Abstract

Background: Sickle cell disease is commonly seen in rural population of south part of Gujarat in India. It is one of the common causes of recurrent hospitalization, morbidity and mortality in pediatric population. This study was therefore undertaken to evaluate the clinical profile of sickle cell disease in a tertiary care hospital.Methods: This was the prospective observational study done from November 2015 to October 2016. All the hospitalized diagnosed case of sickle cell disease and trait in age group of 6 months to 14 years were taken in this study. Sickle cell disease with some genetic or metabolic disease and sickle-beta-thalassemia patients were not included in this study.Results: Total 61 patients were admitted over a one year of study period, out of which 47 were sickle cell disease and 14 sickle cell trait patients. Morbidity events were commonly observed in 5-12 years of age groups (68.85%). Seasonal variation also observed, 47.54% of total cases are seen in winter season. Pain (60.65%) was the most common presenting symptom. Severe pallor (39.34%) and splenomegaly (24.59%) was the most common sign in both groups. Vaso-occlusive crisis (59.01%) was the most common morbidity event observed, of which abdominal pain was the most common site of pain involvement. On laboratory analysis, there was statistically significant difference observed in disease and trait. In patients with sickle cell disease acute painful crisis (59.57%) was the common morbidity event observed while in sickle cell trait patients acute febrile illness (71.42%) observed.Conclusions: Vaso-occlusive crisis is the commonest manifestation in pediatric age group. Comprehensive medical care and management is required to decrease the morbidity and mortality.

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