髓质海绵肾ppt课件.ppt

1 Medullaryspongekidney MSK 2 MSKisarenalmalformationcharacterizedbycysticanomaliesofprecalycealducts whichisfrequentlyassociatedwithnephrocalcinosisandrenalstones amoderateriskofdevelopingurinaryinfectionsandrenalfailure髓质海绵肾是一种先天性的肾髓质囊性病变 其特征为肾锥体部乳头管及集合管呈梭形或囊状扩张 并伴发感染和肾钙质沉着 不是诊断的必须标准 尿路结石 肾衰 3 pathology 4 theprevalenceamongpeoplesubmittedtoi v urography forwhichtheremusthavebeenareason is0 5 1 sowecansafelyassumethattheseprevalencesaretoohigh About3 5 ofrenalstoneformershaveMSK althoughmuchlargerproportions upto20 havealsobeenreported 5 Thediseaseisinterestinginmanyrespects 1 theimpactofimagingtechniquesonitsdiagnosis bothinthepastandinthefuture whentheywillprobablychangeourapproachtothedisorder 2 theelusivepathogenesis wearestillfacingoldandgenerichypotheses anomalouscongenitaldevelopmentofrenaltubuleswithsecondarycysticdilations collectingductdilationsecondarytoobstructionbycalciumsalts renalmanifestationofasystemicconnectivetissuedisorder renalmanifestationofprimaryhyperparathyroidism 6 GUERRINOLENARDUZZI 1902 1985 7 Figure1 RepresentativeurographicappearanceofMSK Typicalpyramidalblushesareevident containingsmallradio opaquespots 8 9 AlthoughitwasfirstrecognizedbyGLenarduzziin1939 itsthoroughdescriptionwastheresultoftheantelitterammultidisciplinarycooperationbetweenaradiologist Lenarduzzi aurologist Cacchi andapathologist Ricci allatthePaduaUniversityHospital 10 CacchiwasafellowunderProfessorRavasini founderoftheInstituteofUrologyinPadua whenLenarduzziwasoperatinganddescribingMSKatthesameSchoolofMedicine TheycertainlysharedsomeMSKpatients RicciwasanassistantinthepathologydepartmentdirectedbyProfessorBompianiinPaduaandperformedthehistopathologicalexaminationonthekidney ROBERTOCACCHIANDVINCENZORICCI 11 pathogenesisofMSK ThepathogenesisofMSKshouldexplaintheinvolvementofanatomicaldistricts thecollectingandprecalycealductsontheonehandandthenephronontheother ofdifferentembryologicalorigins RecentfindingsuggestthatMSKmaybetheconsequenceofadisruptionofsaidinterface thedisruptionoftheureteral bud metanephric blastemainterface 12 AMODERNLOOKATMSK ONTHETRACKOFITSMOLECULARBASES 13 Thelatterareknowntobeassociatedwithalocusonchromosome11p15 whereasWilm stumorisassociatedwithalterationsintheWT 1geneat11p13 Thusthetwolociareveryclose supportingtheviewthattheoverlapbetweenthethreedisorderscoulddependonaderangementoccurringinthe11pchromosomearea alongthelinesoftheso called contiguousgenesyndromes 14 Intravenouspyelogramshowingaccumulationofcontrastinmultiplesmallcystsinvolvingthelowerpoleoftheleftkidneygivingtothepapillaetheappearanceofa bouquetofflowers characteristicofMSK 15 CLINICALASPECTS 1 Recurrentcalciumnephrolithiasisandnephrocalcinosisarethemostcommonsigns Theassociationwithrenalhypercalciuria distaltubularacidosis andhypocitraturia inconjunctionwithurinarystasisinthepapillaryductectasias triggerstheformationofcalciumphosphateand orcalciumoxalatestones 16 2 Hyperparathyroidismisfrequentlyassociated andwasthoughttocauseMSKandalsotriggerstoneformationinthesepatients 3 MSKisassociatedwithotherabnormalitiesofthelowertubule suchasadefectiveurinaryconcentration distalrenaltubularacidosisandhypocitraturia 4 Familialcaseshavebeenreported sometimesassociatedwithrenalagenesia otherrenalmalformationsorabnormalitiesintheurinarytract Anautosomaldominantpatternofinheritancehasbeensuggestedinfamilialcases 17 Clinicalmanifestations 海绵肾如无并发症可无特殊临床症状 而在x线检查中无意地发现 少数病例可有症状常于40岁 50岁时因合并结石和感染 反复的肾盂肾炎被发现 根据临床和放射学上的改变 将病人的初期症状分为3类 1 临床上没有症状或仅有轻微症状 在排泄性尿路造影片上只表现有特征性改变 但尿路平片上没有钙化现象 2 在尿路平片上呈现钙质沉着 病人表现为尿路感染 这种钙质沉着应与原发性甲状旁腺机能亢进及肾小管酸中毒等相区别 3 由于慢性炎症 肾小管结石破出锥体进入肾盏和肾盂 产生结石的典型症状 18 Clinicalmanifestations 1 血尿hematuria这是最常见的症状 约占85 而且反复发作 发作时可伴有腰痛或同时排出细砂样结石 一般为镜下血尿 也可见到个别病例表现为无痛性肉眼全程血尿 2 肾绞痛renalcolic可为早期症状 常多次发作 约占50 为结石排出时的伴随症状 结石排出后症状缓解 少数病例 结石在肾盂内逐渐长大 或嵌顿在输尿管中 需要手术或碎石处理 3 肾盂肾炎pyelonephritis约有50 病人以肾盂肾炎发病 即锥体内乳头管 集合管及扩张的囊腔内发生感染 波及整个尿路 严重者可蔓延到囊腔周围的肾脏组织而影响肾功能 4 全身症状systemicsymptom病程后期可造成肾功能损害 尤其是肾小管功能的损害 可引起全身症状 如贫血 高血压 水肿 水电解质紊乱及酸碱平衡失调 19 clinicaldiagnosis RepresentativeurographicappearanceofMSK Typicalblushesareevident containingsmallradio opaquespots 20 21 22 ultrasonicdiagnosis 23 CTdiagnosis 24 25 EnhancedCT 26 Treatment 髓质海绵肾的治疗包括一般性治疗和针对并发症的治疗 两者不可偏废 都很重要 一般性治疗不管病人有无症状 一经论断应嘱病人多饮水 采用低钙 低草酸饮食 对高钙尿病人应适当长期应用降低尿钙的药物 如噻嗪类利尿剂 并与枸橼酸钾联合应用 以防止或延缓结石形成 注意切勿做不必要的排石治疗 因结石位于肾乳头管内 不能将结石排出 27 Treatment 并发症的治疗并发症包括继发性结石梗阻和感染 当海绵肾结石从乳头管排出并在尿路内停留 长大而引起尿路梗阻时 促使病情恶化 因此 一旦继发性尿路结石形成 即应排石治疗 且不应做长期等待 对不能自行排出的结石应做体外冲击波碎石治疗 继发尿路感染后会加速肾脏的损害过程 因此对继发感染病人应给予有效抗菌素控制感染 对海绵肾结石病人不主张做手术治疗 除非是单侧病变病人 且已证明该侧肾脏因继发结石和感染已无肾功能者 方可考虑单侧肾切除 28 Treatmentwithpotassiumcitrateandrenalstonesinmedullaryspongekidney Forstoneprevention patientswithMSKgenerallyreceivethestandard stoneclinic recommendationsandoftenreceivep