Adrenals

Question 1

How to manage Addisonion crisis?

Answer 1

IV 0.9% Saline
IV hydrocortisone

Question 2

Cushings syndrome - anatomy of the adrenal gland?

Answer 2

Fasiculata tumour secreting cortisol

Question 3

How to manage Addison’s disease?

Answer 3

Hydrocortisone + sick-day rules
Fludrocortisone

Question 4

What is Conn’s syndrome - adrenal anatomy?

Answer 4

Glomerulosa secreting aldosterone

Question 5

Phaeochromocytoma genetic links? (3)

Answer 5

• MEN2
• Von Hippel Lindau Syndrome
• Neurofibromatosis type 1

Question 6

Signs and symptoms seen in Congenital adrenal hyperplasia?

Answer 6

21-OH deficiency :

• Salt losing crisis (due to low aldosterone produced)
• Virilisation (due to high testosterone / androgen produced instead)

Question 7

Why may you have
Hyperplastic adrenal glands?

Answer 7

Cushings disease or ectopic ACTH

Question 8

What are the most common causes of Addison’s disease in the UK

Answer 8

**Autoimmune (UK most common) **

TB (worldwide most common)

Metastasis

Haemorrhage (Waterhouse-Friedrichson Syndrome)
Iatrogenic

Question 9

Adrenal histology:

Wasted adrenal glands likely to be caused by which conditions?

Answer 9

Addison’s disease or long term steroid treatment

Question 10

How to treat Ectopic ACTH causing cushings syndrome? (3)

Answer 10

Ketoconazole
Metyrapone
Mifepristone

Question 11

How to confirm the cause of cushings?

Answer 11

IPSS : inferior petrosal sinus sampling:

Catheter into jugular vein, distinguishes pituitary dependent cushings from ectopic ACTH by measuring ACTH in veins coming from pituitary

Question 12

What is a Paeochromocytoma - adrenal anatomy?

Answer 12

Medulla tumour secreting adrenaline

Question 13

How to test for phaeochromocytoma?

How to treat this medical emergency?

Answer 13

urinary catecholamines will be high (due to medulla secreting access adrenaline)

• Immediate Alpha blockade with phenoxybenzamine
• then add a beta blockade
• then surgery

Question 14

How to test for addisons?

Answer 14

Short synacthen test:
Measure cortisol and ACTH at the start
- give 250ug ACTH, IM
Check cortisol levels at 30 and 60 minutes

The cortisol will be very low <10nM (not making any cortisol in response to exogenous ACTH challenge)
ACTH will be >100g/dl

Question 15

What might this be?

33 yr old, HTN
Urea and electrolytes:
Na = 147
K = 2.8
U = 4.0
Glucose = 4.0mM

Plasma aldosterone raised
Plasma renin suppressed

Answer 15

Conns syndrome (primary hyperaldosteronism)

• too much aldosterone = HTN and suppression of renin at JGA

Question 16

Addison’s disease investigation

Answer 16

9am cortisol to confirm low cortisol

to confirm : short synACTHen test

–> blood sample to measure cortisol before test
–> shorth ACTH synthetic injected to stimulate glands to produce cortisol
–> blood samples after 30 and 60 mins to measure cortisol levels

Question 17

What is Cushing’s syndrome?

Answer 17

High cortisol which can have exogenous cause (glucocorticoid medications) or endogeneous cause (cushings disease, adrenal adenoma, ectopic ACTH)

Question 18

What are the most common causes of Addison’s disease?

Answer 18

Autoimmune (UK most common)

TB (worldwide most common)

Metastasis

Haemorrhage (Waterhouse-Friedrichson Syndrome)
Iatrogenic

Question 19

31yo, profound fatigue, acutely unwell for a few days, vomiting

Test results:
Na = 125
K = 6.5
U = 10
FT4 = <5nM
TSH = >50mU/L

What is this?

Answer 19

1. Primary hypothyroidism
2. Unusual U&Es –> Addisons
   (mineralcorticoid and glucocorticoid deficiency)

If you have both Addisons + hypothyroidism =
Schmidt’s syndrome aka polyglandular autoimmune syndrome type II

Question 20

What is Cushing’s disease?

Answer 20

Pituitary cause of cushings syndrome (pituitary adenoma excess ACTH)

Question 21

What does this patient have?

Obese women, T2DM, HTN, Bruising

Na - 146
K - 2.9
Aldosterone - <75
Renin - Low
Urea - 4.0
Glucose - 14

Answer 21

Cushings syndrome (low potassium and high glucose with cortisol pushing the hypertension)

Low potassium : excess cortisol can act like aldosterone by binding to mineralcorticoid receptors in kidney = stimulate sodium retention at expence of potassium excretion. Also in turn increases blood pressure.

Question 22

What deficiency drives Congenital adrenal hyperplasia?

Answer 22

21-OH deficiency

Question 23

Why is the high dose dexamethasone suppression test NOT DONE anymore?

Answer 23

because 85% are pituitary-dependant without the test and so doing a high-dose test is less accurate (false +ve = 20%) than guessing and so it is not useful

Equally, pituitary MRIs with gadolinium enhancement is not that useful but can be definitive

Question 24

What are the most common causes of Addison’s disease worldwide

Answer 24

Autoimmune (UK most common)

TB (worldwide most common)

Metastasis

Haemorrhage (Waterhouse-Friedrichson Syndrome)
Iatrogenic

Question 25

How to screen for Cushing’s syndrome?

Answer 25

Look for hypercortisolism:
11pm salivary cortisol
–> If low : Rules out cushings
–> if high : Suspicion

Low dose dexamethasone suppression test:
The patient takes 1 mg of dexamethasone at 11 pm, and cortisol is measured at 9 am the next morning.
–> Normal suppression : no cushings, may be peusdo-cushings
–> Abnormal suppression : Cushings syndrome (cushings disease / steroids / ectopic / adrenal adenoma

== Do IPSS to confirm cause

Question 26

How to treat Cushings syndrome from adrenal mass?

Answer 26

Adrenalectomy +/- steroid replacement

• beware of Nelson’s syndrome:
  Leads to pituitary enlargement to compensate releasing loads of ACTH causing alot of hyperpigmentation.

Question 27

What is Addison’s disease?

Answer 27

Not enough cortisol + aldosterone:

Question 28

phaeochromocytoma emergency presentation?

Answer 28

Severe hypertension, arrythmias and death

Because of adrenaline tumour

Question 29

What is nelson’s syndrome?

Answer 29

Adrenalectomy +/- steroid replacement

beware of Nelson’s syndrome:
Leads to pituitary enlargement to compensate for adrenalectomy by releasing loads of ACTH causing alot of hyperpigmentation.