Pattern and Clinico-Laboratory Profile in Thalassemic Children in Sohag City

Background: Thalassemia is one of the most common autosomal recessive disorders worldwide. It is a genetic disorder of hemoglobin synthesis. Beta-thalassemia has a particularly high prevalence in populations in the Mediterranean, Middle East, and India. Long-term blood transfusion, splenectomy, and iron chelation therapy- these interventions play a crucial role in the management of thalassemia patients. Objective: This is a demographic study aimed to determine the age and sex distribution, clinic-laboratory profile, investigation, complications to management of transfusion-dependent B-thalassemia major in children at El-Helal hospital in Sohag city. Patients and Method: This study was carried out prospectively from 1st April 2019 to the 31st December 2021, the study included total of 166 children aged between 6 months to 12 years, clinically diagnosed with transfusion-dependent β-thalassemia major attending hematology clinic at Sohag El-Helal Hospital for Health Insurance. Studied children were subjected to complete history, clinical examination and laboratory evaluation. Results: A total of 166 children that age range from 6 months to 12 years, with a mean age of 9.2±4.4 years, most of them were male 122 (73.5%) and female 44 (26.5%). There was a significant statistical attachment between both sex and the Consanguinity, 151 (91%). Allergic reactions were developed in 12 (7.2 %) patients during blood transfusion. Splenomegaly was reported to be a major complication among study patients. Conclusion: Severe anemia is present in nearly all patients; Serum ferritin concentration accurately reflects high body iron stores. liver, kidney, bone, and endocrine functions significantly deteriorate in almost all studied patients.