adrenal disease jennii stephens, pa-c. diseases of the adrenal gland anatomy and physiology ...
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Adrenal Disease
Jennii Stephens, PA-C
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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
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Adrenal Gland
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Adrenal Anatomy
“salt”
“sex”
“sugar”
“GFR”—salt, sugar, sex, the deeper you go the sweeter it gets
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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
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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
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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)
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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
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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…)
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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
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Pituitary-Adrenal Axis
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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
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Androgens
Produced by the zona reticularis
Primary area of androgen production in women
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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
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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
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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
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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
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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)
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Effects of low aldosterone Addison’s disease
Na excretion K secretion
Thus, get rid of Naget rid of watervolume depletionlow BP
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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
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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
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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.
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A Caucasian Addison’s
patient
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Hyperpigmented scars
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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
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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
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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)
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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
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Imaging Studies
CT—depends on cause Infectious—will often show enlarged adrenals TB, histoplasmosis—calcification of adrenals Autoimmune—atrophic adrenals
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Other studies EKG—changes due to hyperkalemia
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Acute presentationAddisonian Crisis
Prominent n/v Vascular collapse (shock) Confused Cyanotic Hyperpyrexia (may reach 105ºF)
Abdominal symptoms may appear like an acute abdomen
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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
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Treatment of Acute Adrenal Crisis
Begin immediate treatment with salt, fluids and glucocorticoids
draw random plasma cortisol level (before any glucocorticoids given)
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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
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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…)
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Chronic Meds (cont)
Fludrocortisone Very potent mineralocorticoid Oral med Titrate to maintain normal BP, Na, and K
levels No dose adjustment in stressful situations
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Miscellaneous
Should consult endocrinologist Patient should wear medical ID bracelet Prognosis: with adequate therapy, normal
lives
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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
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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
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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
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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.
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Moon facies
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Buffalo hump
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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
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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
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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
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Lab Studies
Tests to determine cause Serum ACTH Overnight dexamethasone suppression test
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Other lab signs of Cushings
Leukocytosis Elevated fasting glucose Low potassium (if adrenal adenoma secreting
aldosterone)
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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)
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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
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Other considerations
Pts on steroids > 4-6wks Need meds to prevent bone loss
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Surgery Results
Before After
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Hypercortisolism
Stress response Women in last 3 months of pregnancy Athletes during times of intense training Depressed patients Alcoholics Malnourished patients Panic disorder
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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
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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
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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
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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
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Pathophysiology
Due to excess catecholamines (epinephrine and norepinephrine)
Elevated blood pressure Increased cardiac contractility Elevated heart rate Glycogenolysis Gluconeogenesis
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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%)
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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
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Lab Studies
A 24hour urine collection for vanillylmandelic acid (VMA) Metanephrines
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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)
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Imaging
90% are in adrenals, 98% in abdomen and pelvis
Once found, may want to rule out familial syndromes
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Treatment
Alpha and beta-blockade prior to surgery Surgical resection usually results in resolution of htn
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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
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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
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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
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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.
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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
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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.
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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”