Adrenal cortex and cushing's syndrome Flashcards

1
Q

Which steroids are produced by the adrenal cortex

A

Glucocorticoids
Mineralcorticoids
Androgens

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2
Q

Process of steroid hormone release

A

CRF–>ACTH–>steorid release, negative feedback

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3
Q

Definition cushing’s syndrome (3 aspects)

A

+Glucocorticoid excess
Loss of negative feedback
Loss of normal cyclical pattern of GC relese

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4
Q

Chief cause of cushing’s syndrome

A

Iatrogenic- oral steroids

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5
Q

80% of endogenous cushing’s caused by, commonest endogenous

A

ACTH excess

Pituitary adenoma- cushings disease

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6
Q

Broad categories of cushing’s

A

ACTH dependent

ACTH independent

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7
Q

ACTH dependent causes (2)

A

Cushings disease
Ectopic ACTH- Small cell lung, pancreatic, medullary thyroid
Rarely CRF tumor

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8
Q

ACTH independent causes (4)

A

Adrenal adenoma/cancer
Adrenal nodular hyperplasia
Iatrogenic

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9
Q

What is cushing’s disease

A

Bilateral adrenal hyperplasia, due to +ACTH from pituitary microadenoma

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10
Q

Epidemiology in cushings disease

A

30-50 yo

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11
Q

How does low dose and high dose dexamethasone test change cortisol levels in CD

A
Low dose, no effect
High dose (>8mg) can halve morning cortisol levels
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12
Q

Specific features in ectopic ACTH production

A

Pigmentation
Hypokalemic metabolic alkalosis
Weight loss
Hyperglycemia

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13
Q

When ectopic ACTH, does high does dexamethasone suppress cortisol levels

A

No

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14
Q

Clinical features

A
Weakness
Insomnia
Mood disorders
Impaired cognition
Easy bruising
Oligo/Amenorrhea
Hirsutism and acne
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15
Q

Which symptoms are ACTH dependent

A

Hirsutism and acne

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16
Q

Signs

A
Central obesity
Mood face
Supraclabicular and dorsal fat pads
Facial plethora
Muscle wasting
Purple abdominal striae
Skin atrophy
Acanthosis nigricans
HTN
Hyperglycemia
Osteoporosis
Pathalogical fractures
Hyperpigmentation
Hyperandrogenism
17
Q

Key diagnostic factors

A
Risk factors
Facial plethora
SC fat pads
Striae
Absence of pregnancy
Menstural irregularities
Absence of malnutrition, alcoholism
Absence of physiological stress
Linear growth deceleration in children
18
Q

Strong risk factors

A

Exogenous cortisol use
Pituitary adenoma
Adrenal adenoma
Adrenal carcinoma

19
Q

History

A
Iatrogenic steroid use
Features unusual for age->osteoporosis
Unexplained psychiatric
Nephrolithiasis
Multiple/progressive symptoms
PCOS
Pituitary adenomas
Adrenal adenomas
20
Q

First line diagnostic test->use one of

A

Late night salivary cortisol >4nmol/L, at lease 2 readings
Overnight 1mg dex suppression testing >50nmol/L
24 hour urinary free cortisol-> >3 times upper limit of normal, at least 2 readings
48 hour 2mg dex suppression testing

Should repeat the diagnostic tests
Confirmed if any two are positive

21
Q

Algorithm for cushing diagnosis

A

Cushings expected–>exclude exogenous–>Perform one of high sensitive tests

If negative->Cushings unlikely
If positive->exclude physiological causes->confirm positive test and perform 1 or 2 additional studies->referral to endocrinologist

If positive->cushings->measure plasma ACTH
If negative->cushings unlikely

Suppressed ACTH->independent of ACTH->imaging of adrenals
XSuppressed ACTH= ACTH-dependent–>MRI of pituitary

22
Q

Physiological causes of cushing’s

A
Physical stress
Malnutrition
Alcoholism
Depression
Pregnancy
Morbid obesity/metabolic syndrome
23
Q

Other tests to perform

A

Glucose

Pregnancy

24
Q

If a pituitary adenoma is found on MRI, at what size should you proceed to treatment

A

6mm

25
Q

Management->ACTH secreting tumor

A

Transphenoidal pituitary adenomectomy
Adjunct:
Medical therapy prior to surgery–> mifepristone or pasiretide or ketoconazole
Post surgical cortisol replacement->hydrocortisone
Non-cortisol replacement->levothyroxine +/- testosterone, estrogen, medroxyprogesterone, somatotropin, desmopressin

26
Q

What is pasireotide and how does it work

A

Somatostatin analogue->binds to receptor expressed by corticotrophs in adenomas->decreaseing cortisol

27
Q

What tole does ketoconazole have

A

Steroidogenesis inhibitor

28
Q

What is the role of mifepristone

A

Glucocorticoid receptor antagonist->blocks cortisol at receptor levels and attenuates effects of elevated cortisol

29
Q

When treated with medical therapy before surgery, what must be monitored for

A

Adrenal insufficiency

30
Q

Postoperatively what symptoms should be checked for

A

BP
Orthostatic hypotension
General sense of energy/fatigue

31
Q

Which hormones may be needed (not including cortisol) post surgery

A
Levothyroxine
Testosterone
Estrogen + progestin (10 days/month)
?GH
Desmopressin
32
Q

Other options for management of ACTH tumor

A

Repeat surgery
Pituitary radiotherapy
Bilateral adrenalectomy

33
Q

Management of ectopic ACTH or CRH syndrome

A

Surgical resection/ablation of tumor/metastasis
Medical therapy- mifepristone, pasireotide, ketoconazole
Chemo/radiotherapy for primary tumor

34
Q

Management of ACTH independent->unilateral adrenal carcinoma or adenoma

A

Unilateral adrenalectomy/tumor resection
Medical therapy before surgery
Chemo/radiotherapy for adrenal carcinoma

35
Q

Management of ACTH independent due to bilateral adrenal disease

A

Bilateral adrenalectomy
Permanent post surgical corticosteroid replacement therapy
Medical therapy before surgery

36
Q

Which conditions are unlikely to having clinical features of cushings

A
Physical stress
Malnutiriton
Anorexia
Intense chronic exercise
Hypothalmic amenorrhea
CBG excess
37
Q

Interpreting high dose dexamethasone suppression test

A

8mg dexamethasone at 11pm- Next morning 8am cortisol Cushing syndrome of pituitary origin

38
Q

What is the inferior petrosal sinus sampling for

A

Confirm side of hypersecretion