Preferred Label : Panbronchiolitis, diffuse;
CISMeF acronym : DPB; PBLT;
Type : Phenotype or locus, molecular basis unknown;
Alternative titles and symbols : DPB; PBLT;
Description : Diffuse panbronchiolitis (DPB) is a rare chronic inflammatory obstructive pulmonary
disease primarily affecting the respiratory bronchioles. 'Diffuse' refers to the distribution
of the lesions throughout both lungs, and 'pan-' refers to the involvement of inflammation
in all layers of the respiratory bronchioles. Onset of the disorder occurs in the
second to fifth decade of life, and is clinically manifest by chronic cough, exertional
dyspnea, and sputum production. Most patients also have chronic paranasal sinusitis.
If untreated, the disorder progresses to bronchiectasis, respiratory failure, and
death (summary by Poletti et al., 2006).;
Inheritance : Multifactorial;
Laboratory abnormalities : Hypoxemia;
Prefixed ID : %604809;
Origin ID : 604809;
UMLS CUI : C0878555;
Automatic exact mappings (from CISMeF team)
Currated CISMeF NLP mapping
HPO term(s)
ORDO concept(s)
Semantic type(s)
UMLS correspondences (same concept)