نتایج جستجو برای: haemophilia

تعداد نتایج: 2327  

2013
M C Ozelo P R Villaça R Pérez-Bianco M Candela J Garcia-Chavez B Moreno-Rodriguez M B Rodrigues I Rodriguez-Grecco M H Solano G Chumpitaz M M Morales-Gana A Ruiz-Sáez

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...

Journal: :Thrombosis and haemostasis 2016
Massimo Franchini Cristina Santoro Antonio Coppola

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...

Journal: :Journal of clinical pathology 1950
C Merskey

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, ...

Journal: :British journal of haematology 2017
Nadine G Andersson Günter Auerswald Chris Barnes Manuel Carcao Amy L Dunn Karin Fijnvandraat Marianne Hoffmann Kaan Kavakli Gili Kenet Rainer Kobelt Karin Kurnik Ri Liesner Anne Mäkipernaa Marilyn J Manco-Johnson Maria E Mancuso Angelo C Molinari Beatrice Nolan Rosario Perez Garrido Pia Petrini Helen E Platokouki Amy D Shapiro Runhui Wu Rolf Ljung

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...

2017
R. Gheisari B. Bomke T. Hoffmann R. E. Scharf

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...

Journal: :Thrombosis and haemostasis 2001
L O Mosnier T Lisman H M van den Berg H K Nieuwenhuis J C Meijers B N Bouma

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...

2010
Silvia Riva Monika Bullinger Edda Amann Sylvia von Mackensen

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...

2014
D B DiBenedetti T M Coles T Sharma L Pericleous R Kulkarni

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...

Journal: :Thrombosis and haemostasis 2015
J Elisa Bach Beat Wolf Johannes Oldenburg Clemens R Müller Simone Rost

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...

2011
Mehmet AKIN Deniz Yilmaz KARAPINAR Can BALKAN Yilmaz AY Kaan KAVAKLI

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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