Week 2 Review Q's Flashcards
Anatomy of suprarenal glands (1-64) steroid biochemistry (65-111) regulation of adrenal hormone syn (112-129) patho of adrenals (130-175) seminar fundamentals of assessment of adrenal disorder (176-197) seminar CAH (198-208) adrenal gland physio (209-239) adrenal hormones pharma (240-288) pathology of adrenals lab (289-304)
Which fo the following has a bigger suprarenal gland?
a. male adult
b. female adult
c. fetus
c. fetus
Which is derived from the fibrous stroma of the gland?
a. true capsule
b. false capsule
a. true capsule
Which pathology is most likely to be found in the bare area of the liver?
an abscess (more common in that area)
anterior and posterior renal fascia join to form which structure?
false capsule
Which is most likely to be affected by liver abscesses?
a. right suprarenal gland
b. left suprarenal gland
a. right suprarenal gland
Which part of the right suprarenal gland is in contact with the bare area of the liver?
lateral side
the suprarenal vein emerges from which part of the suprarenal gland?
a. anterior surface
b. posterior surface
c. medial border
a. anterior surface
Which structure is related to the inferior side of the posterior surface of the right suprarenal gland?
right kidney
Which structure is related to the medial side of the anterior surface of the right suprarenal gland?
IVC
Which part of the right suprarenal gland is in contact with the diaphragm?
a. anterior surface
b. posterior surface
c. medial border
b. (upper part of the) posterior surface
T/F: the left suprarenal gland is concave from the lateral border
true
Which structure is related to the superior side of the anterior surface of the left suprarenal gland?
stomach
Which structure is related to the inferior side of the anterior surface of the left suprarenal gland?
pancreas
Which structure is related to the lateral side of the anterior surface of the right suprarenal gland?
liver
Which structure is related to the lateral side of the posterior surface of the left suprarenal gland?
diaphragm
Which is a branch of the renal artery?
a. Superior suprarenal artery
b. Middle suprarenal artery
c. Inferior suprarenal artery
c. Inferior suprarenal artery
What two things drain into the Left Renal vein?
Left suprarenal vein
Left gonadal vein
Which is a branch of the abdominal aorta?
a. Superior suprarenal artery
b. Middle suprarenal artery
c. Inferior suprarenal artery
b. Middle suprarenal artery
Which cells produce chromogranin A?
chromaffin cells
chromogranin A is s storage protein complex for which hormones?
epinephrine
nonepinephrine
Which spinal cord segment supplies preganglionic sympathetic fibers to the suprarenal medulla?
T8-L1
Which is a branch of the inferior phrenic artery?
a. Superior suprarenal artery
b. Middle suprarenal artery
c. Inferior suprarenal artery
a. Superior suprarenal artery
Nerves supplying the cortex of the suprarenal gland go through which of the following?
a. greater splanchnic nerve
b. coeliac ganglion
b. coeliac ganglion
Which part of the suprarenal gland develops from the mesoderm?
cortex
When do cells of the cortex produce DHEA?
a. first trimester
b. second trimester
c. third trimester
d. during birth
e. after birth
b. second trimester
Which part of the suprarenal gland develops from neural crest cells?
medulla
Nerves supplying the chromaffin cells of the suprarenal gland go through which of the following?
a. greater splanchnic nerve
b. coeliac ganglion
a. greater splanchnic nerve
What’s the main difference between postsynaptic neurons and chromaffin cells?
chromaffin cells don’t have axonal processes
When do cells of the cortex differentiate into 3 zones?
a. first trimester
b. second trimester
c. third trimester
d. during birth
e. after birth
e. after birth
What’s the most common cause of congenital adrenal hyperplasia (CAH)?
absence of 21-hydroxylase.
Which describes the inheritance pattern of congenital adrenal hyperplasia (CAH)?
a. autosomal dominant
b. autosomal recessive
c. X linked
b. autosomal recessive
explain the mechanism of how congenital adrenal hyperplasia (CAH) leads to abnormal genital development in infant girls
CAH may enable the synthesis of cortisol or aldosterone, which leads to the body making more of another hormone: androgen. High androgen causes the manifestations
Which approach to suprarenal gland excision is commonly used in laparoscopy?
a. posterior
b. lateral
c. anterior
c. anterior
T/F: posterior and lateral approaches to suprarenal gland excision involves the removal of rib 11 and rib 12
false, the removal or rib 11 OR rib 12 is needed
What percentage of the suprarenal gland is the medulla?
10%
Which suprarenal gland is being excised in this procedure: The splenic flexure is mobilized inferiorly revealing the kidney and the lateral phrenicocolic and splenorenal ligaments are then fully divided.
a. right suprarenal gland
b. left suprarenal gland
b. left suprarenal gland
Which releases catecholamines?
a. cortex
b. medulla
b. medulla
Which of the following is the thickest layer?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
b. Zona Fasciculata
Which of the following secretes aldosterone?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
a. Zona Glomerulosa
Which releases steroids?
a. cortex
b. medulla
a. cortex
Which of the following secretes cortisol?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
b. Zona Fasciculata
secretes glucocorticoids
Which of the following is responsible for axillary and pubic hair growth during puberty (in females)?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
c. Zona Reticularis
Adrenal androgens are secreted by Zona Reticularis in response to what hormone? What receptor does the hormone use?
