Top Banner
Adrenal Disease Jennii Stephens, PA-C
78

Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland Anatomy and Physiology Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Dec 24, 2015

Download

Documents

Welcome message from author
This document is posted to help you gain knowledge. Please leave a comment to let me know what you think about it! Share it to your friends and learn new things together.
Transcript
Page 1: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Adrenal Disease

Jennii Stephens, PA-C

Page 2: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diseases of the Adrenal Gland

Anatomy and Physiology Decreased adrenal function

Cortex• Addisons Disease• Secondary hypoadrenalism (pituitary dysfunction)

Increased adrenal function Cortex:

• Cushings Syndrome/Disease• Conn Syndrome

Medulla: Pheochromocytoma

Page 3: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Adrenal Gland

Page 4: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Adrenal Anatomy

“salt”

“sex”

“sugar”

“GFR”—salt, sugar, sex, the deeper you go the sweeter it gets

Page 5: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Glomerulosa Mineralocorticoids, aldosterone

• maintains sodium and potassium water balance via the distal tubules of the kidney

Fasciculata Glucocorticoids, cortisol

• regulates carbohydrate, protein and fat metabolism

Adrenal Cortex Layers

Page 6: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Adrenal Cortex Layers Reticularis

Androgens and estrogens These are produced in far greater abundance in gonads

No other tissues have the capability of producing either mineralocorticoids or glucocorticoids

Page 7: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Adrenal medulla

Inner portion of adrenal gland Secretes epinephrine and norepinephrine Acts to reinforce activity of the sympathetic

nervous system Not vital to life (its absence does NOT cause

disease)

Page 8: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Mineralocorticoids Aldosterone Site of action is distal tubules of kidney Retain Nablood volumeblood

pressure Crucial for sodium conservation in

Kidney Salivary glands Sweat glands Colon

Absolutely essential for life

Page 9: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Aldosterone regulation BP

Renin

Angiotensinogen Angiotensin I

Angiotensin II

ALDOSTERONE

ACE

Na reabsorption Blood volume BP

Direct renin inhibitor (Tekturna, aliskiren)

ACE inhibitors (‘pril’: lisinipril, captopril…)

Angiotensin receptor blocker (ARB), (‘sartan’: valsartan, losartan…)

Page 10: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Glucocorticoids (cortisol) blood glucose

hepatic gluconeogenesis glucose uptake and use by many tissues, but not

the brain protein degradation which frees amino acids for

gluconeogenesis lipolysis, releasing fatty acids which can act as

an alternative metabolic fuel for tissues Frees glucose for brain usage

Anti-inflammatory and immunosuppressant Synthetic forms maximize these characteristics

allowing us to use them clinically

Page 11: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pituitary-Adrenal Axis

Page 12: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Glucocorticoids (cont)

Secretion of cortisol is controlled by ACTH from the anterior pituitary pro-opiomelanocortin is broken down to form ACTH

and melanocyte-stimulating hormone (MSH) Thus, high ACTH means high MSHincreased skin

pigmentation

Page 13: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Androgens

Produced by the zona reticularis

Primary area of androgen production in women

Page 14: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diseases of the Adrenal Gland

Anatomy and Physiology Decreased adrenal function

Cortex• Addisons Disease• Secondary hypoadrenalism (pituitary dysfunction)

Increased adrenal function Cortex:

• Cushings Syndrome/Disease• Conn Syndrome

Medulla: Pheochromocytoma

Page 15: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Primary v Secondary

Primary disease Pathology at the organ

Secondary disease Pathology somewhere other than the organ The organ has the ability to function normally

Page 16: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Addison’s

1-4 in 100,000 people Most common in adults 30-50 yo Primary hypoadrenalism

Pay attention to the distinction between primary and secondary characteristics

Page 17: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pathophysiology of Addisons

Due to destruction of >90% of bilateral adrenal cortices (80% of cases)

