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REGUB Medicine: Pit & Adrenal Disease (ii) (Primary adrenal failure…
REGUB Medicine: Pit & Adrenal Disease (ii)
Primary adrenal failure (Addison's disease)
rare
hallmark = v high ACTH (causes weightloss + pigmentation) despite low adrenal cortical hormones
androgen loss not as severe in men as in women
causes
90% AI
5% TB (used to be commoner)
v rare causes (1%)
granulomas
HFE
sarcoid
Waterhouse Friderichsen syndrome: usually bilat adrenal haemorrhage due to severe bacterial infection (usually N meningitides)
mets
loss of adrenal cortex with low hormones
low cortisol: fatigue, weightloss, poor healing, hypoglyc, hypotension
low aldosterone: hypotension, hyponatriemia, hyperkalaemia, dehydration
low androgens: loss of libido + low QOL (esp women)
RAAS activated due to low blood vol but no aldosterone can be made
Hypercortisolism (Cushing's syndrome)
often iatrogenic
subtypes
ACTH dependent
pit adenoma (Cushing's disease/pit Cushing's)
ectopic ACTH production (SCLC) - low ACTH from pit
ACTH independent (adrenal)
tumours
hyperplasia
multisystem comps
dyslipidaemia
HTN
thrombosis
vasculitis
changed behaviours, cognition, memory, mood (e.g. psychosis)
cerebral atrophy
GI bleeding
pancreatitis
PUD
delayed wound healing
Hypertrichosis (excessive hair growth all over body)
personal dermatitis
petechiae
acne
striae rubrae distensae
telangiectasia
bone necrosis, osteoporosis, retardation of long bone growth
muscle atrophy
cataracts, glaucoma
Na retention, K excretion
delayed puberty, foetal growth retardation
hypogonadism
Dx
Outrule/tx alcoholism/depression if present
24hr urine free cortisol
1mg overnight dexamethasone suppression test
low dose 48hr dexamethasone suppression test
if ATCH decreases then adrenal cause
if ACTH remains high then pit or ectopic ACTH
late night salivary cortisol
Tx
transphenoidal surgery to remove pit adenoma
adrenalectomy (for adrenal cushings/failed pit surgery)
pit radiotherapy
medical tx
metyrapone (glucocorticoid synthesis inhibitor)
ketoconazole (long term ACTH supression)
aminoglutethimide (blocks conversion of chol to pregnenolone -steroid precursor)
RU 486 (mifepristone, blocks cortisol action by binding to glucocorticoid R)