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2MB Endocrinology > Adrenal Disease > Flashcards

Adrenal Disease Flashcards

1
Q

What are the two main adrenal medullary tumours?

A

Neuroblastoma

Phaeochromocytoma

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2
Q

From which cells is Phaeochromocytoma derived?

A

Chromaffin cells of adrenal medulla

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3
Q

What do Phaeochromocytoma secrete?

A

Catecholamines

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4
Q

Why is Phaeochromocytoma called the 10% tumour?

A

10% are extra-adrenal
10% are bilateral
10% are malignant
10% are not assoc. with hypertension

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5
Q

Which adrenal disorder is associated with dark/pigmented skin?

A

Addison’s disease

high ACTH

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6
Q

What are the main primary adrenal insufficiencies?

A

Addison’s disease
Congenital adrenal hyperplasia
Malignancy
Adrenal TB

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7
Q

What is the commonest cause of primary adrenal insufficiency?

A

Addison’s disease

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8
Q

Is Addison’s disease an autoimmune condition?

A

Yes

Assoc. with autoantibodies in 70%

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9
Q

List clinical features of Addison’s disease

A 
Dizziness
Low BP
Fatigue, lethargy
Anorexia, weight loss
Skin pigmentation
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10
Q

What would sodium and potassium levels be like in Addison’s disease?

A

Low sodium

High potassium

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11
Q

Which diagnostic test is used for Addison’s disease?

A

Short synACTHen test

give synthetic ACTH and measure cortisol levels

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12
Q

Outline management of adrenal insufficiency

A

Hydrocortisone 30mg

Fludrocortisone (replace aldosterone)

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13
Q

Patients on hydrocortisone for adrenal insufficiency can stop whenever they want. True/False?

A

False

Never stop suddenly but can reduce dose

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14
Q

List causes of secondary adrenal insufficiency

A

Pituitary/hypothalamic tumours

Exogenous steroid use (prednisolone)

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15
Q

What is the effect of exogenous steroid on the H-P-adrenal cortex axis?

A

Suppress CRH and ACTH

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16
Q

What hormone is in excess in Cushing’s syndrome?

A

Cortisol

17
Q

Cushing’s syndrome is commoner in men. True/False?

A

False

Females

18
Q

List clinical features of Cushing’s syndrome

A 
Moon-face
Abdominal striae
Thin skin, easy bruising
Proximal myopathy
Osteoporosis
19
Q

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

A

Pituitary disease/adenoma

Ectopic tumours

20
Q

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

A

Adrenal disease/adenoma

Nodular hyperplasia

21
Q

What is the gold-standard test for diagnosing Cushing’s syndrome?

A

Low-dose dexamethasone suppression test

0.5mg every 6hrs for 2 days; cortisol should be less than 50 in normal circumstances

22
Q

What is the commonest cause of cortisol excess?

A

High-dose steroid therapy (e.g. asthma, IBD)

23
Q

What is primary aldosteronism?

A

Autonomous production of aldosterone, independent of its regulators

24
Q

Which syndrome occurs due to aldosteronism?

A

Conn’s syndrome

25
Q

What is the commonest cause of secondary hypertension?

A

Primary aldosteronism

26
Q

Outline how an aldosterone excess is diagnosed

A

Measure aldosterone : renin ratio
If raised, do saline suppression test
Failure of aldosterone to suppress by 50% with 2L saline confirms aldosteronism

27
Q

What are the main causes of aldosteronism?

A

Adrenal hyperplasiaa
Adrenal adenoma
Genetic mutations

28
Q

Outline management of primary aldosteronism

A

Adrenalectomy if unilateral adenoma

Drug (spironolactone) if bilateral hyperplasia

29
Q

What is the commonest cause of congenital adrenal hyperplasia?

A

21-alpha-hydroxylase deficiency

30
Q

What 3 main effects does 21-alpha-hydroxylase deficiency cause?

A

Low cortisol
Low aldosterone
Excess androgens

31
Q

What is the classical triad of symptoms in Phaeochromocytoma?

A

Hypertension or postural hypotension
Headache
Sweating

32
Q

How is Phaeochromocytoma diagnosed?

A

MRI scan
MIBG
PET scan

33
Q

Outline therapy for phaeochromocytoma

A

Alpha blocker (phenoxybenzamine)
Beta blocker (propranolol/atenolol)
Fluid replacement
Surgical excision

34
Q

Phaeochromocytoma is assoc. with which clinical syndromes?

A 
MEN 2 (multiple endocrine neoplasia type 2)
Von-Hippel-Lindau syndrome
Succinate dehydrogenase mutations
Neurofibromatosis
Tuberose sclerosis
35
Q

Which gene is affected in MEN 1?

A

MEN1 gene (11q)

36
Q

Which gene is affected in MEN 2?

A

RET gene (10q)

37
Q

The MEN1 gene is a tumour suppressor. True/False?

A

True

38
Q

Is the RET gene a tumour suppressor or proto-oncogene?

A

Proto-oncogene

39
Q

Von-Hippel-Lindau syndrome is caused by mutation in which gene?

A

VHL gene

2MB Endocrinology (21 decks)

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