Adrenocortical Hypofunction

Question 1

what are some causes of acute primary adrenocortical insufficiency

Answer 1

rapid withdrawal of steroid treatment

massive adrenal haemorrhage

• newborn, anticoagulant treatment, DIC, septicaemic infection

Question 2

what can occur as a crisis in patients with chronic adrenocortical insufficiency due to stress

Answer 2

acute adrenocortical insufficiency

eg in Addison’s patients after infection or not increasing dose of steroid treatment

Question 3

DIC

Answer 3

disseminated intravascular coagulation

widespread activation of the clotting cascade

Question 4

waterhouse-friderichsen

Answer 4

causes acute adrenocortical insufficiency

adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection eg Neisseria meningitides

Question 5

addison’s disease

Answer 5

destruction of the adrenal cortex leads to glucocorticoid and mineralocorticoid deficiency

signs are capricious

reduced cortisol levels lead to increased CRH and ACTH production

Question 6

what is the most common cause of primary adrenal insufficiency

Answer 6

addison’s

Question 7

causes of Addison’s

Answer 7

80% are due to autoimmunity

(autoimmune adrenalitits results from the destruction of the adrenal cortex by antibodies, 21-hydroxylase as the common antigen)

infection: TB, fungal, HIV

metastatic malignancy: breast and lung

unusually: amyloid, sarcoidosis, haemochromatosis

Question 8

what can be used as a marker in autoimmune causes of Addison’s

Answer 8

21-hydroxylase

autoantibodies present in 70%

Question 9

what is associated with autoimmune causes of Addison’s

Answer 9

other autoimmune diseases eg T1DM, autoimmune thyroid disease, pernicious anaemia

Question 10

what fungal infection can cause Addison’s

Answer 10

histoplasma

Question 11

what is often seen in patients with HIV causing Addison’s

Answer 11

mycobacterium avium complex

kaposi’s sarcoma

Question 12

Kaposi’s sarcoma

Answer 12

mostly seen in people with advanced HIV infection, causes patches of abnormal tissue to grow under skin and in lining of organs etc

Question 13

onset of Addison’s

Answer 13

delayed presentation and insidious onset

signs and symptoms are only present once 90% of the gland has been destroyed

often non-specific signs and symptoms

Question 14

clinical features of Addison’s: vague symptoms

Answer 14

weakness

fatigue

anorexia

nausea and vomiting

weight loss, diarrhoea

pigmentation

postural hypotension

Question 15

what mood changes are seen in Addison’s

Answer 15

depression, psychosis and low self esteem

Question 16

describe the pigmentation seen in Addison’s

Answer 16

especially of new scars and palmar creases

increased ACTH stimulates the production of POMC

Question 17

why is there postural hypotension in Addison’s

Answer 17

due to decreased mineralocorticoids, is common even when supine BP is normal

there is K retention and Na loss - hypovolaemia

mineralocorticoid deficiency also results in hyperkalaemia, hyponatraemia, volume depletion and hypotension

Question 18

what are the results of decreased glucocorticoids in Addison’s

Answer 18

hypoglycaemia (cortisol is a stress hormone that causes blood glucose levels to rise)

Question 19

what are the symptoms of an addisonian crisis

Answer 19

occur due to stress (trauma, infection, surgery)

causes vomiting, abdominal pain, hypotension, shock and death

Question 20

management of an Addisonian crisis

Answer 20

give 100ml IV hydrocortisone

IV fluid bolus to support BP

monitor BG and check for hypoglycaemia

Question 21

diagnosis of adrenal insufficiency

Answer 21

suspicious biochemistry - decreased Na and increased K, hypoglycaemia

short synACTHen test

ACTH levels

renin/aldosterone levels

adrenal autoantibodies

Question 22

short synACTHen test to diagnosis adrenal insufficiency

Answer 22

small amount of ACTH injected (IV/IM) and cortisol response measured

normal: baseline >250nmol/L and post ACTH >550nmol/L

Question 23

ACTH levels in adrenal insufficiency

Answer 23

should be increased

this causes skin pigmentation due to production of POMC

Question 24

renin and aldosterone levels in adrenal insufficiency

Answer 24

increased renin and decreased aldosterone

Question 25

management of adrenal insufficiency

Answer 25

treatment must not be delayed to confirm diagnosis

replace steroids: 15-25mg hydrocortisone a day. give steroids in 2-3 doses to try to mimic diurinal rhythm

replace aldosterone: fludrocortisone - corrects postural hypotension and Na/K balance. Monitor K and BP carefully

education

Question 26

how should steroids be given in the initial management of adrenal insufficiency if the patient is unwell

Answer 26

intravenously first

Question 27

what education must be given for the management of adrenal insufficiency

Answer 27

There must be warning about abruptly stopping steroid use, and emphasis that prescribing doctors/surgeons/nurses etc. must know about steroid prescription.

A steroid treatment card must be carried at all times

Question 28

what causes secondary Adrenocortical Insufficiency

Answer 28

lack of CRH/ACTH

• pituitary/hypothalamic disease
• tumours
• surgery/radiotherapy
• exogenous steroid use

Question 29

what is the most common cause of secondaryAdrenocortical Insufficiency

Answer 29

exogenous steroid use

Question 30

how do the clinical features of secondary Adrenocortical Insufficiency differ to those of Addison’s

Answer 30

skin is pale - no increased ACTH

aldosterone production is usually intact (as this is mainly under RAAS control not the HPA)

Question 31

treatment of secondary Adrenocortical Insufficiency

Answer 31

hydrocortisone replacement

fludrocortisone not required

Question 32

steroid dosing in illness

Answer 32

• double hydrocortisone dose in febrile illness, injury or stress
• fludrocortisone dose does not need to be increased