4,540 results match your criteria Haemophilia[Journal]


Standard and water rehabilitation: An analysis of over 14 years' experience in patients with haemophilia or other clotting factor disorders after orthopaedic surgery.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Dipartimento di Riabilitazione, Fondazione T. Camplani - Casa di Cura "Domus Salutis", Brescia, Italy.

Introduction: In people with haemophilia (PWH) with severe arthropathy, total joint replacement (TJR) can be undertaken if conservative management fails. Post-operative rehabilitation treatment is an important part of the comprehensive management of patients undergoing TJR.

Aim: To compare post-operative standard rehabilitation (SR) and SR plus water rehabilitation (WR) in PWH undergoing TJR. Read More

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http://dx.doi.org/10.1111/hae.13748DOI Listing
April 2019
1 Read

Missense changes in the catalytic domain of coagulation factor X account for minimal function preventing a perinatal lethal condition.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.

Introduction: Inherited deficiencies in the coagulation pathway provide diversified models to investigate the molecular bases of perinatal lethality associated with null-like variants. Differently from X-linked haemophilias, homozygous/doubly heterozygous null variants in the rare autosomally inherited deficiency of factor X (FX) might be incompatible with perinatal survival.

Aim: To provide experimental evidence about the null/close-to-null FX function. Read More

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http://dx.doi.org/10.1111/hae.13761DOI Listing
April 2019
1 Read

Prophylactic protocol for dental care in ligneous gingivitis due to severe plasminogen deficiency: Case report and review of literature.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Clinical Medicine I, Department of Medicine, University of Padua, Padua, Italy.

Introduction: Severe plasminogen (PLG) deficiency causes ligneous conjunctivitis, a rare disease characterized by the growth of fibrin-rich pseudomembranes on mucosal surfaces; gums involvement leads to ligneous gingivitis (LG). Specific therapy for LG is not available yet. We report a prophylactic treatment with enoxaparin and fresh frozen plasma (FFP) for invasive dental procedures in a patient with LG, and a review of literature on LG treatment. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/hae.13745
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http://dx.doi.org/10.1111/hae.13745DOI Listing
April 2019
1 Read

Coping strategies in young and adult haemophilia patients: A tool for the adaptation to the disease.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Department of Basic Psychology and Methodology, Faculty of Psychology, University of Murcia, Murcia, Spain.

Introduction: Chronic diseases, after diagnosis, involve changes that have to favour coping with the new situation. The resources used will help control, manage and adapt to the disease. The psychological aspects may be influencing how the individual faces the situation. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/hae.13743
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http://dx.doi.org/10.1111/hae.13743DOI Listing
April 2019
1 Read

Assessment of Kinect V2 for elbow range of motion estimation in people with haemophilia using an angle correction model.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Department of Physiotherapy, University of Valencia, Valencia, Spain.

Introduction: The joint range of motion (ROM) is an important clinical parameter used to assess the loss of functionality resulting from joint bleedings in people with haemophilia. These episodes require a close follow-up and, to decrease patients' hospital dependence, telemedicine tools are needed. Therefore, this study is aimed to analyse the validity of the Microsoft Kinect V2 sensor with corrected angle measurement to be used in the monitoring of elbow ROM in people with haemophilia. Read More

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http://dx.doi.org/10.1111/hae.13744DOI Listing

Periprocedural management of von Willebrand disease: An institutional experience.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Department of Medicine, Division of Hematology/Oncology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

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http://dx.doi.org/10.1111/hae.13718DOI Listing

Regional variation and cost implications of prescribed extended half-life factor concentrates among U.S. Haemophilia Treatment Centres for patients with moderate and severe haemophilia.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

St. Jude Children's Research Hospital, Memphis, Tennessee.

Background: Extended half-life (EHL) factor VIII (FVIII) and IX (FIX) products are intended to decrease the burden of prophylaxis for patients with haemophilia A or B. Whether these newer concentrates have led to meaningful clinical practice change remains vague.

