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Journals Haemophilia : the Official Jou...

Haemophilia : the Official Journal of the World Federation of Hemophilia

https://read.qxmd.com/read/38600799/genetic-predictors-for-fviii-inhibitors-formation-in-a-greek-population-of-children-with-severe-haemophilia-a-exclusively-treated-with-recombinant-concentrates
#1
LETTER
Aikaterini Michalopoulou, Athina Dettoraki, Zoey Kapsimali, Kleopatra Spanou, Emmanouil Liatsis, Marina Economou, Niki Constantinidou, Helen Pergantou
No abstract text is available yet for this article.
April 10, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38600680/structural-alterations-and-pain-perception-at-the-ankle-joint-in-patients-with-haemophilia
#2
JOURNAL ARTICLE
Alexander Schmidt, Michael Sigl-Kraetzig, Heinrich Richter, Tim Vogler, Fabian Tomschi, Thomas Hilberg
BACKGROUND: Patients with haemophilia (PwH) suffer from chronic pain due to joint alterations induced by recurring haemorrhage. OBJECTIVES: This study aimed to investigate the relationship between structural alterations and pain perception at the ankle joint in PwH. PATIENTS/METHODS: Ankle joints of 79 PwH and 57 healthy controls (Con) underwent ultrasound examination (US) and assessment of pain sensitivity via pressure pain thresholds (PPT)...
April 10, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38578720/bleeding-control-improves-after-switching-to-emicizumab-real-world-experience-of-177-children-in-the-pednet-registry
#3
JOURNAL ARTICLE
Konrad van der Zwet, Marloes de Kovel, Jayashree Motwani, Chris van Geet, Beatrice Nolan, Heidi Glosli, Carmen Escuriola Ettingshausen, Christoph Königs, Gili Kenet, Kathelijn Fischer
INTRODUCTION: Despite the rapid uptake of emicizumab in the paediatric haemophilia A (HA) population, real-world data on the safety and efficacy is limited. AIM: To report on bleeding and safety in paediatric patients receiving emicizumab prophylaxis. METHODS: Data were extracted from the multicentre prospective observational PedNet Registry (NCT02979119). Children with haemophilia A, and ≥50 FVIII exposures or inhibitors present receiving emicizumab maintenance therapy were analysed...
April 5, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38578694/haemophilic-arthropathy-diagnosis-management-and-aging-patient-considerations
#4
JOURNAL ARTICLE
Nihal Bakeer, Walid Saied, Antonio Gavrilovski, Cindy Bailey
Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores...
April 5, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38578181/validity-and-reliability-test-of-the-indonesian-version-of-the-pedhalshort-questionnaire
#5
JOURNAL ARTICLE
Novie Amelia Chozie, Dewi Rosariah Ayu, Bernie Endyarni Medise, Putri Maharani Tristanita Marsubrin, Teny Tjitra Sari, Irene Yuniar, Luh Karunia Wahyuni
INTRODUCTION: The PedHAL questionnaire is employed in measuring the activities of haemophilia children. The PedHALshort was developed in 2022 to determine the most relevant item for the assessment. Haemo-QoL questionnaire assesses the quality of life of haemophilia children. AIM: Determine the validity and reliability of the PedHALshort Indonesia language version compared to the Haemo-QoL in the Indonesian population. METHODS: A cross-sectional study was conducted in Jakarta, Indonesia...
April 5, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38578179/mutational-landscape-inhibitor-development-and-health-care-burden-in-non-severe-haemophilia-a-a-single-centre-australian-experience
#6
JOURNAL ARTICLE
Radha Ramanan, Natalie Evans, Zane Kaplan, James D McFadyen, Huyen A Tran
AIM: To characterise non-severe haemophilia A (HA) patients enrolled on the Australian Bleeding Disorders Registry (ABDR) treated through a state-wide Haemophilia Treatment Centre (HTC) with respect to their mutational profile, inhibitor risk and health-care burden. METHOD: We conducted a single-centre observational study of all non-severe HA patients treated at the Alfred Health HTC registered on the ABDR as of the 26th July 2023. Data were extracted from the ABDR and electronic medical record (EMR) regarding demographics, severity, genetic testing, treatment, inhibitors, bleeding events and procedures...
April 5, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38575549/primary-prophylaxis-implementation-and-long-term-joint-outcomes-in-swedish-haemophilia-a-patients
#7
JOURNAL ARTICLE
Alexandros Arvanitakis, Caroline Jepsen, Nadine G Andersson, Fariba Baghaei, Jan Astermark
INTRODUCTION: Primary prophylaxis is the gold standard in severe haemophilia A (SHA) but time to escalate the prophylaxis regimen varies. AIM: Assess prophylaxis implementation and long-term joint health outcomes in SHA with primary prophylaxis. METHODS: Adult male patients born after 1980, with SHA on primary prophylaxis, started before the age of 3 years and second joint bleed, and no history of FVIII inhibitors, were enrolled. Repeated joint-health examinations were performed with HJHS or HEAD-US; VERITAS-PRO assessed adherence...
