Scientific program

Scientific program

Dear Colleagues, Dear Authors

We have received lots of requests, if there is any embargo introduced from the organizers' side in relation to communication on the scientific program and publication of the 13th C1-inhibitor Deficiency & Angioedema Workshop via your channels. As the Scientific Program is being finalized right now, authors and companies are entitled and authorized to make announcements related to their talks from the 13th of April 2023 an onwards. Nevertheless, publishing the abstract prior to the date of the meeting should be avoided. Thank you for your attention.

Thursday, 4 May 2023
13:00-20:00 Registration
17:00-17:30 Welcome coffee
17:30-19:05 Opening ceremony
Welcome of the Guests
Surprise performance
Presentation "For HAE Patients" Award, Award Ceremony
19:05-20:05 Opening lectures
Chairs: Bruce Zuraw, Michael Kirschfink
I-01 Lactoferrin - the alarmin which knows when is a time to kill and a time to heal
Vladimir Leksa
I-02 HAE with the plasminogen gene mutation K330E and other types of hereditary angioedema with normal C1-INH
Konrad Bork
20:05-22:05 Welcome reception
Friday, 5 May 2023
08:00-10:00 Scientific Session I.
Chairs: Anete Grumach, Tomaš Freiberger
08:00-08:15
O-01
The Bradykinin cascade is activated in patients with Alzheimer’s disease and is activated in vitro by aggregated Aβ protein
Allen Kaplan
08:15-08:30
O-02
C1 inhibitor deficiency is associated with a procoagulant phenotype in both humans and mice
Steven Grover
08:30-08:45
O-03
Th2 predominance and decreased NK cells in patients with hereditary angioedema – a connection with autoimmune disease?
Linda Sundler Björkman
08:45-09:00
O-04
Determining the effects of Moringa Oleifera on hepatic and monocytic cell lines in C1 esterase inhibitor production
Martin Gonzo
09:00-09:15
O-05
Purification and characterization of the C1 inhibitor R444C variant causing type 2 hereditary angioedema; covalent binding to human serum albumin and consequences
József Dobó
09:15-09:30
O-06
Cell-cell contacts and the glycocalyx are relevant structures in bradykinin-mediated endothelial barrier injury
Robin Lochbaum
09:30-09:45
O-07
Monocytes hyperactivity and endothelial dysfunction in hereditary angioedema: the MONOBRAD Study
Nicolas Ozanne
09:45-10:00
O-08
Biological pathway analyses of plasma proteomics in hereditary angioedema due to C1 inhibitor deficiency following lanadelumab treatment
Dan Sexton
10:00-10:30 Coffee break
10:30-12:10 Scientific Session II.
Chairs: Teresa Caballero, Avner Reshef
10:30-10:50
O-09
An updated and comprehensive classification and terminology of angioedema
Avner Reshef
10:50-11:05
O-10
Detection of Bradykinin and its major metabolites by Liquid Chromatography Tandem Mass Spectrometry (LC-MS/MS)
Huamin Henry Li
11:05-11:20
O-11
Diagnosis of angioedema by Artificial Intelligence
Felix Aulenbacher
11:20-11:35
O-12
SERPING1 splicing-affecting variants highly represented in the Czech cohort of HAE-1/HAE-2 patients
Tomas Freiberger
11:35-11:50
O-13
HAErmony-1: Clinical study of adeno associated virus vector-mediated gene therapy of human C1-inhibitor in Hereditary Angioedema Type I and II
Marc Riedl
11:50-12:10 Pregnancy consensus
moderated by Teresa Caballero
12:10-13:10 Lunch break
13:10-14:10 Scientific Session III.
