Pilot Moldovan Cluj

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Pilot Moldovan Cluj

  1. 1. AP i l ot Refere nce ntre for Ce H ered itary dem a in An gi oe Rom a ni a Dumitru MOLDOVAN *, Enikö MIHÁLY ** * University of Medicine and Pharmacy Tîrgu Mureş Allergology Department, Mures County Hospital ** Romanian Hereditary Angioedema Network
  2. 2. What is Hereditary Angioedema?
  3. 3. recurrent, recurrent, non-pruritic, nonpainful angioedema in the subcutis or submucosa Throat Glottal edema, laryngeal edema starting with swallowing difficulties, voice changes, Abdomen hoarseness, dyspnea, in Vomiting, colicky pain, extreme cases suffocation diarrhea, malaise Skin Swelling and feeling of tension and pressure
  4. 4. antige Functi Anti- TYPE C1q n C1- onal C4 INH C1-INH C1-INH HAE-I N ↓ ↓ ↓ - HAE-II N N/↑ ↓ ↓ - AAE-I ↓ ↓ ↓ ↓ - AAE-II (autoimune ↓ N/↓ ↓ ↓ + )
  5. 5. The beginning
  6. 6. 2004 Upgrade course in Allergology and Clinical Immunology organized yearly by the Hungarian Society of Allergology and Clinical Immunology (Budapest, December)
  7. 7. 4th C1 INH Workshop Budapest, Ibis Vaci út Spring, 2005 14:00-15:45 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
  8. 8. Romania is far behind other nations in the diagnosis and treatment of HAE patients HAE is usually No HAE centre, misdiagnosed and no register of HAE patients No drug for the treatment of HAE is available in Romania No genetic studies in HAE Previous intentions to register C1 INH purified extracts from plasma were unsuccessful
  9. 9. Map of HAE in Romania 2005 c cases Only sporadi ta base i dual da i n indiv
  10. 10. International support would be of paramount importance HAE exchange programme Regional / International support for clinicians for complement laboratory
  11. 11. Long term difficulties  unable to access appropriate care − 40% rural − poor income  chronic lack of funds of the state insurance company
  12. 12. Have started to work
  13. 13. Aim • To build-up a national data base
  14. 14. Defining skils WORKSHOPS ARTICLES PPT PRESENTATIONS INTERNET SITE LEAFLETS
  15. 15. Targets for the HAE n e t w o r k (1) Allergist Internal Medicine Dermatologist Peditrician
  16. 16. Targets for the HAE n e t w o r k (2) Gastroenterologist Anesthesiologist ENT specialist Surgeon
  17. 17. Targets for the HAE n e t w o r k (3) General practitioner
  18. 18. Targets for the HAE network Complement laboratory Local HAE experts National board of HAE centres Ministry of Health
  19. 19. HO PS OR KS W
  20. 20. Allergology and Clinical Immunology meetings in Romania and HAE workshops 4th Balkan Congress of Allergy and Clinical Immunology Bucharest September 2005 The Annual Conference of the Romanian Society of Allergology and Clinical Immunology Cluj-Napoca March 2006 3th HAE course Budapest 8-10 March 2006 4th HAE course Budapest 5-7 October 2006 First Congress of the Romanian Society of Allergology and Clinical Immunology Tirgu Mures, April 2007 Summer School Cluj September 2007 Diagnostic and treatment opportunities and limitations in care of patients with primary imunodeficiencies in Romania and Moldavia Iaşi 12-13 octombrie 2007
  21. 21. 4th Balkan Congress of Allergy and Clinical Immunology Bucharest September 2005 HEREDITARY ANGIOEDEMA SYMPOSIUM Chairpersons: Marco Ciccardi, Henriette Farkas Henriette Farkas, Lilian Varga: Pathomechanisms, clinical pictures and diagnosis of hereditary angioedema Marco Cicardi:Hereditary angioedema: from pathophysiology to treatment Lilian Varga, Henriette Farkas: Regional HAE Network Dumitru Moldovan, Csilla Todea: Steps in the improvement of management of hereditary angioedema in Romania
