Asian Journal of Clinical Pediatrics and Neonatology¦ Volume 7 ¦ Issue 4¦ October-December 2019 42 Abstract Clinical Profile of Children with Beta-Thalassemia Dilip Kumar 1 , Aarti. A. Kinikar 2 1 Junior Consultant, Neonatology, Ovum Women and Children Speciality Hospital, Banaswadi, Bangalore, 2 Professor and Head, Department of Pediatrics, Byramjee Jee Jee Bhoy Government Medical college and Sasson General Hospital, Pune, Maharashtra. Background: The pathogenesis of thalassemia is based on in effective erythropoiesis, haemolysis and a tendency to increase iron absorption, this causes monocytes and macrophages compartment hyperplasia and hyperactivity. These increased phagocytic activities reduce the capacity of phagocytic system to defend against pathogenic microorganisms. Finally, severe anemia itself is a risk factor for bacterial infections in thalassemia predominantly pneumonia. Subjects and Methods: The present study was conducted in the thalassemia unit and pediatric ward of a tertiary care, referral and teaching hospital situated in an urban area. Informed consent of parents/ caretaker of the enrolled thalassemic children were taken. Result: Among 32 patients, all except one had physical growth retardation. A thalassemic child who had recurrent infections had 11 times higher chance of having physical growth disturbance. (OR= 11.12; p value = 0.0066) compared to those who did not have recurrent infections. 59.37% of the patients who had recurrent infections had wasting and stunting, 15.62% had only wasting and 21.87% had only stunting. Conclusion: This shows that there was male predominance among the patients with infections. Out of 85 cases, 55.29% were males and 44.7% were females. Male: Female ratio was 1.236: 1. Keywords: Beta-Thalassemia, erythropoiesis, recurrent infections. Corresponding Author: Dr. Aarti. A. Kinikar, Professor and Head, Department of Pediatrics, Byramjee JeeJeeBhoy Government Medical college and Sasson General hospital,Pune, Maharashtra. Received: December 2019 Accepted: December 2019 Introduction The Thalassemias are a group of hereditary anaemias resulting from a defect in haemoglobin production. They are the world‟s most common monogenic disorders. The word „Thalassemia‟ is derived from the Greek word„Thalassa‟ meaning „sea‟. Haemoglobin disorders thought to have originated in countries where malaria was endemic. The disease was initially known to be limited to Mediterranean belt, as a result of migration of population, thalassemia, which was once confined only to the Mediterranean belt, is now found to be distributed in many parts of the world. Although reliable data are still lacking for many regions of the world, recent data indicate that about 7% of the world population is a carrier for this haemoglobin disorder, and that 3, 00,000-5, 00,000 children are born each year with the homozygous state of the disease worldwide. 2 Every year approximately 10,000 to 20,000 are born with thalassemia in India. The carrier rate for thalassemia gene varies from 1-3 % in southern India to 3-15% in northern India. [1] Infections are a frequent complication of thalassemia and they can be fatal. The morbidity and mortality rates for infections vary throughout the world depending on differences in the epidemiology of each infection and on the socio economic status of each country and also vary depending on the preventive and therapeutic strategies adopted by that country. [2] In an Italian multicentric study, infections were the second common cause of death after heart failure in thalassemia. Similar results were reportedin Greece and in Taiwan, while in E-beta thalassemia patients in Thailand, infections were the primary cause of morbidity and mortality. The transfusion and iron chelation therapies have dramatically changed the management and prognosis of thalassemia. However, the benefits from transfusion come together with the disadvantages of the high risk of direct exposure to infection risk and indirectly to transfusion related immunomodulation. The other therapeutic options like splenectomy, nutritional deficiencies etc contribute to the infection risk. [3] The pathogenesis of thalassemia is based on ineffective erythropoiesis, haemolysis and a tendency to increase iron absorption, this causes monocytes and macrophages compartment hyperplasia and hyperactivity. These increased phagocytic activities reduce the capacity of phagocytic system to defend against pathogenic microorganisms. Finally, severe anaemia itself is a risk factor for bacterial infections in thalassemia predominantly pneumonia. [4] Due to frequent blood transfusions, the thalassemia patients are subjected to peroxidative stress injury by the secondary iron overload. Iron induced peroxidative damage can lead to congestive cardiac failure which is the main cause of death ISSN (0): 2347-3363; ISSN (P): 2347-3355 Original Article