Adrenal Medulla- SNS response to stress by secreting Catecholamines:

Epinephrine-increases heart rate and blood pressure

Norepinephrine-constricts arterioles
Fight or Flight Response
Cardiac output
Metabolic rate
Vasoconstriction
Respiratory rate
Adrenal Cortex- mediates the stress response produces SALT, SUGAR &
SEX
Aldosterone = promotes salt retention
Cortisol= sugar Androgens = sex hormones
1)Mineralcorticoids
(Aldosterone)- Regulate electrolyte & fluid homeostasis, Promotes
Na+ retention & K+ elimination by kidneys
If Na+ is low, aldosterone levels will
If K+ is low, aldosterone levels will
Regulated by renin-angiotensin system of kidney
2)Glucocorticoids-Regulate metabolism & critical in stress response
(Cortisol)Stimulate gluconeogenesis & blood glucose, Antiinflammatory, antiallergy, anti-immunity
secondary site of androgen synthesis regulates growth and
development of genitalia and puberty
Carbohydrates-Promotes amino acid conversion to Glucose
Inhibits Glucose uptake by muscles and fat. Insulin resistance
Fat- Increases lipolysis & free fatty acids
Protein- Increases protein breakdown which increases AA that can be
converted to Glucose
Test: cortisol levels: Diurnal Rhythm
NORMALLY checked at 9 am: highest: cortisol lev 5-7am lowest: 36pm
SEVERE STRESS:
10 X normal amount of cortisol released
1) can be lifesaving - enables body to cope with stress
2) death if adrenal cannot produce enough corticosteroids
Adrenal Cortex:
Stress Hypothalamus Stimulates Anterior Pituitary Secretes
ACTH target cell Adrenal cortex Secretes Cortisol specific action h
metabolic activity Helps manage stress

Adrenal Dysfunction:
HYPOfunction Adrenal insufficiency
Congenital adrenal hyperplasia,
Addisons disease, Infections, TB, Sepsis,
Disruption of HPA: Exogenous steroids,
ACTH secreting tumor
Low Cortisol
Low Aldosterone: Addisons disease

Addisons
Disease

Acute
Adrenal crisis
Aka
addisonian
crisis

HYPERfunction

Cortisol: Cushing syndrome

Aldosterone: Hyperaldosteronism
Catecholamines:
Pheochromocytom

- acquired
primary adrenal
insufficiency

ACTH may be high

Usually autoimmune(~80%)
Infections (TB)

Signs

LOW Aldosterone, LOW serum cortisol,

HIGH ACTH, HIGH Renin, LOW Na+, HIGH
K+, Low voltage EKG

Symptoms

Fatigue & muscle weakness Weight loss,

Hypotension, Anorexia/nausea/vomiting,
Hyperpigmentation, Bronzing of skin

Test

800 cortisol level(taper off steroid

use),
ACTH level, Random cortisol in ill patient,
ACTH stimulation test: Giving ACTH
should serum cortisol

TX

restore fluid and electrolyte,

Life long replacement of deficient
adrenal hormones
Glucocorticoids (hydrocortisone)
according to diurnal rhythm. 2/3
am,th,1/3pm
Mineralocorticoids
(fludrocortisone:Florinef)
>May need to change dose prn for stress
to prevent crisis
>Double dose if minor stress, IV if major
stress
Trauma, Surgery, Stress, Abrupt
withdrawal of cortisone meds

causes

signs

Na+ & H20 loss, Hypotension, Volume

Depletion, Tachycardia, Hypoglycemia,
Shock, Death

Treatment:

IV fluids, IV hydrocortisone

Secondary
Adrenal
Insufficiency

Inadequate secretion of ACTH by the

pititary gland
Etiology

Signs & Sxs:

TX

Adrenal Insufficiency
Primary:
Adrenal gland
Hyperpigmentation
Hyperkalemia
Elevated ACTH
Cushingsexcess cortisol

