Adrenal disease Flashcards

1
Q

What are the parts of the adrenal gland?

A
  • Capsule
  • Cortex
  • Medulla
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2
Q

What are the layers of the cortex?

A
  • Zona glomerulosa
  • ZonaFasciculata
  • Zone reticularis
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3
Q

What is the function of the glomerulosa?

A

•Mineralcorticoids: Aldosterone - salt

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4
Q

What is the function of the fasciculata?

A

•Glucocortiocid production: cortisol - sugar

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5
Q

What is the function of the reticularis?

A

•Androgens - sex

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6
Q

Explain the regulation of the renin angiotensin system

A
  • Renin is the major regulator of aldosterone production (zona glomerulosa)
  • Activated in response to decreased blood pressure
  • Leads to the production of angiotensin II which causes direct (vasoconstriction) and indirect (aldosterone) methods of BP elevation
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7
Q

Explain the regulation of cortisol/androgen production

A

•Hypothalamus releases CRH - corticotropin releasing hormone
•CRH acts on the anterior pituitary which is stimulated to release ACTH -adrenocoricotropic hormone
•ACTH acts on the adrenal cortex, stimulating it
to release cortisol
•Negative feedback from cortisol acts on the anterior pituitary and hypothalamus

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8
Q

What normal physiological things effect the release of CRH?

A
  • Illness
  • Stress
  • time of day
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9
Q

What is the appearance of someone with Cushing’s?

A
  • Moon face, flushed
  • Increased abdominal fat with striae
  • Easy bruising
  • Poor wound healing
  • Thin arms and legs due to muscle wasting
  • Thinning of skin
  • Buffalo hump
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10
Q

What are the signs of Cushing’s?

A
  • Benign intracranial hypertension
  • Cataracts
  • Avascular necrosis of femoral head
  • Hypertension
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11
Q

Explain the different measurements of cortisol

A

You should perform 2 of the following:
•24 hour urinary free cortisol
•Urine cortisol: creatinine ratio x3
•Dexamethasone suppression test:
- overnight or low dose test over 48 hours
- plasma cortisol should be undetectable in normal circumstances
•Late night salivary cortisol
- should be undetectable or very low in normals

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12
Q

What are the causes of Cushing’s syndrome?

A

•ACTH dependent:

  • pituitary adenoma
  • Cushing’s disease
  • Ectopic ACTH
  • Ectopic CRH

•ACTH independent:

  • adrenal adenoma
  • adrenal carcinoma
  • nodular hyperplasia
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13
Q

What is the management of adrenal adenoma?

A
  • Laparascopic adrenalectomy

* Short term requirement for hydrocortisone replacement therapy

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14
Q

What are the causes of primary adrenal insufficiency?

A
  • Addison’s disease

* Autoimmune destruction

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15
Q

What are the clinical features of adrenal insufficiency?

A
  • Anorexia and weight loss
  • Fatigue/lethargy
  • Dizziness and low BP
  • Abdominal pain
  • Vomiting and diarrhoea
  • Skin pigmentation
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16
Q

Explain the investigations of adrenal insufficiency

A

•Biochemistry: Low Na, High K+, hypoglycaemia
•Short synacthen test:
- measure plasma cortisol before and 30 minutes after IV ACTH injection
- should see a rise in cortisol
•ACTH levels
•Renin/aldosteone levels
- Increased renin, decreased aldosterone
•Adrenal autoantibodies

17
Q

What is congenital adrenal hyperplasia?

A
  • Autosomal recessive disorder
  • Range of genetic disorders relating to defects in steroidogenic genes
  • Most common = CYP21 affecting the glomerulosa and fasciculata
  • Means there is a reduction/no aldosterone and cortisol
18
Q

What are the signs of congenital adrenal hyperplasia?

A
  • Female: ambiguous genitalia

* Boys: Adrenal crisis (hypotension, hyponatraemia), early virilisation

19
Q

What is the treatment of congenital adrenal hyperplasia?

A

Mineralocorticoid and glucocorticoid replacement

20
Q

Why is there an increased androgen production in congenital adrenal hyperplasia?

A
  • Increased feedback so more ACTH is released

* Because the reticularis is not affected, this continues to be stimulated, producing more androgens

21
Q

What are the signs of late onset congenital adrenal hyperplasia?

A
  • Oligomenorrhoea
  • Hirsuitism
  • Reduced fertility
22
Q

Explain late onset CAH

A
  • Partial 21 alpha hydroxylase deficiency
  • Increased ACTH drive leads to increased 17 OPH and adrenal androgens
  • Diagnosis: Synacthen test with 17OHP
23
Q

What are hypertension and hypokalaemia likely a result of?

A

Primary aldosteronism

24
Q

What is the most common cause of secondary hypertension?

A

Primary aldosteronism

25
Q

What is the best screening tool for primary aldosteronism?

A

Aldosterone-renin-ratio (would be increased)

26
Q

Which drugs should you stop in suspected aldosterone excess?

A

Beta blockers and MR antagonists

27
Q

What is the saline suppression test?

A
  • 2L saline over 4 hours

* 4h aldosterone over 270pmol/l is highly suspicious (it should fall or be suppressed)

28
Q

What is the management of primary aldosteronism?

A

•Surgical:
- unilateral laparoscopic adrenalectomy, only if due to adrenal adenoma
•Medical: use MR antagonists (spironolactone or eplerenone)

29
Q

What are the symptoms/signs of pheochromocytoma?

A
  • Hypertension
  • Episodes of headache, palpitations, pallor and sweating due to catecholamine excess
  • Tremor, anxiety, nausea, vomiting, chest/abdo pain
  • Crises last 15 mins and often well in between
30
Q

Which conditions are associated with pheochromocytoma?

A
  • MEN
  • VHL
  • SDBH and SDHD
  • Neurofibromatosis
31
Q

What is the pre operative treatment of pheochromocytoma?

A
  • Alpha blockade initially to stop catecholamine excess - Phenoxybenzamine, aim for SBP<120mmHg
  • After this then beta blocker if tachycardia
  • Encourage salt intake
32
Q

What is adrenal incidentaloma?

A

Incidentally discovered adrenal lesion discovered through diagnostic imaging for unrelated condition, without prior suspicion of tumour/disease

33
Q

What are the characteristics on imaging that would make you think an incidentaloma is more likely benign?

A
  • Lipid rich
  • Less than 4cm
  • Low density, less than 10HU non contrast CT
34
Q

List the overproduction adrenal disorders

A
  • Primary hyperaldosteronsim - aldosterone
  • ACTH independent Cushing’s - cortisol
  • Congenital adrenal hyperplasia - androgens
  • Phaeochromocytoma - Catecholamines