Endocrinology Conditions Flashcards

1
Q

What is the body’s ideal blood glucose concentration

A

Between 4.4 and 6.1 mmol/l

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2
Q

What is insulin

A

An anabolic hormone produced by the beta cells in the islets of Langerhans in the pancreas that reduces blood sugar levels
Insulin is essential in letting cells take glucose out of the blood and use it as fuel, without insulin the cells cannot do this

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3
Q

How does insulin reduce blood sugar

A

In two ways:

  • Causes cells in the body to absorb glucose from the blood and use it as fuel
  • Causes muscle and liver cells to absorb glucose from the blood and store it as glycogen
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4
Q

What is glucagon

A

An catabolic hormone produced by the alpha cells in the islets of Langerhans in the pancreas that is released in response to low blood sugar levels and stress, and tells the liver to break down stored glycogen into glucose (glycogenolysis) and also to convert protein and fats into glucose (gluconeogenesis)

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5
Q

What is type 1 diabetes

A

Type 1 diabetes mellitus (T1DM) is a disease where the pancreas stops being able to produce insulin.
When there is no insulin being produced, the cells of the body cannot take glucose from the blood and use it for fuel
Therefore the cells think the body is being fasted and has no glucose supply
Meanwhile the level of glucose in the blood keeps rising causing hyperglycaemia

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6
Q

What causes type 1 diabetes

A

This is unclear
May be a genetic component
May be triggered by certain viruses such as Coxsackie B virus and enterovirus

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7
Q

What is diabetic ketoacidosis

A

Occurs in type 1 diabetes where the person is not producing adequate insulin themselves and is not injecting adequate insulin to compensate for this
It occurs when the body does not have enough insulin to use and process glucose
The main problems, and most dangerous, are ketoacidosis, dehydration and potassium imbalance

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8
Q

What is ketoacidosis

A

As the cells in the body have no fuel and think they are starving they initiate the process of ketogenesis so that they have a usable fuel. Over time the patient gets higher and higher glucose and ketones levels. Initially the kidneys produce bicarbonate to counteract the ketone acids in the blood and maintain a normal pH. Over time the ketone acids use up the bicarbonate and the blood starts to become acidic.

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9
Q

How do patients become dehydrated in DKA

A

Hyperglycaemia overwhelms the kidneys and glucose starts being filtered into the urine. The glucose in the urine draws water out with it in a process called osmotic diuresis. This causes the patient to urinate a lot (polyuria). This results in severe dehydration. The dehydration stimulates the thirst centre to tell the patient to drink lots of water. This excessive thirst is called polydipsia.

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10
Q

How do patients with DKA have a potassium imbalance

A

Insulin normally drives potassium into cells. Without insulin potassium is not added to and stored in cells. Serum potassium can be high or normal as the kidneys continue to balance blood potassium with the potassium excreted in the urine, however total body potassium is low because no potassium is stored in the cells. When treatment with insulin starts patients can develop severe hypokalaemia (low serum potassium) very quickly and this can lead to fatal arrhythmias.

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11
Q

How does DKA present

A
Life threatening medical emergency:
Hyperglycaemia
Dehydration
Ketosis
Metabolic acidosis (with a low bicarbonate)
Potassium imbalance

The patient will therefore present with symptoms of these abnormalities:

  • Polyuria
  • Polydipsia
  • Nausea and vomiting
  • Acetone smell to their breath
  • Dehydration and subsequent hypotension
  • Altered Consciousness
  • They may have symptoms of an underlying trigger (i.e. sepsis)
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12
Q

How is DKA diagnosed

A

Hyperglycaemia (i.e. blood glucose > 11 mmol/l)
Ketosis (i.e. blood ketones > 3 mmol/l)
Acidosis (i.e. pH < 7.3)

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13
Q

How is DKA treated

A

FIG-PICK
F – Fluids – IV fluid resuscitation with normal saline (e.g. 1 litre stat, then 4 litres with added potassium over the next 12 hours)
I – Insulin – Add an insulin infusion (e.g. Actrapid at 0.1 Unit/kg/hour)
G – Glucose – Closely monitor blood glucose and add a dextrose infusion if below a certain level (e.g. 14 mmol/l)
P – Potassium – Closely monitor serum potassium (e.g. 4 hourly) and correct as required
I – Infection – Treat underlying triggers such as infection
C – Chart fluid balance
K – Ketones – Monitor blood ketones (or bicarbonate if ketone monitoring is unavailable)

