TABLE 1.
Demographic, clinical, biological and radiologic characteristics of the 15 sickle cell disease patients with functional brain imaging between January 1, 2017 and November 20, 2018.
| Summary statistics | |
|---|---|
| Age (years) | |
| Median [IQR] | 27 [22–35] |
| Range | 19-46 |
| Sex (n (%)) | |
| Male | 5 (33.3) |
| Female | 10 (66.7) |
| Genotype (n (%)) | |
| SS | 9 (60.0) |
| SC | 5 (33.3) |
| Sβ+ | 1 (6.7) |
| Disease-modifying therapy (n (%)) | |
| Hydroxyurea | 11 (73.3) |
| Chronic exchange transfusions | 4 (26.7) |
| Clinical sickle cell complications (n (%)) | |
| Acute chest syndrome | 7 (46.7) |
| Priapism a | 2 (40.0) |
| Sepsis | 0 |
| Stroke | 1 (6.7) |
| Pain | 15 (100.0) |
| Transfusion | 9 (60.0) |
| Avascular necrosis | 4 (26.7) |
| Sickle retinopathy | 6 (40.0) |
| Restrictive lung disease | 1 (6.7) |
| Pulmonary hypertension | 1 (6.7) |
| Leg ulcers | 0 |
| Neurosensitive hearing loss | 0 |
| Radiological findings on conventional MRI/MRA (n (%)) | |
| Silent cerebral infarcts | 3 (21.4) |
| White matter hyperintensities | 7 (50.0) |
| Atrophy | 1 (6.7) |
| Moya Moya | 1 (6.7) |
| Aneurysms | 2 (13.3) |
| No finding | 5 (33.3) |
| Laboratory parameters (mean ± SD, [range]) | |
| Hemoglobin (g/L) | 96 ± 16 [68–125] |
| Hematocrit (L/L) | 0.27 ± 0.05 [0.19–0.35] |
| MCV (fL) | 92 ± 14 [70–117] |
| LDH (U/L) | 397 ± 198 [174–814] |
| Reticulocytes (%) | 9.1 ± 6.4 [1.2–22.0] |
| WBC (109/L) | 7.4 ± 2.4 [3.0–11.8] |
| Platelets (109/L) | 308 ± 207 [72–875] |
| Bilirubin (mmol/L) | 45.8 ± 37.1 [7.0–159.0] |
| HbF (%) | 7.7 ± 6.2 [1.2–19.1] |
| Globular filtration rate (ml/min/1.73 m2) | 113 ± 12 [84–120] |
| Urine protein (g/L) | 0.11 ± 0.7 [0–0.28] |
| Systolic blood pressure (mmHg) (mean ± SD, [range]) | 120 (7) [103–131] |
| Sebastiani severity score (%) | 14.4 ± 10.9 [3.2–49.2] |
a
Only males experience priapism.
Missingness: bilirubin 2 (13.3%), glomerular filtration rate 2 (13.3%), HbF 2 (13.3%), hematocrit 2 (13.3%), hemoglobin 1 (6.7%), LDH 6 (40.0%), MCV 2 (13.3%), platelets 2 (13.3%), reticulocytes 3 (20%), urine protein 6 (40.0%), WBC 1 (6.7%).
Abbreviations: HbF, hemoglobin F; IQR, interquartile range; LDH, lactate dehydrogenase; MCV, mean corpuscular volume; WBC, white blood cells.