Browsing by Author "Othman, Maha (7006122614)"

Background: Management of women with type 2B von Willebrand disease (VWD) during pregnancy is challenging because of dysfunctional von Willebrand factor (VWF) and the complexity resulting from discrepant VWF/factor VIII (VWF/FVIII) levels, impaired platelet-dependent VWF activity, progressive thrombocytopenia, and risks associated with the use of desmopressin. There is a lack of high-quality evidence to support clinical decision making. Objectives: In this study, we examined the current diagnostic and management approaches and outcomes in women with VWD during pregnancy. Methods: Data were collected via 3 avenues: literature review, an international registry, and an international survey on physicians’ practices for the management of pregnancy in women with VWD. The registry and survey were supported by the International Society on Thrombosis and Haemostasis. Results: Data on clinical and laboratory features, management and bleeding complications, and pregnancy outcomes of a total of 55 pregnancies from 49 women across the globe (literature: 35, registry: 20) and data reported by 112 physicians were analyzed. We describe the largest dataset on pregnancies in women with type 2B VWD available to date. The data highlight the following key issues: a) bleeding complications remain a concern in these patients, b) the target safe VWF level and the ideal monitoring approach are unknown, c) there is a wide range of hemostatic management practices in the type and timing of treatment, and d) physicians have diverse views on the mode of delivery and use of neuraxial anesthesia. Conclusion: We conclude that an international consensus and guidance are critically required for better care and improved outcomes in this patient cohort. © 2022

Jean St-Louis MD, FRCPC 1,2| Audrey Abad BSc 3| Sharon Funk DPT 4|Merlyn Tilak MPT 5| Stephen Classey PT 6| Nichan Zourikian BSc, PT 7|Paul McLaughlin PT, MSc, MCSP 8| Sébastien Lobet PT, PhD 9|Grace Hernandez PT10| Stacie Akins PT11| Anna J. Wells PT, MSc, MCSP, SRP12|Marilyn Manco-Johnson MD4| Judy John MD5| Steve Austin MBBS BMedSci FRACP FRCPA6| Pratima Chowdary MD8| Cedric Hermans MD9| Diane Nugent MD10|Nihal Bakeer MD11| Sarah Mangles MD, MBBS, MD (Res), FRCP, FRCPath12|Pamela Hilliard BSc, PT3| Victor S. Blanchette MB, BChir (Cantab), FRCPC13,14|Brian M. Feldman MD, MSc, FRCPC15,161Department of Hematology, CHU Sainte-Justine and Hôpital Maisonneuve-Rosemont, Montréal, Québec, Canada2Department of Medicine, Université de Montréal, Montréal, Québec, Canada3Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada4Hemophilia and Thrombosis Center, University of Colorado, Denver, Colorado, USA5Department of Physical Medicine & Rehabilitation, Christian Medical College, Vellore, India6Haemostasis and Thrombosis Centre, St Thomas’ Hospital, London, UK7Pediatric/Adult Comprehensive Hemostasis Center, CHU Sainte-Justine, Montréal, Québec, Canada8Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Hospital, London, UK9Haemostasis and Thrombosis Unit, Division of Haematology, Cliniques Universitaires Saint- Luc, Brussels, Belgium10The Center for Inherited Blood Disorders (CIBD), Orange County, California, USA11Indiana Hemophilia & Thrombosis Center, Indianapolis, Indiana, USA12Haemophilia, Haemostasis & Thrombosis Centre, Hampshire Hospitals NHS Foundation Trust, Basingstoke, UK13Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada14Department of Pediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada15Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada16Department of Pediatrics, Faculty of Medicine, Institute of Health Policy, Management and Evaluation, The Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada The authors regret a technical error in the graph-generating software resulted in an inaccurate visual representation for the moderate group in Figure 1. The bar graphs were not representative of the actual data obtained in the moderate hemophilia group. The correct bar graphs for the moderate group should display a Median HJHS total score of 27.5 in those 30 to 40 years; 29.5 in those 41 to 50 years, and 29.0 in those > 50 years. This corrected figure does not alter the original data, analysis, or conclusions.[Formula presented] The authors would like to apologise for any inconvenience caused. © 2023

The authors regret the affiliation of Clinical Pathology Department, Faculty of Medicine, Mansoura University, Egypt was omitted for Maha Othman. This has been corrected and added as below. The authors would like to apologise for any inconvenience caused. © 2023

Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women’s Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard. © 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.