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Session 2 CM endo3

CM- Endo -3- Adrenal Disease lect 7-8

From what fetal origin is the adrenal cortex mesodermal origin
During fetal development the adrenal cortex only has two zones what are they and what do they produce fetal zone- produces mainly dehydroepiandrosterone (precursor for estrogen) this zone disappears after birth Definitive Zone- synthesizes fetal cortisol and develops into the adrenal cortex after birth
Where are the adrenal glands typically found in the human adult lies in the retroperitoneum above or medial to upper pole of the kidney
What surrounds the adrenal gland fibrous capsule
Which part of the adrenal gland is larger the cortex or medulla cortex- it accounts for 90% of gland weight
What are the arterial supplies for the adrenal gland inferior phrenic artery, renal artery and the aorta
This part of the adrenal gland develops from primitve neural crest cells adrenal medulla
Part of the adrenal gland that is part of the sympathetic nervous system secretes epinephrine and norepinephrine adrenal medulla
in the adult adrenal gland what are the divisions and what do they each make Zona Glomerulosa- Aldosterone Zona fasciculata- Cortisol Zona Reticularis- androgens
What are the cells that make up the adrenal medulla pheochromocytes- large columnar cells with large nuclei and vesicles containing norepi and epi
What two zones of the adrenal cortex does ACTH regulate zona fasciculata, and reticularis
What is the diurinal pattern of ACTH secretion caused by stimulation from CRH diurinal rythym is peak before awakening and a decline as the day progresses
What is the feeback loop from products of adrenal cortex negative feedback loop where cortisol inhibits synthesis and release of CRH and ACTH
What stimulates zona glomerulosa to secrete aldosterone renal juxtaglomerular apparatus and the renin-angiotensin system
What is the universal precursor for all adrenocortical hormones cholesterol
What converts cholesterol to pregnenolone enzyme P450scc
What is the rate limiting step in producing adrenal hormones transporting cholesterol into the mitochondria and converting it to pregnenolone
what effect will deficiency in any of the hydroxylation enzymes of the adrenal cortex cause back up of precursors and deficiency in primary hormones
What effect does ACTH have on secretion of aldosterone minimal effect on secretion of aldosterone
What is the primary regulator of synthesis and secretion of aldosterone renin-angiotensin system (angiotensin II)
What is the renin-angiotensin system blood pressure/volume is low kidney secretes renin which stimulates production of angiotesnsin I. Angiotensin I is converted in the lung by Angiotensin converting enzyme (ACE) to angiotensin II. Angiotensin II causes vasoconstriction
What are the two signals to secrete aldosterone angiotensin II and excess sodium
what is the effect of aldosterone on the distal tubule of the kidney stimulates sodium retention and potassium excretion or hydrogen ions
What is deoxycorticosterone precursor of Alodsterone that stimulates potassium secretion but doesn't cause as much sodium retention. Release Stimulated by rise in serum potassium
What are the effects of cortisol on insulin, lipids and proteins counteracts insulin- increase blood glucose by stimulating gluconeogenesis, glycogen breakdown in liver promotes break down of lipids (fat distributes to 7th vertebrae buffalo hump) promotes break down of proteins (long term muscle wasting and weakness)
What are the effects of cortisol on gastric secretion, water fluids, and the immune system stimulates gastric acid secretion, acts as a water diuretic hormone retain potassium causing weakness and alkalosis, weakens immune system via negative feedback on interleukin-1
What effect does cortisol have on allergies Cortisol is an anit-inflammatory/Anti-Allergy by reducing histamine secretion and stabalizing lysosomal membranes
What effect does cortisol have on bone decreases bone formation and increases bone resorption favorin osteoporosis in long term use
What does cortisol cause with eosinophils eosinophilia
what does cortisol cause with heart increases cardiac contractions
Primary precursor of natural estrogens dehydroepiandrosterone
steroid produced by the testes adrenal cortex and ovaries converted to testosterone and other androgens parent structure of estrone androstenedione
A sex hormone that enhances increase in muscle mass and stimulation of bone growth. Stimulates axillary and pubic hair growth testosterone
more potent form of testosterone causes development of secondary sex characteristics such as facial hair, deepening voice, hypertrophy of sebaceous glands dihydrotestosterone
What stimulates release of adrenal medulla hormones release stimulated by acetylcholine from nerve endings.