Aim: To characterize the longitudinal use of standard (SHL) and EHL factor concentrates at haemophilia treatment centres (HTCs), using the ATHNdataset, a US database of 138 ATHN-affiliated HTCs. Read More

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http://dx.doi.org/10.1111/hae.13758DOI Listing
April 2019
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Efficacy and safety of prophylaxis with BAY 81-8973 in Chinese patients with severe haemophilia A enrolled in the LEOPOLD II trial.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Bayer, Whippany, New Jersey.

Introduction: BAY 81-8973 (Kovaltry ) is a full-length, unmodified recombinant human factor VIII approved in China for prophylaxis and on-demand treatment in patients with haemophilia A. Limited access to FVIII prophylaxis in China has historically led to this population being undertreated. This subanalysis of LEOPOLD II investigated whether the efficacy and safety of BAY 81-8973 varied between Chinese and non-Chinese patients. Read More

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http://dx.doi.org/10.1111/hae.13751DOI Listing
April 2019
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Extreme skewing of X-inactivation: Rethinking severe haemophilia in women and girls.

Haemophilia 2019 Apr 17. Epub 2019 Apr 17.

Little Red Drop Haematology, The Wesley Hospital, Auchenflower, Queensland, Australia.

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https://onlinelibrary.wiley.com/doi/abs/10.1111/hae.13755
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http://dx.doi.org/10.1111/hae.13755DOI Listing
April 2019
1 Read

Consensus statements on vaccination in patients with haemophilia-Results from the Italian haemophilia and vaccinations (HEVA) project.

Haemophilia 2019 Apr 16. Epub 2019 Apr 16.

Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.

Vaccination against communicable diseases is crucial for disease prevention, but this practice poses challenges to healthcare professionals in patients with haemophilia. Poor knowledge of the vaccination requirements for these patients and safety concerns often result in vaccination delay or avoidance. In order to address this issue, a panel of 11 Italian haemophilia and immunization experts conducted a Delphi consensus process to identify the main concerns regarding the safe use of vaccines in patients with haemophilia. Read More

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http://dx.doi.org/10.1111/hae.13756DOI Listing
April 2019
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The impact of psychosocial determinants on caregivers' burden of children with haemophilia (results of the BBC study).

Haemophilia 2019 Apr 11. Epub 2019 Apr 11.

Centre for Outcomes and Experience Research in Childhood Health, Illness and Disability (ORCHID) Great Ormond Street Hospital for Children NHS Trust, London, UK.

Introduction: Caring for a child with haemophilia is burdensome and impacting on caregivers' emotional and financial status. This paper assesses the impact of psychosocial determinants on caregivers' burden across European countries.

Methods: This non-interventional study enrolled caregiver/child dyads at haemophilia treatment centres (HTCs) using the "HEMOphilia associated CAregiver Burden scale" (HEMOCAB). Read More

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http://dx.doi.org/10.1111/hae.13684DOI Listing

The burden of bleeds and other clinical determinants on caregivers of children with haemophilia (the BBC Study).

Haemophilia 2019 Mar 29. Epub 2019 Mar 29.

Department of Medical Psychology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany.

Introduction: Treatment burden for the people with haemophilia has been documented, as has the burden of caring for a child with a common chronic disease such as asthma or diabetes. However, there remains a paucity of data about caregiver burden in haemophilia.

Aims: The aim of this study was to evaluate the impact of bleeding on caregivers of children with haemophilia. Read More

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http://dx.doi.org/10.1111/hae.13736DOI Listing

Population-based surveillance of haemophilia and patient outcomes in Indiana using multiple data sources.

Haemophilia 2019 Mar 29. Epub 2019 Mar 29.

Indiana Hemophilia and Thrombosis Center, Indianapolis, Indiana.

Introduction: Epidemiological surveillance of haemophilia through linkage of medical records within a US state has not been conducted in 20 years.

Aim: The Indiana Haemophilia Surveillance Project aims to identify all persons with haemophilia who resided in Indiana in 2011-2013 and to determine the percentage of patients in Indiana cared for at a federally recognized haemophilia treatment centre (HTC).

Methods: A retrospective review of medical charts was conducted to identify haemophilia cases during the surveillance years. Read More

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http://dx.doi.org/10.1111/hae.13734DOI Listing
March 2019
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Spontaneous recurrent intracranial haemorrhage in a woman with type 2B von Willebrand disease: A clinical case and a brief literature review.

