李蓉《内科学精品教学》01.2 cushing syndrome 2017.pptVIP

Cushing’s diasease is more * Many of the signs and symptoms of Cushing’s syndrome follow logically from the known action of glucocorticoids. Glucocorticoids affects almost all cells of our body and thus signs of cortisol excess impact multiple phisiologic systems. gluconeogenesis, fat synthesis and lipolysis, ?bone marrow hematopoiesis * bone marrow?produce?red blood cells. Are secondary to weakning and rupture of callagen fibers in the dermis. * Striae on Abdomen and thigh * Central regulatory system * * Melanotropic activity of the elevated plasma level of ACTH, which binds to the human MSH rceptor. * * * Dark Sun-exposed area, pressured area, skin creases，like hand creases, oral mucosa，like tongue and gum. Darken typical 腋窝axilla gesture * * Adam’s apple, voice is low and gruff metastasize * * ? ? 0800h 1400h 2000h 0200h Day 1 0700h keep 24h urine, measure plasma cortisol Day 2 Dexa 3 tablets 3 tablets 3 tablets 2 tablets Day 3 Dexa 3 tablets 3 tablets 3 tablets 2 tablets 0700h keep 24h urine Day 4 0800h measure plasma cortisol and 24h urine cortisol Method (8mg/day) Result Be suppressed: Day 4 plasma cortisol 50% of the basal level High-dose dexamethasone suppression test (HDDST) Discrimination of the causes of Cushing’s syndrome by HDDST HDDST ACTH Cushing’s disease suppressed ↑ Adrenal adenoma/carcinoma No ↓ Ectopic ACTH syndrome No ↑↑↑ Pituitary MRI：microadenoma Pituitary MRI：macroadenoma 6. Imaging examination CT：normal adrenal CT：adrenal adenoma 7. Inferior petrosal sinus sampling (BIPSS) Discrimination of Cushing’s disease and Ectopic ACTH syndrome Invasive Test Cushing’s syndrome (CS) suspected 24hUFC, Plasma cortisol circadian rhythm, 1mg DST Normal Abnormal Low-dose DST CS excluded CS confirmed Abnormal Diagnosis 1. Step 2. Etiologic diagnosis Plasma ACTH High-dose DST 3. Location diagnosis Pituitary enhanced MRI Adrenal enhanced CT Chest enhanced CT/Whole body PET-CT Cushing’s disease ACTH Suppressed by High-dose DST Pituitary