Poole J E, Cohn R J, Roode H, Spector I
Department of Paediatrics, Johannesburg Hospital.
S Afr Med J. 1989 Apr 15;75(8):367-70.
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.
1979年至1984年间，约翰内斯堡的两家医院对29名重型β地中海贫血患者进行了治疗。他们分属两个种族群体——地中海裔和亚裔，其中53%的患者年龄在6岁以下，年龄最大的为20岁。8名患者接受了脾切除术。所有患者均定期输血，生活质量有了很大改善。定期输血的并发症是铁过载，这会导致发病和死亡。尽管皮下注射去铁胺（DFO）可使铁充分排出，但患者仍表现出明显的铁过载，这表明患者在家中使用DFO的依从性较差，这也是重型β地中海贫血治疗中的主要长期问题。需要对高危社区及其医生进行教育，以预防新病例的出现，并减轻经济和情感负担。如果患者在遵守治疗方案方面得到充分支持，他们的预期寿命应会大幅提高。
