RESUMO
OBJECTIVE: To study the epidemiology of sickle cell disease in pediatric age group in a rural hospital of Central India. DESIGN: Prospective descriptive hospital based study. SUBJECTS: 99 admitted patients of sickle cell disease were studied for a period of 1 year. RESULTS: Prevalence of sickle cell disease was 5.7% (99/1753) hospitalizations of which 61.6% (n=61) had homozygous sickle cell disease (HbSS) whereas 38.4% (n=38) had heterozygous state (HbAS). Of these, 62 (63%) were below five years of age. Male : Female ratio was 1.65:1 in HbSS cases and 1.71:1 in HbAS cases. History of consanguinity was present in 7 (7%) of which 5 (8.2%) had HbSS and 2 (5.2%) had HbAS. Incidence was maximum in the Mahar community (70%) followed by Kunbi (8 %) and Teli (6%). Vascular occlusive crisis (23. 3%) was the commonest crisis encountered followed by hyperhemolytic crisis (16.3%). There was no correlation between hemoglobin levels and the occurrence of vascular occlusive crisis. Maximum cases required their first blood transfusion between second and third year of age. Requirement of blod transfusion was more in HbSS cases. Four patients died of which three had HbSS and were below five years of age. Splenic sequestration crisis was the commonest cause of death. CONCLUSION: Sickle cell disease is prevalent in this area and most cases present before 5 year of age. VOC is the commonest crisis seen, but death often occurs due to sequestration crisis and usually below 5 years of age.