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Efficacy of Wilate Prophylaxis in Reducing Nosebleeds in Patients with Severe VWD - A Post-hoc Analysis of the WIL-31 Study.
Clin Appl Thromb Hemost
December 2024
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
Background: Prophylaxis with a von Willebrand factor (VWF) concentrate is recommended in von Willebrand disease (VWD) patients with a history of frequent and severe bleeds. Despite nosebleeds being a frequent manifestation of VWD, few studies have investigated the efficacy of factor prophylaxis in preventing nosebleeds in patients with severe VWD.
Methods: This post-hoc analysis of a prospective, 12-month, phase 3 study assessed the efficacy of wilate in the prevention of nosebleeds in 33 patients aged ≥6 years with severe type 1, type 2 or type 3 VWD.
Background: Glanzmann thrombasthenia (GT) is a rare disease with an autosomal recessive inheritance pattern. This disorder is not so uncommonly encountered in routine clinical practice and laboratory settings in Pakistan let alone in the rest of the world. To describe the bleeding phenotype of GT and treatment outcomes in over one hundred patients in north Pakistan.
View Article and Find Full Text PDFLooking Beyond Thrombocytopenia for Bleeding in a Case of Dengue Fever: From Dengue to Hemophilia C.
Cureus
October 2024
Internal Medicine, Max Smart Super Speciality Hospital, New Delhi, IND.
Article Synopsis
- * A case study details a young man in his early twenties who was hospitalized for Dengue, experiencing dark stools (melena) and vomiting blood.
- * Despite his platelet count returning to normal, his bleeding continued due to a combination of a bleeding ulcer in the gastroesophageal junction and a deficiency in clotting factor XI, indicating he had Hemophilia C.
Case Report: Allelic and biallelic variants in cause factor XI deficiency.
Front Cardiovasc Med
November 2024
Department of Obstetrics and Gynecology, The First Affiliated Hospital of Jinan University, Guangzhou, China.
Factor XI deficiency is a rare inherited coagulation disorder with an estimated prevalence of affecting 1 in 1 million. It is characterized by mild and variable bleeding phenotypes, including bruises, nosebleeds, hematuria, and postpartum hemorrhage. It can be caused by either allelic or biallelic variants in ().
View Article and Find Full Text PDFAssessment of joint health in females with haemophilia: The carriers ultrasound project (CUP) study.
Haemophilia
November 2024
Division of Pediatric Hematology/Oncology, Department of Pediatrics, University of Miami-Miller School of Medicine, Miami, Florida, USA.
Introduction: The needs of haemophilia carriers (HC) have been historically overlooked. It is now recognised that HC manifests bleeding symptoms, including haemarthrosis. The natural history of joint health in HC is not yet defined.
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