endocrine diseases-adrenal Flashcards
difference cushing syndrome and cushing disease
cushing syndrome= adrenal gland excess cortisol
cushing disease= pituitary excess ACTH
symptoms of cushing syndrome
anabolic= elevated glucose- DM catabolic= muscle weakness poor wound healing easy brusing infertility stretch marks high cholesterol depression memory loss skin ulcers uncontrolled muscle protein breakdown increased fat osteoporosis unctonrolled appetitie central fat deposition moonface excess mineralocorticoid action= hypertension
2 main types of hypertension
primary=idiopathic
secondary=endocrine, vascular damage and neoplastic disease, aldosteronism
3 causes of primary hyperaldosternoism
conn’s syndrome
bilateral adrenal hyperplasia
Glucocorticoid remediable aldosteronism
what is conn’s syndrome
unilateral adrenal tumour
aldosterone producing adenoma
phenotype of conn’s
high aldosterone high sodium low renin low potassium ECF expansion hypertension
management of conn’s syndrome
surgical
venous sampling
ct scan
unilateral adrenalectomy
what is bilateral adrenal hyperplasia
idiopathic
most common PA 60-70%
phenotype for bilateral adrenal hyperplasia
high aldosterone low renin high sodium low potassium ECF expansion hypertension
treatment for bilateral adrenal hyperplasia
anti-hypertensives
MR blockers
spironalactone
eplernone
what is glucocorticoid remediable aldosteronism
ACTH driven
autosomal dominant genetic disorder
-hybrid gene of unequal meiotic exchange so get 11obhase promoter and AS coding region
- aka driven by ACTH not angiotensin II
phenotype of glucocorticoid remediable aldosteronism
high aldosterone high sodium low potassium low renin ecf expansion hypertension
treatment for glucocoritcoid remediable aldosteronism
suppress pituitary ACTH secretion
-synthetic glucocorticoid dexamethasone
causes of secondary hyperaldosteronism
high renin
- renin secreting JG tumour
- renal arterial stenosis
what is a renin secreting JG tumour
renin hypersecretion
increase RAAS
phenotype for secondary aldosteronism
high renin high aldosterone MR activation high na low k ecf expansion hypertension
treatment for secondary aldosteronism
surgical removal of tumour
what is renal arterial stenosis
low perfusion pressure so get increased renin
secretion
cycle: vasoconstriction increased stenosis
treatment for renal arterial stenosis
anti-hbp MR blockers statins anti-platelets balloon angioplasty stent
excess cortisol 2 types
cushing syndrome= adrenal problem
cushing disease=pituitary tumour
phenotype for cushing’s
high cortisol low k high na low aldosterone and renin high bp
cushing syndrome versus cushing disease phenotype
cushing syndrome=low plasma ACTH high cortisol
cushing disease= high acth and cortisol
treatment for cushing disease plus 3 main drugs
- trans-sphenoidal hypophysectomy
- adrenalectomy
- pituitary radiotherapy
- adrenal synthesis inhibitors: metyrapone and ketoconazole
- somatostatin analogue: pasireotide
treatment for cushing syndrome
stop steroids
remove tumour
radiotherapy tumour
metabolic syndrome and link to 11bHSD
11bhsd type 1 found in fat, liver and brain
increased in obesity
activates cortisol
11bhsd inhibitors function
improve hba1c and reduce cortisol improving memory
how does cortisol elevate hypertension
- inhibits vascular nitric oxide production from arginine by eNOS (nitric oxide synthase)
- potentiate catecholamine action increasing Cardiac output, resistance and adrenaline
- activate the kidney MR normally prevented by converting to cortisone- increase NA pull
causes of glucocorticoid hyperactivity 6
- cushing syndrome
- cushing disease
- apparent mineralocorticoid excess
- liquorice ingestion and drugs
- CAH 11obhase deficiency
- Pheocromrocytoma
what is apparent mineralocorticoid excess
autosomal recessive loss of function in 11bhsd2
-loss of conversion of cortisol to cortisone
phenotype apparent mineralocorticoid excess
high Na Ecf expansion low k low renin low aldosterone severe childhood hypertension
treatment for apparent mineralocorticoid excess
MR antagonists
spironalactone
eplerone
low sodium and k+ diet
what causes hypertension from liquorice ingestion
glycyrrhizinc acid
and carbenoxolone
inhibitors of 11bhsd2
phenotype liquorice ingested hypertension
high sodium
low potassium, renin, aldosterone
episodic HBP
what is congenital adrenal hyperplasia 11obhase deficiency
blocks in ZF formation of cortisol
how does 11b ohase cause hypertension
decrease cortisol
increase ACTH as decrease feedback so increase substrates DOC and deocortisol that act weakly at MR increase receptors
what type of gene disorder are the CAH
autosomal recessive
what does CAH 21 ohase cause
increased ACTH causes hyperplasia
- enlarged adrenals
- steroidogenesis towards androgens= virilisation
- low sodium
- low blood p
- high renin and potassium
what is pheochromocytoma
catecholaime secreting tumour of adrenal medulla
chromaffin cell tumour secretes adrenaline
phenotype of pheochromocytoma- increase catecholamies adrenaline
increase glucagon decrease insulin increase HR vasoconstriction increase glycogen and lipid breakdown peripheral resistance
treatment for pheochromoctyoma
alpha and beta blockers
what MEN syndrome is pheochromocytoma
MEN 2b
multiple endocrine neoplasia
what genetic inheritance is MEN
autosomal dominant causes overactivity and enlargement of some endocrine glands leading to hormonal changes
symptoms of pheochromocytoma
PHE palpitation headache episodic swelling hypertension DM
how much does endocrine hypertension count for hypertension
10-15%
testing for cushing’s disease
overnight dexamethasone suppression test
-give glucocorticoid dexamethasone as should negative feedback ACTH
-low dose suppress pituitary ACTH in normal
-high dose suppress in pituitary adenoma but not ectopic adenoma ACTH
<50
what is the test for Addison’s disease
synACTHen stimulation test
-give ACTH
if addison’s disease then won’t respond to ACTH <430
tests for acromegaly
OGTT and GH will NOT be suppressed
-glucagon stimulation test
conn’s syndrome test
saline suppression test
difference between primary and secondary adrenal insufficiency and their phenotype
primary=lack of steroids and MR due to adrenal-Addison’s disease
secondary= hypopituitarism lack glucocorticoids but normal mineralocorticoid
causes Addison’s disease
- autoimmune 80%
- infection HIV or TB
- metastases
- amyloid
- haemorrhage waterhouse-friederichsen
- congenital
- surgery
5t’s of addison’s disease
tired tears tummy thin tanned
symptoms of primary adrenal insufficiency
weakness tired anorexia nausea and vomiting weight loss hyperpigmentation postural hypotension vitiligo and other autoimmune conditions
symptoms of secondary adrenal insufficiency
weak, tired anorexia nausea and vomiting hypopigmentation weight loss postural hypotension other hypopituritarism signs
treatment for adrenal insufficiency emergencey
IV access 2-3 litre saline
hydrocorisone 100mg IV 6hrly
treatment maintenance for adrenal insufficiency
hydrocortisone 10-15mg on waking
5-10mg in evening
fludrocortisone 0.95-0.1mg
sick day rules for adrenal insufficiency
double your dose for 2 days
what is an incidentaloma
tumour that is found incidentally on CT when investigating for other
