肾病综合征(英文)

Preparedby:-MohammadAliAl-shehri…..Supervisedby:Dr.NephroticSyndrome..…(NS)NephroticsyndromeNephroticsyndrome(NS)resultsfromincreasedpermeabilityofGlomeulrarbasementmembrane(GBM)toplasmaprotein.Itisclinicalandlaboratorysyndromecharacterizedbymassiveproteinuria,whichleadtohypoproteinemia(hypo-albuminemia),hyperlipidemiaandpittingedema.(4-increase,1-decrease).NephroticCriteria:-*Massiveproteinuria:qualitativeproteinuria:3+or4+,quantitativeproteinuria:morethan40mg/m2/hrinchildren(selective).*Hypo-proteinemia:totalplasmaproteins5.5g/dlandserumalbumin:2.5g/dl.*Hyperlipidemia:serumcholesterol:5.7mmol/L*Edema:pittingedemaindifferentdegreeNephriticCriteria-Hematuria:RBCinurine(grosshematuria)-Hypertension:≥130/90mmHginschool-agechildren≥120/80mmHginpreschool-agechildren≥110/70mmHgininfantandtoddler’schildren-Azotemia（renalinsufficiency）:IncreasedlevelofserumBUN、Cr-Hypo-complementemia:Decreasedlevelofserumc3Classification:A-PrimaryIdiopathicNS(INS):majorityThecauseisstillunclearuptonow.Recent10years,increasingevidencehassuggestedthatINSmayresultfromaprimarydisorderofT–cellfunction.Accountingfor90%ofNSinchild.mainlydiscussed.B-SecondaryNS:NSresultedfromsystemicdiseases,suchasanaphylactoidpurpura,systemiclupuserythematosus,HBVinfection.C-CongenitalNS:rare*1st3montheoflife,onlytreatmentrenaltransplantationSecondaryNSDrug,Toxic,Allegy:mercury,snakevenom,vaccine,pellicillamine,Heroin,gold,NSAID,captopril,probenecid,volatilehydrocarbonsInfection:APSGN,HBV,HIV,shuntnephropathy,refluxnephropathy,leprosy,syphilis,Schistosomiasis,hydatiddiseaseAutoimmuneorcollagen-vasculardiseases:SLE,Hashimoto’sthyroiditis,,HSP,VasculitisMetabolicdisease:DiabetesmellitusNeoplasma:Hodgkin’sdisease,carcinoma(renalcell,lung,neuroblastoma,breast,andetc)GeneticDisease:Alportsyn,Sicklecelldisease,Amyloidosis,CongenitalnephropathyOthers:Chronictransplantrejection,congenitalnephrosclerosisIdiopathicNS(INS):Pathology:-MinimalChangeNephropathy(MCN):80%TheglomeruliappearnormalbasicallyUnderLightmicroscopy,andUnderImmunofluorescence*underElectronmicroscopy–fusionofthefootprocessesofthepodocytes(2)Non—MCN：＜20%*Mesangialproliferativeglomerulonephritis(MsPGN):about10%*Focalsegmentalglomerulosclerosis(FSGS):5%*MembranousNephropathy(MN):2%*Membraneproliferativeglomerulonephritis(MPGN):1%*Others：rare,CresentglomerulonephritisNB:-*Nephroticsyndromeis15timesmorecommoninchildrenthaninadults.*Mostcasesofprimarynephroticsyndromeareinchildrenandareduetominimal-changedisease.Theageatonsetvarieswiththetypeofnephroticsyndrome.Pathophysiology:TheMainTriggerOfprimaryNephroticSyndromeandFundamentalandhighlyimportantchangeofpathophysiology:-ProteinuriaPathogenesisofProteinuria:-IncreaseglomerularpermeabilityforproteinsduetolossofnegativechargedglycoproteinDegreeofprotineuria:-Mildlessthan0.5g/m2/dayModerate0.5–2g/m2/daySevermorethan2g/m2/dayTypeofproteinuria:-A-Selectiveproteinuria:whereproteinsoflowmolecularweight.suchasalbumin,areexcretedmorereadilythanproteinofHMWB-Nonselective:LMW+HMWarelostinurinepathogenesisofhypoalbuminemia*Duetohyperproteinuria-----Lossofplasmaproteininurinemainlythealbumin.*Increasedcatabolismofproteinduringacutephase.pathogenesisofhyperlipidemia:-*ResponsetoHypoalbuminemia→reflextoliver--→synthesisofgeneralizeprotein(includinglipoprotein)andlipidintheliver,thelipoproteinhighmolecularweightnolossinurine→hyperlipidemia*Diminishedcatabolismoflipoproteinpathogenesisofedema:-*Reductionplasmacolloidosmoticpressure↓secondarytohypoalbuminemiaEdemaandhypovolemia*Intravascularvolume↓antidiuretichormone(ADH)andaldosterone(ALD)waterandsodiumretentionEdema*Intravascularvolume↓glomerularfiltrationrate(GFR)↓waterandsodiumretentionEdemaHowmanypathologicaltypescausesnephroticsyndrome?ClinicalManifestation:-INMCNS,Themalepreponderanceof2:1:1.Mainmanifestations:Edema(varyingdegrees)isthecommonsymptomLocaledema:edemainface,aroundeyes(Periorbitalswelling),inlowerextremities.Generalizededema(anasarca),edemainpenisandscrotum.2-Non-specificsymptoms:Fatigueandlethargylossofappetite,nauseaandvomiting,abdominalpain,diarrheabodyweightincrease,urineoutputdecreasepleuraleffusion(respiratorydistress)Investigations:-1-Urineanalysis:-A-Proteinuria:3-4+SELECTIVE.b-24urinecollectionforprotein40mg/m2/hrforchildrenc-volume:oliguria(duringstageofedemaformation)d-Microscopically:-microscopichematuria20%,largenumberofhyalinecastInvestigations:-2-Blood:A-serumprotein:decrease5.5gm/dL,Albuminlevelsarelow(＜2.5gm/dL).B-Serumcholesterolandtriglycerides:Cholesterol＞5.7mmol/L(220mg/dl).C--ESR↑＞100mm/hrduringactivityphase.3.Serumcomplemen:Varywithclinicaltype.4.Renalfunction.KidneyBiopsy:-Consideredin:1-SecondaryN.S2-FrequentrelapsingN.S3-SteroidresistantN.S4-Hematuria5-Hypertension6-LowGFRDifferentialDiagnosisofNS:D.Dofgeneralizededema:-1-Protein–losingenteropathy2-HepaticFailure.3-HF4-Proteinenergymalnutrition5-AcuteandchronicGN6-urticaria?AngioedemaComplicationsofNS:-1-Infections:Infectionsisamajorcomplicationinchil