A study of spectrum of sickle cell anemia and thalassemia in a teaching institute of South India.

BACKGROUND AND AIM

Sickle cell syndrome is a group of inherited hematological disorders with varying degrees of anemia, jaundice, fatiguability along with hepatomegaly and splenomegaly. The clinical presentations can be may vary and therefore require thorough investigations. We tried to evaluate the spectrum of sickle cell anemia and thalassemia in pediatric patients of our hospital.

PATIENTS AND METHODS

In this cross-sectional study, A total of n = 200 consecutive cases were detected during the period of study. A thorough history and detailed clinical examination were done. Hb electrophoresis was done in the present study using HYDRASYS ® Electrophoresis Systems from Sebia.

RESULTS

The overall prevalence of SCD in our study was 6.83% the existence of this is found to be greater in the males as compared to females which is in agreement with prevalence across India with more male than female. Thalassemia was prevalent at the rate of 3.96%, sickle cell anemia had a prevalence of 1.98% sickle thalassemia was 0.89%. N = 20 pairs of Parents recognized genetic counseling i.e., with a single child or who wanted further children readily underwent HPLC analysis.

CONCLUSION

The existence of SCD in our study group is lesser as compared to the South India average. Preventive programs consisting of public education, population screening, genetic counseling, and prenatal diagnosis have been very effective in reducing both rates of β-Thalassemia major. Sickle cell anemia is of prime importance because of its high prevalence, morbimortality and the absence of curative treatments.