CLINICOETIOLOGICAL STUDY OF HEMOLYTIC ANAEMIA IN CHILDREN PRESENTING TO A TERTIARY CARE HOSPITAL OFKASHMIR

Abstract

Anemia is said to be present when the hemoglobin levels are more than two standard deviations below the mean for the childs age and sex. Hemolytic anemias arise when red blood cells have decreased survival either due to an intrinsic abnormality of the cell or due to extrinsic factors or both. Hemolytic anemias constitute important cause of mortality and morbidity in developing countries. These group of anemias have various clinical presentations starting from their age of onset of symptoms, failure to thrive, anemia, jaundice, splenomegaly, cholelithiasis, cardiomegaly, congestive cardiac failure, severe life-threatening infections and chronic disabilities leading to distress in the families. The present study attempts to reveal the clinical and etiological profile of patients with different types of hemolytic anemia admitted in a pediatric tertiary care hospital of Kashmir. Review of literature shows only few epidemiological studies particularly in this part of the country, and no such study in our state though the disease is fairly prevalent. We undertook this study with this idea. Aims and Objectives: The aims and objectives of this study was to determine clinical and etiological profile of hemolytic anemia in children aged 1month to 18 years admitted in tertiary care hospital of Kashmir. Materiaals & Methods: This prospective observational study was carried out in a pediatric tertiary care hospital in Kashmir over a period of two years. Prior to initiation of study, ethical clearance was obtained. All patients admitted in the indoor of department of pediatrics between age group of 1 month to 18 years with evidence of hemolysis were included in the study. 50 cases were included in the study after obtaining written informed consent from parent or guardian. A detailed history, examination and required investigations were collected. Data was entered and assessed in MS Excel. Results: The study was based on 50 patients, almost equal males and females, 1month to 18 years of age. The most common hemolytic anemia was beta thalassemia major (n=17, 34%), followed by autoimmune hemolytic anemia(n=10,20%), hereditary spherocytosis (n=5,10%), thalassemia trait (8%), pyruvate kinase deficiency (8%) and glucose 6 phosphate dehydrogenase (8%), thalassemia intermedia (4%), sickle cell trait (2%), microangiopathic hemolytic anemia (2%), hereditary xerosis (2%) and hereditary stomatocytosis (2%). Highest number of the cases was less than 3 years old (56%). The most common clinical presentation was jaundice, which was present in all cases, followed by splenomegaly (60%), hepatomegaly (30%), hemolytic facies (26%). Splenomegaly was seen in all cases of thalassemia major, thalassemia intermedia, beta thalassemia trait, hereditary spherocytosis, 2 cases of pyruvate kinase deficiency and 3 cases of glucose 6 phosphate dehydrogenase deficiency. Spleen >5cm was seen in 10 patients. Massive splenomegaly with abdominal discomfort was seen in 3 cases. The mean hemoglobin was least in beta thalassemia major followed by beta thalassemia intermedia, Hereditary spherocytosis, autoimmune hemolytic anemia, glucose 6 phosphate dehydrogenase deficiency and the highest mean hemoglobin was seen in pyruvate kinase deficiency. Means for serum ferritin and serum bilirubin were calculated.