Endocrine

Front 1

gigantism

Back 1

childhood excess of growth hormone (before epiphyseal/growth plates close)

Front 2

acromegaly
definition?
findings?
diagnosis?
treatment?

Back 2

def: excess of growth hormone in adults (usually caused by a hyperfunctioning pituitary adenoma that secretes growth hormone)
findings: large hands and feet, deep voice, deep furrows, large tongue (macroglossia), coarse facial features, impaired glucose tolerance (insulin resistance)
diagnosis: high IGF-1 (GH is too pulsatile to measure), failure to suppress serum GH following oral glucose tolerance test
tx: resection of pituitary adenoma + octreotide (somatostatin)

Front 3

pegvisomant

Back 3

growth hormone mimicker-- GH receptor has higher affinity for the drug than for endogenous GH so the receptor cannot dimerize/function

Front 4

laron syndrome

Back 4

growth hormone receptor resistance
low IGF-1 (receptor on liver for GH is dysfunctional)
features: short stature, prominent forehead, depressed nasal bridge, underdevelopment of mandible, truncal obesity, very small penis

Front 5

pseudo Cushing's
causes

Back 5

high cortisol levels due to causes other than ACTH or cortisol producing tumors
- depression
- alcohol
- stress (medical or emotional)

Front 6

most specific signs of Cushings

Back 6

Wide >1 cm purple striae
proximal muscle weakness
thin skin
supraclavicular fat pads

Front 7

sodium and potassium levels in adrenal insufficiency

Back 7

aldosterone deficiency--> dec sodium and inc potassium

Front 8

causes of primary adrenal insufficiency

Back 8

1. addison's - autoimmune (50% have another autoimmune disorder- hashimoto's thyroiditis, DM 1, pernicious anemia (against gastric parietal cells), vitiligo
2. infections- fungal, TB, HIV
3. meds- e.g. ketoconazole- blocks cholesterol entry into steroid synthesis pathway
4. adrenal hemorrhage

Front 9

if you see acute hypotension in an anticoagulated pt in the ICU-- think...

Back 9

bilateral adrenal hemorrhage-- acute adrenal insufficiency-- give roids!

Front 10

treatment for adrenal insufficiency

Back 10

1. hydrocortisone- AM higher dose (15mg) and PM lower dose (5 mg) to simulate normal diurnal rhythm
2. fludrocortisone- replace aldosterone- until there is no edema or postural hypotension

Front 11

test for primary adrenal insufficiency (PAI)

Back 11

check morning cortisol
if <3-- PAI
if > 18, rule out PAI
if 3-18, give ACTH, if cortisol does not go above 18--> PAI

Front 12

what does estrogen do to total cortisol levels? how?

Back 12

it elevates total cortisol levels by increasing the amount of cortisol binding globulin

Front 13

actions of estrogen

Back 13

- closure of epiphyseal plates at puberty
- proliferation of endometrial lining
- breast development
- decrease bone resorption

Front 14

actions of progesterone

Back 14

- breast development
- maintains uterus during pregnancy
- proliferation of endometrial glands (secretion)
- increase in body temp

Front 15

hypergonadotrophic hypogonadism

Back 15

elevated LH and FSH, ovarian failure (primary problem with ovaries-- hypothalamus and pituitary responding correctly)

Front 16

hypogonadotropic hypogonadism

Back 16

low or inappropriately normal LH and FSH
problem with hypothalamus or pituitary (stress, tumor etc)

Front 17

secondary amenorrhea
definition?
causes...
hypothalamus?
pituitary?
ovary?

Back 17

def: had regular periods and they stopped
causes
hypothalamic: endocrine disorders (hyperadrogenism, hyperprolactinemia (inhibits GnRH), hyperthyroidism, Cushing's), functional (stress, anorexia, excessive exercise), structural (tumor)
pituitary (tumor, prolactinoma, stalk effect--GnRH cannot reach pit)
Ovary (premature ovarian failure, pregnancy, polycystic ovary syndrome)

Front 18

premature ovarian failure
causes?

