Coagulopathy in the nephrotic syndrome (NS) is very rare. Simultaneous prolongation of both prothrombin time and activated partial thromboplastin time suggests common coagulation pathway abnormality such as liver dysfunction, Vitamin K deficiency, disseminated intravascular coagulation, or primary fibrinolysis. This results in difficulty in proceeding with renal biopsy and tissue diagnosis. We report one such case of NS with coagulopathy and refractoriness to correction with blood products, which led us to make a diagnosis of AL amyloidosis, which was confirmed with abdominal fat pad biopsy and other work-up.
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http://dx.doi.org/10.4103/1319-2442.284035 | DOI Listing |
Ital J Pediatr
November 2024
Department of Interdisciplinary of Medicine, University of Bari 'Aldo Moro', Bari, 70029, Italy.
Background: Hemophilia B is a rare bleeding disorder in males, characterized by a deficiency in coagulation factor IX (FIX). Replacement of FIX with a recombinant FIX (rFIX) fusion protein, to sustain therapeutic plasma levels, is recommended as both treatment and prophylaxis to prevent bleeding episodes, particularly intracranial hemorrhage (ICH).
Case Presentation: This case report outlines the management of ICH in a 7-month-old infant with severe hemophilia B, following an accidental trauma-related event, resulting in a thin compound fracture of the left occiput.
Int J Rheum Dis
September 2024
Faculty of Medicine, Department of Pediatric Nephrology, Gazi University, Ankara, Turkey.
Pediatr Nephrol
January 2025
Department of Nephrology, Children's Hospital of Nanjing Medical University, 72 Guangzhou Road, Nanjing, 210029, Jiangsu Province, China.
Biomedica
May 2024
Departamento de Medicina Interna, Universidad del Valle, Cali, Colombia; Hospital Universitario del Valle Evaristo García, Cali, Colombia.
Introduction: Stroke in young individuals is becoming increasingly prevalent worldwide. Its causes can vary widely, so a thorough investigation by a multidisciplinary team is needed. Pinpointing the precise underlying pathology responsible for the stroke yields benefits for patients, particularly in recurrent events.
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