Introduction To date, none of the available assessment instruments to evaluate functional abilities for individuals with haemophilia has been translated and validated in Brazil. Aim To translate, and test the construct validity, internal consistency and the reliability of the Haemophilia Activities List (HAL) for the Brazilian population with severe and moderate haemophilia (type A or B) and to investigate differences in the self‐perception of functional abilities in patients adults with haemophilia classified according to the presence of joint bleeding and the performance of orthopaedic surgeries. Methods The translation and transcultural adaptation following the steps: (a) translation; (b) synthesis (consensual version); (c) back‐translation; (d) review by the committee of experts; (e) pretest in the target population; and (f) final version of the instrument. Correlations between HAL scores and the scores of the Health Assessment Questionnaire (HAQ) and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) were determined for construct validation. Reliability was tested using the test and retest method, and internal consistency was calculated by Cronbach's α. Results The HAL‐BR was applied to 52 individuals with haemophilia (34.51 ± 12.87 years). The process of translation and cross‐cultural adaptation produced similar versions between the translations A moderate correlation was observed between HAL and the HAQ (r = −0.55) and WOMAC scores (r = −0.58). The reliability was ICC = 0.972, CI (0.917‐0.997) ICC = 0.876, CI (0.631‐0.978) for inter‐ and intra‐examiners. No difference was found in the total HAL score between the groups with and without bleeding. The group subjected to surgery compared to the group of not subjected to surgery presented in the HAL domains worse function in ‘lying down/sitting/kneeling/standing’, ‘self‐care’ and ‘complex lower extremities activities’ as well as in total HAL score. Conclusion The Brazilian version of the HAL was proven to be a valid and reliable evaluation tool for adults with haemophilia in Brazil.
RESUMOA hemofilia é uma doença de herança genética ligada ao cromossomo X. A principal alteração dessa patologia afeta os mecanismos de coagulação. As articulações mais acometidas são os joelhos, cotovelos e tornozelos. As manifestações clínicas incluem dor, sangramento articular e limitação do movimento. O diagnóstico leva em consideração o quadro clínico e exames de imagem. Neste artigo, apresentamos o caso de um paciente com restrição das atividades diárias e perda da qualidade de vida devido à artropatia hemofílica no tornozelo direito. Após a realização da artroplastia total da articulação afetada, o paciente evoluiu com melhora dos parâmetros pré-operatórios. Nível V de Evidência; Opinião de Especialista.Descritores: Tornozelo; Hemofilia; Hemartrose/cirurgia; Artroplastia de substituição do tornozelo. AbStRActHemophilia is a genetically inherited disease linked to the X chromosome. The main alteration of this pathology affects the coagulation mechanisms. The most affected joints are the knees, elbows and ankles. Clinical manifestations include pain, joint bleeding and limited movement. The diagnosis takes into account the clinical picture and imaging tests. In this article, we present the case of a patient with restricted performance of activities of daily living and loss of quality of life due to hemophilic arthropathy in the right ankle. After total joint arthroplasty of the affected joint, there was improvement in the preoperative parameters of the patient. Level V of Evidence; Expert Opinion.Como citar esse artigo: Silva JLV, Pacheco LRL, Nakahashi ER, Picini M. Artroplastia total do tornozelo (ATT) em paciente hemofílico: relato de caso. Sci J Foot Ankle. 2018;12(1):72-6.
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