Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

IM > W2L10 Multiple Endocrine Neoplasia and Autoimmune Disorders > Flashcards

W2L10 Multiple Endocrine Neoplasia and Autoimmune Disorders Flashcards

1
Q

Def men?

A
  • Disorder w neoplasm in >=2 dif hormonal tissues in several members of fam
  • Usually early age
  • 3 well-defined MEN syndromes – MEN 1, MEN 2a, MEN 2b
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Etio men1

A
  • Autosomal dominant trait
  • 3rd/4th decade
  • MEN1 MC
  • High incidence foregut carcinoids
  • Skin lesion(lipoma, facial angiofibroma)
  • Race(unknown)
  • Sex(M=F)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

The men1 is characterized by

A
  1. Neoplasia Parathyroid gland(90%)
  2. EnteroPancreatic tumor(80%)
  3. Ant Pituitary adenomas(55%)
  4. Adrenal adenoma(30%)
  5. Non endocrine tumours
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Men 1. Patho

A

Inactivate mutation of tumor-suppressor gene MEN1 located at chromosome 11

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Etio Primary hyperparathyroidism

A
  • MC manifest MEN1 (95–100%)
  • Hypercalcemia-teenage (hyperplasia parathy gla)
  • Most aff 40y (adenoma parathy gla)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

SS Primary hyperparathyroidism

A
  1. Ca-containing kidney stone
  2. Kidney failure
  3. Nephrocalcinosis
  4. Bone abn(osteoporosis)
  5. GIT & musculoskeletal complaint
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Treatment Primary hyperparathyroidism

A

Criteria surgery

  1. Individual w s ca lvl >12 mg/dL
  2. Ca nephrolithiasis/ renal dysfx
  3. Neuropathic/muscular symptoms
  4. Bone involve(osteopenia)
  5. <50y
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Enteropancreatic tumors

A
  • 2nd MC MEN1,
  • 30% malignant
  • Parallel w hyperparathyroidism
  • Tumor secrete peptide hormone coz specific cli syn
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

List Enteropancreatic tumors

A
  1. Gastrinoma(MC)
  2. Insulinoma(25%)(2nd MC)
  3. Glucagonoma(occasion)
  4. VIPoma(1%)
    - coz profuse watery diarrhea
  5. Somatostatinoma(0.7%)
    - coz dm, steatorrhea
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Gastrinoma

A
  • Result ZES
  • ZES coz by excess gastrin prod
  • > 1/2 of MEN1
  • > 1 gastrin-prod tumor(localize difficult)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Manifestation gastrinomas

A
  1. Esophagitis
  2. Duodenal ulcer
  3. Ulcers involve proximal jejunum
  4. Diarrhea
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Diag gastrinoma

A
  • Inc gastric acid secretion

- Inc basal gastrin lvl in serum >115 pmol/L

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Insulinomas etio & diagnosis

A
  • Most originate in panc bed
  • MC pancic tumor in MEN1
  • May benign/malig

Diag

  • Hypoglycemia(short fast)
  • Elevate s insulin & C-peptide lvl
  • CT/MRI(large)
  • Intraoperative ultrasonography.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Glucagonoma manifest & diag

A
  1. Manifestation:
    - coz syn hyperglycemia,skin rash
    - anorexia, glossitis, anemia, depression, diarrhea, venous thrombosis
  2. Diagnosis(glucagon lvl high)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Pituitary tumors(20-30%) ss?

A
  1. Prolactinoma(MC)
  2. Acromegaly
  3. Cushing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

TTT of Pituitary Tumors

A
  • Prolactinomas w dopamine agonists (bromocriptine, cabergoline, quinagolide)=return s prolactin lvl to normal
  • Surgical resection(xcure but relieve mass eff)
17
Q

Nonendocrine tumor

A
  • Small facial angiofibroma
  • Subcutaneous lipoma
  • Collagenoma(firm dermal nodule)
18
Q

Adrenal adenomas /hyperplasia

A
  • 50% bilateral.

- Benign & nonfunctional

19
Q

MEN 2A (Sipple’s Syndrome)

A
  • Rare familial multiglandular syn(autosomal dominant trait)
  • Mutate on chromosome 10
  • 1st-degree relative may RET mutation
20
Q

Manifestations men2

A
  1. Medullary thy carcinoma(>90%)
  2. Hyperparathyroidism(20–50%), due hyperplasia/multiple adenomas(70%)
  3. Pheochromocytomas(20–35%)
  4. Hirschsprung’s diseas
21
Q

Laboratory Studies men2

A
  • Pentagastrin calcitonin stimulation test(medullary gland)
  • Urinary catecholamines & metanephrines screen(pheochromocytoma)
  • S ca & PTH lvl(hyperparathyroidism)
  • CT/MRI(adrenal)
22
Q

Treatment men 2

A
  • MTC : Total thyroidectomy

- Thy hormone(hypothyroidism)

23
Q

MEN 2B

A

Familial, autosomal dominant multiglandular syn coz by mutation of (RET) on chromosome 10

24
Q

MEN 2B is characterized by

A
  1. Mucosal neuromas (> 90%) w bumpy, enlarged lip & tongue
  2. Marfan-likehabitus(75%)
  3. Adrenal pheochromocytomas(60%)
  4. medullarythyroidcarcinoma(80%)
  5. intestinal ganglioneuromas
  6. skeletal abn(87%)
  7. delay puberty(43%)
25
Q

Follow up of MEN 2

A
  • Monitor recurrence medullary thy car w calcitonin, CEA & +/- provocative calcitonin testing
  • Annual screening(hyperparathyroidism- s ca &PTH lvl MEN 2A
  • Urinary catecholamine lvl annual for pheochromocytoma
  • Medic dosage & adverse eff

IM (17 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions