Clinical Aspects of the Adrenal Glands Flashcards

1
Q

What types of adrenal disorders are there?

A
  • Hyperfunction
  • Hypofunction
  • Tumours
  • Functional symptoms
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2
Q

What is the common approach taken towards adrenal disorders?

A

clinical suspicion

test for assessing functional status

  • is it functioning
  • is it primary or secondary

what is the aetiology
- is it a tumour

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3
Q

What questions should you be thinking of if you suspect someone has an adrenal tumour?

A
  • can it be removed
  • additional chemotherapy/radiotherapy required
  • how can we follow the course of the disease
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4
Q

what can cause adrenal hypofunction

A

primary adrenal insufficiency
- addison’s disease

adrenal enzyme defects

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5
Q

Give an example of an adrenal enzyme defect which can result in primary adrenal insufficiency.

A

Congenital adrenal hyperplasia (most commonly 21-hydroxylase deficiency)

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6
Q

What can cause Addison’s disease?

A
  • Immune destruction (auto)
  • Invasion
  • Infiltration
  • Infection
  • Infarction
  • Iatrogenic
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7
Q

What is the greatest cause of adrenal failure in the UK?

A

> 85% caused by autoimmune Addison’s

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8
Q

What associated autoimmune diseases are common with Addison’s disease?

A
  • thyroid disease (20%)
  • type 1 DM (15%)
  • premature ovarian failure (15%)
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9
Q

What pathophysiology is found in Addison’s disease?

A
  • +ve adrenal autoantibodies to 21-OHase

- lymphocytic infiltrate of adrenal cortex

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10
Q

What are the common symptoms associated with Addison’s disease?

A
  • weakness, fatigue, anorexia, weight loss (100%)
  • skin pigmentation or vitiligo (92%)
  • hypotension (88%)
  • unexplained vomiting or diarrhoea (56%)
  • salt craving (19 %)
  • postural symptoms (12%)
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11
Q

What are the possible clues to the diagnosis of adrenal failure?

A
  • disproportion between severity of illness & circulatory collapse
  • unexplained hypoglycaemia
  • previous depression or weight loss
  • other endocrine features: hypothyroidism, body hair loss, amenorrhoea
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12
Q

What investigations should be carried out for adrenal insufficiency?

A

routine blood tests

  • U+Es
  • glucose
  • FBCs

random cortisol

  • > 450 nmol/l (not Addison’s)
  • <450nmol/l (adrenal status uncertain)
syacthen test (and basal ACTH)
-If suspicion high & patient unwell, treat with steroids and do Synacthen test later
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13
Q

How should adrenal insufficiency be treated?

A

glucocorticoid replacement

  • given in divided doses to ‘mimic normal diurnal variation’
  • hydrocortisone (20-30mg )
  • prednisolone (7.5mg)
  • dexamethasone (0.75mg)

mineralocorticoid replacement
- fludrocortisone (synthetic steroid)

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14
Q

What is fludrocortisone?

A

Synthetic steroid

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15
Q

What does fludrocortisone bind to?

A

Mineralocorticoid (aldosterone) receptors

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16
Q

How should fludrocortisone be administered?

A

50-300 micrograms daily

adjust dose according to:

  • clinical status (postural BP, oedema)
  • U&E
  • plasma renin level
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17
Q

Who needs special care when it comes to steroids and stress?

A
  • hypoadrenal patients on replacement steroids
  • patients on steroids in doses sufficient to suppress the pituitary adrenal axis
  • patients who have received such treatment during the previous 18/12
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18
Q

How should patients who require special care be managed with a short lived illness or stress?

A

Double glucocorticoid dose

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19
Q

How should patients who require special care be managed with a major illness or operation?

A
  • Especially if nil by mouth or GI upset
  • 100mg hydrocortisone iv stat
  • 50-100mg HC iv 8-hourly
  • As stress abates, reduce HC by 50% per day until back on usual replacement dose
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20
Q

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

A
  • never miss steroid doses
  • double the hydrocortisone dose
  • if severe vomiting or diarrhoea, call for help without delay
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21
Q

Abnormalities of aldosterone can lead to?

A

Hypertension

22
Q

What are the endocrine causes of hypertension?

