4. Kuliah Adrenal Disorders
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Transcript of 4. Kuliah Adrenal Disorders
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ADRENAL DISORDERS
Divisi Endokrin dan Metabolik
Bagian Penyakit Dalam FK SRSP !" Adam Malik
Medan
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Cross section through the adrenalgland cortex and medulla
salt
sugar
sex
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CRHCRH
Anterior lobeof pituitary gland
Anterior lobeof pituitary gland
ACTHACTH
ACTHACTH
CortisolCortisol
CortisolCortisol
Circadian regulation
Circadian regulation Stress:Physical stressEmotional stress HypoglycemiaCold exposure Pain
Stress:Physical stressEmotional stress HypoglycemiaCold exposure Pain
Adrenal cortex
Adrenal cortex+
+
+
Hypothalamus-Pituitary-Adrenal axis
Kirk LF. Am Fam Physician 2000CRH=corticothropin releasing hormone; ACTH=arenocorticothropin hormone.
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Regulation of aldosterone secretion
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Componentof renin-
angiotensinaldosteronsystem
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Action of aldosterone on the renal tubule.
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COMT = Catecholamine Ortho Methyl Transferase)
Production of
catecholamines
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Adrenocortical disorders
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Cushings Syndrome
Supraphysiologic glucocoticoid exposure
excess cortisol!
"Protein catabolic state
"#iberation of amino acids by muscle
"AA are transformed into glucose and glycogen and
then transformed into fat
$he source of excess glucocorticoids may be
exogenous or endogenous
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Causes of Cushings Syndrome
AC$H %ependent &'(!"Cushings %isease &)(!
Primary excretion of AC$H from pituitary" *icroadenoma+ macroadenoma or corticotrophic hyperplasia
" ,asophilic or chromophobe
* /01!
"2ctopic source 1)(! Produce AC$H or CRH
Small cell lung CA most common!+ carcinoid tumors+medullary thyroid+ pancreas+ o3arian+
pheochromocytoma+ small-cell CA of prostate
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Causes of Cushings Syndrome
AC$H 4ndependent
"2xogenous steroid use common!
P5 or topical
*ost common cause o3erall!
"Adrenal adenomas 1'(!
"Adrenal carcinoma )(! *ost common cause in children
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Cause of Cushings Syndrome
Pseudo-Cushings disease
"*imic clinical signs and symptoms
"6on-endocrine causes
Alcoholism
*a7or depression
*orbid obesity Acute illness
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Cushings Syndrome
Symptoms and Sign Percent of Patients
8eight gain+ round facies and
truncal obesity
8ea9ness
Hypertension
Hirsutism in :omen!
Amenorrhea
Cutaneous striae 2cchymoses
5steoporosis
Hyperglycemia
; hours for =? hours
" 8hen the patient is stable+ reduce the dosage to )' mg e3ery > hours" $aper to maintenance theraphy by day ? or ) and add
mineralocorticoid theraphy as reKuired
" *aintain or increase the dose to =''-?'' mgDd if complicationspersist or occur
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Addisons Crisis
*aintenance therapy
Jlucocorticoid and mineralocorticoid
" 5ral dose hydrocortisone 0 1'-=' mg in the morning
and )-1' mg later in day.
" ludrocortisone 0 '+')-'+= mgDd orally in the morning.
Response to theraphy
" Jeneral clinical sign+ good appetite and sense of :ellbeing.
" Signs of Cushings syndrome indicate o3ertreatment
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%isorders of adrenal medullary
function
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Pheochromocytoma
Pheochromocytoma is a rare catecholamine-secretingtumor deri3ed from chromaffin cells.
$umors that arise outside the adrenal gland are termed
extra-adrenal pheochromocytomas or paragangliomas. ,ecause of excessi3e catecholamine secretion+
pheochromocytomas may precipitate life-threateninghypertension or cardiac arrhythmias
4t is associated :ith spectacular cardi3ascular disturbances
and+ :hen corectly diagnosed and treatedcurable.8hen undiagnosedfatal
Pre3alence estimates " '.'1( to '.1( of the hypertensi3epopulation
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Pathophysiology
$he clinical manifestations of a pheochromocytoma resultfrom excessi3e catecholamine secretion by the tumor.
Catecholamines typically secreted+ either intermittently orcontinuously+ include norepinephrine and epinephrine andrarely dopamine.
$he biological effects of catecholamines are :ell 9no:n.
*ost pheochromocytomas contain norepinephrinepredominantly+ in comparison :ith the normal adrenalmedulla+ :hich is composed of roughly &)( epinephrine.
amilial pheochromocytomas are an exception because theysecrete large amounts of epinephrine. $hus+ the clinicalmanifestations of a familial pheochromocytoma differ fromthose of a sporadic pheochromocytoma.
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Receptor catecholamine 0Receptor 62!
Receptor 2P4!
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Pheochromocytoma
Symptoms 0" %ue to the pharmacologic effects excess circulatingcatecholamines
" A typical paroxysm the ) Ps! Pressure " sudden ma7or increase in blood pressure
Pain " abrupt onset of throbbing headache @ chest andabdominal pain
Perspiration " profuse generalied diaphoresis
Palpitation
Pallor
Clinical sign 0 Hypertension+orthostatic hypotension+ grade 44 to 444
retinopathy+ tremor+ :eight loss+ fe3er+ painless hematuria+hyperglycemia+ erythrocytosis
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Pheochromocytoma
%iagnosis 0" %emonstration of excessi3e amounts catecholamines in plasma
or urine or degradation product in urine Brinary metanephrine+ normetanephrine+ 3anilmandelic acid *A!+
and free catecholamine in =?-hour periode
%irect measurement plasma 62 and 2P4. #e3els =''' pgDml areabnormal and suggesti3e Pheochromocytoma
" Clonidine suppression test Clonidine orally '+/ mg@ plasma catecholamine 0 before oral clonidine
and again at 1+= and / hr after oral clonidine Plasma catecholamine )''pgDml
" Jlucagon stimulation test
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Pheochromocytoma
$reatment 0"Surgical resection is only definiti3e therapy
"Preoperati3e preparation :ith alpha bloc9ade reduce the
incidence intraoperati3e hypertensi3e crisis andpostoperati3e hypotension
"$he most commonly used agents arephenoxybenamine 1'-=' mg =-/ timesDd+ or praosin
1mg / timesDday+ ad3anced to ) mg / timesDday
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