本文選題：Stargardt病 + 視神經(jīng)��； 參考：《鄭州大學(xué)》2017年碩士論文

【摘要】：背景和目的Stargardt病(Stargardt disease,STGD)是青少年最常見的一種遺傳性黃斑營養(yǎng)不良性眼底疾病,患者常以雙眼中心視力進(jìn)行性下降且不能矯正為首發(fā)癥狀,嚴(yán)重危害著青少年的視功能,全球患病率約為1/10000。STGD原發(fā)于視網(wǎng)膜色素上皮層,大量脂褐素沉積,感光細(xì)胞受損死亡,數(shù)目減少,最終視網(wǎng)膜萎縮,各層變薄。STGD具有高度的遺傳異質(zhì)性和復(fù)雜的臨床表型。ABCA4基因?yàn)橹饕闹虏』?為常染色體隱性遺傳。目前尚無有效的治療方法。視網(wǎng)膜正中央?yún)^(qū)因含有豐富的黃色素而稱為黃斑區(qū),是視網(wǎng)膜上視覺最敏銳的部位。光學(xué)相干斷層成像研究發(fā)現(xiàn)部分STGD患者視網(wǎng)膜神經(jīng)節(jié)細(xì)胞減少及神經(jīng)纖維層變薄,基于體素的形態(tài)學(xué)研究發(fā)現(xiàn)STGD患者視神經(jīng)、視交叉、外側(cè)膝狀體、視放射及視皮層體積變小,這些研究表明STGD患者病理改變不僅局限于視網(wǎng)膜,整個(gè)視路尚存在病理改變。彌散張量成像(diffusion tensor imaging,DTI)能夠在活體無創(chuàng)性定量研究腦白質(zhì)纖維束的微觀結(jié)構(gòu)及其病理生理變化,廣泛應(yīng)用于視神經(jīng)疾病的研究。相比于傳統(tǒng)DTI,小視野彌散張量成像技術(shù)(reduced field of view diffusion tensor imaging,rFOV-DTI)具有提高視神經(jīng)DTI圖像的分辨率,減少磁敏感偽影等優(yōu)勢(shì)。本研究擬采用rFOV-DTI技術(shù)對(duì)STGD患者視神經(jīng)進(jìn)行研究,以探討rFOV-DTI技術(shù)對(duì)STGD視神經(jīng)病變的臨床應(yīng)用價(jià)值。材料和方法分別收集22例STGD病人(均雙眼發(fā)病,44只眼)做為病變組和年齡性別與之相配的25例正常人(50只眼)做為對(duì)照組。所有研究對(duì)象均進(jìn)行視力檢查,并轉(zhuǎn)換為最小分辨角對(duì)數(shù)表達(dá)(logarithmofminimalangleofresolution,logmar)。運(yùn)用gemri7503.0t機(jī)器對(duì)所有研究對(duì)象進(jìn)行磁共振數(shù)據(jù)采集,包括頭顱常規(guī)mri、視神經(jīng)常規(guī)mri和rfov-dti檢查。運(yùn)用gefunctooladw4.5工作站進(jìn)行rfov-dti數(shù)據(jù)后處理得到視神經(jīng)的平均擴(kuò)散系數(shù)(meandiffusivity,md)圖、部分各向異性(fractionalanisotropy,fa)圖及本征向量圖,在b0圖上沿視神經(jīng)前、中、后三段手動(dòng)繪制3個(gè)感興趣區(qū)(regionofinterest,roi),每個(gè)roi的面積約為6mm2,分別計(jì)算每條視神經(jīng)三個(gè)感興趣區(qū)fa值、md值、λ//值、λ⊥值的平均值。采用兩獨(dú)立樣本t檢驗(yàn)比較病變組與正常對(duì)照組視神經(jīng)dti主要參數(shù)值之間的差異。采用配對(duì)樣本t檢驗(yàn)比較病變組左、右側(cè)視神經(jīng)dti主要參數(shù)值之間的差異。采用kruska-wallis檢驗(yàn)方法比較對(duì)照組及不同分期病人視神經(jīng)dti主要參數(shù)值之間的差異,并采用dunn-bonferroni檢驗(yàn)方法進(jìn)一步比較對(duì)照組及不同分期病人視神經(jīng)dti主要參數(shù)值兩兩之間的差異。采用spearman秩相關(guān)分析病變組logmar與dti主要參數(shù)值之間的關(guān)系。結(jié)果1.所有研究對(duì)象常規(guī)頭顱及視神經(jīng)磁共振未見明顯異常。2.與正常對(duì)照組相比,stgd病人視神經(jīng)fa值降低、md值、λ//值及λ⊥值均升高,差異具有統(tǒng)計(jì)學(xué)意義(p0.05)。病變組左、右側(cè)視神經(jīng)fa值、md值、λ//值及λ⊥值之間的差異無統(tǒng)計(jì)學(xué)意義(p0.05)。3.對(duì)照組及病變組四個(gè)分期病人視神經(jīng)fa值、md值、λ//值、λ⊥值總體分布不同,差異具有統(tǒng)計(jì)學(xué)意義(p0.05)。4.對(duì)照組與i期病人視神經(jīng)fa值、md值、λ//值、λ⊥值總體分布間的差異無統(tǒng)計(jì)學(xué)意義(p0.05);ii期病人fa值低于正常對(duì)照組(p0.05);iii期、iv期病人fa值低于正常對(duì)照組和i期病人(p0.05),md值、λ//值、λ⊥值則高于正常對(duì)照組和i期病人(p0.05),其余組間差異無統(tǒng)計(jì)學(xué)意義。5.病變組logmar與fa值之間呈負(fù)相關(guān)(r=㧟0.641,p0.05),與md值、λ//值、λ⊥值之間呈正相關(guān)(r=0.707,p0.05;r=0.689,p0.05;r=0.668,p0.05)。結(jié)論:1.rFOV-DTI技術(shù)較常規(guī)磁共振更能敏感地發(fā)現(xiàn)STGD視神經(jīng)病變,為STGD視神經(jīng)病理改變的診斷提供影像學(xué)依據(jù)。2.rFOV-DTI參數(shù)的變化可定量評(píng)價(jià)STGD視神經(jīng)纖維破壞的嚴(yán)重程度,且視神經(jīng)rFOV-DTI參數(shù)與LogMRA之間存在相關(guān)。3.STGD I期患者視神經(jīng)尚未出現(xiàn)病理改變,II期、III期、IV期視神經(jīng)出現(xiàn)不同程度的病理改變。
