Addison's disease

Question 1

Adrenal insufficiency is an endocrine disorder that happens when the adrenal gland doesn’t produce enough hormones. There are the 3 layers of the adrenal gland, which of the following is NOT one of these layers?

1 - zone glomerulus
2 - zona media
3 - zona fasiculata
4 - zona reticularis

Answer 1

2 - zona media

Question 2

Adrenal insufficiency is an endocrine disorder that happens when the adrenal gland doesn’t produce enough hormones. There are the 3 layers of the adrenal gland from outside moving in they are:

• zone glomerulus
• zona fasiculata
• zona reticularis

Link the hormones created with the layers that they are produced in:

• glucocorticoids
• androgens
• mineralcorticoids

Answer 2

• zone glomerulus = mineralcorticoids
• zona fasiculata = glucocorticoids
• zona reticularis = androgens

Question 3

What is released by the hypothalamus to stimulate the pituitary gland in the hypothalamus pituitary adrenal axis (HPA)?

1 - corticotropin-releasing hormone (CRH)
2 - cortisol
3 - adrenocorticotropic hormone (ACTH)
4 - renin

Answer 3

1 - corticotropin-releasing hormone (CRH)

Question 4

What is released by the pituitary gland secrete to stimulate the zona fasiculata of the adrenal gland in hypothalamus pituitary adrenal axis (HPA)?

1 - corticotropin-releasing hormone (CRH)
2 - cortisol
3 - adrenocorticotropic hormone (ACTH)
4 - renin

Answer 4

3 - adrenocorticotropic hormone (ACTH)

Question 5

It is called primary adrenal insufficiency as the cause is located within the adrenal gland, which can be acute or chronic. What is the chronic form of primary adrenal insufficiency commonly called?

1 - von Willebrands
2 - cushings syndrome
3 - addisons disease
4 - conns disease

Answer 5

3 - addisons disease

• secondary adrenal insufficiency is typically because the pituitary gland is not functioning properly

Question 6

What is the prevalence of primary adrenal insufficiency in the UK?

1 - 84
2 - 840
3 - 8400
4 - 84,000

Answer 6

3 - 8400
- 8 / 100,000

Question 7

What is the most common age affected by primary adrenal insufficiency in the UK, and does this affect women or men more?

1 - 15 - 20 y/o
2 - 20 - 30 y/o
3 - 30 - 50 y/o
4 - >65 y/o

Answer 7

3 - 30 - 50 y/o

- women are more affected more

Question 8

In primary adrenal insufficiency, what % of the adrenal cortex has to be affected/destroyed in order for symptoms to present?

1 - 25%
2 - 40%
3 - 60%
4 - >90%

Answer 8

4 - >90%

Question 9

In addisons disease which 2 of the following are affected?

1 - mineralcorticoid
2 - catecholamines
3 - androgens
4 - glucocorticoids

Answer 9

1 - mineralcorticoid
- aldosterone deficiency

4 - glucocorticoids
- cortisol deficiency

Question 10

Primary adrenal insufficiency is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. All of the following can cause primary adrenal insufficiency, BUT which is the most common, accounting for up to 80% of cases?

1 - autoimmune
2 - infiltrative (sarcoidosis)
3 - infective (TB)
4 - vascular
5 - haemorrhage/infarction
6 - adrenoleucodystrophy (membrane of adrenal glands is damaged)
7 - congenital adrenal insufficiency
8 - drugs
9 - metastatic deposition

Answer 10

1 - autoimmune

• TB is most common cause in under developed countries where the infection spreads from the lungs to the adrenal glands

Question 11

Metastases can cause primary adrenal insufficiency. What are the most common locations where metastases come from?

1 - breast
2 - lung
3 - renal
4 - all of the above

Answer 11

4 - all of the above

Question 12

Although rare Waterhouse-Friderichsen syndrome (WFS) can cause primary adrenal insufficiency. What is WFS?

1 - complication of TB
2 - complication of meningitis
3 - complication of ascites
4 - complication of pericarditis

Answer 12

2 - complication of meningitis
- specifically meningococcal sepsis

Question 13

Primary adrenal insufficiency is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. Secondary adrenal insufficiency, called secondary because it does not affect the adrenal glands directly and may be from another source such as the pituitary gland or another malignancy. Are glucocorticoids the only thing that are affected in secondary adrenal insufficiency?

Answer 13

• yes
• adrenocorticotropic hormone (ACTH) released from pituitary is specific to glucocorticoids
• mineralocorticoids are unaffected

Question 14

Secondary adrenal insufficiency, called secondary because it does not affect the adrenal glands directly and may be from another source such as the pituitary gland or another malignancy. What is the main cause of secondary adrenal insufficiency?

1 - autoimmune disease
2 - malignancy
3 - infection
4 - iatrogenic

Answer 14

4 - iatrogenic

• long term use of steroids
• suppresses the pituitary-adrenal axis

Question 15

When comparing primary and secondary adrenal insufficiency, which has a raised adrenocorticotropic hormone (ACTH)?

