Patients with Beta thalassemia major

ABSTRACT

Qatif is an area with a high prevalence of haemoglobinopathies mainly thalassaemia and sickle cell anaemia. The purpose of this study is to analyse the clinical features and result of treatment of children with beta thalassaemia. Paediatric department of Qatif Central Hospital. 20 children with beta thalassaemia aged 12 years or younger [8 boys and 12 girls] followed up regularly in the haematology clinic over a period of 6 years [1988-1993]. Their age range is 18 months to 12 years [median, 8 years] and the age range at the first blood transfusion was 6 to 48 months [median, 18]. Physical evaluation revealed that weight was below the 10[th] percentile in 12 patients [60%], height was below the 10[th] percentile in six patients [30%], liver sizes were more than 2 cm below costal margin [BCM] in ten patients and more than 4 cm BCM in seven patients. Four patients had splenectomy, three patients had a spleen less than 2 cm BCM and 13 patients had a spleen more than 2 cm BCM. Cardiac evaluation by EKG and chest x-ray revealed cardiomegaly in five patients [25%]. Evaluation of iron overload by serum ferritin estimation was above 1000 mg/ml in 17 patients [85%]. Liver enzymes were high in 18 patients [90%]. Screening for hepatitis surface antigen and HIV one-two were negative in all cases. This study indicates that children with beta thalassaemia major in Qatif tend to have severe complications due to inadequate treatment, and great efforts are needed to improve the medical care provided to this group of patients if we are to give them a real chance for life