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ENDOCRINOLOGY > 1b Adrenal Disorders > Flashcards

1b Adrenal Disorders Flashcards

1
Q

What hormones are produced in the adrenal cortex?

A

Corticosteroids

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2
Q

What are the corticosteroids?

A

Mineralcorticosteroids
Glucocorticoids
Sex Steroids

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3
Q

What is the precursor for steroid hormones?

A

Cholesterol

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4
Q

What is the effect of angiotensin II on the adrenals?

A

Activation of the following enzymes
Side Chain Cleavage
3 Hydroxysteroid dehydrogenase
21 hydroxylase
11 hydroxylase
18 hydroxylase

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5
Q

What is the action of aldosterone?

A

Increases blood pressure, retains sodium and lowers potassium

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6
Q

What is the effect on ACTH on the adrenals:

A

Activation of the following enzymes
Side Chain Cleavage
3 Hydroxysteroid dehydrogenase
21 hydroxylase
11 hydroxylase
17 hydroxylase

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7
Q

Describe the rhythm of cortisol release?

A

Diurnal secretion

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8
Q

What is primary adrenal failure?

A

Autoimmune disease where the immune system destroys the adrenal cortex, therefore insufficient steroid hormone production

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9
Q

What are the symptoms of an adrenal crisis?

A

Fever
Syncope
Convulsions
Hypoglycaemia
Vomiting
Diarrhea

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10
Q

What are the symptoms of an adrenal crisis?

A

Fever
Syncope
Convulsions
Hypoglycaemia
Vomiting
Diarrhea

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11
Q

Why do patients with Addison’s have a good tan?

A

POMC is a large precursor
protein that is cleaved to form
a number of smaller peptides,
including ACTH, MSH and
endorphins
Thus people who have
pathologically high levels of
ACTH may become tanned

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12
Q

Describe the changes to the skin in patients with Addisons?

A

Autoimmune Vitiligo
Hyperpigmentation
Good Tan

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13
Q

What are the three causes of adrenocortical failure?

A

TB - tuberculous Addisons Disease
Autoimmune destruction - Autoimmune Addison’s disease
Born without the necessary enzymes

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14
Q

What is POMC?

A

Pro-opio melanocortin

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15
Q

Where is POMC made

A

Pituitary

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16
Q

What is POMC broken down into?

A

ACTH, MSH, Endorphins and enkephalins

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17
Q

What are the consequences of Adrenocortical Failure?

A

Fall in blood pressure
*Loss of salt in the urine
*Increased plasma potassium
*Fall in glucose due to glucocorticoid
deficiency
*High ACTH resulting in increased
pigmentation

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18
Q

Describe the visual symptoms of Addison’s?

A

Hyperpigmentation
Vitiligo
Mucous Membrane hyperpigmentation - therefore definately not sun tan as these areas are not accessible
Darkening of hair
Muscles weakness

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19
Q

What is the test for Addison’s?

A

SynACTHen test

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20
Q

How does the SynACTHen test work?

A

Measure cortisol at 9am
Administer injection of SynACTHen and remeasure cortisol levels

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21
Q

What test results of ACTH suggest Addisons?

A

Low Cortisol following synACTHen administration

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22
Q

Why can aldosterone not be given to patients with Addison’s?

A

Aldosterone has too short a half life, therefore would have to be given multiple times a day in order to be effective

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23
Q

What is give to patients with Addisons?

A

Fludrocortisone - does not exist in natural steroids, therefore presence slows metabolism

Prednisolone - longer half life and more potent than cortisol, rapidly absorbed with half life suitable for once daily administration

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24
Q

What is the dose for prednisolone daily?