Answer 15

• primary = high ACTH
• pituitary increases ACTH to get a response from adrenal gland
• secondary = low or absent ACTH
• adrenal gland is ok, but insufficient ACTH to respond

Question 16

Which of the following tests are typically performed in a patient with suspected primary adrenal insufficiency?

1 - 9am cortisol (peaks at this time)
2 - ACTH levels (low ACTH = pituitary source and high ACTH = adrenal source)
3 - Na+ and K+
4 - pH
5 - renin and aldosterone (mineralocortiocoid activity)
6 - adrenal antibodies (autoimmune tests)
7 - all of the above

ACTH = Adrenocorticotropic hormone

Answer 16

7 - all of the above

Question 17

Primary adrenal insufficiency (PAI) is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. When investigating if a patient with PAI, what is the dynamic test that should be performed?

1 - OGTT
2 - ABG
3 - synthetic adrenocorticotropic hormone test
4 - all of the above

Answer 17

3 - synthetic adrenocorticotropic hormone test

• patient administered tetracosactide (synthetic ACTH)
• 250 nanograms administered IM at 9am
• sample taken at 0, 30 and 60 minutes
• normal response would be a rise in cortisol levels

Question 18

Primary adrenal insufficiency (PAI) is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. When investigating if a patient with PAI, the dynamic test, detailed below should be performed. What must clinicians take into account and control for?

• patient administered tetracosactide (synthetic ACTH)
• 250 nanograms administered IM at 9am
• sample taken at 0, 30 and 60 minutes
• normal response would be a rise in cortisol levels

1 - age
2 - steroid use
3 - gender
4 - ethnicity

Answer 18

2 - steroid use

Question 19

If we suspect primary adrenal insufficiency, is it important to investigate for 21-hydroxylase?

Answer 19

• yes
• this is the adrenal autoantibody test
• autoimmune is main cause of primary adrenal insufficiency

Question 20

If a patient has gone undiagnosed or not presented with symptoms of adrenal insufficiency, they can present with adrenal crisis, which is a medical emergency of low cortisol levels. Which of the following are common presentations of adrenal crisis?

1 - hypotension
2 - hypothermia
3 - bradykardia
4 - low Na+ and high K+
5 - all of the above

Answer 20

5 - all of the above

• low Na+ and high K+ = low aldosterone
• hypotension = low Na+ and H2O
• bradykardia = low catecholamines and cortisol

Question 21

Primary adrenal insufficiency (PAI) is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. All of the following are clinical manifestations if the zona glomerulus is affected in the adrenal gland causing low or absent mineralcorticoid (aldosterone), EXCEPT which one?

1 - hypotension
2 - hypovolaemia
3 - bradykardia
4 - hyponatreamia
5 - hyperkalaemia
6 - metabolic acidosis

Answer 21

3 - bradykardia

Question 22

Primary adrenal insufficiency (PAI) is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. All of the following are clinical manifestations if the zona glomerulus is affected in the adrenal gland causing low or absent mineralcorticoid (aldosterone): hypotension, hypovolaemia, hyponatreamia, hyperkalaemia and metabolic acidosis. Which of the following can the patient present with?

1 - nausea
2 - vomiting
3 - cravings for salty foods
4 - fatigue
5 - dizziness that worsens with standing
6 - all of the above

Answer 22

6 - all of the above

Question 23

Primary adrenal insufficiency (PAI) is when there is a lack of glucocorticoid (cortisol) and mineralcorticoid (aldosterone) that is due to a problem with the adrenal gland. If the zona fasiculata is affected then glucocorticoids (cortisol) are low or absent. Which of the following is NOT a symptom of this?

1 - weak/fatigues
2 - disorientated
3 - tachycardia
4 - hyperpigmentation

Answer 23

3 - tachycardia
- cortisol contributes to tachycardia normally

• hyperpigmentation is due to increased melanocyte stimulating hormone
• can be commonly seen in knees and elbows

Question 24

In an adrenal crisis, which commonly presents in patients with chronic primary adrenal insufficiency can be a life threatening emergency. What are the common symptoms a patient may present with?

1 - sudden pain in the lower backabdomen, or legs
2 - severe vomiting and diarrhea
3 - dehydration
4 - low blood pressure
5 - loss of consciousness
6 - all of the above

Answer 24

6 - all of the above

Question 25

If a patient is has gone undiagnosed or not presented with symptoms of adrenal insufficiency can present with adrenal crisis, which is a medical emergency of low cortisol levels. Which of the following are important initial treatment options? 1 - intravenous hydrocortisone 2 - intravenous fluids 3 - hypoglycaemia management 4 - all of the above

Answer 25

4 - all of the above

Question 26

If patients are diagnosed with primary adrenal insufficiency, what are the long term treatment options? 1 - transplant 2 - steroid therapy 3 - monoclonal antibodies 4 - surgery

Answer 26

2 - steroid therapy - steroid to mimic circadian rhythm - mineralocorticoid (if affected, namely Fluorocortisone) - education (sick day rules)

Question 27

In patients diagnosed with primary adrenal insufficiency and is having symptoms we should replace the patients regular steroid with which other steroid? 1 - Hydrocortisone 2 - Prednisolone 3 - Betamethasone 4 - Fludrocortisone

Answer 27

1 - Hydrocortisone - given 15-25mg daily - i.e. 10mg at waking and 5mg at lunch - DO NOT give in the evenings

Question 28

In patients diagnosed with primary adrenal insufficiency and they are having symptoms such as postural hypotension, hyponatraemia and hyperkalaemia, what hormone should they be given orally? 1 - Hydrocortisone 2 - Prednisolone 3 - Betamethasone 4 - Fludrocortisone

Answer 28

4 - Fludrocortisone - core mineralcorticoid - 50-200mcg daily

Question 29

What is a hydrocortisone emergency injection kit used for?

