Hyper/hypoadrenalism Flashcards

(49 cards)

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
What can cause pseudocushings?
depression and alcohol
26
Clinical features of cushings?
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
Ix route in cushings?
Confirm hypercortisolaemia -> localise -> image to confirm
28
Initial tests in cushings
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
When are false positives seen in DST?
obese inpatients, inducers of liver enzymes
30
Localising tests in cushings?
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
what is bilateral inferior petrosal sinus sampling?
Basal:central 2:1 or 3:1 if crh given indicative of cushings disease
32
Treatment for cushings syndrome
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
Medical treatment for cushings syndrome?
If not suitable for surgery/c/i inhibitors of steroidogenesis: ketoconazole, metyrapone, mitotane (adrenolytic) ACTH lowering therapy: dopamine 2 r antagonists- cabergoline
34
when is radiotherapy used in cushings?
following transpehoidal surgery/persisting hypercortisolaemia can cause progressive ant. pituitary failure 80% in remission after 4 years
35
what is nelsons syndrome?
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
What is hyperaldosteronism?
Excess production of aldosterone independant of RAAS, incr na and water retention and reduced renin release
37
CLinical features of hyperaldosteronism?
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
Causes of hyperaldosteronism?
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
Treatment of hyperaldosteronism?
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
Secondary hyperaldosteronism?
high renin from reduce renal perfusion: renal artery stenosis, CCF, diuretics
41
Bartters Syndrome?
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
What is a phaeochromocytoma?
catecholamine producing tumour of the adrenal medula
43
What is the 10% rule in phaeochromocytoma?
10 % familial, extraadrenal, bilateral, familial
44
when shoudl you suspect phaeochromocytoma?
if severe or resistant HTN, in young or with: sweating, pallor or flush, apprehension, palpitations and throbbing headaches
45
What is the classic triad in phaeochromocytoma?
episodic headache, sweating and tachycardic
46
COmplications of phaeochromocytoma?
Stroke, HR, Cardiomyopathy
47
Ix in phaeochromocytoma?
24 hr urinary catecholamines plasma catecholamines if crisis abdo CT
48
what do you need to do before surgery in phaeo?
alpha blockade
49
Syndromes associated with phaeo?
MEN II VHL NF2