MC Withdrawal of long-term steroid

treatment that has suppressed ACTH
Hypothalamic/Pituitary dysfunction, S/p
removal of ACTH-secreting tumor
Same as primary except pallor, ACTH
secretion is low, Electrolytes are normal
Other pituitary hormone deficiencies may
be present
Cortisol replacement: Taper doses, Treat
Pituitary cause, Education: Salt foods
liberally, Do not omit meals, Eat snack, Eat
diet with carbs and proteins, Wear medic
bracelet
Kit of 100mg hydrocortisone IM

Secondary:
Hypothalamic-Pituitary
No hyperpigmentation
Normokalemia
Low ACTH: Can be low normal

Disease

Pituitary origin
Hypersecretion of ACTH from
pituitary
ACTH causes Cortisol

Syndrome

Origin is elsewhere:
Iatrogenic
Exogenous steroids
Adrenal tumor
ACTH secreting tumor
Small cell carcinoma
Hypersecretion of Cortisol

Signs and
symptoms

Metabolic
Complications

Hypercortisolism

Cortisol Excess
Round plethoric face (moon face)
Central obesity with thin limbs, Fat
pad buffalo hump, Mood/psychiatric
changes
Purple striae, Thin skin, easy bruising
Glucose intolerance (DM),
Osteoporosis
Poor wound healing, Hypertension,
Hyperandrogenism: hirsuitism/acne
> Calcium reabsorption from the
bone leads to: osteoporosis &
pathologic fractures
>Cortisol causes insulin resistance
hepatic gluconeogenesis and insulin
resistance.
leads to glucose intolerance and
diabetes mellitus
>Frequent infections & slow wound
healing
Suppressed inflammatory response
can mask severe infections
>Decreased ability to handle stress
Psych problems, mood swings
Screening Labs :
24hr urine cortisol
Serum Sodium
Serum Potassium
Glucose
>Plasma ACTH:
High in Pituitary/Ectopic source
Low in adrenal cause

Confirmation

>ACTH suppression test to identify

cause
>Dexamethasone suppression test
Determines pituitary or adrenal
>Diagnostic imaging: reveal pituitary,
adrenal or ectopic tumor

TX

Treat underlying cause

Pituitary tumor: Transphenoidal
surgery
Refractory cases may need pituitary
radiation or adrenalectomy
Adrenal tumor: remove tumor
Cortisol replacement as necessary
Diet: low calorie, carbohydrate & salt

high potassium
Congenital
Adrenal
Hyperplasia
(CAH)

Pathogenesis

90% of affected
patients
Girl signs

Lab test

Boys with CAH

Autosomal recessive disorders of

adrenal steroidogenesis leading to
deficiency of cortisol
Low cortisol level > corticotropin
releasing hormone (CRH) >
adrenocortical hyperplasia
21-Hydroxylase Deficiency (OHD)
Excess androgen: Ambiguous
genitalia in girls
>Dehydration,
>Shock,
>Salt-loss presentations with
electrolytes imbalance, Hyponatremia,
Hyperkalaemia, >Hypoglycemia,
>Hyperpigementation
Newborn screening programs
using capillary heel blood on filter
paper disks to detect 21-(OH)lase
deficiency
usually unrecognized at birth
because their genitalia are normal
May have hypospadius
> salt wasting crisis which results
in:
dehydration, hypotension,
hyponatremia, hyperkalemia

childhood (nonclassic)

early pubic hair

precocious puberty
accelerated growth

Lab Evaluation &

Diagnosis

Serum electrolytes & glucose

Low Na & high K
Fasting hypoglycemia
Elevated serum urea due to
dehydration
Elevated plasma Renin & ACTH levels
Low Cortisol
High androgens especially
testosterone level
Low Aldosterone
Urinary steroid profile
Chromosomes
Pelvic US
Rule out adrenal tumor
>Hydrocortisone

TX

Inhibits production of androgens

>Fludrocortisone (Florinef) 0.05 - 0.2
mg/day
>Normalizes plasma renin activity
>Triple hydrocortisone during stress.
>During adrenal crisis: IV
hydrocortisone and IV fluids
>Surgery for female external genitalia