| Thursday, 23 May 2019 | |
| 13:00-20:00 | Registration – Kamilla Lobby |
| 17:00-17:30 | Welcome coffee – Kamilla Lobby |
| 17:30-19:30 | Opening ceremony – Lecture Hall: Star Auditorium |
| Greeting of guests | |
| „For HAE Patients” Award | |
| Opening lecture Chairs: K. Bork, B. Zuraw |
|
| I-1 | Which are the clinical, pathophysiological and therapeutic similarities between migraine & hereditary angioedema with C1-inhibitor deficiency? Patricia Pozo-Rosich |
| 19:30-22:30 | Welcome reception (Széchenyi Restaurant & Terrace, Grand Hotel Margitsziget) |
| Friday, 24 May 2019 | |
| 07:45-08:30 | Nurse meeting – Room Toboz (by invitation only) moderated by Iris Leibovich-Nassi |
| 08:30-10:25 | Essays on Assays – Lecture Hall: Star Auditorium Chairs: A. Grumach, to be announced |
| O-1 | Parallel comparison of three different assay methodologies for measuring functional C1-Inhibitor in HAE plasma Priya Chockalingam |
| O-2 | Assessment of C1-INH function – different methods, different results Peter J. Späth |
| O-3 | Cancelled talk: |
| O-4 | Diagnosis of bradykinin-mediated angioedema in the emergency department: usefulness of the early biological workup Samuel Luyasu |
| O-5 | SGP 120 and contact system in hereditary angioedema. Diagnostic tool in HAE with normal C1-inhibitor? Allen Kaplan on behalf of Michael Frank |
| O-6 | Changes of complement parameters during erythema marginatum in patients with hereditary angioedema Kinga Viktória Kőhalmi |
| I-2 | Analysis of C1-inhibitor deficiency: Need for standardization and quality control Michael Kirschfink |
| 10:25-10:55 | Coffee break – Room Magnolia & Kamilla Lobby |
| 10:55-12:25 | Angioedema Market: What is for sale? I (P-01 to P-23) – Room Magnolia Chairs: Anette Bygum, M. Cancian, T. Craig |
| P-01 | A case of hereditary angioedema associated with rheumatoid arthritis: treatment challenges Sladjana Andrejevic |
| P-02 | Acute treatment of pregnant women with hereditary angioedema attacks: administration of recombinant human C1 esterase inhibitor Jonathan Bernstein |
| P-03 | Tranexamic acid plus sodium bemiparin as long term prophylaxis in a patient with FXII-HAE during pregnancy: a case report. Irene Hernandez Martin |
| P-04 | Metabolic complications of late diagnosis in hereditary angioedema Natalia Vélez-Tirado |
| P-05 | HAE Patients in Ukraine: frequency and localization of attacks in 2018 Liudmyla Zabrodska |
| P-06 | Hereditary angioedema with C1-inhibitor deficiency (HAE-C1-INH) in childhood and adolescence Emel Aygören-Pürsün |
| P-07 | Higher annual rate of angioedema attacks in HAE-C1-INH patients above the age of 65 compared to patients aged 18 to 64 years Emel Aygören-Pürsün |
| P-08 | A questionnaire survey study to determine association of dental hygiene practices in Hereditary Angioedema subjects with the incidence of post-procedural angioedema attacks Jonathan Bernstein |
| P-09 | The relationship between disease activity and quality of life – a first-time survey in hereditary angioedema Bettina Ignácz |
| P-10 | Are HAE patients able to distinguish prodromes from attacks, and are they correlated? Iris Leibovich Nassi |
| P-11 | Psychological processes in the adaptation to disease in adults with hereditary angioedema due to C1-inhibitor deficiency: a pilot study from Italian referral centers Maria Bova |
| P-12 | Depression in hereditary angiodema can cause sexual morbidity Emine Nihal Mete Gokmen |
| P-13 | Immigrants’ perspective on living with hereditary angioedema in Denmark- a qualitative study Malin Sandberg |
| P-14 | Description of angioedema episodes prompting a call on the bradykin mediated angioedema reference centre on-call hotline Isabelle Boccon-Gibod |
| P-15 | Quality of Life among HAE patients in South West England (Devon and Cornwall) using HAE-QoL questionnaire designed by Foundation for Biomedical Research of La Paz University Hospital Madrid (FIBHULP) Christine Symons |
| P-16 | First kinetic follow-up of coagulation and fibrinolytic parameters in a single edematous attack of a patient with hereditary angioedema Nóra Veszeli |
| P-17 | Development and verification of a quantitative systems pharmacology disease model of hereditary angioedema Rangaraj Narayanan |
| P-18 | Serum complexes between C1INH and C1INH-autoantibodies for the diagnosis of Acquired Angioedema Alberto López Lera |
| P-19 | The Romanian Hereditary Angioedema Center started the global hereditary angioedema registry activities Noémi-Anna Bara |
| P-20 | Hereditary angioedema: quality of life in 19 patients Ana Lainez |
| P-21 | The French side of the global angioedema registry Isabelle Boccon-Gibod |
