Adrenal Gland Clinical Flashcards

1
Q

The adrenal, simply, controls what?

A

Salt
Sugar
Sex
Stress

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2
Q

What are the layers of the adrenal gland?

A

Zona Glomerulosa
Zona Fasciculata
Zona Reticularis
Medulla

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3
Q

When you have raised suspicion of adrenal dysfunction, how do you approach it?

A
Is it functioning high or low?
Is it primary, secondary or tertiary?
What could be the cause?
If tumour: removable?
Deficiency: can it be fixed?
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4
Q

How is cortisol secretion stimulated?

A

CRH from hypothalamus

ACTH from anterior pituitary

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5
Q

Elevated cortisol inhibits what?

A

Secretion of CRH and ACTH

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6
Q

Hypofunction of the adrenal gland is usually due to what?

A

Adrenal dysgenesis
Adrenal dysfunction
Impaired steroidogenesis

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7
Q

What is the precursor to catecholamines?

A

Dopamine

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8
Q

What are the causes of Adrenal Hyposecretion?

A

Primary Adrenal Insufficiency

Adrenal Enzyme defects

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9
Q

What are the causes of primary adrenal insufficiency?

A

Addison’s Disease

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10
Q

What are the different causes of Addison’s disease?

A
AUTOIMMUNE
Invasion
Infiltration
Infection
Infarction
Iatrogenic
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11
Q

Addison’s disease causes what?

A

Adrenal insufficiency

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12
Q

Which adrenal enzyme deficiency typically leads to hyposecretion?

A

21-hydroxylase

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13
Q

What is the role of 21-hydroxylase?

A

Formation of aldosterone and cortisol from progesterones

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14
Q

Autoimmune dysfunction usually attacks what in autoimmune addison’s?

A

Adrenal autoantibodies to 21-hydroxylase (70%)

Lymphocytic infiltrate of adrenal cortex

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15
Q

Which conditions are typically associated with Autoimmune Addison’s disease?

A
Thyroid disease (20%)
T1DM (15%)
Premature ovarian failure (15%)
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16
Q

What are the common symptoms of primary adrenal failure?

A

Fatigue, weakness, anorexia, weightloss (100%)
Skin pigmentation/vitiligo
Hypotension
Unexplained Vom/diarrhoea

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17
Q

What are the rare symptoms of primary adrenal failure?

A

Salt craving

Postural symptoms

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18
Q

How does Primary Adrenal Failure cause skin pigmentation?

A

Excess ACTH co-stimulates melanocyte sytimulating hormone

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19
Q

Which other factors can give clues to adrenal failure?

A

Disproportionate illness severity and dehydration/hypotension
Unexplained hypoglycaemia
Other endocrine features
Previous depression

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20
Q

How is a diagnosis of adrenal insufficiency made?

A

Symptoms
Bloods
Cortisol (low)
Synacthen test

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21
Q

How do you treat a very unwell patient with high suspicion of Adrenal insufficiency?

A

Treat with steroids and do Synacthen test later

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22
Q

How do you treat a very unwell patient with high suspicion of Adrenal insufficiency?

A

Treat with steroids and do Synacthen test later

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23
Q

What is the short synacthen test?

A

Give 250ug Tetracosactrin
Push adrenal glands
Look at response
(Test for adrenal insufficiency)

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24
Q

How do you interpret cortisol levels in the short synacthen test?

A

Over 2hrs
>500nmol/l normal
<150nmol/l 2ndary adrenal insufficiency
<50nmol/l primary adrenal insufficiency

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25
Q

How do short synacthen test results give a diagnosis of primary adrenal failure?

A

Impaired cortisol response with an elevated ACTH

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26
Q

How do short synacthen test results give a diagnosis of secondary adrenal failure?

A

Lesser cortisol response

ACTH stays low

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27
Q

How is congenital adrenal hyperplasia diagnosis?

