title: Clinical and functional evaluation of the joint status of hemophiliac adults at a Brazilian blood center.
abstract: BACKGROUND
  Hemophilia is a potentially disabling condition as hemophilic arthropathy develops early in life and is progressive, especially in patients treated in an on-demand regime.
  
  
  OBJECTIVE
  This study aimed to describe the structural joint status and the functional independence score of hemophiliac adults and correlate structural damage with the functional deficits found in these patients.
  
  
  METHODS
  Hemophiliacs at the Juiz de Fora Regional Blood Center - HEMOMINAS Foundation, aged 18 years and over and treated in an on-demand regime, were clinically evaluated in respect to structural joint damage using the World Federation of Hemophilia Physical Examination Scale (WFH-PE) and functional deficits using the Functional Independence Score in Hemophilia (FISH). The Spearman rank test was used to evaluate the correlation between the two scores.
  
  
  RESULTS
  Thirty-nine patients were evaluated. The mean age was 36.8 years. Target joints were detected in 69.2% of patients studied. The mean Physical Examination Scale and Functional Independence Score were 16.87 and 25.64, respectively. Patients with mild hemophilia showed no significant joint involvement. Patients with severe or moderate hemophilia had similar results regarding structural damage (p-value < 0.001) and functional deficits (p-value = 0.001). There was statistical significance in the correlation between the two scores (r = -0.850; p-value = 0.01).
  
  
  CONCLUSIONS
  The World Federation of Hemophilia Physical Examination Scale and Functional Independence Score in Hemophilia may be useful to clinically assess structural joint damage and functional deficits in hemophiliacs as the tools are inexpensive and easy to administer and may be able to detect hemophilic arthropathy, which results from recurrent hemarthrosis and is common in the population studied.
authors.affiliation: Universidade Federal de Juiz de Fora - UFJF, Juiz de Fora, MG, Brazil.
authors.forename: Adriana Aparecida
authors.initials: AA
authors.lastname: Ferreira
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Bustamante-Teixeira
authors.forename: Isabel Cristina Gonçalves
authors.initials: IC
authors.lastname: Leite
authors.forename: Camila Soares Lima
authors.initials: CS
authors.lastname: Corrêa
authors.forename: Daniela de Oliveira Werneck
authors.initials: Dde O
authors.lastname: Rodrigues
authors.forename: Danielle Teles
authors.initials: DT
authors.lastname: da Cruz
keywords: Disability evaluation; Hemarthrosis; Hemophilia A; Hemophilia B; Joint diseases