Bohle A, Ratschek M
Pathol Res Pract. 1982 Oct;174(4):357-67. doi: 10.1016/S0344-0338(82)80017-4.
Decompensated benign nephrosclerosis, a disease which was briefly described by Theodor Fahr in 1925, defended by him in 1934, and then forgotten, is reported and differentiated from compensated benign nephrosclerosis. Decompensated benign nephrosclerosis can be differentiated from compensated benign nephrosclerosis by the frequent appearance of interstitial cortical fibrosis and glomerular alterations in the sense of hypertensive glomerulopathy. Hypertensive glomerulopathy results in ascending obliteration of the glomeruli, i.e., their transformation into PAS-positive hyaline globules. In compensated benign nephrosclerosis, interstitial fibrosis, if present at all, can usually be identified only in the subcapsular areas. In decompensated benign nephrosclerosis, however, a pyramid-like structure with its base at the corticomedullary border is formed. The severity of preglomerular vascular alterations does not differ in compensated and decompensated benign nephrosclerosis. Clinically, there is no significant difference between the degree of hypertension and the development of the disease. The nephritic symptoms are more pronounced in decompensated benign nephrosclerosis, which predominantly affects middle-aged men (male:female, 5.7:1), than in compensated benign nephrosclerosis. As a result, decompensated benign nephrosclerosis is frequently diagnosed and treated as chronic glomerulonephritis.
失代偿性良性肾硬化症,这种疾病由西奥多·法尔于1925年简要描述,1934年他为其辩护,之后便被遗忘,本文对其进行了报道,并与代偿性良性肾硬化症进行了区分。失代偿性良性肾硬化症可通过间质皮质纤维化和高血压性肾小球病意义上的肾小球改变的频繁出现与代偿性良性肾硬化症相区分。高血压性肾小球病导致肾小球进行性闭塞,即它们转变为PAS阳性透明小球。在代偿性良性肾硬化症中,间质纤维化(若存在的话)通常仅能在包膜下区域识别。然而,在失代偿性良性肾硬化症中,会形成一种底部位于皮质髓质交界处的金字塔样结构。肾小球前血管改变的严重程度在代偿性和失代偿性良性肾硬化症中并无差异。临床上,高血压程度和疾病发展之间没有显著差异。失代偿性良性肾硬化症的肾炎症状比代偿性良性肾硬化症更明显,失代偿性良性肾硬化症主要影响中年男性(男:女,5.7:1)。因此,失代偿性良性肾硬化症常被诊断并当作慢性肾小球肾炎进行治疗。