Charles Explorer logo
🇬🇧

Current state of care for pediatric hemophiliacs in relation to the musculoskeletal system

Publication at Second Faculty of Medicine |
2008

Abstract

The current state of care for pediatric hemophiliacs in the Czech Republic is recorded in the national registry HEMIS - Clinical Registry for continuous and predictive evaluation of hemophilia treatment in children. Of the total number of 228 children with haemophilia, 200 patients with haemophilia A (80%) and 28 patients with haemophilia B (12%).

With a level below 1% the coagulation factor is 36%, with a level 1-5% it is 28% and with a level higher than 5% it is 36%. The diagnosis of hemophilia in children is made in 80% at the age of the first 6 years.

This is the reason why multidisciplinary coordinated comprehensive care must be provided for children with such disabilities from an early age. To prove the severity of musculoskeletal disorders, we present several cases from before the introduction of effective replacement therapy.

Depending on the location of the bleeding episodes, the joints and muscles predominate. They occur mainly in severe patients without a significant difference between hemophilia A and hemophilia B.

Close cooperation with orthopedic clinics focused on childhood was and is a necessary part of a comprehensive approach using all existing options to alleviate or modify the affected limb.