Case 1 - Adrenal crisis and Adrenal glands booklet Flashcards
Presentation of adrenal crisis
- Use of long term steroids - abruptly stopped due to V/D
- Fatigue
- Weakness
- Hypotensive
- Tachycardia
- Unwell appearance
Investigation findings of adrenal crisis
- Hyponatraemia
- Hyperkalaemia
- Raised urea
- Hypoglycaemia
- Mild anaemia
Immediate management of adrenal crisis
- 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
Advice for patient to prevent similar future episodes of crisis
- 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
Evidence pts can keep to let people know they are on long term steroids
- Steroid card
- Emergency crisis letter
- Medic Alert - can call helpline 24/7 and let any professionals access full medical records
What is produced in cortex?
- Glucocorticoids
- Androgens
- Mineralcorticoids
Glucocorticoids -main one
Cortisol
When is cortisol highest and lowest?
Higest 8am
Lowest midnight
What is most cortisol bound to?
- Cortisol binding globulin (CBG)
- Albumin
- Only a small amount is active free form
What do cortisol immunoassays measure?
- 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
How is cortisol controlled?
ACTH
How are androgens controlled?
ACTH
Important roles of androgens in…
- Adult women
- Both sexes pre-puberty
- Adult men mainly rely on testicular production of androgens
Conversion of androgens
DHEA, DHEA-S and androstenedione converted to more potesmt testosterone and dihydrotestosterone in peripheral tissues
Where do androgens exert effects?
- Sebaceous glands
- Hair follicles
- Prostate gland
- External genitalia
Main mineralocorticoid
Aldosterone
Regulation of aldosterone
Via RAAS
When is renin activated?
- Low blood volume
- Hyponatraemia
- Hyperkalaemia
Where does aldosterone act and what does it cause?
- Renal distal convoluted tubule
- Sodium retention
- Potassium excretion
Adrenal medulla - what does it do?
- Tissue made up of sympathetic nervous system
- Secretes adrenaline, noradrenaline, dopamine and metabolites (metanephrines, nor-metanephrines etc)
What is primary adrenal insufficiency (Addisons disease)?
- Destruction of adrenal gland or genetic defects in steroids syntheisis
- All three zones cortex often affected
Clinical features addisons
- Fatigue
- Weakness
- Anorexia
- Weight loss
- Nausea
- Abdominal pain
- Dizziness
VAGUE AND NON SPECIFIC
What does glucocorticoid loss lead to in Addisons?
- Hypoglycaemia
- Increased pigmentation due to increased ACTH from reduced cortisol negative feedback
What can androgen deficiency in women cause?
- Reduced libido
- Loss axillary and pubic hair
Addison crisis - what is it
MEdical emergency
Need IV fluids and IV hydrocortisone
Commonest cause of addisons
Autoimmune
This is tested for by detection of adrenal autoantibodies
Biochemical markers for Addisons
- Hyponatraemia
- Hyperkalaemia
- Raised urea
- Hypoglycaemia
- Mild anaemia
Confirmatory test for Addisons
- 9am cortisol shown to be low with raised ACTH
- Synacthen test needed for confirmation
Management for addisons
- Lifelong glucocorticoid and mineralocorticoid replacement therapy
- Hydrocortisone first choice
- Mineralocorticoid replacement is given as Fludrocortisone
Illness steroid rules
Double dose of gluccocorticoids in illness
Continue on double dose until resolved
What delivery may steroids need to be given in illness?
IM or IV if surgery or prolonged V/D
Alert mechanisms for those on long term steroid therpay
- Steroid card
- Medical alert jewellery
- Emergency contacts for endocrine team
What can cause secondary adrenal insuffiency (ACTH deficiency)?
Hypopituitarism
Treatment secondary adrenal insufficiency
- Hydrocortisone
- But do not need fludrocortisone
What can long term steroid use cause?
ACTH supression - patients need to be advised to not stop steroids abruptly
What are phaechromocytomas and paragangliomas?
Catecholamine secreting tumours
Occur in 0.1% of people with HTN
Where do phaechromocytomas and paragangliomas arise from?
90% adrenal medulla
other 10% arise from extra adrenal chromaffin tissue (these are called paragangliomas)
Familial aspect of phaechromocytomas and paragangliomas
In 30% of patients
Esp in bilateral, extradadrenal or malignat tumours
Clinical features of phaechromocytomas and paragangliomas
- Headache
- Swelling
- Pallor
- Palpitations
- Anxiety/panic attacks
- HTN
What can phaechromocytomas lead to if untreated?
- Hypertensive crisis
- Encephalopathy
- Hyperglycaemia
- Pulmonary oedema
- Cardiac arrhythmias
- Death
Diagnosis of phaechromocytomas and paragangliomas
- 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
Option if tumour is not located on MRI
MIBG - I-meta-lodobenzylguanidine
When should we genetically test someone with phaechromocytomas and paragangliomas
- 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
Management for phaechromocytomas and paragangliomas?
Surgical excision
Laparoscopically or open
What may some patients need at diagnosis of phaechromocytomas and paragangliomas
- 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)
What can beta blockers be used to control in phaechromocytomas and paragangliomas
Can control reflex tachycardia
