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Prophylactic and therapeutic recombinant factor VIIa administration to patients with Glanzmann's thrombasthenia: results of an international survey
TLDR
rFVIIa seems a potential alternative to platelet transfusion in GT patients, particularly in those with antiplatelet antibodies and/or platelet refractoriness. Expand
The use of recombinant factor VIIa in children with inherited platelet function disorders
Summary. Inherited deficiencies of platelet surface glycoproteins such as Glanzmann's thrombasthenia (GT) or Bernard–Soulier syndrome (BSS) can lead to a severe bleeding diathesis. In the past,Expand
The PFA-100: a potential rapid screening tool for the assessment of platelet dysfunction.
TLDR
The PFA-100 proved to be sensitive in detecting classical defects by giving prolonged closure times in samples from patients with major platelet function defects (e.g. von Willebrand's disease, Glanzmann's thrombasthenia and Bernard Soulier syndrome). Expand
Health status and health‐related quality of life of children with haemophilia from six West European countries
TLDR
HRQoL was satisfactory in this cohort: young children were impaired mainly in the dimension ‘family’ and ‘treatment’, whereas older children had higher impairments in the so‐called ‘social’ dimensions, such as ‘perceived support’and ‘friends’. Expand
C1‐inhibitor concentrate home therapy for hereditary angioedema: a viable, effective treatment option
TLDR
This paper reviews the extent of use of home therapy programmes for hereditary angioedema and summarizes the advantages and potential disadvantages of the concept so far. Expand
The use of intermediate purity factor VIII concentrate BPL 8Y as prophylaxis and treatment in congenital thrombotic thrombocytopenic purpura
TLDR
Effective prophylaxis and treatment is possible in congenital TTP using BPL 8Y, because of viral safety issues and easier administration. Expand
Central venous access devices in children with congenital coagulation disorders: complications and long‐term outcome
TLDR
This study demonstrates a high CVAD infection rate and highlights the long‐term complications of CVAD usage, with infection was the commonest problem encountered, with 52 devices becoming infected. Expand
Can early subclinical gait changes in children with haemophilia be identified using the GAITRite® walkway
TLDR
The aim of this case note review was to determine whether the GAITRite® system (electronic pressure sensitive walkway) could identify early changes in gait patterns in boys with haemophilia compared with their peers. Expand
Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia
TLDR
Evidence is provided supporting early prophylaxis use and the importance of immune tolerance therapy in boys with severe haemophilia and the HJHS is a useful tool for identifying and tracking changes in joint health with respect to therapy or disease progression. Expand
Assessment of Treatment Practice Patterns for Severe Hemophilia A: A Global Nurse Perspective
TLDR
The survey found that 28% of US patients and 38% of UK patients with severe hemophilia A were on primary prophylaxis in 2005, significantly higher than those reported in a 2003 survey. Expand
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