نتایج جستجو برای: severity of hemophilia

تعداد نتایج: 21171297  

2014
Hassan Mansouritorghabeh

Bone marrow transplantation (BMT) is nowadays used in various hematological disorders including leukemias. Hemophilia A is sex linked bleeding disorder in which there are various genetic abnormalities in factor VIII gene. Among various hematological disorders, bleeding disorders mainly hemophilia in now widely treated using plasma derived and recombinant factor VIII concentrates. Day to day tra...

Journal: :iranian journal of medical sciences 0
abdolhossein davoodabadi departments of general and thoracic surgery, kashan university of medical sciences, kashan, iran mohammad mahdi adib hematology and medical oncology, kashan university of medical sciences, kashan, iran behrooz keleidari departments of general and thoracic surgery, kashan university of medical sciences, kashan, iran

hemophilia a is a bleeding disorder caused by defective production of factor viii. the main concern associated with the disease is bleeding, especially after trauma and surgeries. factor viii replacement therapy is associated with substantial decrease of bleeding events during surgery. however, there have been a number of reports of thromboemblic events in this situ-ation. the present report de...

Journal: :Blood 2016
Marshall A Mazepa Paul E Monahan Judith R Baker Brenda K Riske J Michael Soucie

The availability of longitudinal data collected prospectively from 1998 to 2011 at federally funded US hemophilia treatment centers provided an opportunity to construct a descriptive analysis of how outcomes of men with severe hemophilia have been altered by the incremental advances and setbacks in hemophilia care in the last 50 years in the United States. This surveillance collaboration with t...

Journal: :Haematologica 2008
Maurizio Margaglione Giancarlo Castaman Massimo Morfini Angiola Rocino Elena Santagostino Giuseppe Tagariello Anna Rita Tagliaferri Ezio Zanon Maria Patrizia Bicocchi Giuseppe Castaldo Flora Peyvandi Rosa Santacroce Francesca Torricelli Elvira Grandone Pier Mannuccio Mannucci

BACKGROUND The high mutational heterogeneity of hemophilia A is a challenge for the provision of genetic services. We plan to identify the mutation in patients with hemophilia A in order to create a confidential national database of mutations for the optimization of genetic services in Italy. DESIGN AND METHODS The factor VIII gene (F8) was analyzed in 1296 unrelated patients with hemophilia ...

Journal: :Haemophilia : the official journal of the World Federation of Hemophilia 2013
A Srivastava A K Brewer E P Mauser-Bunschoten N S Key S Kitchen A Llinas C A Ludlam J N Mahlangu K Mulder M C Poon A Street

Hemophilia is a rare disorder that is complex to diagnose and to manage. These evidence-based guidelines offer practical recommendations on the diagnosis and general management of hemophilia, as well as the management of complications including musculoskeletal issues, inhibitors, and transfusion-transmitted infections. By compiling these guidelines, the World Federation of Hemophilia aims to as...

Journal: :Haematologica 2011
Esther R van Bladel Mark Roest Philip G de Groot Roger E G Schutgens

BACKGROUND Platelets are an underappreciated factor in the classification of the bleeding tendency of patients with hemophilia. In this cross-sectional study, we investigated platelet activation status and responsiveness in relation to residual factor VIII activity and, within the group with severe hemophilia (<1% residual factor VIII activity), to annual factor VIII consumption. DESIGN AND M...

Journal: :Haemophilia : the official journal of the World Federation of Hemophilia 2007
D M DiMichele W K Hoots S W Pipe G E Rivard E Santagostino

Although immune tolerance induction (ITI) has been used for 30 years to eliminate inhibitors and restore normal factor pharmacokinetics in patients with hemophilia, there is a paucity of scientific evidence to guide therapeutic decision-making. In an effort to provide direction for physicians and hemophilia treatment center staff members, an international panel of hemophilia opinion leaders met...

Journal: :journal of dental research, dental clinics, dental prospects 0
mohammad abrisham mehdi tabrizizadeh alireza ghateh

background and aims. hemophilic patients are faced with poor oral hygiene due to concerns about their dental care. the present study assessed the knowledge of hemophilic patients about oral hygiene and the effect of oral hygiene instruction in patients referred to iranian hemophilia society. materials and methods. this cross-sectional study was carried out on 30 hemophilic patients randomly sel...

Abdolah Gorgi F, Abed-Saeedi J, Alavi S, Eshghi P, Farahani H, Habib- Panah B, Khanali L, Taslimi S, Tehrani Tarighat S,

Background: Treating a chronic disease such as hemophilia is to improve the symptoms and quality of life (QOL) of the patients. This study aimed to study the quality of life among hemophilic children and compare the quality of life between patients receiving prophylactic or on demand treatments. Materials and Methods: In this descriptive-comparative study, we enrolled 60 patients from three ...

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