Chempath: Adrenal Disease

Question 1

What are the layers of the adrenal gland?

Answer 1

From outside to in = GFR M

Zona Glomerulosa
Zona Fasiculata
Zone reticularis

Medulla

Question 2

What hormones are produced by each layer of the adrenal gland?

Answer 2

“The deeper you go, the sweeter it gets”

Glomerulosa = MINERALOCORTICOIDS Aldosterone –> Na/K + BP (Salt)

Fasiculata = GLUCOCORTICOIDS Cortisol –> Glucose, insulin axis (Sugar)

Reticularis = SEX STEROIDS Androgens –> DHEAS and androstenedione (Sex)

Adrenal medulla = Catecholamines (adrenaline + noradrenaline)

Question 3

What are some adrenal diseases?

Answer 3

Addisons = Primary adrenal failure

Cushing = Raised cortisol

Conns = Raised aldosterone

Phaeochromocytoma = Adrenal tumour

Question 4

What is addisons and what are the common causes? what happens to hormones + changes as a result?

Answer 4

Addisons = primary adrenal failures

↓ Aldosterone = ↓ Na, ↑ K, ↓BP
↓ Cortisol = ↓ Glucose

Question 5

What are the different causes of Addisons diease + which are most common in uk / world?

Answer 5

Autoimmune- UK

TB- worldwide

Steroid withdrawal

Adrenal haemorrhage (Waterhouse-Friderichsen syndrome)

Amyloidosis

Question 6

How does steroid withdrawal cause addisons?

Answer 6

Negatively feedsback on the the H-P-A axis (inhibiting endogenous secretion of corticosteroids etc.)

Question 7

How can adrenal haemorrhage / waterhouse-friderichsen syndrome cause addisons?

Answer 7

Haemorrhage –> usually caused by meningogoccal sepsis (stress –> increased cortisol/adrenaline/ACTH production –> increased venous pressure –> haemorrhage)

Question 8

What are the features of Addisons disease?

Answer 8

Lethargy, anorexia, weight-loss, depression

Hyperpigmentation: typically look ‘tanned’ (this is only for primary, aka Addison’s, NOT secondary)

HypoNa, HyperK, postural hypotension, hypoglycaemia

Question 9

What investigations are used in Addisons and what are the results?

Answer 9

Initial = Random cortisol

Definitive = Short Synacthen test:

• Measure cortisol + ACTH at baseline
• Give IM synthetic ACTH
• Check cortisol at 30 + 60 minutes

Cortisol RISES ( > 550nmol/L) -  adrenals working
Cortisol LOW -  adrenals NOT working

Question 10

What is the mx of addisons?

Answer 10

Glucocorticoid replacement –> hydrocortisone (or pred)

Mineralocorticoid replacement –> fludrocortisone

In some cases, e.g. persistent fatigue, DHEA replacement is given by endo

Question 11

What are addisonian crises and how can they be managed?

Answer 11

Present with collapse, shock

Addisonian crisis may occur due to severe infection or surgery causing ↑ stress + acute exacerbation, withdrawal of steroids, adrenal haemorrhage

Treatment: 0.9% NaCl 1L +/- dextrose + IV 100mg hydrocortisone

Question 12

What is Schmidts Syndrome?

Answer 12

Addison’s + Primary hypothyroidism

Question 13

What is conn’s syndrome? what can it be caused by? present

Answer 13

Conns is primary hyperALDOSTERONISM

Caused by tumour (unilateral) or hyperplasia (bilateral)

Can present w/ uncontrollable HTN

Question 14

What are the ix in conns syndrome?

Answer 14

1st line = Plasma aldosterone:renin ration (↑ aldosterone, ↓ renin)

High res CT = see if bilateral or unilateral leison

Adrenal vein sampling may also be useful

others = ↑ BP, ↑ Na, ↓ K, Alkalosis

Question 15

What are the renin and aldosterone levels in conns?

What condition has the opposite of this?

Answer 15

Conns = ↑ aldosterone, ↓ renin

Renal artery stenosis = ↓ aldosterone, ↑ renin

Question 16

What is the mx of conns syndrome?

Answer 16

Adrenal adenoma: SURGERY

Bilateral hyperplasia: aldosterone antagonist (e.g. spiro)

Question 17

What is Cushings syndrome?

Answer 17

This when pts have high cortisol

Question 18

What are the main causes of Cushing syndrome?

Answer 18

Oral steroids- MOST COMMON

Adrenal adenoma (or carcinoma- RARE)

Pituitary tumour secreting ACTH- Cushing’s disease

Ectopic tumour secreting ACTH (e.g. small cell)

Question 19

What investigation is the dynamic test for Cushings syndrome?

Answer 19

Low-Dose Dexamethasone Suppression Test

Failure to suppress cortisol –> send for IPSS (inferior petrosal sinus sampling) - used to check if pituitary cause or not

If cortisol suppressed –> pseudo-Cushing’s (i.e. they may be obese)

Question 20

What is the mx

Answer 20

underlying cause. Surgical excision of tumours.

Question 21

Cause of collapse in addisonian crisis ?

Answer 21

Renal loss of Na -> loss of water

Question 22

VSA 1: A patient is hypertensive (BP: 160/100mmHg). Investigations show the following.

Normal U+Es (na upper lim, k lower)
Aldosterone High
Renin Supressed

What is the likely diagnosis?

Answer 22

Conn’s syndrome

↑ aldosterone, ↓ renin
↑ Na, ↓ K

Question 23

What does phaeochromocytoma cause? ix?

Answer 23

Periodic very high BP

24 hr urine metanephrine (which would be very highly raised eg VMA)

Question 24

Mx of phaeo?

Answer 24

Medical emergency

1. Alpha block
2. beta block
3. surgical removal

Question 25

What is phaeo associated w

Answer 25

MEN 2, NF I, VHL syndrome

Question 26

What is phaeo associated w

Answer 26

MEN 2, NF I, VHL syndrome

Question 27

SBA: Increased insulin sensitivity results in low plasma glucose and occurs in which of the following?:

1. Acromegaly
2. Cushing’s disease
3. Phaeochromocytoma
4. ACTH deficiency
5. PCOS

Answer 27

4. ACTH deficiency
5. Acromegaly- associated with insulin resistance
6. Cushing’s disease- associated with insulin resistance
7. Phaeochromocytoma- associated with insulin resistance
8. PCOS- associated with insulin resistance