Deschênes G, Martinat L
Service de néphrologie pédiatrique, hôpital Armand-Trousseau, 26, avenue du Docteur-Arnold-Netter, 75571 Paris, France.
Arch Pediatr. 2000 Dec;7(12):1318-29. doi: 10.1016/s0929-693x(00)00151-2.
Steroid-sensitive idiopathic nephrotic syndrome is a T-cell disorder associated with a functional renal impairment. The molecular mechanisms leading from the stimulation of the immune system to the clinical expression of the renal disease can be analyzed according to five biological events: 1) a Th2 activation of T-cells by interleukin-13; 2) a yet unidentified glomerular permeability factor from immune origin; 3) a molecular disorientation of slit diaphragms or glomerular basement membrane responsible for proteinuria; 4) a podocyte cytoskeleton rearrangement responsible for foot process effacement; and 5) renal avidity for sodium and edema formation resulting from a primary stimulation of tubular Na,K-ATPase and an increase of endothelial permeability.
类固醇敏感性特发性肾病综合征是一种与功能性肾功能损害相关的T细胞疾病。从免疫系统刺激到肾脏疾病临床表型的分子机制可根据五个生物学事件进行分析：1）白细胞介素-13对T细胞的Th2激活；2）一种来源不明的免疫性肾小球通透因子；3）导致蛋白尿的裂孔隔膜或肾小球基底膜分子紊乱；4）导致足突消失的足细胞细胞骨架重排；5）肾小管钠钾ATP酶的原发性刺激和内皮通透性增加导致的肾脏对钠的亲和力及水肿形成。
