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Endocrine > adrenal gland disorders > Flashcards

adrenal gland disorders Flashcards

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
Q

what effect does aldosterone have on sodium/potassium balance?

A
  • it promotes K+/H+ excretion

- increases Na+ reabsorption

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26
Q

what is aldosterone responsible for?

A
  • sodium/potassium balance
  • blood pressure regulation
  • regulation of extracellular volume
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27
Q

where are mineralocorticoid receptors (MR) found?

A
  • kidneys
  • salivary glands
  • gut
  • sweat glands
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28
Q

what is the receptor for aldosterone?

A

-mineral corticoid receptor (MR)

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29
Q

what type of hormone is aldosterone?

A

steroid hormone

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30
Q

what is adrenal insufficiency?

A

-when there is inadequate adrenocortical function

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31
Q

what are some examples of primary adrenal insufficiency?

A
  • Addison’s disease
  • congenital adrenal hyperplasia (CAH)
  • adrenal TB/malignancy
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32
Q

what are some examples of secondary adrenal insufficiency?

A
  • lack of ACTH stimulation
  • iatrogenic (excess exogenous steroid)
  • pituitary/hypothalamic disorders
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33
Q

what is the most common cause of primary adrenal insufficiency?

A

Addison’s disease

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34
Q

what is Addison’s disease?

A

-the autoimmune destruction of the adrenal cortex

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35
Q

are autoantibodies commonly found in patients with Addison’s disease?

A

yes- autoantibodies are positive in 70% of cases

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36
Q

how much of the adrenal cortex is damages in addison’s before becoming symptomatic?

A

> 90%

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37
Q

what other diseases is Addison’s associated with?

A

-other autoimmune diseases (e.g. T1DM, autoimmune thyroid disease, pernicious anaemia)

38
Q

How does Addison’s disease present?

A
  • anorexia/ weight loss
  • fatigue
  • dizziness and low BP
  • abdominal pain, vomiting, diarrhoe
  • skin pigmentation
39
Q

how is adrenal insufficiency diagnosed?

A

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
Q

what is the management of adrenal insufficiency?

A
  • hydrocortisone given as cortisol replacement
  • fludrocortisone as aldosterone replacement

need to have a steroid treatment card!

41
Q

how does secondary adrenal insufficiency due to exogenous steroid use present differently compared to Addison’s?

A
  • in secondary adrenal insufficiency the skin is pale (as there is no increase in ACTH)
  • aldosterone production is intact
42
Q

what is the management for secondary adrenal insufficiency due to tumours?

A

-surgery/ radiotherapy

43
Q

how do you treat secondary adrenal insufficiency due to exogenous steroid use?

A

-with hydrocortisone replacement

44
Q

what is cushings syndrome?

A

-a disease that causes excess cortisol secretion

45
Q

who is cushings syndrome more common in?

A

-women ages 20 to 40

46
Q

what are some clinical features of cortisol excess?

A
  • easy bruising
  • facial plethora
  • striae
  • proximal myopathy
47
Q

what are some ACTH dependant causes of Cushing’s syndrome?

A
  • pituitary adenoma (cushings disease)
  • ectopic ACTH
  • ectopic CRH
48
Q

what are some ACTH independant causes of Cushing’s syndrome?

A
  • adrenal adenoma
  • adrenal carcinoma
  • nodular hyperplasia
49
Q

what is the difference between cushings syndrome and cushings disease?

A

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
Q

how is cushing’s syndrome diagnosed?

A
  • overnight dexamethasone suppression test
  • 24 hour urinary free cortisol
  • late night salivary cortisol
  • low dose dexamethasone suppression test

repeat to confirm

51
Q

what is the commonest cause of cortisol excess?

A

-iatrogenic cushing’s syndrome

52
Q

what causes iatrogenic cushing’s syndrome?

A

-due to prolonged high dose steroid therapy

53
Q

what is iatrogenic cushing’s syndrome?

A

-the chronic suppression of pituitary ACTH production and adrenal atrophy

54
Q

what effect can long term steroid treatment have on ACTH?

A
  • long term steroid treatment suppresses ACTH production

- this can lead to atrophy of adrenal cortex

55
Q

can steroids be stopped suddenly?

A

no they cant be stopped suddenly there must be a gradual withdrawal of steroid therapy if >4-6 weeks

56
Q

what are some endocrine causes of hypertension?

A
  • acromegaly
  • cushings syndrome
  • prim
57
Q

what would hypertension and hypokalaemia suggest?

A

-primary aldosteronism

58
Q

what is primary aldosteronism?

