Adrenal insufficiency

Question 1

What is primary adrenal insufficiency?

Answer 1

Destruction of the Adrenal leading to lack of Cortisol, Aldosterone, DHEAS.

Question 2

Signs and symptoms of primary adrenal insufficiency as correlated to the hormone lacking

Answer 2

Decreased Cortisol: Hypoglycemia, Confusion, Weakness, Fatigue. Decreased Aldosterone: Hyponatremia & Hyperkalemia, Dehydration, Hypotension, Salt craving, Weakness, Abdominal pain, nausea, decreased appetite. Decreased DHEAS: Fatigue

Question 3

General symptoms (repetitive) of adrenal insufficiency

Answer 3

Salt cravings “I love to drink the juice around the pickles”, Fatigue, Muscle weaknesss, Dizzyness, Nausea, Vomiting, Abdominal pain, “Sense of impending doom, feeling down”, Weight loss

Question 4

General signs of symptoms of adrenal insufficiency

Answer 4

Postural drop in blood pressure, Postural increase in pulse, Weight loss, Abdominal tenderness, Muscle weakness, Hyperpigmentation of gum lines, palmar creases, and scars (Primary AI); Vitiligo (Primary AI)

Question 5

Causes of primary adrenal insufficiency

Answer 5

Autoimmune: Poly Glandular Autoimmune I and II. Infections: TB, histoplasmosis, coccidiomycosis, cryptococcus, HIV, CMV, MAI, toxoplasma, Syphilis Metastatic Disease (lung, breast, stomach and colon cancer, lymphoma). Adrenal Hemorrhage (Waterhouse-Friderichsen Syndrome, Lupus anticoagulant, Anticoagulation). Drugs (interfere with cortisol synth): Metyrapone, Aminoglutethimide, Ketoconazole, Rifampin, Barbiturates, Phenytoin

Question 6

Familial causes of adrenal insufficiency - most common in peds

Answer 6

Congenital Adrenal Hyperplasia, Adrenoleukodystrophy, Adrenomyeloneuropathy, Familial glucocorticoid deficiency, Familial glucocorticoid resistance, Defective cholesterol metabolism

Question 7

APS Type 1

Answer 7

Single gene disorder. Aire is a TF in thymus, defect causes failure to delete auto reactive T cells - leads to autoimmunity. Associated with Hypoparathryoidism, Chronic Mucocutaneous Candidiasis (oral thrush), Adrenal Insufficiency

Question 8

APS Type 2

Answer 8

More common, linked to HLA region of chromo 6. Associated with diabetes, addison’s (100%) and thyroid autoimmunity

Question 9

What is Secondary adrenal insufficiency?

Answer 9

Same thing really. Caused by Prior history of steroid use - can be irreversible (need to taper off) - hypo and pituitary are impaired. Also caused by Pituitary Disease / Hypothalamic Disease, Isolated ACTH Deficiency, post transphenoidal pituitary surgery, Peripheral Resistance to glucocorticoids. Secondary is more common that primary insufficiency

Question 10

How do we test for adrenal insufficiency?

Answer 10

Best is ACTH stimulation. baseline cortisol, ACTH - ACTH 250 ug IV push - cortisol at 1 hr, should double from its baseline in 1 hour and should be at least 550 nmol/L.

Question 11

Primary vs secondary adrenal insufficiency

Answer 11

1 is low ACTH and low cortisol, low Na, high K, low glucose, hyperpigmentation. 2 is high ACTH and low cortisol, low Na (due to low cortisol, although aldosterone still there), normal K (aldosterone is stimulated by renin pathway), low glucose.

Question 12

Adrenal crisis - what is it?

Answer 12

Weight Loss, Fatigue, Weakness, Severe Hypotension, Abdominal Symptoms

Question 13

Tx for adrenal crisis

Answer 13

IV fluids, IV steroids, treat whatever precipitated it.

Question 14

Long term management of addison’s

Answer 14

Medic alert bracelet. Glucocorticoids (prednisone, hydrocortisone, cortisone Acetate, Dexamethasone), Mineralocorticoids – Florinef (for 1 insufficiency). Pt needs to double the glucocorticoids during minor illness or stress (to make it like a normal response), IV steroids for major illness/surgery/hospitalization.

Question 15

Complications of using glucocorticoids

Answer 15

Adrenal Suppression, Avascular Necrosis of the Hip, Cushing’s Syndrome, Diabetes, Hypertension, Obesity, Hypertriglyceridemia, Osteoporosis