Kemény E, Mohácsi G, Sonkodi S, Ormos J
Zentralbl Allg Pathol. 1983;128(5-6):379-89.
13 cases of renal amyloidosis were reclassified on the basis of the chemical types of the major amyloid fibril proteins. The classification (primary, secondary) depending on the absence or presence of a coexisting disease was found not to correlate with the classification based on the chemical types (AL, AA). It is concluded that in association with a disease capable of inducing amyloidosis amyloid of type AA does not necessarily appear. The deposited amyloid may be of AL type. No difference of decisive importance between the AL and the AA type was observed concerning the tissue distribution of the amyloid within the kidney. With respect to the cause of death, however, a significant difference was found between the chemical types. In amyloidosis of AA type, the cause of death was always renal insufficiency, whereas in the cases of AL type cardiac or hepatic insufficiency led to death. Differentiation of the types of amyloid proteins has gained practical importance, as it offers the possibility of selective therapeutic approaches. Their routine differentiation is possible with the potassium permanganate method.
根据主要淀粉样纤维蛋白的化学类型,对13例肾淀粉样变性病例进行了重新分类。发现根据是否存在并存疾病进行的分类(原发性、继发性)与基于化学类型(AL、AA)的分类不相关。得出的结论是,与能够诱发淀粉样变性的疾病相关联时,AA型淀粉样蛋白不一定会出现。沉积的淀粉样蛋白可能是AL型。在肾脏内淀粉样蛋白的组织分布方面,未观察到AL型和AA型之间具有决定性意义的差异。然而,就死亡原因而言,化学类型之间存在显著差异。在AA型淀粉样变性中,死亡原因总是肾功能不全,而在AL型病例中,心脏或肝功能不全导致死亡。淀粉样蛋白类型的区分具有实际重要性,因为它提供了选择性治疗方法的可能性。用高锰酸钾法可以对它们进行常规区分。