Hypofunction

Question 1

Give 4 causes of primary adrenal insufficiency?

Answer 1

Addison’s disease, congenital adrenal hyperplasia, adrenal TB, adrenal malignancy

Question 2

Give 3 causes of secondary adrenal insufficiency?

Answer 2

Lack of ACTH stimulation, iatrogenic (overuse of steroids), hypothalamic/pituitary disease

Question 3

Give 3 causes of acute primary adrenal insufficiency?

Answer 3

Rapid withdrawal of steroids, Addison crisis, adrenal haemorrhage

Question 4

Give 5 causes of chronic primary adrenal insufficiency?

Answer 4

Addison’s disease, autoimmune adrenalitis, infections, metastatic malignancy, sacroidosis/amyloid/haemochromatosis

Question 5

What is the most common cause of primary adrenal insufficiency?

Answer 5

Addison’s disease

Question 6

What happens in Addison’s disease?

Answer 6

Autoimmune destruction of the adrenal cortex (90% destroyed before any symptoms)

Question 7

Are autoantibodies present in Addison’s disease?

Answer 7

Yes, in 70% of cases

Question 8

What other autoimmune diseases is Addison’s disease in association with?

Answer 8

Type 1 diabetes, autoimmune thyroid disease, pernicious anaemia

Question 9

What is the onset of Addison’s disease?

Answer 9

Insidious- it manifests once there has been significant hormone losses

Question 10

What does decreased mineralocorticoid in Addison’s disease result in?

Answer 10

K+ retention and Na+ loss (hyperkalaemia, hyponatraemia). Also volume depletion and hypotension.

Question 11

What does decreased glucocorticoid in Addison’s disease result in?

Answer 11

Hypoglycaemia

Question 12

What are some more vague symptoms of Addison’s disease?

Answer 12

Weakness, fatigue, anorexia, weight loss, nausea, vomiting, abdominal pain, diarrhoea

Question 13

What causes increased pigmentation in Addison’s disease?

Answer 13

Increased ACTH

Question 14

Is hyperpigmentation a feature of secondary adrenal insufficiency?

Answer 14

No

Question 15

What may cause an Addisonian crisis?

Answer 15

Stress

Question 16

What are symptoms of an Addisonian crisis?

Answer 16

Abdominal pain, vomiting, hypotension, shock, death

Question 17

What is the suspicious biochemistry which would suggest a diagnosis of Addison’s?

Answer 17

Hyperkalaemia, hyponatraemia, hypoglycaemia

Question 18

What is the stimulation test used for adrenal insufficiency?

Answer 18

Synacthen test

Question 19

When should you measure cortisol in a synacthen test?

Answer 19

Before, and 30 mins after giving the ACTH injection

Question 20

What would normal baseline readings be in a synacthen test?

Answer 20

Before ACTH= > 250nmol/l. After ACTH= > 550nmol/l.

Question 21

What will the levels of ACTH be in primary adrenal insufficiency?

Answer 21

Raised

Question 22

What will the levels of renin and aldosterone be in Addison’s disease?

Answer 22

Renin- high, aldosterone- low

Question 23

Should you wait for a diagnosis of Addison’s disease before you give treatment?

Answer 23

No

Question 24

What hormones need to be replaced in Addison’s disease?

Answer 24

Cortisol and aldosterone

Question 25

What is used as cortisol replacement in Addison's?

Answer 25

Hydrocortisone

Question 26

How should hydrocortisone be given in Addison's disease?

Answer 26

Give IV if acutely unwell, otherwise give 15-30mg/day in divided doses

Question 27

Why is hydrocortisone given in divided doses?

Answer 27

To mimic the normal diurnal variation of cortisol

Question 28

What is used as aldosterone replacement in Addison's?

Answer 28

Fludrocortisone

Question 29

What needs to be carefully monitored in patients on fludrocortisone?

Answer 29

K+ and BP

Question 30

What must patients with Addison's disease be educated about?

Answer 30

Sick days, wearing ID and the dangers of stopping medication suddenly

Question 31

What are congenital adrenal hyperplasia syndromes?

Answer 31

Rare conditions of steroid synthesis, associated with enzyme defects in the steroid pathways

Question 32

What is the cause of 95% of CAH cases?

Answer 32

21 alpha hydroxylase deficiency

Question 33

How is 21 alpha hydroxyls deficiency inherited?

Answer 33

Autosomal recessive

Question 34

What are some ways that CAH may present in boys?

Answer 34

Adrenal insufficiency, poor weight gain, biochemical pattern of Addison's

Question 35

How does CAH generally present in girls?

Answer 35

Genital ambiguity

Question 36

What are some non-classical presentations of CAH in girls?

Answer 36

Acne, oligomenorrhoea, precocious puberty, infertility

Question 37

How is CAH treated in children?

Answer 37

Glucocorticoid and mineralocorticoid replacement, possible surgical correction and achieving maximal growth potential

Question 38

How is CAH treated in adults?

Answer 38

Controlling androgen excess, restoring fertility and avoiding hormone over-replacement

Question 39

What are the two main causes of secondary adrenal insufficiency?

Answer 39

Failure to stimulate the adrenal cortex or suppression of the adrenal cortex

Question 40

How can there be failure to stimulate the adrenal cortex?

Answer 40

Lack of CRH and/or ACTH from tumours, surgery or radiation

Question 41

How can there be suppression of the adrenal cortex?

Answer 41

Exogenous steroid use as this suppresses release of CRH/ACTH

Question 42

The clinical features of secondary adrenal insufficiency are similar to primary, with the exceptions of what?

Answer 42

The skin is not hyperpigmented, aldosterone production is normal

Question 43

Why is aldosterone production normal in secondary adrenal insufficiency?

Answer 43

Aldosterone is monitored by RAAS, not ACTH

Question 44

What hormone replacement is necessary for secondary adrenal insufficiency?

Answer 44

Hydrocortisone only