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[结节性硬化症的伯恩维尔型肾衰竭:一种因超滤导致的新型肾小球损伤?]

[The kidney failure of Bourneville's tuberous sclerosis: a new form of glomerular damage as a result of hyperfiltration?].

作者信息

Schillinger F, Montagnac R, Grapin J L, Birembaut P, Hopfner C

出版信息

Nephrologie. 1985;6(5):219-23.

PMID:4094635
Abstract

Chronic renal failure, rarely seen in tuberous sclerosis (TS), is usually related to renal hamartomas and/or cysts, but has only been very rarely linked to glomerular changes. In a patient with TS, we have performed repeated renal biopsies at early and advanced stages of renal failure. We have correlated the renal function deterioration with the evolution of focal and segmental glomerulosclerosis. Renal insufficiency would be in relation not only with renal involvement by multiple tumors, but also with the progression of focal and segmental glomerulosclerosis. This would be caused by hemodynamic changes in remnant glomeruli, after reduction in renal mass induced by renal tumors, and would be a new example of renal injury due to hyperfiltration.

摘要

慢性肾衰竭在结节性硬化症(TS)中很少见,通常与肾错构瘤和/或囊肿有关,但与肾小球改变的关联极为罕见。在一名TS患者中,我们在肾衰竭的早期和晚期进行了多次肾活检。我们将肾功能恶化与局灶节段性肾小球硬化的进展进行了关联。肾功能不全不仅与多个肿瘤累及肾脏有关,还与局灶节段性肾小球硬化的进展有关。这可能是由于肾肿瘤导致肾实质减少后,残余肾小球的血流动力学改变引起的,并且是超滤导致肾损伤的一个新例子。

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