Department of Pediatrics, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Department of Pediatrics, Division of Hematology and Oncology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Hemoglobin. 2020 Sep;44(5):344-348. doi: 10.1080/03630269.2020.1812636. Epub 2020 Sep 8.
The endocrinological complications are a great concern in transfusion-dependent β-thalassemia (β-thal) patients. The pituitary iron deposition is regarded as the main cause of hormonal changes in thalassemic patients. In this study, our aim was to explore the association between endocrinological complications and pituitary iron overload by magnetic resonance imaging (MRI). Fifty transfusion-dependent thalassemia (TDT) patients were recruited for the study. Pituitary MRIs of patients were taken using a 1.5 Tesla Philips MRI machine. There was at least one clinical endocrine complication in two of three patients. The iron accumulation was moderate in the liver (60.0%) and was mild in hypophysis (16.0%) and in heart (8.0%). The hypogonadism and diabetes mellitus (DM) were not seen with a significantly increased pituitary iron burden. The hypogonadism was related to cardiac iron deposition ( = 0.04). The short stature was associated with a hepatic iron overload ( = 0.05). The conventional follow-up of patients with TDT might be inadequate and screening of patients with MRI of hypophysis along with heart and liver leads to better results.
内分泌并发症是输血依赖型β-地中海贫血（β-地中海贫血）患者的一大关注点。垂体铁沉积被认为是导致地中海贫血患者激素变化的主要原因。在这项研究中，我们的目的是通过磁共振成像（MRI）来探讨内分泌并发症与垂体铁过载之间的关系。
招募了 50 名输血依赖型地中海贫血（TDT）患者进行研究。使用 1.5 特斯拉飞利浦 MRI 机对患者的垂体 MRI 进行了拍摄。有 2/3 的患者至少有一个临床内分泌并发症。肝脏铁蓄积程度为中度（60.0%），垂体（16.0%）和心脏（8.0%）铁蓄积程度为轻度。性腺功能减退症和糖尿病（DM）并未随着垂体铁负荷的显著增加而出现。性腺功能减退症与心脏铁沉积有关（=0.04）。身材矮小与肝脏铁过载有关（=0.05）。对 TDT 患者的常规随访可能不够充分，对垂体、心脏和肝脏 MRI 的患者进行筛查可取得更好的结果。
