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185 Cards in this Set
- Front
- Back
MEN syndromes' eponymous names
|
MEN 1 = Wermer's syndrome
MEN 2A = Sipple's syndrome |
|
the P's of MEN
|
MEN 1
--a Pair of Pits in a Pan --Parathyroid, Pituitary, Pancreas MEN 2A -- it's just a Pair of two syndromes Pheochromocytoma Parathyroid MEN 2B = pheochromocytoma |
|
MEN 1's tumors
|
--parathyroid
(--> calcium kidney stones) --pituitary --pancreatic: G-VIZ = glucagonomas (rare), VIPomas, insulinomas, Zollinger-Ellison (--> stomach ulcers) |
|
all MEN syndromes' arise how?
|
dominant inheritance
|
|
what do MEN 2A & 2B have in common?
|
ret gene
medullary thyroid carcinoma pheochromocytoma |
|
MEN 2B has something additional of its own, besides what it shares with MEN 2A
|
oral/intestinal ganglioneuromatosis
(associated with marfanoid habitus) |
|
which ketone body is found in greater abundance in diabetic ketoacidosis?
|
beta-hydroxybutyrate
> acetoacetate |
|
diabetic ketoacidosis is usually due to...
the ketone bodies are due to... |
^ insulin requirements from
^ stress (e.g. infection) ^ fat breakdown ^ free fatty acids |
|
6 sxs of diabetic ketoacidosis
|
--Kussmaul respirations
--N/V --abdominal paid --psychosis/delirium --dehydration --fruity breath odor |
|
kussmaul respirations =
|
rapid, deep breathing
|
|
a diabetic ketoacidosis finding I might not think of
|
leukocytosis
|
|
what kind of metabolic acidosis is seen in diabetic ketoacidosis?
|
anion gap
|
|
5 complications of diabetic ketoacidosis
|
CHARM
cerebral edema heart failure arrhythmia Rhizopus infection life-threatening Mucormycosis |
|
carcinoid syndrome is especially caused by
|
metastatic small bowel tumors
|
|
rule of 1/3's applies to ...
and is... |
carcinoid syndrome
1/3 metastasize 1/3 have a 2nd malignancy 1/3 are multiple |
|
the most common tumor of the appendix
|
carcinoid
|
|
zollinger-ellison (6)
|
pancreas or duodenum
stomach: rugal thickening acid hypersecretion recurrent ulcers MEN type 1 |
|
DM type ___ is associated with HLA
|
type 1: HLA-DR3 and 4
|
|
histology of type 1 vs. type 2 DM
|
leukocytic infiltrate
amyloid deposit |
|
(2) rx for prolactinomas
what they are, pharmacologically their result |
bromocriptine
cabergoline (dopamine agonists) shrinkage of the prolactinoma |
|
prolactinoma causes (4) because...
|
amenorrhea
galactorrhea low libido infertility prolactin inhibits GnRH |
|
an important lab result of GH
|
impaired glucose tolerance (insulin resistance)
|
|
Rx for acromegaly
|
adenoma resection
followed by octreotide |
|
^GH is normal in...
