Adrenal Gland: 1/3

Question 1

derivation and type of cells in medulla of adrenal

Answer 1

i. Embryonic Nueral Crest cells

Chromaffin Cells

Question 2

Controlled by: medulla and cortex

Answer 2

medulla- direct innv

cortex- HP axis and RAAS

Question 3

What controls Aldosterone production

Answer 3

RAAS. NOT ACTH

Question 4

cortisol has negatoive fb on what

Answer 4

Hypo and pit

Question 5

Primary Hyperaldosteronism aka?

Answer 5

Conn syndrome

Question 6

MC cuase of Primary Hyperaldosteronism

Answer 6

Adrenocortical neoplasm: solitary aldosterone-secreting adenoma

Question 7

Primary Adrenocortical hyperplasia: cause and describe

Answer 7

Bilateral nodular hyperplasia of the adrenals

caused by overacrtivity of aldosterone synthase gene (18HSD)

Question 8

Under what condition is Aldosterone production no longer controlled by RAAS. Now controlled by ACTH?

Answer 8

*****Glucocorticoid-remedial hyperaldo

Question 9

How does Glucocorticoid-remedial hyperaldo couple ACTH to Aldo production?

Answer 9

Fusion between 11B-hydroxylase gene and aldosterone synthase gene (18HSD)

Question 10

Renin, K, Na levels in Primary Hyperaldosteronism

Answer 10

Renin: low
K+ = low
Na = high

Question 11

4 sx of hypokalemia

Answer 11

Weakness, parathesias, visual disturbances, tetany

Question 12

how to dx Primary Hyperaldosteronism

Answer 12

high Aldo with low renin

Question 13

Cushings syndrome defintion

Answer 13

high cortisol

Question 14

Cushing’s disease will alwayd be due to?

Answer 14

Apituitary tumor–> ACTH –> cortisol

Question 15

3 casuses of Cushing syndrome

Answer 15

• Ectopic ACTH production (SCC of lung)
• Autonomous Cortisol Production (Aderenal)
Iatrogenic

Question 16

Consistent sign of Cushings in Children

Answer 16

growth failure with weight gain

Question 17

What two cortisol tests help dx cushings

Answer 17

24 hour urinary free cortisol

Late-night salivary cortisol (level is normally lowest at night)

Question 18

hat effect does Low-dose dexamethasone (corticosteroid) suppression test have on cushing syndrome? why?

Answer 18

None- problem isn’t in HPA axis

Question 19

What diagnostic effect will high-0dose dexamethasone have

Answer 19

supression of cushings disease: pit adenoma (no effect on SCC of lung)

Question 20

Who to Screen for Cushing Syndrome (3)

Answer 20

1. pts with unusual features: HTN, T2DM, thin skin
2. progessive features
3. kids with reduced height but ^ weight

Question 21

Addison disease aka?

Answer 21

Primary Adrenal insufficiency

Question 22

Primary Adrenal insufficiency basic derangement/mechanism

Answer 22

low cortisol (and Aldo) –> loss of negative fb –> increased ACTH production

Question 23

Causes of Primary Adrenal insufficiency (7) and basic cause

Answer 23

Basic: destruction of adrenal cortex

a. Congenital Adrenal Hyperplasia
b. Adrenoleukodystrophy
c. Autoimmune adrenal insufficiency
d. Infection (AIDS, TB, Waterhouse-Friedrichsen Syndrome)
e. Amyloidosis, sarcoidosis, hemochromatosis
f. Metastatic cancer
g. Metabolic failure (congenital adrenal hyperplasia, drug indiced ACTH inhibition)

Question 24

Classic SX of Primary Adrenal insufficiency (2)

Answer 24

hyperpigmentation (buccal mucosa, scars)

fatigue

Question 25

cLASSIC LABS IN Primary Adrenal insufficiency (3)

Answer 25

Low Na . High K Low Blood Sugar

Question 26

tx for primary adrenal insufficiency

Answer 26

cortsiol, aldosterone

Question 27

Waterhouse-Friderichsen Syndrome what is it and cause

Answer 27

hemorrhagic necrosis of adrenals dt DIC in young children with Neisseria meningitidis infx. lack of cortisol --> hypotension--> death

Question 28

CP of Waterhouse-Friderichsen syndrome

Answer 28

DIC with purpura (non-blnaching), hypotension--> shock

Question 29

Inheritance pattern of congenital adrenal hyperplasia

Answer 29

AR

Question 30

general cause ofcongenital adrenal hyperplasia

Answer 30

deficiency of enzyme that makes cortisol

Question 31

morphology of adrenals in congenital adrenal hyperplasia

Answer 31

hyperplastic up to 15x normal weight

Question 32

MC deiciency in congenital adrenal hyperplasia

Answer 32

21-hydroxylase

Question 33

congenital adrenal hyperplasia sign in females

Answer 33

ambiguous genitalia

Question 34

If 3B-HSD is absent or deficient --> ??

Answer 34

undervirilized males dt no sex steroid production:

Question 35

Tx of congenital adrenal hyperplasia (3)

Answer 35

i. Cortisol and mineralcorticoid replacement ii. Normalize growth and androgens Salt supplementation (lack of aldo--> salt wasting)

Question 36

What generally causes Secondary Adrenal Insufficiency

Answer 36

Any disorder of the Hypothalamus or Pituitary gland that reduces ACTH

Question 37

When is adrenarche abnormal in boys and girls

Answer 37

boys < 9 | girls < 8

Question 38

Which is typically functional: adrenal carcinomas or adenomas?

Answer 38

Carcinomas are functional

Question 39

how does a carcinoma typically metastasize

Answer 39

via LN

Question 40

Classic triad of Pheochromocytoma

Answer 40

Headache, diaphoresis (sweating), palpitations

Question 41

MC sx of Pheochromocytoma

Answer 41

HTN

Question 42

Pheochromocytoma: 5 rules of 10

Answer 42

10% are: 1. Malignant 2. bilateral 3. extra-adrenal 4. familial syndromes 5. childhood

Question 43

Tx of pheochromocytoma (2)

Answer 43

i. Normalize BP | Excision

Question 44

What familial mutations is pheochromocytoma asssociated with? (4)

Answer 44

MEN2A and MEN2B, VHL, Neurofibromatosis type 1

Question 45

MEN1 is associated with what 3 tumors

Answer 45

Pituitary Pancreatic Parathyroid adenomas

Question 46

What is gene mutation and product in MEN1? oncogene or suppressor?

Answer 46

MEN1 gene encodes menin = tumor suppressor

Question 47

MC manifestation of MEN1

Answer 47

primary hyperparathyroidism

Question 48

mutation and function in MEN2A and MEN2B

Answer 48

both - gain of function in RET

Question 49

3 associations with MEN2A

Answer 49

Pheochromocytoma Medullary CA Parathyroid hyperplasia

Question 50

RET mutations occur in cells of what nature

Answer 50

those that developed from NCCs

Question 51

MEN2B is associated with what tumors (3)

Answer 51

Pheochromocytoma Medullary CA of thryoid Neuromas, gangliomas

Question 52

Marfanoid habitus is associated with what MEN?

Answer 52

MEN2B