L12: 2ry HTN & MEN

Question 1

Causes of 2ry HTN

Answer 1

Question 2

What control Mineralocorticoids synthesis?

Answer 2

• Mineralocorticoids (aldosterone 100–150 micrograms/day) synthesis occurs in the zona glomerulosa, predominantly under the control of the renin– angiotensin system (outer layer of the cortex) although ACTH also contributes to its regulation.

Question 3

Causes of Hyperaldosteronism

Answer 3

Question 4

Causes of 1ry Hyperaldosteronism

Answer 4

 Conn’s syndrome (aldosterone-producing adrenal adenoma) 35%.

 Bilateral adrenal hyperplasia 60%

Question 5

Causes of 2ry Hyperaldosteronism

Answer 5

Question 6

Primary Hyperaldosteronism

Answer 6

The condition of excess aldosterone secretion which may result in Hypertension with Hypokalemia and Alkalosis

Question 7

Causes of Primary Hyperaldosteronism

Answer 7

• Conn’s syndrome (aldosterone-producing adrenal adenoma) 35%.
• Bilateral adrenal hyperplasia 60%.

Question 8

CP of Primary Hyperaldosteronism

Answer 8

Question 9

INVx for Primary Hyperaldosteronism

Answer 9

Question 10

What is the screening test for Primary Hyperaldosteronism?

Answer 10

Plasma aldosterone : renin ratio (ARR)

Question 11

Origin of Pheochromocytoma

Answer 11

• Adrenal Pheochromocytoma —> Over 90%
• Extra-adrenal Pheochromocytoma –> develop in the paraganglion chromaffin tissue of the sympathetic nervous system.

Question 12

Def of Pheochromocytoma

Answer 12

rare, catecholamine-secreting tumor derived from chromaffin cells

Question 13

TTT of Primary Hyperaldosteronism

Answer 13

• Surgical for adenoma
• Medical for hyperplasia (Spironolactone and aldosterone receptor antagonist eplerenone)

Question 14

Incidence of Pheochromocytoma

Answer 14

0.01 - 0.1% of hypertensive population

Question 15

Rule of 10

• Pheochromocytoma

Answer 15

Question 16

CP of Pheochromocytoma

Answer 16

Question 17

Managment of Pheochromocytoma

Answer 17

Surgical if resectable:
- Medical Pre-operative treatment is mandatory with Combined α & β blockade

• Alpha blockade must precede beta blockade to avoid severe hypertension

If operation is not possible:
- long term Combined α and β blockade

Question 18

Dx of Pheochromocytoma

Answer 18

Question 19

what is tyh screening test for Pheochromocytoma?

Answer 19

24h urinary catecholamine and metabolites

Question 20

Genetics in MEN1

Answer 20

MEN 1 is an AD disorder due to loss-of-function mutations of the MEN 1 gene.

Question 21

Glands commonly affected in MEN1

Answer 21

Question 22

Tumors in MEN1

Answer 22

Question 23

Genetics in MEN2

Answer 23

AD condition due to activating mutations of the RET proto-oncogene

Question 24

MEN2A

Answer 24

MTC (medullary thyroid cancer) in combination with PCC (Pheochromocytoma) and parathyroid tumors.

Question 25

Glands commonly affected in **MEN2**

Answer 25

TUMORS in thyroid gland, parathyroid gland or adrenal gland

Question 26

MEN2B

Answer 26

MTC in association with PCC and mucosal neuromas, marfanoid habitus, megacolon

Question 27

Def of **MEN**

Answer 27

- Is a group of disorders that affect the endocrine system. - There are tumors in multiple endocrine glands that may be malignant or benign and may cause the glands to become overactive and overproduce hormones.

Question 28

Genettics in **MEN**

Answer 28

- Autosomal dominant inherited disorders

Question 29

Types of **MEN**

Answer 29

Question 30

what is another name for **MEN1**?

Answer 30

(Wermer syndrome)

Question 31

what is the most affected gland in **MEN1**?

Answer 31

parathyroid gland tumors

Question 32

What is th first affected gland in **MEN1**?

Answer 32

parathyroid gland tumors

Question 33

what are manefestations of hypercalcemia?

Answer 33

- Neuropsychiatric - Renal Dysfunction - Musculoskeletal Symptoms - Gastrointestinal Abnormalities - Cardiovascular Disease

Question 34

Neuropsychiatric symptoms - Due to Hypercalcemia

Answer 34

 Anxiety, depression, cognitive dysfunction  Lethargy, confusion → stupor, and Coma.

Question 35

MSK Symptoms - Due to Hypercalcemia

Answer 35

 Profound muscle weakness  Bone pain due to reduction in cortical bone mass may occur in individuals with hyperparathyroidism.

Question 36

Renal Dysfunction - Due to Hypercalcemia

Answer 36

Question 37

GI Symptoms - Due to Hypercalcemia

Answer 37

- Constipation, anorexia, nausea and peptic ulcer (calcium-induces increase in gastrin secretion). - In patients with MEN1 → Zollinger-Ellison syndrome may be present.

Question 38

CVS CP of Hypercalcemia

Answer 38

Question 39

Pituitary Gland tumors in **MEN1**

Answer 39

Question 40

Pancreatic tumors in **MEN1** - Site - Nature & Incidence - Types

Answer 40

Question 41

What are the symptoms of zes?

Answer 41

(due to elevated levels of gastrin):  Peptic ulcers  Inflammation of the esophagus  Diarrhea and abdominal pain.

Question 42

what is the most common functional endocrine pancreatic tumor in **MEN1**? and what is the second most common? and what is the TTT?

Answer 42

Question 43

Adrenal Gland Tumors in **MEN1**

Answer 43

These tumors usually are benign.

Question 44

Other Types of tumors in **MEN1**

Answer 44

Question 45

what is another name of **MEN2**?

Answer 45

(Sipple syndrome)

Question 46

Mutations in **MEN2**

Answer 46

MEN2A and MEN2B are caused by mutations in the RET gene.

Question 47

what is the most commonly affected gland in **MEN2**?

Answer 47

People with multiple endocrine neoplasia type 2 (MEN2) have a 98 – 100 % chance of developing medullary thyroid cance

Question 48

Types of **MEN2**

Answer 48

MEN2 is divided into three types:  Type 2A (MEN2A)  Type 2B (MEN2B)  Familial Medullary Thyroid Carcinoma (FMTC).

Question 49

MEN2A

Answer 49

Question 50

CP of MEN2B

Answer 50

Question 51

Dx of **MEN**

Answer 51

Question 52

TTT of **MEN**

Answer 52

Question 53

Surgical TTT of **MEN**

Answer 53

Question 54

Medical TTT of **MEN**

Answer 54

 If surgery not possible and post-operative replacement  Recent drugs that target the mutant gen

Question 55

Radiation TTT of **MEN**

Answer 55

radiation therapy for tumor ablation