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Outcome in moderate haemophilia.

den Uijl, I, Biesma, D, Grobbee, D and Fischer, K (2014) Outcome in moderate haemophilia. Blood Transfus, 12 Sup. s330-s336.

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Abstract

BACKGROUND: Moderate haemophilia is the rarest form of haemophilia. This study aims to assess short- and long-term outcome, including its association with treatment, in patients with moderate haemophilia. MATERIAL AND METHODS: Seventy-five patients with moderate haemophilia (1-5% factor VIII/ factor IX activity), without a history of inhibitors, treated at the van Creveldkliniek, Utrecht (NL) were included in the study. Life-long data on bleeding and treatment were collected. Joints were evaluated using the Haemophilia Joint Health Score. Adults completed questionnaires on activity (HAL) and quality of life (SF-36, EQ5D). RESULTS: The median age of the patients was 37 years (IQR 23-52 years) and haemophilia A was diagnosed in 89%. Bleeding frequency was low: the median annual bleeding rate was 2.0 bleeds/ year (IQR 0.8-3.7 bleeds/year), including a median of 0 joint bleeds/year (IQR 0.8-3.7 bleeds/year). Joint function was good: 82% scored<10 out of 126 points of the Haemophilia Joint Health Score (HJHS). Nevertheless, 29% of patients with moderate haemophilia had a history of prophylaxis, because of a high bleeding frequency. Median age at first joint bleed was 4.8 years (IQR 3.5-8.5). Use of prophylaxis was more associated with age at first joint bleed (P<0.01) than with baseline factor activity (P=0.12). Most patients (52%) who suffered their first joint bleed before the age of 5 years required prophylaxis later in life. DISCUSSION: The majority of patients with moderate haemophilia have few bleeds and complications; however, a considerable subset of patients with a more severe bleeding pattern need prophylactic treatment. These latter patients may be identified by the onset of joint bleeding before the age of 5 years.

Item Type: Article
Authors :
NameEmailORCID
den Uijl, Ii.denuijl@surrey.ac.ukUNSPECIFIED
Biesma, DUNSPECIFIEDUNSPECIFIED
Grobbee, DUNSPECIFIEDUNSPECIFIED
Fischer, KUNSPECIFIEDUNSPECIFIED
Date : January 2014
Identification Number : https://doi.org/10.2450/2012.0091-12
Uncontrolled Keywords : Adult, Age of Onset, Factor IX, Factor VIII, Hemarthrosis, Hemophilia A, Hemorrhage, Humans, Interpersonal Relations, Male, Middle Aged, Motor Activity, Quality of Life, Severity of Illness Index, Surveys and Questionnaires, Treatment Outcome, Young Adult
Related URLs :
Depositing User : Symplectic Elements
Date Deposited : 17 May 2017 10:28
Last Modified : 17 May 2017 14:49
URI: http://epubs.surrey.ac.uk/id/eprint/827926

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