|
stress
exercise hypoglycemia |
|
diagnosis of acromegaly
|
^ serum IGF-1
failure to suppress GH following oral glucose tolerance test |
|
diabetes insipidus is at its core ___
|
lack of ADH
or lack of response to ADH |
|
central DI causes
nephrogenic DI causes |
pituitary tumor, trauma, surgery, histiocytosis X
--hereditary --2^ hypercalcemia, lithium, demeclocycline |
|
an ADH antagonist
ADH analog |
demeclocycline
desmopressin |
|
diagnosis of diabetes insipidus
|
--water deprivation test --
urine osmolality doesn't ^ --urine specific gravity < 1.006 --serum osmolality > 290 mOsm/L |
|
Rx for central DI
|
intranasal desmopressin
(ADH analog) |
|
Rx for nephrogenic DI
|
hydrochlorothiazide
indomethacin amiloride |
|
SIADH (3) manifestations
|
water retention
hyponatremia (because to maintain ~ normal volume, the body responds with v aldosterone) urine osmolality > serum osmolality |
|
treatment for SIADH
|
demeclocycline
H2O restriction |
|
causes of SIADH
|
ectopic ADH (small cell lung cancer)
CNS disorders/head trauma pulmonary disease drugs e.g. cyclophosphamide |
|
bone effects of 1^ hyperparathyroidism
|
^ alkaline phosphatase
cystic bone spaces filled with brown fibrous tissue bone pain |
|
sxs of 1^ hyperparathyroidism
|
often asymptomatic
may present with weakness and constipation |
|
phosphorous levels in 1^ vs. 2^ hyperparathyroidism
|
hypophosphatemia
hyperphosphatemia |
|
2 kidney effects of 1^ hyperparathyroidism
|
hypercaliuria --> renal stones
^ cAMP in urine |
|
2 reasons why PTH is elevated in secondary hyperparathyroidism
|
hyperphosphatemia
chronic renal disease --> hypovitaminosis D --> v gut Ca++ absorption |
|
calcium levels in
1^ 2^ 3^ hyperparathyroidism |
hyper
hypo hyper |
|
pseudohypoparathyroidism is aka
|
Albright's hereditary osteodystrophy
|
|
pseudohypoparathyroidism features
|
dominant
--kidney unresponsiveness to PTH --hypocalcemia --shortened 4th/5th digits --short stature |
|
hypoparathyroidism 3 causes
|
surgical
autoimmune DiGeorge |
|
findings of hypoparathyroidism
|
hypocalcemia --> tetany, hyperreflexia
Chovstek's sign (tapping facial nerve --> contraction of facial muscles) Trousseau's sign (occlusion of brachial artery --> carpal spasm) |
|
2 causes of PTH-independent hypercalcemia
|
Ca++ ingestion
cancer |
|
hashimoto's thyroiditis is associated with gene...
|
HLA-DR5
|
|
histology of hashmoto's
|
Hurthle cells
lymphocytic infiltrate germinal centers |
|
cretinism causes
|
endemic
defect in T4 formation failure in thyroid formation |
|
6 findings in cretinism
|
pot-bellied
pale puffy-faced protruding umbilicus protuberant tongue |
|
subacute (de Quervain's) thyroiditis characteristics (6)
|
self-limited
often following a flulike illness granulomatous inflammation ^ ESR jaw pain very tender thyroid |
|
thyroid replaced by fibrous tissue
(rock-like) goiter = |
Riedel's thyroiditis
|
|
which type of hypersensitivity is Graves' ?
|
type II
|
|
Graves' disease often presents when?
|
during stress (e.g. childbirth)
Goljan says illness, postpartum, etc... |
|
a cause of hyperthyroidism besides Graves'
|
toxic multinodular goiter
|
|
thyrotoxicosis
|
a complication of Graves' and other hyperthyroid disorders:
stress-induced catecholamine surge death by arrhythmia |
|
toxic multinodular goiter (4)
|
focal patches of follicular cells
^ release of T3 and T4 independent of TSH 2^ mutation in receptor |
|
thyrotoxicosis in a patient with iodine deficiency goiter is made iodine replete
is called |
Jod-Basedow phenomenon
|
|
Jod-Basedow
|
iodine deficient patient
is made iodine replete --> thyrotoxicosis |
|
5 types of thyroid cancers
|
4 carcinomas:
--papillary --follicular --medullary --undifferentiated/anaplastic lymphoma |
|
the most common thyroid cancer
|
papillary carcinoma
|
|
papillary carcinoma of the thyroid
|
most common
excellent prognosis "ground glass nuclei" psammoma bodies nuclear grooves ^ risk with childhood irradiation |
|
___ increases one's risk for papillary carcinoma
|
childhood irradiation
|
|
thyroid papillary carcinoma histopathology
|
"ground glass nuclei"
psammoma bodies nuclear grooves |
|
follicular carcinoma of the thyroid
|
good prognosis
