Clinical Aspects of Adrenal Disorders Flashcards

1
Q

What hormones do the different layers of the adrenal gland produce?

A
  • Mineralcorticoids (aldosterone)
  • Corticosteroids (cortisol)
  • Sex steroid (testosterone, progesterone, estrogen)
  • Medulla (norepinephrine, epinephrine)
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2
Q

What is the approach for investigating an adrenal disorder?

A
  1. Clinical suspicion
  2. Test for assessing functional status
  3. If tumour;
    • Resection?
    • +/- chemo or radiotherapy
  4. Endocrine deficiency may need correction
  5. Aetiology specific treatment
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3
Q

Name two hyposecretion disorders

A
  • Primary adrenal insufficiency

* Adrenal enzyme defects

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4
Q

What is another name for primary adrenal insufficiency

A

Addison’s disease

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5
Q

What is autoimmune Addison’s disease?

A
  • Adrenal failure
  • +ve adrenal autoantibodies
  • Lymphocytic infiltrate of adrenal cortex
  • Associated autoimmune disease common (thyroid disease, IDDM, premature ovarian failure)
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6
Q

What are the symptoms associated with Primary Adrenal Failure

A
  • Weakness, fatigue, anorexia, weight loss
  • Skin pigmentation or vitiligo or diarrhoea
  • Salt craving
  • Postural symptoms
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7
Q

Name possible clues to the diagnosis of adrenal failure

A
  • Disproportion between severity of illness and; circulatory collapse / hypotension / dehydration
  • Unexplained hypoglycaemia
  • Other endocrine features (hypothyroidism, body hair loss, amenorrhoea)
  • Previous depression or weight loss
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8
Q

What is Addison’s disease?

A

Damage to adrenal gland and so it does no produce enough cortisol or aldosterone.

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9
Q

How is Addison’s disease?

A
  • Non-specific symptoms
  • Routine bloods: U+E, glucose, FBC

Random cortisol:
• > 550 nmol/l (not Addison’s)
• < 500 nmol/l (adrenal status uncertain)

• Synacthen test (and basal acth)

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10
Q

What is synacthen?

A

Synthetic ACTH test - give synthetic ACTH and take blood to see if it stimulate cortisol production

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11
Q

What is the treatment for Addison’s disease?

A

If suspicion high and patient unwell, treat with steroids and so Synacthen test later

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12
Q

What is the method for the short synacthen test in Addisons disease?

A

9am: 7ml baseline blood test for cortisol and ACTH
Give 250mig tetracosactrin (synacthen) IM or IV

930am: 7ml blood for cortisol
10am: 7ml blood test for cortisol

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13
Q

What else can the short synacthen test be used to diagnose?

A

Congenital adrenal hyperplasia by using the blood sample to analyse for 17-OH progesterone to exclude 21-hydroylase deficiency

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14
Q

How do you interpret the short synacthen test?

A

Normal:
• Normal test at 9am
• Stimulated plasma cortisol (> 500nmol/l)

If impaired cortisol response, and ACTH is elevated then diagnosis is primary adrenal failure.

If ACTH <10ng/l –> secondary adrenal failure

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15
Q

What drugs are used for glucocorticoid replacement?

A
  • Hydrocortisone
  • Prednisolone
  • Dexamethasone
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16
Q

What drug is used for mineralocorticoid replacement?

A

Synthetic steroid, fludrocortisone

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17
Q

What is the action of fludrocortisone for mineralocorticoid replacement?

A
  • Binds to mineralocorticoid (aldosterone) receptors
  • 50-300 micrograms daily

Adjust dose according to:
• Clinical status (postural BP, oedema)
• U&E
• Plasma renin level

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18
Q

Who needs to take special care with use of steroids?

A
  • Hypoadrenal patients on replacement steroids
  • Patients on steroids in doses sufficient to suppress the pituitary adrenal axis (>7.5mg prednisolone daily, or equivalent)
19
Q

What should be given for minor shot-lived illness or stress?

A

Double glucocorticoid dose

20
Q

What should be given for major illness or operation?

A

100mg hydrocortisone IV stat

• As stress abates, reduce HC by 50% per day

21
Q

What are three important ‘self-care’ riles for patients on steroids?

