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Open access

Omar El Graoui, Saadia Faez and Bouchra Oukkache

Abstract

Acquired hemophilia A (AHA), is a rare bleeding disorder with an incidence of approximately 1 per million/year.The disease is caused by the development of autoantibodies directed against clotting factor VIII and associated with an increased morbidity and mortality. In 50% of cases, FVIII autoantibodies occur in patients lacking any relevant concomitant disease; the remaining cases may be associated with postpartum period, autoimmune diseases, underlying hematologic or solid cancers, and infections.

We report the case of a 32-years-old woman, admitted in hemorrhagic shock, following a non-reversible bleeding after dental extraction. The Laboratory tests prolonged activated prothrombin thromboplastin time (APTT at 54s), and normal Prothombine time (PT at 75%). The mixing test revealed the presence of a circulating anticoagulant with a Rosner index at 15. Factors specific to the intrinsic pathway determined that the patient had a factor VIII deficiency. Acquired hemophilia was diagnosed.

At the local level, CT scan of the face objectified a right maxillary osteitis without sinus damage.The patient received transfusion of Red Blood Cells units and FFP (fresh frozen plasma) concentrates.but kept bleeding until an antibiotic treatment and corticosteroids (60mg/d) were added.

Open access

Aneta Neskoromna-Jędrzejczak, Katarzyna Bogusiak, Krzysztof Chojnowski, Marta Robak and Jacek Treliński

Abstract

The preparation of patients with hemophilia before surgical operations and dental procedures constitutes a significant clinical challenge. This article presents the implantoprosthetic rehabilitation of a patient with severe hemophilia B (factor IX activity <1%). The patient was prepared for the surgical procedure with recombinant factor IX concentrate (Rixubis) during the clinical surgery study. Tooth extraction and the implantation of four dental implants in the mandible were planned: one dental implant of 3.7 mm diameter and 10 mm length in the place of tooth 35, and another of 3.2 mm diameter and 10 mm length in the place of tooth 37. The next two implants were implemented 1 month later: one implant 3.7 mm in diameter and 10 mm in length in the place of tooth 46, and another implant 3.2 mm in diameter and 10 mm in length in the area of tooth 44. Appropriate substitution of the missing coagulation factor, together with the use of local hemostatic therapy, allowed dental implantation to be performed without excessive blood loss in this patient with severe hemophilia B.

Open access

Liesbeth Schrijvers, Martin Bedford, Petra Elfvinge, Karin Andritschke, Bert Leenders and Christine Harrington

Abstract

Background: A broad scoping exercise was undertaken to assess and quantify haemophilia nursing care in Europe. Methods: A web-based survey in English was sent to known networks of haemophilia nurses working in Europe. This survey included questions concerning the haemophilia treatment centre, educational level, work activities, gaining knowledge/ expertise and development in the future. Results: In total, 94 nurses in 14 countries in Europe completed the survey. Overall, the majority (62%) of the nurses had over 20 years’ nursing experience, with 44% having more than 10 years’ experience in haemophilia. The educational level varied; with highest educational level of attainment being 41% at nondegree level nursing entry qualification, 35% BSc and 24% MSc. 21% worked in a centre where they treat only children, 26% only adults and 53% both. All had good access to treatment. The core activities (rated >80%) of a haemophilia nurse were: prepare and administer medication, venepuncture and CVADuse (except cannulation), providing education and telephone advice, coordination of (multidisciplinary) care and assistance with clinical trials. Furthermore, 35% stated that they initiated and performed nurse-led research. In the future, almost all nurses would like to develop their expertise and knowledge base by studying at Masters level and above, to have more responsibility, and to conduct research. Conclusion: This scoping exercise provides a baseline assessment of haemophilia nursing across Europe. The results may provide a basis for a more thorough investigation of the current role and the principles of haemophilia nursing care; future opportunities, and the training requirements to develop the specialty.

