Haemophilia : the official journal of the World Federation of Hemophilia
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With approval of gene therapy for haemophilia likely in the near future, policy frameworks are needed to guide the path forward for this disruptive and novel therapeutic advance. ⋯ This issue of ideal and acceptable factor level expression and other unresolved issues will be brought to the 3rd WFH GTRT in 2020.
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Better access to haemophilia treatment and care is currently changing the life of a great many patients with haemophilia or other inherited bleeding diseases in numerous developing countries. These changes are encouraging while stimulating local research initiatives and projects that deserve to be communicated through scientific publications. This paper addresses several opportunities and challenges, providing guidance to scientists, clinicians, researchers and health professionals from developing countries who are actively involved in multidisciplinary haemophilia care, while wishing to publish their reports in Haemophilia, the official, global, multidisciplinary journal of the World Federation of Haemophilia (WFH), the European Haemophilia and Allied Disorders (EAHAD) organization and the Hemostasis and Thrombosis Research Society (HTRS) of North America, focusing on inherited bleeding diseases. Several strategies and pathways designed to encourage, help and support successful publications from developing countries are herein discussed.
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Due to the global prevalence of oral disease, tooth extraction is the most common surgical procedure required in general population thus likely to be similarly common in patients with haemophilia, especially those in older age and those living in countries with restricted resources. There are little or no consensus about optimal level and duration of factor replacement (FRP) therapy required to prevent bleeding complication following surgery and low levels of evidence to inform protocols and guidelines. The goal of this article was to review the literature regarding haematological treatment protocols and to assess their effectiveness in prevention of bleeding complications during and after tooth extractions in people with haemophilia. ⋯ Based on the haematological regimens, all studies were classified into one of three groups: pre- and postoperative FRP or DDAVP, single preoperative FRP or DDAVP, and no FRP treatment. The overall reported bleeding rate in case of both pre- and postoperative FRP and single dose FRP preoperative is similar, 11.9% and 11.4%, respectively, indicating that minimizing the use of clotting factor concentrate is possible if proper local haemostatic measures are provided. Strictly designed prospective studies with higher number of patients are necessary to get firm conclusions about optimal FRP treatment required to prevent bleeding complications during and after oral surgery in patients with haemophilia.
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Postpartum haemorrhage (PPH) is the main cause of maternal morbidity and mortality globally, but it is far more important in non-developed countries. PPH represents 25% of all maternal deaths worldwide. Women with von Willebrand disease (VWD) and other inherited haemorrhagic disorders are at increased risk of PPH. Our aim was to establish a probable association of severe PPH in women with a history of haemostatic abnormalities. ⋯ Our results show a relationship between PPH and inherited haemostatic disorders. VWD was the most frequent diagnosis. Appropriate and opportune diagnosis before pregnancy of inherited haemostatic disorders may be important to effectively prevent and treat PPH.
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Acquired haemophilia (AH) is a rare bleeding disorder with significant morbidity and mortality. Most patients initially present to physicians without experience of the disease, delaying diagnosis and potentially worsening outcomes. Existing guidance in AH is limited to clinical opinion of few experts and does not address monitoring bleeds in specific anatomical locations. ⋯ Here, we present consensus statements derived from a broad sample of global specialists to address monitoring of location-specific bleeds and evaluating efficacy of bleeding treatment in patients with AH. These statements could be applied in practice by treating physicians and validated by individual population surveys.