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1、DevelopmentalabnormalitiesandinflammationsofthekidneyandurinarytractCysticdiseasesCysticrenaldysplasiaPolycystickidney-Adult(AD)-Infantile(AR)MedullarycysticDialysisassociatedcysticSimplexcystsRenalcysticdiseaseassociatedwithinheriteddiseasesGlomerulocysticdiseasesExtraparenchymalrena
2、lcystsPolycystickidney(ADPKD)Frequency:1:400-1000CRF5-10%ADPKD1:16p13.3(85%)RF:40y.:5%,50y:35%,60y.:70%,70y:95%polycystin-1PKD2:4q21(15%)RF:0,5,15,45polycystin-2ScenarioofthedevelopmentoftheADPKDMutationCell-cell,cell-matrixrelationabnormalityAlteredepithelialproliferation,and.differe
3、ntiationAbnorm.ECMEpith.proliferationFluidsecretionVascularinjuryCYSTInterstitialinflammationfibrosisPolycystickidney–clinicalsignsPainHaematuriaProgressiverenalinsufficiencyProteinuria(notmorethan2g/day)PolyuriaHypertensionPolycystickidney–otheraccompaniingcongenitalanomaliesHepaticc
4、ystsBerryanerysmsMitralprolapse–othervalvularanomalitiesTh.:Dialysis,renaltransplatation†cause:40%:coronary/hypertensiveheartdisease25%:infection15%:aneurysmrupture,hypertensiveintracerebralhaemorrhage20%:othercausesRenaldysplasia (Cysticrenaldysplasia)Nomenclature!!!!SporadicUnilater
5、al,(mightbebi~)MetanephricdifferentiationabnormalityCombinedwithlowerurinarytractanomalies(Vesicoureteraljunctionobstruction,ureteragenesia)Clin.:PalpableabdominalmassClinicalmanifestationofrenaldisordersAcutenephritis:macroscopichaematuria,mildproteinuria,mildhypertensionNephrosis:se
6、vereproteinuria>3.5g/dayhypoalbuminaemia,oedema,hyperlipidemia,lipiduriaAcuterenalinsufficiency:abruptoligo/anuriaChronicrenalinsuff/failure:ClinicalmanifestationofrenaldisordersRenaltubulardefects:polyuria,electrolitdisorders(Metabolicacidosis)Urineinfections:pain,fever,etcNephrolith
7、iasis:colica,haematuriaObstruction:pain,infection,stoneformationClinicalmanifestationsofrenaldisordersChronicrenalinsuff:Decreasedrenalreserve:GFR<=50%Secreatinin,BUNnorm.FreeofsymptomsRenalinsufficiency:GFR20-50%Secreatinin,BUN^anaemia,hypertension,sign:polyuria,Renalfailure:GFR:<20-
8、25%,o