Back 18

low estradiol and high FSH in a woman < 40
causes: chemo or radiation, genetic (fragile x syndrome), idiopathic

Front 19

Turner's syndrome
presentation?
tx?

Back 19

XO- only one x chromosome
"streak ovaries"- not fully developed so cannot produce estrogen (FSH should be high in response)
presentation: short stature and amenorrhea
sometimes: webbed neck, low hairline, wide carrying angle of the arms
tx: estrogen + progesterone replacement (uterus is present)

Front 20

Androgen Insensitivity
genotype?
phenotype?

Back 20

genotypic male XY, but phenotypic female
has testes that produce testosterone, but receptors are defective-- no development of male external genitalia
testosterone is converted to estrogen so there is development of breasts
external genitalia are female bc DHT cannot act on normal androgen receptors
no underarm or pubic hair bc DHT needed for this

Front 21

Polycystic Ovary Syndrome
presentation/findings?
hormone levels/profile?
at risk for?
US features?
tx?

Back 21

hirsuitism, irregular menses, acne, high androgen levels, insulin resistance-- obesity, diabetes (30%), cluster risk factors for CAD, acanthosis nigricans (skin hyperpigmentation-often in armpits)
NOT estrogen deficient (bc testosterone--> estrogen)
hormones: inc testosterone, normal DHEA, normal estrogen, LH: FSH >3 (2/3 of women)
features: inc LH prod--> inc testosterone, peripheral insulin resistance (high insulin levels--> obesity, cholesterol converted to testosterone in ovaries)
inc 17 hydroxylase activity
risk: endometrial cancer (estrogen always present)
US: ring of follicles along the periphery that stopped between primordial and dominant- not sensitive or specific
tx: spironolactone

Front 22

causes of hirsuitism?
tx?

Back 22

causes: supplements, PCOS, ovarian or adrenal tumor, Cushings
tx: ovarian cause: give estrogen--> negative feedback- will lower FSH + LH--> suppress ovarian production of testosterone,
spironolactone- aldosterone (K+ sparing diuretic), testosterone antagonist

Front 23

hirsuitism
def?
areas of body?

Back 23

development of androgen dependent terminal body hair in women
areas: upper abdomen, chest (except around areola= normal)

Front 24

signs of testosterone excess in females

Back 24

anovulation
acne, sweating, hirsutism (excessive hair growth)
virilization: male pattern hair loss, deep voice, loss of subQ fat, clitoromegaly

Front 25

androgen action in females

Back 25

provide substrate for estrogen production in granulosa cells (test--> estradiol)
libido
sebaceous glands
pubic and axillary hair

Front 26

What enzyme is responsible for conversion of testosterone to DHT? what is DHT responsible for?

Back 26

enzyme: 5 alpha reductase
DHT: androgen dependent hair growth (pubic, axillary)

Front 27

testosterone sources in women

Back 27

25% adrenal
25% ovary
50% from conversion of androstenedione and DHEA in the periphery

Front 28

catecholamine synthesis equation
rate limiting step? what inhibits this step?

Back 28

tyrosine--tyrosine hyrdoxylase-->Dopa-->dopamine--> norepinepherine--PNMT-->epinepherine
rate limiting step: tyrosine hydroxylase, inhibited by norepi and epi and metyrosine (drug)

Front 29

what is PNMT? what is necessary for its function?

Back 29

enzyme that converts norepi to epi
needs cortisol

Front 30

signals for catecholamine release

Back 30

low BP or blood volume
standing up
low blood sugar
stress- emotional or physical (illness)
drugs- amphetamines

Front 31

actions of B2

Back 31

bronchodilation
vasodilation
increased release of glucose from glycogen and FFA from adipose tissue
skeletal muscle contractility

Front 32

actions of Beta1

Back 32

increased heart rate and contractility

Front 33

action of alpha 2-

Back 33

decreased catecholamine (epi + norepi) release from presynaptic nerve terminals

Front 34

action of alpha 1

Back 34

vasoconstriction (and sweating?-- sympathetic but mediated by ACh)

Front 35

What are 2 methods of catecholamine metabolism?

