Baseline characteristics of the study population classified by phenotype of SCD
| . | SS-Sβ0 . | SC . | Sβ+ . | . |
|---|---|---|---|---|
| Hemoglobin phenotype . | N = 1751 . | N = 495 . | N = 161 . | P* . |
| Country | <.001 | |||
| Cameroon, n (%) | 898 (51.3) | 3 (0.6) | 22 (13.7) | |
| Ivory Coast, n (%) | 333 (19.0) | 194 (39.2) | 94 (58.4) | |
| Mali, n (%) | 520 (29.7) | 298 (60.2) | 45 (28.0) | |
| Clinical findings | ||||
| Male sex, n (%) | 809 (46.2) | 218 (44.0) | 67 (41.6) | .503 |
| Age, y, median (IQR) | 15.0 (9.0-23.0) | 21.0 (14.0-31.0) | 18.0 (13.0-29.0) | <.001 |
| Body mass index, kg/m2, median (IQR) | 16.8 (14.4-19.6) | 19.4 (16.6-22.4) | 18.7 (16.2-21.3) | <.001 |
| Icterus, n (%) | 949 (55.8) | 43 (8.9) | 22 (13.8) | <.001 |
| Medical history | ||||
| Age of clinical onset, median (IQR) | 4.0 (1.0-8.0) | 12.0 (6.0-20.0) | 8.0 (4.0-16.8) | <.001 |
| More than 1 VOC last year, n (%) | 858 (50.2) | 212 (44.4) | 47 (30.3) | <.001 |
| More than 1 ACS lifetime, n (%) | 201 (12.0) | 23 (4.9) | 13 (8.5) | <.001 |
| Stroke, lifetime, n (%) | 23 (1.3) | 2 (0.4) | 0 (0.0) | .156 |
| Leg ulcer, lifetime, n (%) | 166 (9.5) | 18 (3.6) | 6 (3.7) | <.001 |
| Priapism, lifetime, n (%)† | 126 (15.6) | 30 (13.7) | 4 (6.0) | .007 |
| Osteonecrosis, lifetime, n (%) | 209 (11.9) | 49 (9.9) | 19 (11.8) | .044 |
| Treatment | ||||
| Hydroxycarbamide, n (%) | 27 (1.5) | 6 (1.2) | 1 (0.6) | .777 |
| Blood transfusion history, n (%) | 20 (1.1) | 4 (0.8) | 0 (0) | .571 |
| Echocardiographic parameters | ||||
| LVEF, %, median (IQR) | 64.0 (59.0-71.0) | 69.0 (64.0-68.0) | 69.0 (66.0-74.3) | <.001 |
| TRV >2.5 m/s, n (%) | 95 (28.4) | 16 (20.0) | 4 (25.0) | .127 |
| Biological findings | ||||
| Hemoglobin, g/dL, median (IQR) | 7.9 (7.0-8.9) | 11.2 (10.4-12.1) | 10.9 (9.6-11.9) | <.001 |
| Reticulocytes, 103/μL, median (IQR) | 201 (147-252) | 61.5 (8-120) | 22 (5-145) | <.001 |
| Bilirubin, µmol/L, median (IQR) | 27.0 (18.0-39.0) | 11.5 (8.0-18.0) | 11.0 (7.0-20.0) | <.001 |
| LDH, UI/L, median (IQR) | 822 (554-1246) | 391 (280-537) | 344 (243-598) | <.001 |
| HbF, %, median (IQR) | 8.3 (5.0-13.0) | 1.0 (0.0-3.0) | 6.0 (4.0-8.3) | <.001 |
| Leukocytes, G/L, median (IQR) | 11.6 (9.1-14.8) | 7.5 (5.8-9.3) | 5.9 (4.6-8.2) | <.001 |
| Platelets, G/L, median (IQR) | 401 (292-517) | 304 (230-401) | 275 (200-372) | <.001 |
| Urine ACR >30 mg/g, n (%) | 573 (42.4) | 93 (21.2) | 26 (17.0) | <.001 |
| eGFR mL/min/1.73 m2, median (IQR) | 194 (154-248) | 118 (102-136) | 123 (109-149) | <.001 |
| Hemolysis index, median (IQR) | 0.38 (−0.1:1.0) | −1.03 (−1.5:−0.6) | −1.17 (−1.6: −0.6) | <.001 |
| . | SS-Sβ0 . | SC . | Sβ+ . | . |
|---|---|---|---|---|
| Hemoglobin phenotype . | N = 1751 . | N = 495 . | N = 161 . | P* . |
