Adrenal disease Flashcards

1
Q

Recall the 3 zones of the adrenal gland and what is produced in each one

A

Zone glomerulosa = aldosterone
Zona fasiculata = cortisol
Zona reticularis = androgens

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2
Q

What are the peripheral and adrenal catecholamines?

A

Peripheral: Noradrenaline
Adrenal: Adrenaline

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3
Q

Give 2 likely causes of wasted adrenal glands

A

Addison’s disease
Long term steroid tx

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4
Q

Give 2 likely causes of hyper plastic adrenal glands

A

Cushing’s disease
Ectopic ACTH

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5
Q

How do the measurements of aldosterone and cortisol differ?

A

A: PICOmoles
C: NANOmoles (you make 1000x more C than A)

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6
Q

What diagnosis would you suspect in a patient with fatigue and high TSH and low T4?

A

Primary hypothyroidism

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7
Q

What is Schmidt’s syndrome?

A

Primary hypothyroisidm + Addisson’s disease
Antibodies against thyroid + adrenals

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8
Q

What’s the test for Addison’s, and how should it be performed?

A

SynACTHen test
1. Measure cortisol + ACTH
2. 250ug ACTH IM
3. Check cortisol at 30 + 60m
If fail to stimulate = failure of endocrine function

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9
Q

What is the expected electrolyte abnormality in untreated primary adrenal failure?

A

Hyponatraemia
Hyperkalaemia
(Mineralocorticoid deficiency)

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10
Q

What abnormalities may accompany hyponatraemia and hyperkalaemia in Addisons disease?

A

Hypoglycaemia (glucocorticoid deficiency)
Low BP (mineralocorticoid deficiency)

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11
Q

What is the treatment for a patient in an addisonian crisis?

A

IV Saline 0.9% 1L/hr
IV Hydrocortisone

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12
Q

What is the long term treatment for Addisons disease?

A

Hydrocortisone (Glucocorticoid)
Fludrocortisone (Mineralocorticoid)

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13
Q

What are the 3 differentials to consider when a patient with severe HTN also has an adrenal mass?

A
  1. Phaeochromocytoma (medullary tumour secreting adrenalinę)
  2. Conn’s syndrome (zona glomerulosa tumour: secreting aldosterone)
  3. Cushing’s (zona fasciculata tumour secreting cortisol)
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14
Q

What test is used to identify pheochromocytoma?

A

Urinary catecholamines: HIGH

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15
Q

How should phaeochromocytoma be treated?

A
  1. IMMEDIATE: Alpha blockers (phenoxybenzamine) causes reflex tachy
  2. Beta blockers (to deal with reflex tachycardia)
  3. Surgery
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16
Q

What are the expected aldosterone and renin levels in untreated Conn’s syndrome?

A

HIGH Aldosterone
LOW Renin

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17
Q

What electrolyte abnormalities would you see in Conn’s syndrome?

A

HIGH Na
LOW K+

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18
Q

Describe why Renin is suppressed in Conn’s

A

Adrenal gland autonomously secretes aldosterone
Causes increased Na + water retention + K+ excretion
HTN
Suppresses renin production at JGA

19
Q

How does treatment differ if Conn’s syndrome is caused by unilateral adrenal adenoma or bilateral adrenocortical hyperplasia?

A

Unilateral: Surgery
Bilateral: Spironolactone (aldosterone antagonist)

20
Q

What are the expected aldosterone and renin levels in untreated Cushing’s syndrome?

A

LOW Aldosterone
LOW Renin

21
Q

What biochemical abnormalities would you see in Cushing’s syndrome?

A

HIGH Na
LOW K
HIGH Glucose

22
Q

What 3 tests can be used to investigate for Cushing’s?

A
  1. 9am cortisol (HIGH in ALL)
  2. Sneaky midnight cortisol (HIGH in CUSHING’s)
  3. Dexamethosone suppression test (NO ACTH suppression)
23
Q

What does the term ‘cushing’s disease’ refer to?

