Adrenal Disease Flashcards

1
Q

Why do adrenal carcinomas have a poor prognosis?

A

Symptoms do not appear until nerves have become involved - this occurs late in disease progression pathway.

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2
Q

What features suggest an adrenal carcinoma, rather than an adenoma?

A

Large size
Presence of haemorrhage/necrosis
Frequent and atypical mitoses
A lack of clear cytoplasm
Capsular/vascular invasion

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3
Q

How is adrenal function affected in long-term steroid therapy?

A

It will be reduced (as ACTH release is suppressed by these steroids).

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4
Q

What are causes of acute adrenocortical insufficiency?

A

Rapid removal of long-term steroids
Haemorrhage
Disseminated intravascular coagulation
Sepsis

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5
Q

What is the most common cause of chronic adrenocortical insufficiency?

A

Addison’s disease

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6
Q

What is a neuroblastoma?

A

A cancer usually diagnosed in the first 18 months of life.

Around 40% of these affect the adrenal medulla, however can develop anywhere along the sympathetic chain.

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7
Q

What are phaeochromocytomas?

A

Tumours derived from chromaffin cells in the adrenal medulla.

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8
Q

What is secreted by phaechromocytomas?

A

Catecholamines, causing hypertension.

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9
Q

How does aldosterone raise BP?

A

It increases the rate of salt and water resorption, increasing the volume of blood.

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10
Q

How do steroids work?

A

They act on nuclear receptors, interacting with DNA directly. Produces a change in the way genes are expressed.

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11
Q

What is the role of mineralocorticoids?

A

Regulate the body’s sodium/potassium balance, blood pressure regulation and the maintenance of extracellular volume.

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12
Q

What are primary causes of adrenal insufficiency?

A

Addison’s disease (most common)
Congenital adrenal hyperplasia
Adrenal infection
Malignancy

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13
Q

What are secondary causes of adrenal insufficiency?

A

A lack of ACTH production
Iatrogenic (due to excess exogenous steroids which inhibit CRH and ACTH)
Pituitary/hypothalamic disorders

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14
Q

What is Sheehan’s syndrome?

A

A potentially life-threatening condition in which, following labour, a mother loses a significant amount of blood, producing damage to the pituitary gland.

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15
Q

What is Addison’s disease?

A

Autoimmune destruction of the adrenal cortex.

Symptoms present when >90% of the gland has been destroyed.

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16
Q

How does Addison’s disease present within electrolyte sample?

A

High potassium
Low sodium

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17
Q

How does Addison’s disease show on a synacthen test?

A

ACTH will be very high, renin will be raised and aldosterone will be low.

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18
Q

How is adrenal insufficiency treated?

A

Give hydrocortisone to replace cortisol, and fludrocortisone to mimic aldosterone.

During this, ensure to monitor BP and potassium levels.

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19
Q

How should adrenal-insufficient patients adjust their steroid dose when unwell?

A

Increase dose

At no point should steroid replacement ever be stopped.

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20
Q

How can primary and secondary adrenal insufficiency be differentiated?

A

Secondary adrenal insufficiency will not cause hyperpigmentation (as there is no excess ACTH).

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21
Q

Why is hydrocortisone replacement NOT given in some patients?

A

No hyperpigmentation signals that there is no shortage of aldosterone as an excess of ACTH is not present, hence hydrocortisone therapy is not indicated.

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22
Q

What are common features of Cushing’s syndrome?

A

Easy bruising
Facial plethora
Skin striae
Proximal myopathy
Central obesity

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23
Q

What is Cushing’s often dependent on?

A

ACTH (as this is needed to produce cortisol).

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24
Q

What tests can be used to diagnose Cushing’s syndrome?

A

Overnight dexamethasone suppression test
24hr urinary free cortisol
Low dose dexamethasone suppression test (GOLD standard)

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25
Q

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

A

Exogenous steroid therapy (particularly those given orally)

26
Q

Why should long-term steroids be tapered upon cessation?

