Ped EndoEmerg PLG - Copy

8/19/2019 Ped EndoEmerg PLG - Copy

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PKB V Palembang 18 Feb 2012

Pediatric EndocrinologyEmergency

Rogatianus Bagus PPresented by Jose RL Batubara


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ADRENAL INSUFFICIENCY


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Adrenal Anatomy &

Physiology• The adrenals are endocrine organs

that sit on to o! each "idney


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• Each adrenal gland has t#o arts

– Adrenal $ed%lla inner area'• Secretes catecholamines #hich

mediate stress res onse helre are a erson !or

emergencies'(» Nore ine hrine» E ine hrine» Do amine


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• Adrenal Corte) o%ter area* enclosesAdrenal $ed%lla'

– Secretes steroid hormones• Glucocorticoids + e)ert a

#ides read e,ect onmeta-olism o! car-ohydratesand roteins

• Mineralocorticoids + are essentialto maintain sodi%m and .%id-alance

• se) hormones secondaryso%rce'


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• A erson can s%r/i/e #itho%t a!%nctioning adrenal med%lla(

• A !%nctioning adrenal corte) or thesteady a/aila-ility o! re lacementhormone' is essential for survival.


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The Essential Steroids

• Primary gl%cocorticoid+ – Cortisol a("(a( hydrocortisone'

• Primary mineralocorticoid+ – Aldosterone


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Cortisol

• A gl%cocorticoid• Fre0%ently re!erred to as the 1stress

hormone2 – Released in res onse to hysiological or

sychological stress• E)am les+ e)ercise* illness*

in3%ry* star/ation* e)tremedehydration* electrolyteim-alance* emotional stress*s%rgery* etc(


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Cortisol

• Critical actions on many hysiologicsystems* incl%ding+

– $aintains cardio/asc%lar !%nction – Pro/ides -lood ress%re reg%lation – Ena-les car-ohydrate meta-olism

• acts on the li/er to maintainnormal gl%cose le/els

– Imm%ne !%nction actions• Red%ces in.ammation• S% resses imm%ne system


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Cortisol

• 4hen cortisol is not rod%ced orreleased -y the adrenal glands*h%mans are %na-le to res onda ro riately to hysiologicstressors(

•

Rapid deterioration res%lting inorgan damage andshoc"5coma5death can occ%r*es ecially in children


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Aldosterone

• a mineralocorticoid

• Reg%lates -ody .%id -y in.%encingsodi%m -alance

• The h%man -ody re0%ires certainamo%nts o! sodi%m and #ater in

order to maintain normalmeta-olism o! !ats* car-ohydratesand roteins(


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• 4ater5sodi%m -alance is maintained-y aldosterone(

• 4itho%t aldosterone* signi6cant#ater and sodi%m im-alances canres%lt in organ !ail%re5death(


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4hy #e need cortisol

• Cortisol has a necessary e,ect onthe /asc%lar system -lood /essels*heart' and li/er d%ring e isodes o!

hysiologic stress


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7asc%lar Reacti/ity

• In adrenally8ins%9cient indi/id%alse) eriencing a hysiologic stressor*the /asc%lar smooth m%scle #ill

-ecome non8res onsi/e to thee,ects o! nore ine hrine ande ine hrine* res%lting in /asodilationand ca illary 1lea"ing2(

• The atient may -e %na-le tomaintain an ade0%ate -loodress%re

• The -lood /essels cannot res ond to

the stress and #ill e/ent%ally


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Energy $eta-olism

• In adrenally8ins%9cient indi/id%als%nder increased hysiologic stress*the li/er is %na-le to meta-oli:ecar-ohydrates ro erly* #hich mayres%lt in ro!o%ndly lo# -lood s%gar

that is di9c%lt to re/erse #itho%tadministration o! re lacementcortisol


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• The s eed at #hich atientdeterioration occ%rs is di9c%lt to

redict and is related to the%nderlying stressor* atient age*general health* etc(

•

Yo%ng children can -e at high ris" !orra id deterioration* e/en #hene) eriencing a 1sim le2gastrointestinal disorder(


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Endocrinologist Testimony;

•


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4ho has adrenalins%9ciency>• Anyone #hose adrenal glands ha/e sto ed

rod%cing steroids as a res%lt o!+ – Long8term administration o! steroids – Pit%itary gland ro-lems* incl%ding gro#th

hormone de6ciency* t%mor* etc( – Tra%ma* incl%ding head tra%ma that a,ects

it%itary – Loss o! circ%lation to adrenals5remo/al o! tiss%e – A%to8imm%ne disease –

Cancer and other diseases T? and @I7 mayca%se'

• There is also an inherited !orm o! adrenalins%9ciency

CA@'


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Adrenal Ins%9ciency

• Can occ%r !rom long8termadministration o! steroids o/er8rides-ody2s o#n steroid rod%ction'

E)am les+

– rgan trans lant atients – Long8term C PD – Long8term Asthma – Se/ere arthritis – Certain cancer treatments


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r mary renaIns%9ciencyB Addison2s

Disease• The adrenal glands are damaged

and cannot rod%ce s%9cientsteroid

• o! the time* damage is ca%sed-y an a%to8imm%ne res onse that

destroys the adrenal corte)• Addison2s can a,ect -oth se)es and

all age


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Addison2s sym toms

• This disease has a grad%al onset andcan -e di9c%lt to diagnose+

– Chronic* #orsening !atig%e –

4eight loss – $%scle #ea"ness – Loss o! a etite – Na%sea5/omiting – Lo# -lood ress%re – Lo# -lood s%gar – S"in hy er igmentation – Salt8cra/ing

A %t i! t ti !


