TABLE 1.
Clinical characteristics of SCD patients and controls. Results expressed in median and interquartile range unless otherwise indicated. Statistical comparison by t test or Fisher’s exact test
| N | SCD | N | Controls | P | |
|---|---|---|---|---|---|
| Demographics and genetics | |||||
| Age (years) | 497 | 12 (7–16) | 75 | 14 (9–17) | .021 |
| Female gender in no. (%) | 497 | 243 (48.9%) | 75 | 40 (53.3%) | .5 |
| Alpha-thalassemia in no. (%) | 466 | 66 | .5 | ||
| No alpha-globin gene deletion | 316 (67.5%) | 47 (71.2%) | |||
| Single alpha-globin deletion | 138 (29.5%) | 19 (28.8%) | |||
| Double alpha-globin deletion | 14 (3.0%) | 0(0%) | |||
| Medical history | |||||
| Asthma | 493 | 119 (24.1%) | 75 | 14 (18.7%) | .38 |
| Stroke | 492 | 52 (10.6%) | 75 | 0 | .001 |
| Acute chest syndrome or pneumonia | 492 | 239 (48.6%) | 75 | 2 (2.7%) | <.001 |
| Physical examination | |||||
| Body mass index (kg/m2) | 494 | 17.8(15.7–21.5) | 74 | 21.3 (17.8–24.3) | <.0001 |
| Systolic blood pressure (mmHg) | 480 | 112 (104–119) | 73 | 116 (109–125) | .001 |
| Diastolic blood pressure (mmHg) | 480 | 64(59–71) | 73 | 68 (62–75) | .008 |
| Echocardiogram and PFT | |||||
| Tricuspid regurgitation velocity (m/sec) | 441 | 2.3(2.1–2.5) | 67 | 2.1 (2.0–2.3) | <.0001 |
| Left ventricular internal diameter z score | 469 | 1.3 (0.2–2.2) | 71 | −0.5 (−1.3–0.1) | <.0001 |
| Left atrial diameter (cm) | 477 | 2.9 (2.5–3.3) | 73 | 2.7 (2.4–3.0) | .002 |
| Lateral E/e’ | 476 | 6.4 (5.5–7.4) | 73 | 6.1 (5.4–7.0) | .036 |
| FEV1/FVC (%) | 156 | 84 (80–89) | 34 | 85 (83–90) | .30 |
| Laboratory tests | |||||
| Oxygen saturation (%) | 472 | 98(97–99) | 71 | 99 (99–100) | <.0001 |
| eGFR (Schwartz)* (mL/min/1.73 m2) | 475 | 135(111–165) | 73 | 95 (80–110) | <.0001 |
| Absolute neutrophils (109 per L) | 478 | 4.5 (3.3–6.8) | 74 | 2.7 (2.0–3.7) | <.001 |
| Hemoglobin (g/L) | 480 | 91 (80–104) | 75 | 126 (121–138) | <.0001 |
| Reticulocytes (%) | 463 | 7.6 (4.0-11.6) | 63 | 1.0 (0.8-1-1.4) | <.0001 |
| Ferritin (μg/L) | 420 | 178 (85–620) | 70 | 43 (28–62) | <.0001 |
| Alanine aminotransferase (U/L) | 492 | 31 (18–42) | 75 | 22 (14–34) | .0005 |
| Asparate aminotransferase(U/L) | 480 | 42 (31–54) | 75 | 21 (17–29) | <.0001 |
| Total bilirubin (|imol/L) | 480 | 37.6 (23.9–56.4) | 75 | 8.6 (5.1–12.0) | <.0001 |
| Lactate dehydrogenase (U/L) | 437 | 398 (287–561) | 73 | 180 (152–209) | <.0001 |
| NT-proBNP (ng/L) | 456 | 85 (47–155) | 68 | 36 (20–48) | <.0001 |
Abbreviations: FEV1/FVC, forced expiratory volume in 1 minute to forced vital capacity ratio.
*
eGFR (estimated glomerular filtration rate) = 0.413 × (height in cm/creatinine in mg/dL).