Bensman Albert
Service de Néphrologie Pédiatrique, Hôpital d'Enfants Armand Trousseau, 26, avenue du Dr Arnold Netter, 75012 Paris.
Bull Acad Natl Med. 2009 Nov;193(8):1727-33; discussion 1734.
Severe forms of the nephrotic syndrome are, by definition, steroid-resistant or strongly steroid-dependent. Steroid-resistant forms have two causes. The first is T and B lymphocyte dysfunction, which may result in the production of a lymphokine which increases the permeability of the glomerular filtration barrier. The second is mutation of genes that encode proteins involved in establishing and maintaining the glomerular filtration barrier. Mycophenolate mofetil and rituximab are effective new treatments for severe steroid-dependent nephrotic syndromes. The beneficial effect of cyclosporine on proteinuria may result from stabilization of the actin cytoskeleton in podocytes.
根据定义，重度肾病综合征是类固醇抵抗型或高度类固醇依赖型。类固醇抵抗型有两个病因。第一个是T和B淋巴细胞功能障碍，这可能导致产生一种增加肾小球滤过屏障通透性的淋巴因子。第二个是编码参与建立和维持肾小球滤过屏障的蛋白质的基因突变。霉酚酸酯和利妥昔单抗是治疗重度类固醇依赖型肾病综合征的有效新疗法。环孢素对蛋白尿的有益作用可能源于足细胞中肌动蛋白细胞骨架的稳定。
