Table 3.
Clinical characteristics of patients according to retinal lesions and presence or absence of proliferative retinopathy
| Variable | Normal retina (n = 21) | Retinal lesions (n = 19) | p value | PR− (n = 27) | PR+ (n = 13) | p value |
|---|---|---|---|---|---|---|
| Age (years) | 9.17 ± 4.64 | 19.05 ± 4.77 | 0.00** | 10.57 ± 5.12 | 20.69 ± 4.42 | 0.00** |
| Gendera | ||||||
| Male (n = 16) | 11 (52.4 %) | 5 (26.3 %) | 0.09 | 11 (40.7 %) | 5 (38.5 %) | 0.89 |
| Female (n = 24) | 10 (47.6 %) | 14 (73.7 %) | 16 (59.3 %) | 8 (61.5 %) | ||
| Genotypea | ||||||
| SS (n = 26) | 14 (66.7 %) | 12 (63.2 %) | 0.81 | 19 (70.4 %) | 7 (53.8 %) | 0.3 |
| S/β (n = 14) | 7 (33.3 %) | 7 (36.8 %) | 8 (29.6 %) | 6 (46.2 %) | ||
| Disease duration (years) | 6.26 ± 4.77 | 14.47 ± 4.9 | 0.00** | 7.43 ± 5.25 | 15.85 ± 4.3 | 0.00** |
| VOE/year | 1.62 ± 1.6 | 1.89 ± 1.37 | 0.56 | 1.7 ± 1.54 | 1.85 ± 1.41 | 0.78 |
| Hospitalization/year | 1.14 ± 1.6 | 1.0 ± 1.0 | 0.74 | 1.15 ± 1.51 | 0.92 ± 0.95 | 0.62 |
| Transfusion/year | 2.48 ± 2.66 | 2.37 ± 3.7 | 0.91 | 2.48 ± 3.12 | 2.31 ± 3.35 | 0.87 |
| Bone diseasea (n = 4) | 1 (4.8 %) | 3 (15.8 %) | 0.24 | 2 (7.4 %) | 2 (15.4 %) | 0.42 |
| PHTa (n = 3) | 3 (14.3 %) | 0 (0 %) | 0.08 | 3 (11.1 %) | 0 (0 %) | 0.21 |
| ACSa (n = 1) | 0 (0 %) | 1 (5.3 %) | 0.28 | 1 (3.7 %) | 0 (0 %) | 0.48 |
| Gall bladder diseasea (n = 1) | 0 (0 %) | 1 (5.3 %) | 0.28 | 0 (0 %) | 1 (7.7 %) | 0.14 |
| Splenectomya (n = 8) | 2 (9.5 %) | 6 (31.6 %) | 0.09 | 2 (7.4 %) | 6 (46.2 %) | 0.004** |
| Hydroxyureaa (n = 27) | 14 (66.7 %) | 13 (68.4 %) | 0.9 | 18 (66.7 %) | 9 (69.2 %) | 0.87 |
Values expressed as mean ± standard deviation unless mentioned otherwise
PR proliferative retinopathy, VOE vaso-occlusive events, PHT pulmonary hypertension, ACS acute chest syndrome
** Statistically significant (p < 0.05)
aValues expressed as absolute numbers and percentages