SICKLE CELL DISEASE IN BIHAR: AN EXPERIENCE OF TERTIARY HEALTH INSTITUTES.

Abstract

Sickle cell disease is commonly seen in rural population of western part of India. It is one of the common causes of recurrent hospitalization, morbidity and mortality in paediatric population. As there are limited studies addressing the pattern of sickle cell disease amongst paediatric population in Bihar, This study was taken up to evaluate the clinic-haematological prole of paediatric population with sickle cell in tertiary health centres. METHODS: This was a retrospective observational study. Data were retrieved from haemato-pathology departments of tertiary care centres of Bihar. Data of children diagnosed with sickle cell disease from January 2019 to January 2021 were collected and analyzed to assess the , clinical ,haematological and HPLC prole at the time of diagnosis. RESULTS: 10 patients were included in the study. Clinically, Vasoocclusive crisis was the most common presentation (43.93 %) followed by generalized body ache and joint pain (36.99 %) and acute febrile illness (26.39 %), while 3 (30%) patients presented with severe anemia. Haematological nding was suggestive of moderate anemia, low Mean corpuscular volume and low Mean hemoglobin concentration. CONCLUSION: At the time of diagnosis vasoocclusive crisis and generalized bodyache were the most common manifestations in patients with sickle cell disease while haematological picture was suggestive of microcytic hypochromic moderate anemia. All cases were conrmed with HPLC.There was a positive correlation between age at presentation and severity of anemia at the time of diagnosis.