Adrenal gland Flashcards

1
Q

what is secreted from the zona glomerulosa

A

mineralocorticoids

aldosterone

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2
Q

What is secreted from the zona fasciculata

A

glucocorticoids
cortisol
corticosterone

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3
Q

what is secreted from the zona reticularis

A

adrenal androgens
DHEA
Androstenedione

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4
Q

what is the zona glomerulosa regulated by

A

the renin-angiotensin system

plasma K

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5
Q

What is cortisol and androgen production regulated by

A

hypothalamus and anterior pituitary

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6
Q

Cortisol causes an increase in what

A
cardiac output
BP
renal blood flow and GFR
blood sugar
lipolysis 
proteolysis
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7
Q

what can cortisol cause to do with the mind

A

euphoria/psychosis

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8
Q

Cortisol causes a decrease in what

A
libido
serum Ca and collagen formation
wound healing
capillary dilatation
leukocyte migration and macrophage activity
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9
Q

At which sites does aldosterone bind

A

mineralocorticoid receptors at kidneys
salivary gland
gut
sweat glands

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10
Q

What does aldosterone regulate

A

Na/K balance
BP
ECF volume

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11
Q

what is the most common cause of primary adrenal insufficiency

A

Addison’s disease

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12
Q

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

A

> 90%

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13
Q

Is Addison’s an autoimmune disease

A

yes

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14
Q

What other autoimmune conditions is Addison’s associated with

A

autoimmune thyroid disease
T1DM
pernicious anaemia

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15
Q

what are the symptoms of Addison’s

A
weight loss
anorexia
craving for salt 
N&V
Abdominal pain 
diarrhoea/constipation 
SOB
fatigue/weakness 
skin pigmentation
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16
Q

What is the biochemistry in Addisons

A

increased K
Increased ACTH and renin
Decreased Na
decreased aldosterone

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17
Q

what is the test for Addison’s

A

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

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18
Q

What is the normal baseline for cortisol

A

> 250

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19
Q

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

A

> 550

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20
Q

What is the treatment for Addison’s

A

15-30mg Hydrocortisone BD

fludrocortisone BD - monitor BP and K

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21
Q

what are other causes of primary adrenal insufficiency

A

congenital adrenal hyperplasia
adrenal TB
adrenal malignancy

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22
Q

What is congenital adrenal hyperplasia

A

a rare condition associated with enzyme defects in steroid pathways

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23
Q

What is most commonly the deficiency in congenital adrenal hyperplasia

A

21alpha hydroxylase

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24
Q

What type of inheritance is congenital adrenal hyperplasia

A

autosomal recessive

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25
Q

what is the investigation for congenital adrenal hyperplasia

A

basal 17-OH progesterone concentration

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26
Q

What is the treatment for congenital adrenal hyperplasia

A

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

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27
Q

what would a male with congenital adrenal hyperplasia present with

A

adrenal insufficiency

poor weight gain

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28
Q

what would a female with congenital adrenal hyperplasia present with

A

genital ambiguity

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29
Q

What is the most common cause of secondary adrenal insufficiency

A

iatrogenic - long term exogenous steroid use

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30
Q

what else is a cause of secondary adrenal insufficiency

A

pituitary/hypothalamic disorders

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31
Q

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

A

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

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32
Q

What is the treatment for excess exogenous steroid use

A

hydrocortisone replacement

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33
Q

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

A

> 4-6 weeks

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34
Q

Cushing’s syndrome can be described as

A

excess cortisol

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35
Q

Who is most likely to get Cushing’s syndrome

A

Female

20-40yrs

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36
Q

What are the symptoms of Cushing’s syndrome

A
ease of bruising 
central obesity 
proximal myopathy 
striae 
moon face
osteoporosis 
facial plethora 
hypertension
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37
Q

