ADRENAL GLANDS

Adrenal glands have a) Cortexcortex has two functional parts: 1) Zona fascicular/reticularissecretes glucocorticoids and androgens 2) Zona Glomerulosasecretes mineralocorticoids b) Medullasecrets adrenaline and noradrenalin

Control of the adrenal glands is gi en the !ox"

S#nthesis of adrenal hormones

Glucocorticoids$ Cortisol is the major glucocorticoids in the humans. Cortisol levels are highest in the morning and lowest in the night. Glucocorticoids increase or stimulate follo%ing luconeogenesis

l!cogen deposition "rotein catabolism #at deposition

Mineralocorticoids$ Aldosterone is the predominant mineralocorticoid. Aldosterone increases the $a% reabsorbtion and increases &% and ' %excretion. Aldosterone is important is blood pressure regulation( and is controlled b! the )AA* s!stem Androgens$ Androgens are important for pubert!. Androgens are regulated b! the same '"A axis. Catecholamines$ +nl! small portions of catecholamines are secreted b! the adrenal medulla( the! are mainl! secreted b! the nerve endings.

ADD&S'N(S D&SEASE Etiolog#

)rimar# *+AC,-. Autoimmune. *poradic( "ol!glandular s!ndromes 2uberculosis Corticosteroid !ios#nthetic en/#me defects Congenital adrenal h!perplasias

6rugs met!rapone( ,etocona0ole (etc

'/35A/6* 7etastatic carcinoma 1ilateral adrenalectom!

Rare causes 8!mphoma

Secondar# *0AC,-. :ithdrawal of suppressive glucocorticoids therap! '!pothalamic or pituitar! disease

/ntra.adrenal haemorrhage
9:aterhouse.#riedrichsen s!ndrome) Clinical features$ Asthenia is the cardinal s!mptom. wea,ness( anorexia ma! be other features.

-nexplained weight losss $ausea( vomiting( chronic diarrhea '!perpigmentation .. /t commonl! appears as a diffuse brown( tan( or bron0e dar,ening of parts such as
the elbows or creases of the hand and of areas that normall! are pigmented such as the areola about the nipples. 1luish.blac, patches ma! appear on the mucous membranes. '!per pigmentation is due to AC2' excess( so h!per pigmentation is not feature of secondar! adrenal in sufficienc! 3itiligo ma! paradoxicall! be present in patient with autoimmune Addison4s disease

 Arterial h!potension with postural accentuation is fre;uent . "ostural h!potension is

usuall! ver! earl! sign Axillar! and pubic hair ma! be decreased esp. in women. Clinical features of acute Addison(s disease are: 1. unconsciousness 2. Circulator! shoc, with h!potension. 3. 3omiting and diarrhoea. &n estigations$ 1. AC,- stimulation test: 2his test is done to confirm adrenal insufficienc! 2<=microgms of AC2' are injected /.3 or /7. *erum cortisol levels are chec,ed at = and >= minutes. $ormal subjects plasma cortisol ? @A= nmol5l 91B= Cg5dl)D either at baseline or at >= minutes Cortisol levels fail to increase in response to exogenous AC2' in patients with primar! or secondar! adrenal insufficienc!. A random serum cortisol should not be used for diagnosis adrenal insufficiency. 2) AC,- Le els "rimar! or secondar! adrenal insufficienc! can be distinguished b! measurement of AC2' AC2' will be high in AddisonEs disease and low in secondar! adrenal insufficienc!. 3. long acting AC,- stimulation test long AC2' stimulation test can be used .1 mg depot AC2' i.m. dail! for > da!s

/n secondar! adrenal insufficienc! there is a progressive increase in plasma cortisol

with repeated AC2' administration( whereas in AddisonEs disease cortisol remains less than B== nmol5l 92<.@ Cg5dl) at F hours after the last injection. 4. Aldosterone and renin$ 2hese tests are done supine position. /n Addison4s disease aldosterone is decreased and renin is increased but both are normal in secondar! adrenal insufficienc! 5. 'ther !iochemical a!normalities 6 '!pogl!cemia( h!ponatremia( and h!per,alemia. 7. 'ther tests to esta!lish the cause Autoimmune adrenal failure. antibodies against steroid.secreting cells 9adrenal and gonad)( th!roid antigens( pancreatic G cells and parietal cells. 2h!roid function tests(

full blood count 9to screen for pernicious anaemia)( plasma calcium( glucose and tests of gonadal function. 2uberculosis causes adrenal calcification( visible on plain H.ra! or ultrasound scan.

'/3 I8/*A. /maging of the adrenals b! C2 or 7)/ to identif! metastatic malignanc!

Management$ 2reatment of adiisons disesease is life long replacement of glucocorticoids. )eplacement doses and advice to patient of Addison4s disease are "rednisolone B.< mg dail! 9< mg on wa,ing( 2.<mg at 1F==h) or '!drocortisone 2= mg dail! 91= mg on wa,ing( < mg at12==h( <mgat 1F==h) Mineralocorticoid 8 7ineralocorticoids are ver! rarel! re;uired. #ludrocortisone <=. >== micro grams dail! Ade9uac# of glucocorticoid dose is :udged !#$ Clinical well.being

$ormal cortisol levels during the da! while on replacement h!drocortisone ;ludrocortisone re<lacement is assessed !#$ )estoration of serum electrol!tes to normal blood pressure response to posture 9it should not fall ? 1= mm'g s!stolic after 2 minutesE standing) 2uberculosis must be treated All <atients re9uiring re<lacement steroids should$ &now how to increase steroid replacement dose for intercurrent illness Carr! a E*teroid CardE :ear a 7edic.Alert bracelet 9or similar)( which givesdetails of their condition so
that emergenc! replace ment therap! can be given if found unconscious &eep an 9up.to.date) ampoule of h!drocortisone at home in case oral therap! is impossible( for administration b! self( famil! or ". Management of acute h#<oadrenalism$ Clinical context: h!potension( h!ponatraemia( h!per,alaemia( h!pogl!caemia( deh!dration( pigmentation often with precipitating infection( infarction( trauma or operation. &"= fluids: one liter of $*51=J dextrose in first one hour and then several liters there after. &ng" -#drocortisone 1==mg /.3 stat and then repeat KAhrl! until patient can ta,e orall!. Glucose should be infused if there is h!pogl!caemia. mineralocorticoids activit! . 2reat the precipitating cause esp. infection with antibiotics.

;ludrocortisone is not re;uired. as the high( since cortisol doses provide sufficient

 +ral replacement medication is then started( unless unable to ta,e oral medication(
initiall! h!drocortisone 2= mg( F.hourl!( reducing to 2=.>= mg in divided doses over a few da!s.

C'NGEN&,AL ADRENAL ->)ER)LAS&A*CA-.

+ne of the important cause of h!poadrenalism. CA' is autosomal recessive disorder( deficienc! of en0!mes of cortisol s!nthesis. Commonest deficienc! is 21h!drox!lase resulting in reduced levels of cortisol and increased of AC2'. Clinical features 1. ;emales: ambiguous genitalia( clitoromegal! and h!poadrenalism. 2. Males$ enlarged penis and increased pubic hair and h!poadrenalism. 3. 6eficiencies of 11 h!drox!lase and 1B h!drox!lase present with h!pertension. &n estigations$ )educed levels cortisol and increased levels of 1B h!drox!progesterone and AC2'. +ther investigations are same as Addison4s disease. Management$ )eplacement of glucocorticoids is the main sta! therap!. aim is to suppress the AC2'. 6oses of steroids are same as addisons but higher dose is re;uired in the night and lower in the morning