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1、Nephrotic Syndrome,NS 肾病综合征,Contents,Definition Classification Etiology and Pathogenesis Pathophysiology Clinical manifestation Complication Diagnosis Treatment Prognosis,Definition,NS is an accumulation of symptoms and signs for abnormal increase in perme-ability of the glomerular basement mem- bra
2、ne (GBM) to protein.,Clinical features:EdemaProteinuriaHypoalbuminemiaHyperlipidemia,NSAGN,Epidemiological Investigation Of Urine System In Hospitalization,The second commonest renal disease hospitalized in China,General Information,General Information,Occurs at any age: most common between the ages
3、 of 3 and 5 yearsGender: M:F=3.7:1,Classification of NS,Primary NS (PNS) Secondary NS Congenital NS,See P322-323,Primary Nephrotic Syndrome 原发性肾病综合征(PNS),Classification of PNS,Clinical classification:Simple type NS(SNS) Nephritic NS(NNS),See P322-323,Sensitivity to steroid therapy:Steroid-sensitive
4、NS (SSNS)激素敏感型NS Steroid-resistant NS (SRNS)激素耐药型NS Steroid-dependant NS (SDNS)激素依赖型NS,Classification of PNS,小儿内科学 P323,Pathologic classification: Minimal change NS (MCNS): Non-minimal change NS： Diffuse mesangial proliferation (DMP)弥漫系膜增生性肾炎 Focal segmental glomerulosclerosis (FSGS)局灶节段肾小球硬化 Membra
5、noproliferative glomerulonephritis (MPGN)膜增生性肾小球肾炎Membranous glomerulopathy (MN)膜性肾病,Classification of PNS,Etiology and Pathogenesis,Are unclear by far Possible mechanisms: T cell-mediated immune disorder (MCNS) Immune complex-mediated (Non MCNS),glomerular filtration membrane,分子屏障（孔径屏障）,静电屏障,上皮细胞足突
6、,内皮细胞,基膜,Sialoprotein(-),硫酸肝素糖蛋白(-),Electrostatic Barrier,Molecular Barrier,Negative charge of GBM lose, electrostatic Barrier disappear,increased permeability to proteins will result in MCNS,Etiology and Pathogenesis,Molecular Barrier is damaged, increased permeability to proteins will result in no
7、n-MCNS,Pathology,EM 10,000 MCNS,EM 10,000 Normal glomeruli,Finding on electron microscopy simply reveals effacement of the epithelial cell foot processes,FSGS,normal glomeruli,Non-minimal change dieases,Pathophysiology,Proteinuria Fundamental change Caused by :Damage in Electrostatic Barrier or Mole
8、cular Barrier The quantity of protein passing through GBM is greater than the tubular resorption capacity. Heavy proteinuria :24-hours urine protein quantitation50mg/Kgurine protein/creatinine ratio尿蛋白/尿肌酐: exceeds 2.0,HypoalbuminemiaCaused by Lost from urine Raise of proteolysis by tubular Loss of
9、albumin is more than synthesis in liverhypoalbuminemia: Alb 25g/L,Pathophysiology,微量元素载体蛋白丢失 激素结合蛋白丢失,EdemaCaused by Decreased plasma osmotic pressure The activation of the renin-angiotensinaldo-sterone system (RAAS) increase Characteristic: pitting edema凹陷性水肿,Pathophysiology,HyperlipidemiaCaused by
10、 Increased compensatory synthesis by liver Lipid conversion disorder (LDL) lost from urine (HDL) Serum Cholesterol5.7mmol/L,Pathophysiology,导致肾小球纤维化 导致高凝、血栓形成,肾病综合征病理生理,水肿,impairment of GBM,Increased permeability of GBM,massive proteinuria,Hypoalbuminemia,Plasma oncotic pressure,Edema,compensatory s
11、ynthesis and lipidprotein metabolism disorder,Hyperlipidemia,plasmavolume,Fluid shift to tissue space,Pathophysiology,activiation of RAAS,water-sodium retention,EdemaThe most common complainFeature : pitting, dependent edema Site : first periorbital edema, proceed into scrotum, limbs , ascites, pleu
12、ral effusions,Clinical Manifestations,ProteinuriaGlomerular capillary wall permeability Quantity is greater than the tubular resorption capacity Heavy proteinuria : 24-hours urine protein quantitation50mg/Kg urine protein to creatinine ratio 2.0,Clinical Manifestations,Hypoalbuminemia Result of urin
13、ary protein loss and low protein synthesis Serum albumin 5.7mmol/L, most serum lipoproteins increase（ Ch, TG, LDL , HDL ）,Clinical Manifestations,Clinical classification of PNS,Clinical Manifestations,Complications,The most common complication Lead to failure of treatment or relapse of NS Atypical m
14、anifestations Fever and physical findings may be minimal because of cortical steroid therapy,Infections,Complications,Reasons: Decreased IgG levels Edema acting as a potential culture media Immunosuppressive therapy,Infections,Pathogen: viral , bacterial, particularly streptoccocus pneumoniae, fungi
15、 Site: Respiratory tractUrinary tractSkin Spontaneous peritonitis ，Sepsis, Cellulitis,Complications,Infections,Reasons: salt prohibitionexcessive usage of diuretic ( iatrogenic医源性) hypokalemia低钾血症hyponatremia 低钠血症hypocalcaemia低钙血症,Complications,Electrolyte disturbance,Complications Thrombosis,Increa
16、sed tendency to develop arterial and venous thrombosis,Reasons: Elevated coagulation factors and inhibitors of fibrinolysis Decreased anti-thrombin III Increased platelet aggregation,下肢静脉血栓 下肢水肿固定差别 不随体位改变,下肢动脉血栓 皮温下降、动脉搏动消失、疼痛,肾静脉血栓 急性-发作性血尿、腰痛 慢性-蛋白尿持续不缓解,Urinalysis: protein: 2+4+，granular and red
17、 cell casts24hr total urinary protein quantitation 50mg/kg，Urine protein to creatinine ratio 2.0 Albumin levels25g/L .Serum cholesterol and triglycerides: Total Cholesterol 5.7mmol/L (220mg/dl). Renal function: varying degree of decline Immunologic tests:Serum complement level : vary with clinical type.low level immunoglobin G( IgG) Renal biopsy,
