الفهرس 
BETA-THALASSEMIAOnly 14 pages are availabe for public view
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Abstract
T
he combination of blood transfusion and chelation therapy has dramatically prolonged the life expectancy of beta thalassemia patients. However, frequent blood transfusions, iron overload, poor compliance to therapy and chronicity of the disease have in turn contributed to a whole spectrum of complications including cardiac problems, hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism and other endocrine and metabolic problems in adolescents and young adults suffering from thalassaemia major.
The purpose of the present study was to assessthe morbidity patterns of transfusion-dependent thalassemia major patients, and compare the outcomes of these patients according to age of onset, type, duration and compliance to iron chelation therapy and frequency of blood transfusion.
This study included 447 beta thalassemia patients regularly attending the Pediatric Hematology/oncology Clinic, Ain Shams University Children’s Hospital over the last 10 years.Data was collected from the patients or their caregivers, as well as by reviewing follow up sheets.
The study showed that therewas a higher prevelance of osteoporosis delayed puberty, decreased ejection fraction in patients with serum ferritin more than 1500 ng/dl.
Patients on desferoxamine showed a significantly more decrease in the ejection fraction below 60% than patients on other chelation therapy. The different ICT groups showed a comparable prevalence of cardiac, hepatic, renal morbidities as well as decrease in bone mineral density.
Although, there were significant correlations between the bone density abnormalities, the hepatic morbidities and the mean ferritin in the 2 year before evaluation, yet no correlation existed between the renal morbidities, thyroid and parathyroid disorders and the mean ferritin in the 2 years prior evaluation.
Beta thalassemia Patients with DM, hypogonadism, EF less than 60, cardiomyopathy, and pulmonary hypertensionhad higher mean serum ferritin in the 2years prior to evaluation.
Male Patients and those diagnosed at younger age, had higher rate of hepatic, renal morbidities and had lower ejection fraction.
There were a significantly positive correlation between the prevalence of DM, hypogonadism, cardiomyopathy, the decrease in EF less than 60 and the patient`s compliance.
There was also a significant correlation existed betweenthe frequency of blood transfusion and the prevalence of hypogonadism, and decreased ejection fraction less than 60.
The current study also showed that the 5 years survival rate among the studied patients was 80%, 10 years survival rate was 50%, 20 years survival rate was 20%.