Hemophiliac patients develop disorders of the immune status as a result of transfusion therapy: a decrease in the number of T-lymphocytes and the ratio T-helpers/T-suppressors resulting from a decrease in the number of T-helpers, enhancement of immunoglobulinogenesis, and an increase in the level of circulating immune complexes. A degree of the above changes depends on a dose and period of the use of antihemophilic drugs. Hemophiliac patients require immunological monitoring, clinical substantiation of doses and a prolonged period of administration of blood preparations.
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Int J Comput Assist Radiol Surg
December 2024
Institute of Medical Science, University of Toronto, Toronto, ON, Canada.
Purpose: This study explores the use of deep generative models to create synthetic ultrasound images for the detection of hemarthrosis in hemophilia patients. Addressing the challenge of sparse datasets in rare disease diagnostics, the study aims to enhance AI model robustness and accuracy through the integration of domain knowledge into the synthetic image generation process.
Methods: The study employed two ultrasound datasets: a base dataset (Db) of knee recess distension images from non-hemophiliac patients and a target dataset (Dt) of hemarthrosis images from hemophiliac patients.
Indian J Pharmacol
September 2024
Department of Medicine, Lok Nayak Hospital, New Delhi, India.
Introduction: Very low-dose prophylaxis of antihemophilic factor (AHF) in hemophilia involves administration of minimal amounts of clotting factor concentrates, typically below standard doses, to maintain baseline levels and reduce bleeding episodes. This method uses smaller, more frequent doses to balance bleeding prevention and cost-effectiveness. Close monitoring ensures adequate protection while avoiding overtreatment, offering a tailored, cost-effective approach to improve the quality of life (QoL) of the hemophiliacs.
View Article and Find Full Text PDFJ Med Case Rep
October 2024
Consultant anaesthetists, Leicester Royal Infirmary, Leicester, UK.
Background: This is an interesting and unexpected thromboembolic event in a hemophiliac patient.
Case Presentation: This is a case of a 33-year-old primigravidarum of Indian Asian origin with Factor V and Factor VIII deficiency who developed a case of pulmonary embolism during the course of her pregnancy after presenting to the emergency department in Leicester, United Kingdom, with hemoptysis, tachycardia, and tachypnea. Patient was subsequently diagnosed with pulmonary embolism after a computed tomography pulmonary angiogram and was treated with therapeutic daltaparin, a low-molecular-weight heparin.
Healthcare (Basel)
October 2024
Orthopedics and Traumatology Clinic, IRCCS Policlinico San Matteo Foundation, 27100 Pavia, Italy.
Background: Hemophilia type A and B is associated with spontaneous bleeding in muscle tissues and joints. Acute hemarthrosis, representing 70-80% of all bleedings in severe hemophilia patients, is extremely painful. When surgical procedures are needed in hemophiliac patients, perioperative management should be planned with a multidisciplinary team.
View Article and Find Full Text PDFOrthop Surg
November 2024
Department of Orthopedics, The Second Affiliated Hospital of Anhui Medical University, Hefei, China.
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