D/o of the adrenal cortex - McGowan Flashcards
what hormones does the adrenal gland secrete
mineralocorticoids
glucocorticoids
androgens
catecholamines
peptides
what are the mineralocorticoids and where are they made
aldosterone and corticosterone
found within the Zona Glomerulosa in the adrenal gland
what are the glucocorticoids and where are they made
cortisol and cortisone
zona fasciculata in adrenal gland
what are the androgens and where are they made
estrogen and testosterone
zona reticularis in adrenal gland
what are the catecholamines and where are they made
epinephrine and norepinephrine
medulla of the adrenal gland
what are the peptides and where are they made
Somatostatin and Substance P
medulla of adrenal gland
what is the make up of the adrenal gland
cortex and medulla
what is the function/purpose of glucocorticoids and mineralocorticoids
physiologic stress response (long term)
blood pressure regulation and electrolyte homeostasis
aldosterone and cortisol
what is aldosterone responsible for
controls sodium/potassium/water balance
regulation of Blood volume
BP regulation (RAA system)
what is Cortisol responsible for
glucose production
BP regulation
anti-inflammatory
what are adrenal androgen precursors
DHEA (hehydroepiandrosterone)
converted to sex steroids in gonads or target tissues (estrogen and testosterone)
what are the actions of androgen hormones
male and female reproductive development
secondary sex characteristics
menstruation
muscle strenth/mass
what are the catecholamines responsible for
fight or flight response
regulates HR
regulates contractility
vasodilation/constriction
BP regulation
bronchodilation
glycogenesis
what are the 4 S’s of adrenal hromones
Aldosterone = Salt
Cortisol = sugar
Androgen = Sex
Epi, Norepi = Stress
what are hyperfunctioning adrenal disorders
Cushings syndrome
Pheochromocytoma
Multiple endocrine neoplasia
Adrenal adenoma
Adrenal Cancer
what are hypofunctioning adrenal disorders
Addisons disease
Adrenal deficiency
what can cause hypofunction
primarily due to glandular destruction: autoimmune, infection, surgery, inflammation, infarction, hemorrhage or tumor
what can cause hyperfunction
neoplasms (functional), autoimmune disorders, exogenous administration
What is Cushing Syndrome
secondary to glucocorticoid excess, F>M (4:1)
increased ACTH production (dependent vs independent)
pituitary adenomas
cortisol unchecked
excess blood glucose production
increase lipolysis
increase catabolism
decreased insulin production and increased glucagon
what is the presentation of Cushing syndrome
central obesity
rounded face (moon facies)
enlarged fat pad between shoulders (buffalo hump) abdominal striae (purple* stretch marks)
weight gain, Hirsutism, facial plethora (swelling/erythema)
what is the workup for Cushing syndrome
elevated 24 hour urine free cortisol
elevated midnight plasma cortisol
dexamethasone suppression test
Plasma ACTH
Localizing the source - ACTH dependent - MRI/CT; ACTH independent - CT of adrenals
what can impact 24 hour urine free cortisol tests
anti-epileptics, estrogen, testosterone and exogenous steroids
what is the overnight dexamatheasone suppression test
baseline plasma cortisol (high dose only)
give 1mg or 8mg dexamethasone at 11pm
check plasma cortisol at 8-9am
what is the difference between ACTH dependent and ACTH independent
Dependent: problem upstream/outside of adrenals - pituitary
Independent: problem is in the adrenal gland
What is the treatment of Cushing syndrome
piruitary source (dependent): trans-sphenoidal resection of mass
Ectopic ACTH-secreting tumors: local surgical resection
benign adrenal adenoma: laparoscopic resection
adrenal carcinomas: surgical resection
what is required for longterm treatment of cushing syndrome
long-term glucocorticoid replacement
younger = more likely to fully recover
what are negative prognostic indicators for Cushings
older age at diagnosis
higher pre-op ACTH
longer disease presence
what is a sympathetic nervous system tumor arising from the adrenal medulla
pheochromocytoma
avg. age at dx is 40 yo
cause of death in 1/3 of pts prior to dx
what is secreted from pheochromocytomas
catechomaines (epi and norepi)
what are the rule of 10 for pheo
~10% bilateral
~10% exra-adrenal
~10% malignant
