Comparison of the clinical severity of the disease in the patients homozygous for hemoglobin S with and without CD36 deficiency
| . | CD36− SS patients (n = 14) . | Matched CD36+ SS patients (n = 31) . | P . |
|---|---|---|---|
| Medical follow-up time, y, mean ± SD | 4.3 ± 3.0 (range, 1-12) | 5.5 ± 3.4 (range, 1-15) | .22 |
| Days per year of hospitalization for severe painful crises, mean ± SD5-150 | 3.5 ± 7.0 (range, 0-27) | 5.0 ± 6.4 (range, 0-25) | .27 |
| Frequency of acute and degenerative complications | |||
| Sepsis | 28.6% | 45.2% | .29 |
| Central nervous system damage | 7.1% | 6.5% | .99 |
| Acute chest syndrome | 28.6% | 25.8% | .99 |
| Priapism | 28.6% | 16.1% | .63 |
| Proliferative retinopathy | 14.3% | 9.7% | .64 |
| Aseptic necrosis of the head of long bones | 28.6% | 25.8% | .99 |
| Leg ulceration | 14.3% | 9.7% | .64 |
| Papillary necrosis | 0% | 12.9% | .29 |
| Clinical severity score, mean ± SD | 4.4 ± 2.7 | 3.7 ± 2.8 | .36 |
| . | CD36− SS patients (n = 14) . | Matched CD36+ SS patients (n = 31) . | P . |
|---|---|---|---|
| Medical follow-up time, y, mean ± SD | 4.3 ± 3.0 (range, 1-12) | 5.5 ± 3.4 (range, 1-15) | .22 |
| Days per year of hospitalization for severe painful crises, mean ± SD5-150 | 3.5 ± 7.0 (range, 0-27) | 5.0 ± 6.4 (range, 0-25) | .27 |
| Frequency of acute and degenerative complications | |||
| Sepsis | 28.6% | 45.2% | .29 |
| Central nervous system damage | 7.1% | 6.5% | .99 |
| Acute chest syndrome | 28.6% | 25.8% | .99 |
| Priapism | 28.6% | 16.1% | .63 |
| Proliferative retinopathy | 14.3% | 9.7% | .64 |
| Aseptic necrosis of the head of long bones | 28.6% | 25.8% | .99 |
| Leg ulceration | 14.3% | 9.7% | .64 |
| Papillary necrosis | 0% | 12.9% | .29 |
| Clinical severity score, mean ± SD | 4.4 ± 2.7 | 3.7 ± 2.8 | .36 |
SS indicates homozygous for hemoglobin S.
Severe painful crises are acute events requiring hospital admission and the administration of morphine.