[Abstract]:Background and objective Stargardt disease (Stargardt disease, STGD) is one of the most common genetic macular malnutrition fundus diseases in adolescents. Patients often suffer from a progressive decline in the visual acuity of the binocular center and can not be corrected as the first symptom, which seriously endangers the visual function of young people. The global prevalence rate is about 1/10000.STGD on the retinal pigment. The cortex, a large number of lipofuscin deposits, the death of the photoreceptor cells, the decrease of the number, the eventual atrophy of the retina, and the thinning of the retina, the.STGD has a high genetic heterogeneity and a complex clinical phenotype.ABCA4 based on the main pathogenic gene, which is an autosomal recessive inheritance. There is no effective therapy at present. The central retinal area is rich in the retina. Yellow pigment, called macular region, is the most perceptive part of the retina. Optical coherence tomography found that the retinal ganglion cells in some STGD patients have decreased and the nerve fiber layer thinning. The volume of optic nerve, optic chiasma, lateral geniculate body, optic and visual cortex in STGD patients are smaller, based on the morphologic study of voxel. The study shows that the pathological changes of STGD patients are not only limited to the retina, but there are still pathological changes in the whole visual pathway. Diffusion tensor imaging (DTI) can be used to study the microscopic structure and pathophysiological changes of the white matter fiber bundles in the living body, and it should be widely used in the study of optic nerve diseases. Compared with the traditional DTI, it is less than the traditional DTI. The reduced field of view diffusion tensor imaging, rFOV-DTI has the advantages of improving the resolution of the optic nerve DTI image and reducing the magnetic sensitivity artifact. This study intends to use rFOV-DTI technique to study the optic nerve of STGD patients to explore the clinical application value of rFOV-DTI technology to the optic neuropathy. Materials and methods were used to collect 22 cases of STGD (both binocular and 44 eyes) as the lesion group and 25 normal persons (50 eyes) matched with age and sex (50 eyes) as the control group. All the subjects performed the visual examination and converted to the minimum resolution angle logarithmic expression (logarithmofminimalangleofresolution, LogMAR). The gemri7503.0t machine was used. All the subjects were collected for magnetic resonance data, including routine cranial MRI, routine MRI and rfov-dti examination of optic nerve. The average diffusion coefficient of optic nerve (meandiffusivity, MD), partial anisotropy (fractionalanisotropy, FA) and eigenvector were obtained by rfov-dti data processing by gefunctooladw4.5 workstation. In B0 On the graph, 3 regions of interest (regionofinterest, ROI) were manually drawn along the front, middle, and three segments of the optic nerve. The area of each ROI was about 6mm2. The mean values of FA, MD, / / / / / / value of each optic nerve were calculated respectively. The two independent sample t test was used to compare