الفهرس 
?-THALASSEMIA MAJOROnly 14 pages are availabe for public view
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Abstract
Background: Over the past few decades, there has been a remarkable improvement in the survival of patients with thalassemia in developing countries. Availability of safe blood transfusions, effective and accessible iron chelating medications, the introduction of new and non-invasive methods of tissue iron assessment and other advances in multidisciplinary care of thalassemia patients have all contributed to better outcomes. Objective: To evaluate quality of care in patients with thalassemia major at Ain Shams University Children’s Hospital against the standard of care. Patients and methods: Two hundred patients with beta thalassemia major with age ranges 2 to 17 years were recruited. Data was collected by reviewing the patients’ records. Interview based structured questionnaire was developed to assess the patient’s satisfaction about care received during the past 12 months by using a questionnaire including 14 questions (Likert questions) where patients express their opinion about care received. Results: The mean value of pre-transfusion Hb among our patients was 7.26 ± 0.89 g/dl. Initial extended red cell antigen typing had been done in 4.5% of patients. Almost all the studied patients (198/200) were on chelation therapy either as monotherapy or combined chelation therapy with DFO/DFX being the most frequently used combination. Many patients in our study suffered endocrinal complications with short stature being the most common complication. Twenty-eight patients had delayed puberty while hypothyroidism and hypoparathyroidism were uncommon (only one patient for each). LIC and MIC assessment using MRI was done in 13.5% of the patients. Most patients had moderate to severe degree of hepatic iron overload, while only 7.4% had high risk myocardial iron deposition. HCV infection was prevalent among one fifth of the patients. Conclusion: Specialized care is mandatory for the patients with beta thalassemia. Assessing the quality of care is of utmost value in guiding resources and redirecting therapy.