Magnitude of arthropathy in patients with hemophilia: A single-center experience

oleh: Rawand Polus Shamoon

Format: Article
Diterbitkan: Wolters Kluwer Medknow Publications 2017-01-01

Deskripsi

Background: Hemophilia is a sex-linked bleeding disorder. Affected patients suffer spontaneous or post-traumatic bleeding into various sites of the body, mainly into joints, depending on the level of coagulation factor deficiency. Aims: This descriptive study is designed to assess the prevalence and extent of arthropathy and evaluate the functional status of hemophiliacs in Erbil, Northern Iraq. Settings And Design: A descriptive study of all registered hemophiliacs at Nanakali Hemato-Oncology Teaching Centre, Erbil, Iraq. Patients And Methods: Over 15 months (October 2015 to February 2017), a total of 133 hemophilia patients were studied. Their hospital records were used to retrieve clinical and laboratory data, mainly their coagulation profile. All patients were clinically examined at the daycare center; plain radiography was used to evaluate the degree of joint damage based on Petterson score. The magnitude of joint disease was assessed, and patients' functional status was evaluated depending on Functional Independence Score in Hemophilia (FISH). Statistical Analysis Used: Statistical analysis used MS Excel 2010. Results: Hemophiliacs mean age was 12.9 years. Patients with severe hemophilia presented earlier and had more bleeding episodes. At least one bout of hemarthrosis was recorded in 103/133 patients during the course of their disease with knee joint most frequently involved (in eighty patients) followed by elbow, ankle, wrist, and shoulder. The Petterson score related significantly to age of the patient, number of bleeds, and severity of hemophilia. Majority, 67%, of hemophiliacs found to have the limitation of movement. FISH score significantly related with factor activity level. Functional disability was encountered in 9.7% of cases; majority had severe hemophilia. Conclusions: The incidence and severity of joint bleeding and functional disability were high. The FISH and Pettersson scoring systems are very useful tools in assessing patients with hemophilic arthropathy.