Establish patient on their normal sub-cut insulin regime prior to stopping insulin and fluid infusion

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14
Q

What is the maximum infusion rate of potassium

A

10mmol per hour

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15
Q

What is the long term management of type 1 diabetes

A

Condition is life long so patient education is essential
Components of treatment are:
-Subcutaneous insulin regimes
-Monitoring dietary carbohydrate intake
-Monitoring blood sugar levels on waking, at each meal and before bed
-Monitoring for and managing complications, both short and long term

Insulin is usually prescribed as a combination of a background, long acting insulin given once a day and a short acting insulin injected 30 minutes before intake of carbohydrate (i.e. at meals). Insulin regimes are initiated by a diabetic specialist.

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16
Q

What is lipodystrophy

A

Injecting into the same spot can cause a condition called “lipodystrophy”, where the subcutaneous fat hardens and patients do not absorb insulin properly from further injections into this spot. For this reason patients should cycle their injection sites.

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17
Q

What are the short term complications of type 1 diabetes

A

Short term complications relate to immediate insulin and blood glucose management:

  • Hypoglycaemia
  • Hyperglycaemia (and DKA)
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18
Q

What is hypoglycaemia

A
Low blood sugar level 
Typical symptoms are:
-Tremor
-Sweating
-Irritability
-Dizziness
-Pallor
More severe hypoglycaemia will lead to:
-Reduced consciousness
-Coma
-Death 
(unless treated.)
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19
Q

What are the potential long term complications of type 1 diabetes

A

Chronic exposure to hyperglycaemia causes damage to the endothelial cells of blood vessels. This leads to leaky, malfunctioning vessels that are unable to regenerate. High levels of sugar in the blood also causes suppression of the immune system, and provides an optimal environment for infectious organisms to thrive.

Macrovascular Complications:

  • Coronary artery disease is a major cause of death in diabetics
  • Peripheral ischaemia causes poor healing, ulcers and “diabetic foot”
  • Stroke
  • Hypertension

Microvascular Complications:

  • Peripheral neuropathy
  • Retinopathy
  • Kidney disease, particularly glomerulosclerosis

Infection Related Complications:

  • Urinary Tract Infections
  • Pneumonia
  • Skin and soft tissue infections, particularly in the feet
  • Fungal infections, particularly oral and vaginal candidiasis
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20
Q

What is HbA1c

A

It is counting glycated haemoglobin, which is how much glucose is attached to the haemoglobin molecule. This is considered to reflect the average glucose level over the last 3 months because red blood cells have a lifespan of around 3-4 months. We measure it every 3 – 6 months to track progression of the patient’s diabetes and how effective the interventions are. It requires a blood sample sent to the lab, usually red top EDTA bottle.

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21
Q

What is flash glucose monitoring

A

Eg. FreeStyle Libre
This uses a sensor on the skin that measures the glucose level of interstitial fluid. There is a lag of 5 minutes behind blood glucose. This sensor records the glucose readings at short intervals so you get a really good impression of what the glucose levels are doing over time. The user needs to use a “reader” to swipe over the sensor and it is the reader that shows the blood sugar readings.

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22
Q

What is the pathology of type 2 diabetes

A

Repeated exposure to glucose and insulin makes the cells in the body become resistant to the effects of insulin. It therefore requires more and more insulin to produce a response from the cells and get them to take up and use glucose. Over time, the pancreas (specifically the beta cells) becomes fatigued and damaged by producing so much insulin and they start to produce less. A continued onslaught of glucose on the body in light of insulin resistance and pancreatic fatigue leads to chronic hyperglycaemia. Chronic hyperglycaemia leads to microvascular, macrovascular and infectious complications

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23
Q

What are the risk factors for type 2 diabetes

A
Older age
Ethnicity (Black, Chinese, South Asian)
Family history
Obesity
Sedentary lifestyles
High carbohydrate (particularly refined carbohydrate) diet
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24
Q

How does type 2 diabetes present

A
Sometimes caught on HbA1c screen
Fatigue
Polydipsia and polyuria (thirsty and urinating a lot)
Unintentional weight loss
Opportunistic infections
Slow healing
Glucose in urine (on dipstick)
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25
Q

What is the oral glucose tolerance test (OGTT)

A

An oral glucose tolerance test (OGTT) is performed in the morning prior to having breakfast. It involves taking a baseline fasting plasma glucose result, giving a 75g glucose drink and then measuring plasma glucose 2 hours later. It tests the ability of the body to cope with a carbohydrate meal.

