How mild is mild haemophilia?
Haemophilia
; 29(2): 530-537, 2023 Mar.
Article
em En
| MEDLINE
| ID: mdl-36696281
INTRODUCTION: People with mild haemophilia (PWMH) experience sporadic bleeds and are less likely to receive an early diagnosis, appropriate treatment and medical care. Arthropathy is a key determinant of health-related quality of life (QoL), producing pain, limitations in mobility and daily activities. The aim of this study is to evaluate the incidence, risk factors and QoL associated with arthropathy in PWMH. MATERIALS AND METHODS: Observational, cross-sectional cohort study. Data were collected in a single interview and evaluated by a physiotherapist and an orthopaedist and analysed on demographics; baseline factor levels; as well as clinical (Haemophilia Joint Health Score [HJHS]), ultrasound (Haemophilia Early Arthropathy Detection with Ultrasound [HEAD-US]), radiological (Pettersson score [PS]), pain (visual analogue scale [VAS]) and QoL evaluations. We defined arthropathy when at least one of the joints shown with a HEAD-US score ≥ 1. RESULTS: Eighty-five patients and 510 joints were included. Patients' mean age was 35.9 years-old. Median age was 44.2 in patients with arthropathy versus 14.9 in patients without; the difference was statistically significant (p < .001). In patients over 20 years old, 90.5% shown arthropathy. Only 24 (28%) patients had no joint damage (HEAD-US = 0), and 61 (72%) had at least one joint with a HEAD-US ≥ 1. The ankle was the most affected joint. Patient age was found to be the most important risk factor associated with the development of arthropathy. CONCLUSIONS: Joint damage as a result of prior hemarthrosis was the most relevant factor associated with lower QoL, and emphasised the importance of early diagnosis and appropriate management in this particular population.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Artrite
/
Hemofilia A
Tipo de estudo:
Observational_studies
/
Prevalence_studies
/
Risk_factors_studies
/
Screening_studies
Aspecto:
Patient_preference
Limite:
Adult
/
Humans
Idioma:
En
Revista:
Haemophilia
Assunto da revista:
HEMATOLOGIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Argentina
País de publicação:
Reino Unido