the main parameters of the optic nerve DTI in the lesion group and the normal control group. The difference between the main parameters of the left and right optic nerve DTI in the left and right optic nerve was compared with the paired sample t test. The difference between the main parameters of the optic nerve of the optic nerve in the control group and the different staging patients was compared by the kruska-wallis test, and the dunn-bonferroni test method was used to compare the visual gods of the control group and the different staging patients. The difference between the main parameter values of DTI was 22. Spearman rank correlation was used to analyze the relationship between the LogMAR and the main parameters of the DTI. Results 1. all the subjects with the normal head and the optic nerve MRI did not see the obvious abnormal.2. as compared with the normal control group, and the FA value of the optic nerve in the STGD patients decreased, the MD value, the value of lambda / / / the value and the lambda value were all increased. The difference was statistically significant (P0.05). There was no significant difference between the left of the lesion group, the FA value of the right optic nerve, the value of MD, the value of lambda and the value of lambda value (P0.05), the FA value of the optic nerve in the.3. control group and the four staging patients of the pathological group, the MD value, and the value of [lambda] / / / / / / / / / / / / / / value, the difference was statistically significant (P0.05) the.4. control group and the FA optic FA of the I patients. Value, MD value, and / / / / / / value, there was no significant difference between the total distribution of lambda values (P0.05), and the FA value of II patients was lower than that of the normal control group (P0.05), and the FA of IV patients was lower than the normal control and I patients (P0.05), MD value, and the value of lambda was higher than that of the normal control group and the I phase, and the difference between the other groups was not statistically significant. There was a negative correlation between the value of LogMAR and FA (r=? 0.641, P0.05), which was positively correlated with the value of MD, lambda / / / / / value, and [r=0.707, P0.05; r=0.689, P0.05; r=0.668, P0.05). Conclusion: 1.rFOV-DTI technology is more sensitive than conventional magnetic resonance to detect optic neuropathy, providing imaging based parameters for the diagnosis of optic neuropathological changes The changes can be used to quantitatively evaluate the severity of STGD optic nerve fiber damage, and there is no pathological changes in the optic nerve between the rFOV-DTI parameters of the optic nerve and LogMRA in the.3.STGD I phase, and the pathological changes of the optic nerve in II, III and IV stages.

【學(xué)位授予單位】：鄭州大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類號(hào)】：R445.2;R774.5

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本文編號(hào)：1780124