Riaz Haris, Riaz Talha, Khan Muhammad Ubaid, Aziz Sina, Ullah Faizan, Rehman Anis, Zafar Qandeel, Kazi Abdul Nafey
House officer, Civil Hospital Karachi, Pakistan.
BMC Res Notes. 2011 Aug 11;4:287. doi: 10.1186/1756-0500-4-287.
Beta thalassemia is the most frequent genetic disorder of haemoglobin synthesis in Pakistan. Recurrent transfusions lead to iron-overload manifested by increased serum Ferritin levels, for which chelation therapy is required.
The study was conducted in the Pediatric Emergency unit of Civil Hospital Karachi after ethical approval by the Institutional Review Board of Dow University of Health Sciences. Seventy nine cases of beta thalassemia major were included after a written consent. The care takers were interviewed for the socio-demographic variables and the use of Desferrioxamine therapy, after which a blood sample was drawn to assess the serum Ferritin level. SPSS 15.0 was employed for data entry and analysis.Of the seventy-nine patients included in the study, 46 (58.2%) were males while 33 (41.8%) were females. The mean age was 10.8 (± 4.5) years with the dominant age group (46.2%) being 10 to 14 years. In 62 (78.8%) cases, the care taker education was below the tenth grade. The mean serum Ferritin level in our study were 4236.5 ng/ml and showed a directly proportional relationship with age. Desferrioxamine was used by patients in 46 (58.2%) cases with monthly house hold income significant factor to the use of therapy.
The mean serum Ferritin levels are approximately ten times higher than the normal recommended levels for normal individuals, with two-fifths of the patients not receiving iron chelation therapy at all. Use of iron chelation therapy and titrating the dose according to the need can significantly lower the iron load reducing the risk of iron-overload related complications leading to a better quality of life and improving survival in Pakistani beta thalassemia major patients.Conflicts of Interest: None.
β地中海贫血是巴基斯坦最常见的血红蛋白合成遗传性疾病。反复输血会导致铁过载,表现为血清铁蛋白水平升高,对此需要进行螯合治疗。
本研究在卡拉奇市民医院儿科急诊科进行,经道健康科学大学机构审查委员会伦理批准。在获得书面同意后,纳入了79例重型β地中海贫血病例。对护理人员进行了社会人口统计学变量及去铁胺疗法使用情况的访谈,之后采集血样以评估血清铁蛋白水平。使用SPSS 15.0进行数据录入和分析。本研究纳入的79例患者中,46例(58.2%)为男性,33例(41.8%)为女性。平均年龄为10.8(±4.5)岁,主要年龄组(46.2%)为10至14岁。62例(78.8%)患者的护理人员教育程度低于十年级。本研究中血清铁蛋白平均水平为4236.5 ng/ml,且与年龄呈正比关系。46例(58.2%)患者使用了去铁胺,家庭月收入是使用该疗法的重要因素。
血清铁蛋白平均水平约比正常个体正常推荐水平高十倍,五分之二的患者根本未接受铁螯合治疗。使用铁螯合疗法并根据需要调整剂量可显著降低铁负荷,降低铁过载相关并发症的风险,从而提高巴基斯坦重型β地中海贫血患者的生活质量并改善生存率。利益冲突:无。