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Haemophilia, hemophilia

D Q Tran, V Barry, A Antun, M Ribeiro, S Stein, C L Kempton
INTRODUCTION: Poor adherence to factor replacement therapy among patients with haemophilia can lead to joint bleeding and eventual disability. AIM: The aim of this study was to determine patient-related characteristics associated with adherence to factor replacement in adults with haemophilia. METHODS: Adults with haemophilia were recruited to participate in this cross-sectional study. Adherence was measured using either the Validated Hemophilia Regimen Treatment Adherence Scale (VERITAS)-Pro or the VERITAS-PRN questionnaire...
September 30, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Ruben Cuesta-Barriuso, Ana Torres-Ortuño, Sofía Pérez-Alenda, Juan José Carrasco, Felipe Querol, Joaquín Nieto-Munuera
INTRODUCTION: Sports activities are part of multidisciplinary treatments in people with hemophilia. AIM: The objective of this study was to assess the incidence of sports activities in the quality of life as perceived by children with hemophilia. METHODS: A total of 53 children with hemophilia aged 7 to 13 years and 51 children without hemophilia were evaluated. The perception of quality of life, clinical variables, and the frequency of sports activities were registered...
2016: Pediatric Physical Therapy
A H Miners, S Krishnan, K J Pasi
Essentials No randomized trials have compared long-acting factor VIII (FVIII) with currently used products. A comparison was undertaken using a decision model to predict FVIII use and number of bleeds. In the base case, longer acting FVIII reduced factor use by 17% while resulting in similar bleeds. The value of longer acting FVIII will be largely determined by existing regimens and unit price. Click to hear Prof. Makris's presentation on new treatments in hemophilia SUMMARY: Background Recently, factor VIII (FVIII) products with longer half-lives, such as recombinant FVIII Fc fusion protein (rFVIIIFc), have become available...
August 10, 2016: Journal of Thrombosis and Haemostasis: JTH
Yan Zeng, Ruiqing Zhou, Xin Duan, Dan Long
BACKGROUND: Acquired haemophilia A is a rare bleeding disorder caused by the development of specific autoantibodies against coagulation factor VIII. Rituximab may be an alternative approach to the treatment of acquired haemophilia by eradicating FVIII autoantibodies. OBJECTIVES: To assess and summarise the efficacy and adverse effects of rituximab for treating people with acquired haemophilia A. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's trials registers, comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and conference proceedings...
2016: Cochrane Database of Systematic Reviews
P M Poonnoose, P Hilliard, A S Doria, S N Keshava, S Gibikote, M L Kavitha, B M Feldman, V Blanchette, A Srivastava
OBJECTIVES: This study was undertaken to determine the correlation between the radiological changes in haemophilic arthropathy [X-ray, Ultrasound (US) and MRI] and clinical assessment as determined by the Hemophilia Joint Health Score (HJHS); and to document the US and MRI changes in joints that appear normal on plain X-ray and clinical evaluation. MATERIALS AND METHODS: Of 55 study joints (22 knees and 33 ankles) in 51 patients with haemophilia/von Willebrand disease, with a median age of 15 years (range: 5-17) were assessed using X-rays (Pettersson score) and clinical examination (HJHS) at two centres (Toronto, Canada; Vellore, India)...
July 7, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
S J Lane, N S Sholapur, C H T Yeung, A Iorio, N M Heddle, M Sholzberg, M Pai
INTRODUCTION: Care for persons with haemophilia (PWH) is most commonly delivered through the integrated care model used by Hemophilia Treatment Centers (HTCs). Although this model is widely accepted as the gold standard for the management of haemophilia; there is little evidence comparing different care models. AIM: We performed a qualitative study to gain insight into issues related to outcomes, acceptability, equity and feasibility of different care models operating in the US...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
M Pai, N Santesso, C H T Yeung, S J Lane, H J Schünemann, A Iorio
BACKGROUND: Rigorous and transparent methods are necessary to develop clinically relevant and evidence-based practice guidelines. We describe the development of the National Hemophilia Foundation-McMaster Guideline on Care Models for Haemophilia Management, which addresses best practices in haemophilia care delivery. METHODS: We assembled a Panel of persons with haemophilia (PWH), parents of PWH, clinical experts and guideline methodologists. Conflicts of interest were disclosed and managed throughout...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Michael Wang, María Teresa Álvarez Román, Pratima Chowdary, Doris V Quon, Kim Schafer
The World Federation of Hemophilia and the National Hemophilia Foundation encourage people with haemophilia (PWH) to participate in routine physical activity. The benefits of physical activity for PWH include improvements in joint, bone, and muscle health. Accordingly, a number of studies suggest that levels of physical activity among PWH are similar to those of their healthy peers, especially among individuals who began prophylaxis at an early age (≤3 years). Importantly, several studies found either no increased risk or only a transient increase in risk of bleeding with more intensive physical activity compared with less intensive physical activity...
