Search results for "Haemophilia"

Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab: AICE practical guidance in collaboration with SIBi…

2020

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agen…

A novel nonsense mutation in exon 2 of the factor IX gene resulting in severe haemophilia B

2006

Psychosocial aspects of haemophilia: a systematic review of methodologies and findings

2011

Psychosocial factors have a significant impact on quality of life for patients with chronic diseases such as haemophilia. Interventions to support the psychosocial needs of patients and their families, such as offering information and assistance, clarifying doubts, and teaching coping strategies to minimize the impact of disabilities, may help to maximize patient outcomes and improve quality of life for their families. The aim of this study was to evaluate the current literature on psychosocial aspects of haemophilia. Literature searches were performed using the PubMed database to identify studies evaluating psychosocial stressors in persons with haemophilia. Articles pertaining to the HIV …

Fostering drug.storage,preparation and treatment modalities improves adherence to treatment with turoctocog-alfa in patients with haemophilia A:resul…

2019

Personalized prophylaxis with simoctocog-alfa(human-cl rhFVIII):A real-life experience on children and moderate haemophilia A patients

2019

Combined Point of Care Tools Are Able to Improve Treatment Adherence and Health-Related Quality of Life in Patients with Severe Hemophilia: An Observ…

2019

Introduction: Ultrasound (US) assessment of joints is an evolving point of care tool for the detection of early joint arthropathy (Napolitano M, Kessler CM. Hemophilia A and B. Consultative Hemostasis and Thrombosis, Kitchens, 4th edition); population pharmacokinetic (pop-PK) studies are adopted as a useful instrument to set the prophylaxis regimen for patients with hemophilia, they may improve adherence (Nagao A.et al. Thromb Res. 2019 Jan; 173:79-84) and reduce the annual bleeding rate (ABR). Adherence to continuous intravenous administrations of factor VIII or Factor IX products is challenging, thus patients may experience breakthrough bleedings while on prophylaxis. Repeated US examinat…

Postural Assessment Systems in the Prevention of Haemophilic Arthropathy: A Narrative Review

2022

This narrative review aims to give an overview of some postural evaluation systems currently used in patients with haemophilia. Among them, first, we analyse the HJHS scale, recognized as a specific evaluation tool for haemophilic arthropathy. Second, we focus on other systems usually used in non-haemophilic patients that have also shown good applicability in this patient category, such as gait analysis, stabilometry, and baropodometric examination. This review underlines the use these tools could have in clinical practice to identify the early postural alterations in patients with haemophilia and set up personalised rehabilitation programs.

Orthodontic management in patients with haemophilia. About two clinical cases

2009

The most common congenital bleeding disorder is haemophilia. It is a pathology inherited and caused by a defective or an absence of the coagulation factors, so that haemophiliacs cannot form an efficient clot. These patients have been treated with fear in the dental profession without having achieved the goals and ideals most appropriate according to the requirements of each case, which is demonstrated in the little existing literature available. However, they are currently treated as healthy orthodontic patients thanks to the advances in orthodontics. We present the cases of two brothers with mild classical haemophilia, who were treated by Mc Namara?s disjunctor. The emphasis is on importa…

Activated prothrombin complex concentrate (FEIBA® ) in acquired haemophilia A: a large multicentre Italian study - the FAIR Registry

2019

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…