Case 1 - Adrenal crisis and Adrenal glands booklet

Question 1

Presentation of adrenal crisis

Answer 1

• Use of long term steroids - abruptly stopped due to V/D
• Fatigue
• Weakness
• Hypotensive
• Tachycardia
• Unwell appearance

Question 2

Investigation findings of adrenal crisis

Answer 2

• Hyponatraemia
• Hyperkalaemia
• Raised urea
• Hypoglycaemia
• Mild anaemia

Question 3

Immediate management of adrenal crisis

Answer 3

• IV/IM hydrocortisone 100mg (then 200mg every 24hrs diluted in 5% dextrose, or 50mg every 6hrs, 100mg in severe obese)
• IV fluids
• Correct hypoglycaemia - IV dextrose
• Monitor electrolytes and fluid balance

Question 4

Advice for patient to prevent similar future episodes of crisis

Answer 4

• Inform healthcare professionals early on if problem with taking steroids
• Emergency numbers for endocrinology team
• Ensure pt knows how to adminuter IM hydrocortisone in emergency
• Steroid sick day rules - dose needs increasing if feverish, V/D
• Signs of crisis

Question 5

Evidence pts can keep to let people know they are on long term steroids

Answer 5

• Steroid card
• Emergency crisis letter
• Medic Alert - can call helpline 24/7 and let any professionals access full medical records

Question 6

What is produced in cortex?

Answer 6

• Glucocorticoids
• Androgens
• Mineralcorticoids

Question 7

Glucocorticoids -main one

Answer 7

Cortisol

Question 8

When is cortisol highest and lowest?

Answer 8

Higest 8am
Lowest midnight

Question 9

What is most cortisol bound to?

Answer 9

• Cortisol binding globulin (CBG)
• Albumin
• Only a small amount is active free form

Question 10

What do cortisol immunoassays measure?

Answer 10

• Total cortisol - bound and free
• So some conditions eg oestrogen therapy can stimulate CBG levels but may not affect biologically active free levels of cortisol

Question 11

How is cortisol controlled?

Answer 11

ACTH

Question 12

How are androgens controlled?

Answer 12

ACTH

Question 13

Important roles of androgens in…

Answer 13

• Adult women
• Both sexes pre-puberty
• Adult men mainly rely on testicular production of androgens

Question 14

Conversion of androgens

Answer 14

DHEA, DHEA-S and androstenedione converted to more potesmt testosterone and dihydrotestosterone in peripheral tissues

Question 15

Where do androgens exert effects?

Answer 15

• Sebaceous glands
• Hair follicles
• Prostate gland
• External genitalia

Question 16

Main mineralocorticoid

Answer 16

Aldosterone

Question 17

Regulation of aldosterone

Answer 17

Via RAAS

Question 18

When is renin activated?

Answer 18

• Low blood volume
• Hyponatraemia
• Hyperkalaemia

Question 19

Where does aldosterone act and what does it cause?

Answer 19

• Renal distal convoluted tubule
• Sodium retention
• Potassium excretion

Question 20

Adrenal medulla - what does it do?

Answer 20

• Tissue made up of sympathetic nervous system
• Secretes adrenaline, noradrenaline, dopamine and metabolites (metanephrines, nor-metanephrines etc)

Question 21

What is primary adrenal insufficiency (Addisons disease)?

Answer 21

• Destruction of adrenal gland or genetic defects in steroids syntheisis
• All three zones cortex often affected

Question 22

Clinical features addisons

Answer 22

• Fatigue
• Weakness
• Anorexia
• Weight loss
• Nausea
• Abdominal pain
• Dizziness

VAGUE AND NON SPECIFIC

Question 23

What does glucocorticoid loss lead to in Addisons?

Answer 23

• Hypoglycaemia
• Increased pigmentation due to increased ACTH from reduced cortisol negative feedback

Question 24

What can androgen deficiency in women cause?

Answer 24

• Reduced libido
• Loss axillary and pubic hair

Question 25

Addison crisis - what is it

Answer 25

MEdical emergency Need IV fluids and IV hydrocortisone

Question 26

Commonest cause of addisons

Answer 26

Autoimmune This is tested for by detection of adrenal autoantibodies

Question 27

Biochemical markers for Addisons

Answer 27

* Hyponatraemia * Hyperkalaemia * Raised urea * Hypoglycaemia * Mild anaemia

Question 28

Confirmatory test for Addisons

Answer 28

* 9am cortisol shown to be low with raised ACTH * Synacthen test needed for confirmation

Question 29

Management for addisons

Answer 29

* Lifelong glucocorticoid and mineralocorticoid replacement therapy * Hydrocortisone first choice * Mineralocorticoid replacement is given as Fludrocortisone

Question 30

Illness steroid rules

Answer 30

Double dose of gluccocorticoids in illness Continue on double dose until resolved

Question 31

What delivery may steroids need to be given in illness?

Answer 31

IM or IV if surgery or prolonged V/D

Question 32

Alert mechanisms for those on long term steroid therpay

Answer 32

* Steroid card * Medical alert jewellery * Emergency contacts for endocrine team

Question 33

What can cause secondary adrenal insuffiency (ACTH deficiency)?

Answer 33

Hypopituitarism

Question 34

Treatment secondary adrenal insufficiency

Answer 34

* Hydrocortisone * But do not need fludrocortisone

Question 35

What can long term steroid use cause?

Answer 35

ACTH supression - patients need to be advised to not stop steroids abruptly

Question 36

What are phaechromocytomas and paragangliomas?

Answer 36

Catecholamine secreting tumours Occur in 0.1% of people with HTN

Question 37

Where do phaechromocytomas and paragangliomas arise from?

Answer 37

90% adrenal medulla other 10% arise from extra adrenal chromaffin tissue (these are called paragangliomas)

Question 38

Familial aspect of phaechromocytomas and paragangliomas

Answer 38

In 30% of patients Esp in bilateral, extradadrenal or malignat tumours

Question 39

Clinical features of phaechromocytomas and paragangliomas

Answer 39

* Headache * Swelling * Pallor * Palpitations * Anxiety/panic attacks * HTN

Question 40

What can phaechromocytomas lead to if untreated?

Answer 40

* Hypertensive crisis * Encephalopathy * Hyperglycaemia * Pulmonary oedema * Cardiac arrhythmias * Death

Question 41

Diagnosis of phaechromocytomas and paragangliomas

Answer 41

* Elevated catecholamins or their metabolites (metanephrines) using 24 urine catecholamines and plasma metanephrines * Radiological localisation of tumour via CT/MRI abdomen | Whole body MRI if not localised

Question 42

Option if tumour is not located on MRI

Answer 42

MIBG - I-meta-lodobenzylguanidine

Question 43

When should we genetically test someone with phaechromocytomas and paragangliomas

Answer 43

* Young age presentation * Multifocal, malignant or extra-adrenal disease * Identification of mutation should lead to annual screening for new/recurrent disease and genetic testing for first degree relatives

Question 44

Management for phaechromocytomas and paragangliomas?

Answer 44

Surgical excision Laparoscopically or open

Question 45

What may some patients need at diagnosis of phaechromocytomas and paragangliomas

Answer 45

* Alpha or beta blockade * If alpha - phenoxybenzamine used (sometimes doxazosin) * This should be done before beta blockade to avoid unapposed alpha adrenergic stimulation and risk of hypertensive crisis (if have beta blocker before alpha, no beta 2 vasodilation = htn crisis)

Question 46

What can beta blockers be used to control in phaechromocytomas and paragangliomas

Answer 46

Can control reflex tachycardia