Chemical Pathology 6 - Adrenal Flashcards

(35 cards)

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 catecholamines are secreted by adrenals?

A

ADrenaline (peripheral - noradrenaline)

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3
Q

Causes of wasted adrenal glands

A

Addison’s

Long term steroids

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4
Q

Causes if hyperplastic adrenal glands

A

Cushing’s disease

ectopic ACTH

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5
Q

What is the blood supply to the adrenals like

A

Through IVC to adrenal vein

L → drains to L renal vein

R → drains to IVC

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6
Q

What is Schmidt’s syndrome?

A

Primary hypothyroisidm AND

  • low t4, high TSH

Addisson’s disease (primary adrenal failure)

  • hypoglycaemia (GC def), low sodium + high potassium (MC def)

AKA Polyglandular autoimmune syndrome type II

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7
Q

What are autoantibodies directed against in Schmidt’s syndrome?

A

Thyroid and adrenals

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8
Q

What else can give a similar picture to Schmidt’s syndrome?

A

TB

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9
Q

Causes of Addison’s

A

Autoimmune (UK most common)

TB (worldwide most common)

Metastasis

Haemorrhage (Waterhouse-Friedrichson Syndrome)

Iatrogenic

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10
Q

Features of Addison’s disease

A
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11
Q

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

A

SynACTHen test
Following adminstration of synthetic ACTH, check cortisol at 30 and 60 mins

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12
Q

What results would you expect from the short synACTHen test in Addison’s?

A

ACTH = >100ng/dL; Cortisol = <10nM (30mins); Cortisol = <10nM (60mins)

Not making any cortisol in response to ACTH challenge = failure

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13
Q

How would you manage Addison’s disease?

A

IV 0.9% saline (1L/hour)

IV hydrocortisone (sick day rules)

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14
Q

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

A

Hyponatraemia, hyperkalaemia

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15
Q

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

A
  1. Phaeochromocytoma (medullary tumour)
  2. Conn’s syndrome (aldosterone-secreting tumour - zona glomerulosa)
  3. Cushing’s (zona fasciculata tumour secreting cortisol)
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16
Q

What are some disorders associated with phaeochromocytomas?

A

MEN2 → parathyroid tumour, medullary thyroid cancer, phaeo

von Hippel Lindau syndrome → phaeo, renal cell carcinoma, renal cysts, haemangioblastoma

Neurofibromatosis type 1 → phaeo, peripheral/spinal neurofibromas, café au lait spots, etc.

17
Q

How would you test for phaechromocytomas?

A

Urinary catecholamines - high

18
Q

How should phaeochromocytoma be treated?

A

Medical emergency

  1. Immediate alpha blockade (phenoxybenzamine) → reflex tachycardia
  2. Add beta blockade (deals with reflex tachycardia)
  3. Surgery
19
Q

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

A

High aldosterone, low renin

Primary hyperaldosteronism -> adrenals autonomously secrete aldosterone → HTN → supress renin production at JGA

20
Q

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

A

Both low

At high concentrations, cortisol activates MR – 11b-hydroxysteroid dehydrogenase usually degrades cortisol to stop this happening but at concentration, the enzyme is overwhelmed → HTN

21
Q

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

A
  1. 9am cortisol
  2. 12am salivary cortisol (low = normal)
  3. LDDST (at 11pm → 1mg dexamethasone and measure cortisol before 9am next day)
22
Q

What would LDDST show in Cushing’s disease?

A

cortisol will not be suppressed

23
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 Suppression (i.e. pseudo-Cushing’s Syndrome in OBESITY)

24
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

25
How does obesity affect cortisol?
obesity can change metabolism of cortisol → condition mimicking Cushing's
26
What does the term 'cushing's disease' refer to?
Cushing's syndrome that is caused by a pituitary tumour secreting ACTH
27
How should Cushing's syndrome of indeterminate cause be investigated?
Inferior petrosal sinus sampling (IPSS) with CRH stimulation catheter → jugular vein distinguishes pituitary dependent and ectopic ACTH
28
Causes of Cushing's disease
Oral steroids (over treatment of another condition with oral steroids) 85% Pituitary-dependant Cushing’s disease 5% Ectopic ACTH (SCLC) 10% Adrenal adenoma
29
Why don't we do the high dose dexamethasone test in Cushing's?
85% are pituitary-dependant without test and → less accurate (false +ve = 20%) than guessing and so it is not useful
30
How would you treat Cushing's caused by adrenal mass?
adrenalectomy ± steroid replace
31
What complicaion should you be aware of with adrenelectomy?
Nelson’s syndrome removal of adrenal -\> pituitary enlargement (hypopituitarism by compressing stalk) and +++ ACTH (pigmentation)
32
How would you treat ectopic Cushing's?
ketoconazole, metyrapone, mifepristone
33
Mx of Pituitary adenoma
surgery
34
What is CAH caused by?
21-OH deficiency = 95% of CAH
35
How would CAH present?
Salt-losing crisis (low aldosterone) Virilisation (high testosterone/androgens)