Open access

Sarah Gilgunn

Abstract

Clotting factor replacement therapy has proven a highly effective means of treating haemophilia A and B. But treatment involves frequent and lifelong infusion of factor concentrates and is generally prophylactic rather than curative. It is also extremely expensive, associated with inhibitor formation and does not fully abolish the potential for spontaneous bleeding. Gene therapy offers a potential cure for haemophilia, with the possible continuous expression of a clotting factor gene following the administration of a viral vector carrying the appropriate gene. Recent clinical trials of gene therapy for haemophilia have proven positive in selected patients and new studies are underway.

Open access

David Hopper

Abstract

A 73-year-old woman presented with recent onset significant spontaneous bruising. She also had a recent diagnosis of type 2 diabetes mellitus and was undergoing investigations for biochemical hyperthyroidism. A diagnosis of acquired haemophilia A was confirmed with laboratory testing in addition to thyrotoxic Graves’ disease. She had significant bruising of her left thigh and restricted mobility of the left leg. A significant left vastus lateralis haematoma was diagnosed by ultrasound scan. A course of FEIBA (Factor Eight Inhibitor Bypassing Activity) was commenced and physiotherapy input provided. Kinesio tape was applied to the thigh using a lymphatic drainage technique with the aim of reducing swelling and bruising. Eight days after the initial application of tape, bruising had significantly reduced and her range of movement had returned to normal. This case history demonstrates how kinesio tape may be used as a useful adjunct to standard therapy.

Open access

David Owen

Abstract

David Owen was Labour Health Minister 1974-76 and now sits as an independent social democrat in the House of Lords. Before entering Parliament he trained as a medical doctor at St Thomas’s Hospital, London, where he was Clinical Neurologist and Psychiatric Registrar. He has championed the NHS throughout its existence and is now a powerful advocate for its reinstatement to its original purpose. In this extract from his 2014 book The Health of the Nation, NHS in Peril, David Owen sets out the consequences of the 2012 Health and Social Care Act for the haemophilia community.

Open access

Margit Șerban, Dan Poenaru, Laura Cernat, Delia Savescu, Jenel Pătrașcu, Wolfgang Schramm, Emilia Ursu, Delia Mihailov, Cristian Jinca, Ioana Ioniță and Smaranda Arghirescu

Abstract

The development of FVIII/FIX inhibitor alloantibodies represents a severe complication requiring specific laboratory evaluation for establishing a life-saving therapy regimen. Our preliminary study aimed at elaborating a laboratory strategy for monitoring the effectiveness of Activated Prothrombin Complex Concentrate (APCC) and recombinant activated factor VII (rFVIIa) in haemophiliacs with inhibitors, by checking the reliability and clinical value of three complementary assays: clotting-time based coagulometry, thrombelastography (TEG) and thrombin generation assay (TGA). The investigations were performed on 7 patients with severe haemophilia A with high titer inhibitors treated for 12 episodes of severe bleedings, 5 of them for surgical interventions. After the administration of bypassing agents (BPAs) the clotting-time based assay brought changes only on prothrombin (p<0.01), potentially signaling a thrombotic risk, without any impact on the global hemostasis. TEG displayed prompt significant improvement only after rFVIIa (90μg/kg). TGA revealed significantly improved values for peak and velocity index, time to peak and start tail after both BPAs. Despite some disparities between biological hemostatic phenotype and clinical response to therapy, we concluded that TEG and TGA are the only current exploratory assays, expressing the quality of haemostatic control, representing a real support for a personalized, adapted therapy in hemophilia with inhibitors.

Open access

Peter Collins

Abstract

Advances in the understanding of haemophilia require effective collaboration, both at the national and international levels. Such collaborations are currently playing a major role in elucidating the natural history of acquired haemophilia, and also in clarifying the issue of product-related inhibitor development in previously untreated patients with haemophilia.

Open access

Susan Hook

Abstract

A case study is presented illustrating the everyday challenges faced by patients with haemophilia and inhibitors and how treatment with bypassing agents or just FEIBA prophylaxis can help to control bleeding and improve functional ability.

Open access

Jim Munn

Abstract

The World Federation of Hemophilia’s world congress in Melbourne offered haemophilia nurses from across the world an opportunity to meet and share knowledge and experience in a friendly and supportive setting.