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Haemophilia: the Official Journal of the World Federation of Hemophilia

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https://www.readbyqxmd.com/read/30207633/utility-of-a-screening-tool-for-haemostatic-defects-in-a-multicentre-cohort-of-adolescents-with-heavy-menstrual-bleeding
#1
Ayesha Zia, Joseph Stanek, Myra Christian-Rancy, Sanjay P Ahuja, Stephanie Savelli, Sarah H O'Brien
INTRODUCTION: Heavy menstrual bleeding (HMB) may be expected for many adolescents after menarche. Accurate assessment of HMB, a key component in the diagnosis of a haemostatic defect (HD), is a well-recognized challenge. AIM: Our objective was to determine the diagnostic accuracy of an HMB-specific screening tool for HDs in adolescents with HMB, presenting to a secondary care setting. METHODS: Adolescents with HMB were evaluated for a HD at 4 US centres...
September 12, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30182454/uk-haemophilia-centre-doctors-organisation-guidance-on-the-use-of-extended-half-life-coagulation-factor-concentrates-in-routine-clinical-practice-report-of-a-meeting-on-their-adoption-by-belgian-haemophilia-treaters
#2
LETTER
Cedric Hermans, An Van Damme, Gerry Dolan, Philip Maes, Kathelijne Peerlinck
No abstract text is available yet for this article.
September 4, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30175490/orthotopic-liver-transplantation-for-haemophilia-a-may-not-always-lead-to-a-phenotypic-cure-of-haemophilia-a-a-case-report
#3
LETTER
Yuji Suzuki, Keisuke Kakisaka, Tomoko Matsumoto, Keiji Nogami, Hirokatsu Katagiri, Takeshi Takahara, Yasuhiro Takikawa
No abstract text is available yet for this article.
September 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30175488/identification-of-a-homozygous-missense-mutation-p-cys379gly-in-the-d1-domain-of-von-willebrand-factor-propeptide-in-a-family-with-type-2a-iic-von-willebrand-disease
#4
LETTER
Toshio Shigekiyo, Kengo Udaka, Etsuko Sekimoto, Hironobu Shibata, Shuji Ozaki, Yukio Higuchi, Masanori Matsumoto
No abstract text is available yet for this article.
September 3, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30152566/congenital-coagulation-factor-x-deficiency-genetic-analysis-of-five-patients-and-functional-characterization-of-mutant-factor-x-proteins
#5
Satomi Nagaya, Masashi Akiyama, Morika Murakami, Akiko Sekiya, Hidesaku Asakura, Eriko Morishita
Congenital factor X (FX) deficiency is a rare bleeding disorder that is inherited as an autosomal recessive trait. In this study, a genetic analysis of the FX gene was performed in five families with this disorder. Four heterozygous mutations [p.Gly154Arg, p.Val236Met, p.Gly263Val and p.Arg387Cys] and one pair of compound heterozygous FX gene mutations consisting of p.Gly406Ser and p.Val424Phe were identified. Mutant FX proteins containing the identified amino acid substitutions were also expressed in cultured cells...
August 28, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30144219/challenges-in-diagnosis-and-management-of-acquired-factor-xiii-fxiii-inhibitors
#6
LETTER
Joan D Beckman, Raj S Kasthuri, Alisa S Wolberg, Alice D Ma
No abstract text is available yet for this article.
August 24, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30144218/positioning-extended-half-life-concentrates-for-future-use-a-practical-proposal
#7
LETTER
Angelique Nederlof, Ron A A Mathôt, Frank W G Leebeek, Karin Fijnvandraat, Kathelijn Fischer, Marjon H Cnossen
No abstract text is available yet for this article.
August 24, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30144214/haemophilia-care-in-latin-america-assessment-and-perspectives
#8
Apsara Boadas, Margareth C Ozelo, Maria Solano, Adolfina Berges, Arlette Ruiz-Saez, Adriana Linares, José Luis Lamas, Rafael Aparicio, Luis Aversa, Alejandra Baques, Armando Estrada, Misael Herrejon, Ana Mancia, Rosa Nieves-Paulino, Ieda Pinto, Alessandra Prezoti, Verónica Soto, Dani Ugalde
INTRODUCTION: The study is the first application of the Principles of Haemophilia Care for Europe (PHCE) in other regions of the world, specifically in Latin America. OBJECTIVE: To identify strengths in the care of haemophilia, and the aspects that should be improved. METHODS: The information was obtained through a questionnaire designed according to the PHCE and answered by specialists in mid-2016. The countries included were as follows: Argentina, Brazil, Chile, Colombia, Costa Rica, El Salvador, Mexico, Panama, Dominican Republic and Venezuela...
