Table 1.
Baseline characteristics of children with SCA enrolled in SPHERE
| All participants1 | Normal TCD | Elevated TCD | p value | |
|---|---|---|---|---|
| N | 196 | 149 | 47 | |
| Demographics | ||||
| Age at enrollment, mean±SD, years | 6.8±3.5 | 6.9±3.6 | 6.3±3.2 | 0.288 |
| Male, N (%) | 93 (47) | 71 (48) | 22 (47) | |
| Female, N (%) | 103 (53) | 78 (52) | 25 (53) | 1.000 |
| Intake physical examination | ||||
| Height, cm | 111.5±18.5 | 112.0±19.3 | 110.1±15.8 | 0.553 |
| Weight, kg | 18.6±6.6 | 19.0±6.7 | 17.6±6.2 | 0.213 |
| Splenectomy, N (%) | 1 (1) | 1 (1) | 0 (0) | 1.000 |
| Spleen palpable, N (%) | 49 (25) | 36 (24) | 13 (28) | 0.772 |
| Spleen palpable ≥5 cm, N (%) | 32 (16) | 23 (15) | 9 (19) | 0.146 |
| Oxygen saturation (%), mean±SD | 93.0±4.3 | 94.0±3.9 | 91.7±5.0 | 0.001 |
| Enlarged tonsillar tissue, N (%) | 149 (74) | 108 (73) | 41 (87) | 0.130 |
| Growth parameters, Z-score | ||||
| Weight-for-height | –1.08±1.36 | –1.01±1.46 | –1.31±0.94 | 0.066 |
| Height-for-age | –1.35±1.10 | –1.43±1.10 | –1.11±1.09 | 0.082 |
| Past medical history, N (%) | ||||
| Vaso-occlusive painful event | 182 (93) | 137 (92) | 45 (96) | 0.465 |
| Dactylitis | 150 (77) | 118 (79) | 32 (68) | 0.108 |
| Malaria | 173 (88) | 132 (89) | 41 (87) | 0.415 |
| Acute chest syndrome/pneumonia | 70 (36) | 53 (36) | 17 (36) | 1.000 |
| Splenic sequestration | 68 (35) | 54 (36) | 14 (30) | 0.708 |
| Previous stroke | 0 (0) | 0 (0) | 0 (0) | – |
| Bacteremia/sepsis | 73 (37) | 60 (40) | 13 (28) | 0.262 |
| Transfusion | 135 (69) | 100 (67) | 35 (74) | 0.372 |
| Past hydroxyurea use | 7 (4) | 6 (4) | 1 (2) | 1.000 |
| Lifetime hospitalizations, N (%) | ||||
| ≤5 hospitalizations | 135 (69) | 105 (70) | 30 (64) | 0.602 |
| 6–10 hospitalizations | 30 (15) | 21 (14) | 9 (19) | |
| >10 hospitalizations | 31 (16) | 23 (15) | 8 (17) | |
| Baseline parameters, mean±SD | ||||
| Hemoglobin, g/dL | 7.7±1.1 | 7.9±1.1 | 7.1±1.0 | <0.001 |
| MCV, fL | 78±10 | 79±10 | 82±9 | 0.037 |
| Fetal hemoglobin, % | 9.3±5.4 | 9.6±5.7 | 8.5±4.0 | 0.251 |
| ARC, ×103/L | 566±243 | 558±241 | 594±249 | 0.364 |
| WBC count, ×109/L | 14.5±5.4 | 14.2±5.8 | 15.3±3.7 | 0.251 |
| ANC, ×109/L | 5.5±2.3 | 5.4±2.3 | 5.9±2.3 | 0.172 |
| Platelets, ×109/L | 379±164 | 378±162 | 382±173 | 0.899 |
| ALT, U/L | 21±16 | 20±15 | 23±18 | 0.355 |
| Creatinine, mg/dL | 0.22±0.12 | 0.23±0.11 | 0.19±0.12 | 0.013 |
| TCD velocities, mean±SD | ||||
| TAMV, cm/s | 149±25 | 138±18 | 182±12 | <0.001 |
1
A total of 202 children were enrolled, but 6 were withdrawn following confirmatory diagnostic testing. MCV, mean corpuscular volume; WBC, white blood cell; ANC, absolute neutrophil count; ALT, alanine transferase.