ISSN:
1365-2516
Source:
Blackwell Publishing Journal Backfiles 1879-2005
Topics:
Medicine
Notes:
Summary. Severe factor IX deficiency is an X-linked disorder which gives rise to spontaneous and often life-threatening bleeds. The major source of morbidity worldwide is recurrent haemarthroses, giving rise to joint destruction and deformity. However, the incidence of spontaneous haemarthroses has decreased since the advent of prophylactic home-based, on-demand, early replacement therapy.We present the case of a non-ambulant, 13-year-old boy from Chernobyl, who did not have access to this type of treatment, and whose deformity was managed using the Ilizarov external fixator.An external fixator was applied under general anaesthetic in theatre. Haemostasis was achieved by maintaining his FIX levels at 1.0 IU mL−1 pre- and post-operatively. Three months later, the fixed flexion deformity had been reduced from 50 to 5°. Four months postsurgery, this boy was walking freely without pain. There was no peri- or post-operative bleeding or joint swelling.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1046/j.1365-2516.2003.00753.x