Haemophilia 2019 Mar 29. Epub 2019 Mar 29.

Department of Medicine, Haemophilia Centre, University Hospital of Padua, Padua, Italy.

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http://dx.doi.org/10.1111/hae.13742DOI Listing
March 2019
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Congenital Factor X deficiency in women: A systematic review of the literature.

Haemophilia 2019 Mar;25(2):195-204

Katharine Dormandy Haemophilia Centre, Department of Obstetrics & Gynaecology, Royal Free Hospital, London, UK.

Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. Read More

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http://dx.doi.org/10.1111/hae.13729DOI Listing
March 2019
4 Reads

Publishing in Haemophilia.

Haemophilia 2019 Mar;25(2):181-182

Georgetown University Medical Center, Washington, District of Columbia.

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http://dx.doi.org/10.1111/hae.13732DOI Listing

Tailored prophylaxis with rFXIII (NovoThirteen ) in a young girl with severe FXIII deficiency and previous cephalohaematoma.

Haemophilia 2019 Mar 19. Epub 2019 Mar 19.

Department of Medicine - DIMED, Thrombotic and Haemorrhagic and Coagulation Diseases Unit, Veneto Regional Centre for Haemophilia and Thrombophilia, Padova University Hospital, Padova, Italy.

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http://dx.doi.org/10.1111/hae.13723DOI Listing

Analysis of haemophilia case information of Shandong Province in China: Data from 2010 to 2017.

Haemophilia 2019 Mar 19. Epub 2019 Mar 19.

Shandong Hemophilia Treatment Center, Shandong Blood Center, Shandong, China.

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http://dx.doi.org/10.1111/hae.13724DOI Listing

Mortality of patients with haemophilia in Brazil: First report.

Haemophilia 2019 Mar 15. Epub 2019 Mar 15.

Faculty of Medicine, Universidade Federal de Minas Gerais, Belo Horizonte, Brazil.

Introduction: Brazil has the fourth largest world population of patients with haemophilia. However, mortality rates in this population are unknown.

Aim: To analyse mortality and its causes in Brazilian patients with haemophilia from 2000 to 2014. Read More

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http://dx.doi.org/10.1111/hae.13730DOI Listing

Prevalence and risk factors associated with hepatitis C among Brazilian male patients with haemophilia: A long-term follow-up.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Faculdade de Medicina da, Universidade José do Rosário Vellano, Belo Horizonte, Brazil.

People with haemophilia represent a population with a high prevalence of HCV infection due to the use of blood components and plasma-derived clotting factor concentrates before the introduction of viral-inactivating procedures (in the 1980s) and screening for HCV (in the 1990s). About 80% of HCV-infected patients have chronic HCV infection, and at least 20% develop end-stage liver disease. The aim of the study was to assess current anti-HCV positivity in a large cohort of Brazilian haemophilia patients and to determine associated factors with HCV exposure. Read More

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http://dx.doi.org/10.1111/hae.13728DOI Listing
March 2019
2 Reads

Scope of practice of haemophilia physiotherapists: A European survey.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Secteur des Sciences de la Santé, Institut de Recherche Expérimentale et Clinique, Neuromusculoskeletal Lab (NMSK), Université catholique de Louvain, Brussels, Belgium.

Introduction: European guidelines on the care of haemophilia recommend ready access to a range of services provided by a multidisciplinary team of specialists including physiotherapy. However, the scope of physiotherapy provided is unknown.

Methods: The Physiotherapists Committee of the European Association for Haemophilia and Allied Disorders (EAHAD) conducted a web-based survey to quantify the role and scope of practice of physiotherapists involved in haemophilia care. Read More

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http://dx.doi.org/10.1111/hae.13727DOI Listing

Whole blood ristocetin-induced platelet impedance aggregometry does not reflect clinical severity in patients with type 1 von Willebrand disease.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Background: The haemorrhagic phenotype in patients with von Willebrand disease (VWD) is heterogeneous, and assays of von Willebrand factor ristocetin cofactor activity (VWF:RCo) do not always reflect clinical severity, especially in those individuals classed as type 1 VWD. Recent studies have shown that whole blood ristocetin-induced platelet agglutination (WB-RIPA) using an easy-to-use analyzer, Multiplate® platelet impedance technique, could be informative as a diagnostic test in VWD, although inconsistencies were evident in patients with the type 1 disorder, possibly associated with clinical symptoms.

