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CC Jan 2017 Primary Adrenal Insufficiency

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echoecho's version from 2017-03-12 23:32

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Question Answer
_____ disease, or primary adrenal insufficiency, was first described by Dr. Thomas _______ in the middle of the 19th century in 6 individuals with TB of the adrenal glands?Addison's'; Addison
It is an uncommon condition, with an estimated incidence of ____ to ____ per 10,000 people in the developed world?0.9; 1.4
Secondary adrenal insufficiency is most commonly held seen in clinical practice secondary to suppression of the ______ axis by exogenous ______?1) hypothalamic-pituitary. 2) corticosteroids
*** Due to the ____ and non-specific presentation, many have patients with Addison's disease are not diagnosed until they have had symptoms for many years?Slow
The usual etiology of primary Addison's disease in developed countries (80-90%) is ______ destruction of the adrenal gland, with 40% involving the _____ gland only and 60% involving an _____ _____ syndrome?1) autoimmune. 2) adrenal 3) autoimmune polyendocrine syndrome (APS)
List the several forms of autoimmune polyendocrine syndrome (APS)?include adrenal insufficiency associated with disorders of the 1) parathyroids 2)thyroid 3) gonads 4) pancreas 5)skin 6)GI abnormalities 7) neurologic abnormalities
List the less common causes of adrenal gland destruction?1) infections (TB, HIV, fungal). 2) infiltration (adrenal metastases, primary adrenal lymphoma, sarcoidosis, amylodosis, hemochromatosis. 3) thrombosis. 4) multiple congenital disorder 5) and least commonly, adrenal dysgenesis
Addison's disease results in a decreased level of glucocorticoids, such as _______ and sometimes mineralocorticoids, like ______?1) cortisol 2) aldosterone
memorize
Is mineralocorticoids deficiency seen in secondary or tertiary adrenal insufficiency (dysfunction at the level of the hypothalamus)
Question Answer
Symptoms and signs of Addison's disease associated with the specific pathophysiology and whether it is only in primary adrenal insufficiency or not as well as the frequency (%)?1) fatigue/weakness (glucocorticoid deficiency, adrenal androgen deficiency), not only in primary adrenal insufficiency, 100% frequency. 2) lightheadedness/dizziness (mineralocorticoids deficiency, glucocorticoid deficiency), not only in primary adrenal insufficiency, 12% frequency. 3) salt craving (mineralocorticoids deficiency that results in sodium wasting), only in primary adrenal insufficiency, 16% frequency. 4) anorexia (glucocorticoid deficiency), not only in primary adrenal insufficiency, 100% frequency. 5) gastric pain, nausea, vomiting, diarrhea (glucocorticoid deficiency, mineralocorticoids deficiency), more frequent in primary adrenal insufficiency, 92% frequency. 6) myalgias (glucorticoid deficiency), not only in primary adrenal insufficiency, 6-13% frequency. 7)dry, itchy skin (adrenal androgen deficiency), not only in primary adrenal insufficiency 8) decreased libido (adrenal androgen deficiency), not only in primary adrenal insufficiency 9) weight loss/failure to thrive (glucocorticoid deficiency), not only in primary adrenal insufficiency, 100% frequency 10) low blood pressure (mineralocorticoids deficiency, glucocorticoid deficiency), not only in primary adrenal insufficiency, 88-94% frequency. 11) postural hypotension (mineralocorticoids deficiency, glucocorticoid deficiency), more pronounced in primary adrenal insufficiency. 12) raised serum creatinine (mineralocorticoids deficiency), only in primary adrenal insufficiency 13) Hyponatremia (mineralocorticoids deficiency, glucocorticoid deficiency (leading to SIADH), not only in primary adrenal insufficiency, 88% frequency 14) hyperkalemia (mineralocorticoids deficiency), only in primary adrenal insufficiency, 64% frequency 15) anemia/eosinophilia (glucocorticoid deficiency), not only in primary adrenal insufficiency, 40%/14% frequency. 16) azotemia, not only in primary adrenal insufficiency, 55% frequency 17) hyper pigmentation (palmar crease, recent scars, vermillion border of the lips (overproduction of ACTH (adrenocorticotropic hormone) due to the unresponsive adrenal gland, which is broken down into melanocytes-stimulating hormone), not only in primary adrenal insufficiency, 94% frequency 18)increased TSH (glucocorticoid deficiency OR autoimmune thyroid failure), only in primary adrenal insufficiency. 19) hypercalcemis (glucocorticoid deficiency - especially with hyperthyroidism), only in primary adrenal insufficiency, 6% frequency. 20) hypoglycemia (glucocorticoid deficiency), not only in primary adrenal insufficiency. 21) loss of auxiliary or pubic hair (women), absence of adrenal he/pubarche (adrenal androgen deficiency, not only in primary adrenal insufficiency 21) vitiligo, not only in primary adrenal insufficiency, 10-20% frequency
Is abnormal magnesium levels associated with adrenal insufficiency?no
