4,488 results match your criteria Haemophilia[Journal]


Concurrent homozygous sickle-cell disease and severe haemophilia A: Thromboelastography profiles.

Haemophilia 2019 Feb 14. Epub 2019 Feb 14.

Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Michigan.

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http://dx.doi.org/10.1111/hae.13692DOI Listing
February 2019

Low endoscopy bleeding risk in patients with congenital bleeding disorders.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Division of Gastroenterology, McGill University Health Centre, Montreal, Quebec, Canada.

Introduction: Haemophilia A and haemophilia B, von Willebrand disease (VWD), factor VII deficiency and factor XI deficiency are congenital bleeding disorders predisposing to bleeding during invasive procedures. The ageing population of people with congenital bleeding disorders will likely increasingly require gastrointestinal endoscopy. The bleeding risk postgastrointestinal endoscopy and optimal prophylactic treatment regimens are not well described. Read More

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http://dx.doi.org/10.1111/hae.13691DOI Listing
February 2019

Heat inactivation of extended half-life factor VIII concentrates.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Special Coagulation Laboratory, Division of Hematopathology, Department of Laboratory Medicine, Mayo Clinic, Rochester, Minnesota.

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http://dx.doi.org/10.1111/hae.13700DOI Listing
February 2019

The ongoing imperative for immune tolerance induction in inhibitor management.

Authors:
Louis M Aledort

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Icahn School of Medicine at Mount, New York, New York.

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http://dx.doi.org/10.1111/hae.13670DOI Listing
February 2019

Increasing optimal coagulation factor dosing in the paediatric emergency department: A quality improvement study.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Division of Hematology/Oncology/BMT, Nationwide Children's Hospital, Columbus, Ohio.

Introduction: Haemophilia is a disorder complicated by bleeding episodes that require emergent medical evaluation. Factor replacement dosing can present challenges for emergency department (ED) care.

Aims: We aimed to reduce out-of-range factor dosing in the ED. Read More

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http://dx.doi.org/10.1111/hae.13693DOI Listing
February 2019

Use of global assays to monitor emicizumab prophylactic therapy in patients with haemophilia A with inhibitors.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Division of Hematology/Oncology, VCU School of Medicine, Richmond, Virginia.

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http://dx.doi.org/10.1111/hae.13689DOI Listing
February 2019

Neuromuscular control during gait in people with haemophilic arthropathy.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Department of Human Movement Sciences, Faculty of Behavioural and Movement Sciences, Amsterdam Movement Sciences, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands.

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. Read More

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http://doi.wiley.com/10.1111/hae.13697
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http://dx.doi.org/10.1111/hae.13697DOI Listing
February 2019
1 Read

The impact of haemophilia on the social status and the health-related quality of life in adult Lebanese persons with haemophilia.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Hotel Dieu de France Hospital, Saint Joseph University, Beirut, Lebanon.

Introduction: Health-related quality of life (HRQoL) studies are increasingly needed to prevent and improve the medical care of persons with haemophilia (PWH).

Aim: We assessed the impact of haemophilia on HRQoL and social status of adult Lebanese PWH compared to a reference population.

Methods: In this case-control study, 60 severe and moderate PWH were compared to 112 healthy controls. Read More

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http://dx.doi.org/10.1111/hae.13694DOI Listing
February 2019

Haemophilia in Côte d'Ivoire (the Ivory Coast) in 2017: Extensive data collection as part of the World Federation of Hemophilia's twinning programme.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, Brussels, Belgium.

Introduction: In sub-Saharan African countries, research on haemophilia is limited. Since 2015, a partnership has been established through the World Federation of Hemophilia (WFH)'s twinning programme between the haemophilia treatment centre (HTC) of the Centre Hospitalier universitaire of Yopougon in Abidjan, Côte d'Ivoire, and the Cliniques universitaires Saint-Luc of Brussels, Belgium.

