Flashcards in Adrenal gland pathologies Deck (52):
What are the functions of the adrenal hormone?
1. Needed for metabolism of proteins, lipids, & carbohydrates
2. Affects serum glucose levels
3. Helps maintain BP
4. Helps regulate immune system
What is primary corticoadrenal insufficiency?
1. Ex. Addison’s Disease
A. Originates w/in adrenal gland
B. Results in ↓ secretion of mineralcorticoids, glucocorticoids & androgens
C. Underactive or damaged adrenal glands that limit cortisol production
What is secondary corticoadrenal insufficiency?
1. Ex. Glucocorticoid Deficiency
A. Due to impaired pituitary secretion of ACTH
B. Results in ↓ glucocorticoid secretion
C. Underactive pituitary gland or pituitary gland tumor that inhibits ACTH production
What are the causes of addison's disease?
1. Autoimmune disease
A. Most common cause: 70-90% cases
2. Hemorrhage into adrenal gland
What are the causes of glucocorticoid deficiency?
1. Abrupt withdrawal of long term steroid therapy
A. Most common cause
2. Removal of ACTH producing tumor
3. Pituitary injury
B. Autoimmune Process
true/false: autoimmune adrnela insufficiency is asst. with other autoimmune disorders?
53% do not have any other autoimmune disorder
What are the sxs of adrenal insufficiency?
1. Orthostatic hypotension
A. Present in Addison’s
B. Not usually present in Glucocorticoid Deficiency bc Aldosterone secretion near normal
2. Hyperpigmentation of skin
A. Present in Addison’s
B. Not present in Glucocorticoid Deficiency bc ACTH and MSH are low
What sxs are also present in Addison's disease?
2. weight loss
4. GI disturbances
5. changes in distribution of body hair
What are the fasting glucose and Na dx study results for adrenal insufficiency?
1. Fasting Glucose & Na
A. Both low
What are the plasma cortisol dx study results for adrenal insufficiency?
1. Fasting level by 8 am & 4 pm
2. Normal: high in am (peak) & low in pm
3. Addison’s Dz: low in am & in pm
What are the rapid ACTH (corticotropin) stimulation dx study results for adrenal insufficiency?
1. Obtain baseline cortisol level
2. Administration of cosyntropin (synthetic ACTH)
3. ↑ ACTH level & ↓ cortisol -> Addison’s Dz
What are the normal ACTH stimulation test results?
1. Normal pre-injection serum cortisol:
A. 5 - 25 μg/dL & doubles in 30 - 90 min, reaching at least 20 μg/dL
*If pt responds to an ACTH stimulation test, then likely due to insufficient ACTH production by the pituitary
What are the ACTH stimulation test results in a pt with Addison's dz?
Low or low-normal values that DO NOT rise above 20 μg/dL at 30 min
What are the treatment options for Addison's disease?
1. Oral fludrocortisone (Florinef)
2. Lifelong corticosteroid replacement
3. DHEA in women to improve sexual function and interest
What are the characteristics of oral fludrocortisone (Florinef)?
1. Replaces Aldosterone
2. 0.1 -0.2 mg PO QD
3. Prevents dehydration, hypotension, hyponatremia & hyperkalemia
4. Promotes Na+ & H2O retention, & lowers plasma K+ conc.
What are the characteristics of lifelong corticosteroid replacement for addison's disease?
1. Hydrocortisone (drug of choice)
2. 15-30mg orally BID
3. 2/3 in am, 1/3 in pm
4. ↑ dose w/ infection, trauma, surgery, stress, pregnancy
5. Reduce back to normal dosing as stress subsides
What is the treatment for glucocorticoid deficiency?
1. Lifelong corticosteroid replacement
2. Cortisone or IV hydrocortisone
What is an Addisonian crisis?
1. Severe endocrine emergency
2. Often brought on by acute infection
What are the characteristics of addisonian crisis?
1. Renal sodium wasting
3. Loss of vascular tone
4. Results in:
C. Acute cardiovascular collapse
What are the sxs of addisonian crisis?
1. Syncope/Near Syncope
2. Generalized Fatigue
4. Abd or Back Pain
5. Severe N/V
6. Extreme Hypotension
7. Acute Renal Failure w/ Azotemia
When is therapy initiated in Addisonian crisis?
1. Therapy should be instituted immediately upon suspicion
2. If the patient is severely ill, ACTH stimulation test should be postponed until patient is recovered
How is Addisonian crisis treated?
A. 100 mg IV over 30 sec & repeat q 6-8 h for 1st 24 hr, then
B. 150 mg hydrocortisone IV over the 2nd 24 hr if patient has improved markedly
C. 75 mg IV on the 3rd day
2. Immediate intravascular volume expansion
A. 1 liter of D5NS over 1 to 2 hr, then
B. NS IV until hypotension, dehydration, & hyponatremia corrected
3. Inotropic agents
Until BP stabilizes
4. Restart maintenance oral hydrocortisone 15-30 mg & fludrocortisone (Florinef) 0.1 mg QD after initial 3 day Tx
What is the timeline of improvement after treatment for Addisonian crisis?
Restoration of BP & general improvement should occur w/in 1 hr after initial dose of hydrocortisone
What is the prognosis for Addisonian crisis?
1. Recovery depends on Tx of the underlying cause
A. Infection, surgery, trauma, metabolic stress & adequate hydrocortisone therapy
What pt education needs to occur after an Addisonian crisis?
