Adrenal disorders Flashcards

1
Q

How does the adrenal gland develop embryologically?

A

Fetal medulla becomes encapsulated by fetal cortex

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2
Q

What is hyperaldosteronism?

A

Excess production of aldosterone by zona glomerulosa in adrenal glands

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3
Q

How are the causes of hyperaldosteronism categorised? What do they mean?

A

Primary - problem with adrenal cortex

Secondary - overactivity of RAAS

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4
Q

What are the causes of primary hyperaldosteronism? Which is the most common?

A

Bilateral idiopathic adrenal hyperplasia - most common

Conn’s syndrome

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5
Q

What is Conn’s syndrome?

A

Adrenal adenoma

secretes aldosterone

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6
Q

What are the causes of secondary hyperaldosteronism?

A

Renal artery stenosis

Renin producing tumour e.g juxtaglomerular tumour

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7
Q

How do you differentiate between primary and secondary hyperaldosteronism?

A

Primary
low renin levels
high aldosterone:renin ratio

Secondary
high renin levels
low aldosterone:renin ratio

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8
Q

What are the signs of hyperaldosteronism?

A

Hypertension

Hypernatraemia

Hypokalaemia

LV hypertrophy

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9
Q

How is hyperaldosteronism treated?

A

Conn’s syndrome - adrenal adenoma removed by surgery

Bilateral idiopathic adrenal hyperplasia + overactivity of RAAS - spironolactone

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10
Q

What does spironolactone do?

A

Mineralocorticoid receptor antagonist

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11
Q

What is Cushing’s syndrome?

A

Increased secretion of glucocorticoid cortisol

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12
Q

How are the causes of Cushing’s syndrome categorised?

A

External causes

Endogenous causes

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13
Q

What is the external cause of Cushing’s syndrome?

A

Prescribed glucocorticoids

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14
Q

What are the endogenous causes of Cushing’s syndrome?

A

Cushing’s disease

Adrenal Cushing’s

Non pituitary-adrenal tumours producing ACTH or CRH

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15
Q

What is Cushing’s disease?

A

Pituitary adenoma

secretes ACTH

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16
Q

What is Adrenal Cushing’s?

A

Adrenal tumour

produces excess cortisol

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17
Q

What is an example of a non pituitary-adenoma tumour that produces ACTH or CRH?

A

Small cell lung cancer

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18
Q

How do you differentiate between Adrenal cushing’s and Cushing’s disease/non pituitary-adrenal tumour?

A

Adrenal cushing’s - low ACTH
by negative feedback from excess cortisol

Cushing’s disease/non pituitary-adrenal tumour - high ACTH
being secreted by the tumour

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19
Q

What is the most common cause of Cushing’s syndrome?

What is the most rare cause?

A

Most common - external cause, prescribed glucocorticoids

Most rare - endogenous causes, non pituitary-adenoma tumour secreting ACTH or CRH

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20
Q

What are the signs and symptoms of Cushing’s syndrome?

A

Round pink face

Abdominal obesity

Buffalo hump

Skinny arms and legs

Purple striae on abdomen

Hypertension

Hyperglycaemia

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21
Q

What causes the pink round face in Cushing’s syndrome?

A

Redistribution of fat to face

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22
Q

What causes the abdominal obesity in Cushing’s syndrome?

A

Resdistribution of fat to abdomen

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23
Q

What causes the buffalo hump in Cushing’s syndrome?

A

Redistribution of fat to dorso-cervical fat pads

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24
Q

What causes the purple-striae on the abdomen in Cushing’s syndrome?

A

Cortisol stimulates proteolysis
proteins in skin break down, skin becomes thinner
leading to easy bruising

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25
Q

What causes the skinny arms and legs in Cushing’s syndrome?

A

Cortisol stimulating proteolysis in muscles

get wasting of muscles

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26
Q

What causes the hypertension in Cushing’s syndrome?

A

High levels of cortisol
may bind to aldosterone receptors
gives sodium and water retention

27
Q

What causes the hyperglycaemia in Cushing’s syndrome?

A

Excess cortisol over-stimulates gluconeogenesis in liver

28
Q

How is Cushing’s syndrome treated?

A

External causes - reduce dosage gradually, don’t stop suddenly

Endogenous causes - surgically remove the tumour

29
Q

What is Addisons’s disease?

A

Condition of chronic adrenal insufficiency

30
Q

What are the causes of Addison’s disease? Which is the most common cause?

A

Autoimmune destruction of adrenal cortex - most common cause

Infection - TB, fungal

Infiltration - amyloid, haemachromatosis

Malignancy

Genetic - congenital adrenal hyperplasia

Vascular - haemorrhage, infarction

Iatrogenic - adrenalectomy, drugs

31
Q

What are the symptoms and signs of Addison’s disease?

A

Lethargy

Anorexia
Weight loss

Postural hypotension - dizziness

Increased skin pigmentation

Hypoglycaemia

32
Q

Why is there increased skin pigmentation in Addison’s disease?

