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弹性组织发育异常和弹性组织营养不良作为翼状胬肉和睑裂斑的病理基础。

Elastodysplasia and elastodystrophy as the pathologic bases of ocular pterygia and pinguecula.

作者信息

Austin P, Jakobiec F A, Iwamoto T

出版信息

Ophthalmology. 1983 Jan;90(1):96-109. doi: 10.1016/s0161-6420(83)34594-2.

Abstract

Specimens of normal conjunctiva from three adult patients were examined by electron microscopy, which revealed normal elastogenesis in the substantia propria, but more prominently, in the episcleral tissues. Ultrastructural examination of eight pterygia and three pinguecula also disclosed evidence of elastogenesis, but in these lesions the morphogenetic sequence of fiber formation was distorted, and the elastic fibers were abnormal. The zone of hyalinization of the substantia propria immediately beneath the epithelium was the only site that manifested clear-cut evidence of collagen degeneration, which assumed the forms of effacement of the longitudinal periodicity of the collagen fibers, and of microfibrillar unfurling of the ends of the collagen fibers. Fibroblastic activity and elastic fiber formation were inconspicuous in this region. Beneath the hyalinized zone were collections of eosinophilic granular material. This material was shown ultrastructurally to be composed of excessive numbers of hollow-centered microfibrils (an elastic fiber precursor), with a tendency to clump centrally in the larger aggregated sheets and to acquire electron-dense inclusions. Numerous fibroblasts were found within this material. Finally, the elastotic fibers of light microscopy represented an abnormal maturational phase of elastic fiber production. These abnormal elastic fibers had microfibrils at their peripheries, but numerous electron-dense inclusions were associated with focal zones of amorphous elastin deposition. Occasionally histiocytes appeared to be engulfing these abnormal fibers. We have concluded that a large component of pinguecula and pterygia is the result of newly synthesized elastic fiber precursors and abnormal maturational forms of elastic fibers (elastodysplasia) that undergo secondary degeneration (elastodystrophy). These structures are presumed to be elaborated by actinically damaged fibroblasts of the substantia propria.

摘要

对三名成年患者的正常结膜标本进行了电子显微镜检查,结果显示固有层存在正常的弹性纤维生成,但更显著的是在巩膜上组织中。对八个翼状胬肉和三个睑裂斑的超微结构检查也发现了弹性纤维生成的证据,但在这些病变中,纤维形成的形态发生序列发生了扭曲,弹性纤维也不正常。上皮正下方固有层的玻璃样变区域是唯一表现出明确胶原变性证据的部位,胶原变性表现为胶原纤维纵向周期性消失以及胶原纤维末端微原纤维展开的形式。该区域的成纤维细胞活性和弹性纤维形成不明显。在玻璃样变区域下方是嗜酸性颗粒物质的聚集。超微结构显示,这种物质由大量中空中心的微原纤维(弹性纤维前体)组成,在较大的聚集片中倾向于在中央聚集,并获得电子致密包涵体。在这种物质中发现了大量成纤维细胞。最后,光镜下的弹性变性纤维代表弹性纤维产生的异常成熟阶段。这些异常弹性纤维在其周边有微原纤维,但大量电子致密包涵体与无定形弹性蛋白沉积的局灶区域相关。偶尔可见组织细胞似乎在吞噬这些异常纤维。我们得出结论,睑裂斑和翼状胬肉的很大一部分是新合成的弹性纤维前体和经历继发性变性(弹性纤维营养不良)的弹性纤维异常成熟形式(弹性纤维发育异常)的结果。这些结构被认为是由固有层受到光化学损伤的成纤维细胞形成的。

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