What are the effects of norepi vasoconstricts most arterioles resulting in increase in B/P decreases cardiac output
What are the effects of epi vasoconstricts arterioles in the skin causes vasodilation of blood vessels to liver and muscles increases cardiac output decreases intestinal motility stimulates glycogenolysis increases irritability of CNS
What are catecholamines Norepi and epi
How are catcholamines removed from body metabloized in liver by orthomethylation and deamination then kidney secretes metabolites (metanephrine, normetinephrine, and vanyl mandelic acid VMA)
What is Conn's Syndrome primary hyperaldosteronism caused by adrenal adenoma producing excess aldosterone
What causes secondary hyperaldosteronism low perfusion states fibrosis of kidney excessc licorice ingestion liddle's syndrome bartter"s syndrom
what is liddle's syndrome dysregulation of epithelial Na+ channels due to genetic mutation causes secondary hyperaldosteronism
What is Bartter's syndrome defect in the ascending limp of loop of henle where kidney doesn't respond to aldosterone causing secondary hyperaldosteronism
What can cause low perfusion states leading to secondary hyper aldosteronism CHF, Cirrhosis, nephrotic syndrome and renal artery stenosis
What are the s/sx of excess aldosterone mild to severe hypertension may present as headache or stroke fatigue, parasthesias or paralysis polyuria nocturia polydipsia metabolic alkalosis
What are the diagnostic test results for excess aldosterone Hypernatremia- ↑ increase serum sodium hypokalemia-↓ serum potassium hyperaldosterone- ↑ serum aldosterone hyporeninism- ↓ serum renin level
What are the confirmatory tests for primary aldosteronism saline infusion- should decrease renin and aldosterone primary aldosteronism there will be no decrease in aldosterone level CT scan- should so adrenal tumoer Adrenal vein cath- samples from each adrenal vein. vein with heighest level of aldosterone =tum
What is the Tx fro excess aldosterone control hypertension- ace inhibitors spironolactone- potassium sparing diuretic blocks action of aldosterone it also effects adrogen receptors Surgical excision of tumor (must treat
What are the side effects of spironolactone gynecomastia, menstrual irregularities, testicular atrophy, ataxia, erectile dysfunction, drowsiness and rashes.
What is the Tx fro excess aldosterone control hypertension- ace inhibitors spironolactone- potassium sparing diuretic blocks action of aldosterone it also effects adrogen receptors Surgical excision of tumor (must treat
What are the side effects of spironolactone gynecomastia, menstrual irregularities, testicular atrophy, ataxia, erectile dysfunction, drowsiness and rashes.
what is cushings syndrome excess cortisol production by an adrenal adenoma
What are the s/sx of cushings syndrome rapid weight gain (centripital obesity, buffalo hump, Moon face) excess sweating telangiectasia hirsutism polyuria/dypsia hypertension insulin resistance- hyperglycemia GI problems Osteoporosis Opportunistic infections
How do you dx excess cortisol 24 hour urine cortisol test confirm with plasma cortisol level and ACTH level
What would high Serum ACTH in conjuction with excess cortisol indicate ectopic cushing's syndrome (lung tumor secreting cortisol)
What would Low ACTH with excess cortisol indicate adrenal adenoma
HOw does high dose of dexamethasone help dx excess cortisol dexamethasone is synthetic steroid that is similiar to cortisol. It reduces ACTH in normal people leading to decrease in cortisol. If cortisol remains normal you probably have an adrenal adenoma
what imaging technique would you use to confirm location of adrenal tumor CT scan
what is the tx for cortisol excess reduce exogenous corticosteroid use if caused by medicine medical therapy to control symptoms to minimize surgical risk surgical excision to remove adrenal tumor
What causes adrenal medulla hormone excess chromaffin cell tumor called a pheochromocytoma 90% occur in adrenal medulla the rest found along sympathetic chain, 90% benign
Associated with familial MEN-II syndrome or MEN III MENII=hyperparathyroid + medullary thyroid cancer + pheochromocytoma MEN III= medullary thyroid cancer, Marfan's syndrome, neurofibromatosis, and pheochromocytoma
What are the s/sx of pheochromocytoma looks like hyperthyroid headaches paplitation/tachychardia refractory hypertension sweating anxiety tremors constipation
How do you dx pheochromocytoma 24 urine collection looking for metanephrines, catecholamines and VMA collected on day with sympotms. Patient also has to be off anti hypertensives and anxiety meds
what is the confirmatory test for pheochromocytoma clonidine suppression test IV given night AM draw baseline catechols give clonidine redraw catechols after 3 hours normal: clonidine suppresses catechols Pheo: No suppression of baseline catechols Hyperthyroid: clonidine suppresses catechols