Aim: To investigate the relationship between WB-RIPA, bleeding scores (BS) and VWF-related measurements in type 1 VWD. Read More

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http://dx.doi.org/10.1111/hae.13725DOI Listing
March 2019
3 Reads

Anterior osteophyte resection of the ankle joint to increase range of motion in haemophilic ankle arthropathy.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Orthopaedic Surgery, Kyung Hee University Hospital at Gangdong, Seoul, Korea.

Introduction: Haemophilic ankle arthropathy may cause joint bleeding, pain, stiffness, erosion and progressive motion limitations, including a decreased range of motion (ROM) secondary to anterior osteophyte impingement.

Aim: The purpose of this study was to investigate changes in the ROM and symptoms after synovectomy and anterior osteophyte resection for haemophilic ankle arthropathy.

Methods: We retrospectively reviewed 41 ankles in 35 patients with haemophilic arthropathy who underwent arthroscopic and/or open synovectomy. Read More

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http://dx.doi.org/10.1111/hae.13719DOI Listing

Survey of non-physiotherapy department medical staff in China and their understanding of physiotherapy in comprehensive management of haemophilia.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Physical Medicine & Rehabilitation, Peking Union Medical College Hospital, Chinese Academy of Medical School & Peking Union Medical College, Beijing, China.

Introduction: Physiotherapy plays a very important role in comprehensive care of patients with haemophilia. Due to limited resources and a lack of understanding among medical personnel, physiotherapy has not become a standard component of comprehensive management of haemophilia in China.

Aim: To investigate the understanding and practice of physiotherapy in the comprehensive management of haemophilia by non-physiotherapy department medical staff engaged in haemophilia work in China. Read More

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http://dx.doi.org/10.1111/hae.13720DOI Listing

Outpatient treatment for haemophilic arthropathy with radiosynovectomy: Radiation dose to family members.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Radiology Departament, Clementino Fraga Filho University Hospital, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

One of the key features of good practice in medicine is the doctor-patient communication. Radiation protection standards for radiosynovectomy (RS) is limited. Yttrium-90 is a beta-emitting radioisotope used in RS to treat joint pain from haemophilic arthritis. Read More

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http://dx.doi.org/10.1111/hae.13710DOI Listing

Emicizumab prophylaxis to facilitate anticoagulant therapy for management of intra-atrial thrombosis in severe haemophilia with an inhibitor.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Pediatrics, Division of Hematology and Oncology, University of Michigan, Ann Arbor, Michigan.

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http://dx.doi.org/10.1111/hae.13721DOI Listing

Why plasma-derived factor VIII?

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Tulane University, New Orleans, Louisiana.

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http://doi.wiley.com/10.1111/hae.13705
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http://dx.doi.org/10.1111/hae.13705DOI Listing
March 2019
4 Reads

Characterization of two large duplications of F9 associated with mild and severe haemophilia B, respectively.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Department of Laboratory Medicine, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China.

Introduction: Only two large duplications of F9 causing haemophilia B (HB) have been reported.

Aim: To analyse the pathogenic mechanisms of large F9 duplications.

Methods: We have identified two large duplications of F9 (dup ex 1-6 and dup ex 4-6) associated with mild and severe HB in probands A and B, respectively. Read More

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http://dx.doi.org/10.1111/hae.13704DOI Listing
March 2019
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An exploratory comparison of single intra-articular injection of platelet-rich plasma vs hyaluronic acid in treatment of haemophilic arthropathy of the knee.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Haemophilia Care and Research Center, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.

Introduction: Intra-articular platelet-rich plasma (PRP) injection therapy has been extensively applied in clinical practice to treat musculoskeletal disorders such as osteoarthritis, but the treatment for haemophilic arthropathy is rarely reported.

Aims: This study aimed to compare the efficacy of intra-articular PRP vs hyaluronic acid (HA) injections in treating haemophilic arthropathy of knee joints.