Aim: This study sought to collect accurate, and detailed demographic, clinical, and laboratory data on the whole identified Ivorian haemophilia population. Read More

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http://dx.doi.org/10.1111/hae.13682DOI Listing
February 2019

F8 IVS9+5G>A mutation causes moderate haemophilia A.

Haemophilia 2019 Feb 12. Epub 2019 Feb 12.

Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.

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http://dx.doi.org/10.1111/hae.13702DOI Listing
February 2019

Bleeding and safety outcomes in persons with haemophilia A without inhibitors: Results from a prospective non-interventional study in a real-world setting.

Haemophilia 2019 Feb 6. Epub 2019 Feb 6.

Haemophilia Comprehensive Care Centre, Faculty of Health Sciences, University of the Witwatersrand and NHLS, Parktown, Johannesburg, South Africa.

Introduction: Prospectively collected real-world data on bleeds, haemophilia treatment and safety in persons with haemophilia A (PwHA) without factor VIII (FVIII) inhibitors are limited. A global, non-interventional study (NIS; NCT02476942) prospectively collected real-world data in PwHA who were treated per local routine clinical practice.

Aim: Assess annualized bleeding rate (ABR), haemophilia treatment practices and adverse events (AEs) in adult/adolescent PwHA without inhibitors. Read More

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http://dx.doi.org/10.1111/hae.13655DOI Listing
February 2019
1 Read

Evaluation of a standardized protocol for thrombin generation using the calibrated automated thrombogram: A Nordic study.

Haemophilia 2019 Feb 4. Epub 2019 Feb 4.

Department of Translational Medicine & Centre for Thrombosis and Haemostasis, Lund University, Malmö, Sweden.

Introduction: The thrombin generation assay-calibrated automated thrombogram (TGA-CAT) method is used to measure the overall coagulation capacity in plasma. However, the method is still considered to be a research tool, mainly because of its' lack of standardization.

Aim: Our study aimed to further raise the standardization level for the TGA-CAT method by evaluating a detailed standardization protocol and three reference plasmas' (RP)s ability to normalize results. Read More

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http://dx.doi.org/10.1111/hae.13640DOI Listing
February 2019

Relapse pattern and long-term outcomes in subjects with acquired haemophilia A.

Haemophilia 2019 Jan 29. Epub 2019 Jan 29.

Division of Hematology-Oncology, Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, Quebec, Canada.

Introduction: Acquired haemophilia A (AHA) is a rare autoimmune bleeding disorder caused by neutralizing antibodies against factor VIII (FVIII). Despite significant initial morbidity and mortality, most patients achieve remission with immunosuppressive therapy.

Aim: Long-term follow-up data from the Quebec Reference Centre for Inhibitors (QRCI) were analysed to identify factors predictive of AHA relapse and the influence of relapse on survival. Read More

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http://doi.wiley.com/10.1111/hae.13685
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http://dx.doi.org/10.1111/hae.13685DOI Listing
January 2019
7 Reads

Tissue factor pathway inhibitor is the main determinant of thrombin generation in haemophilic patients.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

INSERM, U1059, SAINBIOSE, Université de Lyon, UJM-Saint-Etienne, Saint-Etienne, France.

The thrombin generation (TG) assay evaluates haemostatic balance, which is influenced by the levels of many coagulation factors and inhibitors. Our objective was to identify the determinant factors of TG in haemophilia A (HA) and haemophilia B (HB) patients and to compare them to those in healthy controls. Coagulation factor and inhibitor levels, and TG, were measured in platelet-poor plasma from 40 patients with HA, 32 patients with HB and 40 healthy subjects. Read More

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http://dx.doi.org/10.1111/hae.13679DOI Listing
January 2019
1 Read

Challenges in diagnosis of von Willebrand disease in the presence of combined mutations of different genes.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Centre d'hémostase pédiatrique et adulte du CHU Sainte-Justine, Montreal University, Montréal, Quebec, Canada.

