Endo 7 - Hypoadrenal disorders

Question 1

What is the ultimate precursor for all steroids?

Answer 1

Cholesterol

Question 2

Outermost to innermost, what are the layers of the adrenal cortex and what do they produce?

Answer 2

Outer = z. glomerulosa = mineralocorticoid (aldosterone)

Middle = z. fasciculata = glucocorticoid

Inner = z. reticular = estradiol

Question 3

What are the causes of adrenocortical failure?

Answer 3

1. TB - tuberculous Addisons disease (commonest worldwide)
2. Autoimmune Addisons disease (commonest UK)
3. Congenital adrenal hyperplasia

Question 4

What are the symptoms of Addisons disease?

Answer 4

1. Tired, exhaustion
2. Pigmentation
3. Hypotension

Question 5

What are the consequences of adrenocortical failure?

Answer 5

1. Fall in BP
2. Loss of salt in urine
3. Increased plasma potassium
4. Fall in glucose due to glucocorticoid deficiency
5. High ACTH - increased pigmentation because ACTH also stimulates MSH

Question 6

What pro hormone is broken into MSH and ACTH

Answer 6

POMC

Question 7

What are some tests and results that indicate Addisons disease?

Answer 7

9AM cortisol - LOW

ACTH - HIGH

Question 8

What is a short synACTHen test

Answer 8

250 micrograms of synacthen given IM

If cortisol levels rise to normal, then they don’t have Addisons

Question 9

What is the commonest cause of Congenital adrenal hyperplasia?

Answer 9

21-hydroxylase deficiency

can be complete/partial

Question 10

What hormones are deficient in complete congenital adrenal hyperplasia?

Answer 10

Aldosterone and cortisol

less than 24 hrs survival

Question 11

Which hormones are in excess in complete congenial adrenal hyperplasia

Answer 11

Sex steroids and testosterone particularly

Question 12

Describe the presentation of congenital adrenal hyperplasia

Answer 12

1. presents as a neonate with a “salt losing Addisonian crisis”
2. Girls may have ambiguous genitalia (civilised by adrenal testosterone)

Question 13

Which hormones are deficient in partial congenital adrenal hyperplasia?

Answer 13

Aldosterone and cortisol

Question 14

Which hormones are in excess in partial congenital adrenal hyperplasia?

Answer 14

Sex steroids and testosterone

Question 15

What is the main problem of partial 21 hydroxylase deficiency?

Answer 15

In later life - hirsutism and virilisation in girls

Precocious puberty in boys die to adrenal testosterone

Question 16

Deficiency of 11 hydroxylase enzyme can cause what?

Answer 16

Excess deoxycorticosterone - which behaves like aldosterone

Can cause hypertension and hypokalaemia

Question 17

Which hormones are deficient in 11-hydroxylase deficiency?

Answer 17

Cortisol and aldosterone

Question 18

Which hormones are in excess in 11-hydroxylase deficiency?

Answer 18

Sex steroids and testosterone and 11-deoxycorticosterone

Question 19

What are the problems with 11-hydroxylase deficiency?

Answer 19

Virilisation, hypertension, hypokalaemia

Question 20

Which 3 genetic causes can cause congenital adrenal hyperplasia

Answer 20

21 hydroxylase deficiency, 11 hydroxylase deficiency, 17 hydroxylase deficiency

Question 21

Which hormones are in excess in 17 hydroxylase deficiency?

Answer 21

Cortisol and sex steroids

Question 22

Which hormones are in excess in 17 hydroxylase deficiency?

Answer 22

11-deoxycorticosterone and aldosterone

Question 23

What are problems associated with 17 hydroxylase deficiency?

Answer 23

Hypertension, low K, no puberty/sex steroids, glucocorticoid deficiency