ACTH
ACTH receptor called melanocortin receptor 2
Which of the following have uniform polyhedral cells with distinct outlines, indistinct nucleoli, and vacuolated cytoplasm?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
b. Zona Fasciculata
Which of the following increases hormone release when renin is high?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
a. Zona Glomerulosa
Which of the following releases adrenal androgens after prolonged ACTH stimulation?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
b. Zona Fasciculata
by Zona Reticularis initially
Which of the following consists of columnar cells arranges in inverted columns?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
a. Zona Glomerulosa
Which of the following contains chromaffin cells?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
d. Medulla
Patient presents with high vaniyll mandelic acid (VMA) levels in urine. Which pathology is most likely?
Pheochromocytoma (tumor of the medulla; excessive catecholamines)
Describe the dual blood supply of the medulla
arterial blood from the medullary arterioles
venous blood from the cortical sinusoids
How does the adrenomedullary vein aid in the efflux of hormones?
tunica media of vein contains longitudinally oriented smooth muscles, their contraction causes the adrenal gland volume to decrease leading to hormone efflux
Which two things work together to form the fight/flight response?
glucocorticoids (secreted in the cortex- they induce conversion of NE to epinephrine) and catecholamines (secreted in the medulla)
What do Chromaffin cells secrete?
secrete noradrenaline and adrenaline (and chromogranin a)
In which area do the anterior and posterior renal fascia NOT fuse?
a. superiorly
b. inferiorly
b. inferiorly
Which of the following drain directly into the IVC?
a. right suprarenal vein
b. left suprarenal vein
a. right suprarenal vein
Which of the following’s hormone release is regulated by juxtaglomerular cells of the kidney and partly by the pituitary?
a. Zona Glomerulosa
b. Zona Fasciculata
c. Zona Reticularis
d. Medulla
a. Zona Glomerulosa
juxtaglomerular cells release renin
What catalyzes the conversion of angiotensinogen to angiotensin I?
Circulating renin
What catalyzes the conversion of angiotensin I to angiotensin II?
angiotensin-converting enzyme (ACE)
made in the lung
What method can be used to treat chronic essential hypertension?
give drugs that inhibit ACE in the lung (it stops the synthesis of angiotensin II, which stops aldosterone secretion)
(we took “Captopril” in a learning topic which is an ACE inhibitor)
patient comes in with muscular weakness, low blood pressure, anemia, hyperpigmentation, and weight loss. What do you suspect?
Addison’s disease (deficiency of mineralocorticoids)
Patient comes in with hypertension, edema due to sodium and water retention and hypokalemia. What do you suspect?
Conn’s syndrome (excessive aldosterone secretion)
patient comes in with obesity, diabetes and hypogonadism. What do you suspect?
Cushing’s syndrome (excessive secretion of glucocorticoids)
explain why Cushing’s syndrome causes slow wound healing?
Cortisol is diabetogenic and delays wound healing by diminishing the activity of fibrocytes. It’s also anti-allergic and diminishes antibody formation
cells of which area stain positive (brown) when treated with potassium bichromate?
The cells of the medulla (Chromaffin cells stain positive, chromaffin reaction)
What do you call a steroid made of 17 carbons containing tetracyclic hydrocarbons?
gonane
In which location is the side chain arranged to the steroid nucleus?
carbon 17 of the D ring
Where does most of the cholesterol used for the steroid synthesis come from?
from the circulation (80%)
Which of the following goes through aromatization to be made?
a. Androgens
b. Bile acids
c. Estrogens
d. Progesterone
c. Estrogens
aromatization in ring A
Which of the following is made by adding an OH to cholesterol?
a. Androgens
b. Bile acids
c. Estrogens
d. Progesterone
b. Bile acids
Which is used to esterify cholesterol?
a. Cholesterol ester hydrolase
b. Acyl-CoA cholesterol acyltransferase I
c. cytochrome P450scc
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
e. 17,20-Lyase
b. Acyl-CoA cholesterol acyltransferase I (ACAT)
How many sites in steroidogenic acute regulatory (StAR) protein are available for phosphorylation by protein kinase C (PKC)?
6
Which of the following is encoded by cyp11a1 gene?
a. Cholesterol ester hydrolase
b. Acyl-CoA cholesterol acyltransferase I
c. cytochrome P450scc
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
e. 17,20-Lyase
c. cytochrome P450scc
How many sites in steroidogenic acute regulatory (StAR) protein are available for phosphorylation by protein kinase A (PKA)?
3
In which two locations does steroid hormone synthesis take place?
ER
mitochondria
Which of the following is responsible for creating androgens from cholesterol?
a. 17 alpha-hydroxylase
b. 21 hydroxylase
c. 17,20-Lyase
d. aromatase
e. aldosterone synthase
c. 17,20-Lyase
mineralocorticoids are synthesized due to the deficiency of enzyme?
a. 17 alpha-hydroxylase
b. 21 hydroxylase
c. 17,20-Lyase
d. aromatase
e. aldosterone synthase
a. 17 alpha-hydroxylase
Androgens are synthesized due to the deficiency of enzyme?
a. 17 alpha-hydroxylase
b. 21 hydroxylase
c. 17,20-Lyase
d. aromatase
e. aldosterone synthase
b. 21 hydroxylase
Which of the following genes codes for 21-hydroxylase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
b. CYP21A2
glucocorticoids are synthesized due to the deficiency of enzyme?
a. 17 alpha-hydroxylase
b. 21 hydroxylase
c. 17,20-Lyase
d. aromatase
e. aldosterone synthase
e. aldosterone synthase
What happens with the total removal of side-chain?
a. androgens
b. progesterone
c. corticosteroids
d. mineralocorticoids
a. androgens
progesterone, corticosteroids & mineralocorticoids= Partial removal
Which of the following genes codes for aromatase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
f. CYP19
Which of the following genes codes for aldosterone synthase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
d. CYP11B2
Which of the following genes codes for 17,20 lyase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
e. CYP17
Which of the following genes codes for desmolase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
a. CYP11A1
desmolase aka SCC enzyme
Leydig cells express high levels of which two hormones? What activates Leydig cells to produce testosterone?
