Adrenal Pathology Flashcards

1
Q

list the causes of primary adrenal insufficiency

A

auto-immune
infection eg TB
adrenal metastasis eg from breast, bone or kidney

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2
Q

what is Addisons disease

A

autoimune destruction of the adrenal glands - most common cause of primary adrenal insufficiency

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3
Q

in primary adrenal insufficiency describe levels of cortisol, aldosterone and ACTH

A

cortisol and aldosterone low

ACTH high due to negative feedback still taking place

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4
Q

describe the clinical presentation of addisons disease

A
myalgia and arthraglia 
abdo pain, diarrhoea and vomiting
dizziness
weight loss and fatigue 
hyperpigmentation of the skin
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5
Q

what is the pathophysiology behind hyperpigmentation of addisons disease

A

increased levels of ACTH cause deposits in the skin

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6
Q

describe sodium, potassium and glucose levels in a patient with Addisons disease

A

sodium low
potassium high
glucose low

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7
Q

how is primary adrenal insufficiency diagnosed

A

short synthacten test - administer synthetic ACTH which should increase levels of cortisol gradually eg after 30 mins levels greater than 550
Addisons disease will show no significant increase in cortisol levels

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8
Q

how is Addisons disease managed

A

steroid replacement therapy

hydrocortisone 15-25mg and fludrocortisone

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9
Q

is dosing of steroid replacement therapy divided up and why

A

yes divided up and cortisol typically peaks in morning and low at night, dosages must follow this regime

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10
Q

what occurs if Addisons disease goes untreated

A

Addisonians crisis - presents with shock, hypogylcaemia and confusion usually following surgery or not taking steroids

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11
Q

describe the management of Addisonians crisis

A

fluid resuscitation, IV hydrocortisone and IV glucose if necessary

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12
Q

what is bilateral adrenal hyperplasia

A

congenital form of adrenal insufficiency due to mutation

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13
Q

what mutation causes bilateral adrenal hyperplasia

A

21 alpha hydroxylase enzyme - causes reduced cortisol and aldosterone whilst increases androgens

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14
Q

bilateral adrenal hyperplasia presents differently in adults and children, how does it present in children

A
dehydration 
salt crisis 
failure to thrive 
girls - ambiguous genitalia 
boys - early virilisation
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15
Q

how does bilateral adrenal hyperplasia present in adult men and women

A

men - acne, short stature, precocious puberty

women - hirsutism, oligomenorrhoea, reduced stature, acne

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16
Q

describe the management of bilateral adrenal hyperplasia

A

same as addisons with steroid replacement therapy

girls - requires karyotyping and gender assignment

17
Q

what is secondary adrenal insufficiency

A

deficiency of ACTH which then leads to reduced cortisol levels

18
Q

list some causes of secondary adrenal insufficiency

A

pituitary disease eg tumour or trauma

long term steroid use - dampens the steroid axis over time

19
Q

in secondary adrenal insufficiency both cortisol and aldosterone are reduced true/false

A

false only cortisol is reduced

this means presentation is similar to addisons except no dehydration or hyperpigmentation

20
Q

how is secondary adrenal insufficiency treated

A

oral hydrocortisone

21
Q

what is primary hyperaldosteronism

A

excessive production of aldosterone independent of RAAS

22
Q

what is the main causes of primary hyperaldosteronism

A

Conns syndrome - aldosterone secreting adrenal adenoma

23
Q

describe the presentation of Conn’s Syndrome

A
polydipsia
polyuria 
headaches 
weakness/muscle cramps/spasms 
hypertension - especially young patients and resistant
24
Q

describe sodium and potassium levels in Conn’s syndrome

A

Na high
K low
CT of abdomen will identify adenoma

25
Q

outline the management of Conns syndrome

A

conservative - aldosterone antagonist eg spironolactone

surgical - removal of adenoma

26
Q

what is Cushings syndrome

A

any condition causing excess cortisol production

27
Q

list exogenous and endogenous causes of Cushings syndrome

A

exogenous - prolonged steroid use

endogenous - ACTH independent and ACTH dependent

28
Q

list ACTH independent causes of Cushings syndrome

A

benign adrenal adenoma

adrenal carcinoma

29
Q

list ACTH dependent causes of Cushings syndrome

A

ectopic ACTH production eg lung tumour

pituitary adenoma secreting ACTH

30
Q

describe the presentation of Cushings syndrome

A
central abdominal obesity with striae 
proximal muscle wasting 
moon face 
virilisation and hirsuitism 
oligomenorrhoea 
hypertension, osteoporosis, easy bruising, thin skin
31
Q

what is the screening test for Cushings syndrome

A

overnight dexamethasone suppression test

32
Q

what is the diagnostic test for Cushings syndrome

A

48 hour dexamethasone suppression test - if cortisol is less than 50 patient does not have Cushings

33
Q

how is the cause of Cushings syndrome investigated

A

CT/MRI of abdomen or plasma ACTH

34
Q

outline the management of Cushings

A

surgical excision of adenoma if present

adrenal enzyme inhibitors - metyrapone or ketoconazole

35
Q

phaeochromocytoma is a tumour of __ and secretes __

A

tumour of adrenal medulla

secretes excess catecholamines

36
Q

list the triad of symptoms that present in phaeochromocytoma

A

hypertension
headaches
sweating
also sob, facial flushing and chest tightness

37
Q

how is a suspected phaeochromocytoma investigated

A

24 hour urine collection of catecholamines

MIBG scan - uses a chromaffin seeking isotope to look for increases uptake

38
Q

how is a phaeochromocytoma managed

A

surgical excision

before surgery requires blood pressure protection through alpha blocker - phenoxybenzamine and beta blocker - atenolol