Diffuse panbronchiolitis

General Information (adopted from Orphanet):

Synonyms, Signs: DPB
PBLT
Number of Symptoms 9
OrphanetNr: 171700
OMIM Id: 604809
ICD-10:
UMLs: C0878555
MeSH: C536174
MedDRA: 10062952
Snomed: 430476004

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Multifactorial
[Orphanet]
Age of onset: Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Rare pulmonary disease
 -Rare respiratory disease

Symptom Information: Sort by abundance 

1
(HPO:0012418) Hypoxemia 18 / 7739
2
(HPO:0002110) Bronchiectasis 73 / 7739
3
(HPO:0012735) Cough 24 / 7739
4
(HPO:0003676) Progressive disorder 148 / 7739
5
(OMIM) Paranasal sinusitis, chronic 1 / 7739
6
(OMIM) Obstructive respiratory impairment 3 / 7739
7
(OMIM) Sputum production 3 / 7739
8
([DEL]MedDRA:10011224) Cough 70 / 7739
9
(MedDRA:10047924) Wheezing 8 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) 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 ...
Clinical Description OMIM Yamanaka et al. (1969) first described a chronic airway disease in Japan. They termed it diffuse panbronchiolitis to distinguish it from chronic bronchitis.

Homma et al. (1983) reported numerous Japanese patients with diffuse panbronchiolitis. Patients with ...

Population genetics OMIM Diffuse panbronchiolitis predominantly affects East Asians, occurring with a frequency of 11 per 100,000 in the Japanese population (Homma et al., 1983). The disease has also been reported in Korea (Kim et al., 1992) and China (Chu et ...