Adrenal Gland Path 2 Smu

Front 1

what is the complication of a deficiency of 11- Beta hydroxylase?

Back 1

excess amounts of mineralocorticoids and therefore HTN

Front 2

which enzyme is deficient if you are producing no sex steroids at all?

Back 2

17 alpha hydroxylase

Front 3

What are the enzyme's that produce an excess of sex hormones?

Back 3

- 21 Hydroxylase defny
- 11 Beta hydroxylase defny

Front 4

What type of syndrome is the androgenital syndrome?

Back 4

autosomal recessive enzyme defect

Front 5

What are the two enzymes that are mostly deficient in androgenital syndrome?

Back 5

-21 hydroxylase defny
- 11 beta hydroxylase defn

Front 6

What is the morphology of adrenogenital syndrome?

Back 6

bilateral congenital adrenal hyperplasia= CAH (with ALL 3 deficiencies)

Front 7

What are the clinical fts of Androgenital syndrome with the exception of 17 Hydroxylase defny

Back 7

female: clitoral hypertrophy, pseudomorphism
Oligomenorrhea, hirsuitism, acne
Male: precocious puberty- accentuation of existing characteristics

Front 8

What occurs as a result of 11 beta hydroxylase defny?

Back 8

11 deoxyc, no GC- increase in ACTH= CAH
Na+ retention, HTN

Front 9

What occurs as a result of 21 hydroxylase defny?

Back 9

increase in sex hormone, no MC/GC increase in ACTH- CAH
Na+ wasting, HTN

Front 10

What is the morphology of adrenogenital syndrome?

Back 10

ALL must have GC defny, ALL must have ACTH defny,

Front 11

What is an alternative name for adrenogenital syndrome?

Back 11

CAH= congenital adrenal hyperplasia

Front 12

what is MEN?

Back 12

Multiple endocrine neoplasia- mutliple glands

Front 13

What is the mode of inheritance of MEN?

Back 13

inherited disease
-occurs in younger age
- multiple organs affected

Front 14

What is seen in the scar on type 1 MEN?

Back 14

the liver, the spleen and a pancreatic tumor(islet cell tumor- therefore it must be associated with gastrinoma)

Front 15

What gastric tumor is associated with MEN 1?

Back 15

Gastrinoma = Zollinger Ellison's disease

Front 16

What is seen in MEN 1- wermer syndrome?

Back 16

- Prolactinoma: mass effect + amenorrhea/ galactorrhea, bone fracture
- Primary parathyroid Hyperplasia: hypercalcemia- kidney stone, acute pancreatitis
- Pancreatic tumor= gastrinoma: multiple non healing ulcers

Front 17

21M, presents multiple non healing gastrinomas

Back 17

They have zollinger ellisons, have to think about prolactinomas and hypercalcemia

Front 18

What is the mode of inheritance of MEN2A

Back 18

autosomal dominant

Front 19

What is the pathogenesis of MEN2A?

Back 19

RET protoncogene activation

Front 20

If a 10y has a family hx of MEN 2 A and they perform a test where the child is postive for RET protoncogene, what do you do next?

Back 20

You remove the thyroid gland- as medullary carcinoma is the most difficult to control, it is the most dangerous

Front 21

What is another name for MEN 2A?

Back 21

Sipple syndrome

Front 22

What is the presentation of a MEN2A patient?

Back 22

1. HTN= pheochromocytoma
2. Hypercalcemia= primary parathyroid hyperplasia
3. medullary carcinoma
Takes 10 years each, therefore can die at age 30

Front 23

What is another name for MEN2 B?

Back 23

William syndrome

Front 24

What is unique about MEN2B?

Back 24

It looks like marfans and has neuromas( schwanoma, or neurilemoma- antony A, antony B and Verocay Bodies

Front 25

What is the marker of MEN2B?

Back 25

Calcitonin- as pt has a meduallary carcinoma

Front 26

What is the marker of MEN2A?

Back 26

Calcitonin and calcium

Front 27

What are the diseases associated with MEN 2B?

Back 27

Medullary carcinoma of the thyroid
Pheochromocytoma
Mucosal/intestinal neuromas

Front 28

What are the diseases of they hyperfunctioning adrenal cortex?

Back 28

- cushing syndrome (increase in GC)
- Conn syndome (increase in aldosterone)
- Adrenogenital syndrome (increase in androgen)
Adrenal tumors

Front 29

What are the diseases of hypofunctioning adrenal cortex?

Back 29

- Addison's disease (chronic)
- Waterhouse- Friedrichsen Syndrome (acute)

Front 30

What is the etiology of Cushing syndrome?

Back 30

- ACTH- secreting pituitary adenoma of basophilic cells
- adrenal cortical tumor of ZONA Fasiculata
- Ectopic ACTH: Small cell carcinoma of the lung, RCC, carcinoid tumor
- Exogenous drug intake: RA, leukemia

Front 31

What are the lab findings in Cushings?

Back 31

increase in cortisol

Front 32

What is the morphology of ACTH secreting pituitary adenoma?

Back 32

increase in cortisol, increase in ACTH- bilateral hyperplasia

Front 33

What is the morphology of tumor of zona fasiculata?

Back 33

increase in cortisol, decrease in ACTH- adrenal cortical adenoma

Front 34

How would you describe adrenal corticoid tumor of zona fasiculata?

Back 34

composed of yellow, firm tissue, well circumscribed

Front 35

What is the morphology ectopic ACTH?

Back 35

increase in cortisol, increase in ACTH

Front 36

What is the morphology of exogenous drug intake?

Back 36

increase in cortisol, decrease in ACTH,= bilateral adrenal cortical atrophy

Front 37

What is the diagnosis of cushing syndrome?

Back 37

-loss of variation of cortisol level- increased level in evening
- high serum and urine cortisol
- Dexamethasone Suppression test: high dose- suppresion of ACTH= decrease in cortisol

Front 38

When do you use the dexamethasone test in cushings?

Back 38

to identify pituitary onset of cushing syndrome

Front 39

What are the c/f of Cushings syndrome

Back 39

over action of GC
- erythropoiesis- plethora of face
- lipogenesis- weight gain, central fat depostion, moon face
- gluconeogenesis- hyperglycemia
- hypercalcemia- osteoporosis, fracture
- hyperpigmentation in trunk (striae)
- atrophy of fast twitch muscle
-delayed wound healing (easy bruising, inhibits fibroblast decrease in collagen)

Front 40

Which area is affected in conn syndrome?

Back 40

tumor of zona glomeulosa