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26
Q

How is pre-diabetes diagnosed

A

Pre-diabetes can be diagnosed with a HbA1c or by “impaired fasting glucose” or “impaired glucose tolerance”. Impaired fasting glucose means that their body struggles to get their blood glucose levels in to normal range, even after a prolonged period without eating carbohydrates. Impaired glucose tolerance means their body struggles to cope with processing a carbohydrate meal.

  • HbA1c – 42-47 mmol/mol
  • Impaired fasting glucose – fasting glucose 6.1 – 6.9 mmol/l
  • Impaired glucose tolerance – plasma glucose at 2 hours 7.8 – 11.1 mmol/l on an OGTT
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27
Q

How is type 2 diabetes diagnosed

A

Diabetes can be diagnosed if the patient fits the criteria on plasma glucose, an oral glucose tolerance test or HbA1c.

HbA1c > 48 mmol/mol
Random Glucose > 11 mmol/l
Fasting Glucose > 7 mmol/l
OGTT 2 hour result > 11 mmol/l

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28
Q

How is type 2 diabetes managed

A

It is curable

Dietary Modification

  • Vegetables and oily fish
  • Typical advice is low glycaemic, high fibre diet
  • A low carbohydrate may in fact be more effective in treating and preventing diabetes but is not yet mainstream advice

Optimise Other Risk Factors

  • Exercise and weight loss
  • Stop smoking
  • Optimise treatment for other illnesses, for example hypertension, hyperlipidaemia and cardiovascular disease

Monitoring for Complications

  • Diabetic retinopathy
  • Kidney disease
  • Diabetic foot
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29
Q

What are the HbA1c treatment targets

A

48 mmol/mol for new type 2 diabetics

53 mmol/mol for diabetics that have moved beyond metformin alone

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30
Q

How is type 2 diabetes medically managed

A

First line: metformin titrated from initially 500mg once daily as tolerated.

Second line add: sulfonylurea, pioglitazone, DPP-4 inhibitor or SGLT-2 inhibitor. The decision should be based on individual factors and drug tolerance.

Third line: Triple therapy with metformin and two of the second line drugs combined, or;
Metformin plus insulin

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31
Q

What are the types of insulin

A

Rapid-acting Insulins

  • These start working after around 10 minutes and last around 4 hours
  • Novorapid
  • Humalog
  • Apidra

Short-acting Insulins

  • These start working in around 30 minutes and last around 8 hours
  • Actrapid
  • Humulin S
  • Insuman Rapid

Intermediate-acting Insulins

  • These start working in around 1 hour and last around 16 hours
  • Insulatard
  • Humulin I
  • Insuman Basal

Long-acting Insulins

  • These starts working in around 1 hour and lasts around 24 hours:
  • Lantus
  • Levemir
  • Degludec (lasts over 40 hours)

Combinations Insulins

  • These contain a rapid acting and an intermediate acting insulin. In brackets is the proportion of rapid to intermediate acting insulin:
  • Humalog 25 (25:75)
  • Humalog 50 (50:50)
  • Novomix 30 (30:70)
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32
Q

What is hyperthyroidism

A

Hyperthyroidism is where there is over-production of thyroid hormone by the thyroid gland.

33
Q

What is thyrotoxicosis

A

Abnormal and excessive quantity of thyroid hormone in the body

34
Q

What is primary Hyperthyroidism

A

Due to thyroid pathology

It is the thyroid pathology itself that is behaving abnormally and producing excessive thyroid hormone

35
Q

What is secondary Hyperthyroidism

A

The condition where the thyroid is producing excessive thyroid as a result of overstimulation by thyroid stimulating hormone
The pathology is in the hypothalamus or pituitary

36
Q

What is Grave’s disease

A

An autoimmune condition where TSH receptor antibodies cause a primary hyperthyroidism. These TSH receptor antibodies are abnormal antibodies produced by the immune system that mimic TSH and stimulate the TSH receptors on the thyroid. This is the most common cause of hyperthyroidism.

