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idiopathische pleuroparenchymale fibro-elastose (aandoening)
idiopathische pleuroparenchymale fibro-elastose
IPPFE
Idiopathic pleuroparenchymal fibroelastosis
Idiopathic pleuropulmonary fibroelastosis
IPPFE - idiopathic pleuroparenchymal fibroelastosis
A rare idiopathic interstitial pneumonia with characteristics of prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion and loss of volume) and reticulation.
Id1172606002
StatusPrimitive
Associated morphologyfibrose
Finding sitestructuur van pleura
Associated morphologyfibro-elastose
Finding sitestructuur van parenchyma pulmonalis
SNOMED CT to Orphanet simple map494428
SNOMED CT to ICD-10 extended map
TargetJ84.1
RuleTRUE
AdviceALWAYS J84.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified