Table 1.
Summary of Demographic Data and Lung Function Tests of Study Subjects at Enrollment
| Group | N | Male Sex [n (%)] | Age (yr) [Median (IQR)] | % Predicted FVC [Median (IQR)] | % Predicted DlCO [Median (IQR)] |
|---|---|---|---|---|---|
| Healthy control subjects | 12 | 5 (42) | 41 (30–48) | ||
| Subjects with HPS | 66 | ||||
| Without clinical ILD | 17 | 6 (38) | 41 (30–47) | 90 (84–94) | 87 (82–98) |
| With clinical ILD | 49 | 19 (39) | 40 (33–48) | 71 (60–80) | 66 (53–77) |
| Longitudinal study | 42 | 14 (40) | 40 (33–46) | 72 (63–81) | 72 (54–80) |
Definition of abbreviations: DlCO = diffusion capacity for carbon monoxide; HPS = Hermansky-Pudlak syndrome; ILD = interstitial lung disease; IQR = interquartile range.