what is the presentation for variable presentation
‘the great masquerader’- variable presentation
HA, Profuse sweating, palpitation and tachycardia, HTN (sustained or paroxysmal), Anxiety/panic attacks
What is the classic triad of PHEo
Palpitations
Headache
Episodic sweating
diagnosis likely if all three + HTN
what can pheo result in
catastrophic HTN crisis, fatal arrhythmias, pulmonary edema or HF
may see ARDS and mutisystem failure
what is the workup for Pheo
plasma fractionated free metanephrines (most sensitive)
other lab tests: elevated plasma catecholamines, 24 hour urine catecholamines, serum chromogranin A (CgA), clonidine suppression test
Non-contrast abdominal CT
MRI with or without Gadolinium contrast
what are the treatment options for pheo
tx of choice: surgical resection
must medically optimize HTN and tachycarrhythmias preop
caution: tx resistant hypotesion post-op => AKI, MI
what is the treatment for malignant pheo
add chemo
recheck BP and plasma free metanephinres
follow up for serum chromogranin
a (CgA)
what is multiple endocrine neoplasia (MEN)
inherited tumor syndromes
- autosomal dominant (genetic testing)
tumor in 2+ endocrine glands
4 distinct types
what is the presentation of MEN1
90% have mutation in Menin gene
abnormalities can be deltected around 14-18 years old
clinical symptoms appear in 20s-30s
mean life expectancy: 55 years
classic triad: parathyroids, pancreatic islets, anterior pituitary
What is the presentation of MEN2 (2A) and Men 3 (2B)
ret protooncogene mutation
difference is clinical presentation
Classic triad: meduallary thyroid carcinoma (MTC), pheochromocytomas, parathyroid tumors
what gene is mutated in MEN4
CDKN1B
what is the presentation of MEN2
classic triad: Medullary thyroid carcinoma (MTC), pheochromocytomas, parathryoid tumors
What is the presentation of MEN3
characterized by mucosal neuromas
marfan like habitus
medullary thyroid carcinomas
what is the presentaiton of MEN4
most rare of MEN subsets
often pituitary, parathyroid and pancreatic tumors
how do you work up MEN
genetic testing
screen patients with known FH
screen for neuroendocrine tumors with targeted tests/imaging
what is the treatment of MEN1
parathyroidectomy if symptomatic
prophylactic thymectomy if getting parathyroidectomy
high does PPIs and control of hyeprcalcemia if gastrinomas
what is the treatment for MEN 2 and MEN3
if known FH and + genetic screening
total thyroidectomy : by age 6 for MEN2, by 6 months for Men 6
no GLP-1 inhibitors for DM
screening for pheo and thyroid cancer
what can cause adrenal insufficiency
primary of secondary causes
primary - adrenal gland dysfunction (cortisol and aldosterone)
secondary - ACTH deficiency due to hypothalamus or pituitary dysfunction
What is a primary adrenal disease
Addison’s Disease (Adrenal Deficiency)
primarily autoimmune in origin
what is the classic presentation of adrenal insufficiency
fatigue, reduced stamina, weakness, anorexia and weight loss
what are the later presenting facors for adrenal insufficiency
N/V, abdominal pain +/- diarrhea
pain: arthralgia, myalgias, CP, abdominal pain, back/leg pain, HA
Psych: irritability, depression, anxiety
hyperpigmentation
what is acute adrenal crisis
more common with primary insufficiency
life threatening
N/V/Fever, dehydration, profound hypotension
progression to shock, unresponsive to fluids or vasopressors
what is the workup for adrenal insufficiency
plasma cortisol
low serum DHEA levels
confirmation with cosynotropin stimulation test (synthetic ACTH)
what is the treatment for adrenal insufficiency
glucocortioid replacement therapy (15-30mg hydrocortisone daily in 2-3 doses)
mineralocorticoid replacement therapy (fludrocortisone acetate)
+/- DHEA supplementation for women
what is the treatment of adrenal crises
loading dose of hydrocortisone 100-300mg IV
rehydration with saline solution (generous resuscitation, plus dextrose)
then, hydrocortisone 50-100mg q6h IV, titrate down to PO as able
broad spectrum ABX if infection ruled out as cause
how do you prevent adrenal criese
patient education of times of increased risk
give extra doses and plenty of refills
stress dose steroids
dose adjustment for hot weather, vigorous exercise
anti-emetics PRN to prevent N/V
consider other routes of administration if unable to take PO due to N/V