  22. 22. The Annual Conference of the Romanian Society of Allergology and Clinical Immunology Cluj-Napoca March 2006 WORKSHOP: HEREDITARY ANGIOEDEMA: PRESENT AND FUTURE Chairpersons: Henriette Farkas, Dumitru Moldovan Florin Dan Popescu (Bucharest, Romania): Differential diagnosis of angioedema without urticaria Henriette Farkas (Budapest, Hungary): Clinical features of herediatary angioedema (HAE) Peter J. Späth (Switzerland): Pathomechanism and Diagnosis of HAE Henriette Farkas (Budapest): Therapy of HAE Sylvia Herget (Germany): IMPACT. International, multicenter, prospective, angioedema, C1-INH trials. The C1-INH program with Berinert P for HAE a ZLB Behring S Visscher , R Verdonk , R Schoemaker , G Choi , M Soeters, M Levi, H Farkas, L Varga, J László, G Temesszentandrási, B Bilo, G Porebski, K Obtulowicz, J Nuijens: Open-label Studies of Recombinant Human C1 Inhibitor (rhC1INH) in Subjects with Acute Attacks of HAE Lilian Varga (Budapest): HAE NETWORK Dumitru Moldovan (Tîrgu Mureş): HAE network in Romania Dumitru Moldovan, Lorena Orosz, Linda Katona (Tîrgu Mureş): Internet site of the Romanian HAE Network Association
  23. 23. 3th HAE course Budapest 8-10 March 2006 4th HAE course Budapest 5-7 October 2006
  24. 24. TICLES AR & A bstr acts
  25. 25. Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C, Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC, Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E, Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz W, Rusicke E, Martinez-Saguer I, Aygoren-Pursun E, Harmat G, Fust G, Li H, Bouillet L, Caballero T, Moldovan D, Spaeth PJ, Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang ZY. Hereditary angiodema: a current state-of-the- art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Management of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2008;100(Suppl 2):S30–S40. Craig TJ, Levy RJ, Wasserman RL, Bewtra AK, Hurewitz D, Obtułowicz K, Reshef A, Ritchie B, Moldovan D, Shirov T, Grivcheva-Panovska V, Kiessling PC,Bernstein JA. Efficacy of Human C1-Esterase Inhibitor (C1-INH) concentrate compared to placebo in patients with acute abdominal or facial HAE attacks. J Allergy Clin Immunol (submitted 2009). Kiessling PK, Craig T, Levy R, Wasserman RL, Bewtra AK, Hurewitz D, Obtulowicz K, Reshef A, Ritchie B, Moldovan D, Shirov T, Grivcheva-Panovska V, Kiessling PC, Bernstein J. Treatment of hereditary angioedema with human C1 esterase inhibitor: results of a global, multicenter, randomized, placebo- controlled, phase II/III dose finding study (I.M.P.A.C.T.1) Clin Experimental Immunol, 154 (Suppl. 1), 1-224, 2008. Moldovan D. Angioedemul ereditar. De la un caz la o reţea naţională. Medic.ro 4, 31, supl.85-88, 2007.
  26. 26. Moldovan D. Angioedemul ereditar: o afecţiune cu potenţial letal. Al VI-lea Congres Naţional de Medicină Internă, 6-8 aprilie, 2006, Călimăneşti-Căciulata. EMC 0903/27/02/2006. Moldovan D. Hereditary angioedema: a life-threatening disorder. J Romanian Soc Allergology Clin Immunol 2005; II, 3:18-24. Moldovan D. Hereditary angioedema: a multifaced disease. EAACI & Ga2len Allergy School, Cluj-Napoca, 5-10 Sept. 2007. Moldovan D. Mission is possible. J Romanian Soc Allergol- ogy Clin Immunol 2005; II, 3:47. Moldovan D. Our first 3 years of experience in managing he- reditary angioedema in Romania. Annual Conference of the Rom Soc Allergol Clin Immunol, Baia Mare, 1-4 mai 2008. Moldovan D. Reţeaua Română de Angioedem Ereditar. Conferinţa „Bolile rare: de la evaluarea nevoilor la stabilirea priorităţilor”. UMF „Victor Babeş” Timişoara, Zalău, 2-3 noiembrie 2007. Moldovan D. Romanian Hereditary Angioedema Network. 5th C1 inh Deficiency Workshop, Budapest, 31 May-3 June 2007. Abstract book, 53. Moldovan D. Reţeaua de angioedem ereditar în România. Conferinţa Naţională Anuală de Alergologie şi Imunologie Clinică, 24-26 Martie 2006, Cluj-Napoca.
