Endo - FA Patho p 333 - 347 Flashcards

1
Q

High-dose dexamethasone suppression test causes what in Cushings dx?

A

Free cortisol suppression

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2
Q

How many mg is administred during High-dose dexamethasone test?

A

8mg - high dose

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3
Q

3 causes for inc cortisol?

A
  • Exogenous corticosteroids - Primary adrenal adenoma, hyperplasia, or carcinoma - ACTH-secreting pituitary adenoma, paraneoplastic ACTH
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4
Q

Difference between Cushing syndrome and disease?

A

ACTH-secreting pituitary adenoma - C. disease incr Cortisol from variety of conditions - C. syndrome

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5
Q

2 paths causing paraneoplastic secretion of ACTH?

A

small cell lung cancer, bronchial carcinoids

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6
Q

Is adrenal tumor an ACTH-independent or dependent Cushing syndrome?

A

ACTH independent Cushing syndrome

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7
Q

Is ectopic ACTH secretion an ACTH-independent or dependent Cushing syndrome?

A

ACTH dependent

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8
Q

CRH stimulation test helps diagnose which pathology?

A

Cushing disease (inc ACTH) vs ectopic ACTH secretion (no inc of ACTH)

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9
Q

What an alternative test used to dx adrenal insufficiency?

A

Metyrapone stimulation test

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10
Q

Metyrapone blocks which step of cortisol synthesis?, What is normal response to it

A

The last one - 11DOC (deoxycortisol) –> cortisol. Normal response is dec cortisol and compensatory inc in ACTH, 11DOC. If they dont INC, then its a primary/secondary ACTH insufficiency, not a cortisol issue

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11
Q

Pt presents w/ sudden onset of massive hemorrhage and shock. What is the adrenal path?

A

Primary Acute adrenal insufficiency or Waterhouse-Friderichsen syndrome

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12
Q

Competitive athlete on school break shows signs of adrenal insufficiency, which type is his pathology?

A

Tertiary - chronic exogenous steroid use precipitated by abrupt withdrawal

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13
Q

skin and mucosal hyperpigmentation is seen in which type of adrenal insuff.?

A

primary

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14
Q

Why can Cushing lead to coronary a disease?

A

Inc cortisol –> inc stimulation of a1R on arterioles –> hypertension –> CAD

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15
Q

Cause of truncal obesity and buffalo hump in Cushing?

A

Inc cortisol –> glucose in blood –> deposit in fat, leading to inc fat deposition on face, trunk, back

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16
Q

Most accurate test for Cushings?

A

Los dose dexamethasone suppression test

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17
Q

Different pathological features of adrenal cortex with ACTH independent and dep Cushing?

A

In ACTH independent - atrophy in ZF, ZR In ACTH dep (Bilat adrenal hyperplasia) - ZF and ZR are diffuse, nodular

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18
Q

TB can cause what kind of adrenal insuff?

A

Chronic—aka Addison disease. Due to adrenal atrophy or destruction by disease

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19
Q

— is a byproduct of  ACTH production from —-.

A

MSH pro-opiomelanocortin

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20
Q

In which type of Adrenal insuff. is Aldosterone synthesis unaffected?

A

2ndary and tertiary

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21
Q

Primary adrenal insuff. is associated with which autoimmune syndromes?

A

Polyglandular syndromes

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22
Q

Septicemia in Waterhouse Friderichsen is usually caused by what pathogen?

A

Neisseria

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23
Q

Which adrenal insuff. spares the skin and mucosa? and why?

A

Tertiary and Secondary, because there is a decr in ACTH production –> less MSH by product

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24
Q

What is the MC tumor of the adrenal medulla in children?

A

Neuroblastoma

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25
Q

Classic histology presentation of Neuroblastoma:

A

Homer-Wright rosettes and small, round, blue/purple nuclei

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26
Q

2 path from neural crest?

A

Neuroblastoma Pheochromocytoma

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27
Q

Homovanillic acid (HVA) a breakdown product of?

A

Dopamine

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28
Q

Signs of primary adrenal insuff?

A

Hypotension (hyponatremic volume contraction), Hyper Kalemia, metabolic acidosis, skin and mucosal hyperpigmentation

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29
Q

—- is a breakdown product of norepinephrine.

