adrenal diseases

Question 1

what is adrenal insufficiency

Answer 1

inadequate adrenocortical function

Question 2

what are the types of adrenal insufficiency

Answer 2

primary (acute and chronic)

secondary

Question 3

what causes secondary adrenal insufficiency, what is most common

Answer 3

anything outwith the adrenal gland:

lack of ACTH stimulation
iatrogenic = most common
pituitary/hypothalamic disorder

Question 4

give an example of an iatrogenic cause of adrenal insufficiency

Answer 4

excess exogenous steroids due to medication

Question 5

what are the cause of chronic primary adrenal insufficiency, what is most common

Answer 5

addison's disease = most common
congenital adrenal hyperplasia 
adrenal TB (rare in uk)
adrenal malignancy (rare)

Question 6

what are the investigations of adrenal insufficiency

Answer 6

CT adrenals

Question 7

what are the causes of acute primary adrenal insufficiency

Answer 7

rapid withdrawal of steroid treatment
massive adrenal haemorrhage
adrenal crisis

Question 8

what is Addison’s disease

Answer 8

type of adrenal hypofunction

Question 9

what causes Addison’s disease

Answer 9

autoimmune destruction of the adrenal cortex

Question 10

what are the symptoms of Addison’s disease, describe their onset

Answer 10

anorexia
fatigue/weakness 
dizziness
abdominal pain
vomiting and diarrhoea
skin pigmentation = buzzword

Question 11

why does skin pigmentation happen in Addison’s disease

Answer 11

high levels of ACTH cross-react with melanocyte receptors

Question 12

what does decreased mineralocorticoids cause in Addison’s disease

Answer 12

hyperkalaemia

hypotension/postural hypotension

Question 13

what does decreased glucocorticoids cause in Addison’s disease

Answer 13

hyopglycaemia

Question 14

what investigations should you do in what does in Addison’s disease

Answer 14

Na and K levels
ACTH levels
short Synacthen test
adrenal antibodies

Question 15

what level of ACTH would you expect in Addison’s disease

Answer 15

HIGH

Question 16

what levels of Na and K would you expect in Addison’s disease

Answer 16

low Na

high K

Question 17

what should make you suspect Addison’s disease until proven otherwise

Answer 17

low Na

high K

Question 18

when would you treat Addison’s disease

Answer 18

as soon as its suspected, don’t wait for diagnosis to be confirmed

Question 19

what is the treatment of Addison’s disease

Answer 19

hydrocortisone

fludrocortisone

Question 20

what are the sick day rules surrounding hydrocortisone treatment in Addison’s disease

Answer 20

Temp >38 = hydrocortisone dose doubled

Temp >39 = hydrocortisone dose tripled

Question 21

when would hydrocortisone be given IV in Addison’s disease

Answer 21

if patient is systemically unwell

Question 22

what must be done when giving fludrocortisone

Answer 22

monitor BP

monitor K

Question 23

what is the treatment of adrenal crisis

Answer 23

ASAP:

IV saline
IV hydrocortisone

Question 24

what is adrenal crisis

Answer 24

severe adrenal insufficiency

Question 25

what is congenital adrenal hyperplasia

Answer 25

rare autosomal recessive condition associated with enzyme defects in the steroid pathway

Question 26

what are the two types of congenital adrenal hyperplasia

Answer 26

classical | non-classical

Question 27

what are the investigations of congenital adrenal hyperplasia

Answer 27

genetic mutation analysis | basal or stimulated 17-OH progesterone

Question 28

what is the treatment of congenital adrenal hyperplasia in adults

Answer 28

control androgen excess restore fertility avoid steroid over-replacement

Question 29

what is the treatment of congenital adrenal hyperplasia in children

Answer 29

glucocorticoid replacement = suppresses ACTH mineralocorticoid replacement in some surgical correction of genitals

Question 30

what are the symptoms of classical congenital adrenal hyperplasia

Answer 30

``` female = genital ambiguity male = scrotal discolouration ``` poor weight gain adrenal insufficiency (around 2-3 weeks) biochemical pattern similar to Addison's

Question 31

what are the symptoms of non-classical congenital adrenal hyperplasia

Answer 31

``` Hirsute Acne Oligomenorrhoea Precocious puberty Infertility or sub-fertility ```

Question 32

what symptoms would you expect in a patient lacking in CRH or ACTH

Answer 32

similar to addison's but will have pale skin and intact aldosterone production

Question 33

what is the treatment of a lack or CRH /ACTH

Answer 33

hydrocortisone replacement

Question 34

what is cushing's syndrome e

Answer 34

excess of cortisol

Question 35

how is mainly affected by cushings

Answer 35

women aged 20-40

Question 36

what causes cushings syndrome

Answer 36

``` pituitary adenomas adrenal adenomas ectopic ACTH production e.g. lung cancer alcohol steroid medication ```

Question 37

what is excess cortisol caused by a pituitary adenoma known as

Answer 37

cushings disease

Question 38

what is the difference between cushings syndrome and cushings disease

Answer 38

