儿科泌尿系统-NS2英文课件
68页1、Nephrotic Syndrome(NS),站,Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia.,The vast majority patients (90% of cases) with NS of childhood are primary.,Definition In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood.,Conditions Of Attack Second only to acute nephri- tis Incidence age: at all ages, but most commonly between 35 years of age,Type 1. Clinical type
2、Simple type NS ; Nephritic NS 2. Response to steroid therapy (P274),Type The initial response to cortico- steroids is a guide to prognosis Steroid-dependent NS Steroid-responsive NS Steroid-resistant NS,Type Total effect Partial effect Non-effect,Type 3. Pathologic type (P282) Minimal change disease, MCD 80% of all NS, little change on light microscopy,Type but electron microscopy shows effacement of foot processes (podocytes) of epithelial cells,Type Other forms (Non-MCNS) MSPGN, FSGN, MGN, MPG
3、N, etc.,Pathogenesis The primary disorder is an increase in glomerular permeability to plasma proteinsloss of proteins, mainly albumin in urine,Pathogenesis Filtrated barrier Charge barrier Molecular barrier The loss of the negative charges on the GBM,Pathogenesis The underlying pathogenesis is unknown, but evidence strongly supports the impor- tance of immune mechanisms (P281),Pathophysiology 1. Proteinuria: fundamental and highly important change of pathophysiology,Pathophysiology 2. Hypoprote
4、inemia: mainly albumin 3. Edema: nephrotic edema (pitting edema),Pathophysiology Hypoproteinemiaplasma oncotic pressure, result in a shift of fluid from intervas- cular to extravascular com- partment edema,Pathophysiology Plasma volumeactivates the reninangiotensinaldo- sterone system, also ADH secretion Na and water reabsorption,Pathophysiology 4. Hyperlipidemia (Hyper- cholesterolemia): Ch,TC, LDL-ch, VLDL-ch,Pathophysiology Caused by: 1. Hypoproteinemia stimulates liver protein synthesis incl
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