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61 Cards in this Set
- Front
- Back
gigantism
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childhood excess of growth hormone (before epiphyseal/growth plates close)
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acromegaly
definition? findings? diagnosis? treatment? |
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) |
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pegvisomant
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growth hormone mimicker-- GH receptor has higher affinity for the drug than for endogenous GH so the receptor cannot dimerize/function
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laron syndrome
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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 |
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pseudo Cushing's
causes |
high cortisol levels due to causes other than ACTH or cortisol producing tumors
- depression - alcohol - stress (medical or emotional) |
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most specific signs of Cushings
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Wide >1 cm purple striae
proximal muscle weakness thin skin supraclavicular fat pads |
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sodium and potassium levels in adrenal insufficiency
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aldosterone deficiency--> dec sodium and inc potassium
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causes of primary adrenal insufficiency
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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 |
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if you see acute hypotension in an anticoagulated pt in the ICU-- think...
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bilateral adrenal hemorrhage-- acute adrenal insufficiency-- give roids!
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treatment for adrenal insufficiency
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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 |
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test for primary adrenal insufficiency (PAI)
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check morning cortisol
if <3-- PAI if > 18, rule out PAI if 3-18, give ACTH, if cortisol does not go above 18--> PAI |
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what does estrogen do to total cortisol levels? how?
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it elevates total cortisol levels by increasing the amount of cortisol binding globulin
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actions of estrogen
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- closure of epiphyseal plates at puberty
- proliferation of endometrial lining - breast development - decrease bone resorption |
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actions of progesterone
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- breast development
- maintains uterus during pregnancy - proliferation of endometrial glands (secretion) - increase in body temp |
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hypergonadotrophic hypogonadism
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elevated LH and FSH, ovarian failure (primary problem with ovaries-- hypothalamus and pituitary responding correctly)
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hypogonadotropic hypogonadism
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low or inappropriately normal LH and FSH
problem with hypothalamus or pituitary (stress, tumor etc) |
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secondary amenorrhea
definition? causes... hypothalamus? pituitary? ovary? |
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) |
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premature ovarian failure
causes? |
low estradiol and high FSH in a woman < 40
causes: chemo or radiation, genetic (fragile x syndrome), idiopathic |
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Turner's syndrome
presentation? tx? |
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) |
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Androgen Insensitivity
genotype? phenotype? |
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 |
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Polycystic Ovary Syndrome
presentation/findings? hormone levels/profile? at risk for? US features? tx? |
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 |
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causes of hirsuitism?
tx? |
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 |
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hirsuitism
def? areas of body? |
development of androgen dependent terminal body hair in women
areas: upper abdomen, chest (except around areola= normal) |
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signs of testosterone excess in females
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anovulation
acne, sweating, hirsutism (excessive hair growth) virilization: male pattern hair loss, deep voice, loss of subQ fat, clitoromegaly |
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androgen action in females
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provide substrate for estrogen production in granulosa cells (test--> estradiol)
libido sebaceous glands pubic and axillary hair |
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What enzyme is responsible for conversion of testosterone to DHT? what is DHT responsible for?
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enzyme: 5 alpha reductase
DHT: androgen dependent hair growth (pubic, axillary) |
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testosterone sources in women
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25% adrenal
25% ovary 50% from conversion of androstenedione and DHEA in the periphery |
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catecholamine synthesis equation
rate limiting step? what inhibits this step? |
tyrosine--tyrosine hyrdoxylase-->Dopa-->dopamine--> norepinepherine--PNMT-->epinepherine
rate limiting step: tyrosine hydroxylase, inhibited by norepi and epi and metyrosine (drug) |
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what is PNMT? what is necessary for its function?
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enzyme that converts norepi to epi
needs cortisol |
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signals for catecholamine release
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low BP or blood volume
standing up low blood sugar stress- emotional or physical (illness) drugs- amphetamines |
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actions of B2
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bronchodilation
vasodilation increased release of glucose from glycogen and FFA from adipose tissue skeletal muscle contractility |
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actions of Beta1
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increased heart rate and contractility
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action of alpha 2-
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decreased catecholamine (epi + norepi) release from presynaptic nerve terminals
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action of alpha 1
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vasoconstriction (and sweating?-- sympathetic but mediated by ACh)
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What are 2 methods of catecholamine metabolism?
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1. COMT- converts catecholamines to metanepherine and normetanepherine- 3% of excretion
2. MAO- converts metanepherine and normetanepherine to vanillyl mandelic acid (VMA)- 65% excretion |
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phechromocytoma
sx? locations? associated familial disorders? |
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) |
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causes of high 24 hr urine catecholamines, VMA and metanepherine
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amphetamines (inc NE release), tricyclic antidepressants and cocaine (inhibit reuptake), stress, withdrawal from alcohol, pheo
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actions of testosterone
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-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 |
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what are sertoli cells responsible for?
what stimulates them? |
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 |
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what are leydig cells responsible for?
what stimulates them? |
testosterone and estrogen production
stimulated by: LH |
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testosterone feedback
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inhibits GnRH and LH
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inhibin inhibits what?
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FSH release from the pituitary
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Klinefelter syndrome
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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 |
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kallman's syndrome
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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 |
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causes of gynecomastia
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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 |
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DiGeorge
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failure of migration of chief cells to the parathyroid gland--hypoparathyroidism
other features: VSD, cleft palate, mental retardation |
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Albright's syndrome
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PTH receptor resistance= pseudohypoparathyroidism
features: short stature, round facies, short fingers esp 4th digit, subQ ossifications, sensorineural abn |
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paget disease of bone
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localized very aggressive resorption-- followed by chaotic, unorganized bone formation- woven bone, poor bone quality ultimately
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rickets
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vitamin D deficiency in kids (before closure of epiphyseal plates)
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Type 1 diabetes
cause? DKA common? tx? Age of onset? genetic predisposition? associations? |
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 |
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Type 2 diabetes
DKA common? Age of onset? associations? genetic predisposition? tx? |
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 |
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Diabetes diagnostic criteria
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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) |
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pre-diabetes
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impaired fasting glucose- FBG 100-125mg/dL
impaired glucose tolerance- OGTT 140-200 mg/dL 92hrs after 75 g of oral glucose) |
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Insulin
fast acting intermediate long-acting |
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 |
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actions of insulin
on skeletal muscle? on adipocytes? on electrolytes? |
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-) |
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sx of hyperglycemia
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polyuria
polydipsia polyphagia (inc appetite) blurred vision weight loss dehydration fatigue |
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precipitating factors of DKA
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infection
infarction ischemia intoxication insulin missed dose |
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mechanism of DKA
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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 |
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Labs in DKA
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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 |
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hyperventilation with fruity odor
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DKA (kussmaul sign plus fruity breath from acetone)
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tx of DKA
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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 |