Patients: Twenty-two haemophilia patients (mean age, 41. Read More

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http://dx.doi.org/10.1111/hae.13711DOI Listing
March 2019
12 Reads

Spinal dorsal rhizotomy plus concurrent left and right gastrocnemii releases in a 7-year-old child with haemophilia A and spastic cerebral palsy.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Heamophilia Centre, Great Ormond St Hospital for Children NHS Foundation Trust, London, UK.

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http://doi.wiley.com/10.1111/hae.13715
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http://dx.doi.org/10.1111/hae.13715DOI Listing
March 2019
10 Reads

Sustained high trough factor IX activity levels with continued use of rIX-FP in adult and paediatric patients with haemophilia B.

Haemophilia 2019 Mar 13. Epub 2019 Mar 13.

Center for Bleeding Disorders and Coagulation, Careggi University Hospital, Florence, Italy.

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http://dx.doi.org/10.1111/hae.13735DOI Listing

Exploring regional variations in the cross-cultural, international implementation of the Patient Reported Outcomes Burdens and Experience (PROBE) study.

Haemophilia 2019 Mar 12. Epub 2019 Mar 12.

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Background: The Patient Reported Outcomes Burdens and Experience (PROBE) study has developed and validated the PROBE questionnaire for assessing patient-reported outcomes in people with haemophilia and participants without bleeding disorders.

Objective: To explore the regional variations in the international implementation of the PROBE questionnaire.

Methods: Data were collected from participants in four regions (Western Pacific, South America, North America and Europe). Read More

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http://dx.doi.org/10.1111/hae.13703DOI Listing
March 2019
1 Read

Professional functioning of young adults with congenital coagulation disorders in the Netherlands.

Haemophilia 2019 Mar 12. Epub 2019 Mar 12.

Department of Pediatric-Hematology and Hemophilia Comprehensive Care Treatment Center, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Introduction And Aim: Suboptimal health-related quality of life and lowered employment rates found in a previous study in young adults (YA) with congenital coagulation disorders (CCD) in the Netherlands underline the need for more insight into professional functioning of YA with CCD and into determinants of professional functioning.

Methods: Young adults (18-30 years) with CCD participated in a cross-sectional study. Professional functioning was assessed with the Work Productivity and Activity Impairment questionnaire (WPAI). Read More

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http://dx.doi.org/10.1111/hae.13698DOI Listing

Acquired haemophilia A and concomitant factor XIII consumption.

Haemophilia 2019 Mar 7. Epub 2019 Mar 7.

Department of Laboratory Medicine and Pathobiology, St. Michael's Hospital, Toronto, Ontario, Canada.

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http://dx.doi.org/10.1111/hae.13690DOI Listing
March 2019
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Erectile dysfunction in patients with haemophilia.

Haemophilia 2019 Mar 28;25(2):283-288. Epub 2019 Feb 28.

British Columbia Provincial Bleeding Disorders Program - Adult Division, St. Paul's Hospital, Vancouver, British Columbia, Canada.

Introduction: Patients with haemophilia (PWH) are experiencing a nearly normal life span with safe factor replacement therapy and effective antiviral treatments for co-infections. As a result, many ageing-related health issues are starting to emerge. One rarely discussed health issue is erectile dysfunction (ED). Read More

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http://dx.doi.org/10.1111/hae.13707DOI Listing
March 2019
4 Reads

Outcomes indicators and processes in transitional care in adolescents with haemophilia: A Delphi survey of Canadian haemophilia care providers.

Haemophilia 2019 Mar 28;25(2):296-305. Epub 2019 Feb 28.

Division of Hematology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada.

Introduction: It is unclear which outcome indicators should be used to measure the success of haemophilia transition programs, and what are key elements of a haemophilia transition program to ensure success.

Aim: To establish by expert consensus a list of important and feasible outcome indicators of successful haemophilia transition, and a list of key elements of transition planning.

Methods: A modified two-stage Delphi survey was developed and disseminated among a panel of Canadian interdisciplinary haemophilia care providers. Read More

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http://dx.doi.org/10.1111/hae.13699DOI Listing
March 2019
3 Reads

Von Willebrand disease screening in women undergoing hysterectomy for heavy menstrual bleeding.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital/The Ohio State University, Columbus, Ohio.

