| Summary: | Background Beta-thalassemia is one of the common genetic disorders in our community. Therefore, it is deemed necessary to study and probe it to encompass all aspects of this disorder.
Aim The aim of this study was to determine the adherence of management of β-thalassemia major in Minia General Hospital to an established Standard for the Clinical Care of Children with Thalassemia in the UK.
Patients and methods This study included 100 children with β-thalassemia major who were receiving regular blood transfusion; data of the patients were collected and analyzed for audit against established Standards for the Clinical Care of Children with Thalassemia in the UK; values were expressed in terms of percentage.
Results The diagnosis of β-thalassemia major in our study was made mainly on the basis of the clinical background confirmed by complete blood count, hemoglobin electrophoresis, and serum ferritin level; the frequency of investigations in our study showed that abdominal ultrasound was performed regularly in most of our patients (93%), followed by liver function (74%), serum ferritin (65%), hepatitis marker (57%), echocardiography (47%), and assessments of weight and height − sitting and standing (43%), follicle-stimulating hormone, luteinizing hormone (7%), thyroid-stimulating hormone, triiodothyronine, thyroxine (7%), fundus examination (7%), audiometry (3%), oral glucose tolerance test (3%), vitamin D level (3%), and DEXA scan (1%); the assessment of puberty was not performed. The most common complication in our study was splenomegaly (80%), followed by bone deformities (76%), slowed growth rates (69%), and infection (47%), followed by bone osteoporosis (27%), heart problems (24%), chronic hepatitis, or liver fibrosis (19%). Iron-chelation therapy was used by 53% of our patients. Deferasirox is the main line (38%); hydroxyurea was used by 41% of our patients.
Conclusion As a conclusion, we found that lines of management of β-thalassemia major in Minia General Hospital were not enough and not performed systematically in comparison with Standards for the Clinical Care of Children with Thalassemia in the UK.
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