Adrenal Disorders Flashcards
where are corticosteroids synthesised?
adrenal cortex
examples of corticosteroids
mineralcorticoids (aldosterone) glucocorticoids (cortisol) sex steroids (androgens, oestrogen)
what is the precursor of a steroid?
cholesterol
what is the effect of angiotensin II on the adrenals?
activates:
3-hydroxysteroid dehydrogenase
11, 21, 18-hydroxylase
what is the effect of ACTH on the adrenals?
activates: 3-hydroxysteroid dehydrogenase
11, 21 and 17-hydroxylase
what is addisons disease?
primary adrenal failure
what is the most common cause of Addisons worldwide?
tuberculosis of the adrenal glands
what is the most common cause of Addisons in the UK?
autoimmune destruction of the adrenal glands
what are the main signs of Addisons disease?
skin and mucous membrane pigmentation
weight loss
vomiting, diarrhoea
low blood pressure
what are the physiological consequences of addisons?
low BP loss of salt in urine increased plasma potassium low blood glucose increased skin pigmentation
why does high ACTH lead to increased pigmentation?
POM-C is cleaved to form ACTH and MSH (and endorphins) so pathologically high ACTH may lead to tanned skin
what tests should be performed if Addisons is suspected?
9am cortisol
short synACTHen test
what is a short synACTHen test?
give 250ug synACTHen IM
measure cortisol response (should increase)
how is Addisons treated?
fludrocortisone 50-100mg
and either hydrocortisone 3x daily(10, 5, 2.5mg) OR prednisolone 3mg daily
what is the most common congenital adrenal hyperplasia?
21-hydroxylase deficiency
what is complete 21-hydroxylase deficiency?
missing 21-hydroxylase from birth
what hormones are absent in complete 21-hydroxylase deficiency?
aldosterone and cortisol
which hormones are in EXCESS in complete 21-hydroxylase deficiency?
sex steroids and testosterone
what are the signs of someone with complete 21-hydroxylase deficiency?
neonate with salt-losing addisonian crisis
what do girls with complete 21-hydroxylase deficiency develop?
ambiguous genitalia, virilised by adrenal testosterone (males usually okay)
how do partial and complete 21-hydroxylase deficiency present differently?
partial may present much later in life as is not fatal without treatment. males may present with precocious puberty and females with hirsutism and virilisation
what is virilisation?
development of male physical characteristics in females/precocious boys
what hormones are deficient in 11-hydroxylase deficiency?
cortisol and aldosterone
what hormones are in excess in 11-hydroxylase deficiency?
sex steroids - testosterone
11-deoxycortisterone