3β-HSD and 17β-HSD are highly expressed in Leydig cells
LH activates Leydig cells
What four actions does PKA do when activated by camp?
1- import of cholesterol via LDL
2- activates CEH enzyme
3- activates Star
4 activates TF CREB
Which of the following converts cholesterol into pregnenolone?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
a. CYP11A1
aka P450scc
What converts estrone into estradiol?
17β-Hydroxysteroid dehydrogenase
What converts androstendione into estrone?
Aromatase
Which of the following is transported via Corticosteroid binding globulin (CBG)?
a. Aldosterone
b. DHEA
c. Cortisol
d. Androgens & Estrogens
c. Cortisol
Which of the following is transported via Albumin?
a. Aldosterone
b. DHEA
c. Cortisol
d. Androgens & Estrogens
a. Aldosterone
Which of the following does not have a specific carrier protein?
a. Aldosterone
b. DHEA
c. Cortisol
d. Androgens & Estrogens
b. DHEA
What percent of steroid hormones freely circulate in the bloodstream?
10%
T/F: steroid hormones do not use secondary messengers, but rather act directly to change gene transcription
true
Which of the following is transported via Sex hormone binding globulin (SHBG)?
a. Aldosterone
b. DHEA
c. Cortisol
d. Androgens & Estrogens
d. Androgens & Estrogens
Which stage of steroid inactivation leads to the transformation of the lipophilic compounds into water soluble metabolites?
a. phase 1
b. phase 2
b. phase 2
Sulfation or glucuronidation
The Sulfation or glucuronidation in phase 2 of steroid inactivation occurs in which carbon positions?
3rd and/or 17th Carbon positions.
Which carbon positions are the two methyl groups of cholesterol located?
at position 18 & 19
Which is used to breaks down cholesterol esters and produces free cholesterol and FFA?
a. Cholesterol ester hydrolase
b. Acyl-CoA cholesterol acyltransferase I
c. cytochrome P450scc
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
e. 17,20-Lyase
a. Cholesterol ester hydrolase
What receptor is used to capture cholesterol from circulatory LDL?
Scavenger receptor class B, type I (SR-BI)
What is the first and rate-limiting step in any steroid synthesis?
Cleavage of 6 carbons (C22-C26) of the side chain (via cytochrome P450scc)
What converst insoluble cholesterol (27C) is into soluble pregnenolone (21C)?
CYP11A1 (aka P450scc aka desmolase)
it does it via three steps (hydroxylation, hydroxylation, Scission)
Which enzyme inactivates steroidogenic acute regulatory (StAR) protein?
PKC
Which enzyme activates steroidogenic acute regulatory (StAR) protein?
PKA
Which is used to convert Pregnenolone into progesterone?
a. Cholesterol ester hydrolase
b. Acyl-CoA cholesterol acyltransferase I
c. cytochrome P450scc
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
e. 17,20-Lyase
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
Which of the following is known as the second SCC enzyme?
a. Cholesterol ester hydrolase
b. Acyl-CoA cholesterol acyltransferase I
c. cytochrome P450scc
d. 3b-Hydroxy steroid dehydrogenase (3bHSD)
e. 17,20-Lyase
e. 17,20-Lyase
Which of the following codes for 11 beta hydroxylase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
c. CYP11B1
Which of the following codes for 3 beta-hydroxysteroid dehydrogenase?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
g. 3 beta-HSD
Which THREE of the following code for enzymes located in the ER?
a. CYP11A1
b. CYP21A2
c. CYP11B1
d. CYP11B2
e. CYP17
f. CYP19
g. 3 beta-HSD
b. CYP21A2 & e. CYP17 & g. 3 beta-HSD
(the rest code for mitochondrial enzymes)
Which TWO of the following are found in the Mitochondria?
a. CYP-450scc / 20,22-lyase
b. 3b-hydroxysteroid dehydrogenase
c. 17a-hydroxylase
d. 21-hydroxylase
e. 11b-hydroxylase
a. CYP-450scc / 20,22-lyase
&
e. 11b-hydroxylase
Which of the following releases LDL from the LDL receptors in endosomes?
a. Cholesterol ester hydrolase
b. endosomal ATPase
c. Acyl-CoA cholesterol acyltransferase I
d. Cholesterol ester hydrolase
b. endosomal ATPase
What suppresses the immune system in long term stress response?
glucocorticoids
What increases blood volume and pressure in long term stress response?
mineralocorticoids
What increases blood glucose in short term stress response?
epinephrine and norepinephrine
Sterol carrier protein-2 (SCP-2) carries free Cholesterol to which of the following sites?
a. inner mitochondrial matrix
b. outer mitochondrial matrix
b. outer mitochondrial matrix
Whos is most likely to get steroidogenic cell hyperplasia?
a. person on vacation
b. person with pet
c. medical student
c. medical student
chronic stress
What activates Aldosterone is synthesis?
angiotensin 2 and plasma potassium
Which receptor does aldosterone act on?
mineralocorticoid receptor (MR)
What’s the function of hormone-sensitive lipase (HSL)?
liberates free Cholesterol from lipid droplets
Hormone-sensitive lipase HSL, AKA cholesteryl ester hydrolase (CEH)
What type of stress induces protein and fat breakdown into sugar? What induces these changes?
long term stress converts proteins and fats into glucose (while short term raises glucose by breaking down glycogen)
glucocorticoids induce this
When is aldosterone secreted?
a. low potassium levels
b. low blood pressure
c. increased sodium levels
d. increased blood volume
b. low blood pressure
released when low blood pressure, volume, and sodium, also when potassium levels are high
What enzymes do Aldosterone-target cells express?