Types Usually autoimmune

• Can be associated with other autoimmune diseases (Graves, type I DM, pernicious anemia…)

• Takes months to years to destroy this much cortex Thrombosis Hemorrhage Infectious causes (HIV, Tb, fungal…) Cancer Certain drugs

Affects both glucocorticoid and mineralocorticoid function

Page 18: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pathophysiology of Secondary hypoadrenalism

Due to lack of ACTH Results in deficiency of cortisol Thus, aldosterone is NOT affected

Causes Pituitary disease, such as tumor Prolonged use of steroid medication is discontinued

without appropriate taper Infection Head trauma Pituitary infarction (Sheehan’s syndrome)

Page 19: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Effects of low aldosterone Addison’s disease

Na excretion K secretion

Thus, get rid of Naget rid of watervolume depletionlow BP

Page 20: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Effects of cortisol deficiency Addison’s disease or Secondary hypoadrenalism

Carbohydrate metabolism disturbed Hypoglycemiaweakness Addison’s disease

ACTH is increased in response to low cortisol This stimulates MSH (melanocyte stimulating

hormone)hyperpigmentation

Page 21: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Chronic presentation

Hyperpigmentation of skin and mucous membranes Caused by ACTH stimulatory effect on

melanocytes Most prominent on

• sun-exposed areas• Knuckles, elbows, knees and new scars• Palmar creases, nail beds, oral cavity, vaginal and

perianal mucosa

Page 22: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.
Page 24: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.
Page 25: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Hyperpigmented fingers and nails

Fingers of a 28-year-old white woman with Addison's disease (underneath) compared to those of a normal woman (top). There is hyperpigmentation of the skin and increased pigmentation of the distal half of the nails that occurred during the period of adrenal insufficiency. The proximal half of the nails are hypopigmented, a reflection of the reduction in ACTH secretion after the institution of glucocorticoid therapy. Courtesy of David N Orth, MD.

Page 26: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

A Caucasian Addison’s

patient

Page 27: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.
Page 29: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Chronic presentation Progressive weakness Chronic, worsening fatigue Poor appetite, craving of salty foods Weight loss Hypotension (Addison’s) GI symptoms (n/v/d) Dizziness (orthostatic hypotension) Irritability and depression Myalgia and flaccid muscle paralysis (from

hyperkalemia) decreased/absent reflexes Addison’s disease only

Page 30: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Chronic presentation

Men Impotence Decreased libido

Women Decreased body hair (from decreased

androgens) Irregular menses or amenorrhea

• Due to chronic ill health/ weight loss/ autoimmune destruction of ovarian tissue

Page 31: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Lab Studies BMP

Hyponatremia, Hyperkalemia, Mild non-anion gap metabolic acidosis (Addison’s only)• due to lack of sodium-retaining and potassium and hydrogen

ion-secreting action of aldosterone Elevated BUN and creatinine (Addison’s only)

• due to hypovolemia, a decreased GFR, and decreased renal plasma flow

Hypercalcemia (unknown mechanism) Hypoglycemia (Both primary and secondary dz)

• Caused by increased peripheral utilization of glucose and increased insulin sensitivity

Urinary and sweat sodium elevated (Addison’s only)

Page 32: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diagnosis of Adrenal Insufficiency ACTH stimulation test (Cortrosyn)

Assesses functional capacity of adrenal glands to make cortisol

1. Draw cortisol level2. Inject ACTH3. Wait 30-60 minutes4. Draw cortisol levels

If normal adrenal functionhigher cortisol If abnormal adrenal functionno change If secondary diseasecan be no change to

higher levels depending on the chronicity of the disease

Page 33: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Imaging Studies

CT—depends on cause Infectious—will often show enlarged adrenals TB, histoplasmosis—calcification of adrenals Autoimmune—atrophic adrenals

Page 34: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Other studies EKG—changes due to hyperkalemia

Page 35: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Acute presentationAddisonian Crisis

