Adrenal Disorders

Question 1

2 things that up regulate ACTH

Answer 1

CRH and ADH

Question 2

Sxs of glucocorticoid deficiency (9)

Answer 2

Weight loss, weakness, fatigue, anorexia, nausea, vomiting, abdominal pain, myalgias, arthralgias.

Question 3

Waterhouse-Friedrichson Syndrome
Characteristics
Cause

Answer 3

• Associated w/ hemorrhage in multiple organs, including adrenals. Lose glucocorticoids and mineralocorticoids → hypotensive shock.
• Typically the pathogen is the meningococcus Neisseria meningitidis.

Question 4

Most common cause of secondary adrenal insufficiency

Answer 4

Pituitary tumor

Question 5

Metabolic changes in Cushing’s (4)

Answer 5

Glucose intolerance / DM, dyslipidemia, HTN, increased mortality from CVD.

Question 6

2 causes of HTN in Cushing’s

Answer 6

Due to mineralocorticoid and glucocorticoid excess.

Question 7

In which cause of excess ACTH is hyper pigmentation most prominent?

Answer 7

Ectopic tumor

Question 8

Treating Cushing’s Disease (4)

Answer 8

• Transphenoidal surgery w/ resection of pituitary adenoma is treatment of choice
• Ketoconazole = adrenal steroid synthesis inhibitor. Used if surgery is not successful.
• If still not cured, to pituitary radiation
• Last resort is bilateral adrenalectomy

Question 9

Nelson’s Syndrome

Answer 9

Excessive growth of ACTH-secreting tumor after bilateral adrenalectomy → hyperpigmentation. Due to loss of negative feedback.

Question 10

Treating Cushing’s from adrenal tumor (2)

Answer 10

Cut out tumor

If still present, use adrenal enzyme inhibitors

Question 11

Treating ectopic ACTH tumor (3)

Answer 11

Cut out tumor
If still present, use adrenal enzyme inhibitors
Adrenalectomy is last resort

Question 12

Mechanism of glucocorticoid anti-inflammatory effects

Answer 12

• NFkB gene mediates immune function / inflammation.
• NFkB normally is bound to inhibitory protein IkB. After a pro-inflammatory signal, IkB becomes phosphorylated / degraded, allowing NFkB to enter nucleus and transcribe inflammatory genes.
• Glucocorticoids induce transcription of IkB → inhibition of NFkB.

Question 13

Spironolactone / eplerenone
Mechanism
Uses
Side effects

Answer 13

• Mechanism – competitive antagonists at MR
• Uses – primary hyperaldosteronism, HTN, and HF
• Side effects – hyperkalemia from both
• Spironolactone: weak progesterone effects may cause irregular menses in women.
• Eplerenone – no progesterone or anti-androgen effects.

Question 14

Pxs at risk of adrenal hemorrhage (3)

Answer 14

Neonates, pxs on anticoagulant therapy, or in adults w/ DIC

Question 15

Rule of 10’s (5 things)

Which disease?

Answer 15

• Pheochromocytoma
• 10% are extra-adrenal. Called paragangliomas. Mainly occur in other areas derived from neural crest, such as organs of Zuckerkandl and carotid body.
• 10% of sporadic cases are bilateral (50% of familial cases are bilateral).
• 10% are malignant
• 10% are NOT associated w/ HTN
• Exception: 25% have germline mutation (familial)

Question 16

3 familial syndromes associated w/ pheochromocytoma
Which genes?
Characteristics

Answer 16

• MEN 2a / 2b – RET gene mutation
• Neurofibromatosis type 1 – NF1 gene mutation. Characterized by pheochromocytoma, neurofibromatosis, café-au-lait spots, and optic nerve gliomas.
• Von Hippel-Lindau Syndrome – VHL gene mutation. Characterized by pheochromocytoma, renal cell carcinoma, hemangioblastoma, and pancreatic endocrine neoplasms.