L02: Adrenal Gland Flashcards

1
Q

What are the 3 layers of the adrenal cortex

A

Zona glomerulosa
Zona fasiculata
Zona reticularis

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2
Q

What hormone does the zona glomerulosa produce

A

Aldosterone

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3
Q

What hormone does the zona fasciulata produce

A

Cortisol

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4
Q

What hormone does the zona reticularis produce

A

DHEA

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5
Q

What is the inner layer of the adrenal gland called

A

Adrenal medulla

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6
Q

What does the adrenal medulla produce

A

Catecholamine: noradrenaline and adrenaline

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7
Q

What role does aldosterone have

A

Salt

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8
Q

What role does cortisol have

A

Sugar

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9
Q

What role does DHEA have

A

Sex

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10
Q

What does CAH stand for

A

Congenital adrenal hyperplasia

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11
Q

What is the major cause of CAH

A

21 Hydroxylase defiency

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12
Q

What stimulates the adrenal gland to produce cortisol

A

Hypothalamus pituitary axis

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13
Q

What does the pituitary gland release to the adrenal gland

A

ACTH

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14
Q

When there is 21 hydoxylase enzyme deficiency what happens to cortisol production

A

Decreases

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15
Q

When there is low cortisol what happen to cortisol feedback back to the hypothalamus

A

Reduced

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16
Q

If there is low cortisol feedback back to the hypothalamus what does the hypothalamus and pituitary do

A

Increase the release of ACTH to try and stimulate the release of cortisol

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17
Q

Instead of producing cortisol due to ACTH binding what does the adrenal glands produce instead

A

DHEA sex steroid (androgen)

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18
Q

Why is DHEA produced

A

It is not affected by the 21 hydroxylase enzyme deficiency

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19
Q

What does excess DHEA cause in new born baby girls

A

Virilization

Hyper plastic adrenals

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20
Q

What is the cause of of 21 hyrodoxylase enzyme

A

Mutation

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21
Q

Which from of 21 hydroxylase enzyme is likely to be mutated

A

CYP21A2

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22
Q

Which 2 categories of hormone is affected by 21 hydoxylase enzyme

A

Glucocorticoids: cortisol
Mineralocorticoid: aldosterone

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23
Q

How do we diagnose CAH

A

Look at level of the steroid 17OHP

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24
Q

What is 17OHP

A

The steroid produced prior to the block of 21 hydroxylase enzyme

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25
Q

What is virilization of the external female genitalia called due to excess androgen

A

46, XX, Disorder sex development (DSD)

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26
Q

How to we stage/classify virilization

A

Using the prader stage

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27
Q

How many stages are there in the prader stage

A

5

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28
Q

What happens to androgen and virilization as you increase the prader stage from 1 to 5

A

Androgens and virilization increases

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29
Q

What are the 3 forms of CAH

A

Classic CAH ‘salt wasting’
Classic CAH ‘simple virlising’
Non-classic CAH

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30
Q

Which hormones are deficiency in classic CAH ‘salt wasting’. Hint is in the name

A

Glucocorticoid deficiency: cortisol

Mineralocorticoid deficiency: aldosterone

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31
Q

What hormone is deficient in classic CAH ‘simple virilizing’

A

Glucocorticoid: cortisol

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32
Q

What hormone is deficient in non-classical CAH

A

Glucocorticoid: cortisol

33
Q

What other condition does non-classical CAH resemble

A

PCOS

34
Q

Which form of CAH is the most severe type

A

Classic CAH ‘salt wasting’

35
Q

Which from of CAH is the mildest one

A

Non-classical CAH

36
Q

What does the severity of CAH depend on

A

The mutation of the 21 hydroxylase enzyme

37
Q

What is the treatment for CAH

A

1) Replacement of glucocorticoid and mineralocorticoid

2) suppression of ACTH to suppress androgen excess

38
Q

Why is it important to give the correct amount of glucocorticoid

A
  • Too much glucocorticoid: suppresses LH and FSH = oligo/amennorhea
  • Too little glucocorticoid= androgen and progesterone excess because you get more release of ACTH and FSH/LH from the anterior pituitary gland = an ovulation, oligomenorrhea, failure to implant
39
Q

What is testicular adrenal rest tissue (TART) occur, describe the process

A

1) during development adrenal and gonads are close together in the urogenital ridge
2) when they separate the both take adrenal and gonadal features
3) so with ACTH stimulate the testicles expand and compress the normal sperm production
4) this impairs infertility if left untreated

40
Q

What are the signs and symptoms of classical CAH

A

Weight loss
Vomiting
Poor feeding
Failure to thrive

41
Q

What are the signs and symptoms in non-classical CAH

A

Oligomenorhhea
Hirtusim
Male pattern baldness
Subfertility

42
Q

To confirm classical CAH what investigation is required

A

Karyotyping and sequencing analysis of CYP21A2 locus
Serum 17OHP
Renal function and blood glucose

