Kosaryan Mehrnoush, Karami Hossein, Darvishi-Khezri Hadi, Akbarzadeh Rosetta, Aliasgharian Aily, Bromand Khadijeh
Department of Pediatric, Thalassemia Research Center, Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran.
Student Research Committee, Thalassemia Research Center, Hemoglobinopathy Institute, Mazandaran University of Medical Sciences, Sari, Iran.
Iran J Public Health. 2019 Jul;48(7):1335-1345.
Electronic registry system of beta-thalassemia patients was run by Thalassemia Research Center (TRC) in 2017. The aim of the current study was presentation of therapeutic status in these patients at Mazandaran Province, Iran.
Therapeutic status variables including: Name of cities and hospitals, age and sex of patients, dependent and non-transfusion-dependent, starting age of the blood transfusion and iron-chelating agents, blood group and Rh, washed blood transfusion, abnormal antibody, transfusion reactions, mean hemoglobin during the last 3 months, type of iron chelators, iron chelators dosage, serum ferritin, and the use of hydroxyurea.
Overall, 1831 patients were registered [891 male (48.7%)]. Mean age of patients was 30±9.7 yr. Average of hemoglobin levels for female and male were 9.1±5.1 and 9.4±6.3 gr/dl, respectively. Seventy-six percent of transfusion-dependent patients (1385) have received iso-group PRBC (packed red blood cells), after crossmatch. The most common blood group among patient was type O-positive (35.7%). Monotherapy with desferrioxamine was most type of used iron-chelating agent in these patients (47.2%). Mean of ferritin was 3300±7800 (ng/ml). Twenty-eight percent of patients (484) have received hydroxyurea; proportion of male and female was approximately equal. T2 weighted magnetic resonance imaging (MRIT2*) was measured in 62.2% of patients. Moderate and severe hepatosiderosis was 10.1% and 2.9%, respectively. Patients with moderate and severe cardiac siderosis were 11% and 5%, respectively.
Registry findings are valuable for treatment management and ensuring patients medications. It will also provide accessibility to various levels of patients' information for health care managers and experts to help them make appropriate decisions.
2017年，地中海贫血研究中心（TRC）启动了β地中海贫血患者电子登记系统。本研究的目的是介绍伊朗马赞德兰省这些患者的治疗状况。
治疗状况变量包括：城市和医院名称、患者年龄和性别、依赖输血和非依赖输血、输血和铁螯合剂的起始年龄、血型和Rh、洗涤输血、异常抗体、输血反应、过去3个月的平均血红蛋白、铁螯合剂类型、铁螯合剂剂量、血清铁蛋白以及羟基脲的使用情况。
总体而言，登记了1831名患者[891名男性（48.7%）]。患者的平均年龄为30±9.7岁。女性和男性的血红蛋白水平平均值分别为9.1±5.1和9.4±6.3克/分升。76%的依赖输血患者（1385名）在交叉配血后接受了同型PRBC（浓缩红细胞）。患者中最常见的血型是O型阳性（35.7%）。去铁胺单药治疗是这些患者中最常用的铁螯合剂类型（47.2%）。铁蛋白平均值为3300±7800（纳克/毫升）。28%的患者（484名）接受了羟基脲治疗；男性和女性的比例大致相等。62.2%的患者进行了T2加权磁共振成像（MRIT2*）测量。中度和重度肝铁过载分别为10.1%和2.9%。中度和重度心脏铁过载患者分别为11%和5%。
登记结果对治疗管理和确保患者用药很有价值。它还将为医疗保健管理人员和专家提供获取各级患者信息的途径，以帮助他们做出适当决策。
