Search results for "Hemophilia B"

New data from the Italian National Register of Congenital Coagulopathies, 2016 Annual Survey

2018

BACKGROUND: In Italy, the National Register of Congenital Coagulopathies (NRCC) collects epidemiological and therapeutic data from patients affected by haemophilia A (HA), haemophilia B (HB), von Willebrand’s disease (vWD) and other rare coagulation disorders. Here we present data from the 2016 annual survey. MATERIALS AND METHODS: Data are provided by the Italian Haemophilia Centres, on a voluntary basis. Information flows from every Centre to a web-based platform of the Italian Association of Haemophilia Centres, shared with the Italian National Institute of Health, in accordance with current privacy laws. Patients are classified by diagnosis, disease severity, age, gender and treatment-r…

Management of dental extraction in patients with Haemophilia A and B: a report of 58 extractions

2013

Objectives: Patients with inherited bleeding disorders are at high risk of bleeding following oral surgery and present challenges to the oral surgeons. Aim of this study was to report our experience in dental extraction in patients exhibiting Haemophilia A and B between 2007 and 2012. Patient and Methods: 58 dental extractions in 15 patients during 19 interventions were performed. Replacement therapy with recombinant and plasma-derived factor VIII and IX was applied systematically in combination with antifibrinolytic treatment and local haemostatic measures. The following data were recorded: type of surgery, applied local haemostatic measures, general substitution, systemic antifibrinolytic…

Electromyographic and Safety Comparisons of Common Lower Limb Rehabilitation Exercises for People With Hemophilia.

2020

Abstract Background Ankles and knees are commonly affected in people with hemophilia and thus are targets for prevention or rehabilitation. However, to our knowledge, no studies have evaluated muscle activity and safety during exercises targeting the lower limbs in people with hemophilia; this lack of information hinders clinical decision-making. Objective The aim of this study was to compare the tolerability of, safety of, and muscle activity levels obtained with external resistance (elastic or machine)-based and non–external resistance-based lower limb exercises in people with hemophilia. Design This was a cross-sectional study. Methods Eleven people who had severe hemophilia and were und…

Neuromuscular control during gait in people with haemophilic arthropathy

2019

Introduction: Effects of haemophilic arthropathy on neuromuscular control during gait are currently unknown. Aims: (a) To assess how haemophilic arthropathy affects the complexity of neuromuscular control during gait; (b) To investigate the relationship between complexity of neuromuscular control and joint impairment. Methods: Thirteen control subjects (CG) walked overground at their preferred and a slow velocity and thirteen people with haemophilic arthropathy (PWHA) walking at their preferred velocity. Surface electromyography (EMG) was collected from eleven leg muscles. Electromyography variance explained by muscle synergies (sets of co-activated muscles that can be recruited by a single…

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…

Safety and Effectiveness of Progressive Moderate-to-Vigorous Intensity Elastic Resistance Training on Physical Function and Pain in People With Hemop…

2020

Abstract Objective Strength training is recommended for people with hemophilia; however, published data are anecdotal and have methodological limitations. The purpose of this study was to evaluate the safety and effectiveness of progressive moderate-to-vigorous intensity elastic resistance training on physical function and pain in this patient population. Methods A randomized controlled trial was conducted in a university laboratory setting where 20 patients (17 with severe, 1 with moderate, and 2 with mild hemophilia) aged 21 to 53 years received evaluations at baseline and 8-week follow-up. Participants were allocated to intervention (progressive strength training) or control (usual daily…

Comparison of the rates of joint arthroplasty in patients with severe factor VIII and IX deficiency: an index of different clinical severity of the 2…

2009

AbstractData from the Italian Hemophilia Centres were collected to perform a retrospective survey of joint arthroplasty in patients with severe hemophilia. Twenty-nine of 49 hemophilia centers reported that 328 of the 347 operations were carried out in 253 patients with severe hemophilia A (HA) and 19 in 15 patients with severe hemophilia B (HB). When results were normalized to the whole Italian hemophilia population (1770 severe HA and 319 severe HB), patients with HA had a 3-fold higher risk of undergoing joint arthroplasty (odds ratio [OR], 3.38; 95% confidence interval [CI], 1.97-5.77; P &lt; .001). These results were confirmed after adjustment for age, HIV, hepatitis C virus (HCV), and…

Gene symbol: f9.

2007

Health-related quality of life and psychological well-being in elderly patients with haemophilia

2011

Many persons with severe haemophilia reach seniority thanks to effective treatment. There is no information on health-related quality of life (HRQoL) of these patients, who had lived for many years when regular replacement therapy was unavailable. Italian patients with severe haemophilia aged ≥65 years born in the 1940s or earlier were compared with men without bleeding disorders matched for age and geography. HRQoL was assessed via generic and disease-specific questionnaires. Potential associations with concomitant illnesses, orthopaedic status, physical functioning, cognitive status and depression were evaluated. In addition, the newly adapted HRQoL questionnaire specific for elderly pers…

Using the Hemophilia Joint Health Score for assessment of children: Reliability of the Spanish version

2018

Introduction: Numerous measuring instruments for the evaluation of hemophilic arthropathy have been developed. One of the most used systems is the Hemophilia Joint Health Score (HJHS) given its sensitivity to clinical changes appearing in the joints because of recurrent hemarthrosis. Objective: Assessing the interrater reliability, using the Spanish version of the HJHS (version 2.1) in children with hemophilia. Design: Reliability study to assess the interrater reliability of the Spanish version of HJHS. Methods: A sample of 36 children aged 7–13 years diagnosed with hemophilia A or B was used. Two physiotherapists performed physical assessments with the Spanish version of the HJHS. Descrip…