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Flashcards in Adrenal gland Deck (96):
1

what is secreted from the zona glomerulosa

mineralocorticoids
aldosterone

2

What is secreted from the zona fasciculata

glucocorticoids
cortisol
corticosterone

3

what is secreted from the zona reticularis

adrenal androgens
DHEA
Androstenedione

4

what is the zona glomerulosa regulated by

the renin-angiotensin system
plasma K

5

What is cortisol and androgen production regulated by

hypothalamus and anterior pituitary

6

Cortisol causes an increase in what

cardiac output
BP
renal blood flow and GFR
blood sugar
lipolysis
proteolysis

7

what can cortisol cause to do with the mind

euphoria/psychosis

8

Cortisol causes a decrease in what

libido
serum Ca and collagen formation
wound healing
capillary dilatation
leukocyte migration and macrophage activity

9

At which sites does aldosterone bind

mineralocorticoid receptors at kidneys
salivary gland
gut
sweat glands

10

What does aldosterone regulate

Na/K balance
BP
ECF volume

11

what is the most common cause of primary adrenal insufficiency

Addison's disease

12

What percentage of the adrenal cortex has to be destroyed for a patient with Addison's to get symptoms

>90%

13

Is Addison's an autoimmune disease

yes

14

What other autoimmune conditions is Addison's associated with

autoimmune thyroid disease
T1DM
pernicious anaemia

15

what are the symptoms of Addison's

weight loss
anorexia
craving for salt
N&V
Abdominal pain
diarrhoea/constipation
SOB
fatigue/weakness
skin pigmentation

16

What is the biochemistry in Addisons

increased K
Increased ACTH and renin
Decreased Na
decreased aldosterone

17

what is the test for Addison's

short synacthen test - measures plasma cortisol before and 30min after the injection of synthetic ACTH

18

What is the normal baseline for cortisol

>250

19

What is the normal cortisol measurement for after the injection of ACTH

>550

20

What is the treatment for Addison's

15-30mg Hydrocortisone BD
fludrocortisone BD - monitor BP and K

21

what are other causes of primary adrenal insufficiency

congenital adrenal hyperplasia
adrenal TB
adrenal malignancy

22

What is congenital adrenal hyperplasia

a rare condition associated with enzyme defects in steroid pathways

23

What is most commonly the deficiency in congenital adrenal hyperplasia

21alpha hydroxylase

24

What type of inheritance is congenital adrenal hyperplasia

autosomal recessive

25

what is the investigation for congenital adrenal hyperplasia

basal 17-OH progesterone concentration

26

What is the treatment for congenital adrenal hyperplasia

glucocorticoid and mineralocorticoid replacement in some
surgical correction
control androgen excess
restore fertility

27

what would a male with congenital adrenal hyperplasia present with

adrenal insufficiency
poor weight gain

28

what would a female with congenital adrenal hyperplasia present with

genital ambiguity

29

What is the most common cause of secondary adrenal insufficiency

iatrogenic - long term exogenous steroid use

30

what else is a cause of secondary adrenal insufficiency

pituitary/hypothalamic disorders

31

What happens in long term exogenous steroid use that causes secondary adrenal insufficiency

when you take high dose steroids for a while and stop abruptly it causes a decrease in cortisol as its not had to produce as much. This causes short term addison like symptoms which corrects when the ACTH recognises that the adrenal isn't producing enough cortisol

32

What is the treatment for excess exogenous steroid use

hydrocortisone replacement

33

Patients who have been on steroids for __________ should be weaned off them to prevent secondary adrenal insufficiency

>4-6 weeks

34

Cushing's syndrome can be described as

excess cortisol

35

Who is most likely to get Cushing's syndrome

Female
20-40yrs

36

What are the symptoms of Cushing's syndrome

ease of bruising
central obesity
proximal myopathy
striae
moon face
osteoporosis
facial plethora
hypertension

37

What is the ACTH dependent causes of Cushing's syndrome

pituitary adenoma (68%)
ectopic ACTH
ectopic CRH

38

What is the ACTH independent causes of Cushing's syndrome

adrenal adenoma (10%)
adrenal carcinoma
nodular hyperplasia

39

What is the investigation for Cushing's syndrome

overnight dexamethasone test
if positive - low dose (2mg) dexamethasone test
repeat to confirm

40

What is the most common cause of Cushing's syndrome

iatrogenic - due to prolonged high dose steroid therapy

41

How does iatrogenic Cushing's syndrome occur

long term high dose steroids causes chronic suppression of ACTH and adrenal atrophy

42

Primary aldosteronism is most likely to present with

hypertension +hypokalaemia
alkalosis

43

What is the biochem of primary aldosteronism

increased Na
decreased K

44

What is primary aldosteronism

autonomous production of aldosterone independent of its own regulators

45

what is the commonest secondary cause of hypertension

primary aldosteronism

46

What are the 2 subtypes of primary aldosteronism

adrenal adenoma
bilateral adrenal hyperplasia

47

what is the more common of the 2 subtypes of primary aldosteronism

bilateral adrenal hyperplasia

48

What is the condition of an adrenal adenoma known as in primary aldosteronism

Conn's syndrome

49

What is the investigation for primary aldosteronism

measure primary aldosterone and renin in a ratio
if ratio is high then do a saline suppression test
failure of plasma aldosterone to suppress by >50% with 2L of normal saline is diagnostic