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http://dx.doi.org/10.1111/hae.13686DOI Listing
January 2019

Sports participation and physical activity in adult Dutch and Swedish patients with severe haemophilia: A comparison between intermediate- and high-dose prophylaxis.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Introduction: Differences in treatment and outcome have been reported for persons with haemophilia (PWH) on intermediate-dose (Dutch) and high-dose (Swedish) prophylaxis, but the potential influence of sports participation has not been considered.

Aim: To compare sports participation and clinical outcome between adult Dutch and Swedish PWH.

Methods: Self-reported sports participation (type and frequency per week), physical functioning (SF-36 : 100-0), joint status (HJHS: 0-144), perceived limitations (HAL : 100-0) and physical activity (IPAQ) were recorded. Read More

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http://dx.doi.org/10.1111/hae.13683DOI Listing
January 2019

Parent's report on oral health-related quality of life of children with haemophilia.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Department of Paediatric Dentistry, Çukurova University, Sarıçam, Adana, Turkey.

Introduction: Among children with haemophilia and their caregivers; problems arising from the teeth and the surrounding tissues have an important role in the treatment of this disease and it affects the quality of life of children and their parents.

Aim: Aim of this study is to evaluate the oral health-related quality of life of children with haemophilia from the perspective of their parents.

Methods: Paediatric oral health-related quality of life (POQL) instrument was used in this cross-sectional study for quality of life measurement. Read More

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http://dx.doi.org/10.1111/hae.13678DOI Listing
January 2019

Expecting the unexpected: Acquired haemophilia A in a patient with homozygous factor V deficiency.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Department of Haematology, Manchester Royal Infirmary, Manchester University Hospitals NHS Foundation Trust, Manchester, UK.

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http://dx.doi.org/10.1111/hae.13669DOI Listing
January 2019
1 Read

Splicing analysis of 26 F8 nucleotide variations using a minigene assay.

Haemophilia 2019 Jan 28. Epub 2019 Jan 28.

Service d'Hématologie Biologique, Centre de Biologie et Pathologie Est, Hospices Civils de Lyon, Lyon, France.

Background: Classically, the study of splicing impact of variation located near the splice site is performed by both in silico and mRNA analysis. However, RNA sample was rarely available.

Objective: To characterize a panel of putative haemophilia A splicing variations. Read More

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http://dx.doi.org/10.1111/hae.13687DOI Listing
January 2019

Recommendations for the clinical interpretation of genetic variants and presentation of results to patients with inherited bleeding disorders. A UK Haemophilia Centre Doctors' Organisation Good Practice Paper.

Haemophilia 2019 Jan;25(1):116-126

Centre for Child & Adolescent Health, University of Bristol, Bristol, UK.

This paper sets out good practice for clinicians involved in interpreting variant reports for patients with inherited bleeding disorders. It is aimed primarily at doctors, nurses and allied healthcare professionals who may not have had specific training in genetic testing methodology or reporting. It deals with uncertainty in classification of variant pathogenicity and the handling of incidental findings. Read More

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http://dx.doi.org/10.1111/hae.13637DOI Listing
January 2019
1 Read

FIXing postinfusion monitoring: Assay experiences with N9-GP (nonacog beta pegol; Refixia ; Rebinyn ).

Haemophilia 2019 Jan;25(1):154-161

Novo Nordisk A/S, Måløv, Denmark.

N9-GP (nonacog beta pegol; Refixia ; Rebinyn , Novo Nordisk A/S, Bagsvaerd, Denmark) is a glycoPEGylated extended half-life recombinant factor IX (rFIX) that exhibits efficacy and potency comparable to unmodified FIX molecules in non-clinical models. Phase 3 clinical trials have confirmed the efficacy and tolerability of N9-GP for the prevention and on-demand treatment of bleeding episodes in patients with haemophilia B. Recent studies have shown that PEGylation affects clotting times in activated partial thromboplastin time (aPTT)-based one-stage activity assays due to interaction between the FIX molecule and certain aPTT reagents. Read More

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http://dx.doi.org/10.1111/hae.13671DOI Listing
January 2019
2 Reads

Coagulation factor VIII is vital for increasing global coagulation after physical exercise.

