Aguirre María Adela, Carretero Marcelina, Nucifora Elsa, Posadas María Lourdes
Hospital Italiano de Buenos Aires.
Rev Fac Cien Med Univ Nac Cordoba. 2021 Mar 12;78(1):83-87. doi: 10.31053/1853.0605.v78.n1.25202.
INTRODUCCIÓN: Amyloidosis is a rare chronic disease, characterized by extracellular deposition of fibrillar proteins in various organs and tissues. The clinical manifestations are based on the site of the deposits and the precursor protein involved. The main diagnostic tool is clinical suspicion. The prognosis is usually bleak. The objective of this study is to describe the events that happened over time to reach what we know today as amyloidosis.
an exploratory, narrative, historical study was carried out. Primary sources and affected documentary sources were reviewed. Development: The first descriptions of patients with amyloidosis date from the mid 1600 AD. These were anatomical descriptions, reported from autopsies of patients. The first approximations to the diagnosis were through stains with iodine and anilines, finally reaching X-ray diffraction, and microscopy with polarized light under staining with Congo red.
Despite being a nosological entity known for four centuries, the difficulties to characterize the origin and understand the pathophysiology of amyloidosis still remain. For decades there were controversies about the nomenclature and the nature of amyloid material. The diagnosis and management of this disease follow the control of advances in technology, currently being a challenge in the management of patients suffering from amyloidosis.
淀粉样变性是一种罕见的慢性疾病,其特征是在各种器官和组织中细胞外沉积纤维状蛋白质。临床表现取决于沉积物的部位和所涉及的前体蛋白。主要的诊断工具是临床怀疑。预后通常不佳。本研究的目的是描述随着时间推移发生的事件,以达到我们如今所知的淀粉样变性。
开展了一项探索性、叙述性的历史研究。查阅了主要资料来源和相关文献资料。发展历程:对淀粉样变性患者的首次描述可追溯到公元17世纪中叶。这些是通过对患者尸体解剖进行的解剖学描述。最初的诊断方法是使用碘和苯胺染色,最终发展到X射线衍射以及在刚果红染色下用偏振光显微镜观察。
尽管淀粉样变性作为一种疾病实体已为人所知达四个世纪,但在确定其起源和理解其病理生理学方面仍然存在困难。几十年来,关于淀粉样物质的命名和性质一直存在争议。该疾病的诊断和管理随着技术进步而不断发展,目前仍是淀粉样变性患者管理中的一项挑战。