Endocrine Part 5

Question 0

What are 2 diagnostic clues for Conn’s syndrome?

Answer 0

unexplained hypokalemia and hypernatremia

Question 1

Usually due to the presence of a functional adrenal adenoma or adrenal hyperplasia

Answer 1

Conn’s syndrome

Question 2

What condition is synonymous with adrenal hyperfunction?

Answer 2

Cushing’s syndrome

Question 3

What is Cushing’s syndrome most commonly due to?

Answer 3

long term use of pharmacologic doses of gluccocorticoids

Question 4

Drugs such as prednisone suppress what 2 things?

Answer 4

ACTH and CRH

Question 5

If Cushing’s syndrome is not due to pharmaceuticals, what is it due to?

Answer 5

tumors (adrenal or pit– particularly tumors involving the corticotroph cell line)

Question 6

Prolonged excess ACTH will eventually result in what?

Answer 6

adrenal hyperplasia

Question 7

Adenomas within the … can result in … which can lead to….

Answer 7

zona fasiculata; excess cortisol; primary Cushing’s syndrome

Question 8

Non-pit and non-adrenal tumors can also result in Cushing’s syndrome as a result of what?

Answer 8

ectopic ACTH

Question 9

Most common tumor associated with the para-neoplastic production of ACTH

Answer 9

carcinoma of the lung

Question 10

80% of Cushing’s syndrome tumors are ACTH dependent. What are 2 tumors leading to this?

Answer 10

Pituitary tumors, lung cancer and other tumors

Question 11

20% of Cushing’s syndrome tumors are independent of ACTH. What are 2 examples?

Answer 11

Benign adrenal tumors/adenomas, malignant adrenal tumors i.e. adrenal cell carcinoma

Question 12

Condition of adrenocortical insufficiency

Answer 12

Addison’s disease

Question 13

What is characteristic of primary Addison’s disease?

Answer 13

Adrenal gland itself is damaged

Question 14

What is characteristic of secondary Addison’s disease?

Answer 14

insufficient ACTH results in atrophy of adrenal cortex

Question 15

What is the most common cause of primary Addison’s disease?

Answer 15

autoimmune destruction of the adrenal cortex

Question 16

What are 3 other autoimmune conditions associated with primary Addison’s disease?

Answer 16

Type 1 DM, Hashimoto’s thyroiditis, Vitiligo

Question 17

What is due to markedly increased levels of ACTH?

Answer 17

hyperpigmentation

Question 18

due to inadequate secretion of ACTH by the anterior pituitary

Answer 18

secondary Addison’s disease

Question 19

Adrenal function loss due to trauma or adrenal hemorrhage from sepsis

Answer 19

Waterhouse-Frederickson syndrome

Question 20

Patients with secondary Addison’s disease have diminished levels of … thus, no stimulation of melanocyte production of melanin

Answer 20

ACTH

Question 21

W-F syndrome is usually caused by what?

Answer 21

severe bacterial infection

Question 22

What is the most common bug leading to W-F syndrome?

Answer 22

n. meningitidis

Question 23

inborn error of metabolism with a specific deficiency of one or more of the enzymes involved in cortisol synthesis

Answer 23

congenital adrenal hyperplasia (CAH)

Question 24

What deficiency accounts for 95% of CAH cases?

Answer 24

21-Hydroxylase deficiency

Question 25

Results in the blockage of cortisol production and causes a subsequent increase in ACTH levels

Answer 25

21-Hydroxylase deficiency

Question 26

What 2 things are increased with 21-hydroxylase deficiency?

Answer 26

17-Hydroxyprogesterone and androstenedione

Question 27

Blockage of cortisol in 21-hydroxylase results in...

Answer 27

increased shunting to cortisol precursors

Question 28

ACTH is (high/low) with 21-Hydroxylase deficiency

Answer 28

high

Question 29

Most significant pathological condition associated with the adrenal medulla

Answer 29

pheochromocytoma

Question 30

catecholamine secreting tumor of the chromaffin cells within the adrenal medulla

Answer 30

pheochromocytoma

Question 31

hallmark symptom of pheochromocytoma

Answer 31

hypertension that may be variable and very labile

Question 32

Think of what in a patient with pheochromocytoma?

Answer 32

MEN IIa or IIb

Question 33

Associated with the presence of a pheochromocytoma, medullary carcinoma of the thyroid, and/or hyperparathyroidism

Answer 33

MEN IIa

Question 34

associated with the presence of a pheochromocytoma, muscosal neuromas, and/or hyperparathyroidism

Answer 34

MEN IIb

Question 35

Biochemically and clinically asymptomatic adrenal masses found incidentally as a result of unrelated imaging

Answer 35

Incidentalomas

Question 36

What percentage of all adrenal tumors are found to be malignant?

Answer 36

1%

Question 37

What are 3 examples of primary adrenal cancer?

Answer 37

neuroblastomas, adrenocortical carcinomas, minority of adrenal pheochromocytomas

Question 38

Essentially a carcinoma of the nervous system and may be seen anywhere along the sympathetic chain

Answer 38

neuroblastomas

Question 39

Neuroblastomas originate from what cells?

Answer 39

neural crest cells

Question 40

Accounts for approx 10% of all childhood cancers in the US

Answer 40

Neuroblastomas

Question 41

Most common solid, extra-cranial malignancy in infants and children

Answer 41

neuroblastoma

Question 42

What percentage of neuroblastomas occur during the first 8 years of life

Answer 42

90%

Question 43

What percentage of neuroblastomas occur during the first 2 years of life

Answer 43

50%