uniform follicles |
|
medullary carcinoma of the thyroid comes from _ cells
|
parafollicular C cells
|
|
medullary carcinoma of the thyroid is associated with
|
MEN 2A and 2B
|
|
medullary carcinoma lab findings
|
calcitonin
|
|
medullary thyroid carcinoma histopathology
|
sheets of cells in amyloid stroma
|
|
undifferentiated/anaplastic thyroid carcinoma
|
older patients
very poor prognosis |
|
thyroid lymphoma is associated with
|
Hashimoto's
|
|
medullary thyroid carcinoma (4)
|
from parafollicular C cells
makes calcitonin sheets of cells in amyloid stroma associated with MEN 2A and 2B |
|
norepinephrine pathway and breakdown products
|
|
| v phenylalanine tyrosine L-dopa dopamine--> HVA norepinephrine --> VMA epinephrine --> metanephrine | | v |
|
_ is elevated in pheochromocytoma
_ is elevated in neuroblastoma |
in the urine:
--VMA (from norepinephrine) --HVA (from dopamine) |
|
a prognostic indicator re: neuroblastoma
|
overexpression of N-myc means
rapid tumor progression |
|
5 P's symptoms of pheochromocytoma
|
pressure (^ BP)
pain (headache) perspiration palptations (tachycardia) pallor |
|
rule of 10's for pheochromocytoma
|
10% malignant
10% bilateral 10% extra-adrenal 10% calcify 10% kids 10% familial |
|
pattern of symptoms in pheochromocytoma
|
"spells" -- relapse and remit
|
|
neuroblastoma location
|
anywhere along the sympathetic chain
|
|
hypothyroidism vs. hyperthyroidism
cardiovascular & respiratory |
bradycardia, dyspnea on exertion
chest pain, palpitations, arrhythmias |
|
hypothyroidism vs. hyperthyroidism
skin and hair |
dry, cool skin;
coarse, brittle hair warm, moist skin; fine hair |
|
hypothyroidism vs.
hyperthyroidism location of myxedema |
facial/periorbital
pretibial |
|
hypothyroidism vs. hyperthyroidism GI sxs
|
weight gain
v appetite constipation weight loss ^ appetite diarrhea |
|
hypothyroidism vs. hyperthyroidism
neurologic on physical exam |
v reflexes
^ reflexes |
|
T3 uptake is otherwise stated...
|
T3 uptake is ~ proportional to saturation of TBG
i.e., if TBG is very saturated, then there will be a lot of T3 uptake onto a resin you bring in |
|
hypothyroidism vs. hyperthyroidism
T3 uptake |
v T3 uptake
^ T3 uptake |
|
rule of 10's in pheochromocytoma
|
10% malignant
10% bilateral 10% extra-adrenal 10% calcify 10% kids 10% familial |
|
endogenous causes of cushing's syndrome
|
cushing's disease
--ACTH from pituitary adenoma ectopic ACTH --small cell lung cancer --bronchial carcinoids adrenal --adenoma --carcinoma --nodular adrenal hyperplasia |
|
adrenal causes of cushing's syndrome
|
adenoma
carcinoma nodular adrenal hyperplasia |
|
cushing's syndrome sxs
|
moon facies
truncal obesity buffalo hump hypertension hyperglycemia (insulin resist.) weight gain skin changes --thinning --striae osteoporosis amenorrhea immune suppression |
|
dexamethasone suppression test
|
healthy:
v cortisol after low dose ACTH pituitary tumor: ^ cortisol after low dose v cortisol after high dose ectopic ACTH-producing tumor or cortisol-producing tumor: ^ cortisol after low and high dose |
|
hyperaldosteronism types
|
primary (Conn's syndrome)
--aldosterone-secreting tumor secondary --kidney perception of low intravascular volume --> ^ renin-angiotensin system |
|
Conn's syndrome is __
|
primary hyperaldosteronism
|
|
Conn's syndrome cause, sxs
|
unilateral or bilateral
aldosterone-secreting tumor hypertension hypokalemia metabolic alkalosis low renin |
|
how to distinguish primary vs. secondary hyperaldosteronism
|
low renin
high renin |
|
secondary hyperaldosteronism
1 core cause |
kidney perception of low intravascular volume -->
overactive renin-angiotensin system |
|
5 things that cause the
1 core cause of secondary hyperaldosteronism |
renal artery stenosis
chronic renal failure CHF cirrhosis nephrotic syndrome --> kidney perception of low intravascular volume --> overactive renin-angiotensin system |
|
addison's disease:
cause |
adrenal atrophy
destruction by disease --autoimmune --TB --metastasis |
|
addison's disease -->
deficiency of _ |
aldosterone
cortisol |
|
addison's disease sxs (4)
|
hypotension
hyperkalemia acidosis skin hyperpigmentation |
|
addison's disease affects what tissues?
