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[淀粉样变性作为早老性和老年性退变的一种表现及根源]

[Amyloidosis as a manifestation and origin of presenile and senile degeneration].

作者信息

Schwartz P

出版信息

Fortschr Med. 1976 May 20;94(15):890-6.

PMID:134966
Abstract
  1. All essential attributes of the amyloidosis in aged persons ("senile amyloidosis") correspond to the condition which in younger individuals develops after infections, particularly following tuberculosis and lymphogranulomatosis, as so-called secondary amyloid degeneration, and also manifests many features of the so-called primary amyloidosis, not connected with infections. 2. Amyloid depositions in the brain, cardiac muscle, and in pancreatic islets (the "senile amyloidotic triad") dominate the morbid anatomic aspect. However, we know no organ or tissue which necessarily remains spared. The number of involved organs and tissues, in general, increases with the progressive aging of the patients. In those persons living long enough, amyloidosis affects every individual and probably all organs and tissues. 3. Contrary to the so-called secondary amyloidosis, in many cases of senile amyloidosis the spleen, liver and kidney remain intact. 4. In the so-called Alzheimers disease, in which both clinically and pathoanatomically a particularly destructive cerebral amyloidosis in relatively young persons prevails, just as in the common senile dementia of aged persons, the brain condition is associated with a systemic amyloid degeneration of many other organs. 5. Several cerebral and cardiac lesions due to amyloid accumulations can probably be diagnosed electrographically. Thus, through these already known morbid anatomical observations we have the promise of an essential enrichment of diagnostic perspectives. 6. In general, the etiologic manifoldness of amyloidosis presently seems to be incomparable. Infections, ionizing radiation, traumatic lesions in human pathology, the introduction of chemically definable substances, infections, and stress consequent to social burdening, proved effective in spontaneous and experimental amyloid degeneration of animals. 7. The demonstration of a tuberculous infection with the help of postmortem radiographs, as well as with the employment of histologic and microbiologic procedures to provide the evidence of acid fast bacilli in calcified remnants of pulmonary foci, proved to be eminently successful methods in the exploration of causes of senile tuberculosis and amyloidosis: Tuberculosis, after its invasion of the organism in early childhood, with its toxic and immunobiologic influences, holds it under its spell for an entire, even very long life and can be considered the most frequent cause of senile amyloidosis. 8. Chromosomal disturbances, with their hereditary manifestations, or, as in cases of mongoloid idiocy, associated with individual deformations, may present as amyloidoses. 9. Amyloid deposits in human pathology may develop by the transformation of normal structures, like cartilage, osteoid tissue, vascular elastic fibers, and also from scar hyalin and from fibrin. 10. We observed the disappearance of cerebral and cardiac amyloid accumulations producing typical defects. 11. Amyloidosis represents one of the most frequent spontaneous diseases of animals...
摘要
  1. 老年人淀粉样变性(“老年性淀粉样变性”)的所有基本特征与年轻人在感染后,特别是在结核病和淋巴肉芽肿病后发生的情况相对应,即所谓的继发性淀粉样变性,并且还表现出许多与感染无关的所谓原发性淀粉样变性的特征。2. 大脑、心肌和胰岛中的淀粉样沉积(“老年性淀粉样变性三联征”)在病理解剖方面占主导地位。然而,我们不知道有哪个器官或组织必然不会受累。一般来说,随着患者年龄的逐渐增长,受累器官和组织的数量会增加。在那些寿命足够长的人中,淀粉样变性会影响每个人,可能还会影响所有器官和组织。3. 与所谓的继发性淀粉样变性相反,在许多老年性淀粉样变性病例中,脾脏、肝脏和肾脏保持完好。4. 在所谓的阿尔茨海默病中,在相对年轻的人中临床上和病理解剖学上都存在特别具有破坏性的脑淀粉样变性,就像在老年人常见的老年性痴呆中一样,脑部病变与许多其他器官的全身性淀粉样变性有关。5. 由于淀粉样蛋白积累导致的一些脑部和心脏病变可能可以通过脑电图进行诊断。因此,通过这些已经为人所知的病理解剖学观察,我们有望极大地丰富诊断视角。6. 一般来说,目前淀粉样变性的病因多样性似乎是无与伦比的。感染、电离辐射、人类病理学中的创伤性病变、引入化学可定义的物质、感染以及社会负担导致的压力,在动物的自发性和实验性淀粉样变性中都被证明是有效的。7. 借助死后X光片证明结核感染,以及采用组织学和微生物学方法在肺部病灶钙化残余物中提供抗酸杆菌的证据,被证明是探索老年性结核病和淀粉样变性病因的非常成功的方法:结核病在儿童早期侵入机体后,凭借其毒性和免疫生物学影响,在整个甚至很长的生命过程中对机体施加影响,可以被认为是老年性淀粉样变性最常见的病因。8. 染色体紊乱及其遗传表现,或者,如在先天愚型痴呆病例中,与个体畸形相关联,可能表现为淀粉样变性。9. 人类病理学中的淀粉样沉积可能通过正常结构的转化形成,如软骨、类骨质组织、血管弹性纤维,也可由瘢痕透明质和纤维蛋白形成。10. 我们观察到产生典型缺陷的脑和心脏淀粉样蛋白积累消失了。11. 淀粉样变性是动物最常见的自发性疾病之一……

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