Over the past 40 years, we observed 284 subjects with @entity13654 ( @entity13654 ) for 2,837 @entity1 -years. We examined the association of the course of clinical events with hematologic and genetic factors. The mean entry age was 21 years, although 15% entered before one year of age. The mean Hb concentration was 11.3 g/dL, the mean fetal hemoglobin was 2.5%, and the mean MCV was 84.4 fL. Twenty-five subjects died at a median age of 37 years. Chronic organ-specific complications occurred in 112 subjects (39.4%), with advanced @entity989 in 65 subjects (22.9%) and @entity2530 ( @entity2530 ) in 42 subjects (14.8%). We identified the beta-globin haplotypes in 82 subjects and the alpha-gene status in 79. Twenty-nine percent had @entity7723 . The beta(CI) haplotype was present in 85.4%. We found a decreased incidence of @entity989 in the beta(CI) subjects compared to the non-beta(CI) subjects (33% vs. 67%; P = 0.049) with a later mean onset age (29 years vs. 21 years; log-rank test, P= 0.026). We also found a consistent pattern of decreased morbidity in subjects who had @entity7723 in comparison to those who did not. We found a reduced risk of chronic organ-specific complications (log-rank test, P= 0.003), lower incidence of sickle crisis (48% vs. 80%, P= 0.001), later onset of @entity1085 (age of onset: 55 years vs. 34 years; P= 0.055), and lower risk of @entity2530 (log-rank test, P= 0.024). Our findings suggest that @entity13654 subjects who have not inherited @entity7723 might benefit from erythrocyte rehydration therapy.

Outcome in XXXX : a four-decade observational study of clinical, hematologic, and genetic factors.

@entity1 :: ('9606', 'Species') :: ['person'] @entity7723 :: ('MESH:D017085', 'Disease') :: ['alpha-thalassemia-2'] @entity1085 :: ('MESH:D005705', 'Disease') :: ['gallbladder disease'] @entity13654 :: ('MESH:D006450', 'Disease') :: ['hemoglobin SC disease', 'Hb SC'] @entity989 :: ('MESH:D012164', 'Disease') :: ['retinopathy'] @entity2530 :: ('MESH:D010020', 'Disease') :: ['osteonecrosis', 'avascular necrosis']
@entity13654 :: (MESH:D006450,Disease) :: ['hemoglobin SC disease']