| Country | <.001 | |||
| Cameroon, n (%) | 898 (51.3) | 3 (0.6) | 22 (13.7) | |
| Ivory Coast, n (%) | 333 (19.0) | 194 (39.2) | 94 (58.4) | |
| Mali, n (%) | 520 (29.7) | 298 (60.2) | 45 (28.0) | |
| Clinical findings | ||||
| Male sex, n (%) | 809 (46.2) | 218 (44.0) | 67 (41.6) | .503 |
| Age, y, median (IQR) | 15.0 (9.0-23.0) | 21.0 (14.0-31.0) | 18.0 (13.0-29.0) | <.001 |
| Body mass index, kg/m2, median (IQR) | 16.8 (14.4-19.6) | 19.4 (16.6-22.4) | 18.7 (16.2-21.3) | <.001 |
| Icterus, n (%) | 949 (55.8) | 43 (8.9) | 22 (13.8) | <.001 |
| Medical history | ||||
| Age of clinical onset, median (IQR) | 4.0 (1.0-8.0) | 12.0 (6.0-20.0) | 8.0 (4.0-16.8) | <.001 |
| More than 1 VOC last year, n (%) | 858 (50.2) | 212 (44.4) | 47 (30.3) | <.001 |
| More than 1 ACS lifetime, n (%) | 201 (12.0) | 23 (4.9) | 13 (8.5) | <.001 |
| Stroke, lifetime, n (%) | 23 (1.3) | 2 (0.4) | 0 (0.0) | .156 |
| Leg ulcer, lifetime, n (%) | 166 (9.5) | 18 (3.6) | 6 (3.7) | <.001 |
| Priapism, lifetime, n (%)† | 126 (15.6) | 30 (13.7) | 4 (6.0) | .007 |
| Osteonecrosis, lifetime, n (%) | 209 (11.9) | 49 (9.9) | 19 (11.8) | .044 |
| Treatment | ||||
| Hydroxycarbamide, n (%) | 27 (1.5) | 6 (1.2) | 1 (0.6) | .777 |
| Blood transfusion history, n (%) | 20 (1.1) | 4 (0.8) | 0 (0) | .571 |
| Echocardiographic parameters | ||||
| LVEF, %, median (IQR) | 64.0 (59.0-71.0) | 69.0 (64.0-68.0) | 69.0 (66.0-74.3) | <.001 |
| TRV >2.5 m/s, n (%) | 95 (28.4) | 16 (20.0) | 4 (25.0) | .127 |
| Biological findings | ||||
| Hemoglobin, g/dL, median (IQR) | 7.9 (7.0-8.9) | 11.2 (10.4-12.1) | 10.9 (9.6-11.9) | <.001 |
| Reticulocytes, 103/μL, median (IQR) | 201 (147-252) | 61.5 (8-120) | 22 (5-145) | <.001 |
| Bilirubin, µmol/L, median (IQR) | 27.0 (18.0-39.0) | 11.5 (8.0-18.0) | 11.0 (7.0-20.0) | <.001 |
| LDH, UI/L, median (IQR) | 822 (554-1246) | 391 (280-537) | 344 (243-598) | <.001 |
| HbF, %, median (IQR) | 8.3 (5.0-13.0) | 1.0 (0.0-3.0) | 6.0 (4.0-8.3) | <.001 |
| Leukocytes, G/L, median (IQR) | 11.6 (9.1-14.8) | 7.5 (5.8-9.3) | 5.9 (4.6-8.2) | <.001 |
| Platelets, G/L, median (IQR) | 401 (292-517) | 304 (230-401) | 275 (200-372) | <.001 |
| Urine ACR >30 mg/g, n (%) | 573 (42.4) | 93 (21.2) | 26 (17.0) | <.001 |
| eGFR mL/min/1.73 m2, median (IQR) | 194 (154-248) | 118 (102-136) | 123 (109-149) | <.001 |
| Hemolysis index, median (IQR) | 0.38 (−0.1:1.0) | −1.03 (−1.5:−0.6) | −1.17 (−1.6: −0.6) | <.001 |
ACR, albumin/creatinine ratio; ACS, acute chest syndrome; LVEF, left ventricular ejection fraction; MCV, mean corpuscular volume; n, number; NA, not applicable.
P, bivariate comparison, analysis of variance for continuous variables, and χ2 test for discontinuous variables.
Percentages calculated for men and boys only.