Title Sąnarių pažeidimo dėl kraujavimo vertinimas hemofilija sergantiems vaikams taikant skirtingus gydymo būdus /
Translation of Title Assessment of the development of blood-induced joint damage in children with hemophilia in relation to different treatment strategies.
Authors Šaulytė Trakymienė, Sonata
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Pages 138
Keywords [eng] hemophilia ; joint damage ; assessment of joint damage ; HJHS
Abstract [eng] Hemophilia care in Lithuania, especially for children, was largely inadequate compared to modern standards. Replacement therapy with clotting factors at bleeding episodes only results in joint damage, however, it is unclear at which age it will manifest. The study purpose was to assess demographic data, analyze treatment and investigate the development of musculoskeletal damage in patients with hemophilia by utilizing a new joint evaluation method (HJHS) to determine its applicability and utility in children treated by on-demand principles and progression of joint damage focusing on the age of remarkable changes in musculoskeletal health and treatment strategy. The prevalence of hemophilia for males aged 0-17 years was 15.3/100 000. Severe hemophilia constituted 60% of all cases. The mean incidence rate between 1992-2011 was 1.57 child per 10 000 live-born boys. Over the six-year period from 2003 to 2008 median annual clotting factor VIII consumption (IUs/year) among patients with severe hemophilia cases increased by 140%. Mean HJHS score in patients aged 4–9 years was 11.6, whereas in patients aged 10–17 years significantly higher – 31.5 (p = 0.0002). Significance of the difference in joint status comparing different treatment strategies was seen already in younger (4–9 years) patients: 2.2 (prophylaxis) vs. 12.5 (on-demand) (p = 0.0002). The HJHS appears to be a useful tool in evaluating musculoskeletal outcome of patients receiving treatment on-demand. Higher HJHS scores were assessed as a sign of progressing joint damage which seems to occur from the age of 10 and onwards. Joint evaluation using a sensitive tool like the HJHS, may show that a primary prophylactic treatment strategy leads to a better outcome already at an early age.
Type Doctoral thesis
Language Lithuanian
Publication date 2013