Al 4-lea Workshop de Deficienţă de C1 INH, Budapesta, 29 aprilie - 1 mai 2005

Friday, 29 April

Opening Ceremony
Chairpersons: H Farkas, M Cicardi

Greeting of guests:
George Füst, Henriette Farkas, Lilian Varga, George Harmat

Sand Animation Performance by Ferenc Cako
"For HAE Patients" Award to George Füst presented by Lilian Varga

Opening lecture
George Füst: Past, Present and Future of Complement Laboratories (20 minutes)

Invited experts' lectures

MM Frank, K Baig, PA Anderson, S Hanish, H Jiang, D Craig, Jr.G Quick,
R Nassar, J Jaggers:
C1 Inhibitor improves outcome in neonatal cardiopulmonary bypass

AE Davis III, C Cramer, S Cai, D Liu:
Anti-inflammatory activities of C1 inhibitor that are independent of protease inhibitor

BL Zuraw, J Herschbach:
Molecular correction of C1 inhibitor genomic DNA

 

Saturday, 30 April

Genetics
Chairpersons: T Foerster, C Druet

E Pappalardo, S Caccia, L Maggioni, LC Zingale B Cicardi and M Cicardi:
Identification of variables causing different clinical expression of hereditary angioedema

S Caccia E Pappalardo, L Maggioni, M Cicardi:
Effect of mutation within the coding region of C1-INH gene on protein function in families with HAE

O Roche, A Blanch, G Fontán, M López-Trascasa:
Characterization of splicing mutations and identification of possible exonic splicing enhancer in the C1-INH

N Monnier, D Ponard, M Abbal, D Vervloet, C Drouet:
Characterization of a mutation of the C1-INH gene at slice sequence and associated with HAE-I

T Förster, W Kreuz, I Martinez-Saguer, E Aygören-Pürsün, K Bork, CR Müller, J Oldenburg:
Mutation analysis of patients with Hereditary Angioedema in Germany

A Tordai, T Hegedus, H Farkas, L Kalmár:
HAEdb: a novel interactive, locus-specific mutation database of the C1 inhibitor gene

H Ren, H Zhang:
Identification of gene mutations of one Chinese type II HAE family

Laboratory and clinical management of C1-INH Deficiency
Chairpersons: M. Frank, E W Nielsen

LO Uttenthal, P Jensen, R Hald, NR Caterer:
Enzyme-linked immunosorbent assay (ELISA) of functional and total C1-esterase inhibitor (C1-INH)

B Bilo, G Porebski, K Obtulowicz, M Kapusta, P Obtulowicz:
The C4 serum level in Polish patients with hereditary angioedema

MD Tarzi, A Hickey, T Förster, HJ Longhurst:
Normal complement C4 does not exculde hereditary angioedema (HAE)

L Varga, G Széplaki, M Kókai, J Laki, J Wieslander, M Daha, P Garred, HO Madsen, É Németh, R Felvinci, B Visy, G Harmat, G Füst, H Farkas:
Total lectin pathway (LP) activity of complement is depressed in hereditary angioedema (HAE)

K Madalinski, M Cedzynski, A Swierzko, E Nowicka, K Obtulowicz , H Gregorek, K Dzierzanowska-Fangrat:
Do the mannan-binding lectin pathway of complement activation and some infection contribute to the pathogenesis of hereditary angioedema?

M-E Moreau, J Robillard, P Ponard, L Bouillet, M Lopez-Trascasa, A Adam, C Drouet:
Metallopeptidase activity and kinin metabolism in hereditary angioedema (HAE): effect of androgen prophylaxis

M Pedrosa, MT Caballero, A Olveira, M Bulnes, J Segura, MC López Serrano:
Usefulness of abdominal ultrasound in the follow-up of patients with hereditary C1-inhibitor deficiency

R Felvinci, B Visy, L Jakab, H Farkas:
The effect of sexual hormone alterations on the frequency of oedematous attacks in patients with HAE

Poster section (Case Reports/ Varia)
Chairpersons: A Davis, W Kreuz

T Förster, W Kreuz, I Martinez-Saguer, E Aygören-Pürsün, J Oldenburg, CR Müller:
Recombinant expression of C1-Inhibitor mutants confirms causality of missense for Hereditary Angioedema - Poster presentation

L Bouillet, D Ponard, C Drouet:
A HAE -III case associated with C1-INH claevage without serpin-protease association

B Piatkowska-Jakubas, AB Skotnicki, D Hawrylecka, P Mensah, G Porebski, K Obtulowicz, B Bilo, A Kwapinska:
C1-inhibitor therapy is effective in capillary leak syndrome due to chemotherapy - case report

E Aygören-Pürsün, I Martinez Saguer, E Rusicke, T Klingebiel, W Kreuz:
Course of two concomitant acute angioedema attacks in a in HAE-Patient treated with s.c.Icatibant

D Zabolotny, L Zabrodska, I Gogunska:
N.M. the first patient with hereditary angioedema from Ukraine

H Ren, H Zhang:
Case report: A case of hereditary angioedema with abdominal pain and ascites

B Visy, K Nagy, É Németh, G Harmat, L Varga, H Farkas:
Haematologic adverse reactions during danazol treatment

G Széplaki, L Varga, Sz Valentin, M Kleiber, I Karádi, L Romics, G Füst, H Farkas:
Adverse effects of long-term danazol prophylaxis on the lipid profiles of patients with hereditary angioedema: a possible risk factor for atherosclerosis

R Perricone, MD Guarino, S Guarino, E Giardina, S Gambardella, C De Carolis, C Perricone, L Fontana, G Novelli:
Germinal mosaicism in HAE