11β-hydroxysteroid dehydrogenase –Type II (11β-HSD-II)
What’s the regulator of the rate-limiting step in steroid biosynthesis?
steroidogenic acute regulatory (StAR) protein
What hormones acts on the mineralocorticoid receptors (MR)?
Aldosterone and cortisol
Circulating cortisol is 3x higher than aldosterone, so how do mineralocorticoid receptors get more aldosterone?
11β-HSD-II converts cortisol to the inactive cortisone, permitting aldosterone to activate mineralocorticoid receptor
Defective 11β-HSD-II leads to which pathologies?
sodium retention, and salt-dependent hypertension
Denovo synthesis of cholesterol requires which precursor?
acetyl coA
What does steroidogenic acute regulatory (StAR) protein do?
speeds the transport of cholesterol from the outer mitochondrial membrane (OMM) to the inner mitochondrial membrane (IMM)
What are the four Aldosterone-target cells?
Kidney, colon, vascular wall, placenta
Which of the following is NOT controlled by ACTH levels?
a. Adrenal androgens
b. Glucocorticoids
c. Mineralocorticoids
c. Mineralocorticoids
mainly controlled by the renin angiotensin system
Which of the following tests deficiencies?
a. Stimulation Tests
b. Suppression Tests
a. Stimulation Tests
Hypercortisolism is also known as
Cushing Syndrome
Which hormones do not have a negative feedback inhibition?
Mineralocorticoids & Sex steroids
Why do patients with Cushing syndrome have purple striae and poor wound healing?
due to weak collagen
Which of the following tests overactivity?
a. Stimulation Tests
b. Suppression Tests
b. Suppression Tests
Which muscle type is most affected in patients with Cushing syndrome?
fast-twitch, type 2 muscles
Most common cause of Cushing syndrome is
Prolonged corticosteroid therapy (iatrogenic)
Which has low ACTH?
a. Adrenal Cushing syndrome
b. Cushing disease
c. Ectopic Cushing syndrome
a. Adrenal Cushing syndrome
Which of the following has a positive high-dose dexamethasone suppression test?
a. Adrenal Cushing syndrome
b. Cushing disease
c. Ectopic Cushing syndrome
b. Cushing disease
dexamethasone= cortisol analog
Which is used to confirm the diagnosis of Cushing?
a. low-dose dexamethasone suppression test
b. high-dose dexamethasone suppression test
a. low-dose dexamethasone suppression test
confirm Cushing but not identify its cause
When is cortisol the highest/lowest?
highest in early mornings (8/9AM) and least at midnight
Which is used to know the cause of Cushing?
a. low-dose dexamethasone suppression test
b. high-dose dexamethasone suppression test
b. high-dose dexamethasone suppression test
Which has characteristically very high ACTH?
a. Adrenal Cushing syndrome
b. Cushing disease
c. Ectopic Cushing syndrome
c. Ectopic Cushing syndrome
On average, which undergoes larger cortical hyperplasia (adrenal enlargement) and why?
a. Adrenal Cushing syndrome
b. Cushing disease
c. Ectopic Cushing syndrome
b. Cushing disease
(Ectopic Cushing syndrome also have it, but the rapid downhill of patients with these cancers often limits the extent of the adrenal enlargement)
Which of the following is caused by “incidentalomas”?
a. Adrenal Cushing syndrome
b. Cushing disease
c. Ectopic Cushing syndrome
a. Adrenal Cushing syndrome
incidentalomas= adrenocortical adenomas that are clinically silent and found incidentally
Most common cause of hypercortisolism?
exogenous administration of steroids
3 clinical features of Hyperaldosteronism
Hypertension
Postassium loss
Sodium retention
What causes Secondary Hyperaldosteronism?
activation of the renin-angiotensin system
What causes the Spironolactone bodies in histology samples?
When you take the anti-hypertensive medication, Spironolactone (prevents your body from absorbing too much salt and keeps your potassium levels from getting too low)
What is Conn’s syndrome?
primary hyperaldosteronism, where the adrenal glands make too much aldosterone
Describe renin levels Conn’s syndrome
decreased plasma levels
Most common cause of Hyperaldosteronism?
idiopathic
Most common cause of Hypoaldosteronism?
Abrupt withdrawal of corticosteroids
dexamethasone is an analog for
cortisol
What is Waterhouse–Friderichsen syndrome (WFS)?
adrenal gland failure due to bleeding into the adrenal glands, commonly caused by severe bacterial infection (N. meningitidis)
What is Addison disease?
primary hypoaldosteronism
When can hypercortisolism cause bilateral cortical atrophy?
When its caused by exogenous steroids, they suppress ACTH, which stops the adrenals from producing cortisol. That leads to atrophy.
