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adrenal gland disorders Flashcards

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1
Q

what part of the adrenal gland is highlighted red?

A

medulla

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2
Q

what part of the adrenal gland is highlighted red?

A

cortex

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3
Q

what is highlighted red?

A

right adrenal gland

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4
Q

what is highlighted red?

A

left adrenal gland

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5
Q

what are the 3 different layers of the adrenal gland?

A
  • Capsule (most outer)
  • Cortex
  • Medulla (most inner)
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6
Q

what layers are in the cortex of the adrenal glands?

A
  • zona glomerulosa
  • zona fasciculata
  • zona reticularis
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7
Q

what regulates the zona glomerulosa?

A
  • Angiotensin II

- K+

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8
Q

what regulates the zona fasciculata?

A

ACTH

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9
Q

what regulates the zona reticularis?

A

ACTH and unknown factors

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10
Q

what does the zona glomerulosa layer of the adrenal gland secrete?

A

mineralocorticoids (e.g. aldosterone)

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11
Q

what does the zona fasciculata in adrenal glands secrete?

A

-glucocorticoids (e.g. cortisol and corticosterone)

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12
Q

what does the zona reticularis in adrenal glands secrete?

A

adrenal androgens (e.g. DHEA and DHEA-sulfate)

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13
Q

what makes up the medulla of the adrenal gland?

A
  • chromaffin cells
  • medullary veins
  • splanchnic nerves
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14
Q

what does the medulla of the adrenal glands secrete?

A

-catecholamines (epinephrine and norepinephrine)

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15
Q

what regulates cortisol and androgen production?

A

-androgen and cortisol production is regulated by hormones produced in the hypothalamus and anterior pituitary gland

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16
Q

what regulates aldosterone?

A

-renin angiotensin system and plasma potassium

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17
Q

do steroid hormones bind extra or intracellularly?

A

intra cellularly

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18
Q

what are the 6 classes of steroid receptors?

A
  • glucocorticoid
  • mineralocorticoid
  • progestin
  • oestrogen
  • androgen
  • vitamin D
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19
Q

what effect does cortisol have on the CNS system?

A
  • mood lability
  • euphoria/ psychosis
  • decrease in libido
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20
Q

what effect does cortisol have on the circulatory/renal system?

A
  • increase in cardiac output
  • increase in blood pressure
  • increase in renal blood flow and GFR
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21
Q

what effect does cortisol have on the metabolic system?

A
  • causes an increase in blood sugar
  • increase in lipolysis
  • increase in proteolysis
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22
Q

what effect does cortisol have on bone/ connective tissue?

A
  • it accelerates osteopersosis
  • decreases calcium
  • decreases collagen formation
  • decreases wound healing
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23
Q

what effect does cortisol have on immunology?

A
  • decreases capillary dilatation/permeability
  • decreases leucocyte migration
  • decreases macrophage activty
  • decreases inflammatory cytokine production
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24
Q

what are the 3 main principles of the clinical use of corticosteroids?

