Early detection of sickle cell disease (SS) done by neonatal screening will help in early prevention and management of complications in postnatal period.
Our objective was to find out prevalence of sickle cell anemia among the population of three districts (Kanker, Dantewada and Raigarh) of Chhattisgarh with clinical and hematological profile of sickle cell disease patients. A cross sectional study was done. A total of 15,701 persons collectively from three districts voluntarily attended the mobile camp and were screened for sickle cell anemia. First solubility test were done and were confirmed by Hb electrophoresis. The prevalence of sickle cell trait (HbAS) was 1,672 (10.6 %), sickle cell disease (HbSS) and inconclusive band was 97 (0.66 %). The HbSS and inconclusive band were subjected to HPLC. Among them 12 (0.076 %) cases were double heterozygous for Hb-S and beta thalassemia minor (SB+), 2 (0.012 %) cases were double heterozygous for Hb-S and Hb-E (S/HBE), 1 (0.006 %) case was double heterozygous for Hb-S and Hb-D Punjab (S/HBD) and 22 (0.14 %) cases had Hb-S with Hb-F level more than 20 % (SSF). Maximum number of HbSS cases were 13 (2.29 %) out of 567 children in the age group 0-5 years and HbAS cases were 124 (15.6 %) out of 794 persons in the age group 21-25 years. On comparison between vaso-occlusive and steady state, homozygous patients showed decrease in Hb, HCT, MCH, RBC in vaso-occlusive crises (p < 0.001) than steady state. Also there was one moderate negative correlation in number of blood transfusion (r = 0.46) with fetal hemoglobin (HbF) level. Patients with high HbF can have severe disease. This happens due to uneven distribution of fetal hemoglobin in F-cells with mean HbF remaining constant but in our study, those who had HbF level above 15-20 % were having fewer crises.
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