Hyper/hypoadrenalism Flashcards

1
Q

What is the difference btw primary and secondary adrenal deficiency in terms of steroids lost?

A

-primary is deficiency of both

secondary is due to suppression usually and only results in glucocorticoid deficiency

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2
Q

Causes of primary adrenal failure?

A
Automimmune adrenalitis
TB
HIV
Haemorrhage (waterhouse freidrichson, SLE)
Tumour replacement (mets from lung, breast, renal)
Adrenoleukodystrophy
CAH
Drugs (ketoconazole, rifampicin
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3
Q

Causes of secondary adrenal failure?

A
Iatrogenic
Pituitary tumour, mets, cranipharyngoma
TB
surgery/radio
trauma
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4
Q

At what point is insufficiency noticable clinically?

A

90%of the gland is destroyed

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5
Q

Symptoms of hypoadrenalism?

A
weakness/fatigue
annorexia and wt loss
N+V, non specific abdo pain
salt craving
postural dizziness
pyrexia
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6
Q

Signs of hypoadrenalism?

A
Weight loss 
HYPERPIGMENTATION?
hyponatreimia
hyperkalaemia
hypercalcaemia
hypoglycaemia 
anameia
females ay have axilliary hair loss and reduced libido
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7
Q

Ix in hypoadrenalism?

A

FBC, U+Es (hypoNA hyperK Incr urea)
Cortisol and ACTH levels at 9am
Cortisol <150nm is suggestive
Increased level of ACTH for level of cortisol is also suggestive

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8
Q

Diagnostic tests in hypoadrenalism?

A

SHort synacthen test- give 250ug acth and sample cortisol levels at baseline, 30 mins, 60 min

Failure to respond suggests adrenal failure

Long synacthen test- give 1000 and see if respnonse over a day. in secondary will be some recovery and response, diseased adrenals will still not respond

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9
Q

Further Ix in hypoadrenalism?

A

Adrenal autoantibodies- directed against 21 hydroxylase in 80%
exclude associated polyendocrine syndrome
Renin to assess mineralocorticoid deficiency
TFTs

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10
Q

Management of hypoadrenalism?

A

Glucocorticoid replacement hydro TDS (eg 10, 5, 5)
Mineralocorticoid- fludro 100mcg
DHEA may improve wellbeing but isnt on prescription

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11
Q

Monitoring needed in hypoadrenalism?

A

signs of glucocorticoid excess
BP
hypertension and oedema
Postural hypotension and salt craving

U+Es, renin, acth levels following replacement if getting pigmentation

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12
Q

Sick day rules in hypoadrenalism?

A

double the dose for any illness, trauma, stress, surgery

have a 100 hydro at home to IM if vomming, get to hosp

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13
Q

Clinical features of addisonian crisis?

A
shock
hypotension
pain
unexplained fever
major stress
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14
Q

causes of addisonian crisis?

A

often known addisons and too ill to take steroids
long term steroid user not taking tablets
bilateral adrenal haemorrhage (less common)

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15
Q

Management of addisonian crisis

A

Bloods for cortisol and acth straight to lab

U+Es- k+ can be high so ecg and calcium gluconate if needed

hydro 100mg IV

Iv fluid bolus to support BP

Monitor BG (hypo a risk)

Culture and abx if risk of infection

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16
Q

ongoing treatment in addisonian crisis?

A

glucose may be needed, give fluids as required, change hydro to oral after 3 days if improvement. GET HELP

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17
Q

Types of hyperadrenalism?

A

Cushings syndrome
Hyperaldosteronism
Phaeochromocytoma

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18
Q

Layers of the adrenals and fx?

A

Glomerulosa- aldosterone
fasciculata- cortisol
reticularis- DHEA
Medulla- adrenaline

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19
Q

When i cortisol usually highest?

A

Morning

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20
Q

What is cushings syndrome?

A

A clinical state produced by chronic glucocorticoid excess + loss of normal feedback

21
Q

Two main categories of Cushings syndrome?

A

ACTH dependent: Pituitary oversecretion, ectopic ACTH secretion, Ectopic CRH secretion (some medullary thyroid and prostate ca)

ACTH independent: Ioatrogenic, adrenocortical carcinoma/ademona
adrenal micronodular dysplasia

22
Q

What is cushings disease?

A

Bilateral adrenal hyperplasia from ACTH secreting pit adenoma (usually micro). No response to low dose DMST but will drop for High dose

23
Q

Where can ectopic secretion of ACTH come from?

A

Small cell lung cancer, carcinoid tumours

24
Q

What are the features of ectopic ACTH secretion?

A

Pigmentation due to increased ACTH, hypokalaemic metabilic acidosis, hyperglycaemia, high dose DMT fails, classical cushing features not present

25
Q

What can cause pseudocushings?