  27. 27. Moldovan D, Dobreanu M, Mihaly E. Hereditary Angioedema: diagnostic difficulties, the Romanian Network for HAE, and IMPACT study. A 36-a Conferinţă Naţională de Imunologie, Tîrgu Mureş, 21-23 septembrie 2006. Moldovan D, Dobreanu M, Popescu FD, Vieru M, Cristea C, Tătaru A. The Romanian Network for Hereditary Angioedema. Present and perspectives. 1st Congress of the Romanian Society of Allergology and Clinical Immunology, Tîrgu Mures, 26-28 April, 2007. Moldovan D, Levy RJ, Visscher S, Relan A, Nuijens JH, Hack CE. Interim results from ongoing open-label studies with recombinant C1 inhibitor (Rhucin; rC1INH) for treatment of patients with acute attacks of hereditary angioedema. Oral presentation. 6th Workshop on Hereditary Angioedema. Budapest, 21-24 May, 2009. Moldovan D, Mihaly E. A MOGYE es a SOTE egyutmukodése a herediter angioodemás betegek ellatásában. Orvostudomanyi ertesito, 82, suppl.1, 39 p, 2009. Moldovan D, Mihály E. Angioedemul ereditar: o imunodeficienţă primară relativ bine conturată. Al 21-lea Simpozion “J Project”. Posibilităţi şi limite în diagnosticul imunodeficienţelor primare în România şi Moldova” 12-13 octombrie 2007, Iaşi. Moldovan D, Mihaly E. Angioedemul ereditar: o imunodeficienţă primară subevaluată. Conferinţa de Imunodeficienţe Primare, J Project, Buziaş 19-20 martie 2009. Moldovan D, Mihály E. Open label treatment with Rhucin. The Experience from Romania. Investigator meeting. Amsterdam 2008.
  28. 28. Moldovan D, Mihaly E, Popescu FD, Dobreanu M. Hereditary C1- inhibitor esterase deficiency: a rather well-defined entity. Rom Rev Lab Med 2009 (in press). Moldovan D, Mihály E, Szöllősi GA. A romániai herediter angiooedema hálózat és nemzetközi együttműködése 36th Congress of the Hungarian Society of Allergology and Clinical Immunology, Siofok, Hungary, May 15-17 2008. Moldovan D, Mihály E, Szöllösi, G. Model de asistenţămedicală multicentrică în bolile rare: Reţeaua Română de Angioedem Ereditar. Sesiunea Ştiinţifică Anuală a Cadrelor didactice, UMF Tîrgu Mureş, 13-14 Dec 2007. Moldovan D, Orosz L, Katona L. Site-ul de Internet al Asociaţiei Române pentru angioedemul ereditar. Conferinţa Naţională de Alergologie şi Imunologie Clinică. Cluj-Napoca, 2006. Moldovan D, Tătaru A. Angioedemul ereditar: de la erori de diagnostic la o reţea naţională. Congresul National de Dermatologie, Bucureşti, 1-4 noiembrie, 2006. Moldovan D, Todea Cs. Angioedemul ereditar: o boală rară, dar uneori fatală. Conferinţa Naţională a Societăţii de Pediatrie, Galaţi, octombrie, 2005. Moldovan D, Todea Cs. Hereditary angioedema: present and perspectives in Romania. 4th C1 inhibitor Deficiency Work- shop. Budapest, 29 April-1 May 2005, Abstract book pg.40.
