Search results for " haemophilia"

Current concepts in the prevention of pathogen transmission via blood/plasma-derived products for bleeding disorders

2015

The pathogen safety of blood/plasma-derived products has historically been a subject of significant concern to the medical community, Measures such as donor selection and blood screening have contributed to increase the safety of these products, but pathogen transmission does still occur. Reasons for this include lack of sensitivity/specificity of current screening methods, lack of reliable screening tests for some pathogens (e.g. prions) and the fact that many potentially harmful infectious agents are not routinely screened for. Methods for the purification/inactivation of blood/plasma-derived products have been developed in order to further reduce the residual risk, but low concentrations…

Hemophilia Clinical Consults: Epidemiology, Clinical Course, and Management of Acquired Hemophilia A

2011

Acquired haemophilia A: a case repor

2009

Addressing the impact of SARS-CoV-2 infection in persons with congenital bleeding disorders: The Italian MECCOVID-19 study

2021

congenital bleeding disorders

The important impact of dental care on haemostatic treatment burden in patients with mild haemophilia

2022

Background: Mild haemophilia (MH) is mainly characterized by haemorrhages secondary to surgery/invasive procedures or trauma. Haemostatic treatment in MH ranges from on demand to short prophylaxis according to the type of bleeding events and the basal clotting factor level. Oral surgery and dental extractions can represent a frequent haemostatic challenge in MH requiring appropriate treatment. However, only few studies on limited numbers of patients are available in the literature regarding the implications of dental management in patients with MH. Objectives: The purpose of the study was to evaluate the impact of dental care on the burden of haemostatic treatment in patients affected by MH…

Predictive factors of immune tolerance treatment response in severe haemophilia A patients with inhibitors: A real‐world report from a single centre,…

2019

Bayesian pharmacokinetic-guided prophylaxis with recombinant factor VIII in severe or moderate haemophilia A

2018

Introduction: Personalised pharmacokinetics (PK) using Bayesian analysis with limited sampling is assumed to help to optimise prophylaxis in haemophilia A (HA) patients. Materials and methods: Our prospective, observational study analysed the influence of PK parameters on clinical variables (bleeding rates, joint status, adherence, and consumption) using myPKFiT (R) in a cohort of twenty-one severe and moderate HA patients on prophylaxis with recombinant FVIII (Advate (R)) in two periods of one year, the first before PK-based tailoring and the second after PK-guided prophylaxis. Intra-individual and inter-individual coefficients of variation (CV) of half-life (t(1/2)) were calculated. Resul…

Surface electrical stimulation of the quadriceps femoris in patients affected by haemophilia A.

2006

Eighteen sessions of surface electrical stimulation was applied to the quadriceps femoris of the left leg of ten male subjects affected by severe haemophilia A, while ten healthy subjects constituted the control group. The isometric strength, the electromyographic activity and the diameter of the rectus femoris were measured in both legs before and after a six-week treatment period. After the treatment, the people affected by haemophilia showed a gain in strength by 13.8% in the stimulated leg and by 17.1% in the non-stimulated one. No changes were detected in the electromyographic activity. On the contrary, the diameter of the rectus femoris of the stimulated leg increased in 24.34%, while…

Results of an orthopaedic survey in young patients with severe haemophilia in Spain

2002

Summary.  This paper outlines the results obtained in a cross-sectional study of a group of young patients with severe haemophilia A and B. The primary aim of the study was to ascertain the level of orthopaedic complications in the group, the effects that these complications have on quality of life, and the medical resources used on these patients. The secondary aim was to relate their current orthopaedic state to the type of treatment received before the study. The study was carried out in 11 hospitals in Spain, where 70 severe haemophilia patients (factor VIII [FVIII] < 2%), with an average age of 21.6 and a median age of 22, and no inhibitors, were monitored. The percentage of patients s…

Autoantibody Inhibitor Eradication In Acquired Hemophilia Associated with Cancer: a Retrospective Analysis

2010

Abstract Abstract 1418 Introduction: Acquired hemophilia (AH), a rare autoimmune disorder primarily of adults, is typically characterized by the presence of IgG oligoclonal antibodies to the clotting factor VIII protein (FVIII). About 10–15% of patients with AH have an underlying malignancy, but the etiologic relationship of cancer to formation of FVIII inhibitor is yet to be determined. To date, there have been no published, comprehensive reviews on the efficacy of various treatments for AH in the context of either solid tumor or hematologic malignancies. Therefore, we have systematically reviewed 86 patients with cancer-associated AH from our own cancer center and from the published liter…