Epistaxis is a potentially life-threatening condition that can compromise a patient's airway, breathing and circulation. Early recognition of underlying bleeding diatheses that are amenable to medical therapy is imperative in order for timely treatment and to reduce morbidity. We present a case in which acquired haemophilia was diagnosed in an 83-year-old man presenting with seemingly uncomplicated epistaxis. The patient suffered multisite haemorrhages and haemodynamic compromise before definitive management with monoclonal antibody therapy. The case highlights the difficulties in managing this rare condition. It also emphasises the importance of clotting assay analysis in patients who present with epistaxis in the absence of an underlying anticoagulant.
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http://dx.doi.org/10.1136/bcr-2015-213528 | DOI Listing |
Superficial arteriovenous malformations are rare fast-flow lesions. They consist of arteriovenous shunts, without cellular hyperplasia or proliferation, which develop in the surrounding tissues (cutaneous, subcutaneous, muscular, bone). Although benign, they are among the most severe of superficial malformations.
View Article and Find Full Text PDFBMC Anesthesiol
January 2025
Department of Anesthesiology, Peking Union Medical College Hospital, Beijing, China.
Background: Halo-pelvic traction is a relatively safe treatment for preoperative spinal deformity correction in patients with severe scoliosis. Common device-related complications include local infection, back discomfort, and nerve compression symptoms. However, there are potential risks of mechanical compression of bronchial structures, especially in patients with severe thoracic lordosis and scoliosis, which can lead to life-threatening airway obstruction.
View Article and Find Full Text PDFArch Ital Urol Androl
January 2025
Department of Medicine and Surgery, Urology Clinic, University of Perugia.
Objective: Fournier's gangrene (FG) is a rare, life-threatening necrotizing fasciitis primarily affecting the perineal, genital, and perianal regions. This rapidly progressing bacterial infection predominantly affects middleaged and elderly men. This multicenter study aims to describe the management in a wide cohort of Fournier's gangrene cases that presented to three tertiary centers with early extensive surgical debridement.
View Article and Find Full Text PDFCurr Opin Pediatr
January 2025
Division of Pediatric Nephrology, The University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Purpose Of Review: This review aims to summarize how scientific advances in complement biology have not only improved the diagnosis and management of aHUS but also continue to offer insights into the pathophysiology of complement-mediated disease that may be leveraged for future therapeutic developments.
Recent Findings: Updated information on the clinical and epidemiological features, pathophysiology, diagnosis, management, and potential for future therapeutic advancements in the treatment of aHUS are reviewed.
Summary: aHUS is a rare but potentially life-threatening disease that requires prompt diagnosis and treatment as well as long-term management via a multidisciplinary team providing coordination of primary and specialty care as well as outreach and education for children and families affected by this life-long disease.
Glob Reg Health Technol Assess
January 2025
Omakase Consulting S.L., Barcelona - Spain.
Background: Activated phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS) is an ultra-rare, potentially life-threatening disease that lacks approved treatments in Spain. This study aimed to apply Multi-Criteria Decision Analysis (MCDA) to assess the value of the first pharmacological treatment for APDS in Spain.
Methods: A multidisciplinary group of 8 experts evaluated the selective PI3Kδ inhibitor leniolisib against Standard of Care (SoC).
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