Prominent n/v Vascular collapse (shock) Confused Cyanotic Hyperpyrexia (may reach 105ºF)

Abdominal symptoms may appear like an acute abdomen

Page 36: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Acute causes

Stress (infection, trauma, surgery, emotional turmoil) with failure to increase steroids in patient with chronic Addison’s

Abrupt cessation of chronic oral steroids COPD patient

Bilateral adrenal hemorrhage Fulminant meningococcemia

Bilateral adrenal artery emboli Sepsis, DIC

Medications: rifampin, ketoconazole, phenytoin

Page 37: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Treatment of Acute Adrenal Crisis

Begin immediate treatment with salt, fluids and glucocorticoids

draw random plasma cortisol level (before any glucocorticoids given)

Page 38: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Treatment

Replace cortisol Acute adrenal crisis: IV

• Clinical improvement (BP response) should be seen within 4-6h of tx

• Taper dose after response Chronic

• Daily oral replacement of both mineralocorticoid and corticosteroid

• For lifetime (can’t ever stop)• Will have to increase doses during periods of

stress

Page 39: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Chronic meds

Hydrocortisone Drug of choice for acute and chronic tx in

Addison’s Has both glucocorticoid and mineralocorticoid

properties Titrate to patient’s general well being and

presence of symptoms Patients should be told to double or triple their

steroid replacement doses in stressful situations (common cold, tooth extraction…)

Page 40: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Chronic Meds (cont)

Fludrocortisone Very potent mineralocorticoid Oral med Titrate to maintain normal BP, Na, and K

levels No dose adjustment in stressful situations

Page 41: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Miscellaneous

Should consult endocrinologist Patient should wear medical ID bracelet Prognosis: with adequate therapy, normal

lives

Page 42: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diseases of the Adrenal Gland

Anatomy and Physiology Decreased adrenal function

Cortex• Addisons Disease• Secondary hypoadrenalism (pituitary dysfunction)

Increased adrenal function Cortex:

• Cushings Syndrome/Disease• Conn Syndrome

Medulla: Pheochromocytoma

Page 43: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Cushings 13 per million patients Usually due to exogenous glucocorticoids 5 women:1 man Usually 25-40 yo

Two distinctions: Syndrome--A group of conditions caused by

increased production of cortisol hormones or by the administration of glucocorticoid hormones

Disease—a form of Cushing’s syndrome caused by an ACTH-secreting pituitary tumor

Page 44: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Presentation

Increased adipose tissue moon facies buffalo hump (on upper back at base of neck) supraclavicular fat pads truncal obesity

facial plethora (flushed) purple striae

usually >1cm in width Most commonly abdomen, buttocks, lower

back, upper thighs and arms, breasts

Page 45: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Moon Facies30-year-old woman with Cushing's disease showing round, plethoric "moon" face, facial hirsutism, and increased supraclavicular fat pads. Williams Textbook of Endocrinology, 8th ed, Foster, DW, Wilson, JD (Eds), WB Saunders, Philadelphia, 1996.

Page 46: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Moon facies

Page 49: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.
Page 51: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Signs/ SymptomsSKIN Striae

CARDIOVASCULAR Hypertension

Edema

Atherosclerosis

GI Peptic ulcers

ENDOCRINE Hypothyroidism

Galactorhea

Menstrual irregularities

MUSCULOSKELETAL Proximal muscle weakness

Osteoporosis

PSYCHOLOGICAL Emotional lability

Fatigue

Depression

Page 52: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pathophysiology Caused by excess levels of cortisol

Exogenous • Most common cause• Usually from oral steroids

Endogenous• Tumors of the adrenal gland • Mets • Cushing’s Disease—pituitary tumor• Lung cancer

Page 53: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Lab Studies

24h Urinary Free Cortisol (UFC) test Screening test Indicator of overall daily cortisol production Values >3X upper normal suggest Cushings