Back 35

1. COMT- converts catecholamines to metanepherine and normetanepherine- 3% of excretion
2. MAO- converts metanepherine and normetanepherine to vanillyl mandelic acid (VMA)- 65% excretion

Front 36

phechromocytoma
sx?
locations?
associated familial disorders?

Back 36

rare tumor that causes release of excess of catecholamines in an episodic pattern
sx: pain, pallor, pressure (high), palpitations and perspiration
Rule of 10s: 10% are outside the adrenal gland (called paraganglioma-arise from symapathetic paraganglia), 10% are malignant, 10% are bilateral
familial: von hippel lindau, tuberous sclerosis, MEN2 (multiple endocrine neoplasia-multiple midline endocrine tumors), neurofibromatosis (nerve tumors, cafe au lait spots)
Sturge-Weber- (AVMs of capillaries, port-wine stain on face)

Front 37

causes of high 24 hr urine catecholamines, VMA and metanepherine

Back 37

amphetamines (inc NE release), tricyclic antidepressants and cocaine (inhibit reuptake), stress, withdrawal from alcohol, pheo

Front 38

actions of testosterone

Back 38

-closure of epiphyseal plates
- growth of larynx
- male hair (diamond pubic hair, facial hair, male pattern baldness)
- muscle development/anabolic
- libido, fertility
- erythropoeitin (higher Hgb in men)
- sebaceous gland growth

Front 39

what are sertoli cells responsible for?
what stimulates them?

Back 39

secretion of androgen binding hormone (binds testosterone), gametogenesis, production of inhibin (inhibits release of FSH and LH from the pituitary), estrogen prod.
stimulated by: FSH

Front 40

what are leydig cells responsible for?
what stimulates them?

Back 40

testosterone and estrogen production
stimulated by: LH

Front 41

testosterone feedback

Back 41

inhibits GnRH and LH

Front 42

inhibin inhibits what?

Back 42

FSH release from the pituitary

Front 43

Klinefelter syndrome

Back 43

XXY- primary hypogonadism, problem with sertoli cells (infertile and will not respond to GnRH replacement)
male but lacking adequate testosterone
fibrosis of sertoli cells leads to small FIRM testicles
eunichoid body habitus- failure to close epiphyseal plates
gynecomastia- high FSH stimulates estrogen production
anemia- testosterone stimulates erythropoetin
osteoporosis
breast cancer, DM, varicose veins
social difficulty

Front 44

kallman's syndrome

Back 44

failure of migration of GnRH and olfactory neural cells--> secondary hypogonadism in men
low testosterone, GnRH and FSH/LH
anosmia or hyponosmia
lack of testosterone leads to small round testicles and lack of pubertal development, eunichoid body habitus
other associations: unilateral renal agenesis, cleft lip/palate, synkinesia (try to move one hand and both move, etc)
may be XR or random

Front 45

causes of gynecomastia

Back 45

imbalance between testosterone and estrogen
drugs: Some Drugs Cause Very Awesome KNockers- spironolactone, digoxin (inhibits Na/K ATPase), cimetidine (H2 blocker), Verapamil, Alcohol, ketoconazole, Nifedipine
marijauna, alcoholic liver disease
Klinefelters, hyperthyroidism
estrogen producing tumor

Front 46

DiGeorge

Back 46

failure of migration of chief cells to the parathyroid gland--hypoparathyroidism
other features: VSD, cleft palate, mental retardation

Front 47

Albright's syndrome

Back 47

PTH receptor resistance= pseudohypoparathyroidism
features: short stature, round facies, short fingers esp 4th digit, subQ ossifications, sensorineural abn

Front 48

paget disease of bone

Back 48

localized very aggressive resorption-- followed by chaotic, unorganized bone formation- woven bone, poor bone quality ultimately

Front 49

rickets

Back 49

vitamin D deficiency in kids (before closure of epiphyseal plates)

Front 50

Type 1 diabetes
cause?
DKA common?
tx?
Age of onset?
genetic predisposition?
associations?