A

Cushing’s syndrome caused by a pituitary tumour secreting ACTH
DISEASE = pituitary DEPENDENT

24
Q

What is the diagnosis in a patient with symptoms of Cushing’s but a normal result in the low-dose dexamethosone suppression test?

A

Normal obese person

25
Q

What is the diagnosis in a patient with symptoms of Cushing’s but only a very slightly suppressed cortisol in the low-dose dexamethosone suppression test?

A

Cushing’s syndrome of indeterminate cause

26
Q

How should Cushing’s syndrome of indeterminate cause be investigated to determine whether pituitary dependent or ectopic?

A

Inferior petrosal sinus sampling with CRH stimulation
Catheter fed into jugular veins.
If ACTH high centrally = Cushing’s disease.

27
Q

Give 3 conditions associated with MEN2a

A

Parathyroid tumour
Medullary thyroid cancer
Phaeochromocytoma

28
Q

Give 3 conditions associated with von Hippel Lindau syndrome

A

Phaeochromocytoma
Renal cell carcinoma
Renal cysts
Haemangioblastoma

29
Q

Give 3 features of Neurofibromatosis type 1

A

Phaeochromocytoma
Peripheral/ spinal neurofibromas
Cafe au lait spots

30
Q

Describe the low dose dexamethasone suppression test

A
  1. At 11pm give 1mg dexamethasone
  2. Measure cortisol before 9am
31
Q

Why is a high dose dexamethasone suppression test rarely used to confirm the cause of Cushing’s syndrome?

A

85% are pituitary dependent without the test and test has 20% false positives (same with MRI)

32
Q

How does Cushing’s cause hypertension?

A

At high conc. Cortisol activates the MR
Increases Na + water reabsorption
11b hydroxysteroid dehydrogenase usually degrades cortisol to stop this, but is overwhelmed at this conc.

33
Q

What is the treatment for Cushing’s disease? What is the treatment for Cushing’s syndrome caused by adrenal adenoma?

A

CD: Transsphenoidal Hypophysectomy
CS: Bilateral adrenalectomy

34
Q

4 causes of Cushing’s syndrome

A

Oral steroids
Pituitary dependent Cushing’s disease
Ectopic ACTH in SCLC
Adrenal adenoma secreting cortisol

35
Q

State the drugs that are used to treat Cushing’s syndrome before and surgery.

A

Before: Metyrapone (inhibits cortisol production) + Mifepristone
After: Hydrocortisone + hormone replacement

36
Q

State 3 causes of adrenocortical failure.

A

TB Addison’s Disesae (commonest worldwide)
AI Addison’s Disease (commonest in UK)
Congenital Adrenal Hyperplasia

37
Q

What is the most common cause of congenital adrenal hyperplasia (CAH)?

A

21-hydroxylase deficiency

38
Q

Why are foetuses with CAH normally fine in utero?

A

In utero, the foetus’ will have maternal cortisol + aldosterone so don’t need to rely on their own endogenous production.

39
Q

What will happen to a baby with complete 21-hydroxylase deficiency after they are born?

A

They will have a salt losing Addisonian crisis due to the lack of aldosterone.
Survive <24 hours if untreated

40
Q

What are the 2 main features of 21-hydroxylase deficiency?

A

Hypotension
Virilisation (female babies will be born with ambiguous genitalia)

41
Q

List 4 features of partial 21-hydroxylase deficiency

A

Male pattern balding
Hirsutism
Irregular menstruation
Clitoral enlargement

42
Q

Describe the presentation of partial 21-hydroxylase deficiency.

A

Present later because they don’t have a salt losing Addisonian crisis.
Present once they start to see the effects of the excess adrenal sex steroids e.g. hirsuitism + precocious puberty
Also slightly hypotensive

43
Q

What would the adrenal glands look like in autoimmune Addisons, TB and metastasis?

A

AI: Atrophied adrenals, very small
TB: Granulomatous infiltration
Metastasis: Large tumour

44
Q

Why do babies with congenital adrenal hyperplasia have enlarged adrenals?

A

ACTH is raised to drive the adrenal cortex to produce its steroids, but only sex steroids can be produced
Excess ACTH makes adrenals grow