April 23, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
G S Schäfer, S Valderramas, A R Gomes, M B Budib, Á L P Wolff, A A T Ramos
INTRODUCTION: Exercise can provide numerous benefits to haemophilia patients, including bleeding reduction in muscles and joints. AIM: This systematic review (SR) aims to evaluate the effects of physical exercise on pain and the musculoskeletal function of patients with hemophilia. METHODS: Literature searches of Pubmed, Web of Science, PEDro, Cochrane, Clinical Trials SciELO and Lilacs were performed. The risks of bias were measured using the JADAD scale...
May 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Annarita Tagliaferri, Caterina Di Perna, Chiara Biasoli, Gianna Franca Rivolta, Gabriele Quintavalle, Gianfranco Cervellin, Marco Barozzi, Laura Benedettini, Corrado Pattacini
Treatment of patients with inherited bleeding disorders (PWIBD) in the emergency department (ED) is challenging. In 2010, a project was started involving all eight hemophilia centers (HC) and all 44 EDs of the Region of Emilia-Romagna (Italy) to improve emergency care for PWIBD. The project incorporates guidelines for emergency treatment, education for ED staff, and a dedicated Web site providing extensive information, proposing treatments, and sharing data with patients' electronic clinical records. A Web algorithm, accessible to PWIBD as well as ED and HC staff, suggests the first dose of concentrate for each type and severity of bleed or trauma...
July 2016: Seminars in Thrombosis and Hemostasis
Satya Bhusan Senapati, Sudhansu Sekhar Mishra, Manmath Kumar Dhir, Srikanta Das
Hemophilia A is an inherited, X-linked, recessive disorder caused by deficiency of clotting factor VIII. Intracranial hemorrhage is the leading cause of morbidity and mortality in these patients. Use of factor replacement products and medications had improved outcome in these patients. But in developing countries many such patients are not able to afford factor replacement products. We report a case of traumatic intracerebellar hemorrage in a hemophilia child. This child presented to us as a case of sub-acute intracerebellar hemorrage, he was managed conservatively with six units of fresh frozen plasma transfusion...
April 2016: Asian Journal of Neurosurgery
H L Sun, M Yang, A S Sait, A von Drygalski, S Jackson
INTRODUCTION: An increased prevalence of hypertension has been reported in patients with haemophilia compared to the age-matched general population, although the causes were unclear. To date, there has been limited data implicating haemophilia-specific risk factors such as renal bleeding. AIM: This two-centre prospective cohort study aimed to assess the prevalence of gross/microscopic haematuria, and the associations between haematuria, blood pressure and renal function...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
D Page, S Crymble, K Lawday, M Long, J Stoffman, L Waterhouse, P Wilton
INTRODUCTION: A network of 25 haemophilia/inherited bleeding disorder comprehensive care centres was established in Canada in the 1970s and 1980s. In 2007, standards of care, focused on the structural and resource requirements necessary to effectively provide optimal care, were adopted. AIM: Assess how human and physical resources affect centres' capacity to attain standards of care. METHODS: The Canadian Hemophilia Society (CHS), with the support of the Association of Hemophilia Clinic Directors of Canada (AHCDC), undertook the assessment...
July 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
Szymon Janczar, Joanna Kosinska, Rafal Ploski, Agata Pastorczak, Olga Wegner, Beata Zalewska-Szewczyk, Adam J W Paige, Maciej Borowiec, Wojciech Mlynarski
We have recently described a severe haemophilia A and moyamoya (SHAM) syndrome caused by Xq28 deletions encompassing F8 and the BRCC3 familial moyamoya gene. The phenotype includes haemophilia A, moyamoya angiopathy, dysmorphia and hypertension. The genetic analysis of the family of our SHAM patient demonstrated carrier state in proband's mother and sister. The patient's mother is apparently well, whereas his currently 18-years-old sister presents with mild haemophilia A, coarctation of the aorta, hypertension, and ventricular arrhythmia...