after positive clonidine test what should be done radiographic imaging to locate and find tumor MRI T-2 weighted shows pheo as hyperdense structure IF MRI is negative do an MIBG test or Octreotide test
what is the tx for pheochromocytoma SURGERY- pretreat with phenoxybenzamine followed by beta blockers. Post operative give fluid replacment to control hypotension
Failure of the adrenal gland leads to what type of adrenal insufficiency Primary Adrenal Insufficiency
What are common causes of primary adrenal insufficiency autoimmune addison's disease infections-TB, histoplasmosis, cryptococcus aids Adrenal Hemorrhage infiltrative disease- amyloidosis, sarcoidosis Adrenoleukodystrophy congenital adrenal hyperplasia metastic tumors medication
What type of adrenal insufficiency would processes outside of the gland cause secondary adrenal insufficiency
What are some common causes for secondary adrenal insufficiency chronic glucocorticoid use pituitary infections hypothalamic/pituitary tumors hemochromatosis sheehan's syndrome trauma iatrogenic- surgery or radiation damage to pituitary
What would be the s/sx of adrenal cortisol insufficiency hypotension tachycardia weight loss hyperpigmentation hyponatremia hypoglycemia nausea, vomiting, anorexia hypercalcemia muscle and joint pain fever
What would be the s/sx of adrenal aldosterone insufficiency hypotension tachycardia dehydration salt craving disturbed renal functioning
What would be the s/sx of adrenal medulla deficiency hypotension because no vascular constriction due to lack of catecholamines
Why do you need to diagnose adrenal insufficiencies quickly hypotension, tachycardia, weakness, anorexia are life threatening
How do you diagnose adrenal insufficiency 8:00 am plasma cortisol check serum electrolytes check ACTH level
HOw do you confirm adrenal insufficiency ACTH stimulation test
what is the ACTH stimulation test done ot test adrenal insufficiency give ACTH then measure cortisol at 0, 30, 60 minutes if cortison doesn't rise primary adrenal insufficiency
What does the metyrapone test aid with in confirming adrenal insufficiency rules out secondary adrenocortical insufficiency
What would CRH stimulation test help confirm helps determine if adrenal insufficiency is tertiary or secondary. Give CRH then Check ACTH ACTH goes up it is tertiary if ACTH is absent or subnormal it is ACTH problem so secondary adrenal insufficiency
What is the treatment for adrenal insufficiency replace cortisol- via hydrocortisone if patient is under stress increase dose replace mineralcorticoids if necessary
What is congenital adrenal hyperplasia what is commonly seen with CAH any one of several autosomal recessive diseases that cause mutations in enzymes in producing cortisol from cholesterol most individual are deficient in sex steroids and have altered sex charachteristics
What is the primary enzyme effected in congenital adrenal hyperplasia 21-hydroxylase deficiency
What are s/sx of congenital adrenal hyperplasia ambiguous genitalia in females vomiting early rapid growth of pubic hair precocious puberty or failure of puberty excessive facial hair, virilization menstrual irregularities infertility hypertension
What would be seen in patient w/ congenital adrenal hyperplasia of 21-oh deficiency high levels of 17-oh progesterone (precursor) High levels of DHEA High levels of urinary 17-ketosteroids Low levels of aldosterone and cortisol X-ray will show bone age older than normal for person's age
What would be seen in patient w/ congenital adrenal hyperplasia of 11-oh deficiency Elevated 17OH-progesterone Elevated serum DHEA level Elevated urinary 17-Ketosteroids Low Aldosterone and Cortisol levels but you wouldn't have problems of low aldosterone because you would have DOC precursor of aldosterone that has similar effects
Very aggressive and often fatal form of adrenal cancer primary adrenal cancer
how do you dx primary adrenal cancer elevated 24 hour unrine cortisol w/ decreased ACTH elevated serum DHEA-s Fine needle aspiration
how often will you see adrenal masses on CT scans of the abdomen when taken for other reasons 1% of all abdomen CT scans will reveal adrenal masses must do a complete workup to rule out adrenal cancer
If you find an adrenal mass on an X-Ray what do you first look for and what tests will you run is the tumor hormonally active cortisol excess test Aldosterone excess test Catecholamine excess Fine needle aspiration to determine metastatic of infiltrative
What would you do if you found the adrenal mass was cancerous Adrenalectomy
Disorder of cortisol secretion with Increased plasma cortisol and DECREASED ACTH primayr hypercortisolism or cushing's SYNDROME
Disorder of cortisol secretion with Increased plasma cortisol and INCREASED ACTH secondary hypercortisolism or cushing's DISEASE
iDisorder of cortisol secretion with lower Plasma Cortisol & INCREASED Plasma ACTH Primary Hypocortisolism: (Addison's disease)
Disorder of cortisol secretion with lower Plasma Cortisol & DECREASED Plasma ACTH Secondary Hypocortisolism: (pituitary tumor)
Created by: smaxsmith