37
Q

What is a toxic multi nodular goitre

A

AKA Pulmmer’s disease
A condition where nodules develop on the thyroid gland that act independently of the normal feedback system and continuously produce excessive thyroid hormone.

38
Q

What is exopthalmos

A

Term used to describe bulging of eyeball out of the socket caused by Grave’s Disease
Due to inflammation, swelling and hypertrophy of the tissue behind the eyeball that forces the eyeball forward

39
Q

What is pretibial myxoedema

A

A dermatological condition where there are deposits of mucin under the skin on the anterior aspect of the leg (the pre-tibial area)
This gives a discoloured, waxy oedematous appearance ti the skin over this area
Specific to Grave’s disease and is a reaction to the TSH receptor antibodies

40
Q

What are the causes of hyperthyroidism

A

Grave’s disease
Toxic multinodular goitre
Solitary toxic thyroid nodule
Thyroiditis (e.g. De Quervain’s, Hashimoto’s, postpartum and drug-induced thyroiditis)

41
Q

What are the universal features of hyperthyroidism

A
Anxiety and irritability
Sweating and heat intolerance
Tachycardia
Weight loss
Fatigue
Frequent loose stools
Sexual dysfunction
42
Q

What are the unique features of Grave’s disease type hyperthyroidism

A

These features all relate to the presence of TSH receptor antibodies:

  • Diffuse goitre (without nodules)
  • Graves eye disease
  • Bilateral exophthalmos
  • Pretibial myxoedema
43
Q

What are the unique features of Toxic multi nodular goitre type hyperthyroidism

A

Goitre with firm nodules
Most patients are aged over 50
Second most common cause of thyrotoxicosis (after Grave’s)

44
Q

What is a solitary toxic thyroid nodule

A

This is where a single abnormal thyroid nodule is acting alone to release thyroid hormone. The nodules are usually benign adenomas. They are treated with surgical removal of the nodule.

45
Q

What is De Quervain’s thyroiditis

A

Describes the presentation of a viral infection with fever, neck pain and tenderness, dysphagia and features of hyperthyroidism. There is a hyperthyroid phase followed by a hypothyroid phase as the TSH level falls due to negative feedback. It is a self-limiting condition and supportive treatment with NSAIDs for pain and inflammation and beta-blockers for symptomatic relief of hyperthyroidism is usually all that is necessary.

46
Q

What is thyroid storm

A

A rare presentation of hyperthyroidism. It is also known as “thyrotoxic crisis”. It is a more severe presentation of hyperthyroidism with pyrexia, tachycardia and delirium. It requires admission for monitoring and is treated the same way as any other presentation of thyrotoxicosis, although they may need supportive care with fluid resuscitation, anti-arrhythmic medication and beta-blockers.

47
Q

How is Hyperthyroidism managed

A
Carbimazole
Propylthiouracil
Radioactive iodine
Beta-blockers
Surgery
48
Q

How is Carbimazole used in Hyperthyroidism

A

Carbimazole is the first line anti-thyroid drug
Usually successful in treating patients with Grave’s disease, leaving them with normal thyroid function after 4-8 weeks
Once the patient has normal thyroid hormone levels, continue on a maintenance carbimazole and either:
-dose titrated to maintain normal levels (titration-block)
-dose is sufficient to block all production and then add in levothyroxine titrated to effect (block and replace)

49
Q

How is Propylthiouracil used in Hyperthyroidism

A

Second line anti-thyroid drug
Used similarly to Carbimazole
Small risk of severe hepatic reactions, including death, which is why Carbimazole is preferred

50
Q

How is radioactive iodine used in Hyperthyroidism

A

Treatment with radioactive iodine involves drinking a single dose of radioactive iodine. This is taken up by the thyroid gland and the emitted radiation destroys a proportion of the thyroid cells. This reduction in functioning cells results in a decrease of thyroid hormone production and thus remission from the hyperthyroidism. Remission can take 6 months and patients can be left hypothyroid afterwards and require levothyroxine replacement.

