Chem Path Flashcards
Functions of cortisol
- antagonises insulin
- causes proteolysis (prod of amino acids)
- anti-inflam
- suppresses immune system
- antagonises vit D to reduce calcium absorption
Functions of aldosterone
- promotes sodium and water retention
- k and h excretion
Hormones produced by the cortex of the adrenal gland
Corticosteroids
Glomerulosa - mineralocorticoids - aldosterone
Fasiculata - glucocorticoids - cortisol
Reticularis - androgens - DHEA
Hormones produced by adrenal medulla
Catecholamines (E and NE)
Describe steroid synthesis
- produced from cholesterol
- rate limiting step = chol side-chain cleaved to pregnenolone (stim by ACTH)
- hydroxylations require cyt-p450 in mitochondria
- 17 and 21 hydroxylations occur in sER
Why is aldosterone not affected by decrease in ACTH?
Stimulated mainly be ang2 and k
Characteristics of CBG
- made by liver
- high affinity
- low capacity
Characteristics of albumin
- low affinity
- high capacity
Why does aldosterone have a shorter half life.
Not bound to plasma protein
3 catecholamine hormones
- dopamine
- epinephrine
- norepinephrine
Action of alpha adrenergic receptors
- vasoconstriction
- decreased insulin release
- sweating
- glycogenolysis
Action of beta adrenergic receptors
- vasodilation
- increased HR
- intestinal smooth muscle relaxation
- bronchodilation
Synthesis of catecholamines
- from phenylalanine
- rate limiting. = tyrosine to DOPA
- hormones in adrenal medulla are complexed to chromogranin proteins in granules
- nerve stim causes granule release
How are catecholamines degraded?
- catechol-O-methyl transferase (COMT) - methylation
- mono amine oxidase (MOA) - deamination
Pattern of inheritance of CAH
Autosomal recessive inborn errors of steroid hormone biosynthesis
Most common defect in CAH
21 hydroxylase
Mechanism of 21 hydroxylase deficiency
- can’t synthesize cortisol so increased ACTH (adrenal hyperplasia)
- accumulation of 17- hydroxyprogesterone (shunting to androgen synthesis)
- elevated 17OHP in serum confirms
3 forms of CAH
- simple virilising
- severe salt-losing
- non-classical
Characteristics of simple virilising form
- mild enzyme defect
- adequate cortisol and aldosterone production
Characteristics of sever salt-losing form
- severe enzyme defect
- potential adrenal crisis due to inadequate aldosterone
- hypotension, shock, hyperkalaemia
- boys more at risk
Characteristics of non classical form
- very mild defect
- no neonatal masculinization
- young women present with menstrual irregularities, infertility and hisuitism
Treatment of CAH
- cortisol and fludrocortisone
- monitor by measuring 17OHP and androgens
Characteristics of 11hydroxylase deficiency
- accumulated 11 deoxycortixosterone has mineralocorticoids activity
- salt retaining
- hypertension and hypokalaemia
- ambiguous genitalia
Diagnosis of CAH
- elevated serum 17OHP or 11DOC
- decreased serum cortisol
- elevated serum ACTH
- elevated serum testosterone
- karyotyping
Definition of primary Addison’s disease
- impaired capacity to secrete cortisol and aldosterone
Definition of secondary Addison’s disease
- lack of ACTH
- cortisol deficiency
- intact aldosterone e
Features of an addisonian crisis
- hypotension
- circulatory shock
- abdominal pain
Clinical features of Addison’s
- fatigue
- muscle weakness
- orthostatic hypertension
- pigmentation (excess ACTH)