Search results for "Haemophilia"

Joint protection in haemophilia

2011

Haemarthroses (intra-articular haemorrhages) are a frequent finding typically observed in patients with haemophilia. Diagnosis and treatment of these bleeding episodes must be delivered as early as possible. Additionally, treatment should ideally be administered intensively (enhanced on-demand treatment) until the resolution of symptoms. Joint aspiration plays an important role in acute and profuse haemarthroses as the presence of blood in the joint leads to chondrocyte apoptosis and chronic synovitis, which will eventually result in joint degeneration (haemophilic arthropathy). Ultrasonography (US) is an appropriate diagnostic technique to assess the evolution of acute haemarthrosis in hae…

Acquired haemophilia in cancer: A systematic and critical literature review

2017

Aim There is a paucity of data on the clinical presentation and management of cancer patients with acquired haemophilia (AH), we here report a systematic literature review on acquired haemophilia in the context of cancer. Methods Treatment outcomes of AH were defined as complete response (CR), partial response (PR) or no response (NR), based on inhibitor eradication, coagulation factor VIII levels and bleeding control. Reported deaths were either related to cancer or bleeding. Results Overall, 105 cases were collected and analyzed according to classification of cancer and efficacy of treatments for inhibitor and malignancy. The mean age was 68 years for both males (range 37-86 years) and fe…

Management of the Sponataneous Bleeding Episodes in Factor VII Deficiency. A Prospective Evaluation of the STER,

2011

Abstract Abstract 3368 Introduction Patients with an inherited factor VII (FVII) deficiency may display a wide range of clinical phenotypes, from an asymptomatic condition to serious hemorrhagic episodes such as fatal central nervous system (CNS) or gastrointestinal (GI) bleeds (Mariani G et al. Thromb Haemost 2005; 93: 481–7). Symptomatic patients can be divided into two major categories: those with mild-to-moderate bleeding tendency and individuals with a severe bleeding tendency which may be more severe than that in hemophilia. The former group mainly experience mucosal bleeding, a clinical picture that mimics that of a platelet disorder and often does not call for treatment. In contrast…

Home‐delivered ultrasound monitoring for home treatment of haemarthrosis in haemophilia A

2015

Confronting the psychological burden of haemophilia

2010

Providing comprehensive care, counselling and support to haemophilic patients, and their parents have always been quite complex for haemophilia treatment centres. Nowadays, starting with recent developments in genetic counselling, prenatal diagnosis and carrier testing, the psychological burden on patients and parents might possibly have increased, compared with even the recent past. The emotional strains and worries associated with a possibly affected newborn and his care through childhood and adolescence may also have a grievous impact on couple dynamics and marital relationship. The impact may be even higher in families in which haemophilia is newly diagnosed. The main psychological prob…

Acquired Hemophilia A Associated with Venous Thrombosis and Very High Inhibitor Titer: A Challenging Scenario

2019

The WFH Haemophilia Centre Twinning Programme: 10 years of growth, 1993-2003

2003

The World Federation of Haemophilia (WFH) Twinning Programme celebrates its tenth anniversary this year. Twinning is one of several international WFH programmes designed to improve haemophilia care at a global level. There are two types of twinning, and the haemophilia treatment centre twinning programme should be distinguished from the WFH haemophilia organization twinning involving national member organizations. The WFH Haemophilia Treatment Centre Twinning Programme helps emerging haemophilia treatment centres develop partnerships with well-established and experienced centres. Twinning can improve diagnosis and clinical care through coaching, training and transfer of expertise, ultimatel…

Joint damage and motor learning during unipedal stance in haemophilia arthropathy: report of two cases

2016

Hemofilia: ejercicio y deporte

2011

El ejercicio físico y el deporte constituyen uno de los pilares básicos en el tratamiento de la hemofilia. Este trabajo describe un resumen de las características de la hemofilia y, a través de una exhaustiva revisión bibliográfica, se analiza la importancia de la condición física, en relación con la prevención y el tratamiento de las lesiones musculoesqueléticas en el paciente hemofílico. La hemofilia es una enfermedad hematológica hereditaria, con lesiones ortopédicas características. Afecta a individuos varones y presenta un déficit de factores de la coagulación que provoca hemorragias incluso espontáneas (en pacientes graves sin tratamiento). Las lesiones más frecuentes son: hemartrosis…

Patient preferences in the treatment of hemophilia A: impact of storage conditions on product choice

2018

Bernd Tischer,1 Renato Marino,2 Mariasanta Napolitano3 1Kantar Health, Munich, Germany; 2Haemophilia and Thrombosis Centre, University Hospital of Bari, Apulia, Italy; 3University of Palermo, Reference Regional Center for Thrombosis and Hemostasis Hematology Unit, Palermo, Italy Objectives: To gain insights into the usage of factor VIII (FVIII) products by patients diagnosed with moderate/severe hemophilia A, and to assess the impact and perceived importance of product storage.Methods: In this study, 200 patients diagnosed with moderate or severe hemophilia A across seven countries participated. Data were collected via a 30-minute, face-to-face interview in six countries and via a web-based…