5th C1 INH Deficiency Workshop, 31 May -3 June 2007, Budapest

Thursday, 31 May

16:30-17:00 Welcome coffee

17:30-20:00 Opening Ceremony
Chairpersons: G. Füst, H. Farkas, L. Varga

Greeting of guests
George Füst
István Karádi (Dean of Semmelweis University, Faculty of Medicine)
Lilian Varga
Henriette Farkas
George Harmat

Saxophone performance
by St. Martin

"For HAE Patients" Award
to Marco Cicardi - presented by Lorenza Zingale

Opening lecture
Marco Cicardi: 1972-2007: dealing with HAE for 35 years

Invited experts' lectures
- Massimo Cugno: The kinin system and the pathophysiology of angioedema
- Péter Gál: Crystal structure of C1 inhibitor: insight into the mechanism of conformational disease

20:30 Welcome Dinner


Friday, 1 June

08:15-9:45 Basic Science and Genetics
Chairpersons: C. Drouet, G. Füst

1. C. Drouet, M. Cicardi, E. Pappalardo, N. Monnier, C. Gaboriaud, D. Ponard, O. Roche, A. Tordai, I. Wagenaar-Bos, R. Perricone, A. Bygum, L. Bouillet, H. Farkas, K. Bork, J. Lunardi, M. Tosi, M. López-Trascasa for the PreHAEAT european group: Mutations of the SERPING1 gene associated with hereditary angioedema in a european population, lessons for their relevance to the disease

2. C. Drouet, D. Ponard, J. Lunardi, N. Monnier, N. Raison, L. Bouillet, S. Cichon, L. Martin: Molecular identification of the hereditary angioedema type III

3. G. Füst, B. Blaskó, G. Széplaki, L. Varga, Zs. Ronai, Z. Prohászka, M. Sasvari-Szekely, B. Visy, H. Farkas: Relationship between copy number of genes (C4A, C4B) encoding the fourth component of complement and the clinical course of hereditary angioedema

4. D. Roem , I. Wagenaar-Bos, E. Hack, M. van Ham: The effect of glycosylation on clearance of C1-Inhibitor

5. C. Suffritti, S. Caccia, E. Pappalardo, L. Maggioni, LC. Zingale, M. Cicardi: Identification of variables causing different clinical expression of inherited C1-inhibitor deficiency (hereditary angioedema)

6. Y. Zhi, H. Zhang, S. Huang: Two novel mutations in C1 inhibitor gene leading to premature stop codons in two Chinese families with HAE

09:45-10:15 Coffee break

10:15-11:30 Laboratory management
Chairpersons: M. Cugno, L. Varga

1. E. Aygören-Pürsün, H. Stoll, E. Rusicke, I. Martinez- Saguer, W. Kreuz: Individually constant C1-Inhibitor levels during the course of the day - C1-Inhibitor shows no evidence of circadian variation

2. AG. Bellatorre, A. Zanichelli, M. Cugno, S. Griffini, L. Maggioni, L.C. Zingale, M. Cicardi: Plasmatic markers of acute attack in patients with angioedema due to C1 inhibitor deficiency

3. Zs. Kelemen, H. Farkas, B. Visy, E. Németh, G. Széplaki, G. Füst, J. Gács, L. Varga: Complement levels in hereditary angioedema

4. L. Varga, A. Bíró, G. Széplaki, L. Tóth, A. Horváth, G. Füst and H. Farkas: Anti-cholesterol antibody levels in hereditary angioedema

5. D. Zabolotny, O. Melnikov, L. Zabrodska, I. Gogunska: Clinico-immunologic characteristics of patients with HAE in Ukraine

11:30-11:45 Break

11:45-13:15 Clinical pictures
Chairpersons: T. Caballero, JHC. Gooi

1. K. Bork, P. Staubach, G. Meng: Frequent and rare symptoms in hereditary angioedema due to C1 inhibitor deficiency

2. JHC. Gooi, GJ. Toogood, NS. Ambrose: Acute abdomen in C1 Inh deficiency (1) Acute pancreatitis and cholelithiasis (2) Gangrenous appendicitis, pneumonia and wound abscess

3. E. Rusicke, I. Martinez-Saguer, E. Aygören-Pürsün, T. Klingebiel, W. Kreuz: Intraindividual and interindividual variations of symptoms in patients with hereditary angioedema

4. EW. Nielsen, H. Lilleng, R. Salvesen: Hemifacial spasm provoked by hereditary angioedema

5. A. Zanichelli, LC. Zingale, D. Lambertenghi Deliliers, M. Cicardi: Frequency, duration and course of angioedema attacks. A prospective study in patients with hereditary angioedema