  29. 29. Moldovan D, Todea Cs. Steps in the improvement of man- agement of hereditary angioedema in Romania. 4th Balkan Congress Allergy Clin Immunol Bucureşti, 22-25 Sept 2005, Abstract book pg. 24. Nuijens J, Verdonk R, Giannetti B, Visscher S, van Doorn M, Resink T, Levi M, Hack C, Farkas H, Obtulowicy K, Caballero T, Perricone R, Cancian M, Montinaro V, Cillari E, Gonzales-Quevedo T, Moldovan D, Schelzinger M, Trig- giani M, Longhurst H, Neri S, Cicardi M. Clinical studies of recombinant human C1 ihibitor (rhC1INH) in Patients with Acute attacks of Hereditary Angioedema. 1st International Leadership Conf. Frankfurt/Main, 19-21 Oct 2007. Popescu FD, Vieru M, Moldovan D. A study of drug-induced angioedema without urticaria 5th C1-INH Deficiency Workshop, Budapest, 31 May-3 June 2007. 32. Szöllösi G, Mihály E, Moldovan D. Romanian Hereditary Angioedema Network, Allergy, 2007,62: S83: 479. 33. Tătaru A, Moldovan D. Angioedemul ereditar: de la fiziopa- tologie la tratamentul modern. Congresul National de Der- matologie, Bucureşti, 1-4 noiembrie, 2006. Moldovan D. Hereditary Angioedema, an example for the understanding and management of rare diseases. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 3-6. Mihály E, Moldovan D. Some genetic issues in hereditary angioedema. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 7-10. Moldovan D, Popescu FD. Pathophysiology of hereditary angioedema. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 11-15.
  30. 30. Moldovan D, Mihály E, Popescu FD. Clinical manifestations and diagnosis of hereditary angioedema. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 16-21. Mihály E, Moldovan D, Dobreanu M. Laboratory diagnosis in evaluation of patients with hereditary angioedema. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 22-28. Moldovan D, Mihály E. The treatment of angioedema caused by C1- Inhibitor deficiency. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 29-34. Mihály E, Moldovan D. Other therapeutic applications of C1- esterase inhibitor. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 35-38. Căpâlnă B, Moldovan D. Hereditary angioedema in children. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 39-45. Nădăşan V, Moldovan D. Hereditary angioedema patient education and professional Web resources. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 46-49. Moldovan D, Mihály E, Dobreanu M, Brassai T, Popescu FD, Vieru M, Deleanu D, Cristea V, Agache I, Ungureanu M, Leru P, Iamandescu B, Tătaru A, NădăN Hereditary Angioedema. Four-years experience. Revista de Medicină şi Farmacie - Orvosi és Gyógyszerészeti Szemle, 2009 55, s3, 50-56.
