Demographic and clinical characteristics of IDPO patients
| No of case and data on diagnosis | |
| Sex | |
| Male | 18 (82%) |
| Female | 4 (18%) |
| Average age at diagnosis of DPO (years) | 37.9 (9.1) |
| Diagnostic opportunity | |
| Medical check-up | 17 (77%) |
| Examination for respiratory symptoms* | 4 (18%) |
| Accidentally while observing other diseases | 1 (5%) |
| Smoking status | |
| Never | 18 (82%) |
| Former | 4 (18%) |
| Family history | 2 (9%) |
| Medical history of lung disease | |
| Pneumothorax | 1 (5%) |
| Infantile asthma | 1 (5%) |
| Pulmonary function test on diagnosis | |
| FVC (mL) | 3379 (741) |
| FVC, %predicted (%) | 85.0 (17.4) |
| FEV1 (mL) | 2729 (641) |
| FEV1, %predicted (%) | 80.0 (18.0) |
| FEV1/FVC (%) | 80.5 (7.4) |
| DLCO, %predicted (%) | 74.9 (18.3) |
| FVC, %predicted <80% | 8 (36%) |
| FEV1/FVC (%) <70% | 2 (9%) |
| DLCO, %predicted (%) <80%† | 10 (63%) |
| KL-6 (U/mL)‡ | 349.0 (231.2) |
| KL-6 > 500 (U/mL) | 3 (17%) |
Data are presented as mean (SD) or n of 22 IDPO cases (%).
*Respiratory symptoms; cough:4, dyspnoea on exertion:3, sputum:2 (multiple answers allowed).
†n=16
‡n=18.
DLco, diffusing capacity of the lung for carbon monoxide; DPO, dendriform pulmonary ossification; FEV1, forced expiratory volume in 1 s; FVC, forced vital capacity; IDPO, idiopathic DPO.