Chairs: Anastasios Germenis, Despina Sanoudou, Matija Rijavec
13:10-13:30
I-03
CRISPR opens the way to genetic therapy of angioedema
Despina Sanoudou
13:30-13:45
O-14
Hereditary angioedema variant curation using a ClinGen framework
Matija Rijavec
13:45-14:00 International consensus on the use of genetics in the management of hereditary angioedema – The 2023 revision and update
Round table discussion and voting by Anastasios Germenis
14:00-16:00 Poster session I. with coffee
Chairs: Timothy Craig, Sandra Christiansen, Markus Magerl
P-01 One-year epidemiologic data from Angioedema Center Vienna, Austria
Tamar Kinaciyan
P-02 Outcome parameters to measure efficacy of prophylactic therapy for hereditary angioedema: a systematic review
Remy Petersen
P-03 Efficacy and safety of Lanadelumab in Russian participants with hereditary angioedema (HAE)
Elena Latysheva
P-04 Mimics of hereditary angioedema in an emergency department
Marko Barešić
P-05 Mechanistic modeling and simulations predict long-term HAE attack prevention with STAR-0215
Jou-Ku Chung
P-06 The impact of dental procedures on angioedema attacks – An observational study of patients enrolled in the Romanian Hereditary Angioedema Registry
Valentin Nadasan
P-07 The analysis of the effect of the COVID-19 pandemic on C1-inhibitor deficient hereditary angioedema patients
Dávid Szilágyi
P-08 Clinical spectrum of high titre anti-Ro/SSA antibodies in patients with hereditary angioedema with C1 inhibitor deficiency
Sladjana Andrejevic
P-09 Efficacy and safety of rituximab in angioedema with acquired C1 inhibitor deficiency
Galith Kalmi
P-10 Hereditary angioedema in the Republic of Panama
Olga M. Barrera
P-11 Vascular neural control blockade in a COVID-19 positive hereditary angioedema patient
Francesca Perego
P-12 A value-based and human centred innovative approach based on persona: Psychosocial needs of hereditary angioedema patients and caregivers
Francesca Perego
P-13 Decreased adhesion to the endothelium leads to elevated neutrophil granulocyte count in hereditary angioedema patients
Erika Kajdácsi
P-14 Psychodynamic factors impacting the degree of trust in the relationship between a doctor and a HAE patient
Ekaterina A. Viktorova
P-15 Targeted sequencing panel for the diagnosis of hereditary angioedema due to C1 inhibitor deficiency
Matija Rijavec
P-16 Route of administration preferences of people with hereditary angioedema for on-demand treatment: A US-based qualitative study
Laurence Boulliet
P-17 Second patient with kininogen 1 mutation in hereditary angioedema
Gaëlle Hardy
P-18 A retrospective study (INTEGRATED) of real-world effectiveness of Lanadelumab in European patients with HAE Type I/II
Markus Magerl
P-19 Quality of life in patients with hereditary angioedema in Latvia
Kanepa Adine
P-20 Burden of hereditary angioedema type I and II: Preliminary results from a real-world study in Europe, Israel and Canada
Henriette Farkas
P-21 Assisted reproductive techniques in patients with Bradykinin-induced angioedema
Tatiana Navarro-Cascales
P-22 Prevalence of non-alcoholic steatohepatitis in adult patients with hereditary angioedema due to C1-inhibitor deficiency (HAE-C1INH)
Teresa Caballero
P-23 Possible psychological fluctuations in severity and frequency of hereditary angioedema swellings
Tomaz Garcez
P-24 Real-life experience after eight months of long-term subcutaneous C1-inhibitor prophylactic treatment in four Hungarian patients with hereditary angioedema due to C1-inhibitor deficiency
Hanga Réka Horváth
P-25 Early symptom relief following treatment with the oral bradykinin 2 receptor antagonist PHVS416 in patients with hereditary angioedema attacks
Marc A. Riedl
16:30- Social program outside with discussion
Saturday, 6 May 2023
08:00-09:30 Scientific Session IV.
Chairs: Hilary Longhurst, Isabelle Boccon-Gibod
08:00-08:15
O-15
Attack-free status across subgroups of patients with hereditary angioedema (HAE) after 96 weeks of berotralstat treatment: results from the APeX-S trial
Avner Reshef
08:15-08:30
O-16
Rationale for the short-term prophylaxis regimen with Sebetralstat in KONFIDENT-S
Michael Smith
08:30-08:45
O-17
One-year results from an open-label study of Donidalorsen in patients with hereditary angioedema
Laura Bordone
08:45-09:00
O-18
The EC85 derived from the oral bradykinin B2 receptor antagonist PHA121 against bradykinin effects in healthy volunteers predicts the onset and duration of its clinical effects in hereditary angioedema
Marcus Maurer
09:00-09:15
O-19
Efficacy and safety of the oral bradykinin B2 receptor antagonist PHVS416 in treatment of hereditary angioedema attacks: topline results of RAPIDe-1 phase 2 trial
Henriette Farkas
09:15-09:30
O-20
Design of ALPHA-STAR, a Phase 1b/2 proof-of-concept trial of STAR-0215 as a long-active preventative therapy in patients with hereditary angioedema (HAE) Types I or II
Marcus Maurer
09:30-10:00 Coffee break
10:00-11:15 Scientific Session V.
