Duchenne型肌营养不良CTGF和TGF-β1表达的临床研究

发布时间：2018-06-04 10:29

本文选题：Duchenne型肌营养不良 + 免疫组织化学　；参考：《中南大学》2014年硕士论文

【摘要】：目的：旨在分析Duchenne型肌营养不良(Duchenne muscular dystrophy, DMD)患者骨骼肌标本中结缔组织生长因子(CTGF)和转化生长因子(TGF-β1)的表达情况,进一步了解其发病机制；探讨DMD患者骨骼肌中CTGF、TGF-β1表达与确诊年龄、病理改变严重程度、临床严重程度之间的关系,进一步明确两个因子的临床意义。方法：选择临床上拟诊DMD的患儿行骨骼肌活检,肌纤维膜上Dystrophin蛋白严重缺失患儿18例入选DMD组。正常对照组为临床怀疑肌病,但肌活检示正常肌肉组织者8例。各组均行免疫组织化学染色,应用Image-proplus6.0图像分析软件检测并比较各组中的CTGF、TGF-β1表达情况,并分析DMD患者CTGF和TGF-β1与确诊年龄、病理改变严重程度、临床严重程度之间的相关性。结果：1.临床资料：18例DMD患者均为男性,平均确诊年龄6.88+2.33岁。首发临床表现以双下肢症状起病多见,如独立行走后步态不稳、易跌倒,上楼梯困难等。2.组织化学染色：所有DMD患者均呈典型肌营养不良改变。3.免疫组织化学染色：CTGF、TGF-β1免疫组织化学染色积分光密度(Integrated optical density, IOD) DMD组与正常对照组比较有显著统计学差异(P0.05)。DMD患者骨骼肌胞浆及肌间间质内CTGF、TGF-β1表达程度与确诊年龄无明显相关(P0.05),与病理改变程度分级和临床严重程度分级呈显著相关(P0.05)。结论：1.DMD患者骨骼肌纤维胞浆、基膜及肌间间质内CTGF、 TGF-β1表达增强；2.临床病变程度和病理改变程度越重,DMD患者骨骼肌肌纤维胞浆及肌间间质内CTGF、TGF-β1表达增强也更明显,推测CTGF、TGF-β1参与DMD纤维化过程,是DMD疾病的共同致病因子。
[Abstract]:Objective: to analyze the expression of connective tissue growth factor (CTGF) and transforming growth factor (TGF- beta 1) in skeletal muscle specimens of patients with Duchenne muscular dystrophy (DMD), and to further understand its pathogenesis, and to explore the expression of CTGF, TGF- beta 1 and the age of diagnosis in skeletal muscle of DMD patients, and the severity of pathological changes in the skeletal muscle of patients with DMD. The clinical significance of the two factors was further clarified by the relationship between clinical severity.
Methods: 18 children with severe loss of Dystrophin protein on the myoffibrous membrane were selected and 18 children were selected from the DMD group. The normal control group was clinically suspected, but the muscle biopsy showed 8 normal muscle organizer. All the groups were stained with immunohistochemical staining, and the Image-proplus6.0 image analysis software was used to detect and compare the results. The expressions of CTGF and TGF- beta 1 in each group were analyzed, and the correlation between CTGF and TGF- beta 1 in DMD patients and the age of diagnosis, severity of pathological changes and clinical severity were analyzed.
Results: 1. clinical data: 18 cases of DMD patients were male, the average age of diagnosis was 6.88+2.33 years old. The first clinical manifestations were common with symptoms of lower limbs, such as unstable gait after independent walking, easy to fall, and staircase difficulties, such as.2. histochemical staining: all DMD patients showed.3. immunohistochemical staining of typical muscular dystrophy: CTGF, The integral light density of TGF- beta 1 immunohistochemical staining (Integrated optical density, IOD) DMD group was significantly different from that of the normal control group (P0.05) the CTGF in the skeletal muscle cytoplasm and intermuscular intermyosa of the patients with.DMD (P0.05), and the expression of TGF- beta 1 was not significantly correlated with the age of diagnosis (P0.05), and was divided into the grade of pathological changes and the clinical severity. The level was significantly correlated (P0.05).
Conclusion: the expression of CTGF and TGF- beta 1 in the skeletal muscle fibrous cytoplasm of 1.DMD patients, the enhancement of the expression of CTGF and TGF- beta in the intermuscular interstitium, the greater the degree of pathological changes and the severity of pathological changes, the enhanced expression of CTGF and TGF- beta 1 in the skeletal muscle fibers and intermuscular interstitium of the DMD patients is also more obvious. It is speculated that CTGF and TGF- beta 1 are involved in the DMD fibrosis process, which is the common cause of DMD diseases. Disease factor.
【学位授予单位】：中南大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R746.2

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