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Abstract hronic hemolytic anemia (CHA) is a common problem in pediatric practice. It is caused by premature destruction of red blood cells(RBCs), exceeding the capacity of bone marrow to produce RBCs. Among the most common cause of CHA are thalassemias, sickle cell disease, pyurvate kinase deficiency, hereditary spherocytosis . Individuals with Chronic hemolytic anemia especially β-thalassemia major suffer from severe anemia and hepatosplenomegaly; they usually come to medical attention within the first two years of life. Without treatment, affected children have severe failure to thrive and shortened life expectancy. Treatment with a regular transfusion program and chelation therapy, aimed at reducing transfusion iron overload, allows for normal growth and development and extends life expectancy into the third to fifth decade. Accordingly, lifelong care is required, and financial expenditures for proper treatment are substantial. Chronic hemolytic anemia as a chronic disease is associated with potentially compromised health-related quality of life for children and their families . Children with chronic hemolytic anemia have good survival but little is known about their quality of life . C The aim of the study is to assess the clinical characteristics as well as frequency of complications and assess quality of life. The response to different therapeutic modalities . Our study was retrospective demographic analysis of chronic hemolytic anemia patients attending to outpatient clinic of hematology unit of Beni –Suef university hospital. In our study 150 patients were enrolled in this study .They were diagnosed as chronic hemolytic anemia by clinical examination and hemoglobin electrophoresis ;108 patients were diagnosed as Bthalassemia, 19 patients were diagnosed as sickle cell anemia ,19 patients were diagnosed as G6PDD, While 4 patients were diagnosed as hereditary spherocytosis. All patients were subjected to detailed history taking, full clinical examination, laboratory investigation (CBC, Hb level pre and post transfusion, Serum ferritin level ,Kidney functions , abdominal US and Hb electropharesis ) . .Among our patients 65males and 85females ,61%had positive family history and 65% had positive consanguinity. According to Egyptian growth curves for height and weight percentile respectively growth failure is detected . Patients can be classified as: Below 3rd percentile:51.9% of all population study. from 3rd to 25th percentile :18.5% >25 th percentile :34% >75th percentile :12% The study demonstrated the major complications affecting chronic hemolytic anemia patients as hepatitis ,the study demonstrated 16% of patients affected by HCV by PCR and other hematological values. The study demonstrated that hydroxyurea is the main stay of treatment of β- thalassemia and sickle cell anemia provided that monitoring of its side effects and revealed normal serum creatinine level, normal serum Na and K and significantly elevated BUN in thalassemic patients. |