Chairs: Connie Katelaris, Marcus Maurer
10:00-10:15
O-21
Transient exposure to NTLA-2002, an investigational CRISPR/Cas9-based gene editing therapy, leads to durable pharmacodynamic responses and attack control in patients with hereditary angioedema
Hilary Longhurst
10:15-10:30
O-22
Prototype of a mobile application to record prodromes and attacks of hereditary angioedema
Iris Leibovich-Nassi
10:30-10:45
O-23
Garadacimab for hereditary angioedema prophylaxis: Long-term efficacy and safety from the VANGUARD Phase 3b open-label extension trial (first interim analysis)
Avner Reshef
10:45-11:00
O-24
Effect of prophylactic immunomodulation in non-human primates treated with BMN 331, an AAV5 gene therapy for hereditary angioedema
Benjamin M. Hock
11:00-11:15
O-25
Garadacimab for hereditary angioedema prophylaxis: Efficacy and safety from a Phase 2 open-label extension trial
Timothy J. Craig
11:15-11:45 Coffee break
11:45-13:00 Scientific Session VI.
Chairs: Konrad Bork, Laurence Bouillet
11:45-12:00
O-26
Prophylaxis of angioedema attacks due to acquired C1-Inhibitor deficiency with PHA121, a novel oral bradykinin B2 receptor antagonist
Remy S. Petersen
12:00-12:15
O-27
Patient case series of hereditary angioedema with normal C1 inhibitor and factor XII mutation: Findings from an Allergy and Immunology Department in Argentina
Ricardo D. Zwiener
12:15-12:30
O-28
Characteristics of acquired angioedema due to C1-inhibitor deficiency (AAE-C1INH) at a large tertiary care hospital in Spain
Patricia Mir Ihara
12:30-12:45
O-29
Angioedema due to acquired C1-inhibitor deficiency associated to monoclonal gammopathies of undetermined significance - Characteristics of a French national cohort
Delphine Gobert
12:45-13:00
O-30
A novel diagnostic parameter for acquired C1-inhibitor deficiency
Zsófia Godnic-Pólai
13:00-14:00 Lunch break
14:00-15:30 Scientific Session VII.
Chairs: Huamin Henry Li, Miloš Ješenak
14:00-14:15
O-31
Long-term prophylaxis with Lanadelumab for children and adolescents with HAE
Ekaterina A. Viktorova
14:15-14:30
O-32
Is pre-procedural prophylaxis needed in patients receiving the newer HAE-C1INH prophylactic therapies?
Teresa Caballero
14:30-14:45
O-33
Quality of life, disease control and mental health in patients with hereditary angioedema in Slovakia – national online survey
Milos Jesenak
14:45-15:00
O-34
Effectiveness of an adapted treatment schedule for long-term prophylaxis in patients with HAE-C1-INH
Johana Gil-Serrano
15:00-15:15
O-35
Lanadelumab effectiveness and safety regardless of dosing and dosing changes in patients with hereditary angioedema from the United States and Canada: Real-world evidence from the EMPOWER Study
Stephen D. Betschel
15:15-15:30
O-36
HAE patients decision to carry on-demand treatment when away from home
Stephen Betschel
15:30-16:00 Coffee break
16:00-18:00 Poster session II.
Chairs: Peter Spӓth, Emel Aygören-Pürsün, Mauro Cancian
P-26 Evolution of health-related quality of life in patients with hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH) and its relationship with disease activity
Ines Fernandez-Concha Llona
P-27 Cardiovascular safety of the orally administered bradykinin B2 receptor antagonist PHA-022121
Brigitte Loenders
P-28 Recombinant human C1 esterase inhibitor on-demand treatment for attacks of hereditary angioedema: A European registry update
Anna Valerieva
P-30 Genetic segregation study in hereditary angioedema with normal C1-inhibitor due to F12 mutation in Southern Spanish population - an observational study
Krasimira Baynova
P-31 Case report: Off-label long-term prophylaxis with C1-esterase-inhibitor (C1-INH) s.c. in a patient with acquired C1-INH-deficiency (C1-INH-AAE)
Eva-Vanessa Ebert
P-32 Specific anti-spike IgG subclasses in patients with C1-INH-HAE (C1-esterase inhibitor deficiency hereditary angioedema) after different types of COVID-19 vaccines
Petra Kiszel
P-33 Patients with hereditary angioedema comply with regular immunization? Pilot study
Anete Grumach
P-34 C1-INH complexes as markers for classical and lectin pathway activation and complex levels in angioedema patients
