Haemophilia : the official journal of the World Federation of Hemophilia
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Immune tolerance induction (ITI) is effective in approximately 70% of haemophilia patients with inhibitors. Poor prognostic factors are age >6 years, ITI started >1 year from inhibitor development, inhibitor peaks >200 BU, inhibitor titre >10 BU when ITI is started and previously failed ITI. The objective of this study was to identify the effectiveness in ITI of a high purity von Willebrand factor/factor VIII (VWF/FVIII) complex concentrate in inhibitor patients at high risk of failure. ⋯ Complete success was obtained in nine patients (53%) after 4-30 months of treatment (median 24), including two of four patients who had previously failed ITI. Seven patients achieved a partial success, with sustained low inhibitor titres (median 1.5 BU, range 1.1-2.8) but abnormal recovery and/or half-life, while the remaining patient withdrew ITI after 12 months when the inhibitor titer was still 70 BU. These findings suggest that high purity VWF/FVIII complex concentrates are effective in ITI, even in patients at high risk of failure.
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Peripheral nerve blocks can be safely used on patients with bleeding disorder, to achieve peri-operative amalgesia.
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Parents of children affected by haemophilia must face, often without prior knowledge, the difficult challenge imposed by such a pathology. To satisfy the need of information, guideline and psychological support for a better quality of life, 30 parents with haemophiliac children have participated in a programme of counselling and psychological support. Such a programme has the aim of guiding the group trough a process of discovery, comparison and personal growth and stimulating adaptive processes of problem-solving and decision-making. ⋯ The use of avoidance -focused coping strategies seems to remain at the same level even if it was low. Also the other psychological aspects investigated, namely depression and anxiety, did receive a positive influence. The results show how significant such programme has been for parents.
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Case Reports
Combined clotting factor deficiencies: experience at a single hemophilia treatment center.
We describe a series of patients with combined factor deficiencies and von Willebrand's disease (VWD) at one haemophilia treatment centre. Although the incidence of VWD is at least 1% in the general population, combined coagulation defects have been infrequently described in the medical literature and are likely under diagnosed. This entity should be considered in patients with a known factor deficiency and either an unexpectedly severe bleeding phenotype, or bleeding that is unresponsive to factor replacement.
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Operating on patients with abnormal coagulation is a challenge frequently faced by surgeons. Achieving haemostasis perioperatively can involve bleeding points that would not ordinarily present a problem with intact clotting function. ⋯ These two cases illustrate a novel use for this antifibrinolytic agent. We suggest that there is a role for topical use of tranexamic acid in perioperative haemostasis in patients with clotting disorders.