adrenal pathway dysfunction Flashcards

1
Q

What biochemical molecule are steroid hormones made from?

A

cholesterol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

which steroid hormones are produced in the adrenal cortex?

A
  1. mineralocorticoids (aldosterone)
  2. Glucocorticoids (cortisol)
  3. Sex steroids (androgens, oestrogens)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Angiotensin 2 activates which enzymes and produces which hormone?

A
  • side chain cleavage
  • 3 hydroxysteroid dehydrogenase
  1. 21 hydroxylase
  2. 11 hydroxylase
  3. 18 hydroxylase

aldosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

ACTH activates which enzymes in the adrenal, producing which hormone?

A

cortisol

  • side-chain cleavage
  • 3 hydroxysteroid dehydrogenase
  1. 17 hydroxylase
  2. 21 hydroxylase
  3. 11 hydroxylase
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

what does aldosterone do?

A

stimulates sodium reabsorption in distal convoluted tubule and collecting duct, stimulates potassium and H+ secretion into urine

causes water to follow sodium back into blood by osmosis, increasing blood volume

therefore

increases blood pressure, sodium and lowers potassium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Addison’s disease is what kind of adrenal failure?

A

primary adrenal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is the most common cause of addison’s disease in the UK?

A

Autoimmune destroying adrenals

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the most common cause of Addison’s disease worldwide?

A

tuberculosis of adrenal glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

These are a symptom of what disease:

  • hyperpigmentation
  • low BP
  • weakness
  • weight loss
  • nausea and GI problems
  • vitiligo
A

Addison’s disease (primary adrenal failure)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Why is there increased pigmentation in Addison’s disease?

A

Lack of negative feedback of cortisol to the anterior pituitary, driving ACTH upwards.

pro-opio melanocortin is the precursor of ACTH and is broken down to ACTH and MSH (melanocyte-stimulating hormone)

Therefore increased production of ACTH also results in equally increased production of MSH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Why do people with addison’s disease have low blood pressure?

A

no cortisol or aldosterone

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what are the consequences of adrenocortical failure?

A
  • fall in BP
  • loss of salt in urine
  • increased plasma potassium
  • fall in glucose due to glucocorticoid deficiency
  • eventual death due to hypotension if untreated
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the test for Addison’s?

A
  1. 9am cortisol
  2. Gie 250micrograms synacthen (synthetic ACTH) and measure cortisol response - will be low-zero
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What will be the levels of ACTH and cortisol in the 9am cortisol test in Addison’s?

A

low cortisol

high ACTH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

why do we give fludrocortisone instead of aldosterone when treating Addison’s disease?

A

fludrocortisone has a long enough half-life to be given once daily

17
Q

what are the drugs that can be given for Addison’s disease and why?

A
  1. Fludrocortisone to replace aldosterone
  2. Prednisolone once daily or hydrocortisone 3x daily
18
Q

What does of fludrocortisone should be given once daily?

19
Q

Which drug: prednisolone or hydrocortisone, has a suitable half-life to mimic cortisol?

A

prednisolone

20
Q

What dose should hydrocortisone be given at, and why?

A

3x daily 10 + 5 + 2.5mg to mimic diurnal rhythm

21
Q

What does of prednisolone should be given?

22
Q

What is the downside of using hydrocortisone 3x daily?

A

produces peaks which are unnatural and can affect metabolism and sleep

23
Q

Congenital 21 hydroxylase deficiency causes an increase in which hormones?

A

sex steroids

24
Q

why do people with congenital 21 hydroxylase deficiency have adrenal hyperplasia?

A

large amount of ACTH produced to try and stimulate hormone release

25
what sexual/reproductive implications does 21 hydroxylase deficiency/congenital adrenal hyperplasia cause in girls?
* cliteromegaly * labial fusion * ambiguous genitalia
26
Which hormones are absent in 21 hydroxylase deficiency?
aldosterone and cortisol
27
what happens after birth in a baby with undiagnosed 21 hydroxylase deficiency?
salt losing addisonian crisis
28
why does a baby with 21 hydroxylase deficiency not have problems while in utero?
foetus gets steroids across the placenta
29
what problems arise later in life with partial 21 hydroxylase deficiency in girls and in boys?
girls - hirsutism, virilisation boys - early puberty due to adrenal testosterone
30
11 hydroxylase deficiency causes excess production of what? Why?
11 deoxycortisone because no cortisol or aldosterone are made due to the deficiency, so instead their precursors are made in excess, and the precursor of aldosterone, 11 deoxycortisone, has a similar effect to aldosterone
31
Excess deoxycortisone and sex steroids/testosterone is caused by? What 3 things does it cause, when in excess?
11 hydroxylase deficiency hypertension, hypokalaemia, virilisation in females
32
17- hydroxylase deficiency results in a lack of?
sex steroids and cortisol
33
17-hydroxylase deficiency causes?
feminisation (no testosterone) hypoglycaemia (no cortisol) hypertension and hyperkalaemia (excess aldosterone) never go through puberty
34
what are the metabolic effects of cortisol?
peripheral protein catabolism hepatic gluconeogenesis increased blood glucose concentration fat metabolism enhanced effects of glucagon