51
Q

What are the strict rules when treating hyperthyroidism with radioactive iodine

A

Must not be pregnant and are not allowed to get pregnant within 6 months
Must avoid close contact with children and pregnant women for 3 weeks (depending on the dose)
Limit contact with anyone for several days after receiving the dose

52
Q

How are beta-blockers used to treat Hyperthyroidism

A

Beta blockers are used to block the adrenalin related symptoms of hyperthyroidism. Propranolol is a good choice because it non-selectively blocks adrenergic activity as opposed to more “selective” beta blockers the work only on the heart. They do not actually treat the underlying problem but control the symptoms whilst the definitive treatment takes time to work. They are particularly useful in patients with thyroid storm.

53
Q

How is surgery used to treat Hyperthyroidism

A

A definitive option is to surgically remove the whole thyroid or toxic nodules. This effectively stops the production of thyroid hormone, however the patient will be left hypothyroid post thyroidectomy and require levothyroxine replacement for life.

54
Q

What is hypothyroidism

A

Describes an inadequate output of thyroid hormones by the thyroid gland

55
Q

What are the causes of hypothyroidism

A

Hashimoto’s thyroiditis
Iodine deficiency
Secondary to treatment of hyperthyroidism
Medications
Secondary hypothyroidism (central causes)

56
Q

What is Hashimoto’s thyroiditis

A

This is the most common causes of hypothyroidism in the developed world. It is caused by autoimmune inflammation of the thyroid gland. It is associated with antithyroid peroxidase (anti-TPO) antibodies and antithyroglobulin antibodies. Initially it causes a goitre after which there is atrophy of the thyroid gland.

57
Q

What is iodine deficiency type hypothyroidism

A

This is the most common cause of hypothyroidism in the developing world. Iodine is added to foods such as table salt to prevent iodine deficiency.

58
Q

What medications can cause hypothyroidism

A

Lithium inhibits the production of thyroid hormones in the thyroid gland and can cause a goitre and hypothyroidism.

Amiodarone interferes with thyroid hormone production and metabolism, usually causing hypothyroidism but it can also cause thyrotoxicosis.

59
Q

What are the causes of secondary hypothyroidism

A

This is where the pituitary gland is failing to produce enough TSH. This is often associated with a lack of other pituitary hormones such as ACTH. This is called hypopituitarism and has many causes:

  • Tumours
  • Infection
  • Vascular (e.g. Sheehan -Syndrome)
  • Radiation
60
Q

How does hypothyroidism present

A
Weight gain
Fatigue
Dry skin
Coarse hair and hair loss
Fluid retention (oedema, pleural effusions, ascites)
Heavy or irregular periods
Constipation
61
Q

How is hypothyroidism investigated

A

Primary Hypothyroidism is caused by thyroid gland insufficiency. Thyroid hormones (i.e. free T3 and T4) will be low. TSH will be high because there is no negative feedback to the brain, so the pituitary produces lots of TSH to try and get the thyroid working.

Secondary Hypothyroidism is caused by pituitary pathology that results in low production of TSH. Thyroid hormones will be low due to the low TSH.

62
Q

How will blood tests show hypothyroidism

A

Primary hypothyroidism:

  • High TSH
  • Low T3 and T4

Secondary hypothyroidism:

  • Low TSH
  • Low T3 and T4
63
Q

How is hypothyroidism managed

A

Replacement of thyroid hormone with oral levothyroxine is the treatment of hypothyroidism. Levothyroxine is synthetic T4, and metabolises to T3 in the body. The dose is titrated until TSH levels are normal. When starting levothyroxine, initially measure TSH levels monthly until stable, then once stable it can be checked less frequently unless they become symptomatic.

If the TSH level is high, the dose is too low and needs to be increased. If the TSH is low, the dose is too high and needs to be reduced.

64
Q

What is Cushing’s syndrome

A

Used to refer to the signs and symptoms that develop after prolonged abnormal elevation of cortisol. Cushing’s Disease is used to refer to the specific condition where a pituitary adenoma (tumour) secretes excessive ACTH. Cushing’s Disease causes a Cushing’s syndrome, but Cushing’s Syndrome is not always caused by Cushing’s Disease.