August 24, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30117632/efficacy-and-safety-of-low-dose-prophylaxis-of-highly-purified-plasma-derived-factor-viii-concentrate-produced-by-the-national-blood-centre-thai-red-cross-society
#9
LETTER
Ampaiwan Chuansumrit, Darintr Sosothikul, Rungrote Natesirinilkul, Yujinda Lektrakul, Ubonwon Charoonruangrit
No abstract text is available yet for this article.
August 17, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30112863/rate-and-appropriateness-of-polypharmacy-in-older-patients-with-hemophilia-compared-with-age-matched-controls
#10
Pier Mannuccio Mannucci, Alessandro Nobili, Emanuela Marchesini, Emily Oliovecchio, Laura Cortesi, Antonio Coppola, Elena Santagostino, Paolo Radossi, Giancarlo Castaman, Lelia Valdrè, Cristina Santoro, Annarita Tagliaferri, Cosimo Ettorre, Ezio Zanon, Giovanni Barillari, Isabella Cantori, Teresa Maria Caimi, Gianluca Sottilotta, Flora Peyvandi, Alfonso Iorio
BACKGROUND: In older people, multiple chronic ailments lead to the intake of multiple medications (polypharmacy) that carry a number of negative consequences (adverse events, prescription and intake errors, poor adherence, higher mortality). Because ageing patients with haemophilia (PWHs) may be particularly at risk due to their pre-existing multiple comorbidities (arthropathy, liver disease), we chose to analyse the pattern of chronic drug intake in a cohort of PWHs aged 60 years or more...
August 16, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30112856/evaluating-the-thrombin-generation-profiles-of-four-different-rfviii-products-in-fviii-deficient-plasma-using-fixa-and-fxia-activation
#11
Donald F Brophy, Erika J Martin, Marisa Ninivaggi, Bassem M Mohammed, John C Barrett, Janice Kuhn, Melinda E Nolte, Emily K Waters, Mirella Ezban
INTRODUCTION: The thrombin generation assay (TGA) can be used to monitor factor replacement therapy in patients with haemophilia. The TGA assay is typically performed using tissue factor as the reaction activator; however, activating with FIXa or FXIa can enhance assay sensitivity when FVIII < 1%. AIMS: To evaluate the sensitivity of the TGA when FIXa (5 nmol/L) and FXIa (0.22 nmol/L) are used to activate the assay in platelet-poor plasma and to compare these data to the one-stage and chromogenic assays...
August 16, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30112811/pain-assessment-and-management-in-haemophilia-a-survey-among-italian-patients-and-specialist-physicians
#12
Annarita Tagliaferri, Massimo Franchini, Gianna F Rivolta, Stefania Farace, Gabriele Quintavalle, Antonio Coppola
INTRODUCTION: Persons with haemophilia (PWH) experience recurrent joint bleeding which leads from early synovitis to irreversible joint damage. Pain strongly affects patients' quality of life, as PWH suffer from acute pain associated with haemarthroses and chronic pain due to arthritic and degenerative complications. AIM: To investigate pain issues among PWH and their treaters in Italy. METHODS: Persons with haemophilia and specialist physicians responded to a survey focused on pain characteristics, assessment, and management by phone call and online, respectively...
August 16, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30112783/retrospective-review-of-acquired-haemophilia-a-from-the-largest-canadian-haemophilia-treatment-centre
#13
LETTER
Jai P Jayakar, Natalya O'Neill, Marie Yan, Rosane Nisenbaum, Marie B Garvey, Jerome Teitel, Michelle Sholzberg
No abstract text is available yet for this article.
August 16, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30102018/effectiveness-of-a-comprehensive-educational-programme-for-accredited-social-health-activists-ashas-to-identify-individuals-in-the-udupi-district-with-bleeding-disorders-a-community-based-survey
#14
S Badagabettu, D M Nayak, A Kurien, V G Kamath, A Kamath, A George
INTRODUCTION: The awareness and knowledge on bleeding disorders is generally poor among the rural population. Accredited Social Health Activists (ASHAs) serve as the facilitators between the rural community and the health care system. Training of ASHAs in screening of rural population for early identification of bleeding disorders can enable prompt referral, timely detection and management of bleeding disorders. AIM: The aim of the study was to evaluate the effectiveness of an ASHA training programme for identification of suspected bleeding disorder cases...
