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Current advances in the care for pati ents with haemophili a


Authors: M. Penka 1,3;  J. Blatný 1,3;  M. Matýšková 1,3;  P. Smejkal 1,3;  E. Tesařová 2,3
Authors place of work: Oddělení klinické hematologi e FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Miroslav Penka, CSc., 2Transfuzní oddělení a krevní banka FN Brno, pracoviště Bohunice, přednostka prim. MU Dr. Eva Tesařová, 3Univerzitní centrum pro trombózu a hemostázu 1
Published in the journal: Vnitř Lék 2009; 55(Suppl 1)(Supplementum 1): 48-51

Summary

Haemophili a is a dise ase known from antiquity and always stirring significant expert and lay discussi on. This is an infrequent dise ase that, nevertheless, is characterized by certain regulariti es that have rather unusu al but often paradigmatic nature. Non- medical issues, at present in particular, are predominantly financi al. It is, therefore, necessary to address the issue of haemophili a in detail and to attempt to provide comprehensive soluti ons based on se amless organizati on and cle ar definiti ons of the character and extent of care as well as on rati onal utilizati on of all available reso urces provided by the current medicine and age. The paper revi ews development of the care for haemophili a pati ents in the past and up to the present time.

Key words:
haemophili a –  substituti on –  CCC/ HTC –  multi‑professi onal care


Zdroje

1. Blatný J. Inhereted co agulati on disorders (Vrozené trombofilní stavy). In: Starý J et al (eds). Dětská hematologi e. Trendy so udobé pedi atri e, svazek 4. Praha: Galén 2005: 189– 203.

2. Blatny J, Kohlerova S, Komrska V et al. Bleeding Prophylaxis in Children with Haemophili a A/ B Using Plasma- derived Concentrates Immunate/ Immunine –  Prospective Survey. J Thromb Haemost 2005; 3 (Suppl 1): Abstract P 1998.

3. Blatny J, Kohlerova S, Zapletal O et al. Prophylaxis with recombinant factor VIIa for the management of bleeding episodes during immune tolerance tre atment in a boy with severe haemophili a A and high‑response inhibitors. Haemophili a 2008; 14: 1140– 1142.

4. Bozděch Z, R.Moster J, Stryhal J et al. Totální protéza kolenního klo ubu u hemofiliků. Acta Chir Orthop Tra um Čech 1983; 50: 228– 233.

5. Colvin BT, Astermark J, Fischer K et al. Inter Disciplinary Working Gro up. Europe an principles of haemophili a care. Haemophili a 2008; 14: 361– 374.

6. Husa P, Roznovský L, Smejkal P et al. Efficacy and safety of chronic hepa­titis C tre atment in haemophilic pati ents. Hepatogastroenterology 2005; 52: 1541– 1544.

7. Matýšková M, Penka M. Hemofili e. Vnitř Lék 1991; 37: 397– 403.

8. Morfini M, Auerswald G, Kobelt RA et al. Prophylactic tre atment of haemophili a pati ents with inhibitors: clinical experi ence with recombinant factor VIIa in Europe an Haemophili a Centres. Haemophili a 2007; 13: 502– 507.

9. Moster R, Penka M, Matěja F. Operační léčba pohybového aparátu u krvácivých stavů. Acta Chir Orthop Tra um Čech 1986; 53: 54– 61.

10. Penka M, Smejkal P, Buliková A et al. Multiple Orthopaedic procedures in Hemophili ac Pati ents. Reports from the 17th Internati onal Congress on Thrombosis, Invited reports to Symposi a, Monduzi editore, Internati onak Proceedings Divisi on, Bologna, Italy, Oct 2002, 63– 66.

11. Penka M, Dušek L, Smejkal P et al. Project HemIS (Haemophili a- Informati on System): multicentric informati on system of evalu ati on of clinical data of pati ents with haemophili a. Haemophili a 2004; 10 (Suppl 3): 21– 22.

12. Penka M. Vrozené krvácivé stavy. In: Indrák K et al (eds). Hematologi e. Vnitřní lékařství –  postgradu ální klinická project. Praha: Triton 2006/ 2007: 110– 117.

13. Salaj P, Brabec P, Penka M et al. Effect of rFVIIa dose and time to tre atment on pati ents with haemophili a and inhibitors: Analysis of HemoRec Registry data from the Czech Republic. Haemophili a 2009; 15: 752– 759.

14. Smejkal P, Mužík J, Penka M et al. The use of FEIBA® and NovoSeven® for tre atment of bleeding episodes in pati ents with hemophili a A and factor VIII inhibitor: a single centre experi ence. Haematologica 2006; 91 (Suppl 1): 156– 157.

15. Smejkal P, Matýšková M, Penka M. Hemofili e. Vnitř Lék 2008; 54: 992– 999.

16. Smejkal P, Matyskova M, Chlupova G et al. Haemophili a pati ents in So uth Moravi a: Survey of the preceeding 20 ye ars. Hämostase ologi e 2008; 28 (Suppl 1): 21– 23.

Štítky
Diabetology Endocrinology Internal medicine

Článok vyšiel v časopise

Internal Medicine

Číslo Supplementum 1

2009 Číslo Supplementum 1
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