医学精品课件:2014 中德班尿崩症.ppt
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1、Tongji HospitalHushuhong2014.9.Diabetes insipidus DI Physiology of urine concentration and dilutionAdenohypophsis(anterior pituitary)ACTH,Gonadotropin (FSH,LH),Prolactin,GH,TSH Neurohypophysis(posterior pituitary):antidiuretic hormone(ADH)oxytocin ADH also known as arginine vasopressin(AVP)in human
2、and other mammals The actions of ADHThe actions of ADH are mediated through V2 receptors by altering the water permeability of the cortical and medullary collecting tubules(CCD and MCD).Concentration of urine CCDMCDUrine Osmolality:Without ADHMin:Urine Osm=50 mOsmWith ADHMax :Urine Osm=1200 mOsm Reg
3、ulator of ADH1.Effective extracellular fliud(ECF)osmolality changes sensed by osmoreceptors in supraoptic and periventricular nuclei (in hypothalamus)2.Effective ECF volume sensed by baroreceptors in aortic arch,carotid sinus,left atrium.Other ADH stimulantsEmotional factors and stressSleepMedcines:
4、chlorpropamide clofibrate opiates nicotine phenobarbitoneDiabetes insipidus(DIDI)Diabetes insipidusDiabetes insipidus(DIDI)is a condition characterized by:Excretion of large amounts of severely diluted urine(polyuria)andExcessive fluid intake (polydipsia)Caused by:1.a deficiency of ADHcentral diabet
5、es insipidus(CDI)or 2.an insensitivity of the kidneys to ADHnephrogenic diabetes insipidus(NDI)*Diabetes:pass through*Insipidus:without tasteCauses of CDI1.SecondaryNeurosurgeryNeurotrauma Suprasellar tumors Infiltrative diseases (histiocytosis X,sarcoidosis)Hypoxic or ischemic Radiation Infection(m
6、eningitis or encephalitis)vascular lesions2.Idiopathic Autoimmune:destruction of the ADH hormone-secreting cells,characterized by lymphocytic inflammation of the pituitary stalk and posterior pituitary Normal pituitary gland:Sagittal T1-weighted image shows normal appearing pituitary gland with post
7、erior pituitary bright spot(arrow)T1-weighted MR images showing a small pituitary gland with absent bright spot of the neurohypophysis(arrow)and thickened pituitary stalk in 18-years-old man with sudden onset of polyuria and polydipsia 3.Genetic Usually an autosomal dominant disease caused by mutati
8、ons involving AVP gene.Causes of NDI1.Primary(familial)X-linked recessive genetic defect of the V2V2 receptor gene.A rare autosomal recessive or dominant pattern of mutation in the aqua-porin gene AQP2AQP2 2.Secondary chronic pyelonephritis/tubulointerstitial disease/CRF/obstructive uropathy/polycys
9、tic diseaseDrugs(lithium,colchcine,fluoride,demeclocycline)hypercalcemiahypokalemiaProtein deprivationAmylodosisSjoegren syndromeClinical featuresPolyuria(including Polyuria(including nocturianocturia)urine volum:4L/day urine SG:1.005 urine osmolality 50-200 mOsm/kg(300 mOsm/kg)PolydipsiaPolydipsia
10、predilection for drinking cold liquidsSymptoms of underlying causeSymptoms of underlying causeComplications:Complications:*Body weight reduction*Obstipation*Hypernatremic dehydration and its neurologic sequelea*Growth retardation(children)*HydronephrosisDiagnostic workupIn a normal well hydrated sub
11、ject with intact function of ADH,the random plasma osmolality is 290mOsm/L and urine osmolality is 300-450mOsm/LIn a patient with DI and free excess to water,plasma osmolality is 290mOsm/L and urine osmolality is 50-200mOsm/LSmultaneous measurement of plasma and urine osmolality establish the diagno
12、sis in most severe DI2012.9.27A male outpatient presented with polydipsia and polyuriasmultaneous measurement of urine osmolality=687mOsm/kg.H2O plasma osmolality=304mOsm/kg.H2OExclusion of DIwater deprivation test-When?Water deprivation test is needed(1)for patients with partial ADH deficiency(2)to
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