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1、HYPERCORTISOLISMCUSHING SYNDROMEContents1. What is Cushing syndrome?2. What is the causes?3. How to diagnose?4. Clinical Features5. Hormone levels6. Functional test7. Localization8. How to treatment? Contents1. What is Cushing syndrome?2. What is the causes?3. How to diagnose?4. Clinical Features5.
2、Hormone levels6. Functional test7. Localization8. How to treatment? Definition Cushing Syndrome A diverse symptom complex resulting from excess steroid hormone (cortisol) production Cushing Disease A hypercortisolism resulting from pituitary ACTH hypersecretionContents1. What is Cushing syndrome?2.
3、What is the causes?3. How to diagnose?4. Clinical Features5. Hormone levels6. Functional test7. Localization8. How to treatment? Capsula GlomerulosaFasciculataReticularisMedullaAldosteroneAndrogenCatecholamineAdrenal StructureCortisolCortisol Secretion Regulation ACTH CRHCortisolHypothalamus Pituita
4、ryAdrenalCauses of Cushing Syndrome ACTH CRHCortisolHypothalamus PituitaryAdrenalEctopic CRH syndromeHypothalamus Cushing syn.Cushing disease (CD)Ectopic ACTH syndromeAdrenal adenomaAdrenal carcinomaMicronodular adrenal diseaseMassive macronodular adrenaldiseaseCauses of Cushing syndrome1. Endogenou
5、s Cushing syndrome (1) ACTH-dependentCushing disease (CD)Ectopic ACTH syndromeEctopic CRH syndrome(2) ACTH-independentAdrenal adenomaAdrenal carcinomaMicronodular adrenal diseaseMassive macronodular adrenal disease(3) Pseudocushing syndrome2. Exogenous Cushing syndrome Contents1. What is Cushing syn
6、drome?2. What is the causes?3. How to diagnose?4. Clinical Features5. Hormone levels6. Functional test7. Localization8. How to treatment? Function Clinical FeaturesFunctions Clinical FeaturesFat Central obesity, “moon face” “buffalo hump”Protein Thin extremities, muscle weakness, Purple striae, brui
7、sing, osteoporosis Glucose IGT, DMElectrolyte Hypertension, hypokalemia, alkalosisFunctions Clinical FeaturesImmunity Infection, impaired wound healing Sex gland Hirsutism, acne, menstrual dysfunction, Erectile dysfunctionBone marrow Increased RBC and WBC，plethoricMental Increased labilitySkin Hyper
8、pigmentation Function Clinical FeaturesHormone Level1. Cortisol levelsPlasma cortisol rhythm (8Am, 4Pm, midnight) 24h Urinary free cortisol, 17-OHCS, 17-KSPlasma ACTH rhythm 2. Functional TestsLow dose dexamethasone suppression test High dose dexamethasone suppression test Metyrapone test Localizati
9、onAdrenal CTLocalizationPituitary MRIDiagnosis1. Confirmation of hypercortisolism (Y/N)Plasma cortisol rhythm (8Am, 4Pm, midnight) 24h Urinary free cortisol, 17-OHCS, 17-KSLow dose dexamethasone suppression test 2. Differentiation of Cushing syndrome (What)Plasma ACTH level High dose dexamethasone s
10、uppression test Metyrapone test CRH stimulation test3. Localization (Where)Adrenal CT, Pituitary MRIDiagnosis Schedules (1)Cushing Syndrome suspectedCortisol rhythm, UFC, 17-OHCS, 17-KSElevatedNormalObesityLow dose dex. testYesNoCushing SyndromeDiagnosis Schedules (2)Cushing SyndromeACTH rhythmEleva
11、tedsuppressedExogenous CushingACTH-dependentYesHigh dose dex. testEctopic ACTH/CRH NoCushing Disease ACTH-independentGlucocorticoid takenYesNoAdrenal Tumor/NoduleContents1. What is Cushing syndrome?2. What is the causes?3. How to diagnose?4. Clinical Features5. Hormone levels6. Functional test7. Loc
12、alization8. How to treatment? Treatment Cause TreatmentCushing D TranssphenoidalEctopic ACTH Surgery+drugEctopic CRH Surgery +drug Adrenal Aden. SurgeryAdrenal Carc. Surgery +drug Micronodular Surgery +replace Macronodular Surgery +replace Nelson SyndromeCause incidence Cor. ACTH Low-Dex. High-Dex.
13、TreatmentCushing D 80 No Yes TranssphenoidalEctopic ACTH 20 No No Surgery+drugEctopic CRH Rare No No Surgery +drug Adrenal Aden. 40-50 No No SurgeryAdrenal Carc. 40-50 No No Surgery +drug Micronodular Less No No Surgery +replace Macronodular Rare No No Surgery +replace Exogenous More No No Stop takingObesity
