Variable | Sickle cell disease (n = 66) |
---|---|
Age (year); mean ± SD | 15.68 ± 7.02 |
Male: female, n (%) | 35 (53.0%): 31 (47.0%) |
Positive family history of SCD, n (%) | 43 (65.2%) |
Clinical characteristics | |
Splenectomized, n (%) | 13 (20.0%) |
Number of sickle crisis/year; median (IQR) | 4 (2–8) |
Sickle crisis ≥ 3/year, n (%) | 35 (54.7%) |
History of silent or manifest stroke, n (%) | 9 (14.1%) |
History of acute chest syndrome, n (%) | 12 (18.8%) |
Cardiopulmonary complications, n (%) | 10 (15.6%) |
Transfusion index (mL/kg/year) | 120 (60–240) |
Iron overload per day (mg/kg); mean ± SD | 0.23 ± 0.15 |
On chelation therapy, n (%) | 53 (80.3%) |
Poor compliance to chelation, n (%) | 21 (42.0%) |
Laboratory characteristics | |
Pre-transfusion hemoglobin (g/dL); mean ± SD | 8.03 ± 1.42 |
HbS (%); mean ± SD | 61.33 ± 20.70 |
HbF (%); median (IQR) | 4.7 (1.3–12.2) |
Serum amylase (U/L); mean ± SD | 56.73 ± 21.43 |
Serum ferritin (ug/L); median (IQR) | 2805 (median 940–4638) |
Serum ferritin level > 2500; n (%) | 33 (50%) |
Radiological characteristics | |
LIC (mg/g liver dry weight); median (IQR) | 11.63 (5.81–20.31) |
Normal; n (%) | 7 (10.6%) |
Mild; n (%) | 16 (24.2%) |
Moderate; n (%) | 24 (36.4%) |
Severe; n (%) | 19 (28.8%) |
Cardiac T2* (msec); mean ± SD | 31.40 ± 6.58 |
Normal; n (%) | 66 (100%) |
Pancreatic MRI (msec); median (IQR) | 53.80 (35.35–84.45) |
Normal; n (%) | 14 (21.2%) |
Mild; n (%) | 43 (65.1%) |
Moderate; n (%) | 8 (12.1%) |
Severe; n (%) | 1 (1.6%) |