TABLE 1.
SUBJECT CHARACTERISTICS
|
P Value |
||||||
|---|---|---|---|---|---|---|
| HPS-1 (n = 7) | HPS-1 PF (n = 34) | NV (n = 85) | HPS-1 vs. HPS-1 PF | HPS-1 vs. NV | HPS-1 PF vs. NV | |
| Age, yr | 31.0 ± 4.2 | 38.6 ± 1.5 | 38.8 ± 1.4 | 0.054 | 0.134 | 0.947 |
| Sex, M/F | 5 / 2 | 11 / 23 | 55 / 30 | |||
| Current smokers | 0 | 0 | 0 | |||
| Former smokers | 0 | 3 | 6 | |||
| Dyspnea or cough | 1 | 27 | 0 | |||
| FEV1, % of predicted | 87.5 ± 4.3 | 85.6 ± 3.4 | 106.7 ± 1.9 | 0.803 | 0.004 | <0.001 |
| FVC, % of predicted | 82.1 ± 5.1 | 79.5 ± 3.4 | 101.5 ± 1.6 | 0.738 | <0.001 | <0.001 |
| TLC, % of predicted | 84.9 ± 5.6 | 78.8 ± 3.3 | 99.5 ± 1.5 | 0.478 | 0.006 | <0.001 |
| DlCO, % of predicted |
85.8 ± 7.7 |
75.0 ± 3.3 |
102.8 ± 1.8 |
0.186 |
0.009 |
<0.001 |
Definition of abbreviations: DlCO = diffusion capacity for carbon monoxide; F = female; HPS-1 = Hermansky-Pudlak syndrome type 1; HPS-1 PF = Hermansky-Pudlak syndrome type 1 with pulmonary fibrosis; M = male; NV = healthy research volunteer; TLC = total lung capacity.