A

vanillylmandelic acid (VMA)

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30
Q

“dancing eyes-dancing feet” are seen in?

A

Neuroblastoma, Subacute sclerosing panencephalitis. - opsoclonus & myoclonus

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31
Q

Name episodic hyperadrenergic symptomsof pheochromocytoma (5 P’s):

A

Pressure (incr BP) Pain (headache) Perspiration Palpitations (tachycardia) Pallor

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32
Q

T or F? Wilms tumor can cross the midline vs. Neuroblastoma which is smooth and unilateral.

A

F - opposite

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33
Q

Why is α-blockade administred in Pheochromocytoma before giving β-blockers prior to tumor resection?

A

to avoid a hypertensive crisis.

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34
Q

What % of Pheo’s calcify and why?

A

10% bc of inc in pTrp

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35
Q

Neuroblastoma has an overexpression of which oncogene?

A

N-myc

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36
Q

What malignancy is associated with c-myc (as opposed to n-myc)

A

Burkitt’s lymphoma

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37
Q

What is a typical histology of Pheochomocytoma?

A

enlarged pleomorphic nuclei, typical of malignancy.

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38
Q

2 tumor markers for Neuroblastoma?

A

Bombesin and neuron specific enolase

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39
Q

Name one path that use Bombesin and neuron-specific enolase as tumor markers?

A

Lung cancer (bronchial carcinoid)

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40
Q

Homer Wright rosettes also seen in what other path?

A

Medulloblastoma

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41
Q

Urine and plasma findings is Pheochromocytoma?

A

 catecholamines and metanephrines in urine and plasma.

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42
Q

Which thyroid disorder is associated with Inc CK?

A

Hypothyroidism - myopathy (Normal Ck in Hyper)

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43
Q

Which thyroid disorder is associated with thinning, fine hair?

A

hyperthyroidism

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44
Q

Thyroid disorder with inc #/sensitivity of B adr receptors?

A

Hyperthyroid

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45
Q

Causes of smooth/diffuse goiter?

A

Graves, Hashimoto, Iodine def, TSH secreting pit adenoma

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46
Q

Causes of nodular goiter?

A

Toxic multinodular goiter, Thyroid adenoma, Thyroid cancer, Thyroid cyst

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47
Q

HLA # associated with Hashimoto’s?

A

HLA - DR3; HLA-DR5

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48
Q

Which thyroid disorder is assoc w/ non Hodgkin Lymphoma?

A

Hashimoto

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49
Q

Most common cause of thyroid disgenesis?

A

Congential hypothyroid (Cretinism)

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50
Q

Neck and jaw pain is assoc with which thyroid disorder?

A

Subacute granulomatous thyroiditis - jaw pain, very tender thyroid

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51
Q

Which disease is assoc with a rock like painless goiter? Why rock-like? (patho)

A

Riedel thyroiditis - thyroid replaced with fibrous tissue w/ inflammatory infiltrate.

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52
Q

What is the Wolff Chaikoff effect?

A

Thyroid gland will downregulate in response to inc iodide

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53
Q

Graves disease has what type of Ig, HS?

A

HS-II, IgG

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54
Q

The release of cytokines in the retroorbital space leads to secretion of _____ by _____ cells? (Eventually resulting in exophthalmus in Graves)

A

Inc secretion of hydrophilic GAGs by fibroblasts

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55
Q

focal patches of hyperfunctioning follicular cells = which thyroid disease?

A

Toxic multinodular goiter

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56
Q

Tall follicular epithelial cells with scalloped colloid is seen in which thyroid disease?

A

Graves

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57
Q

Tx for Thyoid Storm?

A

Propranolol (Nadolol), Propylthiouracil, Corticosteroids, Potassium Iodide

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58
Q

Jod-Basedow phenomenon

A

If a patient that is previously iodine deficient and has partially autonomous thryroid tissue, is then made iodine replete —> thyrotoxicosis

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59
Q

Most common histology of thyroid adenoma?

A

follicular - with no capsular or vascular invasion

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60
Q

Why is thyroid cancer treatment associated with hypocalcemia?

