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Adrenal Disorders Flashcards

1
Q

What catecholamines are secreted by the the adrenal medulla?

A

Adrenaline

Noradrenaline

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2
Q

What veins and nerves are found in the adrenal medulla?

A

Medullary veins

Splanchnic Nerves

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3
Q

What regulates Aldosterone?

A

Renin-Angiotensin-ALDOSTERONE-System (RAAS)

Plasma Potassium

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4
Q

What are the 6 classes of steroid receptors?

A 
Glucocorticoid
Mineralocorticoid
Progestin
Oestrogen
Androgen
Vitamin D
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5
Q

What action do glucocortiocoids have on the circulatory/ renal systems?

A

Increased:

  • Cardiac output
  • Blood pressure
  • renal blood flow
  • Glomerular Filtration Rate
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6
Q

What effect do glucocorticoids have on the CNS?

A

Mood lability
Euphoria/psychosis
Decreased libido

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7
Q

How do Glucocorticoids affect the bone and connective tissue?

A 
Accelerates osteoporosis
Decreases:
- Serum calcium
- collagen formation
- wound healing
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8
Q

What are the 3 main uses of corticosteroids in practice?

A
  • Suppress inflammation
  • Suppress immune system
  • Replacement treatment
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9
Q

Where are the mineralocorticoid receptors found?

A

Kidneys
Salivary glands
Gut
Sweat glands

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10
Q

What can cause PRIMARY adrenal insufficiency?

A
  • Addison’s disease
  • Congenital Adrenal Hyperplasia (CAH)
  • Adrenal TB/malignancy
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11
Q

What causes secondary adrenal insufficiency?

A
  • lack of ACTH stimulation
  • Iatrogenic (removal of steroid Tx)
  • Pituitary/hypothalamic disorders
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12
Q

What is addison’s disease?

A

Autoimmune destruction of adrenal cortex

Autoantibodies positive in 70%

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13
Q

What percentage of the adrenal cortex is usually destroyed before people experience symptoms in Addison’s disease?

A

> 90%

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14
Q

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

A
  • Type 1 DM
  • autoimmune thyroid disease
  • pernicious anaemia
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15
Q

What features of biochemistry would make you suspicious of adrenal insufficiency?

A

Low Na+
High K+
hypoglycaemia

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16
Q

What treatments are used to manage adrenal insufficiency?

A
  • Hydrocortisone as cortisol replacement

- Fludrocortisone as aldosterone replacement

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17
Q

If people are on treatment for adrenal insufficiency, what should they be educated on?

A
  • ‘sick day rules’ due to needing more steroid when ill
  • Cannot stop medication suddenly
  • Need to wear identification
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18
Q

What hormones are diminished in secondary adrenal insufficiency?

A

CRH and ACTH

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19
Q

Where are striae often seen in Cushing’s syndrome and why?

A
  • Around stomach (due to increased central obesity)

- Inside of proximal thighs

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20
Q

What hormone is found in excess in Conn’s syndrome

A

Aldosterone

21
Q

Describe what is meant by Primary Aldosteronism?

A

Autonomous production of aldosterone independent of its regulators (angiotensin II/potassium)

22
Q

What clinical features normally indicate Primary Aldosteronism?

A
  • Significant hypertension
  • Hypokalaemia (in around 30%)
  • Alkalosis
23
Q

What are the main causes of primary aldosteronism?

A
  • Adrenal adenoma (Conn’s)

- Bilateral adrenal hyperplasia (commonest cause)

24
Q

How is Aldosterone excess confirmed?

A
  • Aldosterone to renin ratio (ARR)
  • Saline suppression test
    (Failure of plasma aldosterone to suppress by > 50% with 2 litres of normal saline)
25
How can the type of primary aldosteronism be confirmed?
- Adrenal CT to demonstrate adenoma | - Adrenal vein sampling to confirm adenoma is true source of aldosterone excess
26
What surgery can be used to treat primary aldosteronism?
- Unilateral laparoscopic adrenalectomy | Only if adrenal adenoma confirmed by vein sampling
27
What type of treatment can be used in bilateral adrenal hyperplasia?
Use Mineralocorticoid Receptor antagonists (spironolactone or eplerenone)
28
Aldosterone excess is a common cause of secondary hypertension. TRUE/FALSE?
TRUE
29
What enzyme is deficient in Congenital Adrenal Hyperplasia
21α hydroxylase
30
Is Congenital Adrenal Hyperplasia Autosomal Dominant or Recessive?
Autosomal Recessive
31
What marker is tested for in the suspicion of Congenital Adrenal Hyperplasia
17-OH Progesterone
32
How does Congenital Adrenal Hyperplasia classically present in males?
- Adrenal insufficiency - Often around two to three weeks - Poor weight gain - Biochemical pattern (Low Na, High K, Hypoglycaemic)
33
How does Congenital Adrenal Hyperplasia classically present in females?
Genital ambiguity
34
Describe a "non-classical" presentation of Congenital Adrenal Hyperplasia
``` Hirsutism Acne Oligomenorrhoea Precocious puberty Infertility or sub-fertility ```
35
How are children with CAH usually treated?
- Glucorticoid replacement - Mineralocorticoid replacement **ONLY SOME** - Surgical correction => Achieve maximal growth potential
36
If CAH is not noticed until adulthood, how is it treated at this stage?
- Control androgen excess - Restore fertility - Avoid steroid over-replacement
37
How does Phaeochromocytoma usually present?
- Labile hypertension - Postural hypotension - Paroxysmal sweating/headache/pallor/tachycardia * *sometimes presents with none of these**
38
What colour are phaechromocytomas and why?
Chromaffin cells reduce chrome salts to metal chromium | =>resulting in a brown colour reaction
39
In what section of the adrenal gland is a phaechromocytoma found?
Medulla
40
Why does compression of a phaechromocytoma cause an increase in blood pressure?
Squeezes out noradrenaline and adrenaline | => sympathetic drive increases BP
41
What complications of phaeochromocytoma can appear to present as stand alone illness?
``` Left ventricular failure Myocardial necrosis Stroke Shock Paralytic ileus of bowel ```
42
What biochemical abnormalities are often seen in Phaechromocytoma?
- Hyperglycaemia – adrenaline secreting tumours - low K+ level - raised Hb conc. - Mild hypercalcaemia - Lactic acidosis – in absence of shock
43
What patients should be investigated on suspicion of phaeochromocytoma?
- Family members with syndromes - Resistant hypertension - The young [<50] with hypertension - Classical symptoms - Consider with hypertension and hyperglycaemia
44
How is an excess in catecholamines in phaeochromocytoma confirmed?
Urine – 2x24hour catecholamines or metanephrins
45
What imaging modalities can be used to identify the source of catecholamine excess?
``` MRI Scan (Abdomen/Whole body) MIBG – meta-iodobenzylguanidine PET Scan ```
46
What medical treatments are used in phaeochromocytoma?
- Phenoxybenzamine (α-blocker) - Propranolol, atenolol or metoprolol (β-blocker) **A before B**
47
What surgical treatment can be used in phaechromocytoma?
Laparoscopic surgery Attempt full excision **chemotherapy can also be used if malignant => Radio-labelled MIBG
48
Phaeochromocytoma is linked to what genetic conditions?
- Multiple Endocrine Neoplasia 2 (MEN2) - Von-Hippel-Lindau syndrome - Succinate dehydrogenase mutations (SDH) - Neurofibromatosis - Tuberose sclerosis

Endocrine (24 decks)

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