|
all three cortical divisions
spares medulla |
|
distinguish addison's disease from ___
sxs differences? reason? |
2^ adrenal insufficiency from v pituitary ACTH production
--no skin hyperpigmentation --no hyperkalemia because aldosterone's primary regulation is by renin-angiotensin |
|
3 kinds of adrenal insufficiency
|
Addison's disease
--chronic 1^ adrenal insufficiency 2^ adrenal insufficiency because of v pituitary ACTH production Waterhouse-Friderichsen syndrome --acute 1^ adrenal insufficiency |
|
Waterhouse-Friderichsen syndrome is
|
acute 1^ adrenal insufficiency
due to adrenal hemorrhage associated with N. meningitidis septicemia DIC endotoxic shock |
|
testosterone binding protein levels in men and women
|
sex hormone-binding globulin...
^ SHBG --> v free testosterone --gynecomastia in men v SHBG --> ^ free testosterone --hirsutism in women |
|
thyroid hormones' functions
|
bone growth (synergism with GH)
CNS maturation ^ beta1 receptors in heart ^ basal metabolic rate via ^ Na+/K+ ATPase activity ^ glycogenolysis ^ gluconeogenesis ^ lipolysis |
|
thyroid hormone -->
^ basal metabolic rate --> elevation of (3) |
O2 consumption
RR body temperature |
|
negative feedback in thyroid hormone regulation
|
negative feedback by free T3
to anterior pituitary --> v sensitivity to TRH |
|
a binding protein that binds thyroid hormones
its effects what ^ or v it |
thyroxine-binding globulin binds most T3/T4
only free hormone is active hepatic failure v TBG estrogen ^ TBG |
|
the major enzyme in thyroid hormone synthesis
its effects |
peroxidase
oxidation and organification of iodide coupling of MIT and DIT |
|
iodine in the blood vs. in the thyroid gland lumen
|
I-
oxidation --> I2 |
|
vitamin D:
sources they get converted to... |
D3 from sun
D2 from plants both are converted to 25-OH vitamin D in liver --> 1,25-(OH) 2 vitamin D (active) in kidney |
|
function of vitamin D
|
^ absorption of calcium and phosphate
^ bone resorption of Ca++ and phosphate |
|
regulation of vitamin D
|
vitamin D production is stimulated by
^ PTH v Ca++ v phosphate 1,25 (OH) 2 vitamin D inhibits its own production |
|
function of calcitonin
|
v bone resorption of calcium
|
|
regulation of calcitonin
|
^ serum Ca++
causes calcitonin secretion |
|
hormones that signal by cAMP
|
"FLAT CHAMP" + gCG
FSH LH ACTH TSH CRH hCG ADH (V2) MSH PTH GHRH calcitonin glucagon |
|
hormones that signal by cGMP
|
ANP
NO (think vasodilators) |
|
hormones that signal by IP3
|
GnRH
Oxytocin ADH (V1) TRH "goat" |
|
ADH signals by...
|
V1 Gq --> IP3
V2 Gs --> cAMP |
|
TRH vs. TSH
signal by what? |
TSH by cAMP
TRH by IP3 |
|
GH, GnRH, GHRH signal by what?
