107 Chapter 5 in patients with a high bleeding tendency detected on the BAT. Many OI patients underwent multiple invasive procedures because of fractures and dentinogenesis imperfecta, possibly with a undiscovered history of bleeding. This means that patients are at risk of experiencing unnecessary and potentially debilitating haemorrhagic symptoms if a bleeding tendency is not identified. The use of a structured bleeding questionnaire as is used in this study seems to be far more useful than laboratory measurements. We recommend clinicians who treat OI patients to assess bleeding tendency with the use of a Bleeding Assessment Tool. We also recommend to consider potential interventions to reduce haemorrhagic symptoms with the use of desmopressin and/or tranexamic acid which might be sufficient to reduce haemorrhagic symptoms and (in menorrhagia) improve quality of life. These interventions had as described in literature no evidence of adverse effects, while patients might be prevented from being exposed to potential risks associated with the administration of blood products. Future studies will be required to further define the bleeding phenotype in OI and to investigate a possible correlation with genotype. Also OI specific studies into results of preventive medication on bleeding tendency and validation of reliability and feasibility of the Self-BAT in OI are important issues for future research.
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