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1、TongjiHospitalHushuhong2014 9 DiabetesinsipidusDI Physiologyofurineconcentrationanddilution Adenohypophsis anteriorpituitary ACTH Gonadotropin FSH LH Prolactin GH TSH Neurohypophysis posteriorpituitary antidiuretichormone ADH oxytocin ADHalsoknownasargininevasopressin AVP inhumanandothermammals Thea
2、ctionsofADH TheactionsofADHaremediatedthroughV2receptorsbyalteringthewaterpermeabilityofthecorticalandmedullarycollectingtubules CCDandMCD Concentrationofurine CCD MCD UrineOsmolality WithoutADHMin Urine Osm 50mOsmWithADHMax Urine Osm 1200mOsm RegulatorofADH 1 Effectiveextracellularfliud ECF osmolal
3、itychangessensedbyosmoreceptorsinsupraopticandperiventricularnuclei inhypothalamus 2 EffectiveECFvolumesensedbybaroreceptorsinaorticarch carotidsinus leftatrium OtherADHstimulants EmotionalfactorsandstressSleepMedcines chlorpropamideclofibrateopiatesnicotinephenobarbitone Diabetesinsipidus DI Diabet
4、esinsipidus DI isaconditioncharacterizedby Excretionoflargeamountsofseverelydilutedurine polyuria andExcessivefluidintake polydipsia Causedby 1 adeficiencyofADH centraldiabetesinsipidus CDI or2 aninsensitivityofthekidneystoADH nephrogenicdiabetesinsipidus NDI Diabetes passthrough Insipidus withoutta
5、ste CausesofCDI 1 SecondaryNeurosurgeryNeurotraumaSuprasellartumorsInfiltrativediseases histiocytosisX sarcoidosis HypoxicorischemicRadiationInfection meningitisorencephalitis vascularlesions 2 IdiopathicAutoimmune destructionoftheADHhormone secretingcells characterizedbylymphocyticinflammationofthe
6、pituitarystalkandposteriorpituitary Normalpituitarygland SagittalT1 weightedimageshowsnormalappearingpituitaryglandwithposteriorpituitarybrightspot arrow T1 weightedMRimagesshowingasmallpituitaryglandwithabsentbrightspotoftheneurohypophysis arrow andthickenedpituitarystalkin18 years oldmanwithsudden
7、onsetofpolyuriaandpolydipsia 3 GeneticUsuallyanautosomaldominantdiseasecausedbymutationsinvolvingAVPgene CausesofNDI 1 Primary familial X linkedrecessivegeneticdefectoftheV2receptorgene Arareautosomalrecessiveordominantpatternofmutationintheaqua poringeneAQP2 2 Secondarychronicpyelonephritis tubuloi
8、nterstitialdisease CRF obstructiveuropathy polycysticdiseaseDrugs lithium colchcine fluoride demeclocycline hypercalcemiahypokalemiaProteindeprivationAmylodosisSjoegrensyndrome Clinicalfeatures Polyuria includingnocturia urinevolum 4L dayurineSG 1 005urineosmolality50 200mOsm kg 300mOsm kg Polydipsi
9、apredilectionfordrinkingcoldliquidsSymptomsofunderlyingcause Complications Bodyweightreduction Obstipation Hypernatremicdehydrationanditsneurologicsequelea Growthretardation children Hydronephrosis Diagnosticworkup InanormalwellhydratedsubjectwithintactfunctionofADH therandomplasmaosmolalityis 290mO
10、sm Landurineosmolalityis300 450mOsm LInapatientwithDIandfreeexcesstowater plasmaosmolalityis 290mOsm Landurineosmolalityis50 200mOsm LSmultaneousmeasurementofplasmaandurineosmolalityestablishthediagnosisinmostsevereDI 2012 9 27Amaleoutpatientpresentedwithpolydipsiaandpolyuriasmultaneousmeasurementof
11、urineosmolality 687mOsm kg H2Oplasmaosmolality 304mOsm kg H2OExclusionofDI waterdeprivationtest When Waterdeprivationtestisneeded 1 forpatientswithpartialADHdeficiency 2 todifferentiateDIfromprimarypolydipsia waterdeprivationtest how BedoneinthemorningunderobservationWeighthepatientandmeasureplasmaa
12、ndurineosmolalityevery2hours 4 18hoursdeprivationisenough waterdeprivationtest whenendend Thetestshouldbeendedonceoneofthefollowingoccurs 1 Theurineosmolalityreachesanormalvalueofabove600mOsm kg 2 Theurineosmolalityisstableon2or3successivemeasurements difference 30mOsm L indicatingaPlateuaphase 3 Sy
13、ndromeandsignofdehydration Attheendoftest exogenousADHisadministered ADH5UorDDAVP2ugsc or20ugDDAVPnasally Urineosmolalityisthenmeasuredhourlyforthenext2hours q1hr waterdeprivationtest Interpretation NormalsubjectsorprimarypolydipsiaAfterwaterdeprivation theurineoutputisreducedUrineosmolalityrises600
14、 1200mOsm kgplasmaosmolalityhardlyrises 300mOsm kg urine plasmaosmolalityratiorises 2 0AfterexogenousADH Urineosmsincreaseminimally 5 10 DIpatientsAfterwaterdeprivation Theplasmabutnottheurineosmolalityrises U Posmolalityratioremains 1 5 1 0completeCDI1 0 1 5partialCDIAfterexogenousADHCDI urineosmsi
15、ncrease over50 100 incompleteCDI over10 50 inpartialCDI NDI urineosmsincreasebylessthan5 10 waterdeprivationtestresultsofnormnal CDIandNDIsubjects waterdeprivationtest Case1 female 49yr ID 977049polydipsia polyuriafor2m butnonuctouriaPhysicalexamination normalOGTTandthyroidfunctiontest normal U P 2
16、66diagnosis normalorprimarypolydipsia ADH5usq U P 2 66diagnosis normalorprimarypolydipsia waterdeprivationtest Case2 male aged36yPolydipsiafor6m Urinevolume7000 8000ml d polyuriadespiteofnodrinking nuctouria waterdeprivationtest result U P 0 42diagnosis completeCDI 551 121 121 355 waterdeprivationtest result ADH5usq U P 0 42diagnosis completeCDI 551 121 121 355 waterdeprivationtest Case3 male 27yPolydipsiaandpolyuriafor1y Urinevolume6000ml d noheadacheandvisiualdisordersPE normal Diagnose partia