Which of the following is more common?
a. cushing syndrome
b. cushing disease
c. ectopic cushing syndrome
b. cushing disease
Which is more common in men above the age of forty?
a. Adrenal cushing syndrome
b. cushing disease
c. Ectopic Cushing syndrome
c. Ectopic Cushing syndrome
What is used to screen for hyperaldosteronism?
aldosterone:renin ratio
What is used to confirm hyperaldosteronism?
aldosterone suppression test
Secondary hyperaldosteronism is caused by a deficiency in
ACTH
A patient has an autoimmune disease against the adrenal gland. What kind of hypoaldosteronism can this lead to?
a. primary acute
b. primary chronic
c. secondary
b. primary chronic
primary hyperaldosteronism VERSUS secondary hyperaldosteronism
Which one causes pigmentation and why?
Primary causes high ACTH. To make ACTH POMC is required, which is what stimulates MSH and leads to pigmentation.
(secondary hyperaldosteronism has low ACTH, thus low POMC)
Which neoplasm which stains positive for potassium dichromate?
Pheochromocytoma (neoplasm made from chromaffin cells)
Pheochromocytoma have a granular cytoplasm, what is an explanation for this?
the cytoplasm has many vacuoles filled with catecholamines
Describe clinical features of a patient with pheochromocytoma
chronic hypertension
Describe the gross appearance of pheochromocytoma
tan and well defined tumor
pheochromocytoma stains positive for what two stains?
chromogranin A and S100 (stains sustentacular cells)
What are four things you can test to confirm the presence of a pheochromocytoma?
Catecholamines Vanillylmandelic acid (VMA) Metanephrines Chromogranin A (you can also use nuclear medicine scan to locate the tumor)
Which four genetic defects lead to pheochromocytomas?
RET gene
SDH gene
VHL gene
NF1 gene
What’s a tumor of the sympathetic ganglia and adrenal medulla
neuroblastoma
What’s the most common solid malignancy of childhood and infancy?
brain tumors
What’s the second most common solid malignancy of childhood and infancy?
neuroblastoma
catecholamines metabolites can be used to do which of the following?
a. screen for neuroblastoma
b. confirm neuroblastoma
a. screen for neuroblastoma
Which of the following symptoms is a more specific sign of cushing syndrome?
a. depression
b. abdominal striae
c. buffalo hump
c. moon face
b. abdominal striae
spontaneous bruising, proximal myopathy, and abdominal striae are the most specific signs
Whats needed to diagnose cushing syndrome?
at least two positive tests
Explain how the diurnal variation test of cushing syndrome works?
test cortisol in the morning and at midnight, midnight test concentration should be 75% of the morning concentration
What does it indicate when a patient has a positive low-dose dexamethasone suppression test?
that he has cushing (but we don’t know the cause)
Patient with cushing has high glucose, explain the etiology?
cortisol acts against insulin (it releases the sugar from cells)
Addisons disease that was caused by a pituitary adenoma causes which of the following?
a. high cortisol
b. low ACTH
c. high ACTH
b. low ACTH
Describe the levels of cortisol, aldosterone, and ACTH in primary adrenal insufficiency?
cortisol low
aldosterone low
ACTH high
Describe the levels of cortisol, aldosterone, and ACTH in secondary adrenal insufficiency?
cortisol low
aldosterone normal (electrolytes not changed)
ACTH low
Which of the following causes hyperpigmentation?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
a. primary adrenal insufficiency
Which of the following has normal electrolyte balance?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
b. secondary adrenal insufficiency
because of the presence of aldosterone
How much of the adrenal cortex must be destroyed for the effects to be evident?
90%
Which of the following causes paleness?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
b. secondary adrenal insufficiency
You suspect a patient of adrenal insufficiency and test his cortisol level at 9 AM, which of the following excludes adrenal insufficiency?
a. low cortisol levels
b. normal cortisol levels
c. high cortisol levels
c. high cortisol levels
(a normal result doesn’t exclude adrenal insufficiency. WHY? because a patient with low cortisol may be stressed because of the doctor/hospital visit and that can increases the stress hormone cortisol, causing it do appear normal)
Which of the following has a high renin concentration?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
a. primary adrenal insufficiency
(adrenal doesn’t produce enough aldosterone, so the Na is low and K is high -> low blood pressure. so the renin would increase to try and increase it back to normal)
Which of the following has an abnormal electrolyte balance?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
a. primary adrenal insufficiency
Which of the following has a low renin concentration?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
b. secondary adrenal insufficiency
not sure about this
Which of the following has a low renin concentration?
a. primary hyperaldosteronism
b. secondary hyperaldosteronism
a. primary hyperaldosteronism
(the adrenal makes too much aldosterone-> Na-K pumps work hard so the Na becomes high and K low. This leads to high BP, the Renin-angiotensin system stops renin secretion to lower the BP)
Which of the following has a high renin concentration?
a. primary hyperaldosteronism
b. secondary hyperaldosteronism
b. secondary hyperaldosteronism
What does a positive synacthen stimulation test indicate?
a. adrenal glands functional
b. adrenal glands non-functional
b. adrenal glands non-functional
(positive tests always indicate abnormalities, so in this case it means that cortisol levels don’t respond to the ACTH analog)
Which of the following has a higher response to ACTH analogs?
a. primary adrenal insufficiency
b. secondary adrenal insufficiency
b. secondary adrenal insufficiency
Patient has hypertension, elevated K levels, hypokalemic alkalosis, and low renin levels. Which is the diagnosis?
a. primary hyperaldosteronism
b. secondary hyperaldosteronism
a. primary hyperaldosteronism
What’s the most common cause of primary hyperaldosteronism?
aldosterone-producing adrenal tumor
What’s th metabolite of androstenedione?
