Adrenal Disease Flashcards

1
Q

What differentiates Cushing Syndrome from Cushing Disease?

A

CS - hypercortisolaemia
CD - pituitary tumour

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2
Q

What are the zones of the adrenal cortex and what are their roles?

A

ZG - aldosterone (mineralocorticoids)
ZF - cortisol (glucocorticoids)
ZR - DHEA (androgens)

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3
Q

What does cortisol bind to in the bloodstream?

A

CBG (cortisol binding globulin)

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4
Q

What is the action of cortisol?

A

Stress
- gluconeogenesis
- lipolysis
- proteolysis

BP
- vasoconstriction (sensitivity to catecholamines)

Reduced immune response
- fewer PGs & ILKs
- inhibition of T-lymphocytes

Brain
- mood & memory

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5
Q

How does Cushing Syndrome present?

A
  • severe muscle, bone & skin breakdown (easy bruising, thin skin)
  • T2DM (hyperglycaemia)
  • central obesity (high insulin, activation of lipoprotein lipase)
  • HTN (catecholamine sensitivity = vasoconstriction ; ZG cross-activation = RAAS)
  • reduced sexual function (GRH suppression)
  • frequent infections
  • impaired brain function
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6
Q

Causes of Cushing syndrome

A
  • exogenous medications (steroids, oft for asthma/RA) = atrophy
  • pituitary adenoma (CD = hyperplasia)
  • SCC (LC - ectopic ACTH)
  • adrenal adenoma/carcinoma (suppression of CRH, ACTH; contralateral atrophy)
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7
Q

Symptoms of Cushing disease

A
  • muscle wasting
  • thin extremities
  • easy bruising
  • striae
  • fractures (osteoporosis)
  • moon face
  • buffalo hump
  • truncal obesity
  • DM
  • HTN
  • CVD risk
  • vulnerable to infections
  • poor wound healing
  • amenorrhoea
  • psychiatric (mental disorders)
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8
Q

Diagnosis of Cushing syndrome

A
  • 24h urinary cortisol
  • 9am serum cortisol
  • LOW-DOSE overnight dexamethasone suppression test (+ increases; endogenous)
  • plasma ACTH (low: 1ry INDEPT adrenal adenoma/carcinoma) (high: 2ry DEPENDENT Cushing disease & ectopic)
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9
Q

Treatment of Cushing syndrome

A
  • treat ULC
  • taper exogenous steroids (withdrawal = adrenal crisis)
  • steroid inhibitors (metyrapone, ketoconazole)
  • TSS (excision of pituitary adenoma) + RTx
  • total adrenalectomy (supplemented with hydrocortisone/fludrocortisone)
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10
Q

Differentiate between Cushing disease & ectopic ACTH production

A

HIGH-DOSE DST injection - ectopic sites non-responsive
- CRH stimulation test = rise in CD
- Inf. petrosal sinus sampling (IPSS) - catheter in FV/JV, inject CRH, sample ACTH peripheral vein & petrosal sinus

Ix- pituitary MRI, CT TAP

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11
Q

Red flags of Cushing syndrome indicating adrenal carcinoma/adenoma

A
  • UWL
  • F: acne, deep voice, hirsutism
  • CT: haemorrhage, necrosis, calcification
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12
Q

How would adrenal carcinoma be managed differently to an adenoma?

A

Adenoma - lap

Carcinoma - open (+LN resection +/- nearby organs)

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13
Q

Nelson syndrome

A

Pituitary hyperplasia post-bilateral adrenalectomy
- remove adrenals = no -ve feedback

Presents with: headache, visual impairment, hyperpigmentation (melanocytes)

Treat: TSS

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14
Q

What is the HPA axis?

A

CRH (hypothalamus) -> ACTH (ant. Pituitary) -> Cortisol (ZF adrenal cortex)
<— negative feedback

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15
Q

What are potential complication of prolonged systemic exogenous glucocorticoids?

A
  • Cushing syndrome
  • Posterior subcapsular cataracts (decreased night vision & glares)
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16
Q

What is the most common cause of Cushing’s syndrome?

A

Oral corticosteroid use

17
Q

Pseudo-Cushing’s syndrome

A
  • signs, symptoms & biochemical findings of Cushing’s syndrome
  • no HPA abnormality
  • diurnal variation retained

Cause: chronic alcoholism (repeat tests after 1m abstinence)

18
Q

What is Addison’s disease?

A

Primary adrenal insufficiency due to bilateral adrenal cortex destruction

19
Q

What mechanisms of compensation can mask Addison’s disease?

A

Low cortisol should = low adrenaline
- compensated by NAd

M: testicles 2ry supply of androgens

20
Q

What are the causes of Addison’s disease?

A
  • autoimmune destruction (polyglandular AIS T2)
  • infection (TB, fungal)
  • adrenal haemorrhage (WFS)
  • adrenal vein thrombotic infarction
  • mets
  • cortisol synthesis inhibitors (ketoconazole, suramin)
21
Q

What is an Addisonian crisis?

A

An adrenal crisis in Addison’s disease precipitated by physiologically stressful events:
- surgical procedures
- trauma
- infection
- dehydration

22
Q

What are the characteristic symptoms of Addison’s disease?

A
  • fatigue & weakness
  • hypotension/syncope
  • hyponatraemia & hypoglycaemia
  • hyperkalaemia
  • abdo pain, anorexia, N+V, WL
  • intolerance of temp extremes
  • hyperpigmentation (tanned palmar creases) / vitiligo
  • reduced libido & hair (F)
  • confusion / depression
23
Q

Why does Addison’s disease cause hypotension?

A

Hypocortisolaemia - reduced vascular responsiveness to ATII & NAd (RAAS)

24
Q

How does Addison’s disease cause hyponatraemia?

A

Hypoaldosteronism = Na+ loss
Low cortisol = high ADH = water loss

25
Q

What causes the hyperkalaemia associated with Addison’s disease?

A

Hypoaldosteronism = mild hyperchloraemic acidosis

26
Q

Why do Addison’s patients present with hyperpigmentation?

A

ACTH stimulation of melanocyte activity

27
Q

What is vitiligo?

A

AI destruction of melanocytes.

28
Q

How does an Addisonian crisis present?

A
  • hypoglycaemia
  • shock - vasomotor/circulatory collapse (unresponsive to vasopressors due to low cortisol)
  • death?
29
Q

How is Addison’s disease diagnosed?

A

Bedside - hx, exam findings

Bloods - LOW 9am cortisol, rapid ACTH (no cortisol), Na+
HIGH serum ACTH, plasma renin, K+, hyperchloraemic acidosis

Imaging - abdo CT (enlarged glands - TB/malignancy; small = AID/advanced TB; adrenal haemorrhage/thrombosis)
AXR - adrenal calcifications = infectious cause

30
Q

What medications are used to manage Addison’s disease?

A

Life-long:
Glucocorticoid replacement
- hydrocortisone
Mineralocorticoid replacement
- fludrocortisone
Androgens (F)
- low dose DHEA

31
Q

What drugs are used acutely in an Addisonian crisis?

A
  • hydrocortisone etc.
  • adrenaline
  • glucose
  • IV. 0.9% saline
32
Q

What are ‘sick day rules’ for Addison’s disease?

A
  • dose hydrocortisone during significant trauma/surgery
33
Q

What is Waterhouse-Friderichsen syndrome?

A

Adrenal failure & crisis associated with overwhelming infection & adrenal gland haemorrhage = necrosis
- rare, severe