| P-22 | Designing and delivering Educational Therapeutic Program training kit for HAE patients across France Isabelle Boccon-Gibod |
| P-23 | Daily routine laboratory assessment of C1-INH function – different methods, different results, different interpretations Peter Späth |
| 12:25-13:55 | Lunch – Restaurant Platan |
| 13:55-16:15 | Clinical Pieces of Angioedema Legoland – Lecture Hall: Star Auditorium Chairs: J. Berstein, W. Wuillemin |
| I-3 | Prodromes of HAE: scientific evidence or delusional perception? Avner Reshef |
| O-7 | Clinical and genetic characteristics of patients with Hereditary Angioedema at a large tertiary care hospital in Saudi Arabia Farrukh Sheikh |
| O-8 | D-Dimer and C-Reactive Protein in urticaria and angioedema at the Emergency Room Riccardo Senter |
| O-9 | Validity and Reliability of a New Instrument for the Evaluation of HAE Prodromes Iris Leibovich-Nassi |
| O-10 | Psychosocial burden of Hereditary Angioedema in a Canadian cohort Julia Hews-Girard |
| O-11 | Angioedema and Urticaria at the Emergency Room: epidemiology and clinical management in a tertiary care center in Italy (2009-2018) Mauro Cancian |
| O-12 | Hereditary angioedema with a specific mutation in the plasminogen gene in 18 families Konrad Bork |
| O-13 | Hereditary angioedema with c1-INH deficiency in 96 Brazilian children Anete S. Grumach |
| O-14 | Determinants of breakthrough attacks in hereditary angioedema patients undergoing dental procedures Jonathan A. Bernstein |
| 16:15-16:45 | Coffee break – Room Magnolia & Kamilla Lobby |
| 16:45-17:45 | International consensus on the use of genetics in the management of hereditary angioedema – Lecture Hall: Star Auditorium moderated by Anastasios Germenis |
| 18:45 | Departure to the Dinner, gathering in the Kamilla lobby (REGISTRATION area) |
| 19:00- | Dinner |
| Saturday, 25 May 2019 | |
| 08:30-10:30 | Behind the Scenes – Lecture Hall: Star Auditorium Chairs: M. Cicardi, J. Dobó |
| O-15 | A SERPING1 variant that causes C1-inhibitor deficiency without hereditary angioedema Sonia Caccia |
| O-16 | Activation of complement MASP-3 in healthy donors and in patients with C1-inhibitor deficiency József Dobó |
| O-17 | Simultaneous determination of human plasma serine proteases complexed with C1-inhibitor in vivo Erika Kajdácsi |
| O-18 | Bradykinin, LPS and MASP-1 synergistically regulate endothelial permeability Zsuzsanna Németh |
| O-19 | Pharmacological profile of PHA-022121, a non-peptide bradykinin B2 receptor antagonist, established using the isolated human umbilical vein Francois Marceau |
| O-20 | Changes of coagulation parameters during erythema marginatum in patients with hereditary angioedema Kinga Viktória Kőhalmi |
| O-21 | Plasminogen missense mutation p.Lys330Glu: altered plasminogen glycoforms type I & II and activation susceptibility Arije Ghannam |
| O-22 | The potential of exhaled biomarker analysis in angioedema Judit Pákó Cancelled talk: |
| 10:30-11:00 | Coffee break – Room Magnolia & Kamilla Lobby |
| 11:00-12:30 | The Proof of the Pudding… – Lecture Hall: Star Auditorium Chairs: E. Aygören-Pürsün, M. Riedl |
| O-23 | PHA-022121, the first-in-class orally active bradykinin receptor B2 antagonist for on-demand and prophylactic treatment of HAE Anne S. J. Lesage |
| O-24 | Clinical evaluation of pharmacokinetics, pharmacodynamics, safety, and efficacy dose-response of BCX7353 as an acute treatment for angioedema in patients with hereditary angioedema (HAE) Marcin Stobiecki |
| O-25 | KVD900, a new oral on-demand treatment of hereditary angioedema attacks achieves complete plasma kallikrein suppression: safety, tolerability, pharmacokinetic and pharmacodynamic results from a phase 1 first-in-human study Andreas Maetzel |
| O-26 | Pharmacokinetics, safety, and potency of ATN-249, a novel oral plasma kallikrein inhibitor for hereditary angioedema Ira Kalfus |
| O-27 | Population pharmacokinetic analysis of C1-esterase inhibitor functional activity in the COMPACT open-label extension study is consistent with previous COMPACT studies Zuraw Bruce |
| O-28 | Functional C1-Esterase Inhibitor and Complement Protein 4 Levels were not altered by lanadelumab treatment in HAE Patients in the Phase 3 HELP Study Priya Chockalingam |
| 12:30-14:30 | Lunch – Restaurant Platan / HAE Global Registry Meeting – Room Jasmin (by invitation only) |
| 14:30-16:00 | Angioedema Market: What is for sale? II. (P-24 – P-46) – Room Magnolia Chairs: S. Betschel, S. Christiansen, M. Jesenák |
| P-24 | Genetic segregation study in angioedema with normal C1- inhibitor (n-C1-INH-HAE) in Southern Spanish population Krasimira Baynova |
| P-25 | A deep intronic SERPING1 variant associated with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE) Sofia Vatsiou |