17-oxosteroid
What’s the metabolite of 17- Hydroxyprogesterone?
pregnanetriol
What is the metabolite of cortisol?
tetrahydrocortisol glucuronide
11-Deoxycortisol protein is low in a case of CAH. Which enzyme is low?
a. 11-b-hydroxylase
b. 21- hydroxylase
b. 21- hydroxylase
What gene encodes for 21-hydroxylase?
Cytochrome P450 Family 21 Subfamily A Member 2 (CYP21A2) gene encoding
11-Deoxycortisol protein is high in a case of CAH. Which enzyme is low?
a. 11-b-hydroxylase
b. 21- hydroxylase
a. 11-b-hydroxylase
What gene encodes for 11-b-hydroxylase?
Cytochrome P450 Family 11 Subfamily B Member 1 (CYP11B1) gene
Which of the following is low in a CAH patient?
a. pregnanetriol
b. tetrahydrocortisol glucuronide
c. 17-oxosteroid
b. tetrahydrocortisol glucuronide
no cortisol is made
Where is androstenedione converted to testosterone?
adrenal cortex or in peripheral tissues
Describe the ACTH levels of a CAH patient, why is this?
high ACTH, since the cortisol is low or nonexistent there’s no negative inhibition and the pituitary continuously stimulates the adrenals (->hypertrophy)
Testosterone is converted into dihydrotestosterone by what enzyme? Where does it take place?
5a-reductase in peripheral tissue
Why does a fetus have a bigger suprarenal gland?
fetal adrenal cortisol secretion needed for labor
Which of the following has a greater effect on beta 2 receptors?
a. epinephrine
b. norepinephrine
a. epinephrine
norepinephrine mostly works on alpha receptors
What activates the nicotinic receptors of the medulla?
a. pre-ganglionic fibers of the parasympathetic nervous system
b. post-ganglionic fibers of the parasympathetic nervous system
c. pre-ganglionic fibers of the sympathetic nervous system
d. post-ganglionic fibers of the sympathetic nervous system
c. preganglionic sympathetic nerve fibers (release acetylcholine)
once the nicotinic receptors of the medulla are activated (by acetylcholine) which catecholamines are released? and at which ratio?
epinephrine & norepinephrine at a 4:1 ratio
give two examples of neuroectodermal cells
Chromaffin cells and pheochromocytes
Which of the following is secreted almost entirely from the adrenal medulla?
a. epinephrine
b. norepinephrine
a. epinephrine
only 30% of norepinephrine comes from medulla, where does the rest of the 70% come from?
70% is released from nerve terminals (of the sympathetic nervous system) and diffuses into the vascular system
(post-ganglionic fibers of the sympathetic nervous system)
Which catecholamine receptors decrease insulin secretion in times of stress?
a. a1
b. a2
c. b1
d. b2
e. b3
b. a2
Which catecholamine receptors break down fat tissue in times of stress?
a. a1
b. a2
c. b1
d. b2
e. b3
e. b3
Which catecholamine receptors decrease blood flow to the GI tract in times of stress?
a. a1
b. a2
c. b1
d. b2
e. b3
a. a1
Which catecholamine receptors increase cardiac output in times of stress?
a. a1
b. a2
c. b1
d. b2
e. b3
c. b1
Which catecholamine receptors increase gluconeogenesis in times of stress?
a. a1
b. a2
c. b1
d. b2
e. b3
d. b2
Which of the following is more frequently elevated in Pheochromocytomas?
a. epinephrine
b. norepinephrine
b. norepinephrine
What’s the mechanism of action of hypertension in Pheochromocytomas?
high norepinephrine acts with alpha receptors, which constricts blood vessels and leads to hypertension
What’s the mechanism of action of orthostatic hypotension in Pheochromocytomas?
down-regulation of α-adrenergic receptors resulting from persistent elevation of norepinephrine. Thus the baroreceptor response to blood shifts is blunted.
Which of the following has a lower half-life? why?
a. aldosterone
b. cortisol
a. aldosterone
because it has a lower affinity to albumin, transcortin, and aldosterone binding globin
How does aldosterone affect sodium levels in saliva?
decrease the Na levels
Explain how Atrial natriuretic peptide (ANP) works and its relation with aldosterone
ANP is a natriuretic peptide hormone secreted from the cardiac atria, these cells have volume receptors that respond to increased stretching of the atrial wall due to increased atrial blood volume. They want to reduce the blood volume/pressure, so they increase Na excretion and they stop aldosterone secretion.
Explain how juxtaglomerular cells work and its relation with aldosterone
Juxtaglomerular cells secrete renin in response to a drop in pressure detected by stretch receptors in the vascular walls of the kidney. (secrete renin when they detect low Na) Renin then (together with ACE) help make angiotensin II and that stimulates aldosterone synthesis
Cortisol stimulates which of the following?
a. bone resorption
b. bone formation
a. bone resorption
again, mainly a catabolic hormone
When is cortisol needed to respond to catecholamines?
only when catecholamines are in high concentrations (administered pharmacologically). It increases cardiac output and blood pressure
What does cortisol stimulate in the liver?
gluconeogenesis (only acts anabolically in the liver)
Which of the following cortisol actions is FALSE?
a. depression
b. RBC production
c. ADH stimulation
d. stimulate appetite
c. ADH stimulation
it actually inhibits ADH secretion
A person is running away from a lion, is cortisol necessary to deal with the high amounts of catecholamines in the circulation?
