Scientific programme

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: Identification and characterization of large deletions in the SERPING1 gene – Veronique Frémeaux-Bacchi
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: Who and when: the analysis of the molecular mechanisms of C1-Inhibitor deficiency induced angioedema for the best therapeutic choice – Fleur Bossi
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