Background Sickle cell disease is the commonest type of haemolytic anaemia in Sudan. It is commonly seen in those originating from western Sudan tribes, the affected children show various clinical presentations, laboratory findings, with varied complications and outcomes influenced by genetic, environmental and socioeconomic factors, which require a thorough assessment of patterns of presentations.
Objectives The aim of this study to determine the patterns of presentations of Sickle Cell Disease, in view of demographic data, clinical presentations, laboratory findings and the short outcome.
Methods This a prospective cross-sectional hospital based study, conducted in Ahmed Gasim Specialized Hospital for Children, (Khartoum North,Sudan) during the period from 1/11/2015 to 31/1/2016, covered all patients with Sickle cell disease, their ages were less than 18 years. Data collected by specifically designed questionnaires to fulfil the objectives of this study.
Results One hundred thirty eight patients were studied, aged between 9 months and 17 years with a mean age of 6.26 years (±4.44 SD). Nearly 93% of patients were belonging to western tribes of Sudan. The prevalence of sickle cell disease patients were 2.6% of total admission and 0.7% of the total number of patients seen in the Emergency Room in Ahmed Gasim Specialized Hospital for Children. Also anaemia due to Sickle Cell Disease comprises 43.9% of total anaemic patients. Seventy percent of patients were diagnosed during the first year of life. Painful crises were observed in 59%, haemolytic crises seen in 20.4%, sickle cell hepotpathy in 4.3%, cerebrovascular accidents seen 1.4%, sequestration crises in 0.7%. Ninety fifth percent of patients presented with fever, 71% presented with fatigability, pain reported in 69.6%, jaundice in 52% and limb swelling in 51.4%. Pallor indicative of anaemia is seen in all (100%) of patients, tachycardia seen in 91% and hepatomegaly detected in 63.8%. Growth failure observed in 51.45%. All study population had low haemoglobin, ranging from 2.9 - 9.5 gm/dl with a mean 7.01 gm/dl (±1.1 SD), Reticulocytosis observed in 18.8% and Leucocytosis in 85.5%. Ninety percent recovered from their acute episodes, (5%) were referred to other hospital, only three patients (2.2%) leaved against medical advice, two patients (1.4%) discharged home with complications, there were two deaths (1.4%).
Conclusions The study exemplifies different patterns of presentations in patients with Sickle cell disease with variable percentage. It is suggested to manage considering that patterns and applying the multi-disciplinary approach.
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