A STUDY OF CLINICAL AND HAEMATOPATHOLOGICAL CORRELATION OF HAEMOLYTIC ANAEMIAS IN CHILDREN
Journal Title: Journal of Evolution of Medical and Dental Sciences - Year 2016, Vol 5, Issue 69
Abstract
Anaemia is a manifestation of disease, not a disease in itself. Anaemia is a frequent laboratory abnormality in children. As many as 80 children in developing countries will be anaemic at some point by the age of 18 years. Most children with anaemia are asymptomatic, but have abnormal lab data on routine screening. Physical examination is also many a times normal. Detection and diagnosis are essential for rational analysis and management. Among the anaemias, Haemolytic anaemias are unique. The aetiology is diverse and many hereditary and acquired disorders play a role. Clinical presentation depends on the onset of erythrocyte destruction. The morbidity depends on the aetiology of haemolysis. This study focuses on the prevalence, age of presentation and clinical profile of haemolytic anaemia in children and will help in early diagnosis and planning of appropriate treatment. AIMS AND OBJECTIVES OF STUDY To study the prevalence of various types of Haemolytic anaemia in children admitted in Paediatric wards at a tertiary care Hospital. To study the clinical profile, age at first presentation and the aetiology of the hereditary haemolytic anaemia and to study the utility of laboratory procedures in the diagnosis of hereditary haemolytic anaemia. To study the complications, treatment protocols and the response to treatment in hereditary haemolytic anaemia. METHODOLOGY Study Design: Prevalence study, Study Place: Department of Paediatrics, Coimbatore Medical College, Coimbatore, Study Period: 18 months (Nov 2012 - April 2014). INCLUSION CRITERIA The sample consisted of children aged 1 month to 17 years, presenting with clinical symptoms and signs suggestive of haemolytic anaemia and those with peripheral blood smear suggestive of haemolysis. EXCLUSION CRITERIA Neonates were excluded from the study. MANOEUVRE In the present hospital-based study, the prevalence of various types of haemolytic anaemia in children attending Coimbatore Medical College and Hospital was studied. In children diagnosed with hereditary haemolytic anaemia the clinical profile, complications, utility of the diagnostic procedures, treatment protocols and the response to the treatment were studied. RESULTS/CONCLUSION In the present hospital-based prevalence study, 52 children were diagnosed as having Haemolytic anaemia. In those children diagnosed as hereditary haemolytic disorders, the age at first presentation, clinical profile, complications, treatment protocols and response to the treatment were studied. In our study, the youngest child was 3-month-old female and the oldest was 3-year-old male. Most of the children were presented in the age group between 3 months to 12 months - 8 cases (66.66%). Mean age at presentation is 9 months. In the present study, the frequency of Hb-S related disorders was high constituting 57.57% followed by Thalassemia syndromes constituting 39.36%. Average age at presentation of Thalassemia major was 9 months; Sickle cell anaemia 6 yrs.; Sickle cell trait 4 yrs. and Sickle thalassemia 5.5 yrs. Male: Female ratio for Thalassemia major was 2:1; Male: Female ratio for Sickle cell anaemia was 26:1. The common mode of presentation/complication for Thalassemia major was anaemia with congestive cardiac failure; vaso-occlusive crisis for sickle cell anaemia and anaemia for sickle cell disease. Family history of consanguinity was positive in 69.69% of cases. When growth parameters were studied in children with beta thalassemia major, growth retardation was observed in 75% of cases; and in sickle cell disorders 78.4% of children had growth retardation. Peripheral smear evidence of haemolysis was positive in 90.9% cases suggesting that as the most useful method for screening. In children diagnosed as having Thalassemia major, 72% received 15 blood transfusions at the age of 0-2 yrs. and in children diagnosed as having Sickle cell disorders 50% received 1st blood transfusion at the age of 3-5 yrs. In those children receiving chelation therapy, side effect reported was neutropenia.
Authors and Affiliations
Palaniappan , Booma
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