نتایج جستجو برای: haemophilia
تعداد نتایج: 2327 فیلتر نتایج به سال:
There is a paucity of literature on haemophilia treatment in Latin American countries, a region characterized by rapidly improving systems of care, but with substantial disparities in treatment between countries. The aim of this study was to evaluate the musculoskeletal status of haemophilia patients from Latin America and to examine the relationship between musculoskeletal status and treatment...
http://dx.doi.org/10.1160/TH16-02-0116 Thromb Haemost 2016; 116: 201–203 Dear Sirs, Considering the high quality of replacement therapy available nowadays for haemophiliacs, the most challenging complication of haemophilia is currently the development of inhibitors, which render replacement therapy ineffective, preclude the access of patients to a safe and effective standard of care (particular...
Haemophilia is the only recognized haemorrhagic diathesis which is inherited as a sex linked recessive character. It is a disease of males which is transmitted through apparently unaffected females. The classical case can be recognized with certainty since the laboratory manifestations are specific. The association of a prolonged coagulation time with a normal bleeding time and platelet count, ...
The discussion of prophylactic therapy in haemophilia is largely focused on joint outcomes. The impact of prophylactic therapy on intracranial haemorrhage (ICH) is less known. This study aimed to analyse ICH in children with haemophilia, with a focus on different prophylaxis regimens and sequelae of ICH. We conducted a multicentre retrospective and prospective study that included 33 haemophilia...
We have performed a monocenter study on 29 consecutive patients with acquired haemophilia A who were referred for diagnosis and treatment to the Düsseldorf Haemophilia Comprehensive Care Center between March 2001 and February 2010. Patients, methods: 18 men (age: 44–86 years) and 11 women (age: 20–83 years). For laboratory evaluation, a standardized staged protocol of aPTT, FVIII : C activity a...
TAFI (thrombin activatable fibrinolysis inhibitor) down regulates fibrinolysis after activation by relatively high concentrations of thrombin generated during coagulation via thrombin mediated factor XI activation and subsequent activation of the intrinsic pathway. It is this secondary burst of thrombin that is severely diminished in haemophilia A, a deficiency of coagulation factor VIII. We th...
BACKGROUND Patient-Reported Outcomes (PROs) are considered important outcomes because they reflect the patient's experience in clinical trials. PROs have been included in the field of haemophilia only recently. PURPOSE Comparing the contents of PROs measures used in haemophilia, based on the ICF/ICF-CY as frame of reference. METHODS Haemophilia-specific PROs for adults and children were sel...
Haemophilia A is a rare inherited bleeding disorder characterized by an inability of the blood to clot normally. Patients can experience spontaneous or trauma-induced joint and soft tissue bleeding and must keep coagulation factor VIII (FVIII) accessible at all times; thus, FVIII product storage and stability are critical. Our primary objective was to assess haemophilia A patients' and caregive...
Current screening methods for factor VIII gene (F8) mutations can reveal the causative alteration in the vast majority of haemophilia A patients. Yet, standard diagnostic methods fail in about 2% of cases. This study aimed at analysing the entire intronic sequences of the F8 gene in 15 haemophilia A patients by next generation sequencing. All patients had a mild to moderate phenotype and no mut...
Von Willebrand disease and haemophilia A are the two most common inherited bleeding disorders. The worldwide incidence of VWD is estimated between 1% and 4% of the population without apparent racial or ethnic predilection. In the United States, the incidence of haemophilia A is estimated to be 25 per 100 000 male births. Despite the relatively high frequency of those two bleeding disorders in t...
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