Clinical phenotype of the study population with and without a history of intrapulmonary shunting
| . | Subjects with h/o IPS (mean ± SD) . | Subjects without known h/o IPS (mean ± SD) . | P value . |
|---|---|---|---|
| Number of subjects, N | 23 | 18 | |
| Age (y) | 34.5 ± 10.1 | 31.3 ± 9.4 | .216∗ |
| Sex | M = 15 / F = 8 | M = 5 / F = 13 | .017† |
| Total (mature erythrocyte + reticulocyte) Adhesion in normoxia | 965 ± 742 | 426 ± 796 | .005∗ |
| Total (mature erythrocyte + reticulocyte) Adhesion in hypoxia | 3321 ± 3785 | 1183 ± 1250 | .010∗ |
| Hgb (g/dL) | 8.3 ± 1.8 | 8.4 ± 1.1 | .864∗ |
| WBC (109/L) | 11.2 ± 2.8 | 10.1 ± 3.8 | .275‡ |
| ANC (106/L) | 6719 ± 2567 | 5937 ± 2815 | .359‡ |
| Platelet count (109/L) | 356.9 ± 123.3 | 316.0 ± 108.9 | .275‡ |
| MCV | 92.1 ± 9.9 | 93.6 ± 11.8 | .864∗ |
| Reticulocyte count (109/L) | 441.3 ± 190.1 | 294.2 ± 202.7 | .022‡ |
| LDH (U/L) | 495.4 ± 301.0 | 299.0 ± 147.1 | .004∗ |
| Ferritin (μg/L) | 2298 ± 2975 | 2272 ± 2608 | .747∗ |
| HbS (%) | 62.2 ± 22.2 | 55.2 ± 21.6 | .265∗ |
| HbA (%) | 25.8 ± 21.5 | 30.6 ± 24.5 | .532∗ |
| HbF (%) | 4.4 ± 4.6 | 6.7 ± 6.5 | .211∗ |
| Recently transfused (HbA > 10%) | 14/23 | 12/18 | .702† |
| h/o “in vivo” Hb desaturation (nocturnal or exertional) | 17/23 | 7/18 | .014† |
| . | Subjects with h/o IPS (mean ± SD) . | Subjects without known h/o IPS (mean ± SD) . | P value . |
|---|---|---|---|
| Number of subjects, N | 23 | 18 | |
| Age (y) | 34.5 ± 10.1 | 31.3 ± 9.4 | .216∗ |
| Sex | M = 15 / F = 8 | M = 5 / F = 13 | .017† |
| Total (mature erythrocyte + reticulocyte) Adhesion in normoxia | 965 ± 742 | 426 ± 796 | .005∗ |
| Total (mature erythrocyte + reticulocyte) Adhesion in hypoxia | 3321 ± 3785 | 1183 ± 1250 | .010∗ |
| Hgb (g/dL) | 8.3 ± 1.8 | 8.4 ± 1.1 | .864∗ |
| WBC (109/L) | 11.2 ± 2.8 | 10.1 ± 3.8 | .275‡ |
| ANC (106/L) | 6719 ± 2567 | 5937 ± 2815 | .359‡ |
| Platelet count (109/L) | 356.9 ± 123.3 | 316.0 ± 108.9 | .275‡ |
| MCV | 92.1 ± 9.9 | 93.6 ± 11.8 | .864∗ |
| Reticulocyte count (109/L) | 441.3 ± 190.1 | 294.2 ± 202.7 | .022‡ |
| LDH (U/L) | 495.4 ± 301.0 | 299.0 ± 147.1 | .004∗ |
| Ferritin (μg/L) | 2298 ± 2975 | 2272 ± 2608 | .747∗ |
| HbS (%) | 62.2 ± 22.2 | 55.2 ± 21.6 | .265∗ |
| HbA (%) | 25.8 ± 21.5 | 30.6 ± 24.5 | .532∗ |
| HbF (%) | 4.4 ± 4.6 | 6.7 ± 6.5 | .211∗ |
| Recently transfused (HbA > 10%) | 14/23 | 12/18 | .702† |
| h/o “in vivo” Hb desaturation (nocturnal or exertional) | 17/23 | 7/18 | .014† |