|
GHRH -- cAMP
GnRH -- IP3 GH -- receptor-associated tyrosine kinase (JAK/STAT pathway) |
|
hormones with
cytosolic vs. nuclear receptors |
cytosolic
Vitamin D Estrogen, Testosterone, Progesterone Cortisol, Aldosterone ---------------------------------- T3/T4 |
|
signaling pathways of endocrine hormones (7)
|
cAMP
cGMP IP3 cytosolic steroid receptor nuclear steroid receptor intrinsic tyrosine kinase (MAP kinase pathway) receptor-associated tyrosine kinase (JAK-STAT pathway) |
|
two tyrosine kinase signaling pathways
|
intrinsic tyrosine kinase
(MAP kinase pathway) receptor-associated tyrosine kinase (JAK-STAT pathway) |
|
hormones that signal by
intrisic tyrosine kinase (MAP kinase pathway) |
insulin
IGF-1 FGF PDGF |
|
hormones that signal by
receptor-associated tyrosine kinase (JAK/STAT pathway) |
GH
prolactin cytokine IL-2 |
|
IL-2 signaling pathway
|
receptor-associated tyrosine kinase
(JAK/STAT) |
|
GH, prolactin signaling pathway
|
receptor-associated tyrosine kinase
(JAK/STAT) |
|
ADH causes vasoconstriction by __ receptors
|
V1
|
|
PTH is made by
|
chief cells
|
|
function of PTH
|
^ bone resorption calcium and phosphate
^ kidney reabsorption of calcium in distal convoluted tubule v kidney reabsorption of phosphate ^ 1,25 (OH) 2 vitamin D production |
|
the enzyme in the kidney that makes 1,25 (OH) 2 vitamin D
|
1alpha-hydroxylase
|
|
PTH regulation
|
v free Ca++ ^ PTH
v free Mg++ v PTH --diarrhea --aminoglycosides --diuretics --alcohol abuse ("DADA") |
|
PTH signaling in bone
|
^ production of
M-CSF RANK-L in osteoblasts --> stimulating osteoclasts |
|
a random bone effect by PTH
a random urinary effect by PTH |
enhances bone matrix degradation
^ urinary cAMP |
|
phosphorus homeostasis (4)
|
lower serum phosphorus
--> conversion at kidney of 25-(OH) vitamin D --> 1,25 (OH) 2 vitamin D releases phosphate from bone matrix ^ phosphate absorption from intestine |
|
cortisol function (7)
|
maintains blood pressure
v bone formation anti-inflammatory v immune function ^ gluconeogenesis ^ lipolysis ^ proteolysis |
|
excess cortisol --> regulation...
|
v CRH, ACTH, and cortisol secretion
|
|
aldosterone synthesis metabolites
|
cholesterol
pregnenolone progesterone 11-deoxycorticosterone corticosterone aldosterone |
|
how does cortisol maintain blood pressure
|
permissive effect with epinephrine:
upregulates alpha1 receptors on arterioles |
|
aldosterone synthesis enzymes
and a couple things that regulate them |
desmolase
-- ACTH + -- ketoconazole - 3beta-hydroxysteroid dehydrogenase 21-hydroxylase 11-deoxycorticosterone aldosterone synthase -- angiotensin II + |
|
aldosterone synthesis metabolites and enzymes
|
cholesterol
--desmolase--> pregnenolone --3beta-hydroxysteroid dehydrogenase--> progesterone --21-hydroxylase--> 11-deoxycorticosterone --11beta-hydroxylase--> corticosterone --aldosterone synthase--> aldosterone |
|
conversion between metabolites from aldosterone pathway to cortisol pathway
by enzyme? |
pregnenolone --> 17-hydroxypregnenolone
progesterone --> 17-hydroxyprogesterone 17alpha-hydroxylase |
|
conversion from cortisol pathway to testosterone pathway
|
17-hydroxypregnenolone --> dehydroepiandrosterone
17-hydroxyprogesterone --> androstenedione |
|
conversion from testosterone pathway metabolites to estrogen pathway metabolites
|
androstenedione --> estrone
testosterone --> estradiol [ testosterone --> DHT ] |
|
testosterone --> (2)
by (2) enzymes |
testosterone --> estradiol
-- aromatase testosterone --> DHT -- 5alpha-reductase |
|
cortisol pathway metabolites and enzymes
|
17-hydroxypregnenolone
--3beta-hydroxysteroid dehydrogenase--> 17-hydroxyprogesterone --21-hydroxylase--> 11-deoxycortisol --11beta-hydroxylase--> cortisol |
|
cortisol pathway metabolites
|
17-hydroxypregnenolone
17-hydroxyprogesterone 11-deoxycortisol cortisol |
|
testosterone synthesis pathway metabolites
and enzymes |
dehydroepiandrosterone DHEA
--3beta-hydroxysteroid dehydrogenase--> androstenedione testosterone |
|
estrogen pathway
|
estrone
| | v estradiol |
|
congenital bilateral adrenal hyperplasias:
which enzymes? |
17alpha hydroxylase deficiency
21-hydroxylase deficiency 11beta-hydroxylase deficiency |
|
17alpha-hydroxylase deficiency
hormone and electrolyte abnormalities |
v sex hormones
^ mineralocorticoids HYPERtension hypokalemia |
|
21-hydroxylase deficiency
hormone and electrolyte abnormalities (7) |
^ sex hormones
v mineralocorticoids HYPOtension hyperkalemia ^ plasma renin volume depletion salt wasting can --> hypovolemic shock in the newborn |
|
11beta-hydroxylase deficiency
hormone and electrolyte abnormalities (4) |
^ sex hormones
v aldosterone v corticosterone HYPERtension 2^ ... ^ 11-deoxycorticosterone (it's a mineralocorticoid) |
|
21-hydroxylase deficiency
sex symptoms |
^ sex hormones
masculinization female pseudohermaphroditism |
|
11beta-hydroxylase deficiency
sex symptoms |
^ sex hormones
masculinization |
|
17alpha-hydroxylase deficiency
sex symptoms |
v sex hormones
XY: v DHT --> pseudohermaphroditism (externally phenotypic female, no internal reproductive structures due to MIF) XX: externally phenotypic female normal internal sex organs lacking 2^ sexual characteristics ("sexual infantilism") |
|
hypothalamic pituitary regulation...
