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[New Advances in the Study of VEXAS Syndrome --Review].
Zhongguo Shi Yan Xue Ye Xue Za Zhi
December 2024
Department of Hematology, Zhongda Hospital Affiliated to Southeast University, Nanjing 210009, Jiangsu Province, China.
Article Synopsis
- VEXAS syndrome is an adult-onset autoinflammatory disorder linked to somatic mutations in the X-linked gene affecting the ubiquitin system.
- Patients exhibit a variety of symptoms, including fever, inflammation, and hematological issues like anemia and thrombocytopenia, leading to high morbidity and mortality.
- Current treatments focus on managing symptoms and mutations but are not well-developed, emphasizing the need for supportive care and risk factor management.
Inhibition and evasion of neutrophil microbicidal responses by .
mBio
December 2024
Department of Microbiology and Immunology, College of Medicine, University of Louisville, Louisville, Kentucky, USA.
Unlabelled: species evade degradation and proliferate within alveolar macrophages as an essential step for the manifestation of disease. However, most intracellular bacterial pathogens are restricted in neutrophils, which are the first line of innate immune defense against invading pathogens. Bacterial degradation within neutrophils is mediated by the fusion of microbicidal granules to pathogen-containing phagosomes and the generation of reactive oxygen species (ROS) by the phagocyte NADPH oxidase complex.
View Article and Find Full Text PDFCystic Neutrophilic Granulomatous Mastitis: Imaging Features With Histopathologic Correlation.
J Breast Imaging
December 2024
Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
Cystic neutrophilic granulomatous mastitis (CNGM) is a rare type of granulomatous lobular mastitis (GLM) with a distinct histologic pattern characterized on histopathology by clear lipid vacuoles lined by peripheral neutrophils ("suppurative lipogranulomas"), often containing gram-positive bacilli and strongly associated with Corynebacterial infection (in particular, Corynebacterium kroppenstedtii). Cystic neutrophilic granulomatous mastitis has a distinct histopathologic appearance, but the imaging appearance is less well described and has been limited to case reports and small case series published primarily in pathology literature. Mammographic findings of CNGM include focal asymmetry, skin thickening, and irregular or oval masses.
View Article and Find Full Text PDFAcquired autoinflammatory disorders: A dermatologist's perspective.
Clin Exp Dermatol
December 2024
Department of Dermatology, Venereology and Leprology; Postgraduate Institute of Medical Education and Research; Chandigarh, India.
Article Synopsis
- Autoinflammatory disorders are characterized by an exaggerated response of the innate immune system, primarily involving neutrophils and macrophages, and are differentiated from connective tissue diseases by the absence of anti-nuclear antibodies.
- Many of these disorders are linked to inherited genetic mutations and tend to manifest in childhood or early adulthood, though some, like VEXAS syndrome, highlight the potential for acquired mutations in adults.
- The review focuses on the cutaneous manifestations of various acquired autoinflammatory disorders, including adult-onset Still's disease and Schnitzler syndrome, which have significant diagnostic implications.
Exercise Preconditioning of the Donor Liver Decreases Cold Ischemia/Reperfusion Injury in a Mouse Model.
Transplantation
January 2025
Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA.
Background: Liver transplantation stands as the primary treatment for end-stage liver disease, with demand surging in recent decades because of expanded indications. However, hepatic ischemia/reperfusion injury can lead to liver transplant failure in both deceased donor and living donor transplantation. This study explored whether preconditioning donor livers through exercise training (ExT) could mitigate cold ischemic injury posttransplantation.
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