A
  1. Never miss steroid doses
  2. Double the hydrocortisone dose in event of intercurrent illness
  3. If severe vomiting or diarrhoea, call for help without delay (likely need IM HC)
22
Q

Name endocrine causes of hypertension

A

Primary hyperaldosteronism:
• Unilateral adenoma
• Bilateral hyperplasia

Rare:
• Phaeochomcytoma 
• Cushing's syndrome 
• Acromegaly 
• Hyperparathyroidism 
• Hypothyroidism 
• Congenital adrenal hyperplasia
23
Q

Name three adrenal disorders

A

Cortex:
• Cushing’s syndrome (cortisol, androgens)
• Conn’s syndrome (aldosterone)

Medulla:
• Phaeochromocytoma (catecholamines)

24
Q

What does Cushing’s syndrome cause?

A

Excess corticosteroids

25
Q

What are the actions of cortisol?

A
  • Tissue breakdown - causes weakness of skin, muscle + bone
  • Sodium retention - may cause hypertension + heart failure
  • Insulin antagonism - may cause diabetes mellitus
26
Q

What are the signs and symptoms of Cushing’s syndrome?

A
  • Central obesity
  • Hypertension
  • Glc intolerance
  • Hirsutism
  • Amenorrhoea
  • Osteoporosis
  • Acne
  • Oedema
27
Q

What are the causes of Cushing’s syndrome?

A

ACTH dependent:
• Pituitary tumour (Cushing’s disease)
• Ectopic ACTH secretion (i.e. lung carcinoid)

ACTH-independent:
• Adrenal tumour (adenoma or carcinoma)

28
Q

What is the screening test for Cushing’s syndrome?

A
  • 24hr urinary free cortisol

* 1mg overnight Dexamethasone suppression test

29
Q

What is the approach to hypercortisolism?

A
  1. Screen -> overnight dex test and 24hr urine free cortisol
  2. Confirmation -> 24hr urine and low dose dex test
  3. If ACTH dependent or not?
  4. ACTH: pit/no pit -> high dose dex get
  5. Localisation
30
Q

What tests are used to localise cause of hypercortisolism?

A
  • MRI Sella
  • CT adrenal
  • BIPSS
  • CT Chest
31
Q

What is BIPSS?

A

Bilateral inferior petrosal sinus sampling

32
Q

Describe the pathophysiology of primary hyperaldosteronism

A
  1. Aldosterone-producing tumour
  2. Increase in blood volume, blood pressure and urine K
  3. This decreases stimulation of renin

So Aldo increase and renin decrease, increasing A/R ratio

33
Q

Describe screening of hyperaldosteronism

A
Plasma PA (aldo)/ PRA (renin) ratio
• > 20 - primary hyperaldosteronism 
• < 20 - secondary hyperaldosteronism (essential hypertension)
34
Q

What is PRA and PAC?

A

Plasma renin activity (PRA) and plasma aldosterone concentration (PAC)

35
Q

What is indicated when PAC and PRA both increase?

A

Investigate for cause secondary hyperaldosteronism

  • Renovascular hypertension
  • Diuretic use
  • Renin- secreting tumour
  • Malignant Hypertension
  • Coarctation of Aorta
36
Q

What is indicated when PAC increases and PRA decreases?

A

Investigate for causes of Primary hyperaldosteronism

37
Q

What is indicated when PAC and PRA both decrease?

A

Congenital adrenal hyperplasia

Exogenous mineralocorticoid

DOC-producing tumour
Cushing’s syndrome

11 beta HSD deficiency
Liddle’s syndrome

38
Q

What is the approach to hyperaldosteronism?

A
  1. Clinical suspicion
  2. PRA and PAC screening test and PAC/PRA > 20
  3. 24hr urine aldosterone and sodium confirmatory test
  4. Establish source of aldosterone:
    • CT scan of adrenal glands
    • Upright posture test
    • Plasma 18-hydroxycorticosteroneAdrenal venous sampling if CT scan inconclusive
39
Q

What is phaeochromocytoma?

A

Neuroendocrine tumour of the medulla of adrenal glands

40
Q

What are the symptoms of phaeochromocytoma?

A
• Hypertension 
• Paroxysmal attacks 
   - Headache
   - Sweating
   - Palpitations 
   - Tremor 
   - Anxiety/fear
41
Q

What is a cause of congenital adrenal hyperplasia?

A

21-hydroxylase deficiency

42
Q

What are the effects of 21-hydroxylase deficiency in congenital adrenal hyperplasia?

A

Severe cases
• Neonatal salt-losing crisis
• Ambiguous genitalia (girls)

Incomplete defects
• Pseudo-precocious puberty (boys)
• Hirsutism (women)

43
Q

What is hirsutism?

A

Hairiness