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年轻地中海贫血患者肝脏、心肌和垂体铁沉积的MRI相关性研究。

Correlative study of iron accumulation in liver, myocardium, and pituitary assessed with MRI in young thalassemic patients.

作者信息

Christoforidis Athanasios, Haritandi Afroditi, Tsitouridis Ioannis, Tsatra Ioanna, Tsantali Haido, Karyda Stavroula, Dimitriadis Athanasios S, Athanassiou-Metaxa Miranda

机构信息

Thalassaemia Unit, 1st Paediatric Department, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki.

出版信息

J Pediatr Hematol Oncol. 2006 May;28(5):311-5. doi: 10.1097/01.mph.0000212915.22265.3b.

Abstract

Clinical complications resulting from unevenly iron accumulation in individual organs of patients with beta-thalassemia major can affect both expectancy and quality of life. Magnetic resonance imaging (MRI) offers a quantitative, noninvasive, accurate method for estimating iron levels in various tissues, not easily accessible with other techniques. The aim of this study was to evaluate and correlate the level of iron accumulation in different organs (anterior pituitary, myocardium, and liver) assessed with MRI, in children and young adults with beta-thalassemia major. Thirty children and young adults (13 female and 17 male patients) with homozygous beta-thalassemia, treated conventionally, were studied with hepatic, myocardial, and hypophyseal MRI. For liver and myocardium, we calculated the natural logarithm of the signal-to-air ratio in flash 2-dimensional sequences with electrocardiogram gating, whereas for anterior pituitary, the signal intensity was measured in sagittal T2 sequences. All scans were performed within 3 months. In 13 patients, data regarding liver iron concentrations (LIC) assessed by percutaneous liver biopsy were available. The mean of serum ferritin concentrations for 1 year before scans was calculated for each patient. MRI values in myocardium and liver showed a significant negative correlation to age (r=-0.73 and -0.69, respectively). For pituitary MRI, a linear regression with age was recorded in patients over 14 years of age (r=-0.67), whereas a relatively increased signal intensity reduction was recorded in pubertal subjects. Mean serum ferritin concentrations ranged from 252 to 5872 mug/L with an average of 1525+/-1047 mug/L. No statistical significant correlation was noted between mean ferritin levels versus liver, pituitary, and cardiac MRI values (r=-0.49, -0.28, and -0.1, respectively). Mean LIC values assessed by percutaneous biopsy were 13.76+/-11.6 mg/g of dry tissue. A statistically significant negative correlation was observed between liver MRI readings and LIC determined by biopsy (r=-0.89). None of the 3 organs studied with MRI were significantly correlated to each other. Pituitary to liver MRI values and liver to myocardial MRI values were moderately correlated (r=0.34 and 0.42, respectively). Pituitary MRI was not correlated at all to myocardial MRI (r=-0.001). In conclusion, iron accumulation in thalassemic patients is a procedure progressing with age, which seems to act independently in different organs. MRI represents a reliable, noninvasive method for assessing iron overload in various tissues, non-easily accessible with other techniques. Regular scanning, to recognize preclinically excessive iron deposits and intensified chelation therapy, can prevent serious and fatal complications.

摘要

重型β地中海贫血患者各器官中铁蓄积不均所导致的临床并发症会影响预期寿命和生活质量。磁共振成像(MRI)提供了一种定量、无创且准确的方法来估计各种组织中的铁含量,而其他技术难以轻易获取这些信息。本研究的目的是评估并关联通过MRI评估的重型β地中海贫血儿童和青年不同器官(垂体前叶、心肌和肝脏)中的铁蓄积水平。对30例接受传统治疗的纯合子β地中海贫血儿童和青年(13例女性和17例男性患者)进行了肝脏、心肌和垂体MRI检查。对于肝脏和心肌,我们计算了心电图门控的快速二维序列中信号与空气比值的自然对数,而对于垂体前叶,则在矢状T2序列中测量信号强度。所有扫描均在3个月内完成。13例患者有经皮肝活检评估的肝脏铁浓度(LIC)数据。计算了每位患者扫描前1年血清铁蛋白浓度的平均值。心肌和肝脏的MRI值与年龄呈显著负相关(分别为r = -0.73和-0.69)。对于垂体MRI,14岁以上患者记录到与年龄的线性回归(r = -0.67),而青春期受试者记录到信号强度相对增加的降低。平均血清铁蛋白浓度范围为252至5872μg/L,平均为1525±1047μg/L。平均铁蛋白水平与肝脏、垂体和心脏MRI值之间未发现统计学显著相关性(分别为r = -0.49、-0.28和-0.1)。经皮活检评估的平均LIC值为13.76±11.6mg/g干组织。观察到肝脏MRI读数与活检确定的LIC之间存在统计学显著负相关(r = -0.89)。研究的3个器官中,彼此之间均无显著相关性。垂体与肝脏MRI值以及肝脏与心肌MRI值呈中度相关(分别为r = 0.34和0.42)。垂体MRI与心肌MRI完全不相关(r = -0.001)。总之,地中海贫血患者的铁蓄积是一个随年龄进展的过程,似乎在不同器官中独立起作用。MRI是评估各种组织中铁过载的可靠无创方法,而其他技术难以轻易获取这些信息。定期扫描以识别临床前过量的铁沉积并强化螯合治疗,可以预防严重和致命的并发症。

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