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endocrine > adrenal pathology > Flashcards

adrenal pathology Flashcards

1
Q

what are some examples of primary adrenal insufficiency

A
  • addison’s disease
  • congenital adrenal hyperplasia
  • adrenal TB/malignancy
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2
Q

what are some examples of secondary adrenal insufficiency

A
  • due to lack of ACTH stimulation
  • iatrogenic (excess exogenous steroid)
  • pituitary/hypothalamic disorders
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3
Q

causes of primary adrenal insufficiency

A
  • autoimmune/addison’s disease
  • APS type 1
  • APS type 2
  • infections
  • bilateral adrenal haemorrhage
  • bilateral adrenal metastases
  • bilateral adrenal infiltration
  • bilateral adrenalectomy
  • drug induced
  • genetic disorders
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4
Q

causes of secondary adrenal insufficiency

A
  • pituitary tumours
  • pituitary surgery
  • pituitary irradiation
  • trauma
  • infections
  • pituitary apoplexy
  • sheehan’s syndrome
  • genetic disorders
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5
Q

causes of tertiary adrenal insufficiency

A
  • hypothalamic tumours
  • hypothalamic surgery
  • hypothalamic irradiation
  • infections
  • trauma
  • Cushing’s syndrome
  • drug induced
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6
Q

clinical features of Addison’s disease

A
  • anorexia, weight loss
  • fatigue/lethargy
  • dizziness and low BP
  • abdo pain
  • vomiting
  • diarrhoea
  • skin pigmentation
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7
Q

what is addisons

A

autoimmune destruction of the adrenal cortex

commonest cause of primary adrenal insufficiency

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8
Q

diagnosis of adrenal insufficiency

A

biochemistry

  • decrease Na increase K and hypoglycaemia
  • short synACTHen test
  • ACTH levels should be increased
  • renin should be increased and aldosterone should be decreased
  • adrenal antibodies
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9
Q

management of adrenal insufficiency

A
  • hydrocortisone as cortisol replacement (15-30mg daily)
  • fludrocortisone as aldosterone replacement
  • need education
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10
Q

emergency treatment of adrenal crisis

A
  • IV hydrocortisone
  • IV fluid resuscitation
  • cardiac monitoring
  • careful monitoring of electrolyte fluid balance
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11
Q

sick day rule 1

A

moderate intercurrent illness, surgical procedure under local anaesthetic
double usual daily glucocorticoid use

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12
Q

sick day rule 2

A

severe intercurrent illness, preparation for colonoscopy, acute trauma or surgery

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13
Q

cause of secondary adrenal insufficiency

A
  • pituitary/hypothalamic disease tumours
  • exogenous steroid use
  • genetic
  • pituitary surgery
  • trauma
  • infection
  • sheehan’s syndrome
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14
Q

examples of exogenous steroid use

A
  • high dose prednisolone
  • dexamethasone
  • inhaled corticosteroid
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15
Q

causes of tertiary adrenal insufficiency

A
  • hypothalamic tumours
  • hypothalamic surgery
  • hypothalamic irradiation
  • infections
  • trauma
  • cushing’s
  • drug induced
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16
Q

what is secondary adrenal insufficiency

A

inadequate ACTH stimulating the adrenal glands, resulting in low levels of cortisol being released. This is the result of loss or damage to the pituitary gland.

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17
Q

what is tertiary adrenal insufficiency

A

inadequate CRH release by the hypothalamus. This is usually the result of patients being on long term oral steroids (for more than 3 weeks) causing suppression of the hypothalamus.

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18
Q

difference between addison’s and secondary adrenal insufficiency clinical features

A

secondary:

  • skin pale
  • aldosterone production is intact
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19
Q

what does long term steroid treatment do to ACTH production

A

suppresses it

20
Q

implications of atrophy of adrenal cortex

A
  • unable to respond to stress (illness/surgery)
  • need extra doses of steroid when ill/surgical procedure
  • cannot stop suddenly
  • gradual withdrawal of steroid therapy if >4-6 weeks
21
Q

what is primary aldosteronism

A

autonomous production of aldosterone independent of its regulators

22
Q

what are the cardiovascular actions of aldosterone

A
  • increase cardiac collagen
  • cytokines and ROS synthesis
  • sodium retention
  • altered epithelial function (increased pressor response)
  • increase sympathetic outflow
23
Q

