| 1个遗传性血色病家系的临床MRI诊断 |
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| 引用本文： | 李爱华,黄金明,孔祥泉,叶进,侯晓华.1个遗传性血色病家系的临床MRI诊断[J].临床肝胆病杂志,2011,27(3):302-305. |
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| 作者姓名： | 李爱华 黄金明 孔祥泉 叶进 侯晓华 |
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| 作者单位： | 1. 华中科技大学同济医学院附属协和医院,消化内科,武汉,450022
2. 华中科技大学同济医学院附属协和医院,放射科,武汉,450022 |
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| 摘 要： | 目的探讨一个遗传性血色病（HH）家系（A）及其旁系（B）的临床MRI特点。方法对A、B家系成员进行临床、生化、肝穿活检组织铁染色及多器官MRI检查。结果 （1）部分成员有血色病相关临床表现及异常生化指标;（2）3例患者接受肝活检,发现2例肝细胞内较多铁质沉积,另1例未见明显铁沉积;（3）MRI：部分成员心肝脾胰有不同程度的铁质沉积,表现为低信号影,肾脏、大脑均未见铁沉积。结论 （1）该血色病家系HH患者铁质主要沉积在肝脏,其他脏器如心脏、脾脏、胰腺亦有累及;而其旁系可见部分成员生化异常及脏器铁沉着,但尚未发现相关血色病临床表现,是否处于发病早期尚待随访观察;（2）与肝活检相比,MRI对组织铁沉积诊断较为准确。
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| 关 键 词： | 血色素沉着症 磁共振成像 |
The magnetic resonance imaging findings in a hereditary hemochromatosis pedigree |
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| LI Ai-hua,HUANG Jin-ming,KONG Xiang-quan,et al..The magnetic resonance imaging findings in a hereditary hemochromatosis pedigree[J].Chinese Journal of Clinical Hepatology,2011,27(3):302-305. |
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| Authors: | LI Ai-hua HUANG Jin-ming KONG Xiang-quan |
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| Institution: | LI Ai-hua,HUANG Jin-ming,KONG Xiang-quan,et al.(Division of Gastroenterology,Union Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,China) |
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| Abstract: | Objective To describe the clinical and magnetic resonance imaging(MRI) features of a hereditary hemochromatosis(HH) pedigree(A) and its collateral(B).Methods Clinical,biochemical,liver biopsy and MRI studies were carried out among the family members.Results(1)Some of the pedigree members showed hemochromatosis correlating symptom and physical sign.The biochemical values presented different abnormal elevation.(2)3 patients received liver biopsy,of which 2 patients suffered from extensive hepatic hemosiderin ... |
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| Keywords: | hemochromatosis magnetic resonance imaging |
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