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7. Endocrinology (Medical Knowledge)a > Clinical Aspects of Adrenal Disorders > Flashcards

Clinical Aspects of Adrenal Disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

What does each layer of the adrenal glands secrete?

A
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2
Q

What are the classification of adrenal disorders?

A
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3
Q

Explain the common approach to treating adrenal disorders?

A
  • clinical suspicion
  • test for assessing functional status: functioning? primary or secondary? aetiology?
  • if tumour: resectable? chemotherapy/radiotherapy help?
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4
Q

What does HPA stand for?

A

Hypothalamus-pituitary-adrenal axis

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5
Q

What impact does hypofunction have on the HPA?

A
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6
Q

What can hypofunction of the adrenal glands be caused by?

A

primary adrenal insufficiency

  • addison’s disease: immune destruction, invasion, infiltration, infection, infarction, iatrogenic

adrenal enzyme defect

  • congenital adrenal hyperplasia: most commonly 21-hydroxylase deficiency
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7
Q

What are some causes of Addison’s disease?

A

Immune destruction

Invasion

Infiltration

Infection

Infarction

Iatrogenic

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8
Q

What enzyme most commonly causes adrenal enzyme defect?

A

21-hydroxylase deficiency

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9
Q

What does adrenal enzyme defect cause?

A

Congenital adrenal hyperplasia

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10
Q

What autoantibodies are looked for in Addison’s disease?

A

Positive adrenal autoantibodies to 21-hydroxylase

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11
Q

How is the adrenal cortex involved in Addison’s disease?

A

Lymphatic infiltrate of adrenal cortex

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12
Q

Addison’s disease is often associated with other autoimmune disease, what are some examples?

A

Thyroid disease

Type 1 diabetes mellitus

Premature ovarian failure

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13
Q

What are some common symptoms of primary adrenal failure?

A
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14
Q

What are some clues to a diagnosis of adrenal failure?

A
  • disproportion between severity of illness and hypotension
  • unexplained hypoglycaemia
  • other endocrine features: hypothyroidism, body hair loss, amenorrhoea
  • previous depression or weight loss
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15
Q

What is the most common cause of adrenal failure?

A

Autoimmune Addison’s disease

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16
Q

What is done to diagnose adrenal insuffiency?

A

Routine bloods (U and Es, glucose, FBC)

Random cortisol

Synacthen test (and basal ACTH)

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17
Q

What routine bloods are done when investigating adrenal insuffiency?

A

U and Es

Glucose

FBC

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18
Q

For a random cortisol test, what level is not an Addison’s diagnosis?

A

>550nmol/L

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19
Q

For a random cortisol test, what level is potentially an Addison’s diagnosis?

A

<500nmol/L

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20
Q

Is Addison’s disease a cause of hyper or hyposecretion of the adrenal glands?

A

Hyposecretion

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21
Q

What is the synacthen test?

A

Special chemical test to see how well adrenal glands make cortisol

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22
Q

Explain the process of diagnosing adrenocortical insuficiency?

A
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23
Q

What medicines can be used as glucocorticoid replacement?

A
  • Hydrocortisone 20-30mg
  • Prednisolone 7.5mg
  • Dexamethasone 0.75mg
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24
Q

What can be used as mineral corticoid replacement?

A

Synthetic steroid called fludrocortisone

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25
Q

What does fludrocortisone bind to?

A

Aldosterone receptors

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26
Q

When prescribing people steroids, who needs special care?

A
  • Hypoadrenal patients on replacement steroids
  • Patients on steroids in doses sufficient to suppress the pituitary adrenal axis (>7.5mg prednisolone daily or equivalent)
  • Patients who have received such treatment during the previous 18/12
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27
Q

What action should be taken for people you have prescribed steroids too who are now suffering from a minor short lived illness or stress?

A

Double glucocorticoid dose

28
Q

What action should be taken for people you have prescribed steroids too who are now suffering from a major illness or operation?

A
  • 100mg hydrocortisone IV stat
  • 50-100mg HC IV 8-hourly
29
Q

What are 3 important self-care rules for patients on steroids?

A
  • never miss steroid dose
  • double hydrocortisone dose when ill (flu or UTI)
  • if severe vomiting or diarrhoea call for help without delay
30
Q

What are some endocrine causes of hypertension?

A
  • primary hyperaldosteronism: unilateral adenoma, bilateral hyperplasia
  • pheochromocytoma
  • cushing’s syndrome
  • acromegaly
  • hyperparathyroidism
  • hypothyroidism
  • congenital adrenal hyperplasia
31
Q

What are some causes of hypersecretion of adrenal glands?

A

cortex

  • cushing’s syndrome (cortisol, androgens): adenoma, carcinoma or bilateral hyperplasia
  • conn’s syndrome (aldosterone): adenoma or bilateral hyperplasia

medulla

  • phaeochromocytoma (catecholamines)
32
Q

What does Cushing’s syndrome cause hypersecretion of?