  31. 31. Romanian Hereditary Angioedema Network Founded in 2006
  32. 32. F LE TS L EA
  33. 33. si te rn et In te
  34. 34. ne t.ro W.hae WW
  35. 35. 5th C1 INH workshop Budapest Spring 2007 08:30-10:30Patients’ Associations and management Chairpersons: T. Bowen, A. Menendez, S. Smith-Foltz 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 T. Caballero, S. Cimbollek, R. Cabañas, 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) T. Castaldo: HAE International (HAEI) G. Harmat: In what ways should European action help support the health systems of the Member States? D. Moldovan: Romanian Hereditary Angioedema Network 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 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)
  36. 36. 2008
  37. 37. Annual Conference of Allergology and Clinical Immunology Baia Mare, May 2008 Workshop: C1 inibitor deficiency (Deficienta de C1-inhibitor esteraza) Konrad Bork (Guthenberg University, Maiz, Germany): Clinical manifestations of hereditary angioedema Lilian Varga (Semmelweis University Budapest, Hungary): Diagnosis of hereditary angioedema Henriette Farkas (Semmelweis University Budapest, Hungary): New therapies in hereditary angioedema J Nuijens, R Verdonk, B Giannetti, S Visscher, M van Doorn, T Resinka, M Levic, C Hack, H Farkas, K Obtulowicz, T Caballero, R Perricon*, M Cancia, V Montinaro, E Cillari, T Gonzales- Quevedo, D Moldovan, M Schlezinger, M Triggiani, H Longhurst, S Neri, M Cicardi: Clinical Studies of Recombinant Human C1 Inhibitor (rhC1INH) in Patients with Acute Attacks of Hereditary Angioedema Ioana Agache, Liliana Duca (Transylvania University, Medicine Faculty, Department of Immunology-Allergology, Brasov): Our experience on acquired C1 inhibitor deficiency in autoimmune disorders Dumitru Moldovan, Enikö Mihály, Géza-Attila Szöllösi (University of Medicine and Pharmacy, Tirgu Mures): Three years experience in managing hereditary angioedema in Romania
  38. 38. 14.00 – 14.45 HANO SZIMPÓZIUM Üléselnök: Dr. Farkas Henriette, Dr. Kadocsa Edit 14.00–14.20 Icatibant - a novel treatment for hereditary angioedema Werner Aberer /JERINI szimpózium University of Graz, Department of Dermatology, Graz, Austria 14.25–14.35 Új eredmények a Herediter angioneuroticus oedema kutatásában és kezelésében Farkas H., L. Varga, G. Széplaki, Gy. Temesszentandrási, L. Jakab, B. Fekete, G. Füst, I. Karádi 14.35–14.45 A romániai herediter angiooedema hálózat és nemzetközi együttműködése Moldovan D., Mihály E., Szöllősi GA.
  39. 39. FP7
  40. 40. The National Alliance for Rare Diseases in Romania signed a cooperation protocol with Ministry of Health, and in the coming year are expected a development of centres of excelence.
  41. 41. Good news The Romanian HAE Network have started to work The awareness of HAE is improving 40 of our patients have C1 INH antigen and function measured Some improvement in complement laboratory Two clinical trials, one still ongoing
  42. 42. Map of HAE in Romania 2008 ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗ ∗
  43. 43. 6th C1 INH workshop Budapest May 2009
  44. 44. Map of HAE in Romania 2009
  45. 45. 53 confirmed cases  10 were diagnostized in Romania  21 diagnosed in Budapest, and 22 in Karlsruhe and Groningen  belong to 19 families  7 are type II and 44type I  we didn’t find yet any type III HAE patients
  46. 46. DEZIDERATA HAE REFERENCE CENTRE COMPLEMENT LABORATORIES HAE REGISTRY PATIENTS’ ORGANIZATION REGISTRATION OF DRUGS FOR EMERGENCY AND PREVENTIVE TREATMENT
  47. 47. TASKS OF THE CENTER  Diagnostic procedure  Differential diagnostic procedure  Treatment  Follow-up  Register  Spread of knowledgeed  Educating of colleagues (postgraduate and graduate courses)  Research  Conference activity  Publications  Joining to the international projects (research and trials)
  48. 48. Why Us?
  49. 49. i o na l te rna t G ood i n coope ra ti o n
  50. 50. What can we offer?  HAE training  Diagnostic and therapeutic protocols  Measurements of complement parameters  Telephone and e-mail hot-line service for physicians and patients  Storage of samples (-800 C)  Referals to the most outstanding HAEcentres
  51. 51. Acknowledgments Patients
  52. 52. Acknowledgments Industry
  53. 53. Acknowledgments Network for Complement Related Diseases
  54. 54. Colleagues and friends • Prof. dr. Henriette Farkas • Dr. Lilian Varga • Prof.dr. Gyorgy Fust • Prof. dr. Marco Cicardi • Prof. dr. Konrad Bork • Peter Spaeth • Tom Bowen • Avner Reshef • Timothy Craig
  55. 55. Thank you

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