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安塔利亚州立医院地中海贫血中心:15年经验(1994年至2008年)

The Thalassemia center of Antalya State Hospital: 15 years of experience (1994 to 2008).

作者信息

Canatan Duran

机构信息

Akdeniz Kan Hastaliklari Vakfi, Antelsan Is Merkezi, Kisla Mh Gulluk Cd., Antalya, Turkey.

出版信息

J Pediatr Hematol Oncol. 2013 Jan;35(1):24-7. doi: 10.1097/MPH.0b013e3182755f1e.

DOI:10.1097/MPH.0b013e3182755f1e
PMID:23128337
Abstract

The Thalassemia center of Antalya State Hospital was established in 1994 in Antalya, Turkey. The number of newborns with thalassemia decreased statistically because of actions taken as a result of prevention studies. A total of 388 patients, including 246 with thalassemia major (63.4%), 86 with thalassemia intermediate (22.1%), 23 with sickle cell+β-thalassemia (5.9%), 20 with sickle cell disease (5.1%), and 13 with other hemoglobin abnormalities (3.3%), were studied. Complications were found to be as follows: cardiomyopathy in 45 of them (11.5%), diabetes mellitus in 10 (2.5%), hypothyroidism in 17 (4.3%), hypoparathyroidism in 2 (0.5%), osteoporosis in 53 (13.6%), growth retardation in 110 (28.3%), and hypogonadism in 75 patients (19.3%). The incidence of autoantibody and alloantibody in patients with thalassemia major was 5.6% and 10.5%, respectively. Transfusion-transmission diseases evaluated in patients found the incidence of hepatit A virus IgG to be 97.5%, that of HBs Ab to be 99.5%, HBs Ag to be 0.5%, HCV Ab to be 18%, CMV IgG to be 72.3%, CMV IgM to be 2%, and HIV-Ab to be 0%. Therefore, for the first time in our country the thalassemia center was established as a model and included subunits and a team. It served all patients for 15 years until the team was dispersed; thereafter, most of the patients were not followed up on a regular basis.

摘要

安塔利亚州立医院地中海贫血中心于1994年在土耳其安塔利亚成立。由于预防研究采取的措施,地中海贫血新生儿数量在统计学上有所下降。共研究了388例患者,其中重型地中海贫血246例(63.4%),中间型地中海贫血86例(22.1%),镰状细胞+β地中海贫血23例(5.9%),镰状细胞病20例(5.1%),其他血红蛋白异常13例(3.3%)。发现的并发症如下:其中45例(11.5%)患有心肌病,10例(2.5%)患有糖尿病,17例(4.3%)患有甲状腺功能减退症,2例(0.5%)患有甲状旁腺功能减退症,53例(13.6%)患有骨质疏松症,110例(28.3%)生长发育迟缓,75例(19.3%)患有性腺功能减退症。重型地中海贫血患者自身抗体和同种抗体的发生率分别为5.6%和10.5%。对患者评估的输血传播疾病发现,甲型肝炎病毒IgG的发生率为97.5%,乙肝抗体为99.5%,乙肝表面抗原为0.5%,丙肝抗体为18%,巨细胞病毒IgG为72.3%,巨细胞病毒IgM为2%,艾滋病毒抗体为0%。因此,我国首次将地中海贫血中心作为一个模式建立起来,包括多个亚单位和一个团队。该中心为所有患者服务了15年,直到团队解散;此后,大多数患者没有得到定期随访。

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