Case 1 - Adrenal crisis and Adrenal glands booklet Flashcards

1
Q

Presentation of adrenal crisis

A
  • Use of long term steroids - abruptly stopped due to V/D
  • Fatigue
  • Weakness
  • Hypotensive
  • Tachycardia
  • Unwell appearance
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2
Q

Investigation findings of adrenal crisis

A
  • Hyponatraemia
  • Hyperkalaemia
  • Raised urea
  • Hypoglycaemia
  • Mild anaemia
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3
Q

Immediate management of adrenal crisis

A
  • IV/IM hydrocortisone 100mg (then 200mg every 24hrs diluted in 5% dextrose, or 50mg every 6hrs, 100mg in severe obese)
  • IV fluids
  • Correct hypoglycaemia - IV dextrose
  • Monitor electrolytes and fluid balance
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4
Q

Advice for patient to prevent similar future episodes of crisis

A
  • Inform healthcare professionals early on if problem with taking steroids
  • Emergency numbers for endocrinology team
  • Ensure pt knows how to adminuter IM hydrocortisone in emergency
  • Steroid sick day rules - dose needs increasing if feverish, V/D
  • Signs of crisis
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5
Q

Evidence pts can keep to let people know they are on long term steroids

A
  • Steroid card
  • Emergency crisis letter
  • Medic Alert - can call helpline 24/7 and let any professionals access full medical records
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6
Q

What is produced in cortex?

A
  • Glucocorticoids
  • Androgens
  • Mineralcorticoids
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7
Q

Glucocorticoids -main one

A

Cortisol

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8
Q

When is cortisol highest and lowest?

A

Higest 8am
Lowest midnight

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9
Q

What is most cortisol bound to?

A
  • Cortisol binding globulin (CBG)
  • Albumin
  • Only a small amount is active free form
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10
Q

What do cortisol immunoassays measure?

A
  • Total cortisol - bound and free
  • So some conditions eg oestrogen therapy can stimulate CBG levels but may not affect biologically active free levels of cortisol
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11
Q

How is cortisol controlled?

A

ACTH

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12
Q

How are androgens controlled?

A

ACTH

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13
Q

Important roles of androgens in…

A
  • Adult women
  • Both sexes pre-puberty
  • Adult men mainly rely on testicular production of androgens
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14
Q

Conversion of androgens

A

DHEA, DHEA-S and androstenedione converted to more potesmt testosterone and dihydrotestosterone in peripheral tissues

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15
Q

Where do androgens exert effects?

A
  • Sebaceous glands
  • Hair follicles
  • Prostate gland
  • External genitalia
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16
Q

Main mineralocorticoid

A

Aldosterone

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17
Q

Regulation of aldosterone

A

Via RAAS

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18
Q

When is renin activated?

A
  • Low blood volume
  • Hyponatraemia
  • Hyperkalaemia
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19
Q

Where does aldosterone act and what does it cause?

A
  • Renal distal convoluted tubule
  • Sodium retention
  • Potassium excretion
20
Q

Adrenal medulla - what does it do?

A
  • Tissue made up of sympathetic nervous system
  • Secretes adrenaline, noradrenaline, dopamine and metabolites (metanephrines, nor-metanephrines etc)
21
Q

What is primary adrenal insufficiency (Addisons disease)?

A
  • Destruction of adrenal gland or genetic defects in steroids syntheisis
  • All three zones cortex often affected
22
Q

Clinical features addisons

A
  • Fatigue
  • Weakness
  • Anorexia
  • Weight loss
  • Nausea
  • Abdominal pain
  • Dizziness

VAGUE AND NON SPECIFIC

23
Q

What does glucocorticoid loss lead to in Addisons?

A
  • Hypoglycaemia
  • Increased pigmentation due to increased ACTH from reduced cortisol negative feedback
24
Q

What can androgen deficiency in women cause?

A
  • Reduced libido
  • Loss axillary and pubic hair
25
Q

Addison crisis - what is it

A

MEdical emergency
Need IV fluids and IV hydrocortisone

26
Q

Commonest cause of addisons

A

Autoimmune
This is tested for by detection of adrenal autoantibodies

27
Q

Biochemical markers for Addisons

A
  • Hyponatraemia
  • Hyperkalaemia
  • Raised urea
  • Hypoglycaemia
  • Mild anaemia
28
Q

Confirmatory test for Addisons

A
  • 9am cortisol shown to be low with raised ACTH
  • Synacthen test needed for confirmation
29
Q

Management for addisons

A
  • Lifelong glucocorticoid and mineralocorticoid replacement therapy
  • Hydrocortisone first choice
  • Mineralocorticoid replacement is given as Fludrocortisone
30
Q

Illness steroid rules

A

Double dose of gluccocorticoids in illness
Continue on double dose until resolved

31
Q

What delivery may steroids need to be given in illness?

A

IM or IV if surgery or prolonged V/D

32
Q

Alert mechanisms for those on long term steroid therpay

A
  • Steroid card
  • Medical alert jewellery
  • Emergency contacts for endocrine team
33
Q

What can cause secondary adrenal insuffiency (ACTH deficiency)?

A

Hypopituitarism

34
Q

Treatment secondary adrenal insufficiency

A
  • Hydrocortisone
  • But do not need fludrocortisone
35
Q

What can long term steroid use cause?

A

ACTH supression - patients need to be advised to not stop steroids abruptly

36
Q

What are phaechromocytomas and paragangliomas?

A

Catecholamine secreting tumours
Occur in 0.1% of people with HTN

37
Q

Where do phaechromocytomas and paragangliomas arise from?

A

90% adrenal medulla
other 10% arise from extra adrenal chromaffin tissue (these are called paragangliomas)

38
Q

Familial aspect of phaechromocytomas and paragangliomas

A

In 30% of patients
Esp in bilateral, extradadrenal or malignat tumours

39
Q

Clinical features of phaechromocytomas and paragangliomas

A
  • Headache
  • Swelling
  • Pallor
  • Palpitations
  • Anxiety/panic attacks
  • HTN
40
Q

What can phaechromocytomas lead to if untreated?

A
  • Hypertensive crisis
  • Encephalopathy
  • Hyperglycaemia
  • Pulmonary oedema
  • Cardiac arrhythmias
  • Death
41
Q

Diagnosis of phaechromocytomas and paragangliomas

A
  • Elevated catecholamins or their metabolites (metanephrines) using 24 urine catecholamines and plasma metanephrines
  • Radiological localisation of tumour via CT/MRI abdomen

Whole body MRI if not localised

42
Q

Option if tumour is not located on MRI

A

MIBG - I-meta-lodobenzylguanidine

43
Q

When should we genetically test someone with phaechromocytomas and paragangliomas

A
  • Young age presentation
  • Multifocal, malignant or extra-adrenal disease
  • Identification of mutation should lead to annual screening for new/recurrent disease and genetic testing for first degree relatives
44
Q

Management for phaechromocytomas and paragangliomas?

A

Surgical excision
Laparoscopically or open

45
Q

What may some patients need at diagnosis of phaechromocytomas and paragangliomas

A
  • Alpha or beta blockade
  • If alpha - phenoxybenzamine used (sometimes doxazosin)
  • This should be done before beta blockade to avoid unapposed alpha adrenergic stimulation and risk of hypertensive crisis (if have beta blocker before alpha, no beta 2 vasodilation = htn crisis)
46
Q

What can beta blockers be used to control in phaechromocytomas and paragangliomas

A

Can control reflex tachycardia

47
Q
A