{"orpha":2846,"name":"Congenital pericardium anomaly","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"Congenital pericardium anomaly comprises a group of rare congenital cardiac malformations characterized by the complete (Congenital complete agenesis of pericardium) or partial absence of the pericardium (Congenital partial agenesis of pericardium), or by the presence of pericardial cysts (Pleuropericardial cyst).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2846/","orphanet_url":"https://www.orpha.net/en/disease/detail/2846","names":{"fr":{"name":"Anomalie congénitale du péricarde","definition":"Une anomalie rare du développement embryonnaire qui comprend un groupe de malformations cardiaques congénitales rares caractérisées par l'absence complète (agénésie congénitale complète du péricarde) ou partielle du péricarde (agénésie partielle congénitale du péricarde), ou par la présence de kystes péricardiques (kyste pleuropéricardique)."},"de":{"name":"Perikardfehlbildungen, kongenitale"},"es":{"name":"Anomalía congénita del pericardio","definition":"Este término comprende un grupo de malformaciones cardíacas congénitas poco frecuentes, caracterizadas por la ausencia completa (agenesia completa congénita del pericardio) o parcial (agenesia parcial congénita del pericardio) del pericardio, o por la presencia de quistes pericárdicos (quiste pleuropericárdico)."},"it":{"name":"Anomalia congenita del pericardio","definition":"L'anomalia congenita del pericardio comprende un gruppo di cardiopatie congenite rare, caratterizzate dall'assenza completa (agenesia congenita completa del pericardio) o parziale del pericardio (agenesia congenita parziale del pericardio), oppure dalla presenza di cisti pericardiche (cisti pleuro-pericardica) (si vedano questi termini)."},"nl":{"name":"Congenitale pericardanomalie","definition":"Congenitale pericardanomalie omvat een groep van zeldzame, congenitale hartmalformaties die gekarakteriseerd worden door de volledige (congenitale complete agenesie van het pericard) of partiële afwezigheid (congenitale partiële agenesie van het pericard) van het pericard, of door de aanwezigheid van pericardiale cysten (pleuropericardiale cyste) (zie deze termen)."},"pt":{"name":"Anomalia congénita do pericárdio"},"pl":{"name":"Wrodzona wada osierdzia"},"cs":{"name":"Vrozená anomálie perikardu"},"uk":{"name":"Вроджена аномалія перикарда","definition":"Congenital pericardium anomaly comprises a group of rare congenital cardiac malformations characterized by the complete (Congenital complete agenesis of pericardium) or partial absence of the pericardium (Congenital partial agenesis of pericardium), or by the presence of pericardial cysts (Pleuropericardial cyst) (see these terms)."},"zh":{"name":"先天性心包缺损"},"tr":{"name":"Doğumsal perikard anomalisi"}},"xrefs":[{"source":"ICD-11","code":"LA8D","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1188459532"},{"source":"MONDO","code":"0017300","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"17300","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0685699","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93890,"name":"Rare developmental defect during embryogenesis"},"parents":[{"orpha":88991,"name":"Rare congenital heart disease","classifications":[147,148]}],"children":[{"orpha":99129,"name":"Congenital complete agenesis of pericardium","type":"Morphological anomaly"},{"orpha":99130,"name":"Congenital partial agenesis of pericardium","type":"Morphological anomaly"},{"orpha":99131,"name":"Pleuro-pericardial cyst","type":"Morphological anomaly"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}