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
What is the most common cause of Cushing's syndrome?
Exogenous steroid therapy (particularly those given orally)
26
Why should long-term steroids be tapered upon cessation?
As abrupt withdrawal may trigger an adrenal crisis.
27
What are endocrinological causes of hypertension?
Hyperaldosteronism Phaeochromocytoma Cushing's syndrome Acromegaly
28
What would hypertension and hypokalaemia suggest?
Primary aldosteronism Will present with metabolic alkalosis.
29
What is primary aldosteronism?
The autonomous production of aldosterone, independent of angiotensin II and potassium.
30
What is the most common cause of primary aldosteronism?
Bilateral adrenal hyperplasia
31
How is bilateral adrenal hyperplasia managed?
Spironolactone
32
What is the most common cause of congenital adrenal hyperplasia?
21alpha-hydroxylase deficiency Has an autosomal recessive inheritance pattern - affected individuals will have ambiguous genitalia.
33
How does partial 21alpha-hydroxylase deficiency present?
Hirsutism Menstrual disturbance Infertility Affected individuals are usually female - condition is a result of the inability to convert testosterone to progesterone.
34
How is CAH treated?
Glucocorticoid and mineralocorticoid therapy as can cause adrenal insufficiency. Treat abnormal genitalia via surgery.
35
What is the name of a phaeochromocytoma-like tumor developed outwith the adrenal medulla?
Paraganglioma These may occur anywhere along the sympathetic chain.
36
How can phaeochromocytoma present?
Labile hypertension Postural hypertension Paroxysmal sweating, headache, pallor, and tachycardia
37
What genetic disorders are associated with phaeochromocytoma?
Men2a Men2b Von-Hippel Lindau syndrome
38
At what times may a phaeochromocytoma produce a hypertensive crisis?
Pregnancy Surgery
39
How should phaeochromocytoma be investigated?
Identify catecholamine excess MRI/PET/MIBG scan
40
How is phaeochromocytoma treated?
Surgery - give alpha and beta blockers. After procedure, give fluid/blood replacement.
41
What is an example of an alpha blocker?
Phenoxybenzamine
42
What controls water balance?
ADH
43
What controls sodium balance?
Steroid hormones
44
What is the role of ADH?
To bring water back in, which would otherwise be lost.
45
If ADH levels are high, what is the result?
A small volume of concentrated urine.
46
If ADH is absent, or the kidneys aren't working properly, what is the result?
A large volume of dilute urine is produced.
47
What form of urine has high osmolarity?
Concentrated urine
48
What form of urine has low osmolarity?
Dilute urine
49
What is mineralocorticoid activity?
The reabsorption of sodium in the renal tubules, in exchange for potassium or hydrogen.
50
What can cause a low sodium:water balance?
Too much water Too little sodium
51
Which compartment is sodium restricted to?
ECF Remember water follows solute.
52
What is the most common cause of water overload hyponatraemia?
SiADH
53
What is the most common cause of hypernatraemia?
Insufficient water intake
54
What are causes of reduced sodium?
Adrenal insufficiency Gut (diarrhoea and vomiting) Skin (burns) Decreased intake
55
What are causes of increased water?
SiADH Excessive water intake
56
What are causes of hypernatraemia?
Too little water Too much sodium
57
What causes increased sodium?
Increased dietary intake
58
What are causes of decreased water levels?
Diabetes insipidus Reduced water intake
59
What is given to manage those with too little water?
Dextrose 5%
60
How can water be removed from the body?
Loop diuretics
61