{"orpha":36238,"name":"Staphylococcal necrotizing pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Staphylococcal necrotizing pneumonia is a rare, bacterial, pulmonary infectious disease, caused by a Panton-Valentine leukocidin-producing Staphylococcus aureus strain, characterized by severe respiratory failure, extensive, rapidly progressing pneumonia and hemorrhagic lung necrosis. Patients typically present with influenza-like symptoms, such as fever, cough, and chest pain, as well as hemoptysis, hypotension, leukopenia, and severe respiratory symptoms that rapidly evolve to acute respiratory distress syndrome and septic shock. High mortality is associated.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-36238/","orphanet_url":"https://www.orpha.net/en/disease/detail/36238","names":{"fr":{"name":"Pneumonie nécrosante staphylococcique","definition":"La pneumonie nécrosante staphylococcique est une maladie infectieuse pulmonaire rare, d'origine bactérienne, causée par une souche de <i>Staphylococcus aureus</i> produisant de la leucocidine de Panton-Valentine. Elle se caractérise par une insuffisance respiratoire sévère, étendue, progressant rapidement vers une pneumonie et une nécrose pulmonaire hémorragique. Les patients présentent habituellement des signes évoquant une infection par le virus de la grippe, tels qu'une fièvre, une toux et une douleur thoracique, ainsi qu'une hémoptysie, une hypotension, une leucopénie, et des signes respiratoires sévères qui évoluent rapidement vers un syndrome de détresse respiratoire aigu et un choc septique. La maladie est associée à une mortalité importante."},"de":{"name":"Pneumonie, nekrotisierende, Staphylokokken-induzierte","definition":"Die Staphylokokken-induzierte nekrotisierende Pneumonie ist eine seltene, bakterielle, pulmonale Infektionskrankheit, die durch einen Panton-Valentin-Leukozidin-produzierenden Staphylococcus aureus-Stamm verursacht wird und durch schweres Atemversagen, ausgedehnte, schnell fortschreitende Pneumonie und hämorrhagische Lungennekrosen gekennzeichnet ist. Die Patienten präsentieren sich typischerweise mit grippeähnlichen Symptomen wie Fieber, Husten und Brustschmerzen sowie Hämoptyse, Hypotonie, Leukopenie und schweren respiratorischen Symptomen, die sich schnell zu einem akuten Atemnotsyndrom und septischem Schock weiterentwickeln. Eine hohe Sterblichkeit ist damit verbunden."},"es":{"name":"Neumonía necrotizante estafilocócica","definition":"La neumonía necrotizante estafilocócica es una enfermedad infecciosa pulmonar bacteriana poco frecuente causada por una cepa de Staphylococcus aureus productora de leucocidina de Panton-Valentine y caracterizada por un fallo respiratorio grave, extenso, rápidamente progresivo a neumonía y necrosis pulmonar hemorrágica. Típicamente, los afectados se presentan con síntomas similares a la gripe, tales como fiebre, tos y dolor torácico, así como hemoptisis, hipotensión, leucopenia y síntomas respiratorios graves que evolucionan rápidamente al síndrome de dificultad respiratoria aguda y choque séptico. Está asociado a una elevada mortalidad."},"it":{"name":"Polmonite necrotizzante da stafilococco","definition":"La polmonite necrotizzante da stafilococco è una malattia infettiva polmonare rara di origine batterica, causata dal ceppo dello Staphylococcus aureus che produce leucocidina di Panton-Valentine; è caratterizzata da grave insufficienza respiratoria, polmonite estesa a progressione rapida e necrosi polmonare emorragica. Di solito i pazienti presentano sintomi influenzali, come la febbre, la tosse e il dolore toracico, associati ad emottisi, ipotensione, leucopenia e sintomi respiratori gravi che evolvono rapidamente verso la sindrome da distress respiratorio acuto e lo shock settico. La mortalità è elevata."},"nl":{"name":"Door stafylokokken veroorzaakte necrotiserende pneumonie","definition":"Door stafylokokken veroorzaakte necrotiserende pneumonie is een zeldzame, bacteriële, pulmonale infectieziekte die wordt veroorzaakt door een <i>Staphylococcus aureus</i> stam die Panton-Valentine leukocidine produceert. De ziekte wordt gekarakteriseerd door ernstig respiratoir falen, uitgebreide en snel progressieve pneumonie, en hemorragische longnecrose. Patiënten vertonen doorgaans griepachtige symptomen, zoals koorts, hoest en pijn in de borst, alsook hemoptoë (bloedhoest), hypotensie, leukopenie, en ernstige respiratoire symptomen die snel evolueren naar 'acute respiratory distress'-syndroom en septische shock. Er is een hoge mortaliteit geassocieerd."},"pt":{"name":"Pneumonia necrotizante por estafilococos"},"pl":{"name":"Gronkowcowe martwicze zapalenie płuc"},"cs":{"name":"Stafylokoková nekrotizující pneumonie"},"uk":{"name":"Стафілококова некротична пневмонія","definition":"Staphylococcal necrotizing pneumonia is a rare, bacterial, pulmonary infectious disease, caused by a Panton-Valentine leukocidin-producing Staphylococcus aureus strain, characterized by severe respiratory failure, extensive, rapidly progressing pneumonia and hemorrhagic lung necrosis. Patients typically present with influenza-like symptoms, such as fever, cough, and chest pain, as well as hemoptysis, hypotension, leukopenia, and severe respiratory symptoms that rapidly evolve to acute respiratory distress syndrome and septic shock. High mortality is associated."},"zh":{"name":"葡萄球菌坏死性肺炎"},"tr":{"name":"Stafilokokal nekrotizan pnömoni"}},"xrefs":[{"source":"ICD-10","code":"J15.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CA40.06","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018183","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4706659","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":68416,"name":"Rare infectious disease"},"parents":[{"orpha":101944,"name":"Rare pulmonary disease","classifications":[184]},{"orpha":300579,"name":"Staphylococcal toxemia","classifications":[203]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2020-04-21","source":"28192581[PMID]_26785088[PMID]_24980464[PMID]_22388585[PMID]","terms":[{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032169","label":"Severe infection","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002789","label":"Tachypnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011949","label":"Acute infectious pneumonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031864","label":"Bacteremia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032308","label":"Increased circulating procalcitonin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011897","label":"Neutrophilia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031246","label":"Nonproductive cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032177","label":"Parenchymal consolidation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011919","label":"Pleural empyema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002878","label":"Respiratory failure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032016","label":"Abnormal sputum","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001289","label":"Confusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100758","label":"Gangrene","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001254","label":"Lethargy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001882","label":"Leukopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025439","label":"Pharyngitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002107","label":"Pneumothorax","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025419","label":"Pulmonary pneumatocele","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025144","label":"Shivering","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031273","label":"Shock","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030955","label":"Alcoholism","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000819","label":"Diabetes mellitus","frequency":"Very rare (<4-1%)","criteria":null}]}}