Ocular manifestations of hereditary primary systemic amyloidosis.
Ocular manifestations of hereditary primary systemic amyloidosis.
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遗传性原发性系统性淀粉样变性的眼部表现。
DOI：
10.1001/archopht.1955.00930020666004
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发表时间：
1955
期刊：
影响因子：
--
通讯作者：
W. Block
中科院分区：
文献类型：
--
作者：
H. Falls;J. Jackson;J. H. Carey;J. G. Rukavina;W. Block
遗传性原发性系统性淀粉样变性是淀粉样蛋白问题的一种不寻常的表现，其特征是蛋白质的变化，明显地模仿了普通和模糊的蛋白质。到目前为止，世界文献中只有四篇报告详细描述了临床、遗传和病理状况。最近出现了三个报告，强调眼器官的显着参与。至于这种普遍的和病态的过程的起源，在过去的半个世纪里，文献中出现了许多猜测。一方面，人们认为病理生理学代表了免疫反应的最终结果，另一方面，它被认为是结缔组织基质的异常。在很大程度上，这些观察结果是基于基本的动物实验数据，尽管它们有已知的局限性。最近有人提出继发性（实质性）和原发性（间质性）系统性淀粉样变性可能代表类似的
Hereditary primary systemic amyloidosis, an unusual expression of the amyloid problem, is characterized by a protean symptomatology that remarkably mimics the commonplace and the obscure. To date only four reports have appeared in the world literature describing in some detail the clinical, genetic, and pathologic constellation.* Recently three reports have appeared which emphasize the remarkable involvement of the ocular apparatus.† As to the genesis of this universal and morbid process, much speculation has appeared in the literature in the past halfcentury. On the one hand, it has been held that the pathophysiology represents the endresult of an immunological reaction, and, on the other, it has been regarded as an anomaly of the connective tissue ground substance. For the greater part, these observations have been based upon, with their known limitation, fundamental animal experimental data. It has recently been suggested that secondary (parenchymatous) and primary (mesenchymal) systemic amyloidosis might represent similar