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
Q

What is Cushing’s syndrome?

A

Excess corticosteroids

26
Q

What does Cushing’s syndrome cause?

A

Tissue breakdown
-causes weakness of skin, muscle & bone

Sodium retention
-may cause hypertension & heart failure

Insulin antagonism
-may cause diabetes mellitus

27
Q

What signs or symptoms may be present in Cushing’s syndrome?

A
  • Central obesity
  • Hypertension
  • Glucose intolerance
  • Amenorrhoea or impotency
  • Purple striae
  • Plethoric faeces
  • Easy bruisability
  • Osteoporosis
  • Personality changes
  • Acne
  • Oedema
  • Headache
  • Poor wound healing
28
Q

What are the ACTH dependent causes of Cushing’s syndrome?

A

75% cases:Pituitary tumour (Cushing’s disease)

5% cases: Ectopic ACTH secretion (eg lung carcinoid)

29
Q

What are the ACTH independent causes of Cushing’s disease?

A
  • 20% cases: Adrenal tumour (adenoma or carcinoma)

- Corticosteroid therapy (eg for asthma, IBD)

30
Q

How is hypercortisolism screened for?

A
  • 1mg Overnight DEX suppression test

- 24hr urine free cortisol

31
Q

How is hypercortisolism diagnosed?

A
  • 24hr urine free cortisol

- Low dose Dex test

32
Q

How is it determined if hypercortisolism is ACTH dependent or independent?

A

Paired morning-midnight ACTH cortisol

33
Q

How is it determined if the pituitary is involved in hypercortisolism?

A

High dose Dex test

34
Q

How is the cause of hypercortisolism localised?

A
  • MRI sella
  • CT adrenal
  • BIPSS
  • CT chest
35
Q

What is the pathophysiology of primary hyperaldosteronism?

A
  • Aldosterone producing tumour
  • Increase in blood volume, BP and urine K
  • Decrease in renin
  • Increase in A/R ratio
36
Q

How is primary and secondary hyperaldosteronism differentiated?

A
  • PA/PRA ration>20 is primary hyperaldosteronism

- PA/PRA ratio <20 is secondary hyperaldosetronism

37
Q

What can cause increased PRA and PAC?

A

Investigate for causes of secondary hyperaldosteronism

  • Renovascular hypertension
  • Diuretic use
  • Renin- secreting tumour
  • Malignant -Hypertension
  • Coarctation of Aorta
38
Q

What can cause decreased PRA with increased PAC?

A

Investigate for causes of Primary hyperaldosteronism

39
Q

What can cause decreased PRA and PAC?

A

-Congenital adrenal hyperplasia
-Exogenous mineralocorticoid
-DOC-producing tumour
Cushing’s syndrome
-11 beta HSD deficiency
Liddle’s syndrome

40
Q

What are the screening tests for hyperaldosteronism?

A
  • Plasma renin activity (PRA) and plasma aldosterone concentration (PAC)
  • PAC/PRA> 20 with concomitant serum aldosterone >10ng/dl
41
Q

What are the confirmatory tests for hyperaldosteronism?

A

24hr urine aldosterone >12ug/day and urinary sodium >200mEq/day during 4 days of salt loading

42
Q

How is the aldosterone source established in hyperaldesteronism ?

A
  • 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
Q

What is another name for hyperaldosteronism?

A

Conn’s syndrome

44
Q

What can cause Cushing’s syndrome?

A
  • Adenoma
  • Carcinoma
  • Bilateral hyperplasea
45
Q

What can cause Conn’s syndrome

A
  • Adenoma

- Bilateral hyperplasia

46
Q

Why is pheochromocytoma referred to as the 10% tumour?

A
  • 10% extra-adrenal
  • 10% malignant
  • 10% multiple
  • 10% hyperglycaemia

(30% inherited origin)

47
Q

Pheochromocytoma is associated with paroxysmal attacks of….

A
  • Headache
  • Sweating
  • Palpitations
  • Tremor
  • Pallor
  • Anxiety/fear
48
Q

What is persistent in 70% of pheochromocytoma patients?

A

Hypertension

49
Q

What are >90% of congenital adrenal hyperplasia cases due to?

A

21-hydroxylase deficiency

50
Q

How can severe cases of congenital adrenal hyperplasia present?

A
  • Neonatal salt-losing crisis

- Ambiguous genitalia (girls)

51
Q

What incomplete defects can occur in congenital adrenal hyperplasia?

A
  • Pseudo-precocious puberty (boys)

- Hirsutism (women)