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- W2109055955 abstract "Carriers of severe and moderate haemophilia A and B are expected to have approximately 50% of the normal level of factors VIII and IX. However, due to X chromosome inactivation in early embryonic life, factor levels can vary considerably. This can lead to increased bleeding tendency, which may in turn impact on health-related quality of life (HRQOL).The aim of this study was to assess HRQOL in carriers of severe and moderate haemophilia with and without increased bleeding tendency.One hundred and twenty-four adult carriers and 90 controls were recruited. Bleeding tendency was evaluated using a structured bleeding assessment tool. HRQOL was measured by the short form 36 (SF-36) questionnaire. The SF-36 scores were compared with Swedish normative age-matched data and reported as Z scores.There was no significant difference between the whole groups of carriers and controls in the Z scores of the eight SF-36 domains. The mental component summary (MCS) was lower in carriers, compared with controls (P = 0.048). The subgroup of carriers with an increased bleeding tendency had significantly lower Z scores compared to controls regarding the General Health (P = 0.008), the Social Functioning (P = 0.040) and the Mental Health (P = 0.048) domains. The MCS was significantly lower in this carrier subgroup than in controls (P = 0.033).We conclude that the subgroup of carriers of haemophilia with increased bleeding tendency have impaired HRQOL. The SF-36 results indicate that this condition affects mental rather than physical health." @default.
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- W2109055955 date "2015-09-08" @default.
- W2109055955 modified "2023-10-02" @default.
- W2109055955 title "Association between bleeding tendency and health-related quality of life in carriers of moderate and severe haemophilia" @default.
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- W2109055955 doi "https://doi.org/10.1111/hae.12796" @default.
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