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French national diagnostic and care protocol for Kawasaki disease |
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| Institution: | 1. Service de rhumatologie pédiatrique, centre de référence des maladies auto-inflammatoires rares et des amyloses, CHU de Bicêtre, 78, rue du Général-Leclerc, 94275 Le Kremlin-Bicêtre, France;2. M3C-Necker-Enfants–Malades, hôpital Necker-Enfants–Malades, université de Paris Cité, Paris, France;3. Service de néphrologie, rhumatologie et dermatologie pédiatriques, centre de référence des rhumatismes inflammatoires et maladies auto-immunes systémiques rares de l’enfant (RAISE), hôpital Femme-Mère–Enfant, hospices civils de Lyon, Lyon, France;4. Service des urgences pédiatriques, centre hospitalier intercommunal de Créteil, Créteil, France;5. Service de spécialités pédiatriques et médecine infantile, CHU Timone-Enfants, 13385 Marseille cedex 5, France;6. Service de pédiatrie générale, maladies infectieuses et médecine interne, centre de référence des rhumatismes inflammatoires et maladies auto-immunes systémiques rares de l’enfant, hôpital universitaire Robert-Debré, université hospital, Assistance publique–Hôpitaux de Paris, 75019 Paris, France;7. Pediatric Rheumatology Unit, Gaetano Pini Hospital, Department of Clinical Sciences and Community Health, Research Centre for Adult and Pediatric Rheumatic Diseases, Università degli Studi di Milano, Milan, Italy;8. Association France vascularites, Blaisy-Bas, France;9. Service de médecine interne, hôpital Saint-Antoine, Sorbonne université, AP–HP, 75012 Paris, France;10. Service de pédiatrie, centre de référence des maladies auto-inflammatoires et de l’amylose (CEREMAIA), centre hospitalier de Versailles, Le Chesnay, France;11. Service de radiologie pédiatrique, Institut mutualiste Montsouris, CHU de Bicêtre, Le Kremlin-Bicêtre, France;12. Service de pédiatrie générale et des urgences pédiatriques, hôpital Jean-Verdier, Assistance publique–Hôpitaux de Paris (AP–HP), Bondy, France;13. Service de réanimation pédiatrique et néonatale, DMU 3 santé de l’enfant et adolescent, hôpital Bicêtre, université Paris-Saclay, AP–HP, Le Kremlin-Bicêtre, France;14. Division of Dermatology, Department of Pediatrics, Centre Hospitalier Universitaire Sainte Justine, Université de Montréal, Montréal, Quebec, Canada;15. Service des urgences pédiatriques, hôpital universitaire de Pellegrin, Bordeaux, France;p. Service de pédiatrie, CHU Saint-Étienne, Saint-Étienne, France;q. Service d’accueil des urgences pédiatriques, hôpital Nord, AP–HM, 13005 Marseille, France;1. Service de médecine interne et immunologie clinique, Groupe Hospitalier Universitaire Paris-Saclay, Hôpital Bicêtre, Université Paris-Saclay, Assistance Publique–Hôpitaux de Paris, Le Kremlin-Bicêtre, France;2. Service de neurologie, centre de référence des neuropathies Périphériques Rares (NNERF), Groupe Hospitalier Universitaire Paris-Saclay, Hôpital Bicêtre, Université Paris-Saclay, Assistance Publique–Hôpitaux de Paris, Le Kremlin-Bicêtre, France;3. Service de neurophysiologie, centre de référence des neuropathies périphériques rares (NNERF), Groupe Hospitalier Universitaire Paris-Saclay, Hôpital Bicêtre, Université Paris-Saclay, Assistance Publique–Hôpitaux de Paris, Le Kremlin-Bicêtre, France;4. Service d’anatomie pathologique et neuropathologie, Centre de Référence des Neuropathies Périphériques Rares (NNERF), Groupe Hospitalier Universitaire Paris-Saclay, Hôpital Bicêtre, Université Paris-Saclay, Assistance Publique–Hôpitaux de Paris, Le Kremlin-Bicêtre, France;1. Service de médecine interne, hôpital Cochin, AP–HP, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France;2. Université Paris cité, 75006 Paris, France;1. Service de médecine interne, Hospices civils de Lyon, Hôpital de la Croix-Rousse, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France;2. Hospices civils de Lyon, Pôle IMER, 69003 Lyon, France;3. Service d’ophtalmologie, Hospices civils de Lyon, Hôpital de la Croix-Rousse, 103, grande rue de la Croix-Rousse, 69317 Lyon cedex 04, France;4. Service d’immunologie clinique et médecine interne, CNR RESO, maladies auto-immunes et systémiques rares, Nouvel Hôpital civil, Hôpitaux Universitaires de Strasbourg, Strasbourg, France;5. Université de Lyon, Lyon, France;6. University Lyon, University Claude Bernard-Lyon 1, HESPER EA 7425, 69008 Lyon, France;1. Internal Medicine Service, Hospital Universitario 12 de Octubre, Avenida de Córdoba s/n, Planta 13 derecha, 28041 Madrid, Spain;2. Pathology Department, Hospital Universitario 12 de Octubre, Avenida de Córdoba s/n, Planta -1, 28041 Madrid, Spain;1. Nephrology and Dialysis Unit, Santa Caterina Novella Hospital, Galatina, Italy;2. Department of Biomedical, Experimental and Clinical Sciences “Mario Serio”, University of Firenze, Firenze, Italy;3. Department of Experimental and Clinical Medicine, University of Fireze, Firenze, Italy;4. Nephrology and Dialysis Unit, Department of Pediatrics, Meyer Children''s Hospital, Firenze, Italy;5. Department of Medical Sciences, University of Torino, Torino, Italy;1. Service des Soins Intensifs, Hôpital DELTA (CHIREC), Bruxelles, Belgique;2. Département de Médecine Interne, Hôpital DELTA (CHIREC), Bruxelles, Belgique;3. Université Libre de Bruxelles (ULB), Bruxelles, Belgique;4. Laboratoire de Neurogénétique et Neuroinflammation, Institut Imagine, 24, boulevard du Montparnasse, 75015 Paris, France |
| Abstract: | Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up. |
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