Haemophilia : the official journal of the World Federation of Hemophilia
-
The aim of this study was to investigate the genotoxic effect on the peripheral blood lymphocytes potentially induced by Re-186 in paediatric age group undergoing radiosynovectomy for haemophilic synovitis, by using chromosomal aberration analysis (CA) and the micronuclei (MN) assay for detecting chromosomal aberrations, as well as the sister chromatid exchanges (SCE) technique for assessing DNA damage. ⋯ The major finding of this study with relatively small sample is that, radiosynovectomy with Re-186 does not seem to induce early genotoxic effects on the peripheral blood lymphocytes in paediatric age group.
-
Acupuncture is successfully used in the treatment of degenerative osteoarthritis. The treatment of haemophilic arthropathies can require strong painkillers with severe side-effects. Therefore, a special yet simple acupuncture technique was evaluated in the treatment of these joint problems. ⋯ The side not receiving treatment showed a reduction from 4.1 to 4.0. No side-effects were observed. Even though interpretation of our data are limited due to the small patient numbers, significant improvement of the VAS after treatment suggests that acupuncture has a measurable positive effect in pain management for haemophilic arthropathy of the lower extremities.
-
Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders, whose severity is inversely proportional to the degree of factor deficiency. Haemophilia A and B, inherited as X-linked recessive traits, are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of blood coagulation factor VIII (FVIII) and factor IX (FIX). Together with von Willebrand's disease, a defect of primary haemostasis, these X-linked disorders include 95% to 97% of all the inherited deficiencies of coagulation factors. ⋯ In countries with more advanced molecular facilities and higher budget resources, the most appropriate choice in general is a direct strategy for mutation detection. However, in countries with limited facilities and low budget resources, carrier detection and prenatal diagnosis are usually performed by linkage analysis with genetic markers. This article reviews the genetic diagnosis of haemophilia, genetics and inhibitor development, genetics of von Willebrand's disease and of rare bleeding disorders.
-
Multicenter Study
A retrospective postlicensure survey of FEIBA efficacy and safety.
Patients with haemophilia who develop inhibitors have unique treatment needs; bypassing agents such as Factor Eight Inhibitor Bypassing Activity, Anti-Inhibitor Coagulant Complex (FEIBA; Baxter AG, Vienna, Austria) are part of this therapeutic armamentarium. This study sought to increase comprehension of the full therapeutic profile of FEIBA by evaluating its safety and efficacy in the settings of acute bleeding, surgery, and prophylaxis. Information was collected through a postmarketing surveillance study; questionnaire booklets were distributed to 72 treatment centers in the United States and Europe. ⋯ No thrombotic complications occurred during any treatment episode. Results indicated that FEIBA was safe and effective in acute, surgical, and prophylactic treatment settings, supporting the utility of FEIBA as a treatment option for patients with inhibitors. However, prospective studies are advised.
-
Review Practice Guideline
The obstetric and gynaecological management of women with inherited bleeding disorders--review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization.
The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.