| P-26 | The silo effect in the annotation of SERPING1 variation Anastasios Germenis |
| P-27 | Variant pathogenicity curation in primary angioedema with normal C1 inhibitor (nl-C1-INH-HAE) Maria Zamanakou |
| P-28 | Hereditary angioedema: a report from the Republic of Belarus Irina Guryanova |
| P-29 | A national audit of hereditary and aquired angioedema in New Zealand Karen Lindsay |
| P-30 | Situational analysis of diagnosis and treatment of hereditary angioedema in Latin America Sandra Nieto |
| P-31 | Hereditary angioedema in Belarus: epidemiology, clinical characteristics and access to diagnosis and treatment Francesca Perego |
| P-32 | Comparison of the C1-INH productions of different endothelial cells Erika Kajdácsi |
| P-33 | Sensitive assays for measuring C1-inhibitor Zsófia Jandrasics |
| P-34 | Increased fibrinolysis-induced bradykinin formation in hereditary angioedema confirmed using stored plasma and biotechnological inhibitors François Marceau |
| P-35 | High plasma exposures of KVD900 achieved in First in Human study markedly inhibit plasma prekallikrein activation; early blockade of plasma kallikrein (PKa) may halt attacks in hereditary angioedema (HAE) by reducing contact system activation Edward Duckworth |
| P-36 | The treatment of angioedema attacks by bradykinin B2-receptor antagonist icatibant in hereditary angioedema – a real-life study Noémi Andrási |
| P-37 | Evaluation of the efficacy and safety of home treatment with the recombinant human C1-inhibitor in hereditary angioedema Noémi Andrási |
| P-38 | Characterizing the minority of hereditary angioedema attacks that require more than a single injection of icatibant Irmgard Andresen |
| P-39 | Long-term prophylaxis in hereditary angioedema patients followed in ITAlian Centers for Angioedema (ITACA) and enrolled in HAE Global Registry (HGR) Mauro Cancian |
| P-40 | Shortage in France of plasma derived C1Inh concentrates: state of play and consequences for patients. Laurence Bouillet |
| P-41 | Limelight on erythema marginatum: a review of clinical features and the introduction of a new management strategy in hereditary angioedema Agnes Holdonner |
| P-42 | Hereditary angioedema: dental management in a Brazilian reference center Maria Oliva Alonso |
| P-43 | Development and validation of the self-efficacy assessment questionnaire in the management of hereditary angioedema for patients and family caregivers (HAE-SES) Francisco Sanchez Hernandez |
| P-44 | Headache as a symptom of hereditary angioedema Beáta Visy |
| P-45 | Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures Andrea Zanichelli |
| P-46 | Mass spectrometry based screening for hereditary angioedema disease Volha Skrahina |
| 16:00-16:30 | Coffee break – Room Magnolia & Kamilla Lobby |
| 16:30-17:30 | International consensus on the gynecologic and obstetric management of female patients with hereditary angioedema – updates – Lecture Hall: Star Auditorium moderated by Teresa Caballero |
| 18:45 | Departure to the Dinner, gathering in the main hotel lobby (in front of the RECEPTION DESK) |
| 19:00- | Dinner |
| Sunday, 26 May 2019 | |
| 08:30-09:30 | Lifelike Stories – Lecture Hall: Star Auditorium Chairs: M. Magerl, A. Zanichelli |
| O-29 | Hereditary Angioedema with normal C1 inhibitor: first report of an Argentinian family with factor XII mutation Ricardo D Zwiener |
| O-30 | Acquired angioedema and chronic spontaneous urticaria – one disease or two separated entities? Case report Milos Jesenák |
| O-31 | Oligoarticular Juvenile Idiopathic Arthritis in a child with Type 1 hereditary angioedema: a case report Jimmy H.C. Gooi |
| O-32 | Cardiac tamponade following cardiac surgery in Type 2 HAE patient Jimmy H.C. Gooi |
| 09:30-10:00 | Coffee break – Room Magnolia & Kamilla Lobby |
| 10:00-11:00 | Is Offense the Best Defense? – Lecture Hall: Star Auditorium Chairs: H. Longhurst, G. Porebski |
| O-33 | Clinical characteristic and therapeutic modalities in Polish C1-INH-HAE patients. A pilot cohort study in adults population. Grzegorz Porebski |
| O-34 | Treatment of patients with hereditary angioedema with normal C1-inhibitor: evaluation of 295 patients Anete S Grumach |
| O-35 | Long-term prophylaxis with C1-inhibitor concentrate in patients with hereditary angioedema David Loli-Ausejo |
| O-36 | The needs of individually tailored prophylaxis with C1-INH concentrate in pediatric patients with hereditary angioedema (HAE) – real life data from 6 pediatric patients Inmaculada Martinez Saguer |
| 11:00-12:00 | Bon voyage kit – Lecture Hall: Star Auditorium Chairs: H. Farkas, A. Kaplan, L. Varga |
| 12:00-13:30 | Lunch – Restaurant Platan |
| 13:30 | Departure |