TRH --> |
+ TSH, prolactin
|
|
hypothalamic pituitary regulation...
somatostatin --> |
- GH, TSH
|
|
drugs that + or - prolactin
|
- prolactin secretion:
--dopamine agonists e.g. bromocriptine + prolactin secretion: dopamine antagonists --most antipsychotics --estrogens (OCPs, pregnancy) |
|
prolactin regulates...
|
PRL -->
^ dopamine from hypothalamus v GnRH |
|
these regulate prolactin
|
TRH + prolactin
dopamine - prolactin secretion |
|
islets are most numerous in what part of pancreas?
|
tail
[think about the tail of Alaska with the islands] |
|
islets of langerhans
cell types, products, locations |
alpha -- glucagon (peripheral)
beta -- insulin (central) delta -- somatostatin (interspersed) |
|
insulin regulates _ in pancreas
|
inhibits glucagon release
by alpha cells of pancreas |
|
how does glucose get into beta cell?
|
GLUT-2 (facilitated diffusion)
|
|
___ are tissues that don't need insulin for glucose uptake
|
BRICK L
brain RBCs intestine cornea kidney liver |
|
GLUT-1
|
RBCs
brain |
|
GLUT-2
|
bidirectional
beta islet cells liver kidney small intestine |
|
GLUT-4
|
insulin responsive
adipose skeletal muscle |
|
the glucose transporter that responds to insulin is
|
GLUT-4
|
|
anabolic effects of insulin
|
^ glucose transport
^ glycogen synthesis, storag ^ triglyceride synthesis, storag. ^ protein synthesis (muscle) ^ Na+ retention (kidneys) ^ cellular uptake of K+ and amino acids |
|
besides facilitating the uptake of glucose and K+, insulin also facilitates uptake of _
|
amino acids
|
|
insulin action at the kidneys
|
Na+ retention
|
|
how do beta islet cells release insulin?
(8) |
glucose comes in by GLUT-2 (facilitated diffusion)
aerobic respiration ^^^ ATP closes K+ channel --> depolarization --> opens Ca++ channel ^^^ Ca++ --> exocytosis of insulin |
|
brain energy supply
|
usually uses glucose
uses ketone bodies in starvation |
|
_ is a tissue that always depends on glucose
|
RBCs
|
|
adrenal cortex and medulla
embryonic origins |
cortex: from mesoderm
medulla: from neural crest |
|
zona fasciculata seccretes...
|
cortisol
sex hormones |
|
zona reticularis secretes
|
sex hormones e.g. androgens
|
|
sex hormones from the adrenals come from what histological site?
|
zona fasciculata
*zona reticularis* |
|
acidophils make
|
GH
prolactin |
|
basophils make
|
B-FLAT... Basophils make
FSH LH ACTH TSH |
|
carrier proteins re: pituitary
|
neurophysins
carrier proteins in the posterior pituitary carry hormones in circulation |
|
subunits of pituitary hormones
|
alpha subunit--
common to TSH, LH, FSH, hCG beta subunit-- determines hormone specificity |