Lisa Hurler
P-35 Organization and patient activity
Elena Bezbozhnaya
P-36 Hereditary angioedema with normal C1 inbibitor and cutis laxa: An unusual association
Regis Campos
P-37 Development of HAE awareness in the Czech Republic
Camelia Isaic
P-38 Efficacy of the oral bradykinin B2 receptor antagonist PHVS416 by attack symptom in the RAPIDe-1 phase 2 clinical trial for treatment of hereditary angioedema attacks
Anna Valerieva
P-39 First report of FXII gene pathogenic variant in a Mexican family with hereditary angioedema
Francisco Alberto Contreras
P-40 Hereditary angioedema with a mutation in the plasminogen gene
Signe Purina
P-41 Experience in the use of pathogenetic therapy for arresting hereditary angioedema (HAE) attacks in pediatric patients
Ekaterina A. Viktorova
P-42 Prevailing swelling of the abdominal cavity, as an atypical course of HAE
Liudmyla Zabrodska
P-43 A patient who was diagnosed with hereditary angioedema at two different times
Beáta Visy
P-44 The patient journey: From mesenteric panniculitis and angioedema to acquired angioedema
Ricardo Zwiener
P-45 An uncommon case of postpartum venous thrombosis in a patient with C1INH-HAE
Francesco Giardino
P-46 A young woman with C1-INH deficiency refractory to various treatments: The relevance of having all therapeutic strategies available
Beatrice Piazza
P-47 Safety of COVID-19 vaccines in patients with angioedema with C1 inhibitor deficiency: Data from Italian Network for Hereditary and Acquired Angioedema (ITACA)
Andrea Zanichelli
P-48 Garadacimab for hereditary angioedema prophylaxis in adolescents: Efficacy and safety from the VANGUARD Phase 3 and 3b open-label extension trial (first interim analysis)
Markus Magerl
P-49 Transmission patterns in C1-INH deficiency hereditary angioedema favours a wild-type male offspring: Our experience at Chandigarh, India
Ankur Jindal
P-50 Profile of Hereditary Angioedema from Nepal- A speck of imprint on Everest
Dharmagat Bhattarai
18:30-
Sunday, 7 May 2023
08:00-10:00 Scientific Session VIII.
Chairs: Sladjana Andrejevic, Danny Cohn
08:00-08:15
O-37
The ACARE network – An update on global angioedema projects and initiatives
Marcus Maurer
08:15-08:30
O-38
The angioedema registry of the Italian Network for Hereditary and Acquired Angioedema (ITACA): a tool to monitor HAE course, therapeutic adherence and overall burden of the disease
Mauro Cancian
08:30-08:45
O-39
The impact of puberty on C1INH-HAE course: A survey from the Italian Network for Hereditary and Acquired Angioedema (ITACA)
Riccardo Senter
08:45-09:00
O-40
Comorbidities in angioedema due to C1-INH deficiency: A survey from the Italian Network for Hereditary and Acquired Angioedema (ITACA)
Andrea Zanichelli
09:00-09:15
O-41
Pharmacoeconomic burden for HAE patients in Brazil
Marina Teixeira Henriques
09:15-09:30
O-42
Management of hereditary angioedema with normal C1Inh: About a series of 149 French cases
Laurence Bouillet
09:30-09:45
O-43
Diagnostic criteria of angiotensin converting enzyme inhibitors (ACEi) induced bradykinin angioedema: The experience from the French national reference center for angioedema
Alexis Bocquet
09:45-10:00
O-44
Autoimmune disorders in C1INH-hereditary angioedema: Preliminary data of a prospective study from an ITACA cohort
Mauro Cancian
10:00-10:30 Coffee break
10:30-11:30 Scientific Session IX.
Chairs: Aharon Kessel, Andrea Zanichelli
10:30-10:45
O-45
Health-related quality of life with garadacimab for hereditary angioedema prophylaxis: Results from a phase 2 trial
Avner Reshef
10:45-11:00
O-46
Quality of life in hereditary angioedema in Brazil: A multicentric study
Lucca Nogueira Paes Jannuzzi
11:00-11:15
O-47
Real-world effectiveness data on lanadelumab in HAE long term prophylaxis in Slovakia
Anna Bobcakova
11:15-11:30
O-48
Understanding the impact of HAE attacks on patient reported quality-of-life - analysis of real-world patient data
David Hinds
11:30-12:15 Closing Ceremony
11:30-12:00 ’Grant for Young Investigators’ Ceremony
performed by Stephen Betschel
12:00-12:10 Closing remarks
performed by Allen Kaplan
12:15-14:00 Lunch
14:00- Departure