65
Q

What are the features of Cushing’s syndrome

A

Round in the middle with thin limbs:

  • Round “moon” face
  • Central Obesity
  • Abdominal striae
  • Buffalo Hump (fat pad on upper back)
  • Proximal limb muscle wasting

High levels of stress hormone:

  • Hypertension
  • Cardiac hypertrophy
  • Hyperglycaemia (Type 2 Diabetes)
  • Depression
  • Insomnia

Extra effects:

  • Osteoporosis
  • Easy bruising and poor skin healing
66
Q

What are the causes of Cushing’s syndrome

A

Exogenous steroids (in patients on long term high dose steroid medications)
Cushing’s Disease (a pituitary adenoma releasing excessive ACTH)
Adrenal Adenoma (a hormone secreting adrenal tumour)
Paraneoplastic Cushing’s (when excess ACTH is released from a cancer, small cancer is the most common cause)
Ectopic ACTH (ACTH from somewhere other than the pituitary)

67
Q

How is Cushing’s syndrome diagnosed using dexamethasone suppression tests

A

Dexamethasone suppression test. This involves initially giving the patient the “low dose” test. If the low dose test is normal, Cushing’s can be excluded. If the low dose test is abnormal, then a high dose test is performed to differentiate between the underlying causes.

To perform the test the patient takes a dose of dexamethasone (a synthetic glucocorticoid steroid) at night (i.e. 10pm) and their cortisol and ACTH is measured in the morning (i.e. 9am). The intention is to find out whether the dexamethasone suppresses their normal morning spike of cortisol.

Low Dose Dexamethasone Suppression Test (1mg dexamethasone):
A normal response is for the dexamethasone to suppress the release of cortisol by effecting negative feedback on the hypothalamus and pituitary. The hypothalamus responds by reducing the CRH output. The pituitary responds by reducing the ACTH output. The lower CRH and ACTH levels result in a low cortisol level. When the cortisol level is not suppressed, this is the abnormal result seen in Cushing’s Syndrome.

High Dose Dexamethasone Suppression Test (8mg dexamethasone): The high dose dexamethasone suppression test is performed after an abnormal result on the low dose test.

In Cushing’s Disease (pituitary adenoma) the pituitary still shows some response to negative feedback and 8mg of dexamethasone is enough to suppress cortisol.

Where there is an adrenal adenoma, cortisol production is independent from the pituitary. Therefore, cortisone is not suppressed however ACTH is suppressed due to negative feedback on the hypothalamus and pituitary gland.

Where there is ectopic ACTH (e.g. from a small cell lung cancer), neither cortisol or ACTH will be suppressed because the ACTH production is independent of the hypothalamus or pituitary gland.

68
Q

What investigations can be carried out in suspected Cushing’s syndrome

A

Dexamethasone suppression test

24-hour urinary free cortisol (can be used as an alternative to the dexamethasone suppression test to diagnose Cushing’s syndrome but does not indicate the underlying cause and is cumbersome to carry out.)

FBC (raised white cells) and electrolytes (potassium may be low if aldosterone is also secreted by an adrenal adenoma)

MRI brain for pituitary adenoma

Chest CT for small cell lung cancer

Abdominal CT for adrenal tumours

69
Q

How is Cushing’s syndrome treated

A

The main treatment is to remove the underlying cause (surgically remove the tumour):

  • Trans-sphenoidal (through the nose) removal of pituitary adenoma
  • Surgical removal of adrenal tumour
  • Surgical removal of tumour producing ectopic ACTH

If surgical removal of the cause is not possible another option is to remove both adrenal glands and give the patient replacement steroid hormones for life.

70
Q

What is adrenal insufficiency

A

Where the adrenal glands do not produce enough steroid hormones, particularly cortisol and aldosterone
Steroids are essential for life and therefore the condition is life-threatening unless the hormones are replaced

71
Q

What is Addison’s disease

A

The specific condition where the adrenal glands have been damaged, resulting in a reduction in the secretion of cortisol and aldosterone. This is also called Primary Adrenal Insufficiency. The most common cause is autoimmune.