Answer 29

- when adrenal crisis, vomiting or dehydrated | - main component is 3 vials of hydrocortisone phosphate (100mg/ml)

Question 30

During a stress response, the body generally releases cortisol. However, if the patient is ill or has adrenal insufficiency then this can lead to adrenal crisis, a medical emergency. Illness can affect the bodies response, so it is important to be aware of the sick day rules, which are essentially rules on what to do with medication relating to adrenal glands depending on the severity of the illness. What does sick day 1 rule encompass, which may be caused by infection or anesthesia? 1 - continue with normal steroid dose 2 - double steroid dose for 3-4 days post illness 3 - triple steroid dose for 7 days post illness 4 - add hydrocortisone via IV to normal steroid dose

Answer 30

2 - double steroid dose for 3-4 days post illness

Question 31

During a stress response, the body generally releases cortisol. However, if the patient is ill or has adrenal insufficiency then this can lead to adrenal crisis, a medical emergency. Illness can affect the bodies response, so it is important to be aware of the sick day rules, which are essentially rules on what to do with medication relating to adrenal glands depending on the severity of the illness. What does sick day 2 rule encompass, which may be caused by severe illness, persistent vomiting or trauma? 1 - continue with normal steroid dose 2 - double steroid dose for 3-4 days post illness 3 - triple steroid dose for 7 days post illness 4 - hydrocortisone injection given at 100mg intravenously

Answer 31

4 - hydrocortisone injection given at 100mg intravenously

Question 32

In an adrenal crisis, which commonly presents in patients with chronic primary adrenal insufficiency can be a life threatening emergency. Should we request bloods and tests before we treat the patient?

Answer 32

- no - treat immediately if suspect adrenal crisis - BUT request ACTH and cortisol immediately

Question 33

In an adrenal crisis, which commonly presents in patients with chronic primary adrenal insufficiency can be a life threatening emergency. We should give patients calcium gluconate. What is the primary reason for giving this? 1 - hyperkalaemia can causes cardiac abnormalities 2 - reduces risk of rhabdomyolysis 3 - reduces the risk of renal necrosis 4 - all of the above

Answer 33

1 - hyperkalaemia can causes cardiac abnormalities

Question 34

In an adrenal crisis, which commonly presents in patients with chronic primary adrenal insufficiency can be a life threatening emergency. All of the following must be done EXCEPT which one? 1 - 100mg IV hydrocortisone stat 2 - 500ml IV 0.9% saline bolus 3 - calcium gluconate 4 - dextrose

Answer 34

4 - dextrose - hypoglycaemia should be monitored but not an immediate treatment - calcium gluconate is used to protect the heart from hyperkalaemia - antibiotics may be given if infection is suspected, but need blood, urine and sputum for culture

Question 35

In an adrenal crisis, which commonly presents in patients with chronic primary adrenal insufficiency can be a life threatening emergency. Following the initial treatment with 100mg IV hydrocortisone stat, 500ml IV 0.9% saline bolus and calcium gluconate, we should continue care. Which of the following should be performed? 1 - glucose if patient is hypoglycaemic 2 - IV fluids based on dehydration levels 3 - hydrocortisone 100mg/8h IV or IM 4 - switch to oral steroids if patient stable 5 - all of the above

Answer 35

5 - all of the above

Question 36

There are 4 types of autoimmune polyglandular syndrome. Which 2 can cause Addisons disease? 1 - Type 1 2 - Type 2 3 - Type 3 4 - Type 4

Answer 36

1 - Type 1 2 - Type 2 (most common) - always present in Type 2

Question 37

Polyglandular syndrome type 1 (PST-1) is a rare genetic autoimmune disease that gradually affects more and more hormone hormone producing organs. Which of the following are affected in PST-1? 1 - Addisons disease 2 - hypoparathyroidism 3 - chronic mucocutaneous candidiasis (CMC) 4 - 2 of the above must be present 5 - all myst be present

Answer 37

4 - 2 of the above must be present - CMC = chronic infections of the skin, nails, and mucous membranes

Question 38

Polyglandular syndrome type 2 (PST-2) is a rare genetic autoimmune disease that gradually affects more and more hormone hormone producing organs. Which of the following are affected in PST-2? 1 - Addisons disease 2 - thyroid disease 3 - T1DM 4 - 2 of the above must be present 5 - all myst be present

Answer 38

4 - 2 of the above must be present - Addisons is always present - thyroid disease and/or T1DM

Question 39

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