January 2016: European Journal of Medical Genetics
Marc Dauty, Raphael Gross, Fabien Leboeuf, Marc Trossaert
BACKGROUND: Severe hemophilia is an inherited, lifelong bleeding disorder characterized by spontaneous bleeding, which results in painful joint deformities. Currently two surgical treatments are available to treat haemophilia-related ankle joint destruction: ankle arthrodesis and total ankle replacement. The aim of the present study was to compare these two surgical procedures in haemophiliac subjects. CASE PRESENTATION: Kinematic and dynamic parameters were quantified using a three-dimensional gait-analysis system in two similar clinical cases...
2015: BMC Research Notes
Xiao-yun Lian, Kui-xing Li, Bao-lai Hua, Yong-qiang Zhao
OBJECTIVE: To investigate the benefit of low-dose tertiary prophylaxis in adults with severe haemophilia A(SHA). METHODS: Twenty-two SHA patients aged 18 to 60 years from the Haemophilia Centre of Peking Union Medical College Hospital, Beijing, China, were retrospectively observed from their one year on-demand treatment to one year tertiary prophylaxis using plasma derived factor 8 concentrates at 5-10 IU/kg 2-3x per week. All the patients had already developed arthropathy...
August 2015: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
Ingrid Pabinger, Max Heistinger, Wolfgang Muntean, Sylvia-Elisabeth Reitter-Pfoertner, Sabine Rosenlechner, Thomas Schindl, Gerhard Schuster, Werner Streif, Katharina Thom, Christoph Male
This guideline which is endorsed by the Austrian Society of Haemophilia, the Austrian Society of Paediatrics, and the Austrian Society of Haematology & Medical Oncology is intended to give a clear and practical guidance for diagnosing and treating haemophilia in Austria. In the treatment of haemophilia there are few controlled interventional trials, and recommendations usually have a rather low level of evidence.The main basis for this paper are the new international guidelines by the World Federation of Hemophilia, published in 2013...
November 2015: Wiener Klinische Wochenschrift
A Martínez-Esteve, R M Álvarez-Pérez, R Núñez-Vázquez, J L Tirado-Hospital, R García-Jiménez, J Povedano-Gómez, I Borrego-Dorado
OBJECTIVE: To assess the outcome and adverse-effects of the radioisotope synoviorthesis in paediatric and adolescent patients with haemophilia. MATERIAL AND METHODS: Prospective study of historical cohort was conducted. A total of 20 consecutive haemophiliacs with a mean age of 13.1 years (range 4-17) were included with a mean follow-up of 64.9 months (range 18-109). The diagnosis of synovitis was established on the basis of clinical follow-up including radiological images (radiography and/or MRI)...
January 2016: Revista Española de Medicina Nuclear e Imagen Molecular
L Minuk, S Jackson, A Iorio, M-C Poon, E Dilworth, K Brose, R Card, I Rizwan, B Chin-Yee, M Louzada
INTRODUCTION: Improvements in haemophilia care have increased life expectancy in persons with haemophilia (PWH). This ageing population presents clinicians with management challenges as they develop age-related comorbidities such as cardiovascular disease (CVD). AIMS: To assess the epidemiology of CVD risk factors and events in an ageing Canadian haemophilia population. METHODS: A retrospective, multicentre chart review was carried out at five Canadian Hemophilia Treatment Centres...
November 2015: Haemophilia: the Official Journal of the World Federation of Hemophilia
A Lambing, E Kachalsky, L M Mueller, P Kuriakose
OBJECTIVES: In 2011, 6.98-million offenders were documented in the adult correctional system, with state operating costs designated 12% towards medical care ($11.97 day per inmate) for the general population. Common co-existing health problems identified are: arthritis (13%), hypertension (11%), asthma (10%) and heart problems (6%). Less than 5% of inmates have health issues related to cancer, diabetes, liver or renal problems and communicable diseases. The leading cause of death is suicide (33...
November 2015: Haemophilia: the Official Journal of the World Federation of Hemophilia
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