Adrenal Issues Flashcards

1
Q

What is Addison’s disease?

A

Autoimmune destruction of the adrenal glands

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2
Q

What is the commonest cause of hypoadrenalism in the UK

A

Addison’s disease
Autoimmune destruction of the adrenal glands is the commonest cause of primary hypoadrenalism in the UK, accounting for 80% of cases.

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3
Q

Addison’s disease and results in reduction of which hormones

A

reduced cortisol and aldosterone being produced.

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4
Q

Which electrolyte disturbances will occur as a result of Addison’s disease?

A

hyperkalaemia
hyponatraemia
hypoglycaemia
metabolic acidosis

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5
Q

Addison’s disease sx

A

lethargy, weakness, anorexia, nausea & vomiting, weight loss, ‘salt-craving’
hyperpigmentation (especially palmar creases)*, vitiligo, loss of pubic hair in women, hypotension
crisis: collapse, shock, pyrexia

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6
Q

Addisons disease causes hypo or hyperglycaemia

A

hypoglycaemia

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7
Q

What are the primary causes of hypoadrenalims?

A
primary addison's
tuberculosis
metastases (e.g. bronchial carcinoma)
meningococcal septicaemia (Waterhouse-Friderichsen syndrome)
HIV
antiphospholipid syndrome
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8
Q

What are the secondary causes of hypoadrenalims?

A

pituitary disorders (e.g. tumours, irradiation, infiltration)

Exogenous glucocorticoid therapy

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9
Q

Hypoadrenalism always causes hyperpigmentation

A

false

Primary Addison’s is associated with hyperpigmentation whereas secondary adrenal insufficiency is not

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10
Q

In a patient with suspected Addison’s disease the definite investigation is

A

ACTH stimulation test (short Synacthen test)

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11
Q

Describe ACTH simulation test

A

Plasma cortisol is measured before and 30 minutes after giving Synacthen 250ug IM. Adrenal autoantibodies such as anti-21-hydroxylase may also be demonstrated.

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12
Q

If an ACTH stimulation test is not readily available (e.g. in primary care) then what test can be useful:

A

9 am serum cortisol

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13
Q

Describe results of 9am serum cortisol for addisons

A

> 500 nmol/l makes Addison’s very unlikely
< 100 nmol/l is definitely abnormal
100-500 nmol/l should prompt a ACTH stimulation test to be performed

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14
Q

Patients who have Addison’s disease are usually given what therapy.

A

both glucocorticoid and mineralocorticoid replacement

hydrocortisone: usually given in 2 or 3 divided doses. Patients typically require 20-30 mg per day, with the majority given in the first half of the day
fludrocortisone

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15
Q

Describe patient education in Addison’s disease?

A

emphasise the importance of not missing glucocorticoid doses
consider MedicAlert bracelets and steroid cards
patients should be provided with hydrocortisone for injection with needles and syringes to treat an adrenal crisis
discuss how to adjust the glucocorticoid dose during an intercurrent illness

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16
Q

Management of intercurrent illness in addisons?

A

in simple terms the glucocorticoid dose should be doubled
the Addison’s Clinical Advisory Panel have produced guidelines detailing particular scenarios - please see the CKS link for more deta

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17
Q

Causes of addisonian crisis?

A

sepsis or surgery
adrenal haemorrhage eg Waterhouse-Friderichsen syndrome (fulminant meningococcemia)
steroid withdrawal

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18
Q

Outline management of addisonian crisis?

A

hydrocortisone 100 mg im or iv - continue hydrocortisone 6 hourly until the patient is stable.

1 litre normal saline infused over 30-60 mins or with dextrose if hypoglycaemic

No fludrocortisone is required because high cortisol exerts weak mineralocorticoid action
oral replacement may begin after 24 hours and be reduced to maintenance over 3-4 days

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19
Q

Describe the which hormones are released from the parts of the cortex?

A

Zona glomerulosa – produces and secretes mineralocorticoids such as aldosterone.

Zona fasciculata – produces and secretes corticosteroids such as cortisol. It also secretes a small amount of androgens.

Zona reticularis – produces and secretes androgens such as dehydroepiandrosterone (DHES). It also secretes a small amount of corticosteroids.

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20
Q

What is Cushing’s Syndrome?

A

is used to refer to the signs and symptoms that develop after prolonged abnormal elevation of cortisol.

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21
Q

What is Cushing’s disease?

A

specific condition where a pituitary adenoma (tumour) secretes excessive ACTH. Cushing’s Disease causes a Cushing’s syndrome, but Cushing’s Syndrome is not always caused by Cushing’s Disease.

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22
Q

Endogenous are the commonest causes of cushings disease

A

FALSE

exogenous causes of Cushing’s syndrome (e.g. glucocorticoid therapy) are far more common than endogenous ones.

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23
Q

ACTH dependent causes of cushings?

A

Cushing’s disease (80%): pituitary tumour secreting ACTH producing adrenal hyperplasia

ectopic ACTH production (5-10%): e.g. small cell lung cancer is the most common causes

24
Q

ACTH independent causes of cushings?