A

Samples of cortisol taken and tested for 17-OH progesterone to exclude 21-hydroxylase deficiency

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28
Q

What does a normal response to rapid ACTH stimulation test tell you?

A

Excludes primary adrenocortical insufficiency

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29
Q

What should you test after an abnormal ACTH stimulation test?

A

Test Plasma ACTH

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30
Q

What does suppressed ACTH tell us?

A

Secondary Adrenocortical insufficiency

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31
Q

What does elevated ACTH tell us?

A

Primary adrenocortical insufficiency

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32
Q

You diagnose a patient with Addison’s disease, what do you do next?

A

Check adrenal autoantibodies

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33
Q

What do you do with a patient with Addison’s disease and positive for adrenal autoantibodies?

A

Screen for other features of APS (autoimmune polyendocrine syndromes)

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34
Q

What do you do with a patient with Addison’s disease and negative for adrenal autoantibodies?

A

Men: Check VLCFA (FIRST)
- If -ve, adrenal imaging
Women: Adrenal imaging

35
Q

A patient with Addison’s disease, negative for adrenal autoantibodies and VLCFA positive has what?

A

Adrenoleucodystophy

36
Q

What factors are looked for in adrenal imaging?

A

Infiltation
Infarction
Haemorrhage
Infection

37
Q

What therapies are given for adrenal hyposecretion?

A

Glucocorticoid treatment

Mineralocorticoid replacement

38
Q

What is the typical glucocorticoid replacement therapy?

A

HYDROCORTISONE 20-30mg
Prednisolone 7.5mg
Dexamethasone 0.75mg
Divided doses to mimic diurnal variation

39
Q

What is the typical mineralocorticoid replacement therapy?

A

Synthetic steroid

Fludrocortisone 50-300ug

40
Q

Which patients are likely to need steroid therapy?

A

Hypoadrenal
Patients on steroids who suppress the pituitary adrenal axis
18/12 recent treatment

41
Q

How should steroid therapy be changed for patients with short-lived illness?

A

Double glucocorticoid dose

42
Q

How should steroid therapy be changed for patients with major illness/surgery?

A

100mg hydrocortisone IV

Reduce as stress abates

43
Q

What are the endocrine causes of hypertension?

A
Primary Hyperaldosteronism
Pheochromocytoma 
Cushing's disease
Acromegaly
Hyperparathyroidism
Hypothyroidism
Congenital Adrenal Hyperplasia
44
Q

What conditions of the adrenal cortex cause hypersecretion?

A

Cushing’s syndrome

Conn’s syndrome

45
Q

What conditions of the adrenal medulla cause hypersecretion?

A

Phaeochromocytoma

46
Q

Phaeochromocytoma causes secretion of what?

A

Catecholamines

47
Q

Cushing’s syndrome causes secretion of what?

A

Cortisol

Androgens

48
Q

Conn’s syndrome causes the secretion of what?

A

Aldosterone

49
Q

What is Cushing’s syndrome?

A

Excess corticosteroids

50
Q

What is the role of cortisol?

A

Tissue breakdown
Sodium retention
Insulin antagonism

51
Q

What are the symptoms of Cushing’s syndrome?

A
Central obesity
Hypertension
Glucose intolerance 
Moon face
Striae
Mood changes 
Amenorrhoea
52
Q

Cushing’s syndrome not caused by corticosteroid therapy is divided into which classes?

A

ACTH-dependent

ACTH-independent

53
Q

What are the causes of ACTH-dependent Cushing’s?

A
Pituitary tumour (Cushing's disease)
Ectopic ACTH secretion
54
Q

What type of tumour causes Cushing’s disease?

A

Basophil adenoma

55
Q

What are the causes of ACTH-independent Cushing’s?

A
Adrenal tumour (adenoma, carcinoma)
Primary nodular hyperplasia
56
Q

What is the initial screen for hypercortisolism?

