Table 1 Baseline characteristics
|  | Total (n = 90) | CRS (n = 25) | No CRS (n = 65) | OR | p-value |
|---|---|---|---|---|---|
Median age (yrs) (range) | 29 (16–58) | 30 (21–57) | 28 (16–58) |  | 0.256 |
Sex (%) | Â | Â | Â | 0.64 | 0.425 |
 Male | 31 (34.4) | 7 (28.0) | 24 (36.9) |  |  |
 Female | 59 (65.6) | 18 (72.0) | 41 (63.1) |  |  |
Mean arterial pressure (mmHg) (±SD) | 76.3 (±8.8) | 74.2 (±6.6) | 77.1 (±9.5) |  | 0.262 |
Diabetes mellitus (%) | 5 (5.6) | 2 (8.0) | 3 (4.6) | 1.80 | 0.615 |
Hypertension (%) | 0 | 0 | 0 | Â | Â |
Dyslipidemia (%) | 0 | 0 | 0 | Â | Â |
Hypothyroidism (%) | 26 (28.9) | 7 (28.0) | 19 (29.2) | 0.94 | 0.908 |
Adrenal insufficiency (%) | 7 (7.8) | 4 (16.0) | 3 (4.6) | 3.94 | 0.09 |
Thalassemia type (%) | |||||
 β0/βE thalassemia | 57 (63.3) | 11 (44.0) | 46 (70.8) | 0.325 | 0.018 |
 β0/β0 thalassemia | 26 (28.9) | 12 (48.0) | 14 (21.5) | 3.36 | 0.013 |
 HemoglobinH disease | 7 (7.8) | 2 (8.0) | 5 (7.7) | 1.043 | 0.961 |
Pre-transfusion Hemoglobin (g/dL) mean (±SD) | 7.25 (±1.04) | 7.18 (±1.14) | 7.28 (±1.01) |  | 0.70 |
Splenectomy (%) | Â | Â | Â | 2.71 | 0.055 |
 Yes | 54 (60.0) | 19 (76.0) | 35 (53.8) |  |  |
 No | 36 (40.0) | 6 (24.0) | 30 (46.2) |  |  |
Transfusion-dependent (%) | Â | Â | Â | 2.88 | 0.219 |
 Yes | 75 (83.3) | 23 (92.0) | 52 (80.0) |  |  |
 No | 15 (16.7) | 2 (8.0) | 13 (20.0) |  |  |
Presence of EMH (%) | Â | Â | Â | 4.59 | 0.02 |
 Yes | 31 (34.4) | 15 (60.0) | 16 (24.6) |  |  |
 No | 59 (65.6) | 10 (40.0) | 49 (75.4) |  |  |
Ferritin (ng/mL) mean (±SD) | 1783.2 (±1313.7) | 1577.6 (±1133.2) | 1877.0 (±1376.7) |  | 0.30 |
Hemochromatosis (%) | Â | Â | Â | 2.60 | 0.047 |
 Yes | 42 (46.7) | 16 (64.0) | 26 (40.0) |  |  |
 No | 48 (53.3) | 9 (36.0) | 39 (60.0) |  |  |
Mean liver iron concentration (mg/g) (±SD) | 13.78 (±5.89) | 13.26 (±6.24) | 14.02 (±5.80) |  | 0.70 |
Mean cardiac T2 star (ms) (±SD) | 37.20 (±12.23) | 36.76 (±16.37) | 37.42 (±9.98) |  | 0.96 |
Type of iron chelation (%) | |||||
 Deferoxamine (DFO) | 41 (45.6) | 12 (48.0) | 29 (44.6) | 1.15 | 0.773 |
 Deferiprone (DFP) | 65 (72.2) | 18 (72.0) | 47 (61.5) | 0.99 | 0.977 |
 Deferasirox (DFX) | 13 (14.4) | 2 (8.0) | 11 (16.9) | 0.43 | 0.281 |
 Combined DFO + DFP | 25 (27.8) | 7 (28.0) | 18 (27.7) | 1.01 | 0.977 |
 Combined DFP + DFX | 4 (4.4) | 0 (0) | 4 (6.2) | – | 0.573 |
Presence of pulmonary hypertension (%) |  |  |  | 25.50 | < 0.001 |
 Yes | 17 (18.9) | 14 (56.0) | 3 (4.6) |  |  |
 No | 73 (81.1) | 11 (44.0) | 62 (95.4) |  |  |
Electrocardiogram (%) | Â | Â | Â | Â | 0.138 |
 Normal sinus rhythm | 63 (70.0) | 15 (60.0) | 48 (73.9) |  |  |
 Abnormal rhythm | 7 (7.8) | 1 (4.0) | 6 (9.2) |  |  |
 ST segment change | 20 (22.2) | 9 (36.0) | 11 (16.9) |  |  |
Echocardiogram mean (±SD) | |||||
 Left ventricular ejection fraction [LVEF], (mmHg) |  | 64.8 (±8.17) | 65.0 (±4.67) |  | 0.949 |
 Right ventricular systolic pressure [RVSP], (mmHg) |  | 67.1 (±35.68) | 49 (±2.82) |  | 0.509 |
 Mean pulmonary arterial pressure [mPAP], (mmHg) |  | 50.1 (±42.2) | 23.0 (±5.0) |  | 0.324 |
 Tricuspid velocity [TV], (m/s) |  | 329.7 (±95.7) | 256.7 (±48.6) |  | 0.090 |
E/A ratio of mitral valve |  | 1.21 (±0.31) | 1.55 (±0.54) |  | 0.139 |
E/E’ ratio of mitral valve |  | 11.9 (±2.2) | 11.7 (±3.0) |  | 0.899 |
Serum NT-proBNP (ng/ml) median (IQR) | 137.01 (78.3–195.8) | 141.6 (65.3–217.9) | 99.47 (37.5–161.4) |  | 0.028 |
Mean serum Creatinine (mg/dl) (±SD) | 0.57 (±0.17) | 0.55 (±0.2) | 0.58 (±0.15) |  | 0.37 |
Different in serum creatinine at 0 and 3 months (mg/dl) mean (±SD) | 0.0689 (±0.055) | 0.0688 (±0.057) | 0.069 (±0.055) |  | 1.00 |
24 h urine median (IQR) | |||||
 Protein (mg/24 h.) | 193.0 (103.0-183.0) | 273.0 (186.2–359.8) | 175.0 (102.8–247.2) |  | < 0.001 |
 Potassium (mmol/24 h.) | 26.9 (17.8–36.0) | 30.3 (19.0–41.6) | 25.6 (17.4–33.9) |  | 0.214 |
 Phosphorus (mg/24 h.) | 471.7 (309.4–634.1) | 449.9 (213.3–686.5) | 486.4 (332.5–640.5) |  | 0.93 |
 Magnesium (mEq/24 h.) | 4.1 (2.8–5.4) | 5.48 (3.9–7.0) | 3.85 (2.7–5.0) |  | 0.008 |
 Calcium (mg/24 h.) | 58.9 (13.1–104.7) | 50.8 (1.8–99.8) | 72.85 (26.6–119.1) |  | 0.247 |
Mean urinary NGAL (ng/ml) median (IQR) | 9.55 (3.8–15.3) | 11.67 (3.5–19.9) | 9.0 (4.4–13.6) |  | 0.143 |
Chronic Urinary NGAL > 5 ng/ml (%) |  |  |  | 2.82 | 0.038 |
 Yes | 49 (54.4) | 18 (72.0) | 31 (47.7) |  |  |
 No | 41 (45.6) | 7 (28.0) | 34 (52.3) |  |  |