本文選題：Duchenne型肌營養(yǎng)不良 + 免疫組織化學(xué)��； 參考：《中南大學(xué)》2014年碩士論文

【摘要】：目的：旨在分析Duchenne型肌營養(yǎng)不良(Duchenne muscular dystrophy, DMD)患者骨骼肌標(biāo)本中結(jié)締組織生長因子(CTGF)和轉(zhuǎn)化生長因子(TGF-β1)的表達(dá)情況,進(jìn)一步了解其發(fā)病機(jī)制；探討DMD患者骨骼肌中CTGF、TGF-β1表達(dá)與確診年齡、病理改變嚴(yán)重程度、臨床嚴(yán)重程度之間的關(guān)系,進(jìn)一步明確兩個因子的臨床意義。 方法：選擇臨床上擬診DMD的患兒行骨骼肌活檢,肌纖維膜上Dystrophin蛋白嚴(yán)重缺失患兒18例入選DMD組。正常對照組為臨床懷疑肌病,但肌活檢示正常肌肉組織者8例。各組均行免疫組織化學(xué)染色,應(yīng)用Image-proplus6.0圖像分析軟件檢測并比較各組中的CTGF、TGF-β1表達(dá)情況,并分析DMD患者CTGF和TGF-β1與確診年齡、病理改變嚴(yán)重程度、臨床嚴(yán)重程度之間的相關(guān)性。 結(jié)果：1.臨床資料：18例DMD患者均為男性,平均確診年齡6.88+2.33歲。首發(fā)臨床表現(xiàn)以雙下肢癥狀起病多見,如獨立行走后步態(tài)不穩(wěn)、易跌倒,上樓梯困難等。2.組織化學(xué)染色：所有DMD患者均呈典型肌營養(yǎng)不良改變。3.免疫組織化學(xué)染色：CTGF、TGF-β1免疫組織化學(xué)染色積分光密度(Integrated optical density, IOD) DMD組與正常對照組比較有顯著統(tǒng)計學(xué)差異(P0.05)。DMD患者骨骼肌胞漿及肌間間質(zhì)內(nèi)CTGF、TGF-β1表達(dá)程度與確診年齡無明顯相關(guān)(P0.05),與病理改變程度分級和臨床嚴(yán)重程度分級呈顯著相關(guān)(P0.05)。 結(jié)論：1.DMD患者骨骼肌纖維胞漿、基膜及肌間間質(zhì)內(nèi)CTGF、 TGF-β1表達(dá)增強(qiáng)；2.臨床病變程度和病理改變程度越重,DMD患者骨骼肌肌纖維胞漿及肌間間質(zhì)內(nèi)CTGF、TGF-β1表達(dá)增強(qiáng)也更明顯,推測CTGF、TGF-β1參與DMD纖維化過程,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.
【學(xué)位授予單位】：中南大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R746.2

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