13:15-14:45 Lunch break

14:45-16:00 Varia - Gynecology
Chairpersons: L. Bouillet, H. Longhurst

1. K. Bork, D. Gül, G. Dewald: Hereditary angioedema with normal C1 inhibitor activity including hereditary angioedema with coagulation factor XII gene mutations

2. L. Bouillet, I. Boccon-Gibod, K. Bork, C. Bucher, A. Bygum, T. Caballero, C. Drouet, H. Farkas, H. Longhurst, C. Massot, EW. Nielsen, D. Ponard, M. Cicardi: Disease expression in women with hereditary angioedema

3. L. Bouillet, D. Ponard, N. Monnier, I. Boccon-Gibod, H.Roussset, J. Lunardi, C. Massot, S. Cichon, C. Drouet: Type III angioedema: about 3 informative families

4. S. Cimbollek, T. González-Quevedo, M. Díaz: To become or not to become pregnant with hereditary angioedema

5. D. Lambertenghi Deliliers, R. Castelli, LC. Zingale, M. Cicardi: Lymphoproliferative disease and acquired C1 inhibitor deficiency

6. I. Martinez-Saguer, E. Rusicke, E. Aygören-Pürsün, T. Klingebiel, W. Kreuz: Different manifestation of swelling attacks in patients with Hereditary Angioedema during pregnancy � A follow up of 34 pregnancies

16:00-16:30 Coffee break

17:30 Social program

Revenge - dance production in two acts (National Theater)
Dinner - at Sir Lancelot Restaurant


Saturday, 2 June

08:30-10:30 Patients' Associations and management
Chairpersons: T. Bowen, A. Menendez, S. Smith-Foltz

1. T. Bowen, B. Ritchie, J. Heber, E. Wagner, K. Brosz, J. Brosz, P. Adomaitis, J. Burnham, B. Yang: Canadian 2006 International Consensus Algorithm for the Diagnosis, Therapy and Management of Hereditary Angioedema � Canadian Review and Experience 2007

2. T. Caballero, S. Cimbollek, R. Cabańas, A. Campos, C. Gómez-Traseira, MT. González-Quevedo, M. Guilarte, J. Jurado-Palomo, JI. Larco, MC. López-Serrano, C. Marcos, M. Pedrosa, N. Prior, M. Rubio: Introduction to the Spanish Clinical Group for the Study of Angioedema due to C1 inhibitor deficiency (SGACI)

3. T. Castaldo: HAE International (HAEI)

4. G. Harmat: In what ways should European action help support the health systems of the Member States?

5. D. Moldovan: Romanian Hereditary Angioedema Network (PDF)

6. P. Nordenfelt, L. Mallbris, J. Björkander, P. Hellström, AK. Lefvert, A. Lindfors, L. Lundblad, K. Löfdahl , L. Nordvall, L. Truedsson, S. Werner, CF. Wahlgren: Sweha a swedish project that will survey HAE in Sweden

7. N. Prior, E. Remor, C. Gómez Traseira, MC. López Serrano, V. Cardona, S.Cimbollek, T. González Quevedo, M. Guilarte, D. Hernández , C. Marcos, M. Rubio, T. Caballero: Development of a disease specific health-related quality of life (HRQOL) questionnaire in adults with hereditary angioedema due to C1 inhibitor deficiency (HAE)

8. LC. Zingale, K. Bork, H. Farkas, A. Bygum, L. Bouillet, T. Caballero, H. Longhurst, EW. Nielsen, B. Bilo, C. Bucher, M. Cicardi: The European Register of Hereditary Angioedema: Experience and Preliminary Results

10:30-11:00 Coffee break

11:00-12:00 Poster section
Chairpersons: D. Moldovan, EW. Nielsen

1. L. Beinrohr, J. Dobó, V. Harmat, Zs. Lőrincz P. Gál and P. Závodszky: Crystal structure of C1-inhibitor: understanding the mechanism of heparin potentiation

2. R. Felvinci, E. Németh, L. Varga, B. Blaskó, A. Szilágyi, L. Kalmár , H. Farkas: Examination of twins with hereditary angioneurotic edema

3. J. Gács, É Németh, B Visy, Zs. Kelemen, K. Miklós, J Németh, L. Varga, H. Farkas: Immunregulatory disorders associated with hereditary angioneurotic edema (HAE)

4. V. Grivcheva-Panovska,, K. Stavrik: HAE in Macedonia

5. É. Németh, B. Visy, L. Varga, G. Füst, A. Kiss, I. Takács, H. Farkas: Acquired C1-inhibitor deficiency and multiple myeloma

6. FD. Popescu, M. Vieru, D. Moldovan: A study of drug-induced angioedema without urticaria  (PDF)

7. B. Rosenkranz, J. Knolle: Efficacy and safety profile of the potent and selective bradykinin B2 receptor antagonist Icatibant in healthy volunteers

8. B. Visy, G. Széplaki, Zs. Kelemen, É. Németh, J. Gács, R. Felvinci, L. Varga, G. Harmat and H. Farkas: Does danazol cause liver damage in HAE?