A

iatrogenic: steroids
adrenal adenoma (5-10%)
adrenal carcinoma (rare)
Carney complex: syndrome including cardiac myxoma
micronodular adrenal dysplasia (very rare)

25
What is Pseudo-Cushing's?
mimics Cushing's often due to alcohol excess or severe depression causes false positive dexamethasone suppression test or 24 hr urinary free cortisol
26
What test differentiates cushings and pseudocushings?
insulin stress test may be used to differentiate
27
How is cushings investigated?
Investigations are divided into confirming Cushing's syndrome and then localising the lesion.
28
Metabolic acidosis/alkalosis seen in cushings
hypokalaemic metabolic alkalosis
29
How does cushings affect glucose?
impaired glucose tolerance
30
Ectopic ACTH secretion (e.g. secondary to small cell lung cancer) is characteristically associated with
very low potassium levels.
31
Tests to confirm Cushing's syndrome
The two most commonly used tests are: overnight dexamethasone suppression test (most sensitive) 24 hr urinary free cortisol
32
Localisation test in Cushings?
first-line 9am and midnight plasma ACTH (and cortisol) levels Both low- and high-dose dexamethasone suppression tests may be used to localise the pathology resulting in Cushing's syndrome.
33
When measuring 9am and midnight plasma ACTH (and cortisol) levels - If ACTH is suppressed then what is most likely?
non-ACTH dependent cause is likely such as an adrenal adenoma
34
In cushings what would Normal results look like? (low dose, high dose dexamethasone & ACTH?)
Cortisol following low-dose dexamethasone: Low Cortisol following high-dose dexamethasone: Low ACTH: Normal
35
What would Cushing's syndrome due to other causes (e.g. adrenal adenomas) show on localisation tests?
Cortisol following low-dose dexamethasone: Normal Cortisol following high-dose dexamethasone: Normal ACTH: Low
36
What would Cushing's disease (i.e. pituitary adenoma → ACTH secretion) show on localisation tests?
Cortisol following low-dose dexamethasone: Normal Cortisol following high-dose dexamethasone: Low ACTH: High
37
What would Ectopic ACTH syndrome likely show on localisation tests?
Cortisol following low-dose dexamethasone: Normal Cortisol following high-dose dexamethasone: Normal ACTH: High
38
What can differentiate between pituitary and ectopic ACTH secretion?
Petrosal sinus sampling of ACTH
39
In Cushing's what would CRH stimulation tests show?
if pituitary source then cortisol rises | if ectopic/adrenal then no change in cortisol
40
Primary hyperaldosteronism most commonly caused by?
bilateral idiopathic adrenal hyperplasia is the cause in up to 70% of cases
41
Primary hyperaldosteronism extremely rare cause?
Adrenal carcinoma
42
Features of Primary hyperaldosteronism?
``` hypertension hypokalaemia e.g. muscle weakness this is a classical feature in exams but studies suggest this is seen in only 10-40% of patients alkalosis ```
43
What is first line investigation for Primary hyperaldosteronism? What would this show?
plasma aldosterone/renin ratio is the first-line investigation should show high aldosterone levels alongside low renin levels (negative feedback due to sodium retention from aldosterone)
44
What other tests should be done in Primary hyperaldosteronism?
plasma aldosterone/renin ratio is the first-line investigation following this a high-resolution CT abdomen and adrenal vein sampling is used to differentiate between unilateral and bilateral sources of aldosterone excess Adrenal Venous Sampling (AVS) can be done to identify the gland secreting excess hormone in primary hyperaldosteronism
45
Primary hyperaldosteronism mx
adrenal adenoma: surgery | bilateral adrenocortical hyperplasia: aldosterone antagonist e.g. spironolactone
46
What is Phaeochromocytoma
rare catecholamine secreting tumour
47
Phaeochromocytoma inheritance
10% are familial
48
Phaeochromocytoma associations?
MEN type II, neurofibromatosis and von Hippel-Lindau syndrome
49
10% rules in phaeo?
bilateral in 10% malignant in 10% extra-adrenal in 10% (most common site = organ of Zuckerkandl, adjacent to the bifurcation of the aorta)
50
Features in phaeo are typically
episodic
51
sx phaeo
``` hypertension (around 90% of cases, may be sustained) headaches palpitations sweating anxiety ```
52
24 hr urinary collection of catecholamines test for phaeo
false | metanephrines
53
test for phaeo?
24 hr urinary collection of metanephrines (sensitivity 97%*)
54
Surgery is first line in phaeo
false | stabilized with medical management
55
Outline management in Phaeo?
patient must first however be stabilized with medical management: alpha-blocker (e.g. phenoxybenzamine), given before a beta-blocker (e.g. propranolol) (a before b) Surgery is the definitive management
56
What is Von Hippel-Lindau (VHL) syndrome?
autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3
57
Features of VHL?
haemangiomas manifesting in subarachnoid haemorrhages, vitreous haemorrhage phaeochromocytoma clear-cell renal cell carcinoma Cysts: renal cysts (premalignant), extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours