Abstract

Introduction: In the world, Sickle cell disease (SCD) is the commonest genetic disease. The most common single gene disorders are Haemoglobinopathies. 59th World Health Assembly in 2006, sickle-cell disease (SCD) recognized by WHO as an inherited disorder of hemoglobin as a priority of public health. Sickle hemoglobin (HbS) is abnormal hemoglobin difference in which adenine is substituted by thiamine. Deoxygenating of red blood cells form tactoid aggregates and distorts RBCs into sickle shape in HbS. repeated sickling and desickling RBCs convert into permanent sickle shape which can occur sickle red blood cell anemia, crisis and organ damage. There is extremely variable in Hematological profiles of sickle cell disease. There are very few studies are available on hematological profiles of sickle cell disease. Individuals express the homozygous form (HbSS) manifest the disease whereas with the heterozygous form (HbAS), also known as sickle-cell trait (SCT), are usually asymptomatic carriers. Initially Sickle cell disease is limited to sub-Saharan Africa, Middle-East and some parts of India. However nowadays it is spread to all continents with the migration of populations. In India, sickle cell disease (SCD) is common in Chhattisgarh, Madhya Pradesh, Gujarat, Orissa, Vidarbha, Andhra Pradesh and Tamil Nadu. For sickle cell disease there is more need for hospital care in children than adult patients. Children younger than five years of age suffering from SCD especially with age of 1-3 years are most endangered to mortality. Aim: The main aim of this study is to study hematological profiles clinical manifestation of children with sickle cell disease. Material and method: This is cross- section study which is done in the Department of Paediatrics at Surat Municipal Institute of Medical education and research Gujarat (SMIMER) and from 2016 to 2017 during the period of one year. This study was carried out on patients between the age group 6 months to 14 year old which found with diagnosed of sickle cell disease on routine blood investigations and further investigations were carried out to ascertain the cause of sickle cell. Result: A total of 60 patients with different age group from 6 months to 14 years were admitted during the study period that fulfilled the inclusion criteria. Out of 60 patients 46 were homozygous sickle cell disease and 14 heterozygous sickle cell trait patients. Total 38 (63.3%) male patients and 22(36.7%) female patients were included. Conclusion: Child presenting for detecting sickle cell with the symptom like abdominal pain, musculoskeletal pain, pallor, icterus, splenomegaly and hepatomegaly should lead to high suspicion of sickle cell where its prevalence is more. in pediatric age group commonest manifestation like hemolytic in nature, hematological parameters were suggestive of hypochromic microcytic anemia. In patients with sickle cell anemia, parental counseling and preventive measures like management of pain with simple analgesics, early treatment of infection with antibiotics and regular folic acid supplementation will be more useful for decreasing morbidity and mortality. Hence With comprehensive medical health care life status expectancy can be improved considerably. Keywords: Sickle cell disease, Pediatric, hematological profiles, Acute painful crisis

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