A

3.75mg daily

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25
What is the dosage of fludrocortisone?
50 to 100 mcg daily
26
Which hormones will be completely absent in complete 21-hydroxylase deficiency?
Aldosterone and Cortisol
27
How long can you survive without treatment with 21-hydroxylase deficiency?
less than 24 hours
28
Which hormones will be in excess in complete 21-hydroxylase deficiency?
Sex steroids like testosterone
29
What is the youngest age of presentation with Adrenal Disorders?
As a neonate with a salt losing Addisonian Crisis
30
How do female neonates with Addisonian Crises present?
may have ambigious genitalia - loo like they have a penis and scrotum, therefore immediately given saline to restore salt balance
31
What happens to hormone levels in Partial 21 hydroxylase deficiency
a bit of aldosterone and cortisol to get by with
32
Which hormones are deficient in partial 21 hydroxylase deficienfy?
Cortisol and Aldosterone
33
Which hormones are in excess in partial 21 hydroxylase deficiency?
Sex steroids and testosterone
34
What are the main problems in girls with partial 21 hydroxylase deficiency?
Hirsutism and virilisation
35
What are the main problems in boys with partial 21 hydroxylase deficiency?
Precocious puberty due to adrenal testosterone
36
Deficiency of what hormone causes male escutcheon?
Partial 21 hydroxylase defiency
37
What builds up with a 11 hydroxylase deficiency?
11-deoxycortiocosterone
38
what is important to note about 11-deoxycorticosterone?
Behaves like aldosterone, therefore excess can cause hypertension and hypokalaemia
39
Which hormones are deficient in 11-hydroxylase deficiency
Cortisol and aldosterone
40
Which hormones are in excess in 11-hydroxylase deficiency
Sex steroids, testosterone and 11-deoxycorticosterone
41
What are the problems associated with 11-hydroxylase deficiency?
virilisation, hypertension and low potassium
42
What hormones are deficient in 17-hydroxylase deficiency?
Cortisol and Sex Steroids
43
What hormones are in excess in 17-hydroxylase deficiency?
11-deoxycorticosterone, aldosterone
44
What are the problems associated with 17-hydroxylase deficiency?
Hypertension, Low K, Sex steroid deficiency and glucocorticoid deficiency
45
What time in the day do cortisol levels peak?
8.32 am
46
What are some of the signs seen in patients with cushings?
Red Striae Buffalo Humps Poor Wound Healing Centripetal Obesity Hyperplasia of the adrenal glands Moon Face Thin Skin Proximal Myopathy Diabetes
47
List the four causes of Cushing's syndrome?
1. Taking too many steroids orally 2. Pituitary dependant Cushing's Disease 9\Pituitary adenoma) 3. Ectopic ACTH from lung cancer 4. Adrenal Adenoma secreting Cortisol
48
What are the two ways to determine the cause of Cushing's Syndrome?
24h urine collection for urinary free cortisol Blood diurnal cortisol levels
49
Describe how the cortisol levels of someone with Cushing's syndrome differs from a normal person?
both have diurnal rhythm - but cushings patient will have a midnight sleeping cortisol which is not zero
50
How does the low dose dexamethasone test work?
Dexamethasone is an artificial steroid which will suppress cortisol levels to zero in normal patients, but with Cushing's patients, the levels will fail to fall to zero
51
Which drugs are used to treat Cushings'?
Steroid biosynthesis inhibitors
52
What are the two steroid biosynthesis inhibitors used to treat cushing's?
Metyrapone and ketoconazole
53
How does Metyrapone work?
Inhibits 11-hydroxylase, so cortisol levels drop as steroid synthesis in the zona fasciulata and zona reticularis are arrested at the 11- hydroxylase level
54
How does the buildup of 11-deoxycortisol as a result on using metyrapone affect the axis?
No effect - does not exert a negative feedback effect
55
What are the two main uses of Metyrapone?
Control of Cushing's before surgery to improve patients post-op healing process (wound healing) Control of Cushing's after radiotherapy
56
What are the side effects of metyrapone?
11-deoxycorticosterone accumulates in the zona glomerulosa and has aldosterone like effects, leading to salt retention and hypertension
57
How does Metyrapone lead to Hirsutism?
Increased androgen production due to the lack of 11-hydroxylase leads to hirsutism in women as there is more testosterone produced
58
Describe the mechanism of action for ketoconazole?
Mainly blocks 17 hydroxylase, which inhibits cortisol production
59
What are the side effects of ketoconazole?
Liver damage - monitor liver function weekly
60
Describe the mechanism of Osilidrostat?
Blocks 11 hydroxylase, therefore no cortisol produced
61
What is Conn's syndrome?
Where a benign adrenal cortical tumour causes an excess of aldosterone
62
What are the effects of Conn's syndrome?
Hypertension and hypokalaemia
63
What is used to treat Conn's syndrome?
Spironolactone
64
How does Spironolactone work?
Converted into several active metabolites, including canrenone which is a competitive antagonist of the mineralcorticoid receptor which reverses the effects of aldosterone Also blocks Na+ reabsorption and K+ excretion in the kidney tubules
65
Where is Spironolactone metabolised?
it is highly protein bound and metabolised in the liver
66
What are the side effects of spironolactone?
Menstrual irregularitie Gynaecomastia
67
What are the two drugs used to treat Conn's syndrome?
Spironolactone Epleronone
68
Why is epleronone prefered over spironolactone?
Binds less to androgen and progesterone receptors, therefore less gynaecomastia
69
What are phaeochromocytomas?
Tumours of the adrenal MEDULLA which secrete catecholamines liek Adrenaline and Nor Adrenalline
70
What are the clinical features of a phaeocytochromocytoma?
Hypertension in young people Episodic severe hypertension (after abdominal palpitation
71
Why is a phaeochromocytoma considered a medical emergency?
High blood pressure can cause MI or stroke High Adrenaline = cause ventricular fibrillation
72
Why must you be careful when performing surgery for a phaeo?
Surgery - but have to be careful as anaesthetic can precipitate a hypertensive crisis
73
How are Phaeo's managed?
Alpha Blockade - give IV fluid while this Beta Blockade - added to prevent tachy cardia

ENDOCRINOLOGY (20 decks)

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