L12: 2ry HTN & MEN Flashcards

1
Q

Causes of 2ry HTN

A
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2
Q

What control Mineralocorticoids synthesis?

A
  • 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.
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3
Q

Causes of Hyperaldosteronism

A
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4
Q

Causes of 1ry Hyperaldosteronism

A

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

 Bilateral adrenal hyperplasia 60%

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5
Q

Causes of 2ry Hyperaldosteronism

A
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6
Q

Primary Hyperaldosteronism

A

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

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7
Q

Causes of Primary Hyperaldosteronism

A
  • Conn’s syndrome (aldosterone-producing adrenal adenoma) 35%.
  • Bilateral adrenal hyperplasia 60%.
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8
Q

CP of Primary Hyperaldosteronism

A
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9
Q

INVx for Primary Hyperaldosteronism

A
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10
Q

What is the screening test for Primary Hyperaldosteronism?

A

Plasma aldosterone : renin ratio (ARR)

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11
Q

Origin of Pheochromocytoma

A
  • Adrenal Pheochromocytoma —> Over 90%
  • Extra-adrenal Pheochromocytoma –> develop in the paraganglion chromaffin tissue of the sympathetic nervous system.
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12
Q

Def of Pheochromocytoma

A

rare, catecholamine-secreting tumor derived from chromaffin cells

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13
Q

TTT of Primary Hyperaldosteronism

A
  • Surgical for adenoma
  • Medical for hyperplasia (Spironolactone and aldosterone receptor antagonist eplerenone)
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14
Q

Incidence of Pheochromocytoma

A

0.01 - 0.1% of hypertensive population

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15
Q

Rule of 10

  • Pheochromocytoma
A
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16
Q

CP of Pheochromocytoma

A
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17
Q

Managment of Pheochromocytoma

A

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

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18
Q

Dx of Pheochromocytoma

A
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19
Q

what is tyh screening test for Pheochromocytoma?

A

24h urinary catecholamine and metabolites

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20
Q

Genetics in MEN1

A

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

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21
Q

Glands commonly affected in MEN1

A
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22
Q

Tumors in MEN1

A
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23
Q

Genetics in MEN2

A

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

24
Q

MEN2A

A

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

25
Q

Glands commonly affected in MEN2

A

TUMORS in thyroid gland, parathyroid gland or adrenal gland

26
Q

MEN2B

A

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

27
Q

Def of MEN

A
  • 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.
28
Q

Genettics in MEN

A
  • Autosomal dominant inherited disorders
29
Q

Types of MEN

A
30
Q

what is another name for MEN1?

A

(Wermer syndrome)

31
Q

what is the most affected gland in MEN1?

A

parathyroid gland tumors

32
Q

What is th first affected gland in MEN1?

A

parathyroid gland tumors

33
Q

what are manefestations of hypercalcemia?

A
  • Neuropsychiatric
  • Renal Dysfunction
  • Musculoskeletal Symptoms
  • Gastrointestinal Abnormalities
  • Cardiovascular Disease
34
Q

Neuropsychiatric symptoms

  • Due to Hypercalcemia
A

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

35
Q

MSK Symptoms

  • Due to Hypercalcemia
A

 Profound muscle weakness

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

36
Q

Renal Dysfunction

  • Due to Hypercalcemia
A
37
Q

GI Symptoms

  • Due to Hypercalcemia
A
  • Constipation, anorexia, nausea and peptic ulcer (calcium-induces increase in gastrin secretion).
  • In patients with MEN1 → Zollinger-Ellison syndrome may be present.
38
Q

CVS CP of Hypercalcemia

A
39
Q

Pituitary Gland tumors in MEN1

A
40
Q

Pancreatic tumors in MEN1

  • Site
  • Nature & Incidence
  • Types
A
41
Q

What are the symptoms of zes?

A

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

42
Q

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

A
43
Q

Adrenal Gland Tumors in MEN1

A

These tumors usually are benign.

44
Q

Other Types of tumors in MEN1

A
45
Q

what is another name of MEN2?

A

(Sipple syndrome)

46
Q

Mutations in MEN2

A

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

47
Q

what is the most commonly affected gland in MEN2?

A

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

48
Q

Types of MEN2

A

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

49
Q

MEN2A

A
50
Q

CP of MEN2B

A
51
Q

Dx of MEN

A
52
Q

TTT of MEN

A
53
Q

Surgical TTT of MEN

A
54
Q

Medical TTT of MEN

A

 If surgery not possible and post-operative replacement

 Recent drugs that target the mutant gen

55
Q

Radiation TTT of MEN

A

radiation therapy for tumor ablation