A

Due to surgery leading to possible removal of parathyroid glands

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61
Q

Which two Thyroid cancers are associated with Ret mutation?

A

Papillary CA and MEN 2A & 2B (medullary CA)

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62
Q

Empty appearing nuclei with a central clearing and nuclear grooves - which Thyroid disease?

A

Papillary (Orphan Annie eyes)

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63
Q

Which thyroid cancer stains with Congo Red?

A

Medullary CA - sheets of cells in amyloid stroma

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64
Q

High PTH and low Ca is which Parathyroid disordeR?

A

2ndary Hyper PTH - vitamin D def, chronic renal failure

65
Q

Low Ca and Low PTH?

A

Primary HypoPTH

66
Q

High Ca and low PTH?

A

PTH indep hyper Calcemia - excess intake of Ca, cancer, hyper vitaminosis D

67
Q

Which endocrine disorder is associated with carpal spasm when one takes blood pressure?

A

HypoPTH - Trousseau sign

68
Q

Which endocrine disorder is assoc with shortened 4th/5th digits, and short stature?

A

Pseudohypoparathyroidism type 1A

69
Q

Which parathyroid disease is associated with a defective Gs protein alpha subunit ?

A

Psuedohypoparathyroidism type 1A and Pseudopseudohypoparathyroidism

70
Q

Which parathyroid disorder is associated with maternal imprinting? paternal imprinting?

A

Pseudohypoparathyroidism 1A - mother Pseudopseudohypoparathyroidism - father

71
Q

Which endocrine disorder is associated with a defective Ca 2+ sensing receptor?

A

Familial hypocalciuric hypercalcemia

72
Q

Which endocrine disorder is assoc with constipation, and flank pain?

A

Primary hyperparathyroidism

73
Q

Inc cAMP in urine is seen with which endocrine disordeR?

A

Primary Hyperparathyroidism

74
Q

The cystic bone lesions are filled with brown tissue made of what?

A

Osteoclasts and deposited hemosiderin from hemorrhages

75
Q

Most common manifestations of a pituitary adenoma?

A

Prolactinoma - galactorrhea, amenorrhea

76
Q

Hyperpigmentation, headaches, and bitemporal hemianopsia is associated with which endocrine disorder?

A

Nelson syndrome

77
Q

Major causes of hyperparathyroidism?

A

Pit adenoma, hyperplasia

78
Q

Which parameters increase with primary hyperparathyroidism?

A

calcium (se and urine), inc PTH, inc ALP, inc urinary cAMP

79
Q

In Osteitis fibrosa cystica, what are the cystic bone spaces filled with?

A

filled with brown fibrous tissue - brown because of deposited hemosiderin from hemorrhage

80
Q

Presentation of hyperparathyroidism?

A

weakness, constipation, abdominal/flank pain ( kidney stones, acute pancreatitis) depression

81
Q

Most common cause of secondary hyperparathyroidism? What sets it apart from other causes?

A

chronic renal disease, will show hyperphosphatemia (other causes = hypophosphatemia)

82
Q

Pituitary adenomas are most often associated with the increase of what hormone?

A

PRL - sx/ amenorrhea, galactorrhea, low libido, infertility

83
Q

Treatment for prolactinoma

A
  1. Dopamine agonist - Bromocriptine, Cabergoline 2.Transsphenoidal resection
84
Q

Which test is associated with acromegaly to Dx?

A

Inc serum IGF-1 ; failure to suppress serum GH following oral glucose tolerance test

85
Q

What is increased in serum with acromegaly?

A

IGF-1

86
Q

Which two drugs are used in acromegaly and mech of action?

A

Octreotide - somatostatin analogue (-) GH release Pegvisomant - ( GH-R inhibitor - “Put a peg in that viisible growth, man”)

87
Q

Secondary Nephrogenic DBI is associated with which drugs?

A

Lithium, Demeclocycline (ADH antagonist)

88
Q

Difference in lab values with Central v Nephrogenic DBI?

A

Central - dec ADH, increase in urine osmo with ADH analogue Nephrogenic - normal ADH , no change in urine osm with ADH analogue

89
Q

3 drugs used to treat Nephrogenic DBI?

A

HCTZ, Indomethacin, Amiloride

90
Q

4 major causes of SIADH?

A

Ectopic (Small cell lung cancer) CNS disorders/head trauma Pulm disease Drugs (“Lungs, and brain, and drugs, and cancer)

91
Q

Which endocrine disorder is associated with large tongue ,odor, spaces between the teeth?

A

Acromegaly - tongue has deep furrows, have big sweat glands and are prone to odor, and have spaces between teeth Other poss q stem sx/ arthritis, gout, large toes/fingers/nose/head, impaired glucose tolerance, polyps, thick neck, coarse facial features, carpal tunnel, cardiomegaly

92
Q

What is the carrier protein for ADH?

A

Neurophysin

93
Q

Failing to correct sodium levels slowly in SIADH leads to what pathology?

A

Osmotic demyelination syndrome

94
Q

Why do you not see edema, rales, or JVD in SIADH?

A

It’s a euvolemic condition, only Na is decreased in the body, urinating out a ton of Na, while retaining H2O

95
Q

What is ischemic infarct of the pituitary associated with?

A

postpartum bleeding

96
Q

Most common presentation of Sheehan syndrome?

A

failure to lactate (Opp of GH-oma) and loss of pubic hair Other sx/dec T4, 24hr cortisol,

97
Q

Most common cause of death in Diabetes mellitus?

A

MI - when associated with large vessel atherosclerosis

98
Q

Osmotic damage in DB associated with?

A

increased sorbitol in organs with aldose reductase or dec/no sorbitol DH

99
Q

HLA associations of T1DB?

A

DR3 DR4

100
Q

What type of deposits are seen in islet cells in T2DB?

A

amyloid polypeptide

101
Q

leukocyte infiltration is seen in islets of which type of diabetes?

A

T1DB

102
Q

Most common infection precipitating diabetic ketoacidosis? Most common infection as a result of it?

A

UTI, mucormycosis

103
Q

Inc c peptide levels associated with which Dx?

A

Insulinoma

104
Q

Most common malignancy in SI?

A

Carcinoid syndrome

105
Q

Why are high levels of 5-HT not seen in carcinoid syndrome localized in GI?

A

Undergoes first pass metabolism

106
Q

Which vitamin deficiency is associated with carcinoid syndrome?

A

niacin - pellagra

107
Q

What marker is increased in the urine with carcinoid syndrome?

A

5-HIAA

108
Q

Which side of the heart is affected in carcinoid syndrome (valves)?

A

R sided valvular disease

109
Q

Increased gastrin secretion from a tumor can be localized in which two areas?

A

Pancreas and duodenum

110
Q

A gastrin secreting tumor is associated with which endocrine neoplasia?

A

MEN I

111
Q

proliferation and hyperplasia of parietal cells leading to increase mass of fundic glands is assoc with which dx?

A

Z-E syndrome

112
Q

Why is Z-E syndrome associated with diarrhea?

A

Bc inc gastric acid will inhibit digestive enzmes, cant digest nutrients

113
Q

Increased risk of colorectal polyps and cancer in which endocrine cancer?

A

Acromegaly

114
Q

Mech of action of Pegvisomant? Is Tx for which disease?

A

GH-R antagonist, Acromegaly

115
Q

Which endocrine disorder is associated with saddle nose, prominent forehead, and small genitalia?

A

Laron syndrome

116
Q

Which drugs are associated with Nephrogenic DBI?

A

Lithium, Demeclocycline

117
Q

Tx for Nephrogenic DBI?

A

HCTZ, Indomethacin, Amiloride

118
Q

Tx for SIADH?

A
  • Fluid restriction
  • Salt tablets
  • IV hypertonic
  • saline diuretics
  • Rx/ Conivaptan Tolvaptan Demeclocycline
119
Q

Failure to lactate, absent menstruation, cold intolerance

A

Sheehan syndrome

120
Q

unopposed secretion of GH and Epi can cause what endocrine disorder?

A

DB

121
Q

Why is there retinopathy/nephro/neuropathy with DB?

A

Due to nonenzymatic glycosylation in small vessels

122
Q

Osmotic damage in DB due to?

A

sorbital accumulation in organs with aldose reductase or dec/absent sorbitol DH

123
Q

What level of fasting plasma glucose is Dx for DB?

