伊朗重型地中海贫血与中间型地中海贫血青少年及青年成人肾上腺皮质功能减退的发生率
The Frequency of Adrenal Insufficiency in Adolescents and Young Adults with Thalassemia Major versus Thalassemia Intermedia in Iran.
作者信息
Matin Sara, Jahromi Masoud Ghanei, Karemizadeh Zohreh, Haghpanah Sezaneh, De Sanctis Vincenzo, Soliman Ashraf, Dehbozorgian Javad, Majd Zahra, Rezaei Narges, Karimi Mehran
机构信息
Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Anesthesiology, Jahrom University of Medical Science, Jahrom, Iran.
出版信息
Mediterr J Hematol Infect Dis. 2015 Jan 1;7(1):e2015005. doi: 10.4084/MJHID.2015.005. eCollection 2015.
BACKGROUND
Endocrine dysfunction is not uncommon complication in patients with transfusion-dependent thalassemia and is thought to occur as a consequence of excessive iron overload. The primary objective of this study is to determine the frequency of adrenal insufficiency in patients with thalassemia major and thalassemia intermediate.
METHODS
This cross-sectional study was done at the Shiraz University of Medical Sciences, Shiraz, Southern Iran, in 2013. One hundred and ninety patients were divided into two groups; thalassemia major(TM) and thalassemia intermediate (TI) groups. We measured 8 AM serum cortisol, ACTH and ferritin concentrations in all patients.
RESULTS
The mean age of the TM and TI group were 22.5±5.7 and 23.8±6 years, respectively. 90 patients (47.4%) were splenectomized, 34 (36.2%) with TM and 56 (58.2%) with TI (p :<0.001). The median and interquartile range of serum ferritin levels were 2184±3700 ng/ml and 437±443ng/ml in TM and TI respectively (p< 0.001). Three patients with TM (1.6%) had low basal cortisol and ACTH levels. However, their cortisol response to ACTH stimulation was normal.
CONCLUSIONS
Low basal concentrations of cortisol and ACTH occurred in 1.6% of our adolescents young adult patients with TM suggesting a central defect in cortisol secretion at the basal state. However, cortisol response to standard - dose ACTH was normal in all patients with TM and TI.
背景
内分泌功能障碍在依赖输血的地中海贫血患者中是一种常见并发症，被认为是铁过载过多的结果。本研究的主要目的是确定重型地中海贫血和中间型地中海贫血患者肾上腺功能不全的发生率。
方法
这项横断面研究于2013年在伊朗南部设拉子的设拉子医科大学进行。190名患者分为两组；重型地中海贫血（TM）组和中间型地中海贫血（TI）组。我们测量了所有患者上午8点的血清皮质醇、促肾上腺皮质激素（ACTH）和铁蛋白浓度。
结果
TM组和TI组的平均年龄分别为22.5±5.7岁和23.8±6岁。90名患者（47.4%）接受了脾切除术，其中TM组34名（36.2%），TI组56名（58.2%）（p<0.001）。TM组和TI组血清铁蛋白水平的中位数和四分位数间距分别为2184±3700 ng/ml和437±443 ng/ml（p<0.001）。3名TM患者（1.6%）基础皮质醇和ACTH水平较低。然而，他们对ACTH刺激的皮质醇反应正常。
结论
在我们的青少年和年轻成人TM患者中，1.6%出现基础皮质醇和ACTH浓度低，提示基础状态下皮质醇分泌存在中枢缺陷。然而，所有TM和TI患者对标准剂量ACTH的皮质醇反应均正常。
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