50

What other investigations are used in primary aldosteronism to figure out the subtype

adrenal CT
Adrenal vein sampling

51

What is the surgical treatment for primary aldosteronism

laparoscopic adrenalectomy
- cures hypokalaemia and decreases BP

52

When is surgical treatment used in primary aldosteronism

only for adrenal adenoma

53

What is the medical treatment for primary aldosteronism

MR antagonists = spironolactone

54

When is medical treatment used in primary aldosteronism

only for bilateral adrenal hyperplasia

55

What is the classic triad of pheochromocytoma

labile hypertension
paroxysmal sweating
headache

56

What are the other symptoms of pheochromocytoma

postural hypotension
pallor
tachycardia and bradycardia
palpitations
SOB
constipation
anxiety/fear
weight loss
pyrexia

57

What is the biochem of pheochromocytoma

increased catecholamines (noradrenaline, adrenaline, dopamine)
hyperglycaemia
increased Hb
mild hypercalcaemia
may have decreased K

58

Put in order the greatest concentration of catecholamine to the least

dopamine > noradrenaline > adrenaline

59

What is a pheochromocytoma

adrenal medulla cancer derived from the chromaffin cells (secrete catecholamines)

60

What type of onset does a pheochromocytoma have

insidious

61

The 10% tumour refers to what in a pheochromocytoma

10% malignant
10% bilateral
10% in children
10% extra-adrenal
10% familial
10% associated with hyperglycaemia

62

What are the complications of pheochromocytoma

LV failure
Myocardial necrosis
Stroke (CVA)
shock
paralytic ileus of bowel
arrhythmia

63

What is the investigations for pheochromocytoma

urine - 2x 24hr catecholamine/metanephrine conc
plasma - at time of symptoms

64

On pathology - why would a pheochromocytoma be brown

due to oxidation

65

What other investigations can be used for pheochromocytoma

MRI - abdo, whole body
MIBG
PET scan

66

What is the pharmacological treatment for pheochromocytoma

alpha blocker - phenoxybenzamine
B blocker - propanolol/atenolol/metoprolol
fluid +/- blood replacement

67

What is the surgical treatment for pheochromocytoma

laparoscopic total excision of tumour
chemo if malignant

68

What are the 2 types of adrenocortical hyperplasia

congenital and acquired

69

Congenital adrenocortical hyperplasia is what type of inheritance

autosomal recessive

70

How does congenital adrenocortical hyperplasia occur

due to a deficiency/lack of enzyme for steroid biosynthesis causing increased androgen production and decreased cortisol

71

What would a patient present with in congenital adrenocortical hyperplasia

masculinisation
precocious puberty

72

What would decreased cortisol stimulate in congenital adrenocortical hyperplasia

ACTH release and cortical hyperplasia

73

What happens in acquired adrenocortical hyperplasia

there is endogenous ACTH production causing bilateral adrenal enlargement
e.g. pituitary adenoma
ectopic ACTH

74

What are the 2 subtypes of acquired adrenocortical hyperplasia

diffuse
nodular

75

What is diffuse acquired adrenocortical hyperplasia

ACTH driven

76

What is nodular acquired adrenocortical hyperplasia

usually ACTH independent

77

What are the 2 types of adrenocortical tumours

adenoma (more common)
carcinoma (rare)

78

An adrenocortical adenoma is usually______ in size

small

79

An adrenocortical carcinoma is usually ________ in size

large (>20cm)

80

Which adrenocortical tumour is more likely to be functional

carcinoma

81

Where does an adrenocortical carcinoma spread to

retroperitoneum - kidneys
vascular - liver, lung, bone
peritoneum and pleura
regional lymph nodes

82

What is the 5yr survival for an adrenocortical carcinoma

20-35% (50% dead in 2 yrs)

83

Primary hyperaldosteronism is known as

Conn's syndrome

84

What is Conn's syndrome associated with

diffuse/nodular hyperplasia

85

The most common endogenous cause of hypercortisolism is

ACTH secreting pituitary adenoma (cushing's disease)

86

A neuroblastoma is a congenital cancer of the

adrenal medulla (40%)

87

When are neuroblastomas usually diagnosed

infancy

88

ME2A is also known as

Sipple syndrome

89

What is present in MEN2A

pheochromocytoma (40-50%)
medullary thyroid carcinoma (100%)
Parathyroid hyperplasia

90

What chromosome has a mutation on it in MEN2A

It is a RET oncogene mutation on chromosome 10

91

What is present in MEN2B

mucosal neuromas
marfanoid body habitus
medullary thyroid carcinoma
pheochromocytoma

92

MEN2B is also linked to RET (true/false)

true

93

What present in MEN1

pituitary adenoma
parathyroid hyperplasia
pancreatic tumours
loss/reduced protein function

94

What percentage of MEN1 patients will die

50%

95

What is the treatment for MEN2

prophylactic thyroidectomy - prevents medullar thyroid cancer
screening for pheochromocytoma
screening for parathyroid disease

96

What presents in neurofibromatosis

Axillary freckling
Cafe au lait macules
Neurofibromas
optic gliomas
scoliosis