Scientific programme

Opening ceremony

• Greeting of guests
• ‘For HAE Patients’ Award Prize-giving ceremony

Laudation of Bruce Zuraw by Anthony Castaldo

• Let the treatment fit the disease
  Bruce Zuraw
  „For HAE Patients” Award

Opening lectures I
/Chairs: F. Marceau, M. Riedl/

• C1 Inhibitor – Where we came from to where we are going
  Alvin Schmaier

Friday, 19 May

Opening lectures II
/Chairs: E. Aygören. B. Ritchie/

• Angioedema and idiopathic systemic capillary leak syndrome
  Marco Cicardi

• Angioedema- Histamine or Bradykinin?
  Avner Reshef
  

Scientific lectures

The exploration of molecular mechanisms and advances in diagnosis – dig deep to uncover the truth
/Chairs: C. Drouet, P. Gál, P. Späth/

• Bradykinin displays a biased agonism profile in B2 receptor mutants identified in patients with Hereditary Angioedema
  Rafael Filippelli-Silva

• Design of fusion proteins that bind B1 and B2 receptors for bradykinin: progress toward diagnostic tools
  Xavier Charest-Morin

• Comparing pathways of kinin formation in human whole blood: generating endogenous agonists of the bradykinin B2 and B1 receptors
  François Marceau

• A novel potential triggering factor of HAE attacks:
  complement MASP-1 increases endothelial cell permeability
  Márta L. Debreczeni

• Modeling the activation and inhibition by C1 inhibitor of lectin pathway proteases
  József Dobó

• Algorithm to distinguish histamine and bradykinin-induced angioedema in Emergency Department
  Jacques Hébert

• Bradykinin angioedema solution for biological diagnostic
  Delphine Charignon

• Assay for functional C1 inhibitor deficiency: An ELISA that improves upon the chromogenic method
  Kusumam Joseph

Scientific lectures

The events of an angioedema episode – a tour into hell and back
/Chairs: I. Martinez-Saguer, A. Zanichelli/

• Emerging role for phospholipase enzymes in Hereditary Angioedema with C1-INH Deficiency
  Stefania Loffredo

• An observation of contact system activation in the early angioedema suggests a systemic process prior to a local process
  Arije Ghannam

• Novel high-resolution follow-up of the coagulation and fibrinolytic parameters in a single angioedematous attack of a C1-INH-HAE patient
  Nóra Veszeli

Poster session I. & Coffee break

MINGLING WITH THE AUTHORS – SCIENCE IN THE SHOP-WINDOW I.
/Chairs: T. Bowen, H. Longhurst/

• SERPING1 gene typing in the era of Next-Generation Sequencing (NGS)
  Gedeon Loules

• Hereditary angioedema: Report from the Czech registry
  Roman Hakl

• Radiation as a trigger of attacks in a misdiagnosed patient with hereditary angioedema and Hodgkin’s disease
  Anastasios E. Germenis

• The KLKB1-Ser143Asn polymorphism: A new genetic biomarker predicting the age of disease onset in patients with hereditary angioedema due to C1-INH deficiency (C1-INH-HAE)
  Panagiota Gianni, Maria Zamanakou

• Acquired Angioedema due to C1INH deficiency. Description of 12 cases and screening for free and complexed anti-C1INH autoantibodies
  Albert López-Lera

• Role of Vascular Permeability Factors in patients with Hereditary Angioedema with C1 Inhibitor Deficiency
  Anne Lisa Ferrara

• Expression of bradykinin B1 and B2 receptors on lymphocytes and monocytes during the remission and attack in patients with Hereditary angioedema (HAE)
  Wojciech Dyga

• Genetic variants in SERPING1 and F12 genes in Polish patients with hereditary angioedema type I & II
  Teofila Książek

• Development of a set of sensitive assays for measuring enzyme/c1-inhibitor complexes
  Anna Koncz

• Results from an interim analysis of a Ruconest treatment registry
  in Europe
  Roman Hakl

• The challenging management of idiopathic systemic capillary leak syndrome: survey from an Italian case series
  Maddalena A. Wu

• Complex interplay between autonomic and contact/complement systems underlying attacks of hereditary angioedema due to C1 inhibitor deficiency
  Maddalena A. Wu

• Early versus late administration of icatibant in patients with hereditary angioedema
  Irmgard Andresen