``` syndrome = caused by anything resulting in high cortisol levels disease = caused by high cortisol levels due to a pituitary adenoma ```

Question 39

what are the symptoms of cushings syndrome

Answer 39

``` proximal myopathy/wasting osteoporosis thin skin = easy bruising & striae diabetes central obesity high BP virilism hirsuitism acne olgio/amenorrhoea ```

Question 40

what is the investigations of cushings syndrome

Answer 40

``` high dose oral exogenous steroid (dexamethasone) low dose dexamethasone overnight dexamethasone suppression test 24hr urine collection diurnal cortisol variation ```

Question 41

what is the gold standard investigation for cushings, what results would you expect

Answer 41

low dose DST cortisol <50 = not cushings cortisol >130 = defo cushings

Question 42

what is the treatment for cushings caused by a pituitary issue

Answer 42

Hypophysectomy and external radiotherapy if recurs Bilateral adrenalectomy

Question 43

what is the treatment for cushings caused by an adrenal issue

Answer 43

adrenalectomy

Question 44

what is the treatment for cushings caused by an ectopic issue

Answer 44

remove source | bilateral adrenalectomy

Question 45

what drugs can be used to treat cushings

Answer 45

Metyrapone Ketoconazole Pasireotide LAR = somatostatin analogue

Question 46

when would you prescribe drugs in cushings

Answer 46

if all else fails or while waiting for radiotherapy to work

Question 47

what is primary aldosteronism

Answer 47

autonomous production of aldosterone independent of its regulators

Question 48

what are the regulators of aldosterone

Answer 48

angiotensin II | K+

Question 49

what are the causes of primary aldosteronism, what is most common

Answer 49

adrenal adenoma bilateral adrenal hyperplasia = most common unilateral hyperplasia genetic mutations

Question 50

what is primary aldosteronism caused by adrenal adenoma called

Answer 50

Conn's syndrome

Question 51

what are the symptoms of primary aldosteronism

Answer 51

significant hypotension hypokalaemia alkalosis

Question 52

what are the investigations of primary aldosteronism

Answer 52

ARR-aldosterone to renin ratio, if raised do saline suppression test to confirm PA adrenal CT adrenal vein sampling

Question 53

what is the treatment of primary aldosteronism caused by an adrenal adenoma (conn's)

Answer 53

surgery = unilateral laparoscopic adrenalectomy

Question 54

what is the treatment of primary aldosteronism caused by bilateral hyperplasia

Answer 54

MR antagonist = spironolactone or eplereone

Question 55

describe an adrenocortical adenoma

Answer 55

well circumscribed, encapsulated lesion composed of cells resembling adrenocortical cells yellow surface

Question 56

describe an adrenocortical carcinoma

Answer 56

large frequent, abnormal mitoses lack of clear cells capsular or vascular invasion

Question 57

what is phaeochromocytoma, where does it originate

Answer 57

neuroendocrine tumour of the medulla of the adrenal gland, originating in the chromaffin cells

Question 58

what does phaeochromocytoma secrete

Answer 58

catecholamines

Question 59

what is a phaeochromocytoma located outside of the adrenal called

Answer 59

paraganglioma

Question 60

why is a phaeochromocytomadark brown in colour

Answer 60

potassium dichromate causes oxidation of catecholamines within tumour

Question 61

what is the classical triad of symptoms of phaeochromocytoma

Answer 61

hypertension headache sweating

Question 62

what are the symptoms of phaeochromocytoma

Answer 62

``` hypertension headache sweating palpitations SOB consitpation anxiety/fear weight loss ```

Question 63

what is phaeochromocytoma associated with

Answer 63

MEN2 Von-hippel-lindau syndrome Neurofibromatosis

Question 64

what are the two purposes of the investigations of  phaeochromocytoma

Answer 64

confirm catecholamine excess | identify source of excess catecholamines

Question 65

what investigations confirm excess catecholamines in phaeochromocytoma

Answer 65

2 x 24hr catecholamines or metanephrins in urine | catecholamines in plasma

Question 66

what are the issues in trying to identify catecholamine excess

Answer 66

they are naturally raised in HF | catecholamine secretion is episodic

Question 67

what investigations identify source of raised catecholamines in phaeochromocytoma

Answer 67

MIR MIBG PET scan

Question 68

what is the treatment of phaeochromocytoma

Answer 68

alpha blocker THEN add beta blocker surgery chemo if malignant

Question 69

what alpha blocker is used to treat phaeochromocytoma

Answer 69

phenoxybenzamine

Question 70

what beta blocked can be used to treat phaeochromocytoma

Answer 70

propanolo, atenolol, metoprolol

Question 71

what is a neuroblastoma

Answer 71

rare tumours composed of primitive appearing cells which can show maturation and differentiation towards ganglion cells.

Question 72

where do neuroblastoma arise

Answer 72

40% adrenal medulla | the rest are mainly along the sympathetic chains