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      Type I Kounis syndrome variant: A case report and literature review

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          Abstract

          Kounis syndrome defined as the occurrence of acute coronary syndrome in the setting of allergic reaction due to mast cells activation and inflammatory mediators release that induces coronary vasospasm, plaque erosion, or even stent thrombosis. A 25-year-old postpartum female with asthma and recurrent episodes of chest pain was admitted with ST-segment elevation myocardial infarction in the setting of coronary artery spasms. The patient was started on calcium channel blockers and nitrite-based medication with no improvement. She was noted to have eosinophilia and initiation of corticosteroid-based regimen lead to resolution of chest pain episodes and normalization of eosinophilia. Kounis syndrome should be considered in young patients with chest pain. Coronary vasodilators are considered as the first-line of treatment. The use of corticosteroids has been described in the literature in severe or refractory cases.

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          Kounis syndrome (allergic angina and allergic myocardial infarction): a natural paradigm?

          Inflammatory mediators including histamine, neutral proteases, arachidonic acid products, platelet activating factor and a variety of cytokines and chemokines are increased in blood or urine in both allergic episodes and acute coronary syndromes. The release of mediators during allergic insults has been incriminated to induce coronary artery spasm and/or atheromatous plaque erosion or rupture. A common pathway between allergic and non-allergic coronary syndromes seems to exist. Today, there is evidence that mast cells not only enter the culprit region before plaque erosion or rupture but they release their contents before an actual coronary episode. Kounis syndrome is the concurrence of acute coronary syndromes with conditions associated with mast cell activation including allergic or hypersensitivity and anaphylactic or anaphylactoid insults. It is caused by inflammatory mediators released through mast cell activation. Kounis syndrome, as consequence, of the above pathophysiologic association is regarded as nature's own experiment and magnificent natural paradigm showing novel way in an effort to prevent acute coronary syndromes. Drugs and natural molecules which stabilize mast cell membrane and monoclonal antibodies that protect mast cell surface could emerge as novel therapeutic modalities capable to prevent acute coronary and cerebrovascular events.
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            Coronary hypersensitivity disorder: the Kounis syndrome.

            When allergy or hypersensitivity and anaphylactic or anaphylactoid insults lead to cardiovascular symptoms and signs, including acute coronary events, the result might be the recently defined nosologic entity Kounis syndrome. Vasospastic allergic angina, allergic myocardial infarction, and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells are the 3 reported variants of this syndrome.
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              Kounis syndrome: a new twist on an old disease.

              Kounis syndrome is the concurrence of acute coronary syndromes with conditions associated with mast cell activation, such as allergies or hypersensitivity and anaphylactic or anaphylactoid insults that can involve other interrelated and interacting inflammatory cells behaving as a 'ball of thread'. It is caused by inflammatory mediators such as neutral proteases including tryptase and chymase, arachidonic acid products, histamine, platelet activating factor and a variety of cytokines and chemokines released during the activation process. Platelets with FCεRI and FCεRII receptors also participate in the above cascade. Vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells constitute the three reported variants of this syndrome. Kounis syndrome is a ubiquitus disease that represents a magnificent natural paradigm and nature's own experiment, in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture. Kounis syndrome can complicate anesthesia, vaccination, medical therapy and stent implantation and it seems to be associated with coronary allograft vasculopathy and takotsubo syndrome, it can often be confused with hypersensitivity myocarditis and can be the cause of unexplained sudden death. Kounis syndrome has revealed that the same mediators released from the same inflammatory cells are present in acute coronary events of nonallergic etiology. These cells are not only present in the culprit region before plaque erosion or rupture but they release their contents just before an actual coronary event. Therefore, does Kounis syndrome represent a magnificent natural paradigm and nature's own experiment in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture showing a novel way towards our effort to prevent acute coronary syndromes? Drugs, substances targeting the stem cell factor that is essential for mast cell development, proliferation, survival, adhesion and homing as well as monoclonal antibodies and natural molecules that protect mast cell surface and stabilize mast cell membrane could emerge as novel therapeutic ways capable to prevent acute coronary and acute cerebrovascular events.
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                Author and article information

                Journal
                Avicenna J Med
                Avicenna J Med
                AJM
                Avicenna Journal of Medicine
                Medknow Publications & Media Pvt Ltd (India )
                2231-0770
                2249-4464
                Jan-Mar 2018
                : 8
                : 1
                : 37-39
                Affiliations
                [1] Department of Internal Medicine, Saint Clair Memorial Hospital, Allegheny General Hospital, Pittsburgh, PA, USA
                Author notes
                Address for correspondence: Dr. Abdullah Haddad, 5215 Riverfront Drive, Pittsburgh, PA 15238, USA. E-mail: haddad_abdullah@ 123456yahoo.com
                Article
                AJM-8-37
                10.4103/ajm.AJM_114_17
                5782419
                29404272
                3038d582-79af-41cc-b119-dfaa20a8bf1f
                Copyright: © 2018 Avicenna Journal of Medicine

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

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                Categories
                Case Report

                Medicine
                allergic coronary artery syndrome,coronary artery vasospasm,kounis syndrome
                Medicine
                allergic coronary artery syndrome, coronary artery vasospasm, kounis syndrome

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