Adrenal Disorder

Question 1

Two Types of Adrenal Glands

Answer 1

Adrenal Cortex and medulla

Question 2

Adrenal Cortex Hormones

Answer 2

Mineralocorticoids – produced in Zona glomerulosa
Aldosterone
Stimulates renal tubule sodium reabsorption and potassium excretion
Goal to increase intravascular volume.
Sodium reabsorption = water retention

Glucocorticoids – produced in Zona fasciculata
Cortisol

Androgens – produced in Zona reticularis

Question 3

Adrenal Medulla Hormones

Answer 3

Norepinephrine
Epinephrine
Dopamine

Active in fight or flight sympathetic response, stress, sepsis
Not under pituitary control
Controlled by sympathetic nervous system via
direct neural stimulation

Question 4

Function of Cortisol

Answer 4

Inhibits insulin

Regulates the metabolism of proteins

carbohydrates, and fats

Diurnal excretion
Highest at ~0600
Lowest at ~2400

increases with exercise and stress

Increases in acute trauma, infection

Required for Angiotensin II production, maintains vascular tone

Regulates inflammatory response of immune system

Question 5

Epidemiology and Etiology of Addison’s Disease

Answer 5

Autoimmune (idiopathic) destruction of adrenal cortex is most common cause in U.S.

Chronic use of steroids with blunting of ACTH/CRH response during physiologic stress

Women > men

30-60 yr age of onset

Infection as cause

TB is common cause in developing nations
Disseminated fungal infection

Intolerance to physiologic stress is a main concern

Other causes
Metastatic carcinoma
Iatrogenic
Drugs: ketoconazole, steroids

Question 6

Pathophysiology of Addisons

Answer 6

Chronic deficiency of cortisol, aldosterone, and adrenal androgens/estrogens

Characterized by high ACTH and low corticosteriod

Autoimmune disease

Question 7

Signs/ Symptoms of Addison’s Disease

Answer 7

Constitutional
Weakness, fatigability
Low grade fever

Skin
Hyperpigmentation, creases

GI
Anorexia, weight loss
Abdominal pain
Anorexia, N/V, Salt craving, Signs of dehydration

Cardiac
Hypotension
small heart
Shock

MSK
Myalgias, arthralgias

Neuro
Delayed deep tendon reflexes

Psychiatric
Lethargy, confusion, psychosis

Question 8

Chronic Adrenocortical Insufficiency Signs/ Symptoms

Answer 8

Anxiety, irritability

Orthostatic Hypotension

Skin pigment changes – “tanning” over non-exposed and exposed areas, hand creases

Question 9

Chronic Adrenocortical Insufficiency Lab and Imaging

Answer 9

Hypoglycemia

Plasma ACTH is elevated

Low serum Sodium

Elevated Potassium
Retains K+

Question 10

Diagnosis of Chronic Adrenocortical Insufficiency

Answer 10

Low plasma cortisol @ 8am is diagnostic with elevated plasma ACTH level (>200 pg/mL)

Antiadrenal antibodies in autoimmune disease

Corticotropin (ACTH) Stimulation Test
Stimulates adrenal cortex to produce and secrete cortisol
Failed adrenal response (no cortisol elevation) is confirmatory

Question 11

Chronic Adrenocortical InsufficiencyTreatment

Answer 11

Replacement of glucocorticoids and mineralocorticoids

Hydrocortisone
Give lowest effective dose to avoid Cushingnoid side effects
Increase dose during times of acute illness/stress

DHEA

Medical alert bracelet

Question 12

Adrenal Crisis

Answer 12

Occurs in patients who stop their Steroidal medications

This is most common cause of adrenal insufficiency
abrupt cessation of glucocorticoids

Can occur in stressful periods without prior glucocorticoid dose adjustment:
Infections
Trauma
Surgery

Goal of treatment:

Reverse hypotension and electrolyte abnormalities
Intravenous infusion with isotonic normal saline

