原发性慢性肾上腺皮质功能减退课件

Addisons Disease Primary chronic adrenocortical insufficiency 1 “The ultimate measure of a “The ultimate measure of a man is not where he stands in man is not where he stands in moments of comfort and moments of comfort and convenience, but where he convenience, but where he stands at times of challenge stands at times of challenge and controversy.“and controversy.“ Martin Luther King Jr. 2 Objectives lEtiology lPathophysiology and clinical findings lPrinciple of diagnosis and treatment lDiagnosis and treatment of Addisonian Crisis 3 Outline lDefination of Addisons disease lEtiology lClinical findings lLaboratory findings lDiagnosis lTreatment 4 5 Adrenal Glands lInner Medulla Epinephrine /Norepinephrine lOuter cortex Corticosteroids 6 Corticosteroids lMineralcorticoids Aldosterone lGlucocorticoids Cortisol lAndrogens 7 Functions of Aldosterone lIncreases sodium absorption lIncreases potassium excretion 8 Glucocorticoid Function lImportant for stress response lAnti-inflammatory lStimulates gluconeogenesis 9 The hypothalamic-pituitary-adrenal axis 10 Adrenocortical insufficiency lis defined by deficient production of glucocorticoids or mineralocorticoids or both. 11 Adrenocortical insufficiency primary-CAH Adrenocortical Insufficiency secondary-CAH 12 Primary-CAH lPrimary adrenocortical insufficiency (Addisons disease) is due to destruction of the adrenal cortex . 13 Secondary adrenocortical insufficiency lSecondary adrenocortical insufficiency impaired cortisol production is due to deficient ACTH production. 14 Etiology l Autoimmune destruction，most common cause（65） l Tuberculosis，second common（20 ） l Other rare causes （15）： metastatic carcinoma、chronic infection 、AIDS 15 Pathology of adrenal glands 16 Clinical findings 1.Insufficient level of aldosteron 2. Insufficient level of cortisol 3. Addisonian crisis 17 aldosteron retain sodium excrete potassium sodium in urine potassium in urineH+ in urine serum sodium serum potassiumacidosis blood volume BP vasopressin renin、angiotencin 18 cortisol Anorexia salt craving weight loss Response of CNS depression Cardiovascular Bp Low of heart Small heart Crisis when stress gluconeogenesis Glycogen depletion Hypoglycemia when fasting ACTH MSH hyperpigmentation Kidney excrete water Low serum sodium Primary disease Sex function Irregular menstrual 19 Clinical features of Primary-CAH % Weakness and fatigue100 Weight loss100 Anorexia100 Hyperpigmentation92 Hypotension88 Gastrointestinal symptoms56 Salt craving19 20 Addisons disease 21 Addisons disease 22 Addisons disease 23 Laboratory findings Biochemical laboratory test： lhyponatremia lhyperkalemia lhypoglycemia lanemia 24 Laboratory findings Hormone test： lLow plasma and urine cortisol，low urine 17-OHCS，elevation of the plasma ACTH lACTH Stimulation Test，the most specific test for diagnosis。 25 Laboratory findings lImaging： the CT scan ( adrenals enlarged、Calcification) 26 Diagnosis of Addisons Disease lLow serum cortisol levels lHigh serum ACTH lDecreased serum Aldosterone l17-Hydroxysteroids lLow blood glucose lLow serum sodium lHigh serum potassium lIncreased WBC lClinical Picture/history 27 Differential Diagnosis of Addisons Disease lSecondary adrenocortical insufficiency lOther diseases accomonied by hyperpigmentation debility symptosis 28 Management of Addisons Disease lEducation lSteroid replacement lPrevention of Addisonian Crisis lPromote rest lMedical alert bracelet 29 Steroid replacement glucocorticoids： hydrocortison 8Am20mg 4Pm10mg prednison 8Am5mg 4Pm2.5mg mineralocorticoids： often not use Hormone replacement 30 Addisonian Crisis lAcute Adrenal Insufficiency lPrecipitated by stress states lUntreated fatal lCause must be treated lClinical Manifestations Sever hypotension Shock Circulatory collapse Death 31 Treatment of Addisonian Crisis lAggressive treatment of shock Rapid rehydration IV steroids Vasop