Adrenal Pathology Flashcards
What are the 2 parts of the adrenal glands and what do each produce?
Outer cortex and inner medulla
Cortex: cortisol, androgens + aldosterone
Medulla: catecholamines
Where are the adrenal glands found?
Bilaterally superior to kidneys
What is the precursor to hormones produced by the adrenal cortex and what class are these?
Cholesterol
Steroid hormones
What is the cause of Addisons disease?
Autoimmune adrenal insufficiency - lack of Cortisol
Who is the most likely presenting group of Addisons disease?
Young women
How does Addisons present?
Anorexia and weight loss, fatigue, skin hyperpigmentation (particularly in creases in hands), abdominal pain, vomiting, diarrhoea
What are the biochemical findings in Addisons disease?
Low Na, High K, hypoglycaemia
What test is used to diagnose Addisons disease and what are the results?
Synacthen:
If cortisol same after ACTH = Addisons
How is Addisons treated?
Hydrocortisone + fluids
What is given if patients are aldosterone deficient?
Fludrocortisone
What is congenital adrenal hyperplasia and what causes it?
Autosomal recessive genetic condition
Enzyme defect in steroid pathway
How does congenital adrenal hyperplasia present in males and females?
Males: Adrenal insufficiency (2-3 weeks), Poor weight gain, Biochemical pattern of Addisons (low Na, high K, hypoglycaemia)
Females:
Genital ambiguity
How is congenital adrenal hyperplasia treated?
Acute: Hydrocortisone + fluids
Glucocorticoid and mineralocortiocoid (which will suppress androgens)
What causes Cushings syndrome?
Excess cortisol, mineralocorticoid and androgens
What is Cushings syndrome compared to Cushings disease?
Disease: pituitary cause of excess cortisol
Syndrome: any other cause (adrenal adenoma, ectopic ACTH, pseudocushings)
What is the clinical presentation of Cushings syndrome?
Thin skin, proximal myopathy, buffalo hump, central obesity (on thin legs), marked striae, often have associated diabetes
How is Cushings syndrome screened for a diagnosed?
Screening: overnight dexamethasone suppression test (should switch off cortisol production)
Diagnosis: low dose dexamethasone suppression test (if cortisol remains high = Cushings)
How is Cushings disease identified from Cushings syndrome?
High dose dexamethasone suppression test
How is Cushings treated?
If pituitary - Surgery
Adrenal - Surgery
Ectopic - remove source
What is Conns syndrome?
Primary aldosteronism due to adrenal adenoma
How does Conns syndrome present?
Resistant hypertension (Increased aldosterone = Na retention + K loss = hypertension),
Hypokalaemia,
Hypernatraemia,
Alkalosis
How is conns syndrome diagnosed?
Measure aldosterone + renin and express as ratio (ARR) Saline suppression test (large dose of saline should suppress aldosterone and renin - if not = primary aldosteronism) Adrenal CT (to confirm adenoma = cause) - must also have biochemical diagnosis as can have many benign nodules in adrenal gland
How is Conns syndrome (primary aldosteronism from adrenal adenoma) treated?
If young = surgery
If older = spironolactone
What is a phaeochromocytoma?
Tumour in adrenal medulla or extra adrenal (in sympathetic chain)