Haemophilia 2019 Jan 21. Epub 2019 Jan 21.

Department of Translational Medicine & Centre for Thrombosis and Haemostasis, Lund University, Lund, Sweden.

Background: In a previous smaller study, we found evidence of a diminished global coagulation capacity after maximal exercise in patients with severe haemophilia A (HA).

Aim: To validate these results, we repeated the study in a larger cohort. We also examined if the exercise-induced increased levels of von Willebrand factor (VWF) might prolong the effect of factor concentrate administered just before exercise. Read More

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http://dx.doi.org/10.1111/hae.13652DOI Listing
January 2019
2 Reads

Hindfoot malalignment in adults with haemophilic ankle arthropathy: The importance of early detection and orthotic treatment.

Haemophilia 2019 Jan 21. Epub 2019 Jan 21.

Department of Hematology, La Paz University Hospital-IdiPaz, Madrid, Spain.

Introduction: Haemophilic arthropathy (osteoarthritis secondary to haemophilia) of the ankle may result in painful hindfoot malalignment.

Purpose: To analyse hindfoot alignment in subjects with haemophilic arthropathy of the ankle and evaluate the response (improvement of pain, function and alignment) to the orthotic treatment prescribed in patients with malalignment.

Methods: The study included 163 patients with haemophilia, all of them over 16 years of age. Read More

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http://dx.doi.org/10.1111/hae.13680DOI Listing
January 2019
4 Reads

Moderate haemophilia in focus.

Authors:
Erik Berntorp

Haemophilia 2019 Jan 16. Epub 2019 Jan 16.

Centre for Thrombosis and Haemostasis, Lund University, Malmö, Sweden.

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http://doi.wiley.com/10.1111/hae.13677
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http://dx.doi.org/10.1111/hae.13677DOI Listing
January 2019
1 Read

The characteristics and spectrum of F9 mutations in Chinese sporadic haemophilia B pedigrees.

Haemophilia 2019 Jan 16. Epub 2019 Jan 16.

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

Introduction: Sporadic haemophilia B (HB) without obvious familial history poses challenges for genetic diagnosis and counselling.

Aim: To identify the F9 variants in sporadic HB patients and probe the origin of these de novo mutations.

Method: A total of 294 unrelated HB pedigrees sought genetic diagnosis were analysed in this single-centre study. Read More

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http://doi.wiley.com/10.1111/hae.13681
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http://dx.doi.org/10.1111/hae.13681DOI Listing
January 2019
3 Reads

Bleed volume of experimental knee haemarthrosis correlates with the subsequent degree of haemophilic arthropathy.

Haemophilia 2019 Jan 16. Epub 2019 Jan 16.

Global Drug Discovery, Novo Nordisk A/S, Maaloev, Denmark.

Background: Haemophilic arthropathy is the main morbidity of haemophilia. The individual pathological response to the same number of clinically evident joint bleeds is highly variable; thus, it remains unknown if certain joint bleeding characteristics are critical for the development of arthropathy.

Aim: To study the relation between bleed volume and subsequent development of arthropathy, we aimed to develop quantitative in vivo imaging of active joint bleeds in a mouse model of haemophilia. Read More

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http://doi.wiley.com/10.1111/hae.13672
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http://dx.doi.org/10.1111/hae.13672DOI Listing
January 2019
3 Reads

Incidence and risk factors for hepatocellular cancer in individuals with haemophilia: A National Inpatient Sample Study.

Haemophilia 2019 Jan 7. Epub 2019 Jan 7.

Center for Research on Health Care Data Center, University of Pittsburgh, Pittsburgh, Pennsylvania.

Introduction: Among haemophilic (H) men, hepatitis C virus (HCV) is the leading cause of liver disease and mortality, but demographics and risks of hepatocellular carcinoma (HCC) in H are not well known.

