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Z OLD ESA 1- Metabolism > Disorders of Adenocorticol Function > Flashcards

Disorders of Adenocorticol Function Flashcards

1
Q

What is characterised by decreased activity of the adrenal cortex?

A

Addisons disease

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2
Q

What may Addison’s disease be due to?

A

NAME?

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3
Q

What can cause disease of the adrenal cortex?

A

Autoimmune destruction

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4
Q

What is the result of reduction in activity of the adrenal cortex?

A

Reduces glucocorticoids and mineralocorticoid secretion

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5
Q

What do disorders of the pituitary and hypothalamus leading to decreased secretion of ACTH affect?

A

Glucocorticoids only

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6
Q

What is characterised by increased secretion of glucocorticoids?

A

Cushing’s syndrome

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7
Q

What may Cushing’s syndrome be due to?

A
  • Increased activity of the adrenal cortex due to tumour (adenoma)
  • Disorders in secretion of ACTH caused by pituitary adenoma
  • Ectopic secretion of ACTH
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8
Q

What is the disease caused by pituitary adenoma causing increased ACTH secretion?

A

Cushing’s disease

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9
Q

What is congenital adrenal hyperplasia?

A

A number of clinical conditions that arise as a consequence of a genetic defect in one or more of the enzymes required for synthesis of cortisol

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10
Q

What happens due to the lack of cortisol in congenital adrenal hyperplasia?

A

The pituitary is not subjected to negative feedback control

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11
Q

What is the result of the pituitary not being subject to negative feedback?

A

It secretes large amounts of ACTH

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12
Q

What does excess ACTH secretion in congenital adrenal hyperplasia’s cause?

A

An enlargement of the adrenal cortex (hyperplasia)

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13
Q

What does the severity of the consequences of congenital adrenal hyperplasias depend on?

A

Which enzyme(s) is affected

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14
Q

What are the effects of excess cortisol secretion?

A
  • Increased muscle proteolysis and hepatic gluconeogenesis
  • Increased lipogenesis in adipose tissue
  • Purple striae on lower abdomen, upper arms and thighs
  • Immuno-suppressive, anti-inflammatory and anti-allergic reactions of cortisol
  • Back pain and collapse of ribs
  • Hypertension
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15
Q

What can increased muscle proteolysis and hepatic gluconeogenesis lead to?

A

Hyperglycaemia, and associated polyuria and polydipsia

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16
Q

What is hyperglycaemia due to excess cortisol secretion called?

A

Steroid diabetes

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17
Q

What does increased muscle proteolysis cause?

A

Wasting of proximal muscle

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18
Q

What is the result of proximal muscle wastage?

A

NAME?

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19
Q

What does the increased lipogenesis in adipose tissue on excess cortisol secretion lead to?

A

Deposition of fate in abdomen, neck and face

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20
Q

What does the deposition of fat in excess cortisol secretion lead to?

A

The characteristic body shape, moon-shaped face and weight gain

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21
Q

What do the purple striae caused by excess cortisol secretion reflect?

A

The catabolic effects of protein structures in skin

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22
Q

What does the decreased protein integrity in skin caused by excess cortisol secretion lead to?

A

Easy bruising

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23
Q

Why does excess cortisol secretion lead to easy bruising?

A

Because there is thinning of the skin and subcutaneous tissue

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24
Q

What does the immunological effects of excess cortisol secretion lead to?

A

Increased susceptibility to bacterial infections, anda cne

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25
Q

Why may excess cortisol secretion cause back pain and rib collapse?

A

Due to osteoporosis

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26
Q

Why does excess cortisol production lead to osteoporosis?

A

Because of disturbances to calcium metabolism, and loss of bone matrix proteins

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27
Q

Why may mineralocorticoid effects of excess cortisol lead to hypertension?

A

Due to sodium and fluid retention

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28
Q

Where else may the symptoms of excess cortisol secretion occur?

A

In patients receiving long-term treatment with glucocorticoids for various chronic conditions

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29
Q

What would autoimmune destruction of the adrenal gland?

A

Involve loss of cortisol and mineralocorticoids

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30
Q

How does theloss of cortisol and mineralocorticoids present?

A

It is a complex situation, that may present as an acute emergency (Addisonian Crisis), or as a chronic debilitating disorder (Addison’s disease)

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31
Q

How does Addison’s disease onset?

A

Insidious onset, with initial non-specific symptoms of tiredness, extreme muscular weakness, anorexia, vague abdominal pain, weight loss and occasional dizziness

32
Q

What symptoms of Addison’s disease are extreme?

A

The muscular weakness and dehydration

33
Q

What is a more specific sign of Addison’s disease?

A

Increased pigmentation

34
Q

Where does increased pigmentation occur especially in Addison’s disease?

A
  • Exposed areas of the body
  • Points of friction
  • Buccal mucosa
  • Scars
  • Palmar creases
35
Q

Why does Addison’s disease cause increased pigmentation?

A

Due to ACTH-mediated melanocyte stimulation

36
Q

What happens to blood pressure in Addison’s disease?

A

It decreases

37
Q

Why does Addison’s disease decrease blood pressure?

