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
What are the actions of cortisol?
* Tissue breakdown - causes weakness of skin, muscle + bone * Sodium retention - may cause hypertension + heart failure * Insulin antagonism - may cause diabetes mellitus
26
What are the signs and symptoms of Cushing's syndrome?
* Central obesity * Hypertension * Glc intolerance * Hirsutism * Amenorrhoea * Osteoporosis * Acne * Oedema
27
What are the causes of Cushing's syndrome?
ACTH dependent: • Pituitary tumour (Cushing's disease) • Ectopic ACTH secretion (i.e. lung carcinoid) ACTH-independent: • Adrenal tumour (adenoma or carcinoma)
28
What is the screening test for Cushing's syndrome?
* 24hr urinary free cortisol | * 1mg overnight Dexamethasone suppression test
29
What is the approach to hypercortisolism?
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
What tests are used to localise cause of hypercortisolism?
* MRI Sella * CT adrenal * BIPSS * CT Chest
31
What is BIPSS?
Bilateral inferior petrosal sinus sampling
32
Describe the pathophysiology of primary hyperaldosteronism
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
Describe screening of hyperaldosteronism
``` Plasma PA (aldo)/ PRA (renin) ratio • > 20 - primary hyperaldosteronism • < 20 - secondary hyperaldosteronism (essential hypertension) ```
34
What is PRA and PAC?
Plasma renin activity (PRA) and plasma aldosterone concentration (PAC)
35
What is indicated when PAC and PRA both increase?
Investigate for cause secondary hyperaldosteronism * Renovascular hypertension * Diuretic use * Renin- secreting tumour * Malignant Hypertension * Coarctation of Aorta
36
What is indicated when PAC increases and PRA decreases?
Investigate for causes of Primary hyperaldosteronism
37
What is indicated when PAC and PRA both decrease?
Congenital adrenal hyperplasia Exogenous mineralocorticoid DOC-producing tumour Cushing’s syndrome 11 beta HSD deficiency Liddle’s syndrome
38
What is the approach to hyperaldosteronism?
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-hydroxycorticosterone Adrenal venous sampling if CT scan inconclusive
39
What is phaeochromocytoma?
Neuroendocrine tumour of the medulla of adrenal glands
40
What are the symptoms of phaeochromocytoma?
``` • Hypertension • Paroxysmal attacks - Headache - Sweating - Palpitations - Tremor - Anxiety/fear ```
41
What is a cause of congenital adrenal hyperplasia?
21-hydroxylase deficiency
42
What are the effects of 21-hydroxylase deficiency in congenital adrenal hyperplasia?
Severe cases • Neonatal salt-losing crisis • Ambiguous genitalia (girls) Incomplete defects • Pseudo-precocious puberty (boys) • Hirsutism (women)
43
What is hirsutism?
Hairiness