August 13, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30091822/real-world-comparative-analysis-of-bleeding-complications-and-health-related-quality-of-life-in-patients-with-haemophilia-a-and-haemophilia-b
#15
Jason Booth, Abiola Oladapo, Shaun Walsh, Jamie O'Hara, Liz Carroll, Daniel-Anibal Garcia Diego, Brian O'Mahony
INTRODUCTION: Clinical severity and impact of haemophilia on quality of life have been generally considered to be lower for haemophilia B (HB) compared with haemophilia A (HA) patients. AIMS: To compare annual bleeding rate (ABR), target joint development and health-related quality of life (HRQoL) between adult (≥18 years) severe HA and HB patients using recent data from the Cost of Haemophilia in Europe: a Socioeconomic Survey (CHESS) study. METHODS: Multivariate generalized linear models (GLM) were constructed to assess the relationship between haemophilia type, ABR, HRQoL (derived from EQ-5D index scores) and the presence of target joints while controlling for covariates...
August 9, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30088696/review-of-molecular-mechanisms-at-distal-xq28-leading-to-balanced-or-unbalanced-genomic-rearrangements-and-their-phenotypic-impacts-on-hemophilia
#16
REVIEW
N Lannoy, C Hermans
The distal Xq28 region is very gene-rich, comprising a relatively large number of low-copy repeats (LCRs) predisposing to genomic rearrangements. The best-known rearrangement at this locus is the F8 intron 22 inversion, responsible for up to 45% of severe hemophilia A (HA) cases. An additional inversion of intron 1 of F8 has more recently been described, affecting 2%-5% of patients with severe HA. These "balanced" rearrangements are mediated by intrachromosomal homologous recombination between inversely oriented LCRs located in intron 1 or 22 and other extragenic copies positioned more telomerically outside the F8 gene...
August 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30088693/impact-of-target-joint-and-fviii-inhibitor-%C3%AE-n-bone-properties-in-children-with-haemophilia-a-a-peripheral-quantitative-computed-tomography-study
#17
Panagiota Xafaki, Alexia Balanika, Helen Pergantou, Olympia Papakonstantinou, Helen Platokouki
BACKGROUND: Haemophilic children are prone to low bone mass accrual. OBJECTIVE: To assess bone properties in haemophilic children, using peripheral quantitative computed tomography (pQCT) and to correlate findings with clinical data. SUBJECTS/METHODS: Peripheral quantitative computed tomography scan of both radii and tibiae were performed in 31 haemophilic A children (severe 24, mean age 11.2 years). Seven subjects had a history of inhibitors...
August 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30088692/value-of-prophylaxis-vs-on-demand-treatment-application-of-a-value-framework-in-hemophilia
#18
Diane Nugent, Brian O'Mahony, Gerry Dolan
INTRODUCTION: Therapeutic advances over the past 30 years have led to longer life expectancy and improved quality of life (QOL) for persons with hemophilia. Access to innovative therapy may be compromised if treatment decisions are driven solely by cost. New strategies are needed to assess true therapeutic values, along with financial cost, as physicians, policymakers, payers and manufacturers work together to improve patient care. AIM: To provide an evidence-based assessment of the value of prophylaxis vs on-demand therapy for hemophilia, based on a widely recognized three-tiered value framework approach for assessing a range of therapeutic interventions...
August 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30088320/perioperative-haemostasis-in-children-with-haemophilia-and-inhibitors-during-central-venous-catheter-surgery-the-karolinska-model
#19
LETTER
Helena Hägglöf, Maria Magnusson, Pia Petrini, Tony Frisk, Susanna Ranta
No abstract text is available yet for this article.
August 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/30088316/application-of-optimized-nursing-process-to-perioperative-patients-with-haemophilic-pseudotumours
#20
LETTER
Yaping Chen, Yu He, Xi Zhou, Yuan Xu, Baozhong Zhang, Yong Liu, Xisheng Weng
No abstract text is available yet for this article.
August 8, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
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