Module 11 Endocrine Path (Robbins Ch 24)

Front 1

Most common enzyme deficient in adrenogenital syndrome

Back 1

21-hydroxylase

Front 2

Adrenal hormone whose production is DECREASED in adrenogenital syndrome

Back 2

Cortisol

Front 3

Presenting symptom of nodule in adrenal medulla

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HTN

Front 4

Hyperosmolar coma: more likely type I or II diabetes

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Type II

Front 5

Serum test very important to do post thyroidectomy

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Calcium (to check for hypoparathyroidism)

Front 6

Name for an adrenal cortical adenoma secreting aldosterone

Back 6

Conn syndrome

Front 7

Effect of Conn syndrome on corticotropin levels

Back 7

NONE. Aldosterone does not exhibit feedback on corticotropin

Front 8

Only way to differentiate between follicular adenoma vs carcinoma of thyroid

Back 8

Invasion or metastasis = carcinoma

Front 9

Out of follicular and papillar carcinoma of thyroid, which is more likely to metastasis to lymph nodes and which to distant sites

Back 9

Follicular = distant sites, Papillary = lymph nodes

Front 10

Three likely metastasis site of follicular thyroid carcinoma

Back 10

Bone, lung, liver

Front 11

Rare, but highly aggressive thyroid cancer. Very unlikely to be confined

Back 11

Anaplastic carcinoma

Front 12

Thyroiditis: typically involves part of gland or whole?

Back 12

Whole gland

Front 13

Cancer risk increased with Hashimoto thyroiditis

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non-Hodkin B-cell lymphoma

Front 14

Thyroid cancer that tends to invade locally before having lymph node or distant metastasis

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Medullary carcinoma

Front 15

Give dx based on microscopic appearance: papillary projection in thyroid follicles lined by tall columnar epithelial cells, stromal lymphoid aggregates,

Back 15

Graves disease

Front 16

Give dx based on microscopic appearance: Thyroid Follicular destruction with lymphoid aggregates and Hurthle cell metaplasia

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Hashimoto Thyroiditis

Front 17

Give dx based on microscopic appearance: Thyroid Follicular destruction with presence of giant cells

Back 17

Granulomatous thyroiditis

Front 18

Thyroid cancer that can be multifocal

Back 18

Medullary carcinoma

Front 19

pDiabetes type with Pancreatic amyloid deposition = ?

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Type 2 diabetes

Front 20

Give dx based on microscopic appearance: thyroid with enlarged follicles and flattened epithelial cells

Back 20

Goiter

Front 21

Most patients with goiter: hypo, hyper, or euthyroid

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Euthyroid

Front 22

Cancer ruled out by negative low and high dose dexamethasone suppression test

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Pituitary adenoma

Front 23

Adrenal tumor Cushing's and Conn's disease: Effect on opposite adrenal gland?

Back 23

Cushings: atrophy, Conn's: none

Front 24

Type II diabetics are at risk for developing what liver complication

Back 24

Nonalcoholic steatohepatitis

Front 25

Mucormycosis is at increased risk when diabetics enter:

Back 25

Ketoacidosis

Front 26

Most common cause of primary and secondary hyperparathyroidism

Back 26

Primary - parathyroid adenoma; Secondary - renal failure

Front 27

Easiest way to exclude renal failure as a cause of hyperparathyroidism

Back 27

Low phosphate levels. Phosphate is retained with renal failure

Front 28

Infection at increased risk when type I diabetic enters ketoacidosis

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Mucomycosis (Mucor Circinelloides)

Front 29

Pancreas has fibrous stroma with scattered normal islets. Dx?

Back 29

Chronic pancreatitis

Front 30

Name for bilateral adrenocortical hemorrhage following Neisseria Meningitidis infection

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Waterhouse-Friderichsen syndrome

Front 31

Severe infection with this virus in immunocompromised individuls can diminish adrenal function

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CMV

Front 32

What is Plummer syndrome? Give its alternate name

Back 32

A hyperfunctioning nodule inside a goiter. AKA toxic multinodular goiter

Front 33

Affect of papillary carcinoma on thyroid function

Back 33

NONE

Front 34

Follicular adenoma: usually hot or cold nodules

Back 34

Cold ("toxic" follicular adenoma is an exception)