April 4, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38575529/is-pharmacological-thromboprophylaxis-necessary-in-persons-with-haemophilia-undergoing-major-orthopaedic-surgery
#8
LETTER
Silvia Linari, Lisa Pieri, Christian Carulli, Francesco Demartis, Rajmonda Fjerza, Domenico Prisco, Giancarlo Castaman
No abstract text is available yet for this article.
April 4, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38575526/predicting-joint-involvement-through-tailored-prophylaxis-in-severe-haemophilia-a-is-it-possible
#9
JOURNAL ARTICLE
Maria Del Mar Meijon-Ortigueira, Maria Teresa Alvarez-Roman, Hortensia De La Corte, Nora Butta, Victor Jimenez-Yuste
INTRODUCTION: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. However, both have strengths and weaknesses, and their characteristics need to be integrated to optimize treatment appropriately. In this paper, we present a model that considers together the characteristics of prophylaxis and the relevance of each...
April 4, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38575518/unresolved-hemostasis-issues-in-haemophilia
#10
JOURNAL ARTICLE
Robert F Sidonio, John W Weisel, Darrel Stafford
Despite rapid technological advancement in factor and nonfactor products in the prevention and treatment of bleeding in haemophilia patients, it is imperative that we acknowledge gaps in our understanding of how hemostasis is achieved. The authors will briefly review three unresolved issues in persons with haemophilia (PwH) focusing on the forgotten function that red blood cells play in hemostasis, the critical role of extravascular (outside circulation) FIX in hemostasis in the context of unmodified and extended half-life FIX products and finally on the role that skeletal muscle myosin plays in prothrombinase assembly and subsequent thrombin generation that could mitigate breakthrough muscle hematomas...
April 4, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38571362/the-importance-and-evolution-of-bleeding-disorder-registries
#11
JOURNAL ARTICLE
Huyen Tran, Renchi Yang, Kathelijn Fischer, Michael Makris, Barbara A Konkle
Registries are excellent sources of data to address questions that are typically not evaluated in randomized clinical trials, including natural history, disease prevalence, treatment approaches and adverse events, and models of care. Global and regional registries can provide data to identify differences in outcomes and in haemophilia care between countries, economic settings, and regions, while facilitating research and data sharing. In this manuscript, we highlight five bleeding disorder registries: Country registries from Australia and China, Paediatric Network on Haemophilia Management (PedNet) data on children who have received emicizumab, data from the European Haemophilia Safety Surveillance (EUHASS) system, and data on women and girls with haemophilia from the World Federation of Haemophilia (WFH) registries...
April 3, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38571337/dental-management-of-people-with-complex-or-rare-inherited-bleeding-disorders
#12
JOURNAL ARTICLE
Lochana Nanayakkara, Norjehan Yahaya, Miryam Parreira, Branislav Bajkin
Advances in haematological therapies for people with complex or rare inherited bleeding disorders (IBD) have resulted in them living longer, retaining their natural teeth with greater expectations of function and aesthetics. Dental management strategies need to evolve to meet these challenges. Utilising low level laser diode therapy to reduce pre-operative inflammation to reduce the intraoperative and postoperative burden on haemostasis is described in a case series of 12 patients. For these individuals who previously required further medical management to support haemostasis or experienced such prolonged haemorrhage sufficient to warrant hospital admission, haemostasis was achieved in the dental surgery such that they were able to return home with no further medical intervention or overnight stays...
April 3, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38562115/-not-available
#13
JOURNAL ARTICLE
Andreas Tiede, Sophie Susen, Ton Lisman
Acquired bleeding disorders can develop in previously healthy people irrespective of age or gender but are particularly common in patients with certain underlying conditions. Here, we review recent advances in the management of acquired haemophilia A (AHA), acquired von Willebrand syndrome (AVWS), and patients with hemostatic abnormalities due to chronic liver disease (CLD). Patients with AHA can now benefit from prophylaxis with emicizumab, a therapeutic antibody that mimics the function of activated coagulation factor VIII...
April 2, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38561627/a-novel-fgg-missense-variant-associated-with-fibrinogen-storage-disease-in-a-large-family-from-quebec
#14
LETTER
Marie-Claude Pelland-Marcotte, Adelina-Teona Avram, Hélène Néron, Christine Demers, Julie Castilloux, Julie Gauthier, Marguerite Neerman-Arbez, Alessandro Casini, Georges-Etienne Rivard
No abstract text is available yet for this article.