A

>126 mg/dL

124
Q

What level for the 2 hr oral glucose tolerance test is Dx for DB?

A

> 200 mg/dL

125
Q

Why can you potentially see vomiting with insulin def?

A

inc lipolysis –> inc ketogenesis, ketonemia –> vomiting

126
Q

What type of metabolic issue with insulin def?

A

anion gap metabolic acidosis

127
Q

HLA # for T1DB?

A

HLA DR3 DR4

128
Q

Autoimmune destruction of Beta cells due to?

A

glutamic acid decarboxylase Ab

129
Q

Sx of DB ketoacidosis?

A

Delirium, Kussmaul respirations, Abdom pain, n/v, dehydration, fruity breath odor

130
Q

Infectious complication of DB ketoacidosis?

A

Rhizopus - mucormycosis

131
Q

What is seen with elderly T2Db?

A

hyperglycemia induced dehydraion and inc serum osmo (Hyperosm hyperglycema non ketotic syndrome)

132
Q

Glucose level in hyperosmolar hyperglycemic state?

A

>600 mg/dL

133
Q

Serum Osmo level in hyperosmolar hyperglycemic state?

A

>320mOsm/kg

134
Q

Pt with migratory erythema, hypergylcemia, DVT, weight loss, depression?

A

Glucagonoma

135
Q

Whipple triad? in what Disease?

A

Low blood glucose Sx of hypoglycemia - lethargy, syncope, diplopia resolution of Sx with normalizing of glucose levels Seen in Insulinoma

136
Q

10% of Insulinoma is associated with what, along with ZE syndrome?

A

MEN I

137
Q

Pt with DB or glucose intolerance, steatorrhea, gallstones ?

A

Somatostatinoma

138
Q

Cell type of carcinoid tumor?

A

neuroendocrine cells

139
Q

Why are sx of Carcinoid not seen if tumor is in GI?

A

5-HT goes through first pass metabolism

140
Q

Cardiac issue with carcinoid syndrome?

A

R side valvular disease - TR, Pulm stenosis

141
Q

Lab test in carcinoid

A

5-HIAA Hydroxyindoleacetic acid

142
Q

Dermatitis, diarrhea, and dementia seen in which endocrine disorder?

A

Carcinoid syndrome –> niacin Def

143
Q

Definitive Dx of ZE syndrome?

A

Gastrin levels stay elevated after admin of secretin (would dec Normally)

144
Q

Which thyroid disorder is associated with Inc CK?

A

Hypothyroidism - myopathy (Normal Ck in Hyper)

145
Q

Which thyroid disorder is associated with thinning, fine hair?

A

hyperthyroidism

146
Q

Thyroid disorder with inc #/sensitivity of B adr receptors?

A

Hyperthyroid

147
Q

Hyperpigmentation, headaches, and bitemporal hemianopsia is associated with which endocrine disorder?

A

Nelson syndrome

148
Q

3Ps for MEN1?

A

Parathyroid tumors Pituitary tumors (prolactin or GH) Pancreatic endocrine tumors (Zollinger Ellison syndrome, insulinomas, VIPomas, glucagonomas)

149
Q

what gene is associated with MEN1?

A

MEN1 gene (menin, tumor suppressor) on chromosome 11

150
Q

what MEN is associated with RET gene

A

MEN2A and MEN2B

151
Q

What does RET gene code for?

A

Receptor Tyrosine kinase

152
Q

symptoms of VIPomas?

A

WDHA Watery Diarrhea, Hypokalemia, and Achlorhydria

153
Q

what path is associated with dermatitis (necrolytic migratory erythema), diabetes, DVT, and depression?

A

glucagonoma (also associated with MEN1)

154
Q

2Ps of MEN 2A?

A

Parathyroid hyperplasia Pheochromocytoma

155
Q

1P of MEN 2B?

A

Pheochromocytoma

156
Q

what MEN is associated with medullary thyroid carcinoma?

A

MEN2A, 2B

157
Q

which MEN is associated with mucosal neuromas (oral/intestinal ganglioneuromatosis)

A

MEN 2B

158
Q

which multiple endocrine neoplasia is associated with marfanoid habitus?

A

MEN2B