الفهرس 
Thalassemia in Children and Adolescents: A Single Center Study of Adherence to Evidence Based Management Guidelines and Practice Obstacles
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Abstract
Thalassemia refers to a group of inherited hemoglobinopathies where there
is a quantitative defect in the production of alpha globin or beta globin chains resulting in
precipitation of the unpaired chains, which in turn causes destruction of red blood cell
precursors in the bone marrow (ineffective erythropoiesis) and circulation (hemolysis). It
often requires regular blood transfusions, iron chelation therapy and sometimes
splenectomy for its management. Thus, the therapeutic regime is complex, lifelong and
inconvenient, requiring repeated hospitalizations and blood transfusions, which often
affects the child’s physical and mental health negatively. Treatment adherence is crucial to
Thalassemia control, complications prevention.
Objectives: To evaluate thalassemia major and intermedia management practice and
rate of adherence to guideline at Pediatric Hematology Clinic and Unit, Cairo
University Hospitals, and to analyze the causes of non-adherence to guidelines before
and during the Covid 19 era.
Patients and Methods: This study is a Survey cross-sectional study which was conducted
at the Hematology Clinics in the Children’s Hospitals, Cairo University, in the period from
August 2020 to June 2021, on 100 patients with beta thalassemia major and intermedia
diagnosed and followed-up. Patients were randomly distributed to the physicians and
usually visit the clinic without arranging appointments. On the clinic day, all consecutive
patients and care givers were asked to participate in the study