A

primary hyperaldosteronism

  • unilateral adenoma
  • bilateral hyperplasia

rarer causes

  • phaeochromcytoma
  • cushing’s syndrome
  • acromegaly
  • hyperparathyroidism
  • hypothyroidism
  • congenital adrenal hyperplasia
23
Q

What disorders are associated with hypersecretion of the adrenal cortex

A
  • Cushing’s syndrome

- Conn’s syndrome

24
Q

What condition is associated with hypersecretion of the adrenal medulla?

A

Pheochromocytoma

25
What is Cushing's syndrome?
Excess corticosteroids
26
What does Cushing's syndrome cause?
Tissue breakdown -causes weakness of skin, muscle & bone Sodium retention -may cause hypertension & heart failure Insulin antagonism -may cause diabetes mellitus
27
What signs or symptoms may be present in Cushing's syndrome?
- central obesity - purple striae - plethoric faeces - osteoporosis - oedema - acne - easy bruisability - headaches - personality changes - hypertension - glucose intolerance - amenorrhoea or impotency
28
What are the ACTH dependent causes of Cushing's syndrome?
75% cases: pituitary tumour (Cushing’s disease) 5% cases: ectopic ACTH secretion (eg lung carcinoid)
29
What are the ACTH independent causes of Cushing's disease?
- 20% cases: adrenal tumour (adenoma or carcinoma) | - corticosteroid therapy (eg for asthma, IBD)
30
How is hypercortisolism screened for?
- 1mg overnight DEX suppression test | - 24hr urine free cortisol
31
How is hypercortisolism diagnosed?
- 24hr urine free cortisol | - low dose Dex test
32
How is it determined if hypercortisolism is ACTH dependent or independent?
Paired morning-midnight ACTH cortisol
33
How is it determined if the pituitary is involved in hypercortisolism?
High dose Dex test
34
How is the cause of hypercortisolism localised?
- MRI sella - CT adrenal - BIPSS - CT chest
35
What is the pathophysiology of primary hyperaldosteronism?
- aldosterone producing tumour - increase in blood volume, BP and urine K - decrease in renin - increase in A/R ratio
36
How is primary and secondary hyperaldosteronism differentiated?
PA/PRA >20 : primary hyperaldosteronism | PA/PRA <20 : secondary hyperaldosetronism
37
What can cause increased PRA and PAC?
Investigate for causes of secondary hyperaldosteronism - Renovascular hypertension - Diuretic use - Renin- secreting tumour - Malignant -Hypertension - Coarctation of Aorta
38
What can cause decreased PRA with increased PAC?
Investigate for causes of Primary hyperaldosteronism
39
What can cause decreased PRA and PAC?
- congenital adrenal hyperplasia - exogenous mineralocorticoid - DOC-producing tumour - Cushing’s syndrome - 11 beta HSD deficiency - Liddle’s syndrome
40
What are the screening tests for hyperaldosteronism?
- Plasma renin activity (PRA) and plasma aldosterone concentration (PAC) - PAC/PRA> 20 with concomitant serum aldosterone >10ng/dl
41
What are the confirmatory tests for hyperaldosteronism?
24hr urine aldosterone: >12ug/day urinary sodium: >200mEq/day during 4 days of salt loading
42
How is the aldosterone source established in hyperaldesteronism ?
- CT scan of adrenal glands - upright posture test - plasma 18-hydroxycorticosterone - adrenal venous sampling if CT scan is inconclusive or discordant with posture test
43
What is another name for hyperaldosteronism?
Conn's syndrome
44
What can cause Cushing's syndrome?
- adenoma - carcinoma - bilateral hyperplasia
45
What can cause Conn's syndrome
- Adenoma | - Bilateral hyperplasia
46
Why is pheochromocytoma referred to as the 10% tumour?
- 10% extra-adrenal - 10% malignant - 10% multiple - 10% hyperglycaemia (30% inherited origin)
47
Pheochromocytoma is associated with paroxysmal attacks of....
- Headache - Sweating - Palpitations - Tremor - Pallor - Anxiety/fear
48
What is persistent in 70% of pheochromocytoma patients?
Hypertension
49
What are >90% of congenital adrenal hyperplasia cases due to?
21-hydroxylase deficiency
50
How can severe cases of congenital adrenal hyperplasia present?
- Neonatal salt-losing crisis | - Ambiguous genitalia (girls)
51
What incomplete defects can occur in congenital adrenal hyperplasia?
- Pseudo-precocious puberty (boys) | - Hirsutism (women)