Methods: Adult discharges in H and non-haemophilic (NH) men, with and without HCC were identified in the National Inpatient Sample (NIS) between 1998 and 2014, using ICD-9 codes. Analyses included NIS-provided discharge-level weights to reflect national estimates. Read More

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http://dx.doi.org/10.1111/hae.13668DOI Listing
January 2019
7 Reads

The 1st WFH Gene Therapy Round Table: Understanding the landscape and challenges of gene therapy for haemophilia around the world.

Haemophilia 2019 Jan 3. Epub 2019 Jan 3.

World Federation of Hemophilia, Montreal, Québec, Canada.

In this first in a series of round table meetings, the 1st World Federation of Hemophilia Gene Therapy Round Table was convened to initiate a global dialogue on the expected challenges and opportunities that a disruptive therapy, such as gene therapy, will bring to the haemophilia community. Perspectives from key stakeholder groups, including healthcare professionals, regulators, payors, people with hemophilia and pharmaceutical industry representatives, were sought in the identification of the key issues we expect to face. Didactic presentations and open discussion covered the clinical development of gene therapy in haemophilia; regulatory perspectives of gene therapy; making informed decisions; accessibility, affordability and pricing of gene therapy; and ethical issues of gene therapy clinical trials. Read More

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http://dx.doi.org/10.1111/hae.13673DOI Listing
January 2019
1 Read

Treatment of acquired haemophilia with recombinant porcine factor VIII: A successful strategy in a patient with unstable angina.

Haemophilia 2019 Jan 3. Epub 2019 Jan 3.

Department of Haematology, Leeds Comprehensive Care Haemophilia Centre, St James's University Hospital, Leeds, UK.

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http://dx.doi.org/10.1111/hae.13644DOI Listing
January 2019
4 Reads

Successful coronary artery bypass graft surgery in severe congenital factor VII deficiency: Perioperative treatment with pd-factor VII concentrate.

Haemophilia 2018 Dec 27. Epub 2018 Dec 27.

Division of Haematology, A.O.U. Policlinico Vittorio Emanuele, Catania, Italy.

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http://dx.doi.org/10.1111/hae.13657DOI Listing
December 2018
1 Read

Accurate measurement of extended half-life and unmodified factor VIII low levels with one-stage FVIII assays is dependent on the matrix of calibration curves.

Haemophilia 2019 Jan 27;25(1):e19-e26. Epub 2018 Dec 27.

Clinical Department of Laboratory Medicine, University Hospitals of Leuven, Leuven, Belgium.

Introduction: The monitoring of factor VIII (FVIII) replacement therapy relies on the accurate measurement of FVIII activity over a large concentration range. However, unexplained overestimation of low FVIII levels has recently been reported with extended half-life recombinant FVIIIs.

Aim: The objective of this study was to confirm previous publications indicating that the reagents used to generate the calibration curves determine the accuracy of the measurement of low FVIII levels. Read More

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http://dx.doi.org/10.1111/hae.13656DOI Listing
January 2019
1 Read

Complete remission in a bleeding patient with idiopathic autoimmune factor X deficiency caused by non-neutralizing anti-factor X autoantibody.

Haemophilia 2018 Dec 20. Epub 2018 Dec 20.

Department of Molecular Patho-Biochemistry and Patho-Biology, Yamagata University School of Medicine, Yamagata, Japan.

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http://dx.doi.org/10.1111/hae.13675DOI Listing
December 2018

The invisible child: Sibling experiences of growing up with a brother with severe haemophilia-An interpretative phenomenological analysis.

Haemophilia 2019 Jan 18;25(1):84-91. Epub 2018 Dec 18.

University of Derby, Derby, UK.

Introduction: Haemophilia is an inherited chronic condition that causes bleeding in the joints and soft tissue. Healthy siblings growing up in the family of a person with haemophilia can be affected socially and psychologically.

Aim: To explore qualitatively the experiences of healthy siblings who grew up with a brother with severe haemophilia. Read More

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http://dx.doi.org/10.1111/hae.13659DOI Listing
January 2019

Mycophenolate mofetil as adjunctive therapy in acquired haemophilia A.

Haemophilia 2019 Jan 18;25(1):e59-e65. Epub 2018 Dec 18.

