SOVREMENNAYA PEDIATRIYA.2016.8(80):33-37; doi 10.15574/SP.2016.80.33 

Hemophilia in children: diagnostic and treatment problems 

Tsymbalyuk-Voloshyn I. P.

Lviv Regional Council Public Institution «Western Ukrainian Specialized Children's Medical Centre», Ukraine

Danylo Halytskyy Lviv National Medical University, Ukraine 

Hemophilia is X-linked congenital coagulopathy. Patients with hemophilia need life-long substituted therapy with deficient coagulation factor. In the case of early diagnosis with confirmation the type of hemophilia, it severity and inhibitor status, the disease can be successfully controled with appropriate substituted treatment. An important approach is prohilactic substituted treatment of severe hemophilia. It provides high life quality and decreases the risk of invalidity. An experience of family doctors and surgeons in special features of hemophilia helps to prevent severe or even lethal complications of the disease.

Key words: hemophilia, diagnostic, treatment, children.

References

1. Vygovska YaI. 1999. Hemorrhagic disease. Biblos, Lviv: 240.

2. Danilov IP, Zmachinskij VA, Tsvirko DG, Potapnev MP, Dashkevich EV. 2008. Hemophilia. Meditcynskie novosti. 13: 20-23.

3. Stasyshyn OV. 2011. Prophylactic treatment of hemophilia – a strategy choice. Zdorovja Ukrainy. 4: 48-49.

4. Collins PW, Blanchette VS, Fischer K, Bjцrkman S, Oh M, Fritsch S, Spotts G, Astermark J, Ewenstein B. 2009. Break-through bleeding in relation to predicted factor VIII levels in patients receiving prophylactic treatment for severe hemophilia A. J Thrombosis and Haemostasis.7: 413-420. https://doi.org/10.1111/j.1538-7836.2008.03270.x; PMid:19143924

5. Gouw SC, van der Bom JG, van den Berg HM. 2007. Treatment-related risk factors of inhibitor development in previously untreated patients with hemophilia A: the CANAL cohort study. Blood. 109 (11): 4648-4654. https://doi.org/10.1182/blood-2006-11-056291

6. Ljung R. 2016. Aspects of prophylactic treatment of hemophilia. Thromb J. 14(1): 30. https://doi.org/10.1186/s12959-016-0103-3; PMid:27766056 PMCid:PMC5056524

7. Markis M. 2012. Prophylaxis in haemophilia shoud be life-long. Blood Transfus. 10(2): 165-168.

8. Nathan DG, Orkin SH. 1998. Nathan and Oski's Hematology of Infancy and Childhood. 5-th ed. W.B. Sanders Company, Philadelphia.1; 2.

9. Petrini P. 2001. What factors should influence the dosage and interval of prophylactic treatment in patients with severe haemophilia A and B? Haemophilia. 7(1): 99-102. https://doi.org/10.1046/j.1365-2516.2001.00471.x; https://doi.org/10.1111/j.1365-2516.2001.00471.x; PMid:11136389

10. Manco-Johnson MJ, Abshire TC, Shapiro AD, Riske BMS, Hacker MR, Kilcoyne R, Ingram JD,. Manco-Johnson ML, Funk S, Jacobson L, Valentino LA, Hoots WK, Buchanan GR, DiMichele D, Recht M, Brown D, Leissinger C, Bleak Sh, Cohen A, Mathew P, Matsunaga A, Medeiros D, Nugent D, Thomas GA, Thompson AA, McRedmond K, Soucie JM, Austin H, Evatt BL. 2007. Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe Hemophilia. N Engl J Med. 357: 535-544. https://doi.org/10.1056/NEJMoa067659; PMid:17687129

11. Feldman BM, Pai M, Rivard GE, Poon M-C, Demers C, Robinson S, Luke K-H, Wu JKM, Gill K, Lillicrap D, Babin P, McLimont M, Blanchette VS. 2006. Tailored prophylaxis in severe hemophilia A: interim results from the first 5 years of the Canadian Hemophilia Primary Prophylaxis Study. Journal of Thrombosis and Haemostasis. 4(6): 1228–1236. https://doi.org/10.1111/j.1538-7836.2006.01953.x; PMid:16706965

12. Fischer K, van der Bom JG, Mauser-Bunschoten EP, Roosendaal G, Preis R, de Kleijn P, Grobbee DE, van der Berg M. 2002. The effects of postponing prophylactic treatment on long-term outcome in patients with severe hemophilia. Blood. 99 (7): 2337-2341. https://doi.org/10.1182/blood.V99.7.2337; PMid:11895765

13. Nilsson IM, Berntorp E, Lцfqvist T, Pettersson H. 1992. Twenty-five years' experiens of prophylactic treatment in sever haemophilia A and B. Journal of Interal Medicine. 232(1): 25-32. https://doi.org/10.1111/j.1365-2796.1992.tb00546.x; PMid:1640190