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Ac%te mani!estation o!Addison2s is called AddisonCrisis• Se/ere /omiting5diarrhea• Dehydration• @y otension• S%dden* se/ere ain in -ac"* -elly or

legs• Loss o! conscio%sness• Can -e !atal


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Presentation o! AdrenalCrisis• The atient may resent #ith any

illness or in3%ry as the reci itatinge/ent(

• A atient history o! adrenal ins%9ciency #arrants acare!%l assessment %nder s eci6c rotocols

• Children may deteriorate into adrenal crisis !rom a

sim le !e/er* a gastrointestinal illness* a !all !rom a-icycle or some other in3%ry(

• A mild illness or in3%ry can easily reci itatean adrenal crisis in any age gro%


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Critical ClinicalPresentation• The early indicators o! an adrenal8

crisis onset can -e /ag%e and non8s eci6c( Some or all signs5sym toms

may -e resent(

• In!ants+ – Poor a etite – 7omiting5diarrhea – Lethargy5%nres onsi/e

• Une) lained hy oglycemia – Sei:%re5cardio/asc%lar colla se5death


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• Clearly* the signs5sym toms o!adrenal crisis are similar to otherserio%s shoc"8ty e resentations(

•

For these atients* standard shoc"management re0%iress% lementation #ith corticosteroidmedication Sol%8Corte! or Sol%8

$edrol'• It is im ortant to ANTICIPATE the

e/ol%tion o! an adrenal crisis andmedicate a ro riately %nder thes eci6c rotocols( Do not #ait %ntil a


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Patient $anagement

• Follo# standard A?C and shoc"management treatment(

• ?LS5ILS+ noti!y ALS interce t as soonas ossi-leG transport withoutdelay

• ALS+ administer steroid I$5I75I as

soon as ossi-le a!ter initial li!e8threat and shoc" management ha/e-een initiated(

• Trans ort #itho%t delay toa ro riate hos ital #ith early


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It is im ortant to note that yo% arecaring !or a atient #ith m%lti leiss%es+

1. The precipitating event (a trauma/illness thatmay be a critical issue on its own)and

2. The evolution towards adrenal crisis, whichwill result in organ failure/death if notreversed.


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• This hrase has -een added toParamedic Standing rders incertain ADULT treatment rotocols+


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PEDIATRIC ProtocolU dates• This hrase has -een added to

Paramedic Standing rders incertain PEDIATRIC rotocols+


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Please de6ne


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Sol%8Corte!

• Indications + re lacement o! a-sentcorticosteroid in identi6ed adrenally8ins%9cient atients -eing managed%nder s eci6c treatment rotocolGmany other %ses as #ell

•

Contra-Indications + Do not %se in thene#ly8-orn or any indi/id%al #ith a"no#n hy ersensiti/ity to Sol%8Corte!


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Sol%8Corte!

• Side E ects + in emergency %se*transient hy ertension and5orheadache* sodi%m5#ater retentionmay occ%r( Not %s%al in a H8timedose

• Dosage: Ad%lt+ H mg I7* I$*I

Pediatric+ mg5"g to ama) o! H mg* I7* I$* I

Protect !rom heat


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Sol%8Corte!

• Ad inistration route + I$ or slo# I7-ol%s( Ji/e I7 ?ol%s o/er Kseconds( I7 in!%sion is notacce ta-le !or emergencyadministration

• For yo%ng children* the re!erred I$site is the /ast%s lateralis m%scle


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• Onset of action + !or the indicated %seemergency steroid re lacement inatient e) eriencing stressor' the

onset o! action is inutes . Do notdelay trans ort(


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Sol%8$edrol

• Indications:!rotocol: re lacement o!a-sent corticosteroid in identi6edadrenally8ins%9cient atients -eing

managed %nder s eci6c treatmentrotocolG Ot"er + many %ses*incl%ding ac%te -ronchial asthma

not 6rst8line'G ana hyla)is not

6rst8line'G ac%te e)acer-ation o!m%lti le sclerosis

• Contraindications: any atient #ithsystemic !%ngal in!ection* any

erson #ith "no#n hy ersensiti/ity


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Sol%8$edrol

• Dose+ Ad%lt+ H mg I$5I75I Pediatric+ mg5"g to a ma)

o! H mg I$5I75I

• Ad inistration route + I$ or slo# I7-ol%s( Ji/e I7 ?ol%s o/er K

seconds( I7 in!%sion is notacce ta-le !or emergencyadministration

• For yo%ng children* the re!erred I$site is the /ast%s lateralis m%scle


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