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
  • Is additional Chemotherapy or radiotherapy required
  • How can we follow the course of the disease
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4
Q

What can cause hyposecretion/ primary adrenal insufficiency?

A
  • 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%)
  • T1DM (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) in 70% cases

- 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 / hypotension / dehydration
  • Unexplained hypoglycaemia
  • Other endocrine features (hypothyroidism, body hair loss, amenorrhoea)
  • Previous depression or weight loss
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12
Q

What investigations should be carried out for adrenal insufficiency?

A
  • Routine blood tests: U+Es, glucose and FBCs
  • Random cortisol
    • > 700nmol/l (not Addison’s)
    • <700nmol/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

  • Hydrocortisone (20-30mg )
  • Prednisolone (7.5mg)
  • Dexamethasone (0.75mg)

-Given in divided doses to ‘mimic normal diurnal variation’

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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 (>7.5mg prednisolone daily, or equivalent)
  • Patients who have received such treatment during the previous 18/12 (HPA axis may still be suppressed)
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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 in event of intercurrent illness (eg flu, UTI)
  • If severe vomiting or diarrhoea, call for help without delay (likely to need IM hydrocortisone - some patients or their partners are taught to inject)
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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 - Hypertension - Glucose intolerance - Amenorrhoea or impotency - Purple striae - Plethoric faeces - Easy bruisability - Osteoporosis - Personality changes - Acne - Oedema - Headache - Poor wound healing
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 ration>20 is primary hyperaldosteronism | - PA/PRA ratio <20 is 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 and urinary sodium >200mEq/day during 4 days of salt loading
42
How is the aldosterone source established in hyperaldesteronism ?
- CT scan od 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 hyperplasea
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)