皮质醇增多症的诊治课件.ppt

Hypercortisolism(Cushing’ s Syndrome) Definition A constellation of clinical abnormalities due to chronic exposure to excess of cortisol or related corticosteroid. It is rare disorder It occurs as a result of primary tumors of adrenal gland that hypersecrete cortisol excess ACTH secretion that may be of pituitary or nonpituitary sources Anatomy and Histology Cortex Medulla Adrenal Gland aldosterone cortisol Adrenal androgen catecholamines Zona glomerulosa Zona fasciculata Zona reticularis Normal pattern of ACTH and cortisol secretion Pulsatile secretion Circadian rhythm When stimulated by ACTH, the adrenal gland secretes cortisol and other steroid hormones. ACTH is produced by the pituitary gland and released into the petrosal venous sinuses in response to stimulation by corticotropin-releasing hormone (CRH) from the hypothalamus Etiology and Pathophysiology TABLE 204-2. CAUSES OF CUSHING’ S SYNDROME ACTH-dependent causes ACTH-secreting pituitary tumor ( Cushing’ s disease ) Pituitary ACTH-secreting neoplasm ( ectopic CRH syndrome ) Nonpituitary ACTH-secreting neoplasm ( ectopic ACTH syndrome ) ACTH-independent causes Adrenal adenoma Adrenal carcinoma Micronodular adrenal disease McCune-Albright syndrome Massive macronodular adrenal disease Pseudo-Cushing Syndrome Factitious or surreptitious glucocorticoid administration TABLE 204-3. COMMON CAUSES OF ECTOPIC ACTH SECRETION Small cell carcinoma of the lung 50% Endocrine tumors of foregut origin 35% Thymic carcinoid Islet cell tumor Medullary thyroid carcinoma Bronchial carcinoid Pheochromocytoma 5% Ovarian tumors 2% Abdominal weight gain Red, round ‘moon’ face Thinning extremities ‘Buffalo hump’ High blood pressure High blood sugar Muscle weakness Osteoporosis/Fractures Infections Blood clots Visual field defects Easy bruising Thinning skin Poor wound healing Acne Purple striae Hirsutism Female balding Menstrual irregularity Sleep disorders Excessive hu