Abstract WS20.4: The rs1078761 polymorphism is associated with reduced SPLUNC1 expression and increased pulmonary disease severity in Cystic Fibrosis patients [146] - Aabida Saferali (CA) | |
| Abstract WS20.5: Genetic interaction of NOS-1 and CFTR gene and its association with cystic fibrosis severity [147]- Fernando de Lima Marson (BR) | |
| Abstract WS20.6: High throughput screening as a source for novel cystic fibrosis therapeutic targets - Hugo Botelho (PT) | |
| 17:00-18:30 | Workshop 21 - CFTR Mutations: partners and significance Moderators: M. Amaral (PT) / A. Hinzpeter (FR) |
| Abstract WS21.1: Cystic Fibrosis incidence in Portugal - Paula Pacheco (PT) | |
| Abstract WS21.2: Non-invasive Prenatal Diagnosis (NIPD) of Cystic Fibrosis by quantitative real time Mutant Enrichment with 3’-Modified Oligonucleotides (MEMO) PCR [148] - Marie Claire Vincent (FR) | |
| Abstract WS21.3: Clinical variability in patients with cystic fibrosis and d1152h mutation [149]- Alida Casale (IT) | |
| Abstract WS21.4: Clinical and morphological characteristics of sporadic genetically determined pancreatitis compared with idiopathic pancreatitis: Higher risk of pancreatic cancer in CFTR variants [150] - Xavier Pepermans (BE) | |
| Abstract WS21.5: Evolution of CFTR exon 10 and duplications in primates [151] - Véronique Ladevèze (FR) | |
| Abstract WS21.6: A novel 5’ alternative CFTR mRNA isoform may be a cause of CFTR dysfunction in a patient with nasal polyposis - Emmanuelle Girodon (FR) | |
| Abstract WS21.7: Identification and characterization of two novel mutations in conserved sequences tags (CSTs) of CFTR gene [152] - Rossella Tomaiuolo (IT) | |
| Abstract WS21.8: Exchange proteins directly activated by cyclic AMP: novel CFTR interactors? - Joao Fernandes (PT) | |
| Abstract WS21.9: Screening for Novel F508del-CFTR Correctors Identified by Pharmacophore Modelling through automated microscopy and NBD1 Folding Assays - Ana Romao (PT) | |
| 17:00-18:30 | Workshop 22 - Host-pathogen Interactions Moderators:I. sa Correia (PT) / G. Döring (DE) |
| Abstract WS22.1: Basophil activation is a reliable biomarker of allergic bronchopulmonary aspergillosis (ABPA) in CF: interim results of a longitudinal cohort study [153] - Richard Moss (US) | |
| Abstract WS22.2: P. aeruginosa-induced apoptosis in airway epithelial cells is mediated by gap junctional communication in a CFTR-dependent manner - Davide Losa (CH) | |
| Abstract WS22.3: Role of macrophage proteases in the killing of intracellular bacteria [154] - Noemi Cifani (IT) | |
| Abstract WS22.4: Biofilm formation by cystic fibrosis-relevant Burkholderia cepacia complex (Bcc) bacteria allows them to evade neutrophil anti-microbial activities [155] - Mark Murphy (IE) | |
| Abstract WS22.5: Effect of the Burkholderia cenocepacia exoproteome on CFTR protein expression in human Cystic Fibrosis lung epithelial cell models - Sara Canato (PT) | |
| Abstract WS22.6: The Pseudomonas aeruginosa Pangenome: Impact of Genomic Diversity on Bacterial Pathogenicity and Host Response in Airway Infections - Burkhard Tümmler (DE) | |
| 17:00-18:30 | Workshop 23 - Psychosocial Issues/Nursing - Case Presentations Moderators: M. Kirszenbaum (FR) / J. van der Hulst (NL) / M. Butler (UK) |
| Long Term Hospitalization of a 28 Years Old Female Patient with Cystic Fibrosis Related Diabetes(CFRD) and Turkish Background, Waiting for Lung Transplantation- A Multidisciplinary Approach Is Needed for Problem Solving Issues [156] - Francis Hollander (NL) | |
| The use of clinical hypnosis to manage port access in a 5 year old girl (success with porty and squiggle!) [157]- Kate Blakeley (UK) | |