what is the commonest secondary cause of hypertension

A

primary aldosteronism

24
Q

clinical features of aldosteronism

A
  • significant hypertension
  • hypokalaemia
  • alkalosis
25
Q

what are the subtypes of primary aldosteronism

A
  • adrenal adenoma (Conn’s syndrome)
  • bilateral adrenal hyperplasia
  • rare causes (genetic, unilateral hyperplasia)
26
Q

diagnosis of primary aldosteronism

A
  • confirm aldosterone excess by measuring aldosterone/renin ratio and if raised do saline suppression test (failure of aldosterone to suppress by >50% with 2 litres of normal saline confirms PA)
  • confirm subtype - adrenal CT to confirm adenoma
27
Q

management of primary aldosteronism

A

surgical
-unilateral laparoscopic adrenalectomy if adenoma

medical
-MR antagonists (spironolactone or eplerenone)

28
Q

what is congenital adrenal hyperplasia

A

Congenital adrenal hyperplasia is caused by a congenital deficiency of the 21-hydroxylase enzyme. This causes underproduction of cortisol and aldosterone and overproduction of androgens

29
Q

when is classic CAH typically diagnosed

A

infancy

30
Q

what is non-classic CAH

A
  • partial 21alpha-hydroxylase deficiency

- presents in adolescence

31
Q

presentation of classical CAH

A
  • adrenal insufficiency
  • poor weight gain
  • tall for their age
  • genital ambiguity
  • facial hair
  • deep voice
  • absent periods
  • early puberty
32
Q

presentation of non-classical CAH

A
  • hirsute
  • acne
  • oligomenorrhoea
  • precocious puberty
  • infertility or sub-fertility
33
Q

treatment for CAH in children

A
  • glucocorticoid replacement with hydrocortisone
  • aldosterone replacement with fludrocortisone
  • surgical correction
34
Q

treatment for CAH in adults

A
  • control androgen excess
  • restore fertility
  • avoid steroid over-replacement
35
Q

what are some clues to phaechromocytoma

A
  • labile hypertension
  • postural hypertension
  • paroxysmal sweating, headache, pallor, tachycardia
36
Q

what are extra adrenal tumours called

A

paraganlioma

37
Q

symptoms of paraganglioma/phaechomocytoma

A 
HYPERTENSION
HEADACHE
SWEATING
-palpitations
-breathlessness
-constipation
-anxiety
-weight loss
-flushing
38
Q

signs of paraganglioma/phaechromocytoma

A
  • hypertension
  • postural hypotension
  • pallor
  • bradycardia
  • tachycardia
  • pyrexia
39
Q

signs of complications of paraganglioma/paechromocytoma

A
  • left ventricular failure
  • myocardial necrosis
  • stroke
  • shock
  • paralytic ileus of bowel
40
Q

biochemical abnormalities seen in paraganglioma/phaechromocytoma

A
  • hyperglycaemia
  • low potassium
  • high Hb concentration
  • mild hypercalcaemia
  • lactic acidosis
41
Q

who should we investigate for paraganglioma/phaechromocytoma

A
  • family members with syndromes
  • resistant hypertension
  • under 50s with hypertension
  • classical symptoms
  • consider with hypertension and hyperglycaemia
42
Q

what are the main catecholamines

A

adrenaline, noradrenaline and dopamine

43
Q

diagnosis of phaechromocytoma/paraganglioma

A 
confirm catecholamine excess
-urine and plasma
 MRI scan (abdomen, whole body)
MIBG
PET scan
44
Q

preparation for surgery of paraganglioma/phaechromocytoma

A

full alpha and beta blockade

  • phenoxybenzamine for alpha
  • propanolol, atenolol or metoprolol for beta

fluid and/or blood replacement
careful anaesthetic assessment

45
Q

treatment for phaechromocytoma/paraganglioma

A

surgical

  • laparoscopic
  • total excision
  • chemotherapy if malignant
  • radiolabelled MIBG
46
Q

clinical syndrome associated with phaechromocytoma and paraganglioma

A
  • MEN2
  • Von-Hippel-Lindau syndrome
  • succinate dehydrogenase mutations
  • neurofibromatosis
  • tuberose sclerosis

endocrine (40 decks)

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