A Two Year Hospital Based Prospective Study of Sickle Cell Disorders in Children and Comparison With Other Hospital Based Regional Studies in India

Abstract

ABSTRACT:: Various haemoglobinopathies are major health problem in east godavari district, andhra pradesh. Because of high prevalence of these disorders, we undertook limited hospital based study. AIMS: To study and to evaluate the sickle cell disorders in children who attended outpatient pediatric department, GGH, Kakinada METHODS AND MATERIAL: The present prospective study included 82 patients, referred for screening of hemoglobin disorders from October 2010 to September 2012, with Clinical history of anaemia, jaundice, splenomegaly and fever. Blood samples were collected into EDTA for testing parameters like hemogram, peripheral smear, reticulocyte count, Hb F(fetal hemoglobin) study, osmotic fragility test, Hb H Inclusion Bodies, hemoglobin analysis by HPLC (high performance liquid chromatography) and urine was collected for urobilinogen test. RESULTS: Total numbers of sickle cell disease and trait were 31 out of 82 screened cases with male predominance and six cases were sickle heterozygous conditions. The prevalence of sickle cell disorders is 37%. 54% of sickle cell cases were associated with iron deficiency anemia. Serum bilirubin levels are increased more in sickle cell disease compared to other abnormal Hb disorders. CONCLUSIONS: Our study indicates that sickle cell disease and trait were most common haemoglobinopathies. 93% of sickle cell cases had presented with Microcytic hypochromic anemia. The prevalence of sickle cell disease is 37%, although the present study is Hospital based which cannot represent any community or population, but it emphasizes the need of knowing the magnitude of this problem in this region.