72
Q

What is secondary adrenal insufficiency

A

The result of inadequate ACTH stimulating the adrenal glands, resulting in low cortisol release. This is the result of loss or damage to the pituitary gland. This can be due to surgery to remove a pituitary tumour, infection, loss of blood flow or radiotherapy. There is also a condition called Sheehan’s syndrome where massive blood loss during childbirth leads to pituitary gland necrosis.

73
Q

What is tertiary adrenal insufficiency

A

The result of inadequate CRH release by the hypothalamus. This is usually the result of patients being on long term oral steroids (for more than 3 weeks) causing suppression of the hypothalamus. When the exogenous steroids are suddenly withdrawn the hypothalamus does not “wake up” fast enough and endogenous steroids are not adequately produced. Therefore long term steroids should be tapered slowly to allow time for the adrenal axis to regain normal function.

74
Q

What are the signs and symptoms of adrenal insufficiency

A

Fatigue
Nausea
Cramps
Abdominal pain
Reduced libido
Bronze hyperpigmentation to skin (ACTH stimulates melanocytes to produce melanin)
Hypotension (particularly postural hypotension)

75
Q

What are the investigations in suspected adrenal insufficiency

A

Hyponatraemia (low sodium) is a key biochemical clue. Sometimes the only presenting feature of adrenal insufficiency is hyponatraemia.

Hyperkalaemia (high potassium) is also possible.

Early morning cortisol (8-9am) has a role but is often falsely normal.

A short synacthen test is the test of choice to diagnose adrenal insufficiency.

ACTH. In primary adrenal failure the ACTH level is high as the pituitary is trying very hard to stimulate the adrenal glands without any negative feedback in the absence of cortisol. In secondary adrenal failure the ACTH level is low as the reason the adrenal glands are not producing cortisol is that they are not being stimulated by ACTH.

Adrenal autoantibodies are present in 80% of autoimmune adrenal insufficiency: adrenal cortex antibodies and 21-hydroxylase antibodies

CT / MRI adrenals if suspecting an adrenal tumour, haemorrhage or other structural pathology (not recommended by NICE for autoimmune adrenal insufficiency).

MRI pituitary gives further information about pituitary pathology.

76
Q

What is the short synacthen test

A

ACTH stimulation test
Test of choice for adrenal insufficiency
Ideally performed in the morning
The test involves giving synacthen, which is synthetic ACTH. The blood cortisol is measured at baseline, 30 and 60 minutes after administration. The synthetic ACTH will stimulate healthy adrenal glands to produce cortisol and the cortisol level should at least double. A failure of cortisol to rise (less than double the baseline) indicates primary adrenal insufficiency (Addison’s disease).

77
Q

How is adrenal insufficiency treated

A

Replacement steroids titrated to signs, symptoms and electrolytes
Hydrocortisone is a glucocorticoid hormone and is used to replace cortisol. Fludrocortisone is a mineralocorticoid hormone and is used to replace aldosterone if aldosterone is also insufficient.

Patients are given a steroid card and an emergency ID tag to alert emergency services that they are dependent on steroids for life. Doses should not be missed as they are essential to life. Doses are doubled during an acute illness until they have recovered to match the normal steroid response to illness.

78
Q

What is an addisonian crisis

A

AKA Adrenal crisis
Addisonian crisis is the term used to describe an acute presentation of severe Addisons, where the absence of steroid hormones leads to a life threatening presentation.

They present with:
Reduced consciousness
Hypotension
Hypoglycaemia, hyponatraemia, hyperkaemia
Patients can be very unwell

It can be the first presentation of Addisons Disease or triggered by infection, trauma or other acute illness in someone with established Addisons. It can present in someone on long term steroids suddenly withdrawing those steroids.

Do not wait to perform investigations and establish a definitive diagnosis before treating someone with suspected Addisonian Crisis as this is life threatening and they need immediate treatment.

79
Q

How is an addisonian crisis managed

A

Intensive monitoring if unwell
Parenteral steroids (i.e. IV hydrocortisone 100mg stat then 100mg every 6 hours)
IV fluid resuscitation
Correct hypoglycaemia
Careful monitoring of electrolytes and fluid balance