Front 35

Alternate name for subacute granulomatous thyroiditis

Back 35

de Quarvain thyroiditis

Front 36

Usual factor preceding de Quarvain thyroiditis

Back 36

Viral URI

Front 37

Cell types infiltrating to cause insulitis in Type I diabetes

Back 37

T-cells

Front 38

Thyroid cancer with RET mutation

Back 38

Papillary and medullary carcinomas

Front 39

Amyloid stroma is a common feature of what thyroid tumor

Back 39

Medullary carcinoma

Front 40

Most medullary carcinomas (80%) are sporadic, name two other times they are seen

Back 40

MEN IIA or IIB

Front 41

Alternate name for MEN Type I

Back 41

Wermer syndrome

Front 42

Neck irradiation predisposes to what thyroid cancer?

Back 42

Papillary carcinoma

Front 43

MHC Class II alleles seen with type 1 diabetes

Back 43

HLA-DR3 and DR4

Front 44

Most likely dx for solitary cold nodule in younger person

Back 44

Cancer, most likely follicular adenoma

Front 45

Enzyme causing converstion of glucose to sorbitol

Back 45

Aldose reductase

Front 46

Mechanism of increased atherosclerosis in diabetics

Back 46

Glycosylation of vascular walls

Front 47

MEN syndrome with the "three P's." Name them

Back 47

MEN type I. Pancreas, Pituitary, Parathyroid

Front 48

Most likely causative organism of Waterhouse-Friderichsen syndrome

Back 48

Neisseria Meningiditis

Front 49

Give dx based on microscopic appearance: thyoid with nests of cell in congo red-positive hyaline stroma

Back 49

Medullary carcinoma

Front 50

Product of C cells (aka parafollicular)

Back 50

Calcitonin

Front 51

HLA-DR3 and HLA-DR4 are seen with?

Back 51

Type 1 diabetes

Front 52

Mechanism of neuropathy in diabetics

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Sorbitol accumulation causes osmolar damage

Front 53

Hereditary tendency of graves disease:

Back 53

Fairly high (50% twin concordance)

Front 54

Frequency of exopthalmos in graves disease

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Only 40%

Front 55

Cell type in medullary carcnioma of the thyroid

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C cells (aka parafollicular cells)

Front 56

Condition with antithyroid peroxidase antibodies

Back 56

BOTH Hashimoto and Graves, though higher tighter in Hashimoto

Front 57

If a unilateral adrenal cortical adenoma is present, but the opposite gland is NOT atrophied, what do you know?

Back 57

It's either nonfunctional or secretes aldosterone, NOT cortisol

Front 58

Alternate name for primary chronic adrenocortical insufficiency

Back 58

Addison disease

Front 59

Most common cause of Addison disease (assume NOT a TB-rich country)

Back 59

Autoimmune adrenalitis

Front 60

Co-morbidity common with autoimmune adrenalitis

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Other autoimmune diseases in 50% of cases, often endocrine

Front 61

Two most common mutations in parathyroid tumors

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#1: Cyclin D1, #2: MEN1

Front 62

GNAS1 mutations are common in what endocrine tumor?

Back 62

Somatotroph Pituitary adenoma

Front 63

VHL mutation can be seen in what endocrine tumor?

Back 63

Pheochromocytoma

Front 64

Describe Zollinger-Ellison syndrome

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One or more islet cell adenomas that secrete gastrin; produces intractable PUD

Front 65

Symptoms of vasoactive intestinal polypepite tumor (VIPomas)

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Marked diarrhea, hypokalemia, achlorhydria (low HCl in gastric secretions)

Front 66

Pancreatic tumor that can secrete several various hormones

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Islet cell tumor

Front 67

Strong sign that PUD may be cause by Zollinger-Ellison

Back 67

Disease is intractable - H2 blockers will not heal the ulcers

Front 68

Most common Thyroid malignancy

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Papillary

Front 69

Occurance rate of metastasis TO the thyroid

Back 69

Rare

Front 70

Invasion pattern of parathyroid carcinoma

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Usually Local invasion only

Front 71

Thyroid cancer with cervical node metastasis. Likely Dx?