• Treatment administrated in attacks in hereditary angioedema during pregnancy and breastfeeding
  Marta Sánchez-Jareño

• Late onset of angioedema  attacks due to C1 inhibitor deficiency  – a diagnostic challenge
  Marcin Stobiecki

• Hereditary Angioedema Nationwide Genetic Study in Belarus
  Irina Guryanova

• Off-label subcutaneous use of 1500 IE C1-INH for prophylaxis in HAE? A case report.
  Melanie Nordmann-Kleiner

• The importance of C1q in diagnosis of acquired angioedema (AAE)
  Susanne Trainotti

• Hereditary Angioedema in Ukraine: A National Survey
  Liudmyla Zabrodska 

• Development of a sensitive assay for measuring C1-inhibitor protein
  Dominik Gulyás
  

Saturday, 20 May

Opening lectures III
/Chairs: K. Bork, C. Katelaris/

• Emergent nations of Eastern Europe and Hereditary Angioedema
  Dumitru Moldovan

Scientific lectures

A new identity for C1-INH therapy – a facelift for the better

• Population pharmacokinetics of subcutaneous versus intravenous C1-INH concentrate for the prevention of HAE attacks
  Bruce Zuraw

• Pharmacodynamic effects of subcutaneous C1-INH for the prevention of HAE attacks
  Marco Cicardi

• Safety and efficacy of a C1 inhibitor for the prevention of hereditary angioedema attacks in children: interim results of a Phase 3 study
  Emel Aygören-Pürsün

The diversity of inhibitory therapies and the multitude of genes – hunters and their games
/Chairs: L. Bouillet, A. Grumach, M. Magerl/

• BCX7353, a Once-Daily Oral Kallikrein Inhibitor, is Effective and Safe in the Prophylaxis of Acute Attacks in Patients with Hereditary Angioedema: Results from the First Interim Analysis of the APeX-1 Study
  Emel Aygören-Pürsün

• A Phase 3 open-label extension study of the efficacy and safety of lanadelumab for the prevention of angioedema attacks in patients with hereditary angioedema: trial design
  Marc A. Riedl

• Safety study of ATN-249, a new oral kallikrein inhibitor for hereditary angioedema
  Ira Kalfus

• FXIIa-mediated kallikrein activity discriminates between HAE and normal plasma samples and is a pharmacodynamic marker for CSL312
  Anthony Roberts

• Putative role of KLKB1 gene on HAE pathophysiology
  Renan P. Martin

• Genotyping unknown hereditary angioedema (U-HAE) and idiopathic non-histaminergic acquired angioedema (InH-AAE): Preliminary evidence of pathogenic variants
  Maria Zamanakou

• Hereditary Angioedema With Normal C1 Inhibitor: a cohort of Italian Patients
  Maria Bova

Round table discussion

Genetics of Angioedema: A Precision Medicine promise
/Moderators: N. Brown, A. Germenis/ 

• Molecular analysis of SERPING1 gene: to whom, when and how?
  Margarita López-Trascasa

• Pharmacogenetics of angiotensin-converting enzyme (ACE)
  inhibitor-associated angioedema
  Nancy J. Brown

• Genotyping C1-INH deficiency patients: Methods, pitfalls and biases
  Anastasios E. Germenis

Poster session II & Coffee break

MINGLING WITH THE AUTHORS – SCIENCE IN THE SHOP-WINDOW II.
/Chairs: J. Björkander, T. Craig, E. Hack/

• Hereditary Angioedema in a Brazilian cohort: delay in diagnosis
  Solange O.R. Valle
  

• An investigation into the importance of body mass index in case of patients with hereditary angioedema caused by C1-inhibitor deficiency
  Tamás Szilágyi

• The clinical appearance of idiopathic nonhistaminergic acquired angioedema and its comparison to other hereditary angioedema forms
  Noémi Andrási

• Home treatment with conestat alfa in attacks of hereditary angioedema due to C1-inhibitor deficiency
  Nóra Veszeli

• Canadian physician survey on the medical management of hereditary angioedema
  Lisa Fu

• Ambiguous symptoms of hereditary angioedema may delay diagnosis of concomitant diseases: two case reports
  Inmaculada Martinez Saguer

• First kinetic follow-up of symptoms and complement parameters during a hereditary angioedema attack
  Nóra Veszeli

