{"orpha":91359,"name":"Chronic pneumonitis of infancy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["CPI"],"definition":"Chronic pneumonitis of infancy is a rare pediatric form of interstitial lung disease (ILD).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91359/","orphanet_url":"https://www.orpha.net/en/disease/detail/91359","names":{"fr":{"name":"Pneumopathie chronique de l'enfance","synonyms":["CPI"],"definition":"La pneumopathie chronique de l'enfance est une forme pédiatrique rare de pneumopathie interstitielle."},"de":{"name":"Pneumonie, chronische, des Kindesalters","synonyms":["CPI"],"definition":"Die chronische Pneumonitis des Kleinkindalters ist eine seltene pädiatrische Form der interstitiellen Lungenerkrankung (ILD; s. dort)."},"es":{"name":"Neumonitis crónica del lactante","synonyms":["CPI"],"definition":"La neumonitis intersticial crónica del lactante constituye una variante rara de las enfermedades pulmonares intersticiales de la infancia (EPI)."},"it":{"name":"Polmonite cronica dell'infanzia","synonyms":["CPI"]},"nl":{"name":"Chronische pneumonitis op zuigelingenleeftijd","synonyms":["CPI"],"definition":"Chronische pneumonitis op de kinderleeftijd is een zeldzame pediatrische vorm van interstitiële longziekte (ILD; zie deze term)."},"pt":{"name":"Pneumonite crónica da infância","definition":"A pneumonite crónica da infância é uma forma pediátrica rara de doença pulmonar intersticial (ILD, ver este termo)."},"pl":{"name":"Przewlekłe dziecięce zapalenie płuc","synonyms":["CPI"]},"cs":{"name":"Chronická pneumonitida kojenců","synonyms":["CPI"]},"uk":{"name":"Хронічний пневмоніт немовлят","synonyms":["CPI"],"definition":"Chronic pneumonitis of infancy is a rare pediatric form of interstitial lung disease (ILD, see this term)."},"zh":{"name":"婴儿期慢性肺炎","synonyms":["CPI"]},"tr":{"name":"Süt çocuğunda kronik pnömoni","synonyms":["CPI"]}},"xrefs":[{"source":"ICD-10","code":"J84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB04.6","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1821220054"},{"source":"MONDO","code":"0019621","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3872848","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":264694,"name":"Interstitial lung disease specific to infancy","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Infancy","Neonatal"],"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":"2021-10-13","source":"30505512[PMID]_25755194[PMID]","terms":[{"hpo":"HP:0012735","label":"Cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002207","label":"Diffuse reticular or finely nodular infiltrations","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025179","label":"Ground-glass opacification on pulmonary HRCT","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002883","label":"Hyperventilation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032341","label":"Reduced forced vital capacity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000961","label":"Cyanosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030864","label":"Intercostal retractions","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002789","label":"Tachypnea","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Very rare (<4-1%)","criteria":null}]}}