Nephrotic Syndrome(NS)站.pptxVIP

Nephrotic Syndrome(NS) 第一课件网站 / Definition NS is an accumulation of symptoms and signs and is characterized by proteinuria, hypoproteinemia, edema, and hyperlipidemia. Definition In children under age 5 years the disease usually takes the form of idiopathic (primary) NS of childhood. The vast majority patients (90% of cases) with NS of childhood are primary. Conditions Of Attack● Second only to acute nephri- tis●Incidence age: at all ages, but most commonly between 3~5 years of age Type 1. Clinical type 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, MPGN, etc. Pathogenesis ? The primary disorder is an increase in glomerular permeability to plasma proteins→loss 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. Hypoproteinemia: mainly albumin 3. Edema: nephrotic edema (pitting edema) Pathophysiology ▲ Hypoproteinemia→plasma oncotic pressure↓, result in a shift of fluid from intervas- cular to extravascular com- partment →edema Pathophysiology ▲Plasma volume↓→activates the renin–angiotensin–aldo- sterone system, also ADH secretion↑→ Na and water reabsorption↑ Pathophysiology 4.?Hyperlipidemia (Hyper- cholesterolemia): Ch↑,TC↑, LDL