43
Q

What are the investigation required for non-classical CAH

A

Early morning serum 17OHP

44
Q

If in the investigation of non-classical CAH the serum levels of 17OHP is increased or borderline what test is required

A

ACTH stimulation test with measurement of 17OHP and cortisol
Androgens
Sequencing and analysis of CYP21A2

45
Q

In classical CAH salt wasting what do we give to replace the minerolocortiocid and glucocorticoid deficiency

A

Mineralocoticoid: flufocortisone
Glucocorticoid: hydrocortisone

46
Q

In classic CAH simple virilizing how do we replace the glucocorticoid deficiency

A

Hydrocortisone

47
Q

How do we manage oligomenorhea and hirtusim in non classic CAH

A

Glucocorticoid therapy

Contraceptive pills

48
Q

What does PCOS stand for

A

Polycystic ovary syndrome

49
Q

What are the 3 Rotterdam diagnostic criteria for PCOS

A

1) Polycystic ovary appearance on ultrasound
2) androgen excess: hair growth, acne, male pattern baldness
3) an ovulation: irregular or absence menstrual cycle

50
Q

To diagnose PCOS how many criteria’s should be met out of the 3

A

2 out of 3

51
Q

What other key features does PCOS present with

A

Insulin resistance

52
Q

Is there a treatment available for PCOS

A

NO

53
Q

How is PCOS managed

A
  • lifestyle interventions: diet, exercise, smoking, mental wellbeing
54
Q

How do we diagnose adroitness excess using a biochemical test

A

Look for:
Increased free testosterone
Increase free androgen index

55
Q

What are the pharmacological treatments available for PCOS

A
Combined oral contraceptive pills
Metformin 
Anti-androgens 
IVF
Ovulation induction: clomiphene or letrozole
56
Q

What needs to excluded prior to PCOS

A
CAH
Adrenal cancer 
Adrenal adenoma 
Cushing syndrome 
Ovarian hyperthescosis 
Ovarian tumours
57
Q

What are the 2 questions to discover if there is an adrenal tumour

A

1) if there is hormone excess

2) if there is malignancy

58
Q

Which tumours present with hormonal excess

A

Primary aldosteronism
Cushing’s syndrome
Adrenal androgen excess
Phaechromcytoma

59
Q

What a benign nodules on the adrenal glands that produce cortisol excess called

A

Mild autonomous cortisol excess (MACE)

60
Q

How is MACE different to Cushing’s

A

Patients do no present with typical signs

61
Q

How do we diagnose MACE

A

1mg dexamethasone supression test

62
Q

If the cortisol level are between 51-138 mmol/L what is the MACE

A

MACE-1

63
Q

If the cortisol is greater than 138nmol/L what is the MACE

A

MACE-2

64
Q

What are the cardio-metabolic risks in MACE

A

Hypertension
Type 2 diabetes
Dyslipidaemia

65
Q

How do we diagnose phaechromocytoma

A

Measure metanephrines (metabolites of catecholamines)

66
Q

Which conditions present with a malignant tumour

A

Adrenal carcinoma

67
Q

What are adrenal carcinomas

A

High malignant tumours

68
Q

How do we differentiate between adrenocortical adenoma (benign) and adrenocortical carcinoma (malignant)

A

Imaging
Tumour radiodensity via HU
Tumour diameter

69
Q

What does imaging show for an adenoma and carcinoma

A

Adenoma : fatty

Carcinoma: necrosis

70
Q

What does the tumour radiodensity (HU) show for an adenoma and carcinoma

A

Higher the density i,e above 10 the more likely it is going to be carcinoma

71
Q

What does the tumour diameter show for an adenoma and carcinoma

A

Adenoma: smalller
Carcinoma: larger

72
Q

What is adrenal incidentaloma

A

A finding of a adrenal gland mass upon imaging by accident

73
Q

At what mass is urgent assessment needed due to high chance of malignant cancer

A

Mass is greater than 4cm

74
Q

If the tumour is greater than 4 and has an tumour atentuation greater than 20 what should be carried out

A

Urine steroid metabolomics

75
Q

What is the treatment for phaechromocytoma

A

Alpha blockade
Beta blockade
Surgery

76
Q

If there is an adrenal tumour with hormone excess what should we exclude

A

Primary aldosteronism
Cushing syndrome, MACE
Phaechromocytoma

77
Q

How would we exclude Cushing syndrome

A

Perform a dexamethosine 1mg test

78
Q

How do we exclude primary hyperaldosteronism

A

Measure blood pressure - should be high

Aldosterone/renin ratio- should be high

79
Q

How do we exclude phaechromocytoma

A

Measure plasma metanephrines