AdrenalDysfunctions

CushingSyndrome(Hypercortisolism)
df: hyperfunctioning adrenal gland resulting in increased cortisol levels

Etiology

1.
2.
3.
4.

Cushing'sDisease(pituitaryoverproductionofACTH)
Adrenaloverproduction
EctopicACTH(cancerorcarcinoidsyndrome)
UnknownorexogenoussourceofACTH

Clinical
Manifestations

Fatredistribution
("moonface","buffalohump",truncalobesity)
Skin
(striae,thinning,easybruising)
Osteoporosis
HTN
Menstrualdisorders&erectiledysfunction
Cognitivedysfunction
Polyuria
Musclewasting
Hirsutism

LabStudies

Hyperglycemia
Hyperlipidemia
Hypokalemia
Metabolicalkalosis
Leukocytosis

Establishhypercortisolism

24hrurinecortisol
Overnightlowdosedexamethasonesuppressiontest

MeasureACTH

Low
=adrenalsource
High=pituitaryorcancer

Workup

DetermineACTHsource

Highdosedexamethasonesuppressiontest
NOsuppression
=cancer
Suppressed
=pituitary

DiagnosticImaging

Treatment

Adrenalsource:CTadrenals
Cancer:CTchest
Pituitary:MRIbrainpetrosalvenoussinussamplingforACTH

Surgicalremoval

AddisonDisease(AdrenalInsufficiency)
df:hypofunctioningadrenalglandresultingindecreasedcortisollevels

Clinical
Manifestations

Weaknessandfatigue
AMS
Nausea,vomiting,anorexia
Hypotension
Hyperpigmentation
Fever(acuteadrenalinsufficiency)

Hypoglycemia
Lowaldosterone
Hyperkalemia
Hyponatremia
Metabolicacidosis
Eosinophilia
HighBUN

LabStudies

EstablishHypocortisolism

24hrurinecortisoltest
Overnightlowdosedexamethasonesuppressiontest

Workup

MeasureACTH

High:

indicatesprimaryadrenalfailure
Low:indicatespituitaryfailure(secondary)

CosyntropinStimulationTest

SyntheticACTHisthemostspecifictestforadrenalfunction

Acute:
hydrocortisone+saline
Chronic(nonhypotensive):
prednisone
Chronic(hypotensiveinstability):
fludrocortisone+prednisone

Treatment

Primary
(AddisonsDisease)

Autoimmunedestructionof
adrenals
Infection(TB)
Adrenoleukodystrophy
Metastaticcancertoadrenal
gland

Secondary

Longtermsteroiduse
Hypopituitarism

AcuteAdrenalCrisis

Hemorrhage
Surgery
Trauma
Profoundhypotension
Abruptcessationofchronic
highdosesteroiduse

PrimaryHyperaldosteronism
df: autonomous overproduction of aldosterone despite high blood pressure and low renin activity

Etiology

Solitaryadenoma(Conn'sSyndrome)80%
Bilateralhyperplasia

Clinical
Manifestations

Secondaryhypertension(uncontrollable)
Muscleweakness
Symptomsofdiabetesinsipidus

LabStudies

Hypokalemia
Highaldosterone
Lowrenin
Normoglycemic

Measureplasmaaldosteroneandrenin

Plasmaaldosterone/reninratio>20

Workup

Samplevenousblooddrainingadrenalgland

Highaldosteroneunilateraladenoma

Imaging

CTadrenalglandstoconfirmdiagnosis

Unilateraladenoma:laparoscopicresection
Bilateralhyperplasia:eplerenoneorspironolactone

Treatment

Pheochromocytoma
df: nonmalignant lesion of adrenal medulla autonomously over producing catecholamines despite high blood
pressure

Clinical
Manifestations

Hypertensionthatisepisodicinnature
Headache
Sweating
Palpitationsandtremor

InitialPlasmaorUrineScreens

Workup

Plasmafreemetanephrinelevels
Plasmavanillylmandelicacid(VMA)levels
Plasmaorurinarycatecholaminelevels

Imaging

CTorMRIofadrenalglandsfordiagnosis

MIBGnuclearisotopescan

Treatment

Diagnosispheochromocytomaoutsidetheadrenalglands

1st)Phenoxybenzamine(alphablocker)
2nd)Propranolol
3rd)Laparoscopicremoval
***)Metastaticdiseaseistreatedmedically

CongenitalAdrenalHyperplasia(CAH)
df: inherited defect of steroid synthesis leads to ACTH-induced hyperplasia

AllVariantsHave

IncreasedACTH
Lowcortisol
Enlargedadrenalglands
Tx:steroidreplacement

EnzymeDeficiency

MarqueFindings

OtherFindings

21hydroxylase

Hypotension
Virilization
Hyperkalemia

Mostcommon
Dx:17hydroxyprogesterone
Hyponatremia(saltwasting)
Lowaldosterone
Femalesambiguousgenitalia
Males:precociouspuberty

17hydroxylase

Hypertension
Lowandrogens
Hypokalemia

Increasedaldosterone
Hypokalemia
Males:ambiguousgenitalia
Females:lack2sexualcharacteristics

11hydroxylase

Hypertension
Virilization
Hypokalemia

Lowaldosterone