A

As abrupt withdrawal may trigger an adrenal crisis.

27
Q

What are endocrinological causes of hypertension?

A

Hyperaldosteronism
Phaeochromocytoma
Cushing’s syndrome
Acromegaly

28
Q

What would hypertension and hypokalaemia suggest?

A

Primary aldosteronism

Will present with metabolic alkalosis.

29
Q

What is primary aldosteronism?

A

The autonomous production of aldosterone, independent of angiotensin II and potassium.

30
Q

What is the most common cause of primary aldosteronism?

A

Bilateral adrenal hyperplasia

31
Q

How is bilateral adrenal hyperplasia managed?

A

Spironolactone

32
Q

What is the most common cause of congenital adrenal hyperplasia?

A

21alpha-hydroxylase deficiency

Has an autosomal recessive inheritance pattern - affected individuals will have ambiguous genitalia.

33
Q

How does partial 21alpha-hydroxylase deficiency present?

A

Hirsutism
Menstrual disturbance
Infertility

Affected individuals are usually female - condition is a result of the inability to convert testosterone to progesterone.

34
Q

How is CAH treated?

A

Glucocorticoid and mineralocorticoid therapy as can cause adrenal insufficiency.

Treat abnormal genitalia via surgery.

35
Q

What is the name of a phaeochromocytoma-like tumor developed outwith the adrenal medulla?

A

Paraganglioma

These may occur anywhere along the sympathetic chain.

36
Q

How can phaeochromocytoma present?

A

Labile hypertension
Postural hypertension
Paroxysmal sweating, headache, pallor, and tachycardia

37
Q

What genetic disorders are associated with phaeochromocytoma?

A

Men2a
Men2b
Von-Hippel Lindau syndrome

38
Q

At what times may a phaeochromocytoma produce a hypertensive crisis?

A

Pregnancy
Surgery

39
Q

How should phaeochromocytoma be investigated?

A

Identify catecholamine excess
MRI/PET/MIBG scan

40
Q

How is phaeochromocytoma treated?

A

Surgery - give alpha and beta blockers.

After procedure, give fluid/blood replacement.

41
Q

What is an example of an alpha blocker?

A

Phenoxybenzamine

42
Q

What controls water balance?

A

ADH

43
Q

What controls sodium balance?

A

Steroid hormones

44
Q

What is the role of ADH?

A

To bring water back in, which would otherwise be lost.

45
Q

If ADH levels are high, what is the result?

A

A small volume of concentrated urine.

46
Q

If ADH is absent, or the kidneys aren’t working properly, what is the result?

A

A large volume of dilute urine is produced.

47
Q

What form of urine has high osmolarity?

A

Concentrated urine

48
Q

What form of urine has low osmolarity?

A

Dilute urine

49
Q

What is mineralocorticoid activity?

A

The reabsorption of sodium in the renal tubules, in exchange for potassium or hydrogen.

50
Q

What can cause a low sodium:water balance?

A

Too much water
Too little sodium

51
Q

Which compartment is sodium restricted to?

A

ECF

Remember water follows solute.

52
Q

What is the most common cause of water overload hyponatraemia?

A

SiADH

53
Q

What is the most common cause of hypernatraemia?

A

Insufficient water intake

54
Q

What are causes of reduced sodium?

A

Adrenal insufficiency
Gut (diarrhoea and vomiting)
Skin (burns)
Decreased intake

55
Q

What are causes of increased water?

A

SiADH
Excessive water intake

56
Q

What are causes of hypernatraemia?

A

Too little water
Too much sodium

57
Q

What causes increased sodium?

A

Increased dietary intake

58
Q

What are causes of decreased water levels?

A

Diabetes insipidus
Reduced water intake

59
Q

What is given to manage those with too little water?

A

Dextrose 5%

60
Q

How can water be removed from the body?

A

Loop diuretics

61
Q
A