A
  • suppress inflammation
  • suppress immune system
  • replacement treatment
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25
what effect does aldosterone have on sodium/potassium balance?
- it promotes K+/H+ excretion | - increases Na+ reabsorption
26
what is aldosterone responsible for?
- sodium/potassium balance - blood pressure regulation - regulation of extracellular volume
27
where are mineralocorticoid receptors (MR) found?
- kidneys - salivary glands - gut - sweat glands
28
what is the receptor for aldosterone?
-mineral corticoid receptor (MR)
29
what type of hormone is aldosterone?
steroid hormone
30
what is adrenal insufficiency?
-when there is inadequate adrenocortical function
31
what are some examples of primary adrenal insufficiency?
- Addison's disease - congenital adrenal hyperplasia (CAH) - adrenal TB/malignancy
32
what are some examples of secondary adrenal insufficiency?
- lack of ACTH stimulation - iatrogenic (excess exogenous steroid) - pituitary/hypothalamic disorders
33
what is the most common cause of primary adrenal insufficiency?
Addison's disease
34
what is Addison's disease?
-the autoimmune destruction of the adrenal cortex
35
are autoantibodies commonly found in patients with Addison's disease?
yes- autoantibodies are positive in 70% of cases
36
how much of the adrenal cortex is damages in addison's before becoming symptomatic?
>90%
37
what other diseases is Addison's associated with?
-other autoimmune diseases (e.g. T1DM, autoimmune thyroid disease, pernicious anaemia)
38
How does Addison's disease present?
- anorexia/ weight loss - fatigue - dizziness and low BP - abdominal pain, vomiting, diarrhoe - skin pigmentation
39
how is adrenal insufficiency diagnosed?
Bloods test: - decrease in Na, increase in K - hypogylcamia - ACTH levels raised (causes skin pigmentation) - renin raised - aldosterone lower - adrenal autoantibodies SHORT SYNACTHEN TEST - measure plasma cortisol before and after 30 mins after iv/im ACTH injection - normal: baseline > 250 nmol/L, post ACTH >550nmol/L
40
what is the management of adrenal insufficiency?
- hydrocortisone given as cortisol replacement - fludrocortisone as aldosterone replacement need to have a steroid treatment card!
41
how does secondary adrenal insufficiency due to exogenous steroid use present differently compared to Addison's?
- in secondary adrenal insufficiency the skin is pale (as there is no increase in ACTH) - aldosterone production is intact
42
what is the management for secondary adrenal insufficiency due to tumours?
-surgery/ radiotherapy
43
how do you treat secondary adrenal insufficiency due to exogenous steroid use?
-with hydrocortisone replacement
44
what is cushings syndrome?
-a disease that causes excess cortisol secretion
45
who is cushings syndrome more common in?
-women ages 20 to 40
46
what are some clinical features of cortisol excess?
- easy bruising - facial plethora - striae - proximal myopathy
47
what are some ACTH dependant causes of Cushing's syndrome?
- pituitary adenoma (cushings disease) - ectopic ACTH - ectopic CRH
48
what are some ACTH independant causes of Cushing's syndrome?
- adrenal adenoma - adrenal carcinoma - nodular hyperplasia
49
what is the difference between cushings syndrome and cushings disease?
cushings disease is specifically cortisol excess due to a problem with the pituitary whereas cushings syndrome is cortisol excess with a problem outwith of the pituitary
50
how is cushing's syndrome diagnosed?
- overnight dexamethasone suppression test - 24 hour urinary free cortisol - late night salivary cortisol - low dose dexamethasone suppression test repeat to confirm
51
what is the commonest cause of cortisol excess?
-iatrogenic cushing's syndrome
52
what causes iatrogenic cushing's syndrome?
-due to prolonged high dose steroid therapy
53
what is iatrogenic cushing's syndrome?
-the chronic suppression of pituitary ACTH production and adrenal atrophy
54
what effect can long term steroid treatment have on ACTH?
- long term steroid treatment suppresses ACTH production | - this can lead to atrophy of adrenal cortex
55
can steroids be stopped suddenly?
no they cant be stopped suddenly there must be a gradual withdrawal of steroid therapy if >4-6 weeks
56
what are some endocrine causes of hypertension?
- acromegaly - cushings syndrome - prim
57
what would hypertension and hypokalaemia suggest?
-primary aldosteronism
58
what is primary aldosteronism?
-autonomous production of aldosterone independant of its regulators (angiotensin II/potassium)
59
what effects does aldosterona have?
- increase collagen - increase in sympathetic outflow - altered endothelial function and increase in pressor response - increase in cardiac collagen - sodium retention - cytokines and ROS synthesis
60
