Treatment
IV hydrocortisone 50mg q6hrs or 100mg q8hrs
What is the dose of glucocorticoids for septic shock?
What are the mechanisms of relative adrenal insufficiency in critical illness?
High dose ACTH stimulation test (250 micrograms)
Which is the standardized test used in the diagnosis of CIRCI in adults?
Which is a more sensitive test?
Vitiligo or hyperpigmentation
Which is the sign only seen in primary adrenal insufficiency and not secondary AI?
What are the other clinical manifestations of chronic PAI?
Hyponatremia, hyperkalemia, anemia, eosinophilia, hypercalcemia, lymphocytosis, azotemia
What are the biochemical abnormalities noted on labs in primary adrenal insufficiency?
Medical alert bracelet
Which is an important entity to be worn at all times by persons with PAI?
What else is important to keep on hand?
1 to 3L of normal saline bolus with IV Hydrocortisone 100mg bolus within the first hour followed by 50mg q6 hrs. Post 24hrs oral hydrocortisone at 2-3 times the usual dose with tapering to the usual dose in 2-3 days
What is the treatment of acute adrenal crisis?
What are the clinical manifestations of adrenal crisis?
Systolic blood pressure <90 mmHg for more than one hour following both adequate fluid resuscitation and vasopressor administration (Need for dopamine at >15 mcg/kg/min, or norepinephrine or epinephrine at >0.25 mcg/kg/min to maintain mean BP at >60 mm Hg (80 mm Hg if previous hypertension).
What is the definition of refractory shock/indication for use of glucocorticoids in septic shock?
Gastroenteritis
What is the most common cause of adrenal crisis?
Which are the other precipitants of adrenal crisis?
AM cortisol, ACTH, renin, aldosterone, 21 hydroxylase antibodies
Which are the initial lab tests that must be done to determine the cause of PAI?
HITT
Which hematologic condition is associated with adrenal hemorrhage?
Which is the other hematologic condition associated with adrenal hemorrhage?
Hydrocortisone (10mg in the morning, 5mg in the afternoon) + Fludrocortisone 0.1mg +/- DHEA 25mg
What is the typical regimen to treat patients with chronic primary adrenal insufficiency?
Change in baseline cortisol at 60 min of <9 mcg/dL after cosyntropin (250 mcg; ie, high dose ACTH stimulation) administration OR random plasma cortisol of <10 mcg/dL
How is CIRCI diagnosed by current guidelines?
Premature ovarian failure
Which is the most common autoimmune disease seen in association with Addison's disease?
21 hydroxylase deficiency
Which is the enzymatic defect in non-classical/late-onset congenital adrenal hyperplasia?
Elevated very long-chain fatty acids
Which is the finding on labs suggestive of X-linked adrenoleukodystrophy and adrenomyeloneuropathy?
Minor: 2 to 3 times the dose of hydrocortisone orally for 2 to 3 days, Moderate: 25 to 75mg/day orally or IV for 1 to 2 days, Severe: 100mg IV followed by 50mg q6hrs with rapid tapering and switch to an oral regimen
What are the sick day and surgical stress rules for patients with chronic primary adrenal insufficiency?
Acute deterioration in health status associated with absolute hypotension (systolic blood pressure <100 mm Hg) or relative hypotension (systolic blood pressure ≥20 mm Hg lower than usual), with features that resolve within 1 to 2 hours after parenteral glucocorticoid administration.
How is adrenal crisis defined?
What are the risk factors for the development of adrenal crisis?
Schmidt's syndrome
What is another name for autoimmune polyglandular syndrome type 2?
Which are the disease entities associated with APS II?
Cholesterol desmolase
Which is the enzyme blocked by ketoconazole and heparin?
OCPs, cyproterone, spironolactone. If fail-then glucocorticoids
What is the treatment for non-classical/late-onset congenital adrenal hyperplasia?
IV Methylprednisolone in a dose of 1 mg/kg/day followed by slow tapering over 2 weeks
What is the dose of glucocorticoids in patients with early ARDS (up to day 7 of onset; PaO2/FiO2 of <200)?
What is the dose of glucocorticoids for late persistent ARDS (after day 6 of onset)?
Basal 17-hydroxyprogesterone value greater than 200 ng/dL and confirmed with an ACTH stimulation test serum 17-hydroxyprogesterone value exceeding 1500 ng/dL. (Rarely, stimulated values at 60 minutes in affected patients the range between 1000 ng/dL and 1500 ng/dL the diagnosis should be confirmed with genotyping of the CYP21A2 gene.
How do you diagnose late-onset/non-classical congenital adrenal hyperplasia?
Pseudomonas aeruginosa
Which is the most common organism implicated in causing Waterhouse-Friderichsen syndrome in adults?
Which are the other infectious causes of PAI?
Autoimmune regulator (AIRE) gene
What is the genetic mutation seen in autoimmune polyglandular syndrome type 1 patients?
What are the clinical manifestations of APS I?
Phenylethanolamine N-Methyltransferase
Which is the enzyme which is facilitated by cortisol to convert endogenous norepinephrine to epinephrine?
Which are the other actions permissive actions of cortisol?