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http://dx.doi.org/10.1111/hae.13709DOI Listing
February 2019
2 Reads

Genetic variants of VWF gene in type 2 von Willebrand disease.

Haemophilia 2019 Mar 28;25(2):e78-e85. Epub 2019 Feb 28.

Fundação HEMOMINAS, Minas Gerais, Brazil.

Introduction: von Willebrand disease (VWD) is the most common inherited bleeding disorder. Few studies have explored the molecular basis of type 2 VWD.

Aim: This study aimed to identify variants associated with type 2 VWD. Read More

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http://dx.doi.org/10.1111/hae.13714DOI Listing
March 2019
3 Reads

Safety of buccal infiltration local anaesthesia for dental procedures.

Haemophilia 2019 Mar 28;25(2):270-275. Epub 2019 Feb 28.

Dublin Dental University Hospital, School of Dental Science, Trinity College Dublin, Special care dentistry Division of Child and Public Dental Health, Dublin, Ireland.

Aim: To assess the safety of buccal infiltration local anaesthetic (LA) without additional factor replacement in patients with haemophilia (PWH) and association with clinical experience of the operator.

Methods: A consecutive sample of participants with mild, moderate and severe haemophilia scheduled to have dental treatment were recruited from a comprehensive care centre in Ireland. Infiltration LA was administered using a standard technique. Read More

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http://dx.doi.org/10.1111/hae.13695DOI Listing

Once-weekly prophylaxis with glycoPEGylated recombinant factor VIII (N8-GP) in severe haemophilia A: Safety and efficacy results from pathfinder 2 (randomized phase III trial).

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Division of Hematology, Oncology, and Blood and Marrow Transplantation, Department of Internal Medicine, University of Iowa Carver College of Medicine, Iowa City, Iowa.

Introduction: Turoctocog alfa pegol (N8-GP) is a site-specific, 40 kDa glycoPEGylated recombinant factor VIII (FVIII) product with an extended half-life. The comprehensive main phase of the pivotal pathfinder 2 trial showed N8-GP dosed every 4 days (Q4D) provided favourable safety and efficacy for preventing bleeds in 175 patients with haemophilia A.

Aim And Methods: We investigated the safety and efficacy of N8-GP prophylaxis when administered weekly (Q7D) for 24 weeks to patients with low bleeding rates in the pathfinder 2 extension trial. Read More

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http://dx.doi.org/10.1111/hae.13712DOI Listing
February 2019
3 Reads

Newborn screening for haemophilia: The views of families and adults living with haemophilia in the UK.

Haemophilia 2019 Mar 28;25(2):276-282. Epub 2019 Feb 28.

School of Life Sciences, University of Warwick, Coventry, UK.

Introduction: As genomic sequencing become more efficient and cost-effective, the number of conditions identified through newborn screening globally is set to dramatically increase. Haemophilia is a candidate condition; however, very little is known about the attitudes of the haemophilia community towards screening.

Aim: This study aimed to outline the perspectives of adults with haemophilia and their families towards newborn screening. Read More

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http://dx.doi.org/10.1111/hae.13706DOI Listing
March 2019
1 Read

Vocational experiences and career support opportunities among Canadian men with moderate and severe haemophilia.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Division of Hematology, Department of Medicine, St. Paul's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.

Aim: The purpose of this research was to provide haemophilia treatment centres (HTCs) with guidance for the potential development of appropriate and timely interventions related to employment and vocational counselling and supports.

Methods: A multi-method approach was employed, where initial focus groups (n = 13) and review of the literature were used to construct a structured survey instrument (n = 75).

Results: Focus group participants made choices about employment with keen awareness of how their bleeding disorder might limit them physically; they described the role of social networks in career choices; and they wrestled with issues of disclosure. Read More

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http://dx.doi.org/10.1111/hae.13701DOI Listing
February 2019
1 Read

Central venous access device catheter fragmentation at time of removal in four patients with haemophilia A.

Haemophilia 2019 Feb 28. Epub 2019 Feb 28.

Department of Pediatrics, University of Illinois College of Medicine, Peoria, Illinois.

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http://dx.doi.org/10.1111/hae.13708DOI Listing
February 2019
3 Reads