Hemophilia A is an X-linked genetic hemorrhagic disorder characterized by a factor VIII deficiency. The availability of secured substitution products has led to a dramatic improvement of life expectancy in hemophiliac patients. Nowadays, adult hemophiliac patients may develop Chronic Kidney Disease (CKD) resulting from age-related comorbidities (hypertension, obesity, diabetes). In addition, the high prevalence of viral infections in this population exposes patients to an increased risk of CKD. The risk of hemorrhage in hemophiliac patients is a challenge for their clinical management, both for diagnostic procedures (kidney biopsy in particular) and for renal replacement therapy (dialysis or renal transplantation) when it is needed. This work provides an update of the literature data concerning the management of hemophiliac patients in nephrology, illustrated by the cases of two patients.
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http://dx.doi.org/10.1016/j.nephro.2018.10.002 | DOI Listing |
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.
Introduction: Total joint replacement is the optimal treatment option for patients with severe haemophilic arthritis. Current research emphasizes patient-reported outcomes as a vital measure for evaluating surgical outcomes and patient satisfaction. Nevertheless, very limited information about the subjective experience of perioperative haemophiliacs in the literature, highlighting the need for exploration in this area.
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