Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Yr 2 LCRS - Endo > 6 - Hyperadrenal Disorders > Flashcards

6 - Hyperadrenal Disorders Flashcards

(52 cards)

Start Studying
1
Q

What are the clinical feature’s of Cushing’s Syndrome?

A
  • too much cortisol
  • centripetal obesity
  • moon face
  • buffalo hump
  • proximal myopathy
  • reduced sensation and power in the legs
  • persistent pain in hips and legs
  • difficulty walking
  • hypertension
  • hypokalaemia
  • red striae
  • thin skin
  • shiny skin
  • easy bruising
  • osteoporosis
  • diabetes (T2DM) = hyperglycaemia
  • peripheral oedema (pitting)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the difference between Cushing’s Syndrome and Cushing’s Disease?

A

Cushing’s Syndrome
- refers to excess cortisol in the body, regardless of the cause.

Cushing’s Disease
- caused by a pituitary gland tumor (usually benign) that over-secretes the hormone ACTH.

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

What is Proximal Myopathy?

A

Wastage of muscles

Symmetrical weakness of proximal upper and/or lower limbs.

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

What are the causes of Cushing’s?

A
  • Taking too many steroids
  • Pituitary dependent Cushing’s Disease
  • Ectopic ACTH from lung cancer
  • Adrenal Adenoma secreting cortisol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How can the causes of Cushing’s be divided?

A

Exogenous Causes
- Taking too many steroids

Endogenous Causes

  • Pituitary dependent Cushing’s Disease
  • Ectopic ACTH from lung cancer
  • Adrenal Adenoma secreting cortisol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What investigations can be done to determine the cause of Cushing’s Syndrome?

A
  • 24hr urine collection for urinary free cortisol
  • Blood diurnal cortisol levels
  • Low dose dexamethasone suppression test
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is special about normal cortisol release in the body?

A

It is diurnal

  • cortisol is usually highest at 9am
  • cortisol is lowest at midnight, if the person is asleep

In Cushing’s, the person has high cortisol all the time

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

How do you conduct a low dose dexamethasone suppression test (LDDST)?

A

Dexamethasone
= artifical steroid
= like cortisol but very potent
= corticosteroid

0.5mg 6 hourly for 48 hours

Measure cortisol all day

Normals will suppress cortisol to zero

Any cause of Cushing’s will fail to suppress cortisol

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

How can you diagnose Cushing’s?

A
  • Basal (9am) cortisol: 800nM or above

- End of LDDST: 680nM or above

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

What hormone is produced too much in Cushing’s Syndrome?

A

Cortisol

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

What does Cushingoid mean?

A

Having the constellation of symptoms and signs caused by an excess of cortisol hormone.

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

What overall categories of treatments are there for Cushing’s?

A

Enzyme Inhibitors (prevent steroid synthesis)

Receptor Blocking Drugs (block cortisol receptor)

Pituitary Surgery (Transsphenoidal Hypophysectomy)

Bilateral Adrenalectomy

Unilateral Adrenalectomy for single adrenal mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Which part of the adrenal gland is overactive in Cushing’s and Conn’s?

A

The adrenal cortex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are two examples of drugs that are inhibitors of steroid biosynthesis?

A

Metyrapone

Ketoconazole

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What two examples of MR antagonists?

A

Spirinolactone

Epleronone

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

What is the overall function of inhibitors of steroid biosynthesis as treatment for Cushing’s?

A

They stop cortisol production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the mechanism of action of Metyrapone as treatment for Cushing’s Syndrome?

A

Inhibition of 11-beta-hydroxylase

Steroid synthesis in the zone fasciculata (and of corticosterone in the reticularis) is arrested at the 11-deoxycortisol stage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the pathway of cortisol biosynthesis starting from cholesterol?

A

Cholesterol

  • cytochrome P450 enzymes

Pregnenelone

  • 17a-hydroxylase

17a-hydroxypregnenolone

  • 3beta-hydroxysteroid dehydrogenase

17a-hydroxyprogesterone

  • 21-hydroxylase

11-deoxycortisol

  • 11beta-hydroxylase

Cortisol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the principal glucocorticoid in humans?

A

Cortisol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Which part of the adrenal cortex produces cortisol?

A

Zona Fasciculata

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Which part of the adrenal cortex produces aldosterone?

Study These Flashcards
A

Zona Glomerulosa

22
Q

What is the pathway of aldosterone biosynthesis starting from cholesterol?

Study These Flashcards
A

Cholesterol

  • cytochrome P450 enzymes

Pregnenolone

  • 3beta-hydroxysteroid dehydrogenase

Progesterone

  • 21-hydroxylase

11-deoxycorticosterone (this can also go on to produce corticosterone instead of aldosterone via 11beta-hydroxylase)

  • aldo synthase

Aldosterone

23
Q

What type of hormones are aldosterone and cortisol?

Study These Flashcards
A

Steroid Hormones

24
Q

What are the biochemical effects of Metyrapone as treatment?