No, they’re only necessary if catecholamines are administered pharmacologically
Which of the following causes hyperpigmentation?
a. cushing syndrome
b. cushing disease
b. cushing disease
high ACTH
A deficiency in which of the following enzymes leads to feminine phenotypes?
a. 11β hydroxylase
b. 17α hydroxylase
c. 21 hydroxylase
b. 17α hydroxylase
What prevents edema in conn’s syndrome?
Atrial natriuretic peptide (ANP)
What’s the most common cause of Addison’s disease?
autoimmune destruction of the adrenal cortex
What’s the second most common cause of Addison’s disease?
Tuberculosis, it infiltrates and destructs the adrenal gland (causes cortisol deficiency -> high ACTH)
Patient comes in complaining of loss of appetite, salt craving, and pigmentation of gums. What’s the diagnosis? explain the cause each symptom
Addison’s
Salt craving= low aldosterone
Loss of appitite= low cortisol
Pigmentation= high ACTH leading to MSH stimulation
How does serotonin increase glucocorticoid secretion?
serotonin enhances CRH, which leads to ACTH release. ACTH activates the adrenal cortex, leading to glucocorticoid release
What converts cholesterol to pregnenolone?
by an enzyme called 20-22 desmolase
Which of the following is more freely available in the plasma?
a. Aldosterone
b. Glucocorticoid
a. Aldosterone
How does epinephrine increase glucocorticoid secretion?
by enhancing ACTH secretion, leading to the activation of the adrenal cortex and glucocorticoid release
How does serotonin increase glucocorticoid secretion?
by enhancing CRH secretion
What enhances the clearance/biotransformation of glucocorticoids?
hyperthyroidism ((phenobarbital rifampine carbamazopine phenytoin *** NOT in drug list))
Which hormones increase transcortin levels?
estrogen, progesterone, and thyroid hormones
What reduces the clearance/biotransformation of glucocorticoids?
estrogen pregnancy hypothyroidism liver disease malnutrition low protein diet
Which of the following does cortisol function as?
a. glucocorticoid
b. mineralocorticoids
c. both
d. neither
c. both
Which of the following does cortisol bind to?
a. albumin
b. transcortin
b. transcortin
Which is more potent?
a. Cortisol
b. Cortisone
a. Cortisol
Which requires a higher dose to produce the same effect?
a. Cortisol
b. Cortisone
b. Cortisone
Which of the following has a higher mineralocorticoid activity?
a. Betamethasone
b. 9-Flurocortisol
c. Triamcinolone
d. Prednisolone
b. 9-Flurocortisol
Which of the following has the highest glucocorticoid activity?
a. Dexamethasone
b. 9-Flurocortisol
c. Triamcinolone
d. Prednisolone
a. Dexamethasone
Dexamethasone & Betamethasone have very high glucocorticoid activity
Which of the following has the highest glucocorticoid activity?
a. Aminoglutethimide
b. Betamethasone
c. Cortisone
d. Dexamethasone
b. Betamethasone
Betamethasone>Dexamethasone
Which of the following bind to albumin?
a. glucocorticoids
b. glucocorticoids synthetic analogs
b. glucocorticoids synthetic analogs
Which of the following glucocorticoid functions is FALSE?
a. increase vitamin D action
b. decrease protein synthesis
c. increase myocardium sensitivity to catecholamines
d. decrease calcium absorption
a. increase vitamin D action
antagonize the action of vit D
Which of the following have better oral bioavailability? Why?
a. glucocorticoids
b. glucocorticoids synthetic analogs
b. glucocorticoids synthetic analogs
cortisol gets degraded by first-pass metabolism in the liver
Which of the following do glucocorticoids NOT inhibit?
a. histamine release
b. neutrophils
c. antibody formation
d. lymphocytes
b. neutrophils
it inhibits their migration, so they increase in the blood
How do glucocorticoids decrease vascular permeability?
by increasing the expression of vasocortin
Explain how glucocorticoid use can lead to secondary osteoporosis?
Glucocorticoids antagonize the action of vitamin D, thus decreasing calcium absorption. Low calcium causes release of parathyroid hormone. PTH breaks down bone to raise calcium levels.
Glucocorticoids can also suppress collagen formation which is an organic component in the bone
Which adrenal cortex zones are affected during Addison’s disease?
zona glomerulosa and fasiculata
What medications are used to treat Addison’s disease?
you need both mineralocorticoid and glucocorticoid activity, so give cortisol and flurocortisol
Why are glucocorticoids used to treat asthma?
Increase expression of beta 2 adrenoreceptor, which increase catecholamines (primarily epinephrine) that then acts on both beta 1&2 receptors
Activation of beta 2 receptor causes relaxation of the smooth muscles of the lungs
Which inhibits both prostaglandins and leukotrienes? explain the mechanism.
a. annexin A1
b. NSAIDs
a. annexin A1
(aka lipocortin) its an endogenous inhibitor of phospholipase A2. Phospholipase A2 is responsible for the formation of arachidonic acid, which starting material in the synthesis of prostaglandins and the leukotrienes
Why are Glucocorticoids contraindicated in patients with Glaucoma?
they increase intraocular pressure
Why are glucocorticoids used to treat septic shock?
it increases the cardiac output, restores blood pressure, and restore plasma glucose levels
Which do NSAIDs inhibit?
a. prostaglandins
b. leukotrienes
a. prostaglandins
Describe the binding of transcortin (CBG) to glucocorticoids
high affinity but low capacity
80% of glucocorticoids are bound to CBG
What hormone decreases transcortin levels?
testosterone
Which converts cortisone to physiologically active cortisol?
