Table I:
Baseline Demographics, Laboratory and Clinical Data of Patients in the Cohort
| Variable | N | All SCD | N | HbSS/HbSβ0 Thalassaemia | N | HbSC/HBSβ+ Thalasaemia |
|---|---|---|---|---|---|---|
| Age (years) | 331 | 29 (20, 41) | 236 | 28.0 (20.5, 39.5) | 89 | 35 (20, 43) |
| Female sex | 331 | 187 (56.5) | 236 | 133 (56.4) | 89 | 51 (57.3) |
| Weight (kg) | 323 | 67.9 (59.2, 80.1) | 230 | 65.5 (57.1, 75.8) | 87 | 75.6 (65.8, 92.9) |
| Height (cm) | 125 | 170.3 (162.5, 175.7) | 89 | 170.3 (163, 175.9) | 33 | 168 (162, 173) |
| Systolic blood pressure (mm Hg) | 329 | 121 (111, 132) | 236 | 119 (109, 130) | 87 | 128 (118, 134) |
| Diastolic blood pressure (mm Hg) | 329 | 71 (64, 79) | 236 | 70 (62, 76) | 87 | 77 (70, 83) |
| WBC count (109/l) | 330 | 10.2 (7.6, 12.5) | 236 | 10.8 (8.4, 13) | 88 | 8.6 (6.5, 10.5) |
| Haemoglobin (g/l) | 330 | 95 (81, 109) | 236 | 88 (78, 100) | 88 | 120 (105, 130) |
| Reticulocyte count (109/l) | 305 | 189.6 (117.3, 275.9) | 222 | 218.7 (148.5, 303.4) | 79 | 115.6 (83.2, 184.8) |
| Haemoglobin F (%) | 143 | 4.9 (1.9, 9.4) | 124 | 6.5 (3.2, 10.4) | 29 | 0.9 (0.5, 2.6) |
| Blood urea nitrogen (mmol/l) | 311 | 2.86 (2.14, 3.93) | 226 | 2.86 (2.14, 3.57) | 79 | 3.21 (2.50, 3.93) |
| Creatinine (μmol/l) | 331 | 61.88 (53.04, 79.56) | 236 | 61.88 (53.04, 75.14) | 89 | 70.72 (61.88, 88.40) |
| eGFR (ml/min/1.73m2) | 331 | 135 (113.9, 152.1) | 236 | 140.4 (120.1, 155.9) | 89 | 120.7 (105.9, 137.1) |
| Hyperfiltration* | 331 | 166 (52.2) | 236 | 138 (58.5) | 89 | 25 (28.1) |
| Lactate dehydrogenase (u/l) | 267 | 853 (623, 1155) | 189 | 943 (752, 1280) | 73 | 582 (508, 789) |
| Total bilirubin (μmol/l) | 297 | 32.50 (20.52, 56.44) | 213 | 41.04 (25.66, 73.55) | 78 | 17.10 (13.68, 25.66) |
| Indirect bilirubin (μmol/l) | 71 | 34.21 (20.52, 63.28) | 52 | 40.37 (29.08, 68.42) | 18 | 18.81 (13.68, 23.95) |
| Ferritin (μg/l) | 135 | 249 (71, 880) | 95 | 307 (124, 1340) | 37 | 122 (44, 322) |
| Specific gravity | 169 | 1.01 (1.01, 1.01) | 121 | 1.01 (1.01, 1.01) | 44 | 1.01 (1.01, 1.01) |
| Proteinuria (yes) | 180 | 30 (16.7) | 131 | 26 (19.9) | 45 | 4(8.9) |
| Haemoglobinuria (yes) | 167 | 15 (9.0) | 119 | 15 (12.6) | 44 | 0 (0) |
| Hydroxycarbamide use (yes) | 329 | 110 (33.4) | 234 | 98 (41.9) | 89 | 12 (13.5) |
| ACE-I/ARB therapy (yes) | 329 | 36 (10.9) | 235 | 24 (10.2) | 88 | 12 (13.6) |
| Chronic RBC transfusion (yes) | 331 | 13 (3.9) | 236 | 11 (4.7) | 89 | 2 (2.3) |
| History of diabetes (yes) | 331 | 15 (4.5) | 236 | 7 (3.0) | 89 | 8 (9.0) |
| History of acute chest syndrome (yes) | 314 | 254 (80.9) | 226 | 197 (87.2) | 82 | 55 (67.1) |
| History of stroke (yes) | 304 | 41 (13.5) | 216 | 36 (16.7) | 82 | 5 (6.1) |
| History of leg ulcers (yes) | 271 | 39 (14.3) | 197 | 38 (19.3) | 71 | 1 (1.4) |
| History of avascular necrosis (yes) | 233 | 91 (39.1) | 162 | 64 (39.5) | 66 | 25 (37.9) |
| History of priapism** (yes) | 117 | 45 (38.5) | 86 | 37 (43) | 30 | 8 (26.7) |
All SCD groups included 6 patients (SE = 2, SD = 2, SHPFH = 2) not categorized as mild (HbSC/HBSβ+ thalasaemia) or severe genotype (HbSS/HbSβ0 thalassaemia).
Continuous variables presented as median and interquartile range (IQR). Categorial variables presented as count (percentage) - N(%).
Hyperfiltration defined as: as eGFR > 130 ml/min per 1.73 m2 for women and > 140 ml/min per 1.73 m2 for men
Male subjects only.
ACE-I/ARB – angiotensin converting enzyme inhibitors/angiotensin receptor blocker; eGFR – estimated glomerular filtration rate; RBC – red blood cellSCD – sickle cell disease; SD - haemoglobin SD disease; SE - haemoglobin SE disease; SHPFH - sickle cell-hereditary persistence of fetal haemoglobin; WBC – white blood cell