Arthur Bloom Haemophilia Centre, University Hospital of Wales, Cardiff, UK.

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http://doi.wiley.com/10.1111/hae.13658
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http://dx.doi.org/10.1111/hae.13658DOI Listing
January 2019
10 Reads

A single-centre study of management of pregnant women with von Willebrand disease.

Haemophilia 2019 Jan 18;25(1):e54-e57. Epub 2018 Dec 18.

Hospices Civils de Lyon, Unité d'Hémostase Clinique/Centre Régional de Traitement de l'Hémophilie, Hôpital Louis Pradel, Bron Cedex, France.

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http://dx.doi.org/10.1111/hae.13653DOI Listing
January 2019
1 Read

Potential limits of AAV-based gene therapy with the use of new transgenes expressing factor IX fusion proteins.

Haemophilia 2019 Jan 6;25(1):e11-e18. Epub 2018 Dec 6.

Research Department of Haematology, University of London Cancer Institute, London, UK.

Introduction: The variety of treatment for haemophilia B (HB) has recently improved with the emergence of both AAV-based gene therapy and bioengineered human factor IX (hFIX) molecules with prolonged half-life due to fusion to either albumin (Alb) or immunoglobulin Fc fragment (Fc).

Aim: Adeno-associated viral vectors (AAV) mediating expression of hFIX-Alb and hFIX-Fc fusion proteins was investigated for gene therapy of HB to explore if their extended half-life translates to higher plasma levels of FIX.

Methods: Single-stranded cross-packaged AAV2/8 vectors expressing hFIX-Alb, hFIX-Fc and hFIX were evaluated in vitro, and in mice. Read More

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http://dx.doi.org/10.1111/hae.13651DOI Listing
January 2019
2 Reads

Posturographic and ankle muscle activation characteristics in patients with haemophilia.

Haemophilia 2019 Jan 6;25(1):136-143. Epub 2018 Dec 6.

Department of Sports Medicine, University of Wuppertal, Wuppertal, Germany.

Introduction: The objective of this work was to examine the interrelations of posturographic and surface EMG (SEMG) characteristics of ankle muscles in patients with haemophilia while standing naturally.

Methods: Surface EMG of five bilaterally recorded ankle muscles was conducted in 24 patients with haemophilia (PwH, age: 42 [11] years, mean [SD], 22 A, 2 B, 21 severe, 3 moderate) with median (quartiles) WFH orthopaedic joint score of 30 (20/39) points and 24 non-haemophilic controls (Con, age: 42 [12]). Force plate (IBS) signals were captured simultaneously during bipedal stance with eyes open. Read More

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http://doi.wiley.com/10.1111/hae.13650
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http://dx.doi.org/10.1111/hae.13650DOI Listing
January 2019
14 Reads

Test-retest properties of the Patient Reported Outcomes, Burdens and Experiences (PROBE) questionnaire and its constituent domains.

Haemophilia 2019 Jan 6;25(1):75-83. Epub 2018 Dec 6.

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

Background: The Patient Reported Outcomes, Burdens and Experiences (PROBE) study aims to develop and validate questionnaire for assessing health status in patients with haemophilia and participants without bleeding disorders.

Objective: To investigate the test-retest properties of the PROBE questionnaire.

Methods: The PROBE questionnaire covers four domains and is comprised of 29 questions. Read More

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http://dx.doi.org/10.1111/hae.13649DOI Listing
January 2019
1 Read

A rare presentation of homozygous factor X deficiency in a pregnant patient: A case report and review of the literature.

Haemophilia 2019 Jan 3;25(1):e57-e59. Epub 2018 Dec 3.

Universitaire Ziekenhuizen Leuven, Leuven, Belgium.

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http://dx.doi.org/10.1111/hae.13654DOI Listing
January 2019
1 Read

A case of moderate haemophilia A with inhibitor, carrying the p.R1800H mutation, complicated with juvenile idiopathic arthritis.

Haemophilia 2019 Jan 3;25(1):e51-e54. Epub 2018 Dec 3.