• Hereditary Angioedema in Mexican paediatric population
  Melissa I. Espinosa

• Management of Hereditary Angioedema in Slovak Republic – National Survey Results
  Milos Jesenak

• Quality of life in 41 patients with hereditary angioedema: first report from Iranian National Registry of Hereditary Angioedema
  Maryam Ayazi

• Hereditary angioedema in Bulgaria: clinical and therapeutic characteristics
  Anna Valerieva

• A global multicenter registry of patients with different forms of angioedema without urticaria
  Francesca Perego

• Improvement in diagnostic delays over time in patients with hereditary angioedema: findings from the Icatibant Outcome Survey
  Andrea Zanichelli

• A Survey of Hereditary Angioedema in Israel
  Iris Leibovich-Nassi

• Delayed diagnosis of hereditary angioedema in an adult patient.
  A case report
  Erika J. Sifuentes

• Emergency call number for isolated angioedema:
  an 4 month experience in France
  Isabelle Boccon-Gibod

• Hereditary Angioedema: analysis of 287 attacks treated with Berinert® in the French Cohort COBRA
  Laurence Bouillet

• Management of hereditary angioedema: C1-inhibitor esterase and icatibant acetate self-administration for acute attacks
  Ana Rodríguez

• Off-Label Intramuscular Administration of Conestat Alfa (rhC1inh) in HAE Patients: a Case Series
  Anna Valerieva

• Are we ready to propose a pharmacological approach for Hereditary Angioedema with normal C1 Inhibitor (HAEnlC1INH)?
  Anete S. Grumach
  

• New mutations in C1 inhibitor gene leading to hereditary angioedema
  Camila L. Veronez

• Clinical and analytical characteristics in children with hereditary angioedema due to C1-inhibitor deficiency
  Teresa Caballero

• Hereditary Angioedema Rapid Triage (HAE-RT) Tool: A Delphi Study
  Lisa Fu

• The effect of menopause on hereditary angioedema:
  a descriptive study about 48 postmenopausal French patients
  Aurore Billebeau

• Normal C1-INH Angioedema in Israel: Phenotyping and F12 Gene Sequencing
  Avner Reshef

• The Determinants of Hereditary Angioedema Disease Severity: Geno-Phenotypical Aspects
  Nihal Mete Gokmen

• Hereditary angioedema, epilepsy and behavioral problems – Case presentation
  Katarina Stavrikj

Sunday, 21 May

Opening lectures IV
/Chairs: H. Li, P. Nordenfelt/

• Neutrophils and neutrophil signaling in inflammation
  Attila Mócsai

Scientific lectures

Accomplishments in clinical practice – a tribute to healthcare professionals

• Management of patients with hereditary angioedema with normal C1 inhibitor and without F12 gene mutations
  Konrad Bork

• Hereditary angioedema presenting in childhood is diagnosed in adulthood by non-pediatric physicians: findings from the Icatibant Outcome Survey
  Hilary Longhurst

• How age, gender, and concomitant diseases influence the clinical course of hereditary angioedema
  Inmaculada Martinez Saguer

• Comorbidities of patients with hereditary angioedema due to
  C1-inhibitor deficiency in Hungary
  Kinga V. Kőhalmi

Scientific lectures

Education&Nursing&QoL – novel hopes for a brighter future
/Chairs: H. Boysen, I. Leibovich-Nassi, W. Lumry/

• Launching a new website for the HAE International Nursing Organization
  Iris Leibovich-Nassi

• Patient’s educational therapeutic program “EDUCREAK”: fourth year assessment
  Isabelle Boccon-Gibod

• Smartphone application to record data on attack and treatment patterns in patients with hereditary and acquired angioedema: findings from a Danish cohort
  Kristian B. Kreiberg

• Hereditary Angioedema in Latin America: Are we improving?
  Anete S. Grumach

• Can routine prophylactic subcutaneous C1-inhibitor [C1-INH(SC)] alleviate psychological and physical disabilities caused by HAE? Findings from the COMPACT study (NCT01912456)
  William R. Lumry

• Health Related Quality of Life in adult patients with C1-INH-HAE
  Donatella Lamacchia

• Study of Health-Related Quality of Life and disease activity in adults with HAE in Sweden
  Patrik Nordenfelt