What is the ACTH dependent causes of Cushing’s syndrome

A

pituitary adenoma (68%)
ectopic ACTH
ectopic CRH

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38
Q

What is the ACTH independent causes of Cushing’s syndrome

A

adrenal adenoma (10%)
adrenal carcinoma
nodular hyperplasia

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39
Q

What is the investigation for Cushing’s syndrome

A

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

40
Q

What is the most common cause of Cushing’s syndrome

A

iatrogenic - due to prolonged high dose steroid therapy

41
Q

How does iatrogenic Cushing’s syndrome occur

A

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

42
Q

Primary aldosteronism is most likely to present with

A

hypertension +hypokalaemia

alkalosis

43
Q

What is the biochem of primary aldosteronism

A

increased Na

decreased K

44
Q

What is primary aldosteronism

A

autonomous production of aldosterone independent of its own regulators

45
Q

what is the commonest secondary cause of hypertension

A

primary aldosteronism

46
Q

What are the 2 subtypes of primary aldosteronism

A

adrenal adenoma

bilateral adrenal hyperplasia

47
Q

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

A

bilateral adrenal hyperplasia

48
Q

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

A

Conn’s syndrome

49
Q

What is the investigation for primary aldosteronism

A

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
Q

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

A

adrenal CT

Adrenal vein sampling

51
Q

What is the surgical treatment for primary aldosteronism

A

laparoscopic adrenalectomy

- cures hypokalaemia and decreases BP

52
Q

When is surgical treatment used in primary aldosteronism

A

only for adrenal adenoma

53
Q

What is the medical treatment for primary aldosteronism

A

MR antagonists = spironolactone

54
Q

When is medical treatment used in primary aldosteronism

A

only for bilateral adrenal hyperplasia

55
Q

What is the classic triad of pheochromocytoma

A

labile hypertension
paroxysmal sweating
headache

56
Q

What are the other symptoms of pheochromocytoma

A
postural hypotension 
pallor
tachycardia and bradycardia
palpitations
SOB
constipation 
anxiety/fear
weight loss
pyrexia
57
Q

What is the biochem of pheochromocytoma

A
increased catecholamines (noradrenaline, adrenaline, dopamine) 
hyperglycaemia
increased Hb
mild hypercalcaemia 
may have decreased K
58
Q

Put in order the greatest concentration of catecholamine to the least

A

dopamine > noradrenaline > adrenaline

59
Q

What is a pheochromocytoma

A

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

60
Q

What type of onset does a pheochromocytoma have

A

insidious

61
Q

The 10% tumour refers to what in a pheochromocytoma

A
10% malignant
10% bilateral 
10% in children 
10% extra-adrenal 
10% familial 
10% associated with hyperglycaemia
62
Q

What are the complications of pheochromocytoma

A
LV failure 
Myocardial necrosis 
Stroke (CVA)
shock
paralytic ileus of bowel 
arrhythmia
63
Q

What is the investigations for pheochromocytoma

A

urine - 2x 24hr catecholamine/metanephrine conc

plasma - at time of symptoms

64
Q

On pathology - why would a pheochromocytoma be brown

A

due to oxidation

65
Q

What other investigations can be used for pheochromocytoma

A

MRI - abdo, whole body
MIBG
PET scan

66
Q

What is the pharmacological treatment for pheochromocytoma

A

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

67
Q

What is the surgical treatment for pheochromocytoma

A

laparoscopic total excision of tumour

chemo if malignant

68
Q

What are the 2 types of adrenocortical hyperplasia

A

congenital and acquired

69
Q

Congenital adrenocortical hyperplasia is what type of inheritance

A

autosomal recessive

70
Q

How does congenital adrenocortical hyperplasia occur

A

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

71
Q

What would a patient present with in congenital adrenocortical hyperplasia

A

masculinisation

precocious puberty

72
Q

What would decreased cortisol stimulate in congenital adrenocortical hyperplasia

A

ACTH release and cortical hyperplasia

73
Q

What happens in acquired adrenocortical hyperplasia

A

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

74
Q

What are the 2 subtypes of acquired adrenocortical hyperplasia

A

diffuse

nodular

75
Q

What is diffuse acquired adrenocortical hyperplasia

A

ACTH driven

76
Q

What is nodular acquired adrenocortical hyperplasia

A

usually ACTH independent

77
Q

What are the 2 types of adrenocortical tumours

A

adenoma (more common)

carcinoma (rare)

78
Q

An adrenocortical adenoma is usually______ in size

A

small

79
Q

An adrenocortical carcinoma is usually ________ in size

A

large (>20cm)

80
Q

Which adrenocortical tumour is more likely to be functional

A

carcinoma

81
Q

Where does an adrenocortical carcinoma spread to

A

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

82
Q

What is the 5yr survival for an adrenocortical carcinoma

A

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

83
Q

Primary hyperaldosteronism is known as

A

Conn’s syndrome

84
Q

What is Conn’s syndrome associated with

A

diffuse/nodular hyperplasia

85
Q

The most common endogenous cause of hypercortisolism is

A

ACTH secreting pituitary adenoma (cushing’s disease)

86
Q

A neuroblastoma is a congenital cancer of the

A

adrenal medulla (40%)

87
Q

When are neuroblastomas usually diagnosed

A

infancy

88
Q

ME2A is also known as

A

Sipple syndrome

89
Q

What is present in MEN2A

A

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

90
Q

What chromosome has a mutation on it in MEN2A

A

It is a RET oncogene mutation on chromosome 10

91
Q

What is present in MEN2B

A

mucosal neuromas
marfanoid body habitus
medullary thyroid carcinoma
pheochromocytoma

92
Q

MEN2B is also linked to RET (true/false)

A

true

93
Q

What present in MEN1

A

pituitary adenoma
parathyroid hyperplasia
pancreatic tumours
loss/reduced protein function

94
Q

What percentage of MEN1 patients will die

A

50%

95
Q

What is the treatment for MEN2

A

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

96
Q

What presents in neurofibromatosis

A
Axillary freckling 
Cafe au lait macules 
Neurofibromas
optic gliomas
scoliosis