{"orpha":90066,"name":"Pneumonia caused by Pseudomonas aeruginosa infection","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare pulmonary disease characterized by primary or nonbacteremic pneumonia most frequently arising in an intensive care setting, or bacteremic pneumonia, which is typically associated with neutropenia. Chronic lower respiratory tract infection with development of episodes of pneumonia is common in patients with cystic fibrosis. Acute infections are potentially life-threatening. Patients present with fever, chills, dyspnea, cyanosis, productive cough, as well as signs of severe systemic toxicity. Alveolar hemorrhage, necrosis, and, eventually, cavity formation, are commonly seen.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-90066/","orphanet_url":"https://www.orpha.net/en/disease/detail/90066","names":{"fr":{"name":"Pneumonie due à une infection à Pseudomonas aeruginosa","definition":"Maladie pulmonaire rare caractérisée par une pneumonie non bactériémique ou primaire qui survient la plupart du temps en contexte de soins intensifs, ou par une pneumonie bactériémique généralement associée à une neutropénie. Une infection chronique des voies respiratoires supérieures, accompagnée d'épisodes de pneumonie, est courante chez les patients atteints de mucoviscidose. Les infections aiguës peuvent engager le pronostic vital. Les patients présentent une fièvre, des frissons, une dyspnée, une cyanose, une toux productive, ainsi que des signes de toxicité systémique sévère. Une hémorragie alvéolaire, une nécrose et, à terme, une cavitation sont fréquemment observées."},"de":{"name":"Pneumonie durch Pseudomonas aeruginosa-Infektion","definition":"Eine seltene Lungenerkrankung, die durch eine primäre oder nicht bakteriämische Lungenentzündung, die am häufigsten auf der Intensivstation auftritt, oder eine bakteriämische Lungenentzündung, die in der Regel mit einer Neutropenie einhergeht, gekennzeichnet ist. Chronische Infektionen der unteren Atemwege mit schubweiser Lungenentzündung sind bei Patienten mit Mukoviszidose häufig. Akute Infektionen sind potenziell lebensbedrohlich. Die Patienten zeigen Fieber, Schüttelfrost, Dyspnoe, Zyanose, produktiven Husten sowie Anzeichen einer schweren systemischen Toxizität. Alveolarblutungen, Nekrosen und schließlich die Bildung von Kavernen sind häufig zu beobachten."},"es":{"name":"Neumonía causada por infección por pseudomonas aeruginosa","definition":"Es una enfermedad pulmonar poco frecuente caracterizada por neumonía primaria o no bacteriémica que se produce con mayor frecuencia en el contexto de cuidados intensivos, o una neumonía bacteriémica, y que suele estar asociada a neutropenia. La infección crónica de las vías respiratorias bajas con desarrollo de episodios de neumonía es común en pacientes con fibrosis quística. Las infecciones agudas son potencialmente mortales. Los pacientes presentan fiebre, escalofríos, disnea, cianosis y tos productiva, así como signos de toxicidad sistémica grave. Suele observarse hemorragias alveolares, necrosis y, finalmente, formación de cavidades."},"it":{"name":"Polmonite dovuta a infezione da Pseudomonas aeruginosa","definition":"Si tratta di una pneumopatia rara, caratterizzata da polmonite primaria o non batterica (che in genere si manifesta in un contesto di terapia intensiva), oppure da polmonite batterica, di solito associata a neutropenia. L'infezione cronica delle vie respiratorie inferiori con episodi di polmonite è comune nei pazienti con fibrosi cistica. Le infezioni acute sono potenzialmente letali. I pazienti presentano febbre, brividi, dispnea, cianosi, tosse produttiva e i sintomi di una grave tossicità sistemica. Sono comuni l'emorragia alveolare, la necrosi e la formazione di cavità."},"nl":{"name":"Pneumonie veroorzaakt door Pseudomonas aeruginosa-infectie","definition":"Een zeldzame aandoening van long, gekenmerkt door primaire of niet-bacteriëmische pneumonie die meestal ontstaat in een context van intensieve zorg, of bacteriëmische pneumonie die doorgaans geassocieerd is met neutropenie. Chronische infectie van onderste luchtwegen met ontwikkeling van episodes van pneumonie is gangbaar bij patiënten met cystische fibrose. Acute infecties zijn potentieel levensbedreigend. Patiënten presenteren zich met koorts, rillingen, dyspneu, cyanose, productieve hoest, alsook verschijnselen van ernstige systemische toxiciteit. Alveolaire hemorragie, necrose, en, tot slot, vorming van holtes, worden meestal waargenomen."},"pt":{"name":"Pneumonia por Pseudomonas aeruginosa serótipo O11","definition":"Doença pulmonar rara caracterizada por pneumonia primária ou não bacteriémica que surge habitualmente em ambiente de cuidados intensivos, ou de pneumonia bacteriémica habitualmente associada a neutropenia. A infeção crónica do trato respiratório inferior com o surgimento de episódios de pneumonia é comum em doentes com fibrose quística. As infeções agudas são potencialmente fatais. Os doentes apresentam febre, calafrios, dispneia, cianose, tosse produtiva, e sinais de toxicidade sistémica grave. Habitualmente também se observa hemorragia alveolar, necrose e, eventualmente, cavitação."},"pl":{"name":"Zapalenie płuc wywołane przez Pseudomonas aeruginosa"},"cs":{"name":"Pneumonie způsobená infekcí Pseudomonas aeruginosa"},"uk":{"name":"Пневмонія, спричинена інфекцією Pseudomonas aeruginosa","definition":"A rare pulmonary disease characterized by primary or nonbacteremic pneumonia most frequently arising in an intensive care setting, or bacteremic pneumonia, which is typically associated with neutropenia. Chronic lower respiratory tract infection with development of episodes of pneumonia is common in patients with cystic fibrosis. Acute infections are potentially life-threatening. Patients present with fever, chills, dyspnea, cyanosis, productive cough, as well as signs of severe systemic toxicity. Alveolar hemorrhage, necrosis, and, eventually, cavity formation, are commonly seen."},"zh":{"name":"铜绿假单胞菌感染性肺炎"},"tr":{"name":"Psödomonas aeruginosa enfeksiyonunun neden olduğu pnömoni"}},"xrefs":[{"source":"ICD-10","code":"J15.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA40.05","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"801706385"},{"source":"MONDO","code":"0037398","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0920182","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"13613","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":50.0,"area":"Europe","status":"Validated","source":"European Medicines Agency[OTHER]"}],"phenotypes":null}