皮质醇增多症的诊治
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D.X 2mg q6h P.O 2 days Urinary free cortisol reduced 50%: Cushing’s
来自百度文库
disease (Pituitary adenoma)
Urinary free cortisol NOT reduced
50%:Adrenal tumor, carcinoma, ectopic ACTH Syndrome
ACTH Stimulation test
ACTH 25u intravenously 8h
2-5 fold increase in urinary free cortisol in Cushing’ s disease Plasma cortisol and urinary free cortisol increase in half of adrenal adenoma patients No response in adrenal carcinoma
Normal Appearance
With Cushings
Easy bruising Thinning skin Poor wound healing Acne Purple striae Hirsutism Female balding Menstrual irregularity
Sleep disorders Excessive hunger Excessive thirst Frequent urination Sweating
more above basal level : Cushing’s disease (Pituitary adenoma)
No response in adrenal carcinoma , tumor, ectopic
ACTH Syndrome
Imaging diagnosis
Pituitary CT has a sensitivity of about 50% for identifying microadenomas MRI has increased sensitivity but is not 100% predictive If diagnostic doubt need bilateral inferior petrosal sinus sampling for ACTH Adrenal ultrasonography---first choice Abdominal CT will allow identification of adrenal pathology Somatostatin scintigraphy to identify sites of ectopic hormone production
Metyrapone Test
Etiology diagnose (especially for pituitary or adrenal)
Metyrapone 2-3g (30mg/kg) P.O at midnight Urinary 17-OHCS, Plasma ACTH,11-deoxycortisol
ACTH-independent causes
Adrenal adenoma Adrenal carcinoma
Micronodular adrenal disease
McCune-Albright syndrome Massive macronodular adrenal disease
Pseudo-Cushing Syndrome
Anatomy and Histology
Adrenal Gland
Cortex
Medulla
Zona glomerulosa aldosterone
Zona fasciculata cortisol
Zona reticularis Adrenal androgen catecholamines
Normal pattern of ACTH and cortisol secretion
17-hydroxycortisteriod(17-羟皮质类固醇) 17-ketosteriods(17-酮皮质类固醇)
Plasma ACTH
Screening Tests for Cushing’s
Courtesy of www.CSRF.com
Suppression tests
Screening test
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
Anxiety Confusion Concentration loss Memory loss Depression Suicidal thoughts Panic attacks
Illustration from Mayo Clinic Family Health Book, 2d. ed, 1996
Factitious or surreptitious glucocorticoid administration
TABLE 204-3. COMMON CAUSES OF ECTOPIC ACTH SECRETION
Small cell carcinoma of the lung Endocrine tumors of foregut origin
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 )
And may include any number of these: 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
DX 0.5 mg q6h P.O 2 days
Urinary free cortisol decreased: Normal
Urinary free cortisol NOT decreased: Cushing’
s Syndrome
Suppression tests
Large dose DX suppression test
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
TABLE 204-1. CLINICAL FEATURES OF GLUCOCORTICOID EXCESS
Frequency(%)
Weight gain “Moon facies” Hypertension Violaceous striae Hirsutism Glucose intolerance Proximal muscle weakness Plethora Menstrual dysfunction Acne Easy bruising Osteopenia Dependent edema Hyperpigmentation Hypokalemic metabolic alkalosis
1mg DX P.O at midnight
Plasma cortisol (PF) at 7-8 am next day
PF suppressed: Normal PF NOT suppressed: Cushing’ s Syndrome
Suppression tests
Low dose DX suppression test
Hypercortisolism
(Cushing’ s Syndrome)
Definition
A constellation of clinical abnormalities
due to chronic exposure to excess of
cortisol or related corticosteroid.
90 75 75 65 65 65 60 60 60 40 40 40 40 20 15
Diagnosis
Clinical manifestations Lab findings
Plasma cortisol and rhythm (RIA) Urinary free cortisol
Courtesy of www.CSRF.com
With Cushings Normal Appearance With Cushings Normal Appearance
With Cushings
Normal Appearance
FIGURE . Multiple wide striae on the abdomen of a patient with Cushing's disease.
