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Successful surgical interventions in pediatric patients with type 3 disease of von Willebrand

https://doi.org/10.21682/2311-1267-2019-6-4-55-61

Abstract

There were two pediatric patients under our supervision at Saint-Petersburg State Pediatric Medical University clinic from 2018 to 2019 with type 3 disease of von Willebrand, who needed the surgical treatment. The surgery was made with Haemate® P which is used as for long-term prophylaxis, as for short-term prophylaxis with surgical treatment for disease of von Willebrand. Application of Haemate® P was 100 % successful in both cases.

About the Authors

T. A. Andreeva
City Center for the Treatment of Hemophilia Patients, City Polyclinic № 37
Russian Federation

Cand. of Sci. (Med.), Hematologist, Head, Author ID: 954196, SPIN-code: 8529-6758.

6 Gorokhovaya St., S.-Petersburg, 191186



I. A. Lavrichenko
City Center for the Treatment of Hemophilia Patients, City Polyclinic № 37
Russian Federation

Hematologist.

6 Gorokhovaya St., S.-Petersburg, 191186



O. A. Krasheninnikova
City Center for the Treatment of Hemophilia Patients, City Polyclinic № 37
Russian Federation

Clinical Laboratory Diagnostics Docto.

6 Gorokhovaya St., S.-Petersburg, 191186


A. K. Gurina
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

6th year Student of the Faculty of Pediatrics, SPIN-code: 3711-8953, Author ID: 1038862.

2 Litovskaya St., S.-Petersburg, 194100



A. A. Bykov
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

5th year Student of the Faculty of Pediatrics, SPIN-code:4437-9031.

2 Litovskaya St., S.-Petersburg, 194100



А. L. Nesterchuk
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

6th year Student of the Faculty of Pediatrics, SPIN-code: 1278-8292.

2 Litovskaya St., S.-Petersburg, 194100



K. A. Papayan
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

Cand. of Sci. (Med.), Associate Professor Department of Faculty of Pediatrics, SPIN-code: 7437-0916, Author ID: 783499.

2 Litovskaya St., S.-Petersburg, 194100



V. V. Avramenko
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

Cand. of Sci. (Med.), Orthopedist-traumatologist, Head ofthe Surgical Department No. 2.

2 Litovskaya St., S.-Petersburg, 194100



A. G. Veselov
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

A.                 Cand. of Sci. (Med.), Orthopedist-traumatologist Surgical Department No. 2, SPIN-код: 7502-2280.

2 Litovskaya St., S.-Petersburg, 194100



S. I. Yakunin
Saint-Petersburg State Pediatric Medical University, Ministry of Health of Russia
Russian Federation

Cand. of Sci. (Med.), Pediatric Surgeon, Head ofthe Surgical Department ofthe Consultative and Diagnostic Center.

2 Litovskaya St., S.-Petersburg, 194100


References

1. Windyga J., von Depka-Prondzinski M.; European Wilate® Study Group. Efficacy and safety of a new generation von Willebrand factor/ factor VIII concentrate (Wilate®) in the management of perioperative haemostasis in von Willebrand disease patients undergoing surgery. Thromb Haemost 2011;105(6):1072–9. doi: 10.1160/TH10-10-0631.

2. Gill J.C., Lethager S., Di Paola J., Mannucci P., Bernstein J., Manco-Johnson M., Bergman G., Nichols W.L. and the Humate-P Surgery Study Group. Von Willebrand factor/factor VIII (VWF/FVIII) concentrate (Humate-P) for surgical prophylaxis of excessive bleeding in patients with severe von Willebrand disease (VWD). Poster presented at the Meeting of the World Federation of Haemophilia 2006; Poster #898.

3. Lethagen S., Kyrle P.A., Castaman G., Haertel S., Mannucci P.M. and the Haemate P Surgical Study Group. Von Willebrand factor/factor VIII concentrate (Haemate P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery. J Thromb Haemost 2007;5:1420-30. doi: 10.1111/j.1538-7836.2007.02588.x.

4. Selvam S., James P. Angiodysplasia in von Willebrand disease: Understanding the clinical and basic science. Semin Thromb Hemost 2017;43(6):572-80. doi:10.1055/s-0037-1599145.

5. Franchini M., Mannucci P. Gastrointestinal angiodysplasia and bleeding in von Willebrand disease. ThrombHaemost 2014;112:427-31. doi: 10.1160/TH13-11-0952.

6. Castaman G., Federici A.B., Tosetto A.S., Marca L.A., Stufano F., Mannucci P.M., Rodeghiero F. Different bleeding risk in type 2A and 2M von Willebrand disease: a 2-year prospective study in 107 patients. J Thromb Haemost 2012;10(4):632-8. doi: 10.1111/j.1538-7836.2012.04661.x.

7. Frankini M., Targher G., Lippi G. Prophylaxis in von Willebrand disease. Ann Hematol 2007;86:699-704. doi: 10.1007/s00277-007-0343-1.

8. Gill J.C., Castaman G., Windyga J., Kouides P. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood 2015;126(17):2038-46. doi: 10.1182/blood-2016-04-711259.

9. Scharrer I. Surgery in von Willebrand disease patients. Biomedical Progress 2002;15:18-22. doi: 10.1097/MBC.0000000000000500.

10. Kurth A.A., Ludwig G., Scharrer I. [Prevalence, pathophysiology, diagnosis and treatment of von Willebrand syndrome in orthopedic trauma patients]. Orthopade 1999;28(4):366-74. doi: 10.1007/PL00003619.

11. Oldenburg J., Young G., Santagostino E., Escuriola Ettingshausen C. The importance of inhibitor eradication in clinically complicated hemophilia A patients. Expert Rev Hematol 2018;11(11):857-62. doi: 10.1080/17474086.2018.1521718.

12. Lassila R., Holme P.A., Landorph A., Petrini P., Onundarson P.T., Hillarp A. Semin Thromb Hemost 2011;37:495-502. PMID: 22102192.

13. Berntorp E., Petrini P. Long-term prophylaxis in von Willebrand disease. Blood Coagul Fibrinolysis 2005;16(Suppl. 1):S23-S26. doi:10.1097/01.mbc.0000167659.23262.18.

14. Lethagen S. Clinical experience of prophylactic treatment in von Willebrand disease. Thromb Res 2006;118(Suppl. 1):S9-S11. doi: 10.1016/j.thromres.2006.01.021.

15. Berntorp E., Abshire T.; vWD Steering Committee. The von Willebrand disease prophylaxis network (VWD PN): exploring a treatment concept. Thromb Res 2006;118 Suppl. 1: S19-S22. doi: 10.1016/j.thromres.2006.01.016.


Review

For citations:


Andreeva T.A., Lavrichenko I.A., Krasheninnikova O.A., Gurina A.K., Bykov A.A., Nesterchuk А.L., Papayan K.A., Avramenko V.V., Veselov A.G., Yakunin S.I. Successful surgical interventions in pediatric patients with type 3 disease of von Willebrand. Russian Journal of Pediatric Hematology and Oncology. 2019;6(4):55-61. (In Russ.) https://doi.org/10.21682/2311-1267-2019-6-4-55-61

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ISSN 2311-1267 (Print)
ISSN 2413-5496 (Online)
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