Adrenal Disorders (Hypo) Flashcards

1
Q

What does the adrenal cortex make?

A

Corticosteroids

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2
Q

What is the definition of a steroid?

A

Comes from choelstrol

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3
Q

Where in the adrenal gland are corticosteroids made?

A

Adrenal cortex

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4
Q

What corticosteroids are made in the adrenal cortex?

A
  1. Mineralocorticoids (Aldosterone)
  2. Glucocorticoids (Cortisol)
  3. Sex steroids (Androgens, oestrogens)
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5
Q

What adrenal enzymes does angiotensin II activate?

A
  1. Side Chain Cleavage
  2. 3 Hydroxysteroid dehydrogenase
  3. 21 hydroxylase
  4. 11 hydroxylase
  5. 18 hydroxylase
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6
Q

What is aldosterone’s action?

A

Controls blood pressure, sodium and lowers potassium

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7
Q

What adrenal enzymes does ACTH active?

A
  1. Side Chain Cleavage
  2. 3 Hydroxysteroid dehydrogenase
  3. 21 hydroxylase
  4. 11 hydroxylase
  5. 17 hydroxylase
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8
Q

What enzyme has diurnal rhythm?

A

Cortisol

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9
Q

What is primary adrenal failure called?

A

Addison’s disease

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10
Q

What is Addison’s disease caused by in the UK?

A

Autoimmune disease where the immune system decides to destroy the adrenal cortex

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11
Q

What is commonest cause of Addison’s disease worldwide?

A

Tuberculosis

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12
Q

What does the pituitary secrete in Addison’s disease?

A

ACTH and hence MSH

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13
Q

Why does an Addison’s patient have low blood pressure?

A

No cortisol or aldosterone

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14
Q

What skin condition may coexist with Addison’s disease?

A

Autoimmune vitiligo

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15
Q

Why does an Addison’s patient have increased pitgmentation?

A

POMC is a large precursor protein that is cleaved to form a number of smaller peptides, including ACTH, MSH and endorphins

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16
Q

What are the pathophysiological causes of adrenocortical failure?

A
  1. Adrenal glands destroyed (2)
  2. Enzymes in the steroid synthetic
    pathway not working (1)
17
Q

What are the causes of adrenocortical failure?

A
1. Tuberculous Addison’s disease 
(commonest worldwide)
2. Autoimmune Addison’s disease 
(commonest in UK)
3. Congenital adrenal hyperplasia
18
Q

What are the consequences of adrenocortical failure?

A
  1. Fall in blood pressure
  2. Loss of salt in the urine
  3. Increased plasma potassium
  4. Fall in glucose due to glucocorticoid deficiency
  5. High ACTH resulting in increased pigmentation
  6. Eventual death due to severe hypotension
19
Q

What is POMC?

A

Pro-opio melanocortin

20
Q

Where is POMC synthesized?

A

Pituitary

21
Q

What is POMC broken down into?

A
ACTH
MSH
Endorphins
Enkephalins
Other peptides
22
Q

What tests do you preform once there is clinical suspicion for Addision’s?

A
  1. 9am cortisol = low
  2. ACTH = high
  3. Short synACTHen test
    - Give 250 ug synacthen IM
    - Measure cortisol response
23
Q

How does an Addison’s patient respond to synACTH?

A

Cortisol at 9am = 100 (270-900)
Administer injection IM of synacthen
Cortisol at 9.30 = 150 (>600)nM

24
Q

What is the treatment of adrenal failure?

A
  1. Prednisolone 3mg daily
    or
    Hydrocortisone three times daily (10 + 5 + 2.5)
  2. Fludrocortisone 50 to 100 mcg daily
25
Q

What is congenital adrenal hyperplasia most commonly caused by?

A

21-hydroxylase deficiency

26
Q

Which hormones will be totally absent in complete 21-hydrosylase deficiency?

A

Aldosterone and cortisol

27
Q

How long can you survive in complete 21-hydrosylase deficiency?

A

Less than 24hrs

28
Q

Which hormones will be in excess in complete 21-hydrosylase deficiency?

A

Sex steroids and testosterone

29
Q

What is the presentation of complete 21-hydrosylase deficiency?

A

As a neonate with a salt losing Addisonian crisis

30
Q

Why is the foetus not affected by complete 21-hydrosylase deficiency in utero?

A

Foetus gets steroids across placenta

31
Q

How may infant girls be affected by complete 21-hydrosylase deficiency?

A

Might have ambiguous genitalia (virilised by adrenal testo)

32
Q

Which hormones are deficient in partial 21-hydrosylase deficiency?

A

Cortisol and aldosterone

33
Q

Which hormones will be in excess in partial 21-hydrosylase deficiency?

A

Sex steroids and testosterone

34
Q

At what age will they present?

A

Any age, as they survive

35
Q

What is the main problem in later life in partial 21-hydroxylase deficiency for girls?

A

Hirsutism and virilisation

36
Q

What is the main problem in later life in partial 21-hydroxylase deficiency for boys?

A

Precocious puberty

37
Q

What is the main problem in later life in partial 21-hydroxylase deficiency due to?

A

Adrenal testosterone