4. kuliah adrenal disorders

8/10/2019 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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4. kuliah adrenal disorders

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