Adrenal Gland Flashcards

1
Q

What causes Cushing’s syndrome?

A

Excess cortisol

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2
Q

What is the commonest cause of Cushing’s?

A

Exogenous cortisol (high-dose steroid medications)

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3
Q

What causes exogenous Cushing’s?

A

Prolonged high-dose steroid medications cause negative feedback on the pituitary and hypothalamus (decrease CRH ACTH). Cortisol production is shut down but the exogenous production is still great enough to cause Cushing’s

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4
Q

What is Cushing’s disease?

A

Pituitary adenoma causing Cushing’s syndrome

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5
Q

What are the ACTH dependent causes of Cushing’s?

A
Pituitary adenoma (Cushing's disease)
Ectopic ATCH (or CRH) due to tumour (carcinoma, carcinoid)
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6
Q

What are the ACTH independent causes of Cushing’s?

A

Adrenal adenoma or carcinoma

Nodular adrenal hyperplasia

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7
Q

Why does Cushing’s cause hyperglycaemia?

A

Cortisol excess can inhibit the uptake of glucose in the muscles causing hyperglycaemia and insulin resistance

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8
Q

What are the signs + symptoms of Cushing’s?

A
Muscle wasting, proximal myopathy
Thin extremities 
Thin skin
Easy bruising and poor wound healing
Abdominal striae
Osteoporosis (whereas in obesity you would get increased bone density)
Buffalo hump
Central obesity
Hyperglycaemia
Appearance change over time
Diabetes mellitus
Psychosis, depression
Facial plethora (redness)
Cataracts 
Interscapular and supraclavicular fat pats
Chemosis
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9
Q

What are the signs + symptoms caused by excess aldosterone in Cushing’s?

A

Hypertension

Oedema

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10
Q

What are the signs + symptoms caused by excess androgen in Cushing’s?

A
Virilism
Hirsutism
Acne
Oligomenorrhoea
Amenorrhoea
Female frontal balding
Lack of libido and impotence
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11
Q

What screening tests are done to establish cortisol excess in Cushing’s?

A

Overnight dexamethasone suppression test (normally cortisol is suppressed)
24hr urinary free cortisol
Late night salivary cortisol

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12
Q

What is the gold standard/diagnostic test for Cushing’s syndrome and what result is considered abnormal?

A

Low dose dexamethasone suppression test (done after a positive overnight dexamethasone suppression test)
>130nmol/L = abnormal
<50nmol/L = no Cushing’s
50-130nmol/L = other tests to bes considered

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13
Q

What test can be used for Cushing’s disease?

A

Raised ACTH (can be pituitary adenoma or ectopic ACTH)
Raised ACTH and cortisol in response to exogenous CRH (ectopic sources rarely respond)
Pituitary MRI

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14
Q

How is exogenous Cushing’s treated?

A

Stop medication if possible

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15
Q

What are the treatment options for Cushing’s disease?

A
  1. Hypophysectomy (surgical removal of pituitary adenoma)
  2. External radiotherapy
  3. Medical therapy to reduce ACTH
  4. Bilateral adrenalectomy (last resort)
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16
Q

What are the treatment options for an adrenal (ACTH independent) cause of Cushing’s?

A
  1. Adrenalectomy

2. Radiotherapy + adrenolytic drugs

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17
Q

What are the treatment options for an ectopic (ACTH dependent) cause of Cushing’s?

A
  1. Adrenalectomy

2. Radiotherapy + adrenolytic drugs (if carcinoma)

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18
Q

What drug treatments are available for Cushing’s syndrome?

A

Metyrapone
Ketoconazole
Pasireotide LAR

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19
Q

What is primary hyperaldosteronism?

A

When the adrenal cortex produces excess aldosterone

20
Q

What is the pathogenesis of primary hyperaldosteronism?

A

Autonomous production of aldosterone independent of its regulators
This leads to sodium retention and potassium loss resulting in hypokalaemia and hypertension

21
Q

What are the causes/subtypes of primary hyperaldosteronism?

A
Bilateral adrenal hyperplasia
Adrenal adenoma
Adrenal carcinoma
Genetic mutations
Unilateral adrenal hyperplasia
22
Q

What is the most common cause/subtype of primary hyperaldosteronism?