The clinical diagnosis of primary adrenal insufficiency consists of demonstrating an inappropriately low early morning serum cortisol concentration (< ____ mcg/dL ). This is strongly suggestive of adrenal insufficiency though by the time the patient has developed low serum cortisol concentrations in primary adrenal insufficiency, adrenal destruction is essentially _______?< 3 mcg/dL; complete
The clinic diagnosis of primary adrenal insufficiency consists of measuring a serum early morning ACTH (adrenocorticotropic hormone). If the morning ACTH is _____ and the morning cortisol is low, then the patient has Addison's disease. Due to a larger normal range, afternoon serum cortisol levels are NOT helpful in diagnosis?high
The clinical diagnosis of primary adrenal insufficiency consists of measure meant of the response of serum cortisol to ACTH ______. In primary adrenal insufficiency, there is little, if any increase in _____ production. Cortisol production will increase in secondary and tertiary adrenal insufficiency?stimulation; low
List the factors that are important in the evaluation of patients with primary adrenal insufficiency?1) patient's age and gender. 2) clinical hx and identification of s/s of autoimmune-mediated endocrine disorders
In patients thought to have autoimmune primary adrenal insufficiency, the presence of other endocrine gland involvement can be determined by measuring serum?1) calcium, 2) phosphorus. 3) glucose. 4) thyrotoxicosis
What serum hormone should be measured if the patient is hypocalcemic?PTH
The possibility of _______ should be investigated in women with oligomenorrhea or amenorrhea by measure ___ and ____ and in possibly hypogonadal men by measuring serum _____ and _____?1) FSH and LH. 2) testosterone and LH
Abdominal CT, while not used to dx Addison's disease, can be helpful, how?1) detecting enlarged adrenal glands or adrenal calcification. 2) 4/o autoimmune disease by suggesting an infectious, hemorrhagic or metastatic cause
Measurement of ______ can be helpful for differentiating primary from secondary adrenal insufficiency but it is not a primary test?aldosterone
Treatment of Addison's disease includes lifelong replacement of the deficient hormones, usually in the form of ______, give examples of brand names?1) hydrocortisone (A-Hydrocortisone, Cortef, Solu-CORTEF)
Treatment of Addison's disease includes prednisone tablets for ______ replacement?cortisol
What can be used for aldosterone replacement?fludrocortisone (Florinef)
Give the dose of glucocorticoid of choice for the management of CHRONIC primary adrenal insufficiency?short-acting glucocorticoid such as hydrocortisone (15-025 mg daily in adults) in 2 or 3 divided doses
One should use the ______ glucocorticoid dose that relieves s/s of glucocorticoid deficiency?lowest
Short-acting multi-dose regimens roughly mimic the normal _____ rhythm?diurnal
What two oral medications are longer acting and are an option, especially for patients who have difficulty with dosing multiple times per day?prednisone or Dexamethadone
Most patients with primary adrenal insufficiency eventually require _________ replacement to prevent sodium loss, intravascular volume depletion and hyperkalemia?mineralocorticoids
Give a name and dose of a mineralocorticoids that can be used as the usual dose? What lower dose may be sufficient in patients receiving hydrocortisone, which has some mineralocorticoids activity?1) Fludrocortisone is given orally in a usual dose of 0.1 mg/day. 2) 0.05 mg/day
Higher doses are required during _____ _____ and______?acute illness; surgery
During minor illness, the patient can increase the dose of glucocorticoids to ____ to ____ times the usual daily dose for 3 days w/o consulting a clinician (known as the ___ X ___ rule)?1) two to three times the usual dose 2) 3x3 rule
The increased dose will decrease ____ and _____ and will not compromise the immune process?fever, malaise
The appropriate dose and timing of glucocorticoids for patients undergoing surgery is _______?controversial
Current recommendations for glucocorticoid supplementation at surgery take into account the ______ of the operation? The doses can range from ____ mg to ___mg per day?1) severity. 2) 25mg to 150 mg
If nausea and vomiting preclude oral administration, then _____ replacement must be used?parenteral
*** SUMMARY = Adrenal insufficiency can be primary (at the level of the _____ gland), secondary (at the level of the _____-____ axis) or tertiary (at the level of the _______)?1) adrenal. 2) hypothalamic-pituitary 3) hypothalamus
*** SUMMARY = Most primary adrenal insufficiency seen in the US is ______ in etiology?autoimmune
*** SUMMARY = What are the initial 2 tests that are drawn in the morning?1) cortisol. 2) ACTH (adrenocorticotropic hormone)
***SUMMARY = What test will help define whether the insufficiency is primary?ACTH stimulation test
*** SUMMARY = Treatment involves administration of?glucocorticoids and, in some patients, mineralocorticoids as well
memorize