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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