Adreno Cortex
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Ronald Chrisbianto Gani405090223
Faculty of Medicine 2009Tarumanagara University
ENDOCRINE SYSTEM BLOCK
CASE 3
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PHYSIOLOGY OF CORTEX ADRENAL
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ADRENAL GLAND
Sherwood’s Human Physiology 7th Ed
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Color Atlas of Physiology 5th Ed
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STEROIDOGENIC PATHWAY
Sherwood’s Human Physiology 7th Ed
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Color Atlas of Physiology 5th Ed
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CONTROLOF
CORTISOLSECRETION
Sherwood’s Human Physiology 7th Ed
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CIRCARDIAN RHYTHM
Color Atlas of Physiology 5th Ed
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CUSHING’S SYNDROME
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DEFINITION
• A constellation of clinical features that result from chronic exposure to excess glucocorticoids of any etiology
Harrison’s Principle of Medicine 18th Ed
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EPIDEMIOLOGY
• 1-2 / 100.000 per year• Mostly in women, exception in prepubertal
cases, men dominates• 90% caused by microadenoma (<1cm in size)• 5-10% caused by macroadenoma (>1cm in
size)
Harrison’s Principle of Medicine 18th Ed
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ETIOLOGYCauses of Cushing Syndrome F : M ratio %
ACTH-dependent Cushing’s 90
Cushing's disease (= ACTH-producing pituitary adenoma) 4 : 1 75
Ectopic ACTH syndrome (due to ACTH secretion by bronchial or pancreatic carcinoid tumors, small cell lung cancer, medullary thyroid carcinoma, pheochromocytoma and others)
1 : 1 15
ACTH-Independent Cushing's 4 : 1 10
Adrenocortical adenoma 5 – 10
Adrenocortical carcinoma 1
Rare causes: PPNAD, primary pigmented nodular adrenal disease; AIMAH, ACTH-independent massive adrenal hyperplasia; McCune-Albright syndrome
< 1
Abbreviations: ACTH, adrenocorticotropic hormone; AIMAH, ACTH-independent macronodular hyperplasia; PPNAD, primary pigmented nodular adrenal disease
Harrison’s Principle of Medicine 18th Ed
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ECTOPIC ACTH SECRETION
Cecil Medicine 23rd Ed
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SIGN & SYMPTOMSBody System Signs and Symptoms
Body fat Weight gain, central obesity, rounded face, fat pad on back of neck ("buffalo hump")
Skin Facial plethora, thin and brittle skin, easy bruising, broad and purple stretch marks, acne, hirsutism
Bone Osteopenia, osteoporosis (vertebral fractures), decreased linear growth in children
Muscle Weakness, proximal myopathy (prominent atrophy of gluteal and upper leg muscles)
Cardiovascular Hypertension, hypokalemia, edema, atherosclerosis
Metabolism Glucose intolerance/diabetes, dyslipidemia
Reproductive system Decreased libido, in women amenorrhea (due to cortisol-mediated inhibition of gonadotropin release)
Central nervous system Irritability, emotional lability, depression, sometimes cognitive defects, in severe cases, paranoid psychosis
Blood & Immune Increased susceptibility to infections, increased white blood cell count, eosinopenia, hypercoagulation with increased risk of deep vein thrombosis and pulmonary embolism
Harrison’s Principle of Medicine 18th Ed
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SIGNS & SYMPTOMS
Harrison’s Principle of Medicine 17th Ed
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Harrison’s Principle of Medicine 18th Ed
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MOON FACE
Sherwood’s Human Physiology 7th Ed
First time diagnosed 4 month later
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RADIOLOGIC EVALUATION
• Abdominal CT –Scan• For suspected ectopic ACTH production
high-resolution CT• Hypersecretion of pituitary ACTH MRI with
gadolinium contrast• May not be able to detect small
microadenoma• False-positive to cyst and nonsecretory lesion
Harrison’s Principle of Medicine 18th Ed
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ALGORITHMTO
SUSPECTEDCUSHING’SSYNDROME
Harrison’s Principle of Medicine 18th Ed
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Diagnostic Tests to Determine the Type of Cushing's Syndrome
Test Pituitary Macroadenoma
Pituitary Microadenoma
Ectopic ACTH or CRH Production
Adrenal Tumor
Plasma ACTH level
↑ to ↑↑ N to ↑ ↑ to ↑↑↑ ↓
Percent who respond to high-dose dexamethasone
<10 95 <10 <10
Percent who respond to CRH
>90 >90 <10 <10
Harrison’s Principle of Medicine 18th Ed
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DIFFERENTIAL DIAGNOSIS
• Pseudo-Cushing Syndrome– Caused by chronic alcoholism– Abdnormalities in steroid output, modestly
elevated urine cortisol, blunted circardian rythm of cortisol levels, resistance to supression using the overnight dexamethasone test
– Discontinuation of alcohol and/or improvement of emotional status, result of steroid testing return to normal
Harrison’s Principle of Medicine 18th Ed
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INCINDENTALOMA
• Incidental masses discovered during radiographic testing for another condition
• Asymptomatic Adrenal Mass• Found in ~6 % of adult subjects to autopsy• Uncommon in age <30 years
Harrison’s Principle of Medicine 17th Ed
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ADRENAL MASSES
Harrison’s Principle of Medicine 18th Ed
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ALGORITHMFOR
INCINDENTALOMA
Harrison’s Principle of Medicine 17th Ed
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Harrison’s Principle of Medicine 18th Ed
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TREATMENT
• Surgical Therapy – Resection via transsphenoidal approach
• Radiation Therapy– to pituitary gland for patient who cannot undergo
surgery, length of time to get full response is very long (up to 10 years), possibility of hypopituitarism
• Medical Therapy (combination with surgery)– Hypercortisolism correction ketoconazole
(1600mg) and metyrapone (2g)
Cecil Medicine 23rd Ed
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TREATMENT FOR ADRENAL ADENOMA
• laparoscopic, treated with glucocorticoid and mineralocorticoid pre and post operative
• Despite operative intervention, most patient die within 3 years, metastases most often occur in liver and lung, drugs for corticoadrenal carcinoma mitotane
• Metastase to bones refrater to drugs, need radiotherapy
Harrison’s Principle of Medicine 18th Ed
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TREATMENT FOR BILATERAL HYPERPLASIA
• Surgical exploration of the pituitary via a transsphenoidal approach finding microadenoma.
