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      Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease

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          Abstract

          Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. A set of 20 synthetic operative statements is herein provided, including the definition of Kawasaki disease, its protean presentations, clinical course and seminal treatment modalities of all disease phases. The application of these recommendations should improve prognosis of Kawasaki disease and prevent the progression to permanent vascular abnormalities, thereby diminishing morbidity and mortality.

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          Most cited references80

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          Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association

          Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries.
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            The Immunology of Multisystem Inflammatory Syndrome in Children with COVID-19

            Highlights Hyperinflammation in MIS-C differs from that of acute COVID-19 T-cell subsets discriminate Kawasaki disease patients from MIS-C IL-17A drives Kawasaki, but not MIS-C hyperinflammation. Global profiling reveals candidate autoantibodies with pathogenic potential
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              Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

              Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, p<0·0001). Serious adverse events were similar between both groups: two patients had high total cholesterol and one neutropenia in the intravenous immunoglobulin plus prednisolone group, and one had high total cholesterol and another non-occlusive thrombus in the intravenous immunoglobulin group. Addition of prednisolone to the standard regimen of intravenous immunoglobulin improves coronary artery outcomes in patients with severe Kawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Japanese Ministry of Health, Labour and Welfare. Copyright © 2012 Elsevier Ltd. All rights reserved.
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                Author and article information

                Contributors
                alessandra.marchesi@opbg.net
                donato.rigante@unicatt.it
                rolando.cimaz@unimi.it
                angeloravelli@gaslini.org
                isabella.tarissi@opbg.net
                alessandrorimini@gaslini.org
                fabiocardinale@libero.it
                marco.cattalini@unibs.it
                andrea.dezorzi@opbg.net
                rosamaria.dellepiane@policlinico.mi.it
                patrizia.salice@policlinico.mi.it
                aurelio.secinaro@opbg.net
                andrea.taddio@burlo.trieste.it
                paolo.palma@opbg.net
                may.elhachem@opbg.net
                elisabetta.cortis@aslroma2.it
                mariacristina.maggio@unipa.it
                giocors@alice.it
                alberto.villani@opbg.net
                Journal
                Ital J Pediatr
                Ital J Pediatr
                Italian Journal of Pediatrics
                BioMed Central (London )
                1824-7288
                25 January 2021
                25 January 2021
                2021
                : 47
                : 16
                Affiliations
                [1 ]GRID grid.414125.7, ISNI 0000 0001 0727 6809, Bambino Gesù Children’s Hospital, ; Rome, Italy
                [2 ]GRID grid.414603.4, Department of Life Sciences and Public Health, , Fondazione Policlinico A. Gemelli IRCCS, ; Rome, Italy
                [3 ]GRID grid.8142.f, ISNI 0000 0001 0941 3192, Università Cattolica Sacro Cuore, ; Rome, Italy
                [4 ]Pediatric Rheumatology, ASST Gaetano Pini-CTO, Milan, Italy
                [5 ]GRID grid.4708.b, ISNI 0000 0004 1757 2822, University of Milan, ; Milan, Italy
                [6 ]GRID grid.5606.5, ISNI 0000 0001 2151 3065, Pediatrics and Rheumatology Unit, , IRCCS Istituto Giannina Gaslini, University of Genoa, ; Genoa, Italy
                [7 ]GRID grid.419504.d, ISNI 0000 0004 1760 0109, IRCCS Istituto Giannina Gaslini, ; Genoa, Italy
                [8 ]Department of Pediatrics, AOU Policlinico Giovanni XXIII, Bari, Italy
                [9 ]GRID grid.7637.5, ISNI 0000000417571846, Pediatrics Clinic, , University of Brescia, ASST Spedali Civili di Brescia, ; Brescia, Italy
                [10 ]GRID grid.414818.0, ISNI 0000 0004 1757 8749, UOC of Pediatrics, UOS of Pediatric Immunology, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, ; Milan, Italy
                [11 ]GRID grid.414818.0, ISNI 0000 0004 1757 8749, Section of Pediatric Cardiovascular Diseases, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, ; Milan, Italy
                [12 ]GRID grid.5133.4, ISNI 0000 0001 1941 4308, University of Trieste, ; Trieste, Italy
                [13 ]GRID grid.418712.9, ISNI 0000 0004 1760 7415, Institute of Child and Maternal Health, IRCCS Burlo Garofolo, ; Trieste, Italy
                [14 ]GRID grid.416628.f, ISNI 0000 0004 1760 4441, UOC of Pediatrics, S. Eugenio Hospital, ; Rome, Italy
                [15 ]GRID grid.10776.37, ISNI 0000 0004 1762 5517, Università degli Studi di Palermo, ; Palermo, Italy
                Article
                962
                10.1186/s13052-021-00962-4
                7830049
                33494789
                aedaa199-2416-4458-b20c-a926dab9b247
                © The Author(s) 2021

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 16 October 2020
                : 4 January 2021
                Categories
                Review
                Custom metadata
                © The Author(s) 2021

                Pediatrics
                kawasaki disease,coronary artery abnormalities,intravenous immunoglobulin,aspirin,children

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