what is the commonest secondary cause of hypertension?
primary aldosteronism (PA)
61
what are clinical features of primary aldosteronism (PA)?
- significant hypertension - hypokalaemia - alkalosis
62
what are subtypes of primary aldosteronism?
- adrenal adenoma - bilateral adrenal hyperplasia (commonest cause) - genetic mutations and unilateral hyperplasia (rare)
63
How is primary aldosteronism (PA) diagnosed?
Step 1: confirm aldosterone excess - measure plasma aldosterone and renin and express a ration (ARR- aldosterone to renin ratio) - if ratio raised then investigate further with saline suppression test - failure of plasma aldosterone to suppress by >50% with 2 litres of normal saline confirms PA Step 2: confirm subtype - adrenal CT to demonstrate adenoma - sometimes adrenal vein sampling to confirm adenoma is true source of aldosterone excess
64
what is the management of primary aldosteronism if there is an adrenal adenoma?
Surgical | -unilateral laparoscopic adrenalectomy
65
what is the management of primary aldosteronism if there is bilateral adrenal hyperplasia?
MR antagonist (spironolactone or eplerenone)
66
what is congenital adrenal hyperplasia (CAH)?
-congenital adrenal hyperplasia is an inherited group of disorders characterised by a deficiency in one of the enzymes necessary for cortisol synthesis
67
what causes congenital adrenal hyperplasia?
- due to 21 alpha-hydroxylase deficiency | - autosomal recessive
68
what are the 2 types of congenital adrenal hyperplasia (CAH)?
- classic CAH | - non classic CAH
69
when is classic CAH normally diagnosed?
-in infancy
70
what is non- classic CAH due to?
a partial 21alpha- hydroxylase deficiency
71
who does non-classic CAH usually present in?
-in adolescence/ adulthood with hirsutism, mental disturbances, infertility due to anovulation
72
how does classic congenital adrenal hyperplasia present?
- salt wasting | - simple virilising
73
how does non classic congenital adrenal hyperplasia present?
-hyperandrogenaemia
74
what is the diagnosis of congenital adrenal hyperplasia?
- Basal (or stimulated) 17-OH progesterone | - increasingly supported by genetic mutation analysis
75
what is the presentation of classical CAH in male neonates?
adrenal insufficiency: - often seen in the first 2 to 3 weeks - poor weight gain - biochemical pattern of Addison's disease
76
what is the presentation of classical CAH in female neonates?
-genital ambiguity (virilisation)
77
what is the presentation of classical CAH in females?
- hirsute - acne - oligomenorrhoea - precocious puberty - infertility or sub fertility
78
what is the treatment for CAH in children?
- timely recognition - glucocorticoid replacement - mineralocorticoid replacement in some - surgical correction - achieve maximal growth potential
79
what is the treatment for CAH in adults?
- control androgen excess - restore fertility - avoid steroid over replacement
80
what is pheochromocytoma?
-a type of neuroendocrine tumor that grows from cells called chromaffin cells
81
what is a paraganglioma?
-a type of neuroendocrine tumor that forms near certain blood vessels and nerves outside of the adrenal glands
82
what effect does phaeochromocytoma have on blood pressure?
-it increases it causing hypertension
83
what is the presentation of phaeochromocytoma?
Classic triad (90% cases) - hypertension - headache - sweating - palpitations - breathlessness - constipation - anxiety/fear - weight loss - flushing (uncommon)
84
what is the classical triad for phaeochromocytoma?
- hypertension - headache - sweating
85
what are the signs of phaeochromocytoma?
- hypertension - postural hypotension - pallor - bradycardia and tachycardia - pyrexia
86
what are some complications of phaeochromocytoma?
- left ventricular failure - myocardial necrosis - stroke - shock - paralytic ileus of bowel
87
how is phaeochromocytoma diagnosed?
confirm catecholamine excess by : - uring (2 x 24 hour catecholamines or metanephrines) - plasma Then identify the source of catecholamine excess by doing: - MRI scan of the abdomen and whole body - MIBG scan - PET scan
88
what is the treatment for phaeochromocytoma?
Laparoscopic surgery, long-term follow up and genetic testing= gold standard!! (if malignant do chemo) -to prepare patients for surgery they need to be put on medication: Full alpha and beta-blockade (A before B) -phenoxybenzamine (alpha blocker) -propranolol, atenolol or metoprolol (beta blocker)
89
what are some clinical syndrome associations with phaeochromocytoma?
- multiple endocrine neoplasia 2 (MEN2) - Von Hippel Lindau syndrome - succinate dehydrogenase mutations - neurofibromatosis - tuberose sclerosis
90
what are some endocrine causes of hypertension that are curable?
- cushing's syndrome - conn's syndrome - phaeochromocytoma

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