9. D. Zabolotny, O. Melnikov, L. Zabrodska, I. Gogunska: Cryterium immunology in forming group of risk among relation patients with HAE.

10. Yuxiang Zhi, Hongyu Zhang: Clinical study on Chinese patients with Hereditary angioedemay

12:30- Outside programme in Lajosmizse, including lunch, dinner, "Puszta" (Ranch) Olympic Games, dance


Sunday, 3 June

08:30-10:15 Treatment
Chairpersons: W. Kreuz, L. Zingale

1. K. Bork, P. Staubach: Treatment of skin swelling attacks with pasteurized C1 inhibitor concentrate in patients with hereditary angioedema

2. H. Farkas, Gy. Temesszentandrási, B. Visy, Gy. Harmat, L. Varga, G. Füst, G. Széplaki, B. Fekete, I. Karádi, L. Jakab: A decade of human C1-inhibitor concentrate therapy

3. JHC. Gooi, S. Savic, PMD. Wood: Coronary heart disease risk in C1 Inh deficiency

4. W. Kreuz, E. Rusicke, I. Martinez-Saguer, E. Aygören-Pürsün, T. Klingebiel: Self administration of C1-inhibitor-concentrate in patients with hereditary or acquired angioedema

5. M. Pedrosa, T. Caballero, T. Lobera, G. Gala, C. Panizo, J. Jurado, R. Cabańas: Long term prophylaxis with intravenous plasma human C1 inhibitor concentrate (phC1INH) in three patients with hereditary angioedema (HAE)

6. G. Széplaki, R. Szegedi, L. Varga, Z. Prohászka, Z. Széplaki, L. Romics, I. Karádi, G. Füst, H. Farkas: Proatherogenic lipid profile does not lead to increased carotid intima media thickness in HAE patients with longterm danazol prophylaxis

7. LC. Zingale, A. Zanichelli, D. Lambertenghi Deliliers, AG. Bellatorre, L. Maggioni, M. Cicardi: Use of human C1 inhibitor concentrate in 473 Italian patients with C1 inhibitor deficiency: survey of 1001 infusions

10:15-10:45 Coffee break

10:45-12:45 Clinical trials
Chairpersons: K. Bork, M. Cicardi

1. M. Bas, TK. Hoffmann, J. Greve, H. Bier, G. Kojda: Treatment of laryngopharyngeal and lingual hereditary angioedema with bradykinin-B2-receptor-antagonist Icatibant

2. JJ. Hofstra, CW. Choi, P. Strengers, JJ. Marcar, MM. Levi,RA. Cuperus: Pharmacokinetics, clinical efficacy and safety of C1 inhibitor concentrate (C1-esteraseremmer-N) for treatment of hereditary (and acquired) angioedema

3. P. Kiessling: BerinertŽ P � standard of care for the treatment of acute attacks in HAE: first results of an ongoing prospective open label study in North America

4. RJ. Levy, SD. Goodman: A companion survey for subjects with C1 esterase inhibitor deficiency enrolled in a phase III open label extension study (CE1145_3003) of human pasteurized C1 esterase inhibitor concentrate (BerinertŽ P)

5. H. Li, R. Levy, D. McNeil, T. Schmalbach: Interim open-label results of EDEMA3Ž: a phase 3 study of subcutaneous DX-88 (ecallantide) in patients with hereditary angioedema

6. W. Lumry, T. Schmalbach: Clinical experience with DX-88 (ecallantide) in patients with hereditary angioedema

7. J. Nuijens, R. Verdonk, T. Resink, S. Visscher, M. van Doorn, G. Choi, M. Soeters, M. Levi, C. Hack, H. Farkas, L. Varga, B. Bilo, G. Porebski, K. Obtulowicz, M. Pedrosa and T. Caballero: Open-label studies of recombinant human C1 Inhibitor (rhC1INH) in patients with acute attacks of hereditary angioedema

8. A. Reshef, I. Leibovich: Our experience with Icatibant, a new bradykinin B2 receptor antagonist, in acute Hereditary Angioedema attacks

12:45-13:00 Closing Remarks
Peter Späth

13:00-14:30 Lunch

14:30 Departure