{"orpha":707,"name":"Plague","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Y. pestis infection","Yersinia pestis infection"],"definition":"A rare zoonotic disease characterized by life-threatening, highly contagious and rapidly progressing infection caused by the Gram-negative bacterium <i>Yersinia pestis</i>, affecting rodents, and transmitted to human accidentally. It manifests differently depending on the route of infection. Bubonic plague results from the bite of an infected rodent flea. Pneumonic plague, less frequent but more dangerous, may arise through inhalation of infectious droplets or spread of bubonic plague to the lungs. Septicemic plague occurs from dissemination into the bloodstream, either as a primary infection or as a complication of bubonic or pneumonic forms, leading to disseminated intravascular coagulation, necrosis, and multi-organ failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-707/","orphanet_url":"https://www.orpha.net/en/disease/detail/707","names":{"fr":{"name":"Peste","synonyms":["Infection à Y. pestis","Infection à Yersinia pestis"],"definition":"Zoonose rare caractérisée par une infection potentiellement létale, hautement contagieuse et à progression rapide, causée par la bactérie Gram-négative <i>Yersinia pestis</i>, qui touche les rongeurs et se transmet accidentellement à l'homme. La maladie se manifeste différemment selon la voie de transmission. La peste bubonique résulte de la piqûre d'une puce infectée provenant d'un rongeur. La peste pulmonaire, moins fréquente mais plus dangereuse, peut se développer par inhalation de gouttelettes infectées ou par propagation de la peste bubonique aux poumons. La peste septicémique survient à la suite d'une dissémination dans la circulation sanguine, soit sous forme d'infection primaire, soit de complication des formes bubonique ou pulmonaire, entraînant une coagulation intravasculaire disséminée, une nécrose et une défaillance multiviscérale."},"de":{"name":"Pest","synonyms":["Yersinia pestis-Infektion"],"definition":"Eine seltene Zoonose, die durch eine lebensbedrohliche, hoch ansteckende und schnell fortschreitende Infektion gekennzeichnet ist, die durch das gramnegative Bakterium <i>Yersinia pestis</i> verursacht wird, Nagetiere befällt und versehentlich auf den Menschen übertragen wird. Sie äußert sich je nach Infektionsweg unterschiedlich. Die Beulenpest wird durch den Biss eines infizierten Nagetierflohs verursacht. Die seltenere, aber gefährlichere Lungenpest kann durch Einatmen infektiöser Tröpfchen oder durch Ausbreitung der Beulenpest in die Lunge entstehen. Die septische Pest entsteht durch die Verbreitung in die Blutbahn, entweder als Primärinfektion oder als Komplikation der Beulenpest oder der Lungenpest, was zu disseminierter intravasaler Gerinnung, Nekrose und Multiorganversagen führt."},"es":{"name":"Peste","synonyms":["Infección por Y. pestis","Infección por Yersinia pestis","Yersiniosis"],"definition":"Es una enfermedad zoonótica poco frecuente caracterizada por una infección potencialmente mortal, altamente contagiosa y de rápida progresión causada por la bacteria gramnegativa <i>Yersinia pestis</i>, que afecta a roedores y que se transmite accidentalmente a humanos. Se manifiesta de forma diferente según la vía de infección. La peste bubónica se produce por la picadura de una pulga de roedor infectada. La peste neumónica, menos frecuente pero más peligrosa, puede originarse por inhalación de gotitas infecciosas o por la propagación de la peste bubónica a los pulmones. La peste septicémica se produce por diseminación al torrente sanguíneo, ya sea como infección primaria o como complicación de las formas bubónica o neumónica, lo que provoca coagulación intravascular diseminada, necrosis y fallo multiorgánico."},"it":{"name":"Peste","synonyms":["Infezione da Y.pestis","Infezione da Yersinia pestis"]},"nl":{"name":"Pest","synonyms":["Infectie door Y. pestis","Infectie door Yersinia pestis"],"definition":"Een zeldzame zoönotische aandoening, gekenmerkt door levensbedreigende, zeer besmettelijke en snel progressieve infectie veroorzaakt door de Gram-negatieve bacterie <i>Yersinia pestis</i>, die knaagdieren aantast en per toeval op mensen wordt overgedragen. De manifestatie verschilt naargelang de route van de infectie. Builenpest is het gevolg van een beet van een geïnfecteerde vlo van een knaagdier. Longpest, minder frequent maar gevaarlijker, kan ontstaan door inhalatie van besmettelijke druppels of uitzaaiing van builenpest naar de longen. Septikemische pest treedt op door disseminatie in de bloedbaan, hetzij als primaire infectie of als complicatie van builen- of longpest, wat leidt tot gedissemineerde intravasculaire stolling, necrose, en multi-orgaanfalen."},"pt":{"name":"Peste","synonyms":["Infeção por Y. pestis","Infeção por Yersinia pestis"],"definition":"A peste é uma infecção bacteriana grave causada pela bactéria Gram-negativo <i>Yersinia pestis</i>."},"pl":{"name":"Dżuma","synonyms":["Jersinioza"]},"cs":{"name":"Mor","synonyms":["Yersinióza"]},"uk":{"name":"Чума","synonyms":["Інфекція Y. pestis","Інфекція Yersinia pestis"],"definition":"Plague is a severe bacterial infection caused by the Gram-negative bacterium <i>Yersinia pestis</i>."},"zh":{"name":"鼠疫","synonyms":["耶尔森菌病"]},"tr":{"name":"Veba","synonyms":["Yersiniyoz"]}},"xrefs":[{"source":"ICD-10","code":"A20.0","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.1","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.2","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.3","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.7","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.8","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-10","code":"A20.9","qualifier":"BTNT","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"1B93","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1596449540"},{"source":"MONDO","code":"0019095","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010930","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0032064","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10035148","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":659712,"name":"Rare yersiniosis","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.2,"area":"Europe","status":"Validated","source":"European Centre for Disease prevention and Control 2011[OTHER]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":237.0,"area":"Africa","status":"Validated","source":"World Health Organization 2019[OTHER]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":6.0,"area":"Latin America","status":"Validated","source":"World Health Organization 2019[OTHER]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":2.0,"area":"South East Asia","status":"Validated","source":"World Health Organization 2019[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-11-18","source":"27722868[PMID]_29070654[PMID]_29628173[PMID]_26004640[PMID]","terms":[{"hpo":"HP:0012378","label":"Fatigue","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002840","label":"Lymphadenitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008066","label":"Abnormal blistering of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000739","label":"Anxiety","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031864","label":"Bacteremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0020084","label":"Carbuncle","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025143","label":"Chills","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030953","label":"Conjunctival hyperemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031258","label":"Delirium","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000958","label":"Dry skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011355","label":"Localized skin lesion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004372","label":"Reduced consciousness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001649","label":"Tachycardia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009811","label":"Abnormality of the elbow","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011949","label":"Acute infectious pneumonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025085","label":"Bloody diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0040181","label":"Chapped lip","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001259","label":"Coma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000716","label":"Depression","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025043","label":"Enlarged mesenteric lymph node","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004387","label":"Enterocolitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012219","label":"Erythema nodosum","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000206","label":"Glossitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000365","label":"Hearing impairment","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002248","label":"Hematemesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0032564","label":"Ileitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002037","label":"Inflammation of the large intestine","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011499","label":"Mydriasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001350","label":"Slurred speech","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002317","label":"Unsteady gait","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100584","label":"Endocarditis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100533","label":"Inflammatory abnormality of the eye","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001287","label":"Meningitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025439","label":"Pharyngitis","frequency":"Very rare (<4-1%)","criteria":null}]}}