Disorders of adrenocortical dysfunction Flashcards

1
Q

Where is the adrenal gland?

A

→ On top of the kidney

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2
Q

What type of tissue is the adrenal cortex?

A

→ Glandular tissue

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3
Q

What are the 3 areas of the adrenal cortex, where are they and what do they produce?

A

→ Zona glomerulosa - outside - aldosterone
→ Zona fasciculata - middle - cortisol
→ Zona reticularis - inside - testosterone

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4
Q

What does the medulla produce?

A

→ Adrenaline

→ Noradrenaline

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5
Q

Where is noradrenaline also produced?

A

→ Sympathetic nervous system

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6
Q

Where is the 21 hydroxylase enzyme found?

A

→ Only in the adrenal

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7
Q

What is produced in the second half of the menstrual cycle?

A

→ 17 OH is decreased

→ Progesterone is increased

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8
Q

What is produced in the first half of the menstrual cycle?

A

→ Ovary produced testosterone
→ Aromatase is produced
→ Testosterone is converted to estradiol

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9
Q

What does testosterone become in men?

A

→ Testosterone is reduced
→ By 5 alpha reductase
→ Into dihydrotestosterone (main androgen in men)

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10
Q

What is congenital adrenal hyperplasia?

A

→ Genetically XX

→ Adrenal glands can’t make progesterone

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11
Q

How does cortisol increase plasma glucose levels?

A

→ Increases gluconeogenesis
→ Decreases glucose utilization
→ Increases glycogenolysis
→ Increases glycogen storage

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12
Q

What are the effects of cortisol?

A
→ Increases plasma glucose
→ Increases lipolysis 
→ Proteins are catabolized - releases AA
→ Na+ and H2O retention maintains BP 
→ Anti inflammatory 
→ Decreases gastric acid production
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13
Q

What is the definition of Cushings syndrome?

A

→ Excessive levels of the steroid hormone cortisol

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14
Q

What is Cushings disease?

A

→ Excess cortisol

→ Due to an ACTH secreting pituitary tumor

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15
Q

What are the clinical features of Cushings syndrome?

A
→ Change in body shape
→ Central obesity 
→ Moon face
→ Buffalo hump
→ Thin skin 
→Osteoporosis 
→ Diabetes
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16
Q

What is the difference between a moon face and an obese face?

A

→ The obese face has indentation around the eyes

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17
Q

What are the changes in sex hormones in Cushings syndrome?

A

→ Excess hair growth
→ Irregular periods
→ Problems conceiving
→ Impotence

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18
Q

What are the 3 screening tests for Cushings?

A

→ Urinary free cortisol
→ Diurnal rhythm
→ Overnight dexamethasone suppression testing

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19
Q

What is the diurnal rhythm of cortisol?

A

→ Cortisol peaks in the morning

→ At night it is undetectable

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20
Q

When do you test for cortisol?

A

→ At midnight
→ There should be none
→ If there is cortisol at midnight then something is wrong

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21
Q

What is the renal threshold for cortisol?

A

→ 3-5% of cortisol appears in the urine

→ Freely filtered

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22
Q

How do you do a urine test?

A

→ 24h collection of urine

→ to check for abnormal cortisol levels

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23
Q

If you want to inhibit cortisol production from the pituitary what do you give?

A

→ Cortisol

→ Negative feedback

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24
Q

How do you clinically suppress cortisol and why?

A

→ Dexamethesone

→ Brain can’t tell the difference between cortisol and DMSone

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25
Q

What happens if a Dexamethesone tablet is given to a normal person?

A

→ negative feedback
→ ACTH production stops
→ Cortisol decreases

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26
Q

What are 3 possibilities if the dexamethesone test doesn’t work?

A

→ True Cushings
→ Pseudocushings
→ Exogenous steroids

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27
Q

Why does the dexamethesone test not work even if you don’t have Cushings?

A

→ Baseline cortisol levels may be high

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28
Q

What is the low dose dexamethesone test and what is the purpose of this?

A

→ 4 tablets for 2 days
→ Cortisol decreases below 50
→ Rules out people with pseudocushings

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29
Q

How do you know if a person has Cushings from a low dose dexamethesone test?

A

→ If after the test the cortisol levels are still high

30
Q

What are 3 causes of Cushings?

A

→ Adenoma in pituitary - disease
→ Tumor in adrenal gland - Syndrome
→ Ectopic ACTH production - syndrome

31
Q

What is the cortisol feedback loop?

A
→ CRH is released from the hypothalamus 
→ goes to pituitary 
→ Stimulates ACTH 
→ ACTH stimulates adrenal gland to make cortisol 
→ Cortisol inhibits hypothalamus
32
Q

What does the pituitary gland do when there is high cortisol?

A

→ When it exposed to large amounts of cortisol

→ Downregulates ACTH

33
Q

What is the higher dexamethesone test?

A

→ 4x higher dose of DMsone
→ Amount of cortisol should drop to 1/2
→ If it stays high
→ not pituitary

34
Q

What are ACTH levels like in ectopic ACTH production?