A

-autonomous production of aldosterone independant of its regulators (angiotensin II/potassium)

59
Q

what effects does aldosterona have?

A
  • 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
Q

what is the commonest secondary cause of hypertension?

A

primary aldosteronism (PA)

61
Q

what are clinical features of primary aldosteronism (PA)?

A
  • significant hypertension
  • hypokalaemia
  • alkalosis
62
Q

what are subtypes of primary aldosteronism?

A
  • adrenal adenoma
  • bilateral adrenal hyperplasia (commonest cause)
  • genetic mutations and unilateral hyperplasia (rare)
63
Q

How is primary aldosteronism (PA) diagnosed?

A

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
Q

what is the management of primary aldosteronism if there is an adrenal adenoma?

A

Surgical

-unilateral laparoscopic adrenalectomy

65
Q

what is the management of primary aldosteronism if there is bilateral adrenal hyperplasia?

A

MR antagonist (spironolactone or eplerenone)

66
Q

what is congenital adrenal hyperplasia (CAH)?

A

-congenital adrenal hyperplasia is an inherited group of disorders characterised by a deficiency in one of the enzymes necessary for cortisol synthesis

67
Q

what causes congenital adrenal hyperplasia?

A
  • due to 21 alpha-hydroxylase deficiency

- autosomal recessive

68
Q

what are the 2 types of congenital adrenal hyperplasia (CAH)?

A
  • classic CAH

- non classic CAH

69
Q

when is classic CAH normally diagnosed?

A

-in infancy

70
Q

what is non- classic CAH due to?

A

a partial 21alpha- hydroxylase deficiency

71
Q

who does non-classic CAH usually present in?

A

-in adolescence/ adulthood with hirsutism, mental disturbances, infertility due to anovulation

72
Q

how does classic congenital adrenal hyperplasia present?

A
  • salt wasting

- simple virilising

73
Q

how does non classic congenital adrenal hyperplasia present?

A

-hyperandrogenaemia

74
Q

what is the diagnosis of congenital adrenal hyperplasia?

A
  • Basal (or stimulated) 17-OH progesterone

- increasingly supported by genetic mutation analysis

75
Q

what is the presentation of classical CAH in male neonates?

A

adrenal insufficiency:

  • often seen in the first 2 to 3 weeks
  • poor weight gain
  • biochemical pattern of Addison’s disease
76
Q

what is the presentation of classical CAH in female neonates?

A

-genital ambiguity (virilisation)

77
Q

what is the presentation of classical CAH in females?

A
  • hirsute
  • acne
  • oligomenorrhoea
  • precocious puberty
  • infertility or sub fertility
78
Q

what is the treatment for CAH in children?

A
  • timely recognition
  • glucocorticoid replacement
  • mineralocorticoid replacement in some
  • surgical correction
  • achieve maximal growth potential
79
Q

what is the treatment for CAH in adults?

A
  • control androgen excess
  • restore fertility
  • avoid steroid over replacement
80
Q

what is pheochromocytoma?

A

-a type of neuroendocrine tumor that grows from cells called chromaffin cells

81
Q

what is a paraganglioma?

A

-a type of neuroendocrine tumor that forms near certain blood vessels and nerves outside of the adrenal glands

82
Q

what effect does phaeochromocytoma have on blood pressure?

A

-it increases it causing hypertension

83
Q

what is the presentation of phaeochromocytoma?

A

Classic triad (90% cases)

  • hypertension
  • headache
  • sweating
  • palpitations
  • breathlessness
  • constipation
  • anxiety/fear
  • weight loss
  • flushing (uncommon)
84
Q

what is the classical triad for phaeochromocytoma?

A
  • hypertension
  • headache
  • sweating
85
Q

what are the signs of phaeochromocytoma?

A
  • hypertension
  • postural hypotension
  • pallor
  • bradycardia and tachycardia
  • pyrexia
86
Q

what are some complications of phaeochromocytoma?

A
  • left ventricular failure
  • myocardial necrosis
  • stroke
  • shock
  • paralytic ileus of bowel
87
Q

how is phaeochromocytoma diagnosed?

A

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
Q

what is the treatment for phaeochromocytoma?

A

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
Q

what are some clinical syndrome associations with phaeochromocytoma?

A
  • multiple endocrine neoplasia 2 (MEN2)
  • Von Hippel Lindau syndrome
  • succinate dehydrogenase mutations
  • neurofibromatosis
  • tuberose sclerosis
90
Q

what are some endocrine causes of hypertension that are curable?

A
  • cushing’s syndrome
  • conn’s syndrome
  • phaeochromocytoma

Endocrine (53 decks)

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