{"orpha":454700,"name":"Acquired Creutzfeldt-Jakob disease","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A group of human prion diseases characterized by progressive, invariably fatal neurodegeneration resulting from accidental transmission of prions. The group comprises iatrogenic Creutzfeldt-Jakob disease (CJD), which results from transmission of CJD prions in the course of medical procedures or treatments, and variant CJD (transmission via consumption of products from prion-diseased cows or via blood transfusion from an affected individual).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-454700/","orphanet_url":"https://www.orpha.net/en/disease/detail/454700","names":{"fr":{"name":"Maladie de Creutzfeldt-Jakob acquise","definition":"Groupe de maladies humaines à prions, caractérisées par une neurodégénérescence progressive, invariablement mortelle, résultant d'une transmission accidentelle de prions. Le groupe comprend la maladie de Creutzfeldt-Jakob (MCJ) iatrogène, qui résulte de la transmission de prions de la MCJ au cours de procédures ou de traitements médicaux, et la variante de la MCJ (transmission par la consommation de produits provenant de vaches atteintes de prions ou par la transfusion sanguine d'un individu affecté)."},"de":{"name":"Creutzfeldt-Jakob-Krankheit, erworbene","definition":"Eine Gruppe menschlicher Prionenkrankheiten, die durch eine fortschreitende, ausnahmslos tödliche Neurodegeneration infolge einer zufälligen Übertragung von Prionen gekennzeichnet sind. Die Gruppe umfasst die iatrogene Creutzfeldt-Jakob-Krankheit (CJK), die durch die Übertragung von CJK-Prionen im Rahmen medizinischer Verfahren oder Behandlungen entsteht, und die variante CJK (vCJK), die durch Übertragung beim Verzehr von Produkten prionenkranker Kühe oder durch Bluttransfusion von einer betroffenen Person erfolgt."},"es":{"name":"Enfermedad de Creutzfeldt-Jakob adquirida","definition":"Es un grupo de enfermedades priónicas humanas caracterizadas por una neurodegeneración progresiva e invariablemente letal a consecuencia de la transmisión accidental de priones. El grupo comprende la enfermedad de Creutzfeldt-Jakob (ECJ) iatrogénica, resultante de la transmisión de priones de ECJ en el curso de procedimientos o tratamientos médicos, y la variante de la ECJ (transmisión a través del consumo de productos de derivados afectadas por la enfermedad priónica o mediante la transfusión de sangre de un individuo afectado)."},"it":{"name":"Malattia di Creutzfeldt-Jakob acquisita","definition":"Si tratta di un gruppo di malattie umane da prioni, caratterizzate dalla degenerazione neurologica progressiva e sempre letale, causata dalla trasmissione accidentale di prioni. Il gruppo comprende la malattia di Creutzfeldt-Jakob (CJD) iatrogena, causata dalla trasmissione dei prioni della CJD durante le procedure o i trattamenti medici, e la variante della CJD (la trasmissione avviene con il consumo di prodotti provenienti da mucche colpite dalla malattia da prioni o attraverso la trasfusione di sangue da un soggetto affetto)."},"nl":{"name":"Verworven ziekte van Creutzfeldt-Jakob","definition":"Een groep van humane prionziekten, gekarakteriseerd door progressieve, steevast fatale neurodegeneratie als gevolg van accidentele transmissie van prionen. Deze groep omvat iatrogene ziekte van Creutzfeldt-Jakob (CJD), die het gevolg is van transmissie van CJD-prionen tijdens medische procedures of behandelingen, en variant van CJD (transmissie via consumptie van producten van koeien met prionziekte of via bloedtransfusie van een aangetast individu)."},"pt":{"name":"Doença adquirida de Creutzfeldt-Jakob"},"pl":{"name":"Nabyta choroba Creutzfeldta i Jakoba"},"cs":{"name":"Získaná Creutzfeldtova-Jakobova choroba","synonyms":["Získaná Creutzfeldt-Jakobova choroba"]},"uk":{"name":"Набута хвороба Крейтцфельдта-Якоба","definition":"A group of human prion diseases characterized by progressive, invariably fatal neurodegeneration resulting from accidental transmission of prions. The group comprises iatrogenic Creutzfeldt-Jakob disease (CJD), which results from transmission of CJD prions in the course of medical procedures or treatments, and variant CJD (transmission via consumption of products from prion-diseased cows or via blood transfusion from an affected individual)."},"zh":{"name":"获得性克雅氏病","definition":"一组人类朊病毒疾病，其特征在于因朊病毒意外传播而引起的进行性，致命的神经退行性。该组疾病包括医源性克雅氏病（CJD），其是由于在医疗程序或治疗过程中传播了CJD 朊病毒，以及变异型CJD（通过食用病毒病病牛的产品或通过患病个体的输血传播）。"},"tr":{"name":"Edinsel Creutzfeldt-Jakob hastalığı"}},"xrefs":[{"source":"OMIM","code":"123400","qualifier":"NTBT","icd_relation":null,"validated":true},{"source":"MONDO","code":"0018686","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18686","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681100","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98006,"name":"Rare neurologic disease"},"parents":[{"orpha":576360,"name":"Acquired human prion disease","classifications":[181]},{"orpha":306695,"name":"Miscellaneous movement disorder due to neurodegenerative disease","classifications":[181]}],"children":[{"orpha":576379,"name":"Iatrogenic Creutzfeldt-Jakob disease","type":"Disease"},{"orpha":576370,"name":"Variant Creutzfeldt-Jakob disease","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}