{"orpha":598363,"name":"Multisystem inflammatory syndrome in children and adults","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["MIS-C/A"],"definition":"A rare systemic disease characterized by a life-threatening hyperinflammatory state several weeks after infection with SARS-CoV-2, predominantly occurring in children. The primary infection is typically mild or asymptomatic, and patients are generally previously healthy individuals. Typical presenting signs and symptoms are persistent fever, gastrointestinal symptoms, mucocutaneous inflammation, lymphopenia, and high levels of circulating inflammatory markers. Some patients develop severe disease with cardiac involvement, hypotension, and shock. Presentation is similar in adults, although the severity of cardiac dysfunction, incidence of thrombosis, and mortality may be higher.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-598363/","orphanet_url":"https://www.orpha.net/en/disease/detail/598363","names":{"fr":{"name":"Syndrome inflammatoire multisystémique chez l'enfant et l'adulte","synonyms":["MIS-C/A"],"definition":"Maladie systémique rare caractérisée par un état hyperinflammatoire potentiellement létal plusieurs semaines après une infection par le SARS-CoV-2, survenant principalement chez les enfants. La primo-infection est généralement légère ou asymptomatique, et les patients sont généralement des personnes en bonne santé. Les signes et symptômes typiques sont une fièvre persistante, des symptômes gastro-intestinaux, une inflammation cutanéo-muqueuse, une lymphopénie et des taux élevés de marqueurs inflammatoires circulants. Certains patients développent une forme grave avec atteinte cardiaque, hypotension et choc. La présentation est similaire chez les adultes, bien que la gravité du dysfonctionnement cardiaque, l'incidence de la thrombose et la létalité puissent être plus élevées."},"de":{"name":"Multisystemisches inflammatorisches Syndrom bei Kindern und Erwachsenen","synonyms":["MIS-A","MIS-C","MIS-C/A","PIMS","PIMS-TS","Pädiatrisches hyperinflammatorisches Syndrom mit Multiorganbeteiligung"]},"es":{"name":"Síndrome inflamatorio multisistémico en la infancia y la edad adulta","synonyms":["SIM-A","SIM-C","SIM-C/A","SIMP","SIMP-TS","Síndrome inflamatorio multisistémico pediátrico"],"definition":"Es una enfermedad sistémica poco frecuente caracterizada por un estado hiperinflamatorio potencialmente mortal varias semanas después de la infección por SARS-CoV-2, que ocurre predominantemente en niños. La infección primaria suele ser leve o asintomática y los pacientes suelen ser personas previamente sanas. Los signos y síntomas de presentación típicos son fiebre persistente, síntomas gastrointestinales, inflamación mucocutánea, linfopenia y niveles elevados de marcadores inflamatorios circulantes. Algunos pacientes desarrollan una enfermedad grave con afectación cardíaca, hipotensión y shock. La presentación es similar en adultos, aunque la gravedad de la disfunción cardíaca, la incidencia de trombosis y la mortalidad pueden ser mayores."},"it":{"name":"Sindrome infiammatoria multisistemica del bambino e dell'adulto","synonyms":["MIS-A","MIS-C","MIS-C/A","PIMS","PIMS-TS","Sindrome infiammatoria multisistemica pediatrica"]},"nl":{"name":"Multisystemisch inflammatoir syndroom bij kinderen en volwassenen","synonyms":["MIS-C/A"],"definition":"Een zeldzame systemische aandoening, gekenmerkt door een levensbedreigende hyperinflammatoire toestand meerdere weken na infectie door SARS-CoV-2, die hoofdzakelijk optreedt bij kinderen. De primaire infectie is doorgaans mild of asymptomatisch, en doorgaans zijn patiënten voorheen gezonde individuen. Typische presenterende verschijnselen en symptomen zijn aanhoudende koorts, gastro-intestinale symptomen, mucocutane ontsteking, lymfopenie, en hoge gehaltes van circulerende inflammatoire merkers. Sommige patiënten ontwikkelen een ernstige ziekte met betrokkenheid van hart, hypotensie en shock. De presentatie is gelijkaardig bij volwassenen, maar hartdisfunctie kan ernstiger zijn, en de incidentie van trombose alsook de mortaliteit kunnen hoger liggen."},"pt":{"name":"Síndrome inflamatório multissistémico em crianças e adultos","synonyms":["PIMS","Síndrome inflamatório multissistémico pediátrico"]},"pl":{"name":"Wieloukładowy zespół zapalny u dzieci i dorosłych","synonyms":["PIMS","MIS-A","MIS-C","MIS-C/A","PIMS-TS","Pediatryczny wieloukładowy zespół zapalny"]},"cs":{"name":"Multisystémový zánětlivý syndrom u dětí a dospělých","synonyms":["MIS-C/A"]},"uk":{"name":"Мультисистемний запальний синдром у дітей та дорослих","synonyms":["MIS-C/A"],"definition":"A rare systemic disease characterized by a life-threatening hyperinflammatory state several weeks after infection with SARS-CoV-2, predominantly occurring in children. The primary infection is typically mild or asymptomatic, and patients are generally previously healthy individuals. Typical presenting signs and symptoms are persistent fever, gastrointestinal symptoms, mucocutaneous inflammation, lymphopenia, and high levels of circulating inflammatory markers. Some patients develop severe disease with cardiac involvement, hypotension, and shock. Presentation is similar in adults, although the severity of cardiac dysfunction, incidence of thrombosis, and mortality may be higher."},"zh":{"name":"Multisystem inflammatory syndrome in children and adults","synonyms":["MIS-C/A"]},"tr":{"name":"Multisystem inflammatory syndrome in children and adults","synonyms":["MIS-C/A"]}},"xrefs":[{"source":"ICD-10","code":"U10.9","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"RA03","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1195031154"},{"source":"MONDO","code":"0035375","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680268","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":280373,"name":"Rare pediatric systemic disease","classifications":[231]},{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"33640145[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"33640145[PMID]"}],"phenotypes":null}