CRH stimulation test
Etiology diagnose (especially for pituitary ACTHdependent or ectopic ACTH syndrome) A newer approach is to combine a CRH stimulation test with a dexamethasone suppression test(4mg ). method : 1 µg / kg of CRH is administered intravenously. ACTH and cortisol levels are measured before CRH injection and 15, 30, 45, 60, 90 and 120 minutes after injection. A rise in the cortisol value of 20 percent or more above basal level or a rise in the ACTH value of at least 50 percent above basal level is considered evidence for an ACTH-dependent lesion
Thymic carcinoid Islet cell tumor Medullary thyroid carcinoma Bronchial carcinoid
50% 35%
Pheochromocytoma Ovarian tumors
5% 2%
Clinical manifestations
Symptoms Vary
来自百度文库
disease (Pituitary adenoma)
Urinary free cortisol NOT reduced
50%:Adrenal tumor, carcinoma, ectopic ACTH Syndrome
ACTH Stimulation test
ACTH 25u intravenously 8h
2-5 fold increase in urinary free cortisol in Cushing’ s disease Plasma cortisol and urinary free cortisol increase in half of adrenal adenoma patients No response in adrenal carcinoma
Normal Appearance
With Cushings
Easy bruising Thinning skin Poor wound healing Acne Purple striae Hirsutism Female balding Menstrual irregularity
Sleep disorders Excessive hunger Excessive thirst Frequent urination Sweating
more above basal level : Cushing’s disease (Pituitary adenoma)
No response in adrenal carcinoma , tumor, ectopic
ACTH Syndrome
Imaging diagnosis
Pituitary CT has a sensitivity of about 50% for identifying microadenomas MRI has increased sensitivity but is not 100% predictive If diagnostic doubt need bilateral inferior petrosal sinus sampling for ACTH Adrenal ultrasonography---first choice Abdominal CT will allow identification of adrenal pathology Somatostatin scintigraphy to identify sites of ectopic hormone production
Metyrapone Test
Etiology diagnose (especially for pituitary or adrenal)
Metyrapone 2-3g (30mg/kg) P.O at midnight Urinary 17-OHCS, Plasma ACTH,11-deoxycortisol
ACTH-independent causes
Adrenal adenoma Adrenal carcinoma
Micronodular adrenal disease
McCune-Albright syndrome Massive macronodular adrenal disease
Pseudo-Cushing Syndrome
Anatomy and Histology
Adrenal Gland
Cortex
Medulla
Zona glomerulosa aldosterone
Zona fasciculata cortisol
Zona reticularis Adrenal androgen catecholamines
Normal pattern of ACTH and cortisol secretion
17-hydroxycortisteriod(17-羟皮质类固醇) 17-ketosteriods(17-酮皮质类固醇)
Plasma ACTH
Screening Tests for Cushing’s
Courtesy of www.CSRF.com
Suppression tests
Screening test
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
Anxiety Confusion Concentration loss Memory loss Depression Suicidal thoughts Panic attacks
Illustration from Mayo Clinic Family Health Book, 2d. ed, 1996
Factitious or surreptitious glucocorticoid administration
TABLE 204-3. COMMON CAUSES OF ECTOPIC ACTH SECRETION
Small cell carcinoma of the lung Endocrine tumors of foregut origin
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 )
And may include any number of these: 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
DX 0.5 mg q6h P.O 2 days
Urinary free cortisol decreased: Normal
Urinary free cortisol NOT decreased: Cushing’
s Syndrome
Suppression tests
Large dose DX suppression test
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
TABLE 204-1. CLINICAL FEATURES OF GLUCOCORTICOID EXCESS
Frequency(%)
Weight gain “Moon facies” Hypertension Violaceous striae Hirsutism Glucose intolerance Proximal muscle weakness Plethora Menstrual dysfunction Acne Easy bruising Osteopenia Dependent edema Hyperpigmentation Hypokalemic metabolic alkalosis
1mg DX P.O at midnight
Plasma cortisol (PF) at 7-8 am next day
PF suppressed: Normal PF NOT suppressed: Cushing’ s Syndrome
Suppression tests
Low dose DX suppression test
Hypercortisolism
(Cushing’ s Syndrome)
Definition
A constellation of clinical abnormalities
due to chronic exposure to excess of
cortisol or related corticosteroid.
90 75 75 65 65 65 60 60 60 40 40 40 40 20 15
Diagnosis
Clinical manifestations Lab findings
Plasma cortisol and rhythm (RIA) Urinary free cortisol
Courtesy of www.CSRF.com
With Cushings Normal Appearance With Cushings Normal Appearance
With Cushings
Normal Appearance
FIGURE . Multiple wide striae on the abdomen of a patient with Cushing's disease.
CRH stimulation test
Etiology diagnose (especially for pituitary ACTHdependent or ectopic ACTH syndrome) A newer approach is to combine a CRH stimulation test with a dexamethasone suppression test(4mg ). method : 1 µg / kg of CRH is administered intravenously. ACTH and cortisol levels are measured before CRH injection and 15, 30, 45, 60, 90 and 120 minutes after injection. A rise in the cortisol value of 20 percent or more above basal level or a rise in the ACTH value of at least 50 percent above basal level is considered evidence for an ACTH-dependent lesion
Thymic carcinoid Islet cell tumor Medullary thyroid carcinoma Bronchial carcinoid
50% 35%
Pheochromocytoma Ovarian tumors
5% 2%
Clinical manifestations
Symptoms Vary