Why do we not check random serum cortisol? Because it fluctuates too much

Page 54: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Lab Studies

Tests to determine cause Serum ACTH Overnight dexamethasone suppression test

Page 55: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Other lab signs of Cushings

Leukocytosis Elevated fasting glucose Low potassium (if adrenal adenoma secreting

aldosterone)

Page 56: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Imaging

Only after lab screening tests Why? 10% incidence of nonfunctioning

pituitary or adrenal adenomas CT abdomen (if suspect primary adrenal

problem) MRI pituitary (if suspect pituitary problem)

Page 57: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Treatment

Dependent on the cause Exogenous steroid

Reduce the cortisol to lowest possible amount Endogenous steroid

Surgical resection of causative tumor Ketoconazole: inhibits key steps in

mineralocorticoid and glucocorticoid synthesis

Page 58: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Other considerations

Pts on steroids > 4-6wks Need meds to prevent bone loss

Page 60: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Hypercortisolism

Stress response Women in last 3 months of pregnancy Athletes during times of intense training Depressed patients Alcoholics Malnourished patients Panic disorder

Page 61: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diseases of the Adrenal Gland

Anatomy and Physiology Decreased adrenal function

Cortex• Addisons Disease• Secondary hypoadrenalism (pituitary dysfunction)

Increased adrenal function Cortex:

• Cushings Syndrome/Disease• Conn Syndrome

Medulla: Pheochromocytoma

Page 62: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Conn Syndrome

Primary hyperaldosteronism Up to 1% of HTN pts Characterized by

HTN Hypokalemia (fatigue, muscle cramps, h/a,

palpitations) Causes

Adenoma or cancerous growth Hyperplasia

Treatment depends on the cause

Page 63: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Diseases of the Adrenal Gland

Anatomy and Physiology Decreased adrenal function

Cortex• Addisons Disease• Secondary hypoadrenalism (pituitary dysfunction)

Increased adrenal function Cortex:

• Cushings Syndrome/Disease• Conn Syndrome

Medulla: Pheochromocytoma

Page 64: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pheochromocytoma

Rare catecholamine-secreting tumor from the adrenal gland

1-8 cases per million persons Up to 0.2% of hypertensive

individuals 10% of these are malignantmaking

the diagnosis is critical Occurs in all races and equally

among sexes Peak incidence is between 3rd and

5th decades

Page 65: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Pathophysiology

Due to excess catecholamines (epinephrine and norepinephrine)

Elevated blood pressure Increased cardiac contractility Elevated heart rate Glycogenolysis Gluconeogenesis

Page 66: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Rule of 10s

10% are bilateral 10% occur in children 10% are outside the adrenal glands 10% are familial 10% without blood pressure elevation 10% are malignant (may be closer to 25%)

Page 67: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Clinical Presentation Spells with

headache palpitations diaphoresis in association with severe hypertension

Spells may occur monthly to several times a day Duration of spells may be seconds to hours

With time, spells worsen in severity and become more frequent

May present in association with Von-hippel Lindau, Neurofibromatosis, and MEN II syndrome

Page 68: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Lab Studies

A 24hour urine collection for vanillylmandelic acid (VMA) Metanephrines

Page 69: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Indications for workup

HTN refractory to multiple meds Wide swings in blood pressure Unexplained spells of dizziness Orthostatic hypotension in the absence of

medication Family history of pheochromocytoma Incidental adrenal mass (4-6.5% of

patients with adrenal mass will have pheo)

Page 70: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Imaging

90% are in adrenals, 98% in abdomen and pelvis

Once found, may want to rule out familial syndromes

Page 71: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Treatment

Alpha and beta-blockade prior to surgery Surgical resection usually results in resolution of htn

Page 72: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Case Presentation 1 42yo male presented to his PCP for

fatigue (increasing and worse later in the day) weight loss (40lb over 5yr)

Previous episodes of fatigue were attributed to hypothyroidism due to increased TSH and his levothyroxine was increased.

