Adrenal Cortex (continued)

Question 1

What is the primary test for primary vs. secondary hypocortisolism?

Answer 1

ACTH stimulation test
cortisol levels increase: normal
cortisol levels increase only slightly: hypoaldosteronism

Increase in primary hypocortisiolism (Addison’s ); inappropriately low in secondary hypocortisolism

Question 2

Understand this chart.

Answer 2

Understand chart

Question 3

Another name for primary hyperaldosteronism?

Answer 3

Conn’s Syndrome

Question 4

What is Conn’s syndrome caused by?

Answer 4

a small unilateral adenoma on either side (most common);

bilateral adrenal hyperplasia (idiopathic hyperaldosteronism;

or adrenal carcinoma (rare)

Question 5

What is an initial clue for presence of Conn’s syndrome?

Answer 5

detection of hypertension with hypokalema

Question 6

What are some clinical features of primary hyperaldosteronism?

Answer 6

• increased whole body sodium, fluid, and circulating blood volume
• infrequent hypernatremia
• increased peripheral vasconstriction and TPR
• BP (from borderline to severe HTN)
• edema (rare) (sodium escape, i.e a major increase in sodium and water retention is prevented by “sodium escape” but mechanism is unclear)
• models left ventricular hypertrophy
• potassium depletion and hypokalemia (causing weakness and fatigue)
• Increased hydrogen ion excretion and new bicarbonate create metabolic alkalosis
• Positive Chvostek or Trousseau’s sign (suggestive of alkalosis leading to low Ca2+)
• Normal cortisol level
• suppressed renin (major feature)

Question 7

What is secondary hyperaldosteronism?

Answer 7

refers to a state in which there is an appropriate increase in aldosterone in response to activation of RAAS

Question 8

What are the 2 types of secondary hyper-aldosteronism?

Answer 8

secondary hyper-aldosteronism with HTN
secondary hyper-aldosteronism if hypotension

Question 9

Major cause of secondary hyperaldosteronism with HTN?

Answer 9

primary over secretion of renin, secondary to a decrease in renal blood flow and/or pressure( most cases)

renin secreting tumor (rare

renal arterial stenosis, narrowing via atherosclerosis, fibromuscular hyperplasia

hypokalemia and metabolic alkalosis

modest to high elevated renin and aldosterone

Question 10

What are some DDs to remember before diagnosing someone of secondary hyperaldosteronism with HTN?

Answer 10

Hypokalemia in a hypertensive patient not taking diuretics

hypo-secretion of renin with elevated aldosterone that fails to respond to a volume contraction: Conn’s syndrome

Hyper-secretion of renin with elevated aldosterone: renal vascular

Question 11

What are the features of secondary hyper-aldosteronism with hypotension.

Answer 11

• sequestration of blood on the venous side of hte systemic circulation (common cause)’
• results in dec. CO and thus dec. BF and pressure to the renal artery
• the following conditions produce:
  CHF
  Constriction of vena cava
  hepatic cirrhosis

Question 12

What are “inborn errors of metabolism”?

Answer 12

When there are single enzyme defects that lead to defiencient cortisol secretion and the syndrome called CAH.

Question 13

What is the most common of the congenital enzyme deficiencies?

Answer 13

21 B-hydroxylase deficiency

Question 14

What is corticosterone?

Answer 14

a weak glucocorticoid

Question 15

What are the consequences of 21 B-hydroxylase deficiency?

Answer 15

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis;= hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the female fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty, growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 16

What is treatment for 21 B-hydroxylase defiency?

Answer 16

bringing glucocorticoid and mineralocorticoid back to normal range which also suppresses adrenal androgen secretion; in addition, hydrocortisone to act as feedback inhibition on pituitary

Question 17

What are the consequences of 11 B- hydroxylase deficiency?

Answer 17

clinical features of increased androgens similar to the preceding form including virilization of female fetus

Principal difference with this form is the hypertension produced by 11-deoxycortiocsterone, along with hypokalemia and suprressed renin secretion

Question 18

Treatmetn for 11 B- hydroxylase deficiency?

Answer 18

For all forms of CAH is gluocorticoids such as hydrocortisone and dexamethasone

Question 19

What are the clinical features of 17 a hydroxylase deficiency? (excluding testes and ovaries)

Answer 19

zona fasciculata, reticularis: decreased adrenal androgens, decreased cortisol, and increased ACTH.

increased 11-dexoycorticosterone leading to HTN

reduced circulating angiotensin II reduces stimulation os zona glomerulosa and aldosterone secretion

Question 20

What are the consequences of 21 B-hydroxylase deficiency?

Answer 20

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 21

Be able to make a chart on the effects of 21 B-OH, 11 B OH, and 17 a-OH on glucocorticoids, ACTH, BP, Mineralocorticoid Aldo DOC, Androgen, Estrogen?

Answer 21

Reproduce chart.

Question 22

What are the consequences of 21 B-hydroxylase deficiency?

Answer 22

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 23

What are the consequences of 21 B-hydroxylase deficiency?

Answer 23

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 24

What are the consequences of 21 B-hydroxylase deficiency?

Answer 24

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 25

What are the consequences of 21 B-hydroxylase deficiency?

Answer 25

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 26

What are the consequences of 21 B-hydroxylase deficiency?

Answer 26

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 27

What are the consequences of 21 B-hydroxylase deficiency?

Answer 27

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 28

What are the consequences of 21 B-hydroxylase deficiency?

Answer 28

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 29

What are the consequences of 21 B-hydroxylase deficiency?

Answer 29

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 30

What are the consequences of 21 B-hydroxylase deficiency?

Answer 30

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 31

What are the consequences of 21 B-hydroxylase deficiency?

Answer 31

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 32

What are the consequences of 21 B-hydroxylase deficiency?

Answer 32

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 33

What are the consequences of 21 B-hydroxylase deficiency?

Answer 33

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 34

What are the consequences of 21 B-hydroxylase deficiency?

Answer 34

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height

Question 35

What are the consequences of 21 B-hydroxylase deficiency?

Answer 35

mineralocorticoid deficiency in 75% cases

neonates may present with salt-wasting crisis; salt wasters tend to have hyponatremia, hyperkalemia, and raised plasma renin

elevated 17 hydroxyprogesterone

increased androgens, leading to virilization of the femal fetus and sexual ambiguity at birth

males are phenotypically normal at birth but develop precocious pseudopuberty and growth acceleration, premature epiphyeseal plate closure and diminished final height