a. 11-β-hydroxysteroid dehydrogenase type-1
b. 11-β-hydroxysteroid dehydrogenase type-2
a. 11-β-hydroxysteroid dehydrogenase type-1
How do glucocorticoids affect muscle capacity when taken acutely versus chronically?
increase muscle work capacity acutely, but is associated with myopathy chronically
Which has a side effect of oral candida?
a. Metyrapone
b. Beclomethasone
c. Methylprednisolone/polyethylene glycol
b. Beclomethasone
Which converts cortisol to inactive cortisone?
a. 11-β-hydroxysteroid dehydrogenase type-1
b. 11-β-hydroxysteroid dehydrogenase type-2
b. 11-β-hydroxysteroid dehydrogenase type-2
Which is used to treat rheumatoid arthritis?
a. Metyrapone
b. Beclomethasone
c. Methylprednisolone/polyethylene glycol
c. Methylprednisolone/polyethylene glycol
How can we minimize the side effects of glucocorticoid drugs without changing the dosage?
by changing the route of administration
Describe the solubility of most glucocorticoids? What does that mean for their route of administration?
very lipid-soluble. Most of them cannot be taken by IV
What are the only glucocorticoids that can be given via IV?
hydrocortisone succinate/phosphate
How can we minimize the side effects of glucocorticoid drugs by changing the dosage? (2 ways)
1- mimic the circadian rhythm by giving two doses in the morning and one at night
2- alternative therapy (day on, day off) which has less effect on the hormonal axis
What inhibits the enzyme 11 Beta hydroxylase?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
c. Metyrapone
What is used for the treatment of Cushing’s syndrome?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
b. Mifepristone
What inhibits many enzymes of the steroid biosynthetic pathway?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
d. Ketoconazole
What is an anti-fungal?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
d. Ketoconazole
What inhibits the rate-limiting enzyme in the biosynthesis of glucocorticoids?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
a. Aminoglutethimide
What inhibits the receptor for glucocorticoids?
a. Aminoglutethimide
b. Mifepristone
c. Metyrapone
d. Ketoconazole
b. Mifepristone
Which is used to differentiate between the different forms of cushing’s syndrome?
a. Metyrapone stimulation test
b. Dexamethasone suppression test
c. ACTH and cosyntropin test
b. Dexamethasone suppression test
Patient takes a metyrapone suppression test and we find out that his ACTH levels are low. What does this indicate and why?
he has a secondary adrenal insufficiency. Metyrapone causes low cortisol levels, this means no negative feedback inhibition is available. ACTH should be high, and if it isn’t then there’s a problem with the pituitary.
Patient takes an ACTH and cosyntropin test and his cortisol levels remain constant. What does this indicate and why?
Primary adrenal insufficiency. Cosyntropin is an ACTH analog, so if high ACTH doesn’t stimulate glucocorticoid synthesis that means there is a defect in the adrenal gland.
A patient with an adrenal insufficiency takes an ACTH and cosyntropin test and his cortisol levels increase. What does this indicate and why?
Secondary adrenal insufficiency. Cosyntropin is an ACTH analog, so if high ACTH stimulates glucocorticoid synthesis that means that there’s nothing wrong with the adrenal, and the pituitary is just not stimulating it.
Which enzyme is responsible for the conversion of deoxycortisol to cortisol?
11 beta hydroxylase enzyme
(Google says: In the human adrenal, the mitochondrial cytochrome P450 enzyme CYP11B1 catalyzes the conversion of 11-deoxycortisol to cortisol)
What does administering dexamethasone in a normal patient cause?
suppress ACTH which then suppresses cortisol production
high-dose dexamethasone test suppressed the cortisol concentration in a patient with cushing syndrome. What’s the cause of cushing?
pituitary (cannot be ectopic or adrenal because they wouldn’t respond)
Which of the following has low renin levels?
a. primary hyperaldosteronism
b. secondary hyperaldosteronism
a. primary hyperaldosteronism
How can glucocorticoids result in secondary diabetes?
they induce hepatic gluconeogenesis and inhibit the uptake of glucose by the cells
Which of the following causes cushing syndrome?
a. adrenal adenoma
b. adrenal carcinoma
c. both
c. both
Give an example of a cancer that may induce cushing syndrome
small cell lung carcinoma
Which is more common in the adrenal gland?
a. primary pituitary tumors
b. metastatic tumors
b. metastatic tumors
A patient with known hyperaldosteronism complains of numbness and tingling. What causes this?
low potassium levels
A deficiency of which hormone(s) causes hyponatremia?
cortisol and aldosterone
A deficiency of which hormone(s) causes hyperkalemia
aldosterone
A deficiency of which hormone(s) causes hypoglycemia?
cortisol
T/F: autoimmune adrenalitis (Addison’s disease) is almost always found as an isolated disease
false, its associated with other autoimmune diseases in half the cases (EX/autoimmune polyendocrinopathy syndromes (APS))
Which age does Waterhouse-Friderichsen Syndrome most likely affect?
children
How can abrupt cessation of steroid therapy adrenal crisis?
Longe term corticosteroid therapy shuts off ACTH stimulation to the adrenals, leading to adrenal atrophy. Studen withdrawal will not allow it to recover gradually and resume normal function-> crisis
Why does the tumor surface of pheochromocytoma turn dark brown when immersed in potassium dichromate solution?
due to oxidation of stored catecholamines synthesized by the tumor
What do you call an extra-adrenal pheochromocytoma?
paraganglioma (a rare neuroendocrine neoplasm that may develop at various body sites, including the head, neck, thorax and abdomen)