A

Cortisol, androgens

33
Q

What does Conn’s syndrome cause hypersecretion of?

A

Aldosterone

34
Q

What are some causes of primary Cushing’s syndrome?

A

Adenoma, carcinoma or bilateral hyperplasia

35
Q

What are some causes of primary Conn’s syndrome?

A

Adenoma or bilateral hyperplasia

36
Q

What is a cause of hypersecretion from the adrenal medulla?

A

Phaeochromocytoma

37
Q

What is the clinical presentation of Cushing’s syndrome?

A
38
Q

What are some ACTH dependent causes of Cushing’s syndrome?

A
  • Pituitary tumour (Cushing’s disease)
    • 75% of cases
  • Ectopic ACTH secretion (such as lung carcinoid)
    • 5% of cases
39
Q

What are some ACTH indendent causes of Cushing’s syndrome?

A

adrenal tumour (20%)

  • adenoma or carcinoma
40
Q

Explain the approach to hypercortisolism?

A
41
Q

What are some examples of screening tests for Cushing’s syndrome?

A
  • 24 hour urinary free cortisol
    • Normal 14-135nmol/24 hour
  • 1mg overnight dexamethasone suppression test taken at midnight
    • Normal <50nmol/L at 9am
42
Q

How do levels of cortisol and ACTH normally compare?

A

Concentration levels mimic each other over the course of the day

43
Q

What is Conn’s syndrome also known as?

A

Primary aldosteronism

44
Q

What does RAAS stand for?

A

renin-angiotensin-aldosterone system

45
Q

What system is involved in the pathophysiology of primary hyperaldosteronism (Conn’s disease)?

A

RAAS

46
Q

Explain the pathophysiology of Conn’s syndrome?

A

Aldosterone producing tumour causes increased blood volume, blood pressure and urine potassium

47
Q

What is used to screen for Conn’s syndrome?

A

Aldosterone to renin ratio?

48
Q

What is the cut off for the aldosterone to renin ratio for primary hyperaldosteornism?

A

Ratio >20

49
Q

What does it mean if aldosterone to renin ratio is <20?

A

Secondary hyperaldosteronism

Essential hypertension

(not primary hyperaldosteronism)

50
Q

What does PRA stand for?

A

Plasma renin activity

51
Q

What does PAC stand for?

A

Plasma aldosterone concentration

52
Q

What are causes of increased plasma renin activity (PRA) and increased plasma aldosterone concentration (PAC)?

A
53
Q

What are causes of decreased plasma renin activity (PRA) and increased plasma aldosterone concentration (PAC)?

A
54
Q

What are causes of decreased plasma renin activity (PRA) and decreased plasma aldosterone concentration (PAC)?

A
55
Q

Explain what happens after clinical suspicion of hyperaldosteronism to diagnose it?

A
56
Q

What screening test are done after clinical suspicion of hyperaldosteronism?

A

Plasma renin activity (PRA)

Plasma aldosterone concentration (PAC)

Look at PAC/PRA ratio, >20 means primary aldosterone

57
Q

What is a confirmatory test for hyperadosteornism?

A

24 hour urine aldosterone

58
Q

What investigations are done to establish the aldosterone source in hyperaldosteronism?

A

CT scan of adrenal glands

Upright posture test

Plasma 18-hydroxycorticosterone

59
Q

What is phaeochromocytoma?

A

Rare tumour of adrenal gland tissue that causes release of epinephrine and norepinephrine

60
Q

What does phaeochromocytoma cause the hypersecretion of?

A

Epinephrine and norepinephrine

61
Q

What is the clinical presentation of phaeochromocytoma?

A
  • Hypertension
  • Paroxysmal attacks
    • Headache
    • Sweating
    • Palpitations
    • Tremor
    • Pallor
    • Anxiety
62
Q

Is phaeochromocytoma possible due to inherited origin?

A

30% of the time

63
Q

What genes and familial syndromes are responsible for genetic phaeochromocytoma?

A
  • Genes
    • RET, VHL
  • Familial syndromes
    • MEN2
64
Q

Explain the process of investigating an adrenal mass?

A

1) Clinical suspicion
2) Endocrine workout

  • Free metanephrine in plasma or urine
  • ALD/PRA ratio

3) Imaging work up

  • CT
  • Chemical shift MRI
65
Q

What is congenital adrenal hyperplasia most often due to?

A

In >90% of cases is due to 21-hydroxylase deficiency

66
Q

What do severe cases of congenital adrenal hyperplasia cause?

A

Neonatal salt crises

Ambiguous genitalia (girls)

7. Endocrinology (Medical Knowledge)a (28 decks)

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