SFP: adrenal pathology II Flashcards
What is adrenogenital syndrome?
Adrenal cortex secretes excess DHEA and androstenedione, both of which will be converted to testosterone in peripheral tissues.
What are the main causes of androgen excess?
Adrenal carcinoma or congenital adrenal hyperplasia.
What is congenital adrenal hyperplasia?
Inherited metabolic errors leading to a deficiency or absence of enzymes involved in corticosteroid synthesis.
In congenital adrenal hyperplasia, what happens to steroidogenesis?
It is shunted away from glucocorticoid production; this often leads to excess androgen to use up excess cholesterol.
What enzymes are typically impacted in congenital adrenal hyperplasia?
21 hydroxylase (most common) or 11 hydroxylase.
What causes hyperplasia in congenital adrenal hyperplasia?
Lack of glucocorticoid production ramps up ACTH, causing the adrenal hyperplasia.
What is the life-threatening form of congenital adrenal hyperplasia?
The salt wasting form aka no secretion of aldosterone. This means we cannot properly control our BP.
What is the clinical presentation of congenital adrenal hyperplasia?
Salt wasting, hyponatremia/hyperkalemia, hypotension, acidosis, excess androgen production, virilization.
How do we treat congenital adrenal hyperplasia?
Exogenous glucocorticoids/mineralocorticoids (in salt wasting form).
What may cause acute adrenocortical insufficiency?
Superimposed on chronic insufficiency, continued exogenous steroid use/rapid withdrawal, adrenal parenchymal hemorrhage.
Describe massive adrenal parenchymal hemorrhage.
Often caused by anticoagulants, patients with DIC, pregnancy, or bacteremia/sepsis.
What is waterhouse-friderichsen syndrome?
An overwhelming bacterial infection often due to Neisseria meningitis causes adrenal gland wipeout, rapid hypotension, DIC, and septic shock.
What is Addison’s disease?
A chronic form of adrenal insufficiency, often associated with autoimmune causes.
Describe the onset of Addison’s disease.
Often insidious; symptoms don’t appear until 90% of the cortex is impacted.
Describe autoimmune adrenalitis.
Autoimmune destruction of steroidogenic cells/antibodies to steroidogenic enzymes in the adrenal gland; huge cause of Addison’s disease.
What gene mutation is associated with autoimmune Addison’s disease?
AIRE (autoimmune regulatory gene).
What are infectious causes of Addison’s disease?
TB, fungi, and CMV or mycobacterium avium associated with HIV.
What tumors tend to cause Addison’s disease?
Metastatic tumors, often from lung and breast cancers.
What do adrenal glands look like in autoimmune Addison’s disease?
Atrophic gland.
What do adrenal glands look like in infectious TB Addison’s disease?
Granulomatous inflammation.
What is the clinical presentation of Addison’s disease?
Hyperpigmentation of the skin (from excess ACTH production), GI issues, low mineralocorticoid activity, and hypoglycemia (from glucocorticoid deficiency).
What is secondary adrenocortical insufficiency?
Any disorder of the hypothalamus or pituitary that reduces ACTH output and impact adrenal functioning.
What can prolonged administration of exogenous glucocorticoids do to the adrenal glands?
Cause secondary insufficiency! Long-term use suppresses ACTH secretion that may lead to adrenal dysfunction over time.
Describe response to exogenous ACTH in primary and secondary adrenocortical insufficiency.
In primary, the plasma cortisol won’t increase. This is because the cortex of the gland is shot, so the exogenous ACTH still is not able to stimulate much. In secondary, there will be a quick increase in cortisol. This is because the cortex is ok and finally has the ACTH stimulus it needs to function properly.
