先天性上睑下垂患者提上睑肌中异常细胞外物质的研究  

Abnormal Extracellular Material in the Levator Palpebrae Superioris Congenital Ptosis

作　　者：任晓霞[1] 康宛夏[1] 李光玲[1] 

机构地区：[1]山西省眼科医院眼整形科,太原030002

出　　处：《中国实用眼科杂志》2007年第7期709-712,共4页Chinese Journal of Practical Ophthalmology

摘　　要：目的探讨先天性上睑下垂患者提上睑肌的组织病理学改变,从而对其病因进行探讨。方法对30例43眼先天性上睑下垂患者按肌力大小不同分为轻度（7mm以上）、中度（4～6）mm、重度（0～3）mm3组,行常规提上睑肌缩短术,通过光镜、电镜和免疫组化的方法检测切除下的前部提上睑肌组织标本,应用图像分析系统对提上睑肌组织中细胞外基质中的Ⅲ型胶原和纤维连接蛋白作定量检测,即测定其平均积分光密度（IOD）。结果光镜下所有标本显示提上睑肌组织有不同程度的肌纤维数目减少,排列疏散,走形紊乱,间质中胶原纤维增多,肌浆内出现大量脂肪空泡等病理性改变。另外,在所有这30例（43眼）患者的提上睑肌中出现了一种异常的细胞外物质,这些异常的细胞外物质表现为少见的、排列成块状和带状的无定形物质。在电镜下显示它们由平行排列的原纤维颗粒组成的粗糙束带构成。通过免疫组化的方法证实这些物质中含有Ⅲ型胶原和纤维连接蛋白,而没有发现Ⅰ、Ⅱ、Ⅳ、Ⅴ、Ⅵ和Ⅶ型胶原,同时这种物质也不被肌动蛋白、肌球蛋白、肌凝蛋白和淀粉样P物质或A物质染色。Ⅲ型胶原和纤维连接蛋白的积分光密度在各组间的表达差异有统计学意义（p〈0.05）。结论在30例（43眼）先天性上睑下垂患者的提上睑肌标本中,我们发现了一种少见的无定形细胞外物质,免疫组化方法证明为Ⅲ型胶原和纤维连接蛋白,这种新物质,我们称之为“无定形胶原样物质”,它与患者提上睑肌功能呈负相关,可能是抗体在识别抗原时,激活肌内膜纤维母细胞而产生的胶原和纤维,是提肌在变性过程中肌细胞合成功能紊乱的一种表达方式,可能代表一种提肌组织发育不全的产物,它的来源和全部组成值得进一步研究。所以将提肌变性可导致先天性上睑下垂作为解释是最可靠的,而引起变�Objective To study the pathologic abnormalities of the levator palpebrae superioris in congenital ptosis. Methods 43 eyes of 30 cases with congenital ptosis were divided into three groups, muscle expansion of group Ⅰ was above 7mm, group Ⅱ was 4-6mm, group Ⅲ was 0-3mm. By means of light and electron microscopy and immunohistochemistry,anterior levator tissue specimen escised during routine levator resections were examinded. Integration optic density （IOD） of collagen type and fibeonectin in congenital ptosis＇s lebator muscles were measured with computerized image analysis. Results L All specimens showed lack of muscle fibers which arranged irregulary, there were a large of adipose in the muscles.In addition,in the 30 cases,an abnormal extracellular material was present.By light microscopy this appeared as an unusual amorphous material arranged in clumps and bands, and electron microscopy showed it to consist of parallel coarse bundles of fibrillogranular material.Collagen Ⅲ and fibronectin were identified within this material by immunohistochemistry.There was no detectable collagen Ⅰ、Ⅱ、Ⅳ、Ⅴ、Ⅵ and Ⅶ,and the material did not stain for actin,myosin, myolobin,amyloid P component,or amyloid A.The expression of Collagen and fibronectin in the different groups was significant（p〈0.05）. Conelusion In 43 samples of levator palpebrae superioris from patients with congenital that stained positively for collagen Ⅲ and fibronectin on immunohistochemistry,this novel material,which we call ＂amorphocollagenoid＂ may represent a product of dysgenesis of the levator tissues. The source and full composition of this material merit further study.

关 键 词：先天性上睑下垂 提上睑肌 细胞外异常物质 免疫组化 Ⅲ型胶原 纤维连接蛋白 

分 类 号：R777.1[医药卫生—眼科]