| Psychological Support for a Patient with Cystic Fibrosis Prior to Lung Transplantation - Catherine O'Leary (UK) | |
| Metrics that Matter: Making the Invisible Visible as Adherence Support Moves a Patient from a Dependence on Unscheduled Rescue to Planned Prevention - Charlotte Hinchliffe (UK) / Charlotte Rose (UK) | |
| Caring for an Ageing CF Population: When Drugs Turn Nasty [158]- Jamie Duckers (UK) | |
| The Ethics of Treatment Refusal in a 16 Year Old with Cystic Fibrosis - Harriet Conniff (UK) | |
| 19:00-20:30 | Pari Satellite Symposium |
| Saturday 15 June 2013 | |
| 09:00-10:30 | Symposium 25 - Innovative Therapeutic Strategies directed at the Basic Defect Moderators: M. Hug (DE) / G. McElvaney (IE) |
| Stem cell therapy for CF - Christina Mauritz (DE) | |
| UK Consortium multidose clinical trial: protocol and baseline data [159]- Jane Davies (UK) | |
| Readthrough therapies: a common target for genetic disease with PTC? [160] - Alexandre Hinzpeter (FR) | |
| Using rectal biopsies from CF patients to assess potentiation residual CFTR function - Marcus Mall (DE) | |
| 09:00-10:30 | Symposium 26 - Burkholderia cepacia complex: still a problem? Moderators: N. Høiby (DK) / T. Kidd (AU) |
| Bcc infection resistence and virulence [161] – Miguel Valvano (UK) | |
| Genomic adaption to the CF lung - Isabel sa Correia (PT) | |
| Bcc infection: what have we learned about infection control and therapy [162] – John Govan (UK) | |
| B.multivorans – How should we treat? [163]– Andrew Jones (UK) | |
| 09:00-10:30 | Symposium 27 - Biomarkers of exacerbation and lung function decline Moderators: M. Conese (IT) / M. Cámara (UK) |
| Bacterial signal molecules as biomarkers in CF - Miguel Cámara (UK) | |
| Sputum biomarkers for the prediction of acute pulmonary exacerbation [164] - Theodore Liou (US) | |
| Plasma biomarkers to determine severity of lung function in CF [165]- André Cantin (CA) | |
| Biomarkers of lung function decline in children with CF [166]- Frank Accurso (US) | |
| 09:00-10:30 | Symposium 28 - CF Liver Disease: what have the animal models taught us so far? Moderators: HJ Verkade (NL) / S. Freedman (US) |
| Liver – gut interaction in CF mice [167]- Steve Freedman (US) | |
| The HCO3 umbrella hypothesis in biliary diseases –involved in CF liver disease? [168] – Ulrich Beuers (NL) | |
| The importance of the gallbladder in CF bile salt homeostasis – Dominique Debray (FR) | |
| The development of liver fibrosis: Present and future targets of intervention [169] - Detlef Schuppan (US) | |
| 09:00-10:30 |
Symposium 29 - Comparison of Outcomes across the EU |
| Does your patient in the registry really have CF? - Kris De Boeck (BE) | |
| Liver disease in CF registries [170] - Anders Lindblad (SE) | |
| EU-wide comparison of longitudinal changes in lung function and nutritional measures [171] - Hanne Olesen (DK) | |
| Using Registries for Comparative Outcomes Research [172] - Chris Goss (US) | |
| 09:00-10:30 |
Symposium 30 - Nursing CF across the adult lifespan |
| The young adult [173]- Diana Bilton (UK) | |
| On being a father with CF [174] - M. Oudeman (NL) | |
| Nursing after a transplant [175] - Karen Denaux (BE) | |
| CF diagnosis in adulthood [176]- Kristine Colpaert (BE) | |
| 11:00-12:30 | Plenary Moderators: S. Elborn (UK) |
| CFTR2. The importance of understanding genotype [177]- Garry Cutting (US) | |
| Delivering quality care in CF. New challenges and new solutions? [178]- Diana Bilton (UK) | |
| ECFS: Your Society: Review of the year and plans for next - Stuart Elborn (UK) | |
| 12:30-13:00 | Closing Ceremony |