A

→ ACTH will be high

35
Q

What are the lab features of Cortisol excess?

A

→ Hypokalaemia
→ Metabolic alkalosis
→ Hyperglycaemia

36
Q

Where does aldosterone bind in the kidney?

A

→ Mineralocorticoid receptor

37
Q

What is the function of the mineralocorticoid receptor?

A

→ Retain sodium

38
Q

How is the electrical balance retained at the mineralocorticoid receptor?

A

→ Na+ retained

→ K+ lost

39
Q

What happens if there are no more K+ ions to exchange in the mineralocorticoid receptor?

A

→ Use H+

→ Metabolic alkalosis

40
Q

Why does cortisol not usually bind to the mineralocorticoid receptor?

A

→ Cortisol can bind to the mineralocorticoid receptor
→ 11 beta hydroxysteroid dehydrogenase 2 is part of the mineralocorticoid receptor
→ Cortisol → cortisone by the enzyme (destroyed)
→ Only aldosterone can bind to the receptor

41
Q

What happens during hypovolaemia to retain Na+ (mineralocorticoid receptor)?

A

→ The catalytic site of 11 beta hydroxysteroid dehydrogenase 2 has a saturation point which is set higher than normal cortisol

→ Saturation point is lower than extreme cortisol

→ In extreme cortisol the enzyme gets saturated and cortisol can access the receptor

→ so maximal sodium can be retained

42
Q

What does the POMC gene provide?

A

→ Painkiller
→ Antibiotics
→ Hunger suppressor

43
Q

What does the alpha MSH gene do?

A

→ Powerful appetite suppressor

→ Involved in pigmentation

44
Q

What happens if you give CRH to a normal person?

A

→ ACTH double

45
Q

What happens if you give CRH to an adrenal tumor person?

A

→ suppression of ACTH
→ Too much cortisol
→ cannot respond to CRH

46
Q

What happens if you give CRH to an ectopic ACTH person?

A

→ ACTH are high

→ doesn’t rise

47
Q

What happens if you give CRH to someone with Cushings?

A

→ ACTH rises a lot

→ Pituitary cells are still functional but there are more of them

48
Q

What are 2 ways of localizing a pituitary tumor?

A

→ MRI

→ IPSS

49
Q

What are 2 ways of finding an adrenal tumor?

A

→ CT

→ MRI

50
Q

What are ways of finding ectopic cells?

A

→ Octreotide scan

→ ACTH sampling

51
Q

What are 2 cortisol blockers?

A

→ Metyrapone

→ Ketoconazole

52
Q

What are the clinical features of Addisons?

A

→ Hyponatremia
→ Hyperkalaemia
→ Salt craving
→ Nausea

53
Q

What are some causes of Addisons?

A

→ Autoimmune
→ TB
→ Steroid withdrawal
→Enzyme defect

54
Q

What happens if you give a person synthetic ACTH?

A

→Cortisol levels should rise

55
Q

What is the insulin tolerance test?

A

→ Dose of insulin

→ Hypoglycaemic (stress)

56
Q

How do you replace cortisol?

A

→ Hydrocortisone

→ mimic diurnal rhythm

57
Q

What is the most common cause of congenital adrenal hyperplasia?

A

→ 21 hydroxylase deficiency

→ Yupik Eskimos

58
Q

What do excess sex steroids cause?

A

→ Virilization
→ Hirsutism
→ Infertility

59
Q

What is non classical adrenal hyperplasia?

A

→ 11 beta hydroxylase deficiency

60
Q

What are symptoms of people who have non classical adrenal hyperplasia?

A

→ Cannot produce cortisol
→ Deoxycorticosterone is raised ( same effects as aldosterone)
→ Hypertension

61
Q

What is phaeochromocytoma?

A

→ Tumor of enterochromaffin cells of the adrenal medullar

→ produce adrenaline

62
Q

What is used to replace absent mineralocorticoid?

A

→ 21 hydroxylase deficiency

63
Q

How is renin released?

A

→ Afferent arteriole brings blood in glomerulus and senses blood pressure
→ Macula densa in DCT senses salt
→ JG cells produce renin

64
Q

What is primary excess of aldosterone?

A

→ Tumor producing aldosterone

→ Renin levels are low

65
Q

What is secondary aldosterone syndrome?

A

→ Renin secreting tumor

→ renal artery stenosis

66
Q

What is the treatment for Conns?

A

→ Spironolactone

→ Amiloride

67
Q

What is phaeochromocytoma?

A

→ Tumor of enterochromaffin cells of the adrenal medullar

→ produce adrenaline

68
Q

What are the symptoms of phaeochromocytoma?

A

→ high BP (paroxysmal)
→ Sweating
→ Anxiety

69
Q

What do you look for in the urine of someone with phaeochromocytoma?

A

→ Metapnephrines

70
Q

How do you manage phaeochromocytoma?

A

→ Alpha blockade

→ Beta blockade - propanolol