Endocrinology

Question 1

The term hormone is derived from a Greek phrase meaning:

Answer 1

“To set in motion”

Question 2

Triad of MEN1

Answer 2

Parathyroid, pancreatic islet, and pituitary tumors

Question 3

Triad of MEN2

Answer 3

Medullary thyroid carcinoma, pheochromocytoma, hyperparathyroidism

Question 4

MEN1 gene encodes this putative tumor-suppressor gene

Answer 4

Menin

Question 5

MEN1 gene is located in:

Answer 5

11q13

Question 6

MEN2 is caused by activating mutations of this gene

Answer 6

RET protooncogene

Question 7

Three major types of endocrine conditions

Answer 7

Hormone excess
Hormone deficiency
Hormone resistance

Question 8

Two prevalent causes of hormone deficiency

Answer 8

Hashimoto thyroiditis, type 1 diabetes mellitus

Question 9

Hormone resistance in obesity

Answer 9

Leptin

Question 10

Hormone resistance in catabolic states

Answer 10

Growth hormone

Question 11

Most important diagnostic tools in endocrinology

Answer 11

Immunoassays

Question 12

Cortisol values increase by how much between midnight and dawn

Answer 12

Fivefold

Question 13

Screening for hypothyroidism in women (age, frequency of testing)

Answer 13

Age 35, every 5 years thereafter

Question 14

Screening test for vitamin D deficiency

Answer 14

Serum 25-OH vitamin D

Question 15

Acquired pituitary failure in the newborn is caused by:

Answer 15

Birth trauma

Question 16

Mutations in this gene cause septo-optic dysplasia

Answer 16

HESX1 gene

Question 17

Hypothalamic dysfunction and hypopituitarism resulting from dysgenesis of the septum pellucidum or corpus callosum

Answer 17

Septo-optic dysplasia

Question 18

Syndrome resulting from defective hypothalamic GnRH synthesis

Answer 18

Kallman syndrome

Question 19

Initial genetic cause of Kallman syndrome

Answer 19

KAL gene

Question 20

Treatment of Kallman syndrome that restores fertility

Answer 20

SC pulsatile GnRH

Question 21

This very rare genetically heterogeneous disorder is characterized by mental retardation, renal abnormalities, obesity, and hexadactyly, brachydactyly, or syndactyly.

Answer 21

Bardet-Biedl syndrome

Question 22

Most patients with Bardet-Biedl syndrome are blind by what age?

Answer 22

30

Question 23

This is a contiguous gene syndrome that results from deletion of the paternal copies of the imprinted SNRPN gene, the NECDIN gene, and possibly other genes on chromosome 15q.

Answer 23

Prader-Willi syndrome

Question 24

The development of hypopituitarism develops over how many years after cranial irradiation?

Answer 24

5-15 years

Question 25

Most common hormone deficiency after cranial irradiation

Answer 25

Growth hormone

Question 26

Second most common hormone deficiency after cranial irradiation

Answer 26

Gonadotropin

Question 27

Third most common hormone deficiency after cranial irradiation

Answer 27

ACTH

Question 28

This occurs most often in post-partum women; it usually presents with hyperprolactinemia and MRI evidence of a prominent pituitary mass that often resembles an adenoma, with mildly elevated PRL levels.

Answer 28

Lymphocytic hypophysitis

Question 29

Treatment of lymphocytic hypophysitis

Answer 29

Glucocorticoid

Question 30

Visual recovery after sellar surgery of apoplexy is inversely correlated with:

Answer 30

Length of time after the acute event

Question 31

ACTH reserve is most reliably assessed by:

Answer 31

ACTH and cortisol levels during insulin-induced hypoglycemia

Question 32

Normal GH response to insulin-induced hypoglycemia, GHRH, L-arginine, or L-dopa

Answer 32

GH >3 mcg/L

Question 33

Insulin dose in insulin tolerance test

Answer 33

0.05-0.15 U/kg

Question 34

Glucose level should be below this value for testing GH response to hypoglycemia

Answer 34

<40 mg/dL

Question 35

Cortisol response in insulin tolerance test

Answer 35

Increase by >7 mcg/dL or to >20 mcg/dL

Question 36

Mean growth velocity in later childhood

Answer 36

~6 cm/year

Question 37

Bone age of peak growth rate in girls

Answer 37

12

Question 38

Bone age of peak growth rate in boys

Answer 38

13

Question 39

Three causes of abrogated GH receptor function

Answer 39

(1) Caloric deprivation and malnutrition (2) Uncontrolled diabetes (3) Chronic renal failure

Question 40

Skelatal maturation is best evaluated by measuring a radiologic bone age, which is baed mainly on the degree of bone growth plate fusion in:

Answer 40

Wrist

Question 41

Dose of GH to restore growth velocity

Answer 41

0.02-0.05 mg/kg

Question 42

Target growth velocity during GH treatment in children

Answer 42

~10 cm/year

Question 43

Treatment of GH insensitivity and growth retardation due to mutations of the GH receptor

Answer 43

IGF-1

Question 44

Sequential order of hormone loss in adult GH deficiency

Answer 44

GH > FSH/LH > TSH > ACTH

Question 45

Previous head and neck irradiation increases mortality rates in patients with hypopituitarism, especially from:

Answer 45

Cerebrovascular disease

Question 46

Most common presenting feature of adult hypopituitarism

Answer 46

Hypogonadism