A

Bilateral adrenal hyperplasia

23
Q

What is Conn’s syndrome?

A

Primary hyperaldosteronism caused by an adrenal adenoma

24
Q

What mutation is Conn’s syndrome linked to?

A

Mutation in K+ channels (KCHNJ5 mutations)

25
Q

What are the signs + symptoms of primary hyperaldosteronism?

A

Hypertension
Hypokalaemia
Metabolic alkalosis

26
Q

What are the principles of investigation of primary hyperaldosteronism?

A

Confirm Aldosterone excess

Confirm cause/subtype

27
Q

How is aldosterone excess confirmed in primary hyperaldosteronism?

A
Aldosterone:renin ratio
(if ratio is >750 saline suppression is needed)
Saline suppression (failure of aldosterone to suppress by >50% with 2L of saline confirms primary hyperaldosteronism)
28
Q

What is the diagnostic test for adrenal adenoma in primary hyperaldosteronism?

A

Adrenal vein sampling (CT can also be done)

29
Q

What is the treatment for adrenal adenoma in primary hyperaldosteronism?

A

Unilateral laparoscopic adrenalectomy

30
Q

What is the treatment for bilateral adrenal hyperplasia in primary hyperaldosteronism?

A

Mineralocorticoid receptors antagonists (spironolactone, eplerenone)

31
Q

What is a phaeochromocytoma?

A

Catecholamine producing tumour (derived from chromaffin cells and arise from sympathetic paraganglia cells

32
Q

What turns dark brown in response to the tumour cells in phaechromocytoma?

A

Potassium dichromate (due to oxidation of catecholamines in the tumours cells)

33
Q

What is the definitive evidence of malignancy in phaeochromocytoma?

A

Metastasis

34
Q

What mutation is involved in familial phaeochromocytoma?

A

Succinate dehyrdogenase gene mutations

35
Q

Phaeochromocytoma is the 10% tumour. What are the 10% features of the tumour?

A

10% are extra-adrena (in the sympathetic chain (paraganglioma))
10% are bilateral (50% in familial cases)
10% are malignant (defined by metastasis)
10% are not associated with hypertension
10% are in children
10% are associated with hyperglycaemia
10% are familial

36
Q

What is a paraganglioma?

A

A phaeochromocytoma in the sympathetic chain

37
Q

What genetic things are associated with phaeochromocytoma?

A
MEN2A,  MEN 2B 
Von Hippel-Lindau
Succinate dehydrogenase mutations
Neurofibromatosis
Tuberous sclerosis
38
Q

What is the triad of symptoms for phaeochromocytoma?

A

Hypertension
Headache
Sweating

39
Q

What others signs + symptoms might patients have for phaeochromocytoma (besides the triad)?

A
Insidious onset
Postural hypotension
Palpitation
Breathessness
Constipation
Weight loss
Flushing
Bradycardia and tachycardia
Pallor
Pyrexia 
Polydipsia and polyuria
40
Q

What are the signs of complications of phaeochromocytoma?

A
Left ventricular failure
Myocardial necrosis
Stroke
Shock
Paralytic ileus of bowel
41
Q

How is catecholamine excess confirmed in phaeochromocytoma?

A

Urine catecholamines and metanephrines

42
Q

How is the source of catecholamine excess identified in phaeochromocytoma?

A

CT (phaeochromocytoma)
MIBG (tumour scan)
PET scan
MRI (paraganglioma)

43
Q

What are the complications of phaeochromocytoma?

A
Paroxysmal hypertension
Heart failure
MI
Arrhythmia
Stroke
44
Q

What is the pharmacological treatment for phaeochromocytoma?

A

Pre-operative and post-operative
Alpha blocker (phenoxybenzamine) + beta blocker (propranolo, atenolol, metaprolol)
ALPHA BEFORE BETA

45
Q

What is the treatment for malignant phaeochromocytoma?

A

Chemotherapy

Radio-labelled MIBG

46
Q

What surgery is done for phaeochromocytoma?

A

Total excision of the tumour