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小儿β地中海贫血患者垂体含铁血黄素沉着症与内分泌紊乱的关系

Relationship Between Pituitary Siderosis and Endocrinological Disorders in Pediatric Patients with Beta-Thalassemia.

作者信息

Yılmaz Kamil, Kan Ahmet, Çetincakmak Mehmet Guli, Uzel V Hulya, Yılmaz Deniz, Deniz Muhammed Akif, Hattapoglu Salih

机构信息

Pediatrics, Dicle University School of Medicine, Diyarbakir, TUR.

Pediatric Allergy, Dicle University School of Medicine, Diyarbakir, TUR.

出版信息

Cureus. 2021 Jan 23;13(1):e12877. doi: 10.7759/cureus.12877.

Abstract

Introduction Excess iron accumulation occurs mainly in organs such as reticuloendothelial cells, the pituitary gland, and the pancreas in beta-thalassemia because of blood transfusions. In the present study, it was aimed to investigate the relationship between T2* values on magnetic resonance imaging (MRI) and clinically diagnosed pituitary endocrinological disorders in children with thalassemia major. Methods This study enrolled patients diagnosed with beta-thalassemia at pediatric hematology outpatient clinics. In the study, in addition to the medical history of the patients, routinely performed tests, including hemoglobin electrophoresis, routine biochemical tests, and tests for pubertal development (follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, testosterone, etc.), as well as iron deposition measured by hepatic MRI T2* (STAR) sequence, were retrospectively assessed. A total of 29 patients were enrolled. Results Hypothyroidism was detected in 34.6% (9/26) of patients, short stature in 37% (10/27), and pubertal retardation in 50% (14/28) of the patients. There was no significant correlation between hypothyroidism and pituitary MRI T2* values. No significant correlation was found between laboratory parameters and pituitary MRI examination. Although the sensitivity of T2* levels could rise above 80%, their specificity remained low. This is one of the major limitations of the pituitary MR T2* study for the prediction of short stature. The best lower cut-off level of MR T2* to predict short stature was found 14.6 ms. Conclusion The diagnostic specificity pituitary MR examination levels for short stature were detected as low. Thus, the clinical standardization and validation of pituitary MR T2* values examination are needed before clinical follow-up and multifaceted studies are needed.

摘要

引言

由于输血,过量铁蓄积主要发生在β地中海贫血患者的网状内皮细胞、垂体和胰腺等器官中。本研究旨在探讨磁共振成像(MRI)的T2*值与重型地中海贫血患儿临床诊断的垂体内分泌疾病之间的关系。

方法

本研究纳入了在儿科血液科门诊诊断为β地中海贫血的患者。在研究中,除了患者的病史外,还回顾性评估了常规进行的检查,包括血红蛋白电泳、常规生化检查和青春期发育检查(促卵泡生成素(FSH)、促黄体生成素(LH)、雌二醇、睾酮等),以及通过肝脏MRI T2*(STAR)序列测量的铁沉积。共纳入29例患者。

结果

34.6%(9/26)的患者检测出甲状腺功能减退,37%(10/27)的患者身材矮小,50%(14/28)的患者青春期发育迟缓。甲状腺功能减退与垂体MRI T2值之间无显著相关性。实验室参数与垂体MRI检查之间未发现显著相关性。虽然T2水平的敏感性可升至80%以上,但其特异性仍然较低。这是垂体MR T2研究预测身材矮小的主要局限性之一。预测身材矮小的MR T2最佳下限值为14.6毫秒。

结论

垂体MR检查对身材矮小的诊断特异性较低。因此,在临床随访之前需要对垂体MR T2*值检查进行临床标准化和验证,并且需要进行多方面的研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/382f/7899051/da7462aa890a/cureus-0013-00000012877-i01.jpg

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