本文選題：肝豆狀核變性 + 磁共振成像��； 參考：《安徽醫(yī)科大學(xué)》2014年碩士論文

【摘要】：背景肝豆狀核變性于1912年由Kinnier Wilson首次詳細描述而得名，故又稱Wilson病(Wilson’s disease，WD)，是一種常染色體隱性遺傳性代謝障礙疾病。肝豆狀核變性患者體內(nèi)銅代謝異常已被大量臨床試驗及病理學(xué)研究所證實，銅代謝異常成為目前診斷肝豆狀核變性的主要標準之一。同時WD是目前少數(shù)可以用藥物控制的神經(jīng)系統(tǒng)遺傳性疾病。臨床治療以金屬螯合劑驅(qū)銅治療為主，，青霉胺為其特效藥物。但是部分患者經(jīng)青霉胺治療后，療效欠佳，甚至出現(xiàn)病情加重的情況。進一步研究發(fā)現(xiàn)銅代謝與鐵代謝密切相關(guān)。臨床實驗及活體組織學(xué)標本證實WD患者體內(nèi)鐵代謝異常。磁敏感加權(quán)成像(SWI)是一種利用組織間磁敏感性不同而成像的新技術(shù)，能夠測量腦組織鐵含量的改變。 目的探討SWI在WD患者腦部的應(yīng)用價值及WD患者相位值改變與血清銅、銅藍蛋白、銅氧化酶之間的關(guān)系。 方法對20例確診WD患者及20名年齡性別相匹配的健康志愿者進行常規(guī)MRI平掃及SWI序列掃描，在校正相位圖上手動繪制感興趣區(qū)（ROI），分別測量雙側(cè)尾狀核頭、蒼白球、殼核、紅核、黑質(zhì)、丘腦的相位值。WD組與正常對照組各核團相位值比較采用兩樣本t檢驗行統(tǒng)計學(xué)分析。WD組各核團相位值與患者血清銅、銅藍蛋白、銅氧化酶進行Spearman相關(guān)分析。 結(jié)果WD組雙側(cè)尾狀核頭、蒼白球、殼核、紅核、黑質(zhì)、丘腦可見異常低信號。與正常對照組比較，WD組各核團信號減低更顯著，相位值明顯降低，差異有統(tǒng)計學(xué)意義（P0.05），且兩組間比較雙側(cè)殼核差異最顯著。 WD組雙側(cè)尾狀核頭、蒼白球、殼核、紅核、黑質(zhì)、丘腦相位值與患者血清銅、銅藍蛋白、銅氧化酶活力均無顯著相關(guān)關(guān)系。 結(jié)論WD患者腦內(nèi)灰質(zhì)核團有異常順磁性物質(zhì)沉積，SWI相位值可作為評估WD患者腦內(nèi)順磁性物質(zhì)的有效手段。
[Abstract]:Background Hepatolenticular degeneration was first described in detail by Kinnier Wilson in 1912. It is also called Wilson disease Wilsonian disease (WDN), which is an autosomal recessive hereditary metabolic disorder. The abnormality of copper metabolism in patients with hepatolenticular degeneration has been confirmed by a large number of clinical trials and pathological studies. The abnormality of copper metabolism has become one of the main criteria for the diagnosis of hepatolenticular degeneration. Meanwhile WD is one of the few genetic diseases of nervous system that can be controlled by drugs. In clinical treatment, metal chelating agent was used to drive copper mainly, penicillamine was its special drug. However, some patients were treated with penicillamine, the curative effect was not good, even the condition became worse. Further studies show that copper metabolism is closely related to iron metabolism. Clinical trials and in vivo histological specimens confirmed abnormal iron metabolism in WD patients. Magnetic sensitivity weighted imaging (SWI) is a new imaging technique which can measure the changes of iron content in brain tissue by using different magnetic sensitivity among tissues. Objective to investigate the application value of SWI in WD patients' brain and the changes of phase value and serum copper in WD patients. Methods the relationship between ceruloplasmin and copper oxidase was studied in 20 patients with WD and 20 healthy volunteers matched by age and sex. The region of interest was manually drawn on the corrected phase map. The bilateral caudate head, globus pallidus, putamen, red nucleus, substantia nigra were measured. Phase value of thalamus. WD group was compared with normal control group. Two samples t test were used to analyze the phase value of thalamus group and serum copper and ceruloplasmin. Results abnormal low signals were observed in both caudate nucleus head, globus pallidus, putamen nucleus, red nucleus, substantia nigra and thalamus in WD group. Compared with the control group, the signal intensity and phase value of all nuclei in WD group were significantly decreased, the difference was statistically significant (P 0.05), and the difference between the two groups was the most significant, and the bilateral caudate nucleus head, globus pallidus, putamen nucleus, red nucleus, substantia nigra were most significant in WD group. Phase value of thalamus and serum copper, ceruloplasmin, Conclusion the phase value of abnormal paramagnetic substance deposition in gray nucleus of WD patients can be used as an effective method to evaluate the paramagnetic substance in WD patients.
【學(xué)位授予單位】：安徽醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R445.2;R742.4

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