PE Patient appeared ill BP 96/66 Generalized hyperpigmentation of sun-exposed areas

f/u 2 mos later, same hyperpigmentation with BP of 80/74, weight stable

Page 73: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Case 1 (cont)

Lab Na 127 (136-142) Chloride 93 (96-106) TSH 12.4 (0.5-5.0) CBC nl Morning cortisol 1.5 (6-24)

What are you suspicious of? Addison’s disease

Page 74: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Questions What test should be ordered? ACTH stimulation test

Results revealed cortisol levels less than 1 with NO response to ACTH

Outcome: patient was placed on hydrocortisone and referred to endo. 1 month later was seen for follow-up, BP had improved to 92/62, he reported feeling better, was able to ‘play football with my son for the first time in years’, and had gained 9 pounds.

What etiology do you suspect with both thyroid and adrenal hypofunction?

Autoimmune destruction

Page 75: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Case presentation 2 A 43-year-old white man presents for evaluation of a 2

year history of recurrent episodes of palpitations, diaphoresis, headache, and acute anxiety

Admitted for a-fib which resolved with medical treatment

Over 2 years, the attacks became more frequent increasing to two times per week.

During asymptomatic times, his BP was nl. During one episode in the office, his blood pressure

was 200/120 which resolved as his sxs resolved.

Page 76: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Questions

What test should be ordered? Urine VMA and metanephrines

The results reveal elevated levels of both

What further testing should be done? Abdominal CT/MRI

Abdominal CT reveals a large complex right adrenal mass

Page 77: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Questions

What is the diagnosis? Pheochromocytoma

What is the treatment? Surgery

Outcome: this patient had complete resolution of symptoms with alpha and beta blockade prior to surgery. His HTN and symptoms were completely resolved without medicine following surgical removal of the benign tumor.

Page 78: Adrenal Disease Jennii Stephens, PA-C. Diseases of the Adrenal Gland  Anatomy and Physiology  Decreased adrenal function Cortex Cortex Addisons DiseaseAddisons.

Sources cited1. Sherwood, Lauralee. 1993. Human Physiology from Cells to Systems. West Publishing Company: St.

Paul, MN.2. www.cmaj.ca/cgi/content/full/171/5/4513. www.med.sc.edu:85/ghaffar/tolerance2000.htm4. www.emedicine.com/derm/topic761.htm5. salmon.psy.plym.ac.uk/year1/stressho.htm6. www.niddk.nih.gov/health/endo/pubs/addison/addison.htm7. www.umm.edu/endo/endocrin/addison.htm8. www.nlm.nih.gov/medlineplus/ency/imagepages/1146.htm9. http://cushings.homestead.com/Photos.html10. http://www.cc.nih.gov/ccc/papers/vonhip/adrenal.html11. http://cnserver0.nkf.med.ualberta.ca/cn/Schrier/Vol3/Fig.%204-36A.jpg12. http://www.travelin-tigers.com/zlyn/health.htm13. http://www-medlib.med.utah.edu/WebPath/ENDOHTML/ENDO001.html14. http://www.emedicine.com/derm/topic761.htm15. http://www.netmedicine.com/img_ekg/ekg16.jpg16. http://www.pens.org/articles/edwards-e.htm17. http://www.emedicine.com/med/topic432.htm18. http://www.endotext.com19. Wilson, William C. 2004. Preventing a fatal outcome in Addison’s disease. JAAPA 17 (8): 35-38.20. http://www.emedicine.com/med/topic432.htm21. www.vh.org/adult/provider/anatomy/MicroscopicAnatomy/Section15/Plate15293.html22. http://textbook.cecilmedicine.com/content/23. Nguyen-Martin, Mary Ann and Gary Hammer. Hospital Physician February 2006. “Pheochromocytoma:

An Update on Risk Groups, Diagnosis, and Management”