儿科学精品教学（广西医科大学）泌尿系统疾病.pptVIP

Prednison 2mg/（kg·d）for 8 weeks 1、steroid-sensitive NS 2、steroid-resistant NS 3、steroid-dependent NS 4、relapse and frequently relapse typing in hormone curative effect minimal change disease(８０%) non-minimal change disease: (1）mesangial proliferative nephritis(系膜增生性肾炎) (2）focal segmental glomerulosclerosis(局灶性节段性肾小球硬化) (3）membranous nephropathy (膜性肾病) (4）membranoproliferative nephritis (膜增生性肾炎) Pathologic typing CAUSES OF NEPHROTIC SYNDROME Disease Children(%) Adults(%) Minimal change GN 75 20 Membanous GN 5 40 MPGN I 5 5 Other GN 5 20 正常肾小球电镜表现 微小病变(MCD)电镜：足突融合 PNS的病因及发病机制尚未明确 ①微小病变型—静电屏障损伤（T细胞免疫失调） ②非微小病变型—分子屏障损伤（免疫复合物） ③遗传基础：激素敏感型（HLA-DR7） 频复发（HLA-DR9） 家族性表现 ④自1998年以来对足细胞及裂孔隔膜的认识从超微结构跃升到细胞分子水平。“足细胞分子”(nephrin、CD2-AP、podocin、a-actinin-4)是发生蛋白尿的关键分子。 massive proteinuria normal ： qualitation（—） quantitation: 30～100mg/d NS patient: qualitation （+++～++++） quantitation：40mg/(h.m2) or 50mg/(kg.d) pathophysiology why NS patients have massive proteinuria？ permeability of glomerular basement membrane increased 1.静电屏障作用(static electricity barrier（selective proteinuria — MCD) 2. 分子滤过屏障作用(molecule filtration barrier ) （non-selective proteinuria —non-MCD) Proteinuria Albumin Immunoglobulins (immunity) Transferrin (anemia) Vitamin D-BP (rickets) Low serum proteins fluid shift into interstitial spaces Low Blood Volume Edema Kidneys Respond Retain Na and fluids!!!! Sequence of Events in Nephrotic Syndrome Pathophysiology Glomerular injury Increased permeability of glomerular basement membrane Albuminuria Decreased serum albumin Decreased plasma oncotic pressure Decreased peripheral capillary return Increased renal