内科学教学课件：Addisons disease

1、Addisons diseaseThe First Affiliated Hospital of Chongqing Medical UniversityUnited StatespresidentJohn F. Kennedy(1917-1963), probably the single most famous case of Addisons disease1. Physiology of the Adrenal GlandMineralocorticoids (Aldosterone)Glucocorticoids (Cortisol)AndrogensRegulation of Gl

2、ucocorticoid The Hypothalamic-Pituitary-Adrenal AxisPituitaryAdrenal glandACTHCortisolNegative feedbackHypothalamusCRHRegulation of Mineralocorticoid The Renin-Angiotensin-Aldosterone SystemAdrenalAldosteronReninNaH2O , KBlood volumeAngiotensinKidney2. DefinitionChronic primary adrenal cortex insuff

3、iciency.Loss of all three types of adrenal hormones. AldosteronCortisolAndrogen3.CausesTuberculosisAuto-immune disease4. ManifestationMuscle weakness，fatigue，anorexia, diarrheaWeight loss, low blood pressure (Hypotension) even shock(i) Aldosterone deficiencyAdrenalAldosteronReninNaH2O , KBlood volum

4、eAngiotensin4. ManifestationHyperpigmentation: (ii) Cortisol deficiencyHyperpigmentationHyperpigmentationMechanism of hyperpigmentationPituitaryAdrenal glandACTHCortisolNegative feedbackHypothalamusCRHMSH(Melanocyte-stimulating hormone)Melanocyte Melaninpigment（黑色素）黑色素细胞Cortisol ACTH The similar act

5、ion of MSHMelanin pigment deposition Hyperpigmentation4.Manifestation(ii) Cortisol deficiencyHypoglycemia 4.Manifestation(iii) Androgen deficiencyLoss of axillary and pubic hair Loss of libido5. Adrenal Crisis Underlying adrenal insufficiency Acute stressAdrenalCrisisInfectionSurgeryTraumaClinical f

6、eatures Fever Nausea, vomiting and diarrheaAbdominal painDehydrationHypotension, even shock 5. Adrenal Crisis(i) Adrenal function testsPlasma/urinary cortisol: Plasma ACTH: 6. Tests (ii) ACTH stimulating test:ACTH 250ug iv 6. Tests Measure 0min, 30min, 60min, plasma cortisolNormal cortisol500nmol/LA

7、ddisons disease: low 6. Tests (iii) Abnormal electrolyte and metabolism Hyponatremia (Na+ )Hyperkalemia (K+ )HypoglycemiaAnemia (iv) Imaging examinationCT or MRI of adrenal gland. 6. Tests Autoimmune adrenal failureNormal adrenal 7. DiagnosisTypical manifestations: Hyperpigmentation hypotension, hyp

8、oglycemiaEvidence of tests: Cortisol ACTH ACTH stimulating test (+) Na+ , Hypoglycemia8. Differential diagnosisPrimary vs. Secondary adrenal insufficiencyPituitaryAdrenal glandACTHCortisolHypothalamusCRHSencondary（Sheehans syndrome)Primary(Addisons disease) Cortisol , ACTH No hyperpigmentation Hypop

9、igmentation8. Differential diagnosisSecondary adrenal insufficiency e.g. Sheehans syndrome9.TreatmentEducation: all life replacement.Cortisol replacement:Hydrocortisone 20mg (8AM) 10mg (4PM) Prednisone 5mg (8AM) 2.5mg (4PM) Dexamethasone 0.75mg (8AM) 9.TreatmentAldosterone replacement Intake enough salt: 8-10mg/day. 9-Fludrocortisone. 9.Treatment 9.TreatmentAdrenal CrisisA life-threatening emergency! Reversal of Hypoglycemia，hyponatremia and hypotensio