Replace glucocorticoid

Question 13

Cushing’s Disease

Answer 13

Caused by excess secretion of ACTH usually by a pituitary adenoma
Cushing’s disease is the most common cause of endogenous hypercortisolism

Question 14

Cushing’s Syndrome

Answer 14

Adrenocortical tumors
Non-pituitary ACTH secreting tumors
Small cell lung carcinoma
Ectopic CRH
Bronchial carcinoid
Chronic exposure to Glucocorticoid hormones such as prednisone are the most common exogenous cause  (Iatrogenic)

Question 15

2 Cushing’s Syndrome Subcategories

Answer 15

ACTH Dependent

Cushing’s Disease
Non-pituitary ACTH secreting tumor
Ectopic CRH

ACTH Independent
Adrenocortical tumors

Question 16

Cushing’s Syndrome Signs and Symptoms

Answer 16

Central obesity (90%), “Moon face”, “Buffalo hump”
Supraclavicular fat pads, protuberant abdomen, thin extremities
Hypertension (85%), Decreased glucose tolerance (80%)
Amenorrhea (↑androgens), Weakness (↓K+)
K+ excretion and Na+ retention , CA++ loss
Immunosuppression (↓ PMNs)
Hirsutism (70%); Osteoporosis (55%)
Thirst, polyuria, renal calculi
Acne, Purple striae
Impaired wound healing, susceptible to infections
Mental : poor concentration, depression, psychosis
Enhances catecholamine activity

Question 17

Initial Lab Diagnosis for Cushings Syndrome

Answer 17

24-Hour Urinary Free Cortisol Level – levels high in Cushing’s in 3 separate collections

Late night salivary cortisol level
High in Cushing’s (normally, it is low)

Low Dose Dexamethasone Suppression Test (DST)

Dexa 1mg at 11PM. Check cortisol at 8:00 AM. Will diagnose Cushings if cortisol is high (>0.5 mcg/dL)

Normal <0.5mcg/dL

Question 18

Treatment for Cushing’s Syndrome

Answer 18

Treatment depends on the specific reason for cortisol excess.

If the cause is long-term use of glucocorticoid hormones, the dosage is gradually reduced to the lowest dose adequate for control of the indicated disorder
Ketoconazole, Metyrapone

For pituitary adenoma/Adrenal adenoma: surgery
After surgery need to maintain on

Question 19

Cushing’s Complications

Answer 19

Untreated, can cause death
Complications of hypertension and diabetes
Increased susceptibility to infections
Osteoporosis, nephrolithiasis, psychosis

Question 20

Pheochromocytoma

Answer 20

Excessive catecholamines secreted from a tumor in the adrenal medulla:
norepinephrine, epinephrine, dopamine
typically norepinephrine
If familial, typically epinephrine
0.05%-0.2% hypertensive patients have a pheo
peak incidence 3rd-5th decade

Question 21

Pheochromocytoma Signs/Symptoms

Answer 21

Hypertension, postural tachycardia and hypotension-episodic

Classic Triad:
Attacks of severe headache, palpitations, profuse sweating range from monthly to multiple episodes daily
Cardiac arrythmias, enlargement
Intracerebral hemorrhage
Hypertensive encephalopathy
Epigastric pain, weakness, constipation
Heart failure

Question 22

People that should be examined for Pheochromocytoma

Answer 22

Patients with very difficult to control hypertension
Patients requiring more than 4 blood pressure medications
Patients with onset of hypertension before 35yo or after the age of 60
Symptoms: Headache, sweating and tachycardia/palpitatios and +/- high blood pressure

Question 23

Labs and Treatments for Pheochromocytoma

Answer 23

Urinary catecholamines, total and fractionated metanephrines, vanillylmandelic acid (VMA), and creatinine – in a 24 hour urine collection

Direct plasma assay of epinephrine and norepinephrine, free metanephrines

CT/MRI of adrenals

Question 24

Treatments for Pheochromocytoma

Answer 24

Surgical removal tumor or adrenal gland always indicated for all pts given high incidence of malignancy (unless
already metastatic)

Prognosis: complete cure if benign or if malignant caught early