Joint health and treatment modalities in Nordic patients with moderate haemophilia A and B - The MoHem study.
Haemophilia
; 26(5): 891-897, 2020 Sep.
Article
en En
| MEDLINE
| ID: mdl-33021747
ABSTRACT
INTRODUCTION:
The prevalence of arthropathy in moderate haemophilia A (MHA) and B (MHB) is not well known.AIM:
We evaluated joint health in Nordic patients in relation to their treatment modality.METHODS:
A cross-sectional, multicentre study covering MHA and MHB in Sweden, Finland and Norway. Arthropathy was evaluated by ultrasound (HEAD-US) and Haemophilia Joint Health Score (HJHS).RESULTS:
We report on 145 patients median age 28 years (IQR 13-52) and 61% MHA. Baseline factor VIII/factor IX activity (FVIII/FIXC) was 2 IU/dL (median) (IQR 2-4) lower for MHB (2 IU/dL, IQR 1-2) than MHA (3 IU/dL, IQR 2-4) (P < .01). Eighty-five per cent of MHA and 73% MHB had a history of haemarthrosis (P = .07). Age at first joint bleed was lower for MHA (5 years [median], IQR 3-7) than MHB (7 years, IQR 5-12) (P = .01). Thirty-eight per cent received prophylaxis, started at median 10 years of age (IQR 4-24). Median joint bleeds and serious other bleeds during the last 12 months were both zero (IQR 0-1). Total HEAD-US captured 0/48 points (median) (IQR 0-2) and HJHS 4/120 points (IQR 1-10) with strong correlation between them (r = .72). FVIII/FIX C ≤ 3 IU/dL was associated with higher HJHS (P = .04). Fifteen per cent had undergone orthopaedic surgery.CONCLUSION:
The current joint health in Nordic moderate haemophilia patients was rather good, but a subgroup had severe arthropathy. FVIII/FIX C ≤ 3 IU/dL and MHA were associated with a more severe bleeding phenotype. We suggest primary prophylaxis to all patients with FVIII/FIXC ≤ 3 IU/dL.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Hemofilia B
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Hemofilia A
Tipo de estudio:
Clinical_trials
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Observational_studies
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Prevalence_studies
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Risk_factors_studies
Límite:
Adolescent
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Adult
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Female
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Humans
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Male
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Middle aged
País/Región como asunto:
Europa
Idioma:
En
Revista:
Haemophilia
Asunto de la revista:
HEMATOLOGIA
Año:
2020
Tipo del documento:
Article
País de afiliación:
Noruega