Back 50

diabetes due to pancreatic beta islet cell destruction
cause: autoimmune in 95%
DKA is common (both as initial presentation and complication)
tx: always requires insulin replacement
age of onset: bimodal - major peak at <20 yo and 2nd smaller peak at 50-60
genetic predisposition: yes but only 25-50% identical twin concordance
associations: HLA DR3&4 other autoimmune diseases

Front 51

Type 2 diabetes
DKA common?
Age of onset?
associations?
genetic predisposition?
tx?

Back 51

heterogeneous disorder
most commonly associated with insulin resistance and eventual beta cell dysfunction with impaired insulin secretion
DKA is rare
Age: generally
Associations: obesity and ethnicity with African Americans, Native Americans and Asians having high incidence
genetics: nearly 100% monozygotic twin concordance, polygenic, no HLA assoc
tx: many but try lifestyle changes first, then oral meds, and insulin if needed last

Front 52

Diabetes diagnostic criteria

Back 52

dx based on ANY of these 3
1. Fasting Blood Glucose (FBG) > 126 mg/dL
2. symptoms of diabetes (polydipsia, polyuria, unexplained weight loss) + casual plasma glucose > 200 mg/dL
3. 2-hour glucose > 200mg/dL during oral glucose tolerance test (OGTT)

Front 53

pre-diabetes

Back 53

impaired fasting glucose- FBG 100-125mg/dL
impaired glucose tolerance- OGTT 140-200 mg/dL 92hrs after 75 g of oral glucose)

Front 54

Insulin
fast acting
intermediate
long-acting

Back 54

fast acting- Aspart and Lispro- peak action at 30-90 minutes--good for bolus dose with meal
intermediate- NPH
long acting- Glargine and Detemir- no peak action, lasts 24 hours, good for basal insulin dose

Front 55

actions of insulin
on skeletal muscle?
on adipocytes?
on electrolytes?

Back 55

anabolic
muscle: inc glucose uptake into cells (dec blood glucose), inc glycogenesis, inc uptake of AA (lower AA in blood)--> protein synthesis
adipocytes: inc uptake of FFA from blood and storage in adipocytes (activates lipoprotein lipase to free FA from lipoproteins in blood, inhibits hormone sensitive lipase in adipocytes-inhibits lipolysis)
electrolytes: inc uptake of Phosphate, Potassium and (lower blood K+ and PO43-)

Front 56

sx of hyperglycemia

Back 56

polyuria
polydipsia
polyphagia (inc appetite)
blurred vision
weight loss
dehydration
fatigue

Front 57

precipitating factors of DKA

Back 57

infection
infarction
ischemia
intoxication
insulin missed dose

Front 58

mechanism of DKA

Back 58

dec insulin, inc glucagon-- inc blood glucose
cells do not have glucose and must find other energy source- inc gluconeogenesis and glycogenolysis, inc ketogenesis from lipolysis, inc proteolysis

Front 59

Labs in DKA

Back 59

hyperglycemia
ketone levels high (acidosis- low pH)- metabolic acidosis so bicarb is low
osmolality changes (inc serum K+ because insulin is not around to cause uptake into cells)- dec total potassium through urine

Front 60

hyperventilation with fruity odor

Back 60

DKA (kussmaul sign plus fruity breath from acetone)

Front 61

tx of DKA

Back 61

FLUIDS
if K+ is okay then give insulin (if already low K+ can cause hypokalemia from pulling K+ into cells)- IV drip and then subQ injections-- do not withdraw IV until SubQ is on board-- or back into DKA