A

Decreased cortisol
reduced negative feedback on anterior pituitary
increased production of ACTH
requires POMC
broken down into ACTH and MSH (melanocyte stimulating hormone)
MSH binds to melanocrotin receptors on melanocytes
ACTH itself can bind to these and contribute

33
Q

How is Addison’s disease treated?

A

Lifelong glucocorticoid replacement e.g. hydrocortisone

Lifelong mineralocorticoid replacement e.g. fludrocortisone

34
Q

How can Addisonian’s crises be prevented?

A

Increase dose of glucocorticoid replacement at times of stress e.g. illness
because cortisol would naturally increase

If vomiting, use injection instead of tablets
because can’t absorb tablets

35
Q

What is an Addisonian crisis?

A

Life-threatening emergency

caused by adrenal insufficiency

36
Q

What can trigger an Addisonian crisis?

A

Severe stress

  • trauma
  • infection
  • over exertion
  • abrupt steroid drug withdrawal
37
Q

Why do Addisonian crises occur?

A

Cortisol is too low to cope with the stress

38
Q

What are the signs and symptoms of Addisonian crisis?

A

Nausea, vomiting

Fever

Hypotension

Vascular collapse

39
Q

How is Addisonian’s crisis treated?

A

Rehydration with fluids

IV hydrocortisone - cortisol replacement

Correction of hypoglycaemia

40
Q

What inheritance pattern does congenital adrenal hyperplasia show?

A

Autosomal recessive

41
Q

What is the cause of congenital adrenal hyperplasia?

A

Defect in 21-hydroxylase enzyme

42
Q

What are the consequences of a lack of the enzyme 21-hydroxylase?

A

Low glucocorticoids e.g. cortisol
Low mineralocorticoids e.g. aldosterone

High androgens e.g. testosterone
pathway redirected to here

43
Q

How does congenital adrenal hyperplasia present?

A

Hypoglycaemia

Hypotension

Hyponatraemia

Hyperkalaemia

Virilisation - male characteristics
ambiguity of genitalia

44
Q

How is congenital adrenal hyperplasia managed?

A

Treat addisonian crisis

Long term replacement of glucocorticoids, mineralocorticoid

Determine sex of baby

45
Q

What is a phaeochromocytoma?

A

Tumour of the adrenal medulla chromaffin cells
secretes catecholamines, mainly noradrenaline

46
Q

What is a paraganglioma?

A

Extra-adrenal tumour

chromaffin tissue origin

47
Q

What are the symptoms of phaeochromocytoma and paraganglioma?

A

Severe hypertension

Palpitations
arrythmias

Diaphoresis - excessive sweating

Hyperglycaemia

Weight loss

Headaches

48
Q

How are phaeochromocytomas and paragangliomas managed?

A

Alpha blockers - phenoxybenzamine

Beta blockers

49
Q

In which order should alpha and beta blockers be given? Why?

A

Alpha before beta

Because otherwise get vasoconstriction only, makes hypertension worse

50
Q

What tests are done when an adrenal hormone deficiency is suspected?

A

Electrolytes

9am cortisol

Stimulation test

51
Q

What is the stimulation test for cortisol deficiency?

A

Inject synthetic ACTH, called synacthen

Cortisol should increase in response

52
Q

What are the test results when there is an adrenal hormone deficiency?

A

Electrolytes - hyponatraemia, hyperkalaemia

9am cortisol - low

Stimulation test - cortisol remains low

53
Q

What are the tests done when an adrenal hormone excess is suspected?

A

Electrolytes

Midnight cortisol

Suppression test

54
Q

What is the suppression test for excess cortisol?

A

Give dexamethasone steroid drug

Should negatively feedback on anterior pituitary, decreasing ACTH secretion
Cortisol should decrease

55
Q

What are the test results when there is an adrenal hormone excess?

A

Electrolytes - hypernatraemia, hypokalaemia

Midnight cortisol - high when it should be low

Suppression test - cortisol remains high

56
Q

What tests are done to assess adrenal medulla function?

A

24h urine catecholamimes e.g. adrenaline, noradrenaline

24h urine metanephrines

Plasma metanephrines

57
Q

What are metanephrines?

A

Metabolites of adrenaline and noradrenaline

Called metadrenaline and normetadrenaline

58
Q

What are the test results of adrenal medulla function with phaeochromocytoma?

A

24h urine catecholamines - high

24h urine metanephrines - high

Plasma metanephrines - high

59
Q

What scans are done to look for adrenal tumours?

A

CT scan

MRI scan

PET scan

60
Q

What are the causes of adrenal hormone deficiency?

A

Addison’s disease

ACTH deficiency from hypopituitarism

Steroid induced ACTH suppression

61
Q

What are the symptoms of ACTH deficiency?

A

Similar to primary adrenal failure - Addison’s disease

Except no hyperpigmentation as ACTH not raised
No hyperkalaemia as no mineralocorticoid deficiency

Hyponatraemia due to reduced cortisol meaning reduced diuresis

62
Q

What are some examples of steroid drugs that suppress ACTH?

A

Dexamethasone

Prednisolone

63
Q

What are steroid drugs used to treat?

A

Inflammatory disorders

  • asthma
  • inflammatory bowel disease
  • rheumatoid arthritis

Suppress immune response to organ transplantation.