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

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http://dx.doi.org/10.1111/hae.13648DOI Listing
January 2019
2 Reads

Over two decades of orthopaedic surgery in patients with inhibitors-Quantifying the complication of bleeding.

Haemophilia 2019 Jan 3;25(1):21-32. Epub 2018 Dec 3.

Department of Trauma & Orthopaedics, Royal Free Hospital, London, UK.

Patients with haemophilia who have developed inhibitors against factor VIII (FVIII) or factor IX present a significant concern to those surgeons who operate on them. The evidence base for bypassing agents such as recombinant factor VIIa and activated prothrombin complex concentrate has amassed over several decades. The literature is open to positive interpretation on the successful use of these agents in the treatment of inhibitor-positive patients. Read More

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http://doi.wiley.com/10.1111/hae.13647
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http://dx.doi.org/10.1111/hae.13647DOI Listing
January 2019
9 Reads

Analytical variation in factor VIII one-stage and chromogenic assays: Experiences from the ECAT external quality assessment programme.

Haemophilia 2019 Jan 29;25(1):162-169. Epub 2018 Nov 29.

Department of Haematology, Erasmus University Medical Centre Rotterdam, Rotterdam, The Netherlands.

Background: Both one-stage (OSA) and chromogenic substrate assays (CSA) are used to measure factor VIII (FVIII) activity. Factors explaining analytical variation in FVIII activity levels are still to be completely elucidated.

Aim: The aim of this study was to investigate and quantify the analytical variation in OSA and CSA. Read More

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http://dx.doi.org/10.1111/hae.13643DOI Listing
January 2019
1 Read

Adherence to prophylactic haemophilic treatment in young patients transitioning to adult care: A qualitative review.

Haemophilia 2018 Nov;24(6):862-872

Pfizer, Ballerup, Denmark.

Recombinant prophylactic treatment (PTX) has greatly improved morbidity, mortality and health-related quality of life (HRQoL) in patients with severe haemophilia. Yet, treatment adherence appears suboptimal in adolescents and young adults with haemophilia (YWH). Young patients experience major biopsychosocial changes challenging their adherence through the transition from parental to self-care, from paediatric to adult care. Read More

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http://doi.wiley.com/10.1111/hae.13621
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http://dx.doi.org/10.1111/hae.13621DOI Listing
November 2018
5 Reads

Thromboelastometry profiles after "in vitro" addition of a new plasma-derived factor V concentrate to whole blood from parahaemophilia patients.

Haemophilia 2019 Jan 23;25(1):e38-e42. Epub 2018 Nov 23.

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

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http://dx.doi.org/10.1111/hae.13636DOI Listing
January 2019
2 Reads

A novel approach to immune tolerance induction in haemophilia A with factor VIII inhibitor.

Haemophilia 2019 Jan 23;25(1):e48-e50. Epub 2018 Nov 23.

UC Davis, Sacramento, California.

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http://doi.wiley.com/10.1111/hae.13646
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http://dx.doi.org/10.1111/hae.13646DOI Listing
January 2019
10 Reads

Cardiovascular disease-related hospitalization and mortality among persons with von Willebrand disease: A nationwide register study in Sweden.

Haemophilia 2019 Jan 23;25(1):109-115. Epub 2018 Nov 23.

Department of Translational Medicine, Skåne University Hospital, Lund University, Malmö, Sweden.

Introduction: It has been hypothesized that persons with von Willebrand disease (VWD) may be protected against arterial thrombosis despite having atherosclerosis.

Aim: To calculate a nationwide estimate of the absolute and comparative burden of cardiovascular disease (CVD) hospitalization and mortality among persons with VWD using birthdate and sex-matched comparisons from the general population in Sweden.

Methods: Persons with VWD regardless of the type and severity, diagnosed by a medical doctor, who lived in Sweden for some time during the observation period 1987 through 2008 were included. Read More

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http://dx.doi.org/10.1111/hae.13642DOI Listing
January 2019
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