April 1, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38549492/riding-the-wave-of-change-providing-solid-ground-to-support-nursing-with-patient-transitions-to-novel-haemophilia-therapies
#15
JOURNAL ARTICLE
Erica Crilly, Cathy Harrison, Jennifer Maahs, Marlene Beijlevelt, Brian Ramsay, Cyrus Githinji, Marcela Sisdelli, Anjalin Dsouza
INTRODUCTION: Haemophilia nursing practice has experienced a shift in the past decade, as the historic chief focus on factor infusions shifted to extended half-life products, bispecific antibody therapies and other non-replacement therapies. This evolution has driven a need for changes in nursing practice in many haemophilia treatment centres. AIM: This article intends to provide insights to the haemophilia nurse to champion practice changes at their haemophilia treatment centres...
March 29, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38549463/classification-of-recombinant-factor-viii-products-and-implications-for-clinical-practice-a-systematic-literature-review
#16
REVIEW
Cihan Ay, Mariasanta Napolitano, Abel Hassoun, Radovan Tomic, Cedric Martin, Wilfried Seifert, Karen Pinachyan, Johannes Oldenburg
INTRODUCTION: Consensus over the definition of recombinant factor VIII (rFVIII) product classification in haemophilia A is lacking. rFVIII products are often classified as standard half-life (SHL) or extended half-life (EHL); despite this, no universally accepted definition currently exists. One proposed definition includes half-life, area under the curve, and technology designed to extend half-life; however, the International Society on Thrombosis and Haemostasis defines activity over time as the most intuitive information for building treatment regimens and the World Federation of Hemophilia describes rFVIII product classification in terms of infusion frequency...
March 29, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38545868/the-good-the-bad-and-the-ugly-of-pain-in-haemophilia-recent-evidence-on-the-epidemiology-molecular-mechanisms-and-knowledge-gaps-preventing-optimal-treatment
#17
REVIEW
Silvia Benemei, Consalvo Mattia, Matteo Nicola Dario Di Minno
INTRODUCTION: Haemophilia is an inherited, X-linked blood clotting disorder caused by the deficiency of coagulation factors VIII (FVIII, haemophilia A) or IX (FIX, haemophilia B). Spontaneous bleeds are common in severe forms of haemophilia and can also occur in moderate and mild haemophilia. Severe or repeated bleeding at a joint can evolve into chronic haemophilic arthropathy, with functional damage of the joint, disability, and intense chronic articular pain. Nonetheless, acute and chronic pain may emerge due to secondary conditions related to bleedings...
March 28, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38539060/haemophilia-in-the-era-of-novel-therapies-where-do-inhibitors-feature-in-the-new-landscape
#18
JOURNAL ARTICLE
Shannon L Meeks, Karen L Zimowski
INTRODUCTION: The advent of therapeutic recombinant factor VIII (FVIII) and factor IX (FIX) protein infusions revolutionized the care of persons with haemophilia in the 1990s. It kicked off an era with the increasing use of prophylactic factor infusions for patients and transformed conversations around the ideal trough activity levels as well as the ultimate goals in tailored, individualized care. Our knowledge surrounding the immunologic basis of inhibitor development and treatment derives from a time when patients were receiving frequent factor infusions and focused on immune tolerance induction following inhibitor development...
March 27, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38539058/challenges-in-ageing-persons-with-haemophilia
#19
JOURNAL ARTICLE
Michael Makris, Riitta Lassila, Megan Kennedy
As treatments for individuals with inherited bleeding disorders improve, life expectancy increases and is approaching that of the normal population. Concomitant with this we are now seeing the problems of ageing in the bleeding disorder population. Although the clear-cut association between low clotting factor levels and risk of bleeding is well recognised, a relationship between high levels, some non-factor therapies and thrombotic risk also exists. The management of thrombosis in persons with inherited bleeding disorders is complex but manageable with modern treatments and collaboration in decision making between health care professionals and patients...
March 27, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://read.qxmd.com/read/38532560/women-and-girls-with-inherited-bleeding-disorders-focus-on-haemophilia-carriers-and-heavy-menstrual-bleeding
#20
JOURNAL ARTICLE
Cedric Hermans, Jill M Johnsen, Nicola Curry
Raising awareness and improving recognition, accurate classification, and enhanced access to new treatments represent current key challenges for carriers of haemophilia. Women and girls carrying genes for haemophilia often experience significant bleeding and/or low factor levels. The bleeding associated with female haemophilia is frequently overlooked, has a weak correlation with factor levels, and manifests differently than in males, with heavy menstrual bleeding being a predominant symptom. Recent changes in terminology now allow the diagnosis of haemophilia in females with low factor levels and differentiate between symptomatic and asymptomatic carriers of the gene...
March 26, 2024: Haemophilia: the Official Journal of the World Federation of Hemophilia
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