R Perricone, D De Nardo, M Noia, MD Guarino, S Guarino, C De Carolis, C Perricone, L Fontana:
Successful IVIg treatment of angioedema attacks in a patient with acquired C1-INH deficiency and IgG deficiency

Quality of live
Chairpersons: T Caballero, A Price

N Prior, E Remor, MC López Serrano, T González Quevedo, S Cimbollek, T Caballero:
Health-related quality of life in adults with hereditary angioedema: development of a disease specific questionnaire in Spain (phase I)

E Rusick, E Aygören-Pürsün, I Martinez-Saguer, W Kreuz:
Improvement of clinical course in Hereditary Angioedema following modification of life style

É Németh B Visy, H Farkas:
Psychiatric disorders in Hungarian patients with hereditary angioangioneurotic edema

HAE Centers
Chairpersons: H Farkas, Y Mykal

H Farkas, G Füst, L Varga:
Mission though not impossible

D Zabolotny, I Gogunska, L Zabrodska:
Start of the program to study hereditary angioedema (HAE) in Ukraine

R Stefanov, P Krastev, M Stefanova:
HAE patients healthcare and management in Bulgaria - history, present and future

K Stavric, S Peova, K Mironska, L Kareva, S Nikolovska, Lj Pavlova, M Spirovski:
Diagnosis and treatment of hereditary angioedema in Macedonia

D Moldovan, Cs Todea:
Hereditary angioedema: present and perspectives in Romania

S Cimbollek, T González-Queved, M Díaz Fernández:
Experience and goals in Familiar angioedema from the South of Spain

A Blanch, O Roche, T Caballero, N Sastre, D Callejo, M López-Trascasa:
Patient registry and approach to the prevalence of HAE in Spain

K Obtulowicz, G Porebski, B Bilo, M Kapusta, P Obtulowicz:
Diagnostic and therapeutic problems in management of patients with HAE in Cracow/Poland

MM Gompels, RJ Lock, M Abinum, CA Bethune, G Davies, C Grattan, AC Fay, HJ Longhurst, L Morrison, A Price, M Price, D Watters.:
C1 inhibitor deficiency: UK Consensus Document. Improving standards in the UK

Patients' Associations
Chairpersons: A. Castaldo, M. Raguet

A Menendez, I Nagy, V Penna, A Price, M Raguet, S Smith, A Castaldo, M Cicardi:
HAEI - International Patient Organization for C1 Inhibitor Deficiencies

C Picavet, L Baas:
Dutch patients and the patients association

Intellectual experience, than entertainments for all five senses

 

Sunday, 1 May

Therapy
Chairpersons: K Bork, A Agostoni

U Huffer:
Experiencies with HAE Long-Term Medication: A Report from the German Patient Organization

I Martinez-Saguer, E Aygören-Pürsün, E Rusicke, S Knaub, T Klingebiel, W Kreuz:
Pharmacokinetics of Pasteurized C1 Inhibitor, (Berinert in 40 Patients with Hereditary Angioedema

C Symons:
Plans for a UK Register of patients who self-infuse C1-INH concentrate at home

K Bork, G Meng, P Staubach:
Treatment of abdominal pain attacks with pasteurized C1 inhibitor concentrate in patients with hereditary angioedema

JM Muńoz, J Jurado, T Caballero, E Vázquez, N Prior, C López-Serrano, V Martorell:
Oral surgery in patients with C1 inhibitor deficiency. Our results and protocol

M Bulnes, MT Caballero, M Pedrosa, N Prior, C Cabańas, MC Lopez Serrano:
Clinical evaluation of tolerance and viral safety of C1-inhibitor concentrate in patients with recurrent angioedema caused by hereditary or acquired C1-inhibitor deficiency

W Kreuz, I Martinez Saguer, E Aygören-Pürsün, E Rusicke, T Klingebiel:
Individual replacement therapy (IRT) with a pasteurized C1-Inhibitor concentrate compared to prophylaxis with danazol in patients with Hereditary Angioedema (HAE)- a prospective study comparing quality of life parameters

A Zanichelli, LC Zingale, B Cicardi, L Maggioni, E Pappalardo, M Cicardi:
Use of C1 inhibitor concentrate for treatment of angioedema attacks in patients with C1 inhibitor deficiency. Survey of 1102 infusion in 503 patients

Results of new clinical trials
Chairpersons: M. Cicardi, B Zuraw

S Knaub:
C1 Esterase Inhibitor, Human (Pasteurized) in Subjects with Hereditary Angioedema

K Bork, J Frank, W Kreuz, L Dong, B Rosenkranz, J Knolle:
Novel approach to treatment of hereditary angioedema with Icatibant, a bradykinin receptor antagonist

B Rosenkranz, L Dong, J Knolle:
Phamacokinetic and pharmacodynamic profile of Icatibant, a bradykinin B2 receptor antagonist

J Nuijens, R Verdonk, M van Doorn, R Schoemaker, A Cohen, E Hack, G Choi, M Soeters, M Levi, H Farkas, L Varga, J László, G Temesszentandrási, B Bilo, G Porebski and K Obtulowicz:
Clinical studies of Recombinant Human C1 Inhibitor in Subjects with Hereditary Angioedema, HAE

T Craig:
Multicenter, Open-Label, Repeat-Dosing Study of DX-88 in HAE: Interim Results of the EDEMA2 Study

B Zuraw:
A Multicenter, Double-Blind, Placebo-Controlled Study of DX-88 in Hereditary Angioedema: Results of the EDEMA1 Study

M Cicardi:
DX-88 Clinical Experience Across EDEMA Trials by Localisation of HAE Attack