Study These Flashcards
A

Cortisol synthesis blocked

ACTH secretion increased

Plasma deoxycortisol increased (precursor to cortisol which is not converted because 11beta-hydroxylase enzyme is blocked)

25
What are the uses of metyrapone?
Control of Cushing's syndrome prior to surgery Control of Cushing's symptoms after radiotherapy (which is usually slow to take effect)
26
How do you adjust the dose of metyrapone for a patient?
Adjust oral dose according to cortisol Aim for mean serum cortisol of 150-300 nmol/L
27
Why is meytrapone often given to patients with Cushing's syndrome prior to surgery?
Improves patient's symptoms - friable skin improved etc Promotes better post-op recovery - better wound healing - less infection (cortisol is immunosuppressant) etc
28
What is a biochemical downside to using metyrapone as treatment for Cushing's Syndrome?
It also inhibits part of the aldosterone pathway which has a tail that makes corticosterone. Build up of 11-deoxycorticosterone in zona glomerulosa. It has aldosterone-like (mineralocorticoid) activity - water retention - hypertension Build up of all the precursors to 11-deoxycorticosterone as well. This leads to increased androgen production - acne - hirsutism in women
29
What are the uses of Ketoconazole?
Anti-Fungal Agent = was once its main use = withdrawn in 2013 due to risk of hepatotoxicity = don't want to block cortisol production during most anti-fungal therapy Inhibits Steroidgenesis - off-label use in Cushing's Syndrome - blocks cortisol production - has this action at higher concentrations THEREFORE: Used to treat and control symptoms of Cushing's Syndrome prior to surgery
30
What is the mechanism of action of Ketoconazole?
Blocks production of: - glucocorticoids - mineralocorticoids - sex steroids Does this by blocking multiple steps in steroidgenesis - cytochrome P450 - androstenedione step etc
31
How is Ketoconazole pharmacologically active?
It is orally active.
32
What is the main side effect of Ketoconazole use?
Liver Damage - possibly fatal - hepatotoxicity Need to monitor patient's liver function weekly, clinically and biochemically.
33
If pituitary surgery is chosen as treatment for a patient with Cushing's Syndrome, which one is performed?
Transsphenoidal Hypophysectomy
34
What is Conn's Syndrome?
Primary aldosteronism It is s an endocrine disorder characterized by excessive secretion of the hormone aldosterone.
35
Why do the adrenal glands secrete excess aldosterone in Conn's Syndrome?
Benign adrenal cortical tumour (zona glomerulosa)
36
What physiological states does Conn's Syndrome leave the body in?
Hypertension Hypokalaemia High blood pressure with low potassium is very uncommon, except in Conn's Syndrome.
37
How do you diagnose Conn's Syndrome?
If it's a tumour causing it (primary hyperaldosteronism), then the renin-angiotensin system should be suppressed [This excludes secondary hyperaldosteronism which is hyperaldosteronism not caused by a adrenal tumour]
38
What class of drug would you use to treat Conn's Syndrome?
Mineralocorticoid Receptor Antagonists (MR Antagonists) - not stopping aldosterone production - we stop aldosterone's action on its receptor
39
What is the mechanism of action of Spironolactone?
Converted to several active metabolites (including canrenone) These are competitive antagonists of the mineralocorticoid receptors Blocks Na+ reabsorption and K+ excretion in the kidney tubules It is therefore a potassium sparing diuretic.
40
What is the use of Spironolactone?
Primary Hyperalodosteronism (Conn's Syndrome)
41
What are the main side effects of Spironolactone?
Activates progesterone receptor (agonist) - causing menstrual irregularities in women Blocks androgen receptor (antagonist) - causing gynaecomastia in men
42
What is the mechanism of Eplerone?
A Minerlocorticoid Receptor (MR) Antagonist
43
Why is Eplerone a better tolerated MR Antagonist treatment of Conn's Syndrome compared to Spironolactone?
Less binding to androgen and progesterone receptors than spironolactone. Therefore, no menstrual irregularities and gynaecomastia.
44
What is a Phaeochromocytoma?
A tumour of the adrenal medulla which secrete catecholamines
45
What hormones are synthesise by the adrenal medulla?
Catecholamines (adrenaline and noradrenaline)
46
What do Phaeochromocytomas cause in the body?
The tumours become very large and secrete lots of adrenaline at once. This can cause severe hypertension (acute) - sudden - comes and goes with surges of adrenaline - these episodes are called attacks - episodic severe hypertension
47
What population group does a Phaeochromocytoma normally present in?
Young People
48
What are the clinical features of a Phaeochromocytoma?
- Hypertension in young people - Episodic severe hypertension (after abdominal palpation) - More common in certain inherited conditions (certain genes)
49
What are the risks associated with the clinical features of a Phaeochromocytoma?
Severe hypertension can cause MI or stroke High adrenaline can cause ventricular fibrillation and death Medical emergency
50
Why must a Phaeochromocytoma be managed before surgery occurs to remove it?
Eventually need surgery Patient needs to have their hypertension managed first before surgery This is because anaesthetic can precipitate a hypertensive crisis
51
How do you manage a Phaeochromocytoma?
You block the receptors that adrenaline binds to 1. ALPHA BLOCKADE - patient made need IV fluid as this commences - this is because if they've had a phaeo for a very long time, they'll be extremely vasoconstricted - the fluid stops a crash in blood pressure 2. BETA BLOCKADE - to prevent tachycardia
52
What are some key facts about Phaeochromocytomas?
Extremely rare 10% extra-adrenal (somewhere in sympathetic chain) 10% malignant 10% bilateral

Yr 2 LCRS - Endo (28 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions