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http://dx.doi.org/10.1007/BF02121232 | DOI Listing |
J Allergy Clin Immunol Glob
February 2025
Department of Medicine, Division of Allergy, Immunology & Rheumatology, Jacobs School of Medicine and Biomedical Sciences, Clinical Translational Research Center, University at Buffalo, Buffalo, NY.
Background: Hereditary angioedema (HAE) is a rare inherited disorder that predisposes an individual to develop vasogenic edema. Bradykinin release, which increases vascular permeability, results in angioedema. C1 esterase inhibitor (C1-INH) is a major regulator of critical enzymes involved in bradykinin generation and mutations in genes that encode the C1 inhibitor of complement factor 1, which prevent its synthesis (type I HAE), form a dysfunctional protein (type II HAE), or have normal functioning C1-INH (type III HAE, aka HAE-III).
View Article and Find Full Text PDFInt J Cardiol
January 2025
CHANGE Research Working Group, Universidad Científica del Sur, Lima, Peru.
Cardiovasc Hematol Disord Drug Targets
October 2024
Department of Hospital Therapy with a Course of Medical and Social Expertise, Federal State Budgetary Educational Institution of Higher Education, "Ryazan State Medical University Named after Academician I.P. Pavlov" of the Ministry of Health of the Russian Federation, Ryazan, 390026, Russia.
J Clin Invest
March 2024
Lung and Vascular Inflammation Section, Laboratory of Allergic Diseases, and.
Clarkson disease, or monoclonal gammopathy-associated idiopathic systemic capillary leak syndrome (ISCLS), is a rare, relapsing-remitting disorder featuring the abrupt extravasation of fluids and proteins into peripheral tissues, which in turn leads to hypotensive shock, severe hemoconcentration, and hypoalbuminemia. The specific leakage factor(s) and pathways in ISCLS are unknown, and there is no effective treatment for acute flares. Here, we characterize an autonomous vascular endothelial defect in ISCLS that was recapitulated in patient-derived endothelial cells (ECs) in culture and in a mouse model of disease.
View Article and Find Full Text PDFCureus
March 2024
Emergency and Intensive Care Medicine, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, JPN.
Kounis syndrome is an acute coronary syndrome (ACS) caused by an allergic reaction that almost always occurs immediately and simultaneously with allergic symptoms. We present a case of Kounis syndrome type III that developed after complete resolution of contrast-induced anaphylaxis in a 60-year-old man with a coronary stent placed in the proximal left anterior descending (LAD) artery branch for ischemic heart disease. Contrast-enhanced computed tomography revealed anaphylactic shock.
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