title: Beta-thalassaemia--the Johannesburg experience.
abstract: Twenty-nine patients with beta-thalassaemia major were treated in two Johannesburg hospitals between 1979 and 1984. They belonged to two ethnic groups--Mediterranean and Asian--and 53% were under the age of 6 years, the oldest being 20 years. Eight patients underwent splenectomy. All were regularly transfused and their quality of life greatly improved. The complication of regular transfusions is iron overload, which contributes to morbidity and mortality. Despite adequate iron excretion as a result of subcutaneous desferrioxamine (DFO) infusions, the patients showed significant iron overload, which suggested poor compliance in the home use of DFO and was the main long-term problem in the treatment of beta-thalassaemia major. Education needs to be directed at communities at risk and their doctors so that new cases can be prevented and the financial and emotional cost eased. If patients are given full support in complying with therapy, their life expectancy should be greatly increased.
authors.affiliation: Department of Paediatrics, Johannesburg Hospital.
authors.forename: J E
authors.initials: JE
authors.lastname: Poole
authors.forename: R J
authors.initials: RJ
authors.lastname: Cohn
authors.forename: H
authors.initials: H
authors.lastname: Roode
authors.forename: I
authors.initials: I
authors.lastname: Spector
mesh_terms: D000293:Adolescent; D000328:Adult; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use; D017707:Erythrocyte Transfusion; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007501:Iron / Q000378:metabolism; D013019:South Africa; D013156:Splenectomy; D013789:Thalassemia / Q000150:complications / Q000628:therapy*
chemical_list: D006454:Hemoglobins; D007501:Iron; D003676:Deferoxamine