TABLE 4.
PULMONARY FUNCTION STUDIES IN PATIENTS WITH SICKLE CELL DISEASE WITHOUT AND WITH PULMONARY HYPERTENSION
| Without PHT | With PHT | p Value | |
|---|---|---|---|
| No. of patients | 15 | 24 | |
| FEV1, L | 2.4 ± 0.1 (1.6, 3) | 1.8 ± 0.1 (0.8, 2.6) | < 0.001 |
| FEV1, % pred | 85.1 ± 3.2 (68, 108) | 70.6 ± 3.6 (27, 113) | 0.008 |
| FVC, L | 3.0 ± 0.1 (2, 3.9) | 2.4 ± 0.1 (0.9, 3.8) | 0.005 |
| FVC, % pred | 81.6 ± 2.3 (67.4, 103) | 70.2 ± 3.6 (24, 108) | 0.02 |
| FEV1/FVC ratio | 0.80 ± 0.02 (0.67, 0.91) | 0.78 ± 0.02 (0.65, 0.87) | 0.54 |
| TLC, L | 4.6 ± 0.2 (3.3, 5.7) | 4.0 ± 0.2 (2.2, 6.4) | 0.07 |
| TLC, % pred | 85.3 ± 2.9 (69.4, 106) | 79.9 ± 3.6 (38, 116) | 0.30 |
| FRC, L | 2.3 ± 0.1 (1.8, 3.1) | 2.8 ± 0.8 (1, 3.4) | 0.65 |
| FRC, % pred | 74.1 ± 3.6 (45, 93) | 67.8 ± 4.2 (31, 102) | 0.31 |
| RV, L | 1.5 ± 0.1 (0.7, 2.3) | 1.5 ± 0.1 (0.7, 2.6) | 0.78 |
| RV, % pred | 90.7 ± 6.7 (38, 132) | 97.5 ± 4.9 (59, 156) | 0.41 |
| RV/TLC ratio | 1.09 ± 0.06 (0.61, 1.46) | 1.29 ± 0.03 (0.98, 1.84) | 0.06 |
| DlCO, ml/min/mm Hg | 16.5 ± 1.2 (10.5, 27.2) | 13.7 ± 0.9 (4.9, 24.8) | 0.07 |
| DlCO, % pred | 65.8 ± 4.5 (48, 106) | 55.5 ± 3.3 (23.7, 85) | 0.07 |
| DlCOC, ml/min/mm Hg/L/Hb | 23.0 ± 2.0 (13, 41) | 18.5 ± 1.4 (6.4, 32) | 0.07 |
| DlCOC, % pred | 90.7 ± 6.5 (58, 135) | 71.9 ± 4.7 (31.6, 110.5) | 0.02 |
| Patients with positive methacholine test or clinically active asthma, n (%) | 3 (20%) | 4 (16.6%) |
Definition of abbreviations: DlCO = diffusion capacity for carbon monoxide; DlCOC = diffusion capacity for carbon monoxide corrected for hemoglobin level.
Data are presented as mean ± SEM (min, max).