• Total adrenalectomy cure rate almost 100%, need for lifelong mineralocorticoid and glucocorticoid replacement and a 10–20% probability of a pituitary tumor developing over the next 10 years (Nelson's syndrome)
Harrison’s Principle of Medicine 18th Ed
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TREATMENT FOR CUSHING
Harrison’s Principle of Medicine 18th Ed
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MINERALOCORTICOID EXCESS / HYPERALDOSTERONISM
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HYPERALDOSTERONISM• Twice in women than men• Occurs in age of 30 – 50• ~1% of unselected hypertension patient• Classified to– Primary hyperaldosteronism
• With adrenal tumor (Conn’s syndrome)• Without adrenal tumor (bilateral cortical nodular hyperplasia,
idiopathic hyperaldosteronism, and/or nodular hyperplasia) unknown cause, no hypokalemia, less evidence of radiologic pahology, lower aldosterone level
– Secondary Hyperaldosteronism• Response to activation of RAA system or at Bartter syndrome
Harrison’s Principle of Medicine 18th Ed
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ETIOLOGY
Cecil Medicine 23rd Ed
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ETIOLOGY
Cecil Medicine 23rd Ed
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PRIMARY VS SECONDARY
Harrison’s Principle of Medicine 17th Ed
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SIGNS & SYMPTOMS• Hypokalemia• Very severe diastolic hypertension• Headaches• Potassium depletion weakness & fatigue• Impairment of urinary concentrating ability polyuria
polydipsia• Left ventricular hypertrophy secondary to hypertension,
but disproportionate to essential hypertension• Proteinuria • Renal failure• Hypertension complication structural damage of cerebral
circulation, retinal vasculature, kidneyHarrison’s Principle of Medicine 18th Ed
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DIAGNOSIS
• Ditegakkan dengan kadar aldosteron yang tinggi dan renin yang rendah
• Kalium plasma merupakan petunjuk diagnostik• Kriteria diagnosis hiperaldosteronisme primer– Hipertensi diastolik tanpa edema– Hiposekresi renin yg tidak merespon thd
penurunan volume plasma– Hipersekresi aldosteron yg tidak berkurang setelah
volume expansion
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ALGORITHMFOR
SUSPECTEDMINERALO-CORTICOID
EXCESS
Harrison’s Principle of Medicine 18th Ed
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ANTIHYPERTENSIVE DRUGS
Harrison’s Principle of Medicine 17th Ed
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TREATMENT
• Primary Hyperaldosteronism– Adenoma : • laparoscopic surgical excision• Sodium restriction and spironolactone• Medical intervention may be used fro chronic therapy
but usually limited to side effects in men
– Bilateral Hyperplasia• Surgery only when significant• Symptomatic hypokalemia cannot be controlled by
medical intervention
Harrison’s Principle of Medicine 18th Ed
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ADRENAL ANDROGEN EXCESS
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ADRENAL ANDROGEN EXCESS
• Excess DHEA & androstenedione converted to testosterone in extraglandular tissue virilization
Harrison’s Principle of Medicine 18th Ed
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ADRENAL INSUFFICIENCY
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CLASSIFICATIONOF
ADRENALINSUFFICIENCY
Harrison’s Principle of Medicine 18th Ed
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PRIMARY ADRENAL INSUFFICIENCY (ADDISON’S DISEASE)
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SECONDARY ADRENAL INSUFFICIENCY
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SIGNS&
SYMPTOMS
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SIGN & SYMPTOMS
Harrison’s Principle of Medicine 17th Ed
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SIGNS&
SYMPTOMS(Hyperpigmentation)
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ALGORITHMFOR
ADRENALINSUFFICIENCY
Harrison’s Principle of Medicine 18th Ed
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TREATMENT
• Glucocorticoid replacement– Hydrocortisone 15–25 mg
• Mineralocorticoid replacement– Fludrocortisone 100–150 μg
• Adrenal androgen replacement– DHEA 25–50 mg
Harrison’s Principle of Medicine 18th Ed
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TREATMENT
Harrison’s Principle of Medicine 18th Ed
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REFERENCES• Fauci AS, Kasper DL, Longo DL, Jameson JL, et al, editors.
Harrison’s Principle of Internal Medicine. 18th ed. USA : McGraw Hill Medical, 2011
• Fauci AS, Braunwald E, Kasper DL, Longo DL, Jameson JL, et al, editors. Harrison’s Principle of Internal Medicine. 17th ed. USA : McGraw Hill Medical, 2008
• Sudoyo AW, Setiyohadi B, Alwi I, Simadibrata M, Setiadi S, et al, editors. Buku Ajar Ilmu Penyakit Dalam 5th ed. Jakarta : Pusat Penerbitan Ilmu Penyakit Dalam FKUI, 2006
• Arrend WP, Armitage JO, Clemmons JR, Drazzen JM, Griggs J, et al. Goldman’s Cecil Medicine. Philadelphia : Elsevier Saunders 2008
• Sherwood L. Human Physiology from Cells to System. 7th Ed. USA : Brooks / Cale Cengage Learning 2010