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引用次数: 0
摘要
淀粉样变性是一组基于异常纤维生成的浸润性疾病,即某些蛋白质--淀粉样前体--在特定条件下能够形成不溶解的线状结构--纤维。淀粉样变性的现代分类以主要纤丝蛋白(淀粉样前体)的特异性原则为基础。全身型淀粉样变性主要包括涉及血浆蛋白的疾病。某些类型的淀粉样变性往往会影响某些靶器官。疾病的预后主要取决于哪个器官受到的影响最严重。系统性老年淀粉样变性主要影响心血管系统。血清淀粉样蛋白 A(AA 型)是一种在肝脏合成的急性期蛋白。在全身性 AA 淀粉样变性中,心血管系统受到影响的情况相对较少,约占 0.1-10%。
Amyloidosis is a group of infiltrative diseases based on abnormal fibrillogenesis, i.e. the ability of some proteins - amyloid precursors, under certain conditions, to form insoluble thread-like structures - fibrils. Modern classifications of amyloidosis are based on the principle of specificity of the main fibrillogen protein - the amyloid precursor. Systemic forms of amyloidosis mainly include diseases involving blood plasma proteins. Certain types of amyloidosis tend to affect certain target organs. The prognosis of the disease largely depends on which organ is most severely affected. In systemic senile amyloidosis, the cardiovascular system is predominantly affected. Serum amyloid A (AA type) is an acute-phase protein synthesized in the liver. The cardiovascular system is affected relatively rarely in systemic AA amyloidosis, in approximately 0.1-10% of cases.