肾病综合征课件_13

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1、肾病综合征NephroticSyndrome武汉大学第一临床学院刘红燕DefinitionNephroticsyndrome(NS)isdefinedbythepresenceoffollowing:Heavyproteinuria(>3.5g/d)Hypoalbuminemia(serumalbumin<30g/L)PeripheraledemaHyperlipidemia/hypercholesterolemiaMajorcausesofNS(primaryoridiopathic)Mini

2、malchangedisease(微小病变型）Mesangialproliferativeglomerulonephritis（系膜增生性肾小球肾炎）Focalsegmentalglomerulonephritis（局灶节段硬化性肾小球肾炎）Membranousglomerulonephritis（膜性肾病）Membranoproliferativeglomerulonephritis（膜增殖性肾小球肾炎/系膜毛细血管性肾小球肾炎）MajorcausesofNS(secondaryorsyste

3、mic)Antoimmune:systemiclupuserythematosus(SLE)Endocrine:diabeticnephropathyInfections:HepatitisB,C,HIVAllergenic:Henoch-SchonlenpurpuraNeoplastic:Hodgkin’sandnon-Hodgkin’slymphomas,multiplemyelomaOthers:Amyloidosis,Hereditarynephritis,medicationsPatho

4、physiologyHeavyprotinuriaHypoalbuminemiaEdemaHyperlipidemiaHeavyproteinuriaDamagetothecharge-selectivebarrierofGMB→ultrafiltrationofnegativelychargedalbuminDamagetothesize-selectivebarrierofGMB→passsageoflagermolecularproteinsProteinfiltration>reabsor

5、ptioncapacityoftubulesHeperperfusion/Hyperfiltrationfactors(hypertension,protein-richdiet)→proteinuriaincreaseConsequenceofurinarylossofplasmaproteinsSerumprotein↓orhypoalbuminemiaIgG/Complement↓→infectionCoagulationcomponentalternation(anticoagulants

6、↓)→thrombosisHormone-bindingproteins↓/metal-bindingproteins↓→endocrineormetabolicabnormalitiesLagerproteinsmayincreaseHypoalbuminemiaUrinarylossalbumincatabolism↑bytubulesMalnutritionduetoGIchangesEdemaReducedplasmaoncoticpressure(colloidosmoticpressu

7、re)Hypovolemia→renin-angiotensin-aldosteron↑;Sympatheticnervouseandvasopressionsystems↑PrimaryrenalNa+retension(non-plasmaHormoneeffectsonthekidney)HyperlipidemiaOneofthesentinelfeaturesofNSwithnumerousalterationsinlipidsprofiles(hypercholesterolemia,

8、hypertriglyceridemia,LDLandVLDL↑)OverproductionbytheliveroflipoproteinsDecreasedcatabolismoflipoproteinsandtriglycerideMinimalchangesnephropathy(MCD,微小病变型肾病）OverviewandTerminologyFirstdescribedin1913byMonkas“lipoidnephrosis”otherterms“nildisea