1. Pts should have MedicAlert & card stating condition in case of an emergency
A. Card should alert emergency personnel to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions
B. When traveling, a needle, syringe, & an injectable form of cortisol should be carried for emergencies
C. Pt should know how to increase medication during periods of stress or mild URI, etc
Define cushing's syndrome
1. Cluster of clinical abnormalities caused by excessive adrenocortical hormones
A. Primarily cortisol, but includes aldosterone & androgen
What is the most common cause of cushing's syndrome?
1. Cushing’s Disease accounts for 70% cases of Cushing’s Syndrome
A. ACTH excess
B. Most common cause is pituitary adenoma
What is the etiology of Cushing's syndrome?
1. Pituitary hypersecretion of ACTH
2. Ectopic ACTH secretion by tumor outside pituitary
3. Administration of steroids
A. Most common cause non-physiologically
4. Cancerous adrenal tumor
What does cortisol excess lead to?
1. Anti-inflammatory effects
2. Excessive catabolism of proteins & fat to support hepatic glucose production
3. Can be ACTH dependent or ACTH independent
What are the complications of Cushing's disease?
2. Peptic ulcer
3. Impaired Glucose Tolerance or DM
4. Ischemic HD
Where are the characteristic fat pads in Cushing's disease?
1. Above clavicles
2. Over upper back
A. Buffalo hump
3. On face
A. Moon facies
4. Truncal obesity
What are the sxs of Cushing's disease?
1. ↑ Risk of infection
A. Poor wound healing
2. ↓ Resistance to stress
4. Purple striae
5. Muscle weakness
A. Muscle atrophy arms & legs
6. Hirsutism – excess androgen production
What are the dx studies for Cushing's disease?
4. ↑ Serum & urinary free cortisol
5. ↑ Salivary cortisol
6. Dexamethasone suppression test
Screening test for Cushing’s Syndrome
7. Salivary cortisal assays
8. CRH stimulation test
What is the Screening test for Cushing’s Syndrome?
Dexamethasone suppression test
How is the dexamethasone test performed?
1. Give Dexamethasone 1mg PO @ 11pm & draw fasting serum cortisol level @ 8 am next day
A. Cortisol level <1.8mcg/dL excludes Cushing’s Syndrome
2. Several drugs can interfere w/ test
A. OCP, ASA, MSO4, Methadone, Lithium, MAOI, diuretics
3. If test normal, signs & sx’s suggest hypercortisolism, further testing done
What is a positive salivary cortisol assay for Cushing's disease?
1. Cortisol value > 2.0 ng/mL (5.5 nmol/L)
A. 100% sensitivity & 96% specificity for Dx Cushing's syndrome
2. Usually done on 2 consecutive days
What is a positive CRH stimulation test?
1. Distinguishes pituitary adenomas vs ectopic ACTH syndrome or cortisol-secreting adrenal tumors
2. Draw fasting ACTH & cortisol levels, then
IV bolus of CRH
3. 2-8 redraws @ 5, 10, 15, 30, 45, 60, 90, & 120 min. after CRH injection
What are the ACTH releasing tumors?
1. Benign carcinoid tumors of lung
2. Islet cell tumors of pancreas
3. Medullary carcinoma of thyroid
4. Small cell tumors of lung
5. Tumors of thymus gland
What is the treatment for cushing;s disease?
Resection of pituitary adenoma
What is the treatment of Adrenal tumors?
Surgery is the treatment for benign as well as cancerous tumors of the adrenal glands
What is the treatment for ectopic ACTH syndrome?
1. Eliminate all of the cancerous tissue that is secreting ACTH
E. Combination of above treatments
What is the prognosis for cushing's syndrome?
1. Cushing’s Syndrome post adrenalectomy (benign tumor):
A. 5-year survival of 95%
B. 10-year survival of 90%
2. Cushing’s Disease from pituitary adenoma is similar to above following successful surgery
3. Adrenocortical carcinoma stage I-II
A. 5-year survival of 60%, but mets develop in 40% w/in 2 yr
1. Catecholamine-secreting tumor that arises from chromaffin cells of the adrenal medulla & the sympathetic ganglia
2. ≈10% are malignant
What is the epidemiology of pheochromocytoma?
1.Affect ≈ 0.1–0.4% of the hypertensive population
2. Male = Female
3. Most noted in 4th or 5th decade
What is the classic triad of pheochromocytoma?
HA, sweating, tachycardia
What are the HTN characteristics for pheochromocytoma?
1. ≈ 50% sustained HTN
2. ≈ 50% paroxysmal HTN
3. Suspect in pt < 20 yrs w/ HTN
What other sxs are present for pheochromocytoma?
1. Pain (flank, abdominal, pain)
2. Anxiety or panic attacks
3. Asymptomatic incidental adrenal mass on imaging
4. Cardiac arrythmias
What dx studies are used for pheochromocytomas?
1. 24 hour urine catecholamines & metanephrine
2. Plasma fractionated metanephrines
3. Clonidine suppression test
4. CT or MRI of abd/pelvis
What is a positive 24 hour urine catecholamines & metanephrine test?
1. Norepi > 170 mcg/24 hr
2. Epi > 35 mcg/24 hr
3. Dopamine 700 mcg/24 hr
4. Normetanephrine > 900 mcg/24 hr
What are the characteristics of plasma fractionated metanephrines?
Not as specific, but sensitive
Higher rate of false (+)
Avoid acetaminophen for 48 hours prior
What is the clonidine suppression test?
1. Confirmatory test for false (+) of plasma metanephrines
2. (Normally suppresses release of catecholamines)
A. Clonidine 0.3 mg PO given
B. Plasma catecholamines & fractionated metanephrines measured before & at 3 hrs after dose
C. Plasma norepinephrine > 2000 pg/mL is diagnostic of pheochromocytoma
-Normal response < 500 pg/mL