Disorders Of Adrenocortical Function Flashcards

1
Q

What is congenital adrenal hyperplasia?

A

Impaired cortisol synthesis due to a deficiency of one of the 5 enzymes needed for cortisol synthesis

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2
Q

Where are adrenaline and noradrenaline made?

A

Adrenal medulla

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3
Q

What are the three stages of cushings disease investigation?

A

Screening, confirmation and differentiation of the cause

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4
Q

What are the three differential diagnosis for positive screening?

A

True Cushing’s syndrome, pseudo Cushing’s syndrome and exogenous steroids

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5
Q

What are the symptoms of pseudocushings syndrome?

A

Depression, alcoholism, anorexia and obesity

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6
Q

When does ectopic ACTH production happen?

A

When the neuroendocrine cells become malignant

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7
Q

What happens in terms of aldosterone and cortisol in extreme situations (like bleeding to death)?

A

You dont have enough aldosterone so cortisol oversaturates the cortisol -> cortisone enzyme and then causes sodium retention

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8
Q

What imaging do you do for a pituitary tumour?

A

MRI or inferior petrosa sinus sampling

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9
Q

What imaging do you do for an adrenal tumour?

A

CT or MRI

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10
Q

What imaging do you do for an ectopic tumour?

A

ACTH sampling or octreotide scan

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11
Q

What is an octreotide scan?

A

Putting a nuclear tracer on somatostatin

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12
Q

Why do patients need to take steroid replacement tablets when they have tumour removal surgery?

A

The tumour suppresses the glands ability for secretion, so it needs time to replenish

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13
Q

What are some of the causes of addisons syndrome?

A

Autoimmune, TB, steroid withdrawal, metastasis, infiltration, apoplexy, infection, enzyme defect and drugs

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14
Q

What are the four dynamic tests for adrenal insufficiency?

A

Short Synacthen, long synacthen test, insulin tolerance test and glucagon test

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15
Q

What are the two treatments for Addison’s disease?

A

Hydrocortisone and fludrocortisone

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16
Q

What is the most common deficiency in congenital adrenal hyperplasia?

A

21- hydroxylase

17
Q

Why is lots of testosterone produced in congenital adrenal hypoplasia?

A

No cortisol means no negative feedback and lots of ACTH -> lots of cholesterol -> lots of testosterone

18
Q

What does excess testosterone cause?

A

Virilisation, hirsutism, infertility

19
Q

What does 11 beta hydroxylase deficiency cause?

A

No aldosterone or cortisol but high deoxycorticosterone and testosterone

20
Q

How do you test for congenital adrenal hypoplasia?

A

Synacthen test or prednisone suppression

21
Q

What would you see in the synacthen test in a 11 beta hydroxylase deficient patient?

A

No cortisol rise and increased 17OH progesterone levels

22
Q

What should happen in a prednisone suppression test?

A

Androgens should fall into normal range

23
Q

How do you treat congenital adrenal hypoplasia?

A

Glucocorticoid therapy, surgery to virilised female genetalia and treatment of mother to prevent foetal virilisation

24
Q

Where is aldosterone produced?

A

Zone glomerulosa of the adrenal cortex

25
What are the clinical signs of primary excess aldosterone syndromes?
High aldosterone and low renin
26
What are the clinical signs of secondary excess aldosterone syndromes?
High aldosterone and high renin
27
What are the names of some primary aldosterone excess syndromes?
Conn’s, bilateral adrenal hyperplasia, steroid treatable hypertension and aldosterone producing adrenal carcinoma
28
What are the names of some secondary aldosterone excess conditions with hypertension?
Renal artery stenosis, renin secreting tumour, malignant nephrosclerosis
29
What are the names of some secondary aldosterone excess conditions with a normal BP?
CCF, cirrhosis, nephrotic syndrome and dehydration
30
What is a phaeochromocytoma?
Tumour of the enterochromaffin cells of the adrenal medulla
31
Where do extra adrenal phaeochromocytomas occur?
In sympathetic ganglia or organ of zuckerkandl
32
What catecholamines do phaeochromocytomas produce?
Adrenaline, noradrenaline and dopamine
33
What is the rule of 10 with phaeochromocytomas?
10% bilateral, 10% malignant and 10% extra adrenal
34
What are some phaeochromocytoma- associated syndromes?
Neurofibromatosis type 1, von hippel-Lindau disease, multiple endocrine neoplasia type 2, hereditary paraganglioma syndrome
35
Why is the thyroid gland removed in multiple endocrine neoplasia type 2 patients?
Stop the formation of a medullary thyroid carcinoma
36
What are the main symptoms of phaeochromocytoma?
Sweating, headache, tachycardia
37
What are the pharmacological symptoms of phaeochromocytoma?
Eosinophilia, hyperglycaemia, hypercalcemia, raised urinary metonephrines and catecholamines
38
What types of drugs are used to manage phaeochromocytomas?
Alpha and beta blockade