A

Overnight Dexamethasone suppression test

24hr urine free cortisol

57
Q

How is Hypercortisolism confirmed?

A

24hr urine free cortisol

Low dose dexamethasone test

58
Q

How is ACTH dependent/independent hypercortisolism differentiated?

A

Paired morning-midnight ACTH cortisol

59
Q

If hypercortisolism is confirmed to be ACTH dependent, what is the next step?

A

Determination whether or not in the pituitary

High dose dexamethasone test

60
Q

How is an ACTH secreting tumour located?

A

MRI Sella
CT adrenals/chest
BIPSS

61
Q

What sources produce the most abnormal ACTH?

A

Ectopic sources

62
Q

What is the test for lateralising pituitary lesion?

A

Simultaneous bilateral inferior petrosal sinus and peripheral vein sampling

63
Q

What is the typical cause of Conn’s Syndrome?

A

Adenoma

Bilateral hyperplasia

64
Q

Elevated aldosterone causes what?

A

Elevated blood volume
Elevated blood pressure
Elevated urine K+
↓ Renin

65
Q

How is suspected hyperaldosteronism screened?

A

Plasma PA/PRA ratio

66
Q

What does a PA/PRA ratio > 20 tell us?

A

Primary hyperaldosteronism

67
Q

What does PA/PRA ratio < 20 tell us?

A

(Less reliable)
Secondary hyperaldosteronism
Essential Hypertension

68
Q

What do you test for in a patient with hypertension and hypokalemia?

A

Plasma renin activity (PRA)

Plasma aldosterone concentration (PAC)

69
Q

↑Renin ↑Aldosterone in a patient with hypertension and hypokalemia tells us what?

A

Investigate for Secondary hyperaldosteronism

70
Q

What are the causes of secondary hyperaldosteronism?

A
Renovascular hypertension
Diuretic use
Renin-secreting tumour
Malignant hypertension
Coarctation of the aorta
71
Q

↑Aldosterone (PAC) ↓Renin (PRA) in a patient with hypertension and hypokalemia tells us what?

A

Investigate for primary hyperaldosteronism

72
Q

↓Renin ↓Aldosterone in a patient with hypertension and hypokalemia tells us what?

A

Congenital adrenal hyperplasia
Exogenous mineralocorticoid
Cushings/Liddle’s
11 beta HSD deficiency

73
Q

How is hyperaldosteroneism confirmed?

A

Urinary Sodium
24hr urine aldosterone
4 days of salt loading

74
Q

How is the source of aldosterone found?

A

CT adrenals
Upright posture test
Plasma 18-hydroxycorticosterone

75
Q

What are the symptoms of phaeochromocytoma?

A

Hypertension

Paroxysmal attacks

76
Q

How do paroxysmal attacks present?

A
Headache
Sweating
Palpitations
Tremor 
Pallor
Anxiety
77
Q

What makes Phaeochromocytoma the 10% tumour?

A

10% extra adrenal
10% malignant
10% multiple
10% hyperglycaemic

78
Q

How do you test for phaeochromocytoma?

A

24hr urine:
Total metanephrines (solo in low suspicion)
Catecholamines
Plasma metanephrines

79
Q

What should be done next ina

patient with Increased metanephrines or catecholamines?

A

Localisation

Adrenal/abdominal MRI/CT

80
Q

What other tests should be performed in patients with phoechromocytoma?

A

Genetic testing

Tumour phenotype

81
Q

What must never be performed on a pheochromocytoma?

A

Biopsy

82
Q

What is the most common cause of congenital adrenal hyperplasia?

A

90% due to 21-hydroxylase deficiency

83
Q

How does Congenital adrenal hyperplasia present?

A

Neonatal salt-losing crisis
Ambiguous genitalia (girls)
Pseudo-precocious puberty (boys)
Hirsutism

84
Q

What hormone changes are seen in 21-hydroxylase deficiency?

A

Decreased aldosterone, cortisol

Increased sex hormones