A

Due to sodium and fluid depletion

38
Q

What causes postural hypotension in Addison’s disease?

A

Fluid depletion

39
Q

What kind of episodes does Addison’s disease cause?

A

Hypoglycamic

40
Q

When do the hypoglycaemic episodes caused by Addisons disease particularly occur?

A

On fasting

41
Q

What can exacerbate the effects of Addison’s disease?

A

Stress, such as trauma of severe infection

42
Q

What can the exacerbation of the symptoms of Addison’s disease with stress cause?

A
  • Nausea
  • Vomiting
  • Extreme dehydration
  • Hypotension
  • Confusion
  • Fever
  • Coma
43
Q

What is the exacerbation of the symptoms of Addison’s disease with stress called?

A

An Addisonian crisis

44
Q

What does an Addisonian crisis constitute?

A

A clinical emergency

45
Q

How is an Addisonian crisis treated?

A

IV cortisol and fluid replacement

46
Q

How is adrenocortisol function measured clinically?

A
  • Plasma cortisol
  • ACTH levels
  • 24hr urinary secretion of cortisol and its breakdown products
  • Dynamic function tests
47
Q

What are the breakdown products of cortisol?

A

17-hydroxysteroids

48
Q

Give 2 examples of dynamic function tests

A
  • Dexomethasone surpression tests

- ACTH stimulation tests

49
Q

What can clinical tests of adrenocortisol function be used in?

A

Differential diagnosis of adrenocortisol disease

50
Q

What is dexamethasone?

A

A potent steroid

51
Q

What would normally happen when dexamethasone is given orally?

A

Would suppress the secretion of ACTH and therefore cortisol

52
Q

How does dexamethasone suppress?

A

Feedback inhibition

53
Q

What dexamethasone suppression of plasma cortisol is characteristic of Cushing’s disease?

A

> 50%

54
Q

Why is a dexamethasone suppression of >50% characteristic of Cushing’s disease?

A

Because for the diseases pituitary, even though its relatively insensitive to cortisol, it does retain some sensitivity to potent synthetic steroids

55
Q

Where does dexamethasone suppression not normally occur?

A

In adrenal tumours or ectopic ACTH production

56
Q

What is the result of dexamethasone suppression not normally occurring in adrenal tumours or ectopic ACTH production mean?

A

Rules them out, suggesting the result is due to a pituitary tumour

57
Q

What is Synacthen?

A

A synthetic analogue of ACTH

58
Q

What does the intramuscular administration of Synacthen normally cause?

A

An increase in plasma cortisol by >200nmol/L

59
Q

What does a normal response to Synacthen achieve?

A

The exclusion of Addison’s disease

60
Q

What do the steroid receptors form?

A

Part of a family of nuclear DNA-binding proteins

61
Q

What does the family of nuclear DNA-binding proteins formed by steroid receptors include?

A

The thyroid and vitamin D receptors

62
Q

What do all steroid receptors have?

A

3 main regions

63
Q

What are the 3 main regions of a steroid receptor?

A
  • A hydrophobic hormone-binding region
  • A DNA binding region
  • A variable region
64
Q

What is the DNA binding region of a steroid receptor rich in?

A

Cysteine and basic amino acids

65
Q

What exists in the hormone binding regions of steroid receptors?

A

Sequence homology

66
Q

What is the % homology of the hormone bind region of the glucocorticoid receptor with the mineralocorticoid receptor?

A

64%

67
Q

What is the % homology of the hormone bind region of the glucocorticoid receptor with the androgen receptor?

A

62%

68
Q

What is the % homology of the hormone bind region of the glucocorticoid receptor with the oestrogen receptor?

A

31%

69
Q

What is the % homology of the hormone bind region of the glucocorticoid receptor with the thyroid receptor?

A

24%

70
Q

What is the result of the sequence homology of steroid receptors?

A

Cortisol will bind to the mineralocorticoid and androgen receptors with low affinity

71
Q

When may cortisol binding to mineralocorticoid and androgen receptors be significant?

A

When high levels of hormone present

72
Q

What does aldosterone do?

A
  • Stimulates Na + reabsorption in exchange for K + (or H + )
  • Stimulates growth and development of male genital tract and male secondary sexual characteristics, including height, body shape, facial and body hair, lower voice pitch
  • Anabolic effects on muscle protein
73
Q

What does over-secretion of aldosterone cause?

A
  • Increases Na + and water retention
  • Loss of K +
  • In females-
  • Hair growth (hirsutism)
  • Acne
  • Menstrual problems
  • Virilisation
  • Increased muscle bulk
  • Deepening voice
74
Q

What does an increased Na + and water retention, and loss of K + cause?

A

Hypertension and muscle weakness

75
Q

What does under secretion of aldosterone cause?

A

Hypotension

76
Q

What does oestrogen do?

A
  • Stimulates growth and development of female genital tract, breasts and female secondary characteristics, including broad hips, accumulation of fat in breasts and buttocks, body hair distribution
  • Weakly anabolic
  • Decrease circulating cholesterol levels

Z OLD ESA 1- Metabolism (17 decks)

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