A

depression and alcohol

26
Q

Clinical features of cushings?

A

Appearance: facial rounding, acne, hirsuitism, supraclavicular/intrascapular fat pad, striae, centripetal obesity, easy bruising, thin skin

Catabolic: proximal myopathy, striae, bruising, osteoporosis

Glucocorticoid: obesity, incr glu or DM, increased infection and poor healing

Mineralocorticoid: HTN, hypocalcaemia

Also: gonadal dysfunction, mood change, recurrent achilles tendon rupture,

27
Q

Ix route in cushings?

A

Confirm hypercortisolaemia -> localise -> image to confirm

28
Q

Initial tests in cushings

A

24hr urinary free cortisol
1mg overnight DMST

Midnight and morning salivary cortisol
Low dose DMST (longer and lower)

Perform 2- if both abnormal, confirmed, 1 do more tests, both normal then excluded

29
Q

When are false positives seen in DST?

A

obese inpatients, inducers of liver enzymes

30
Q

Localising tests in cushings?

A

ACTH- if this is low, likely adrenal cause so CT them
if detectable ACTH keep looking

High dose DST: response makes cushings disease more likely

CRH test, cortisol will raise, not really with ectopic

31
Q

what is bilateral inferior petrosal sinus sampling?

A

Basal:central 2:1 or 3:1 if crh given indicative of cushings disease

32
Q

Treatment for cushings syndrome

A

Transphenoidal surgery: cushings disesase, long lasting remission in 50-60%

adrenal surgery: can get rid of one if isolated adenoma

bilateral adrenalectomy: control it in refractory cushings but can -> nelsons

ectopic ACTH- cut it out

33
Q

Medical treatment for cushings syndrome?

A

If not suitable for surgery/c/i

inhibitors of steroidogenesis: ketoconazole, metyrapone, mitotane (adrenolytic)

ACTH lowering therapy: dopamine 2 r antagonists- cabergoline

34
Q

when is radiotherapy used in cushings?

A

following transpehoidal surgery/persisting hypercortisolaemia

can cause progressive ant. pituitary failure

80% in remission after 4 years

35
Q

what is nelsons syndrome?

A

occurs follwing adrenalectomy, high acth levels and hyperpigmentation from enlarging pit. tumour. loss of negative feedback means at least 50% incidence in 10 years so monitor for 6 starting 6mo post op

36
Q

What is hyperaldosteronism?

A

Excess production of aldosterone independant of RAAS, incr na and water retention and reduced renin release

37
Q

CLinical features of hyperaldosteronism?

A

hypertension, hypokalaemia in someone not on diuretics
na mildly raised or normal
can be asymptomatic
or signs of hypoK+ eg cramps parasthesiae, poluria polydipsia

38
Q

Causes of hyperaldosteronism?

A

2/3 are an aldosterone secreting adenoma (Conns)
1/3 are bilateral adrenocortical hyperplasia

Rarely: adrenal carcinoma, GRA (acth regulatory element fixes to aldosterone synthase gene so aldosteron production goes up and under control of acth, suspect if FH)

39
Q

Treatment of hyperaldosteronism?

A

COnns: adrenalectomy- spironolactone 4 weeks before controls BP and K+

Hyperplasia: spironolactone or eplerenone (doesnt cause gynaecomastia)

GRA: dex 1mg for 4 weeks

Carcinoma, poor prognosis, mitotane

40
Q

Secondary hyperaldosteronism?

A

high renin from reduce renal perfusion: renal artery stenosis, CCF, diuretics

41
Q

Bartters Syndrome?

A

Major cause of congenital salt wasting, NA and CL both lost through a defective channel
FTT
polyuria
polydipsia
BP normal
low na and increased renin and aldosterone

Hypokalaemia and metabolic alkalosis
k+ replacement is treatment

42
Q

What is a phaeochromocytoma?

A

catecholamine producing tumour of the adrenal medula

43
Q

What is the 10% rule in phaeochromocytoma?

A

10 % familial, extraadrenal, bilateral, familial

44
Q

when shoudl you suspect phaeochromocytoma?

A

if severe or resistant HTN, in young or with: sweating, pallor or flush, apprehension, palpitations and throbbing headaches

45
Q

What is the classic triad in phaeochromocytoma?

A

episodic headache, sweating and tachycardic

46
Q

COmplications of phaeochromocytoma?

A

Stroke, HR, Cardiomyopathy

47
Q

Ix in phaeochromocytoma?

A

24 hr urinary catecholamines
plasma catecholamines if crisis
abdo CT

48
Q

what do you need to do before surgery in phaeo?

A

alpha blockade

49
Q

Syndromes associated with phaeo?

A

MEN II
VHL
NF2