Back 71

Papillary carcinoma

Front 72

Usual age, location of craniopharyngioma

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Child or young adult, suprasellar region

Front 73

Embryologic origin of craniopharyngioma

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Remnant of Rathke pouch

Front 74

Microscopic appearance of craniopharyngioma

Back 74

Squamous elements + debris containing cholesterol crystals (primitive tooth structures)

Front 75

Likely dx for high prolactin + hypernatremia

Back 75

Craniopharyngioma

Front 76

Ways to segregate high prolactin caused by craniopharyngioma versus prolactinoma

Back 76

Craniopharyngioma: suprasellar, destructive of surrounding structures, hypernatremia. These do NOT occur with prolactinoma

Front 77

Symptoms (other than galactorrhea) seen with prolactinoma

Back 77

Decreased libido, amenorrhea, infertility

Front 78

Easiest way to segregate pancreatic adenocarcinoma versus islet cell tumor

Back 78

Adenocarcinoma does not secrete hormones

Front 79

Fatty replacement of pancreas. Dx?

Back 79

Cystic fibrosis

Front 80

Pseudocysts in the pancreas are a complication of?

Back 80

Pancreatitis

Front 81

Usual serum calcium levels in hyperparathyroidism secondary to renal failure

Back 81

Normal (kidney failure tends to increase PO4/decrease Ca, so PTH balances out)

Front 82

Most common location of neuroblastoma

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Retroperitoneum - Adrenal glands or nearby ganglia

Front 83

Endocrine product of neuroblastoma, urine test used

Back 83

Catecholamine precursors, homovanillic acid (HVA)

Front 84

Hormone which causes can hyperpigmentation in Cushing's

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ACTH (or it's precursors)

Front 85

Inheritance pattern of MODY

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Autosomal dominant

Front 86

Mutation in MODY2

Back 86

Inactivation mutation in glucokinase

Front 87

Mutation in MODY3

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Hepatocyte nuclear factor alpha-1

Front 88

Worse hyperglycemia: MODY3 or MODY2

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MODY3

Front 89

Reason for hyperglycemia in MODY

Back 89

Reduced insulin secreition in response to glucose

Front 90

Newborn with retroperitoneal mass + HTN. Likely Dx?

Back 90

Neuroblastoma

Front 91

Three things than can be screened in urine for pheochromocytoma

Back 91

Free catecholamines, metanephrine, vanillylmandelic acid (VMA)

Front 92

Reason secondary hyperparathyroidism occurs in ADPKD

Back 92

Kidney funciton down --> PO4 up, Vit D down --> Calcium down --> PTH up

Front 93

Appearance of nuclei in papillary carcinoma of thyroid

Back 93

Clear

Front 94

Mutations seen in 30% of papillary thyroid carcinomas; What family are these genes in?

Back 94

RET or NTRK1; Receptor tyrosine kinases

Front 95

What is Sheehan syndrome? Why does it occur?

Back 95

Postpartum pituitary necrosis. Pituitary enlarges during pregnancy, but blood supply does not. Blood loss during childbirth causes infarction

Front 96

More at risk for Sheehan syndrome: anterior or posterior pituatary?

Back 96

Anterior

Front 97

Anatomical abnormality in empty sella

Back 97

Arachnoid herniates through diaphragma sella

Front 98

Most frequent presentation symptom of empty sella

Back 98

Hyperprolactinemia

Front 99

What is the "stalk section" effect?

Back 99

Seen with empty sella: herniation cuts off prolactin inhibition, but other anterior pituitary functions are normal

Front 100

Typical patient for empty sella

Back 100

Obese woman

Front 101

If patient has Cushing's syndrome plus feminization, where is the tumor probably located?

Back 101

Adrenal cortex

Front 102

Location of organ of Zuckerkandl and tumor that arises from it

Back 102

Aortic bifurcation; Pheochromocytoma

Front 103

Mutation seen in "toxic" follicular adenoma

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Activation of TSH receptor pathway

Front 104

Thyroid cancer with PAX8-PPAR gamma fusion gene

Back 104

Follicular carcinoma

Front 105

Mutation in AIRE gene causes:

Back 105

Autoimmune polyendocrinopahty

Front 106

Name a recently discovered type I diabetes polymorphism

Back 106

CTLA4

Front 107

Enzyme deficient in salt-wasting form of adrenogenital syndrome

Back 107

21-hydroxylase

Front 108

Name a recently discovered type II diabetes polymorphism

Back 108

TCF7L2

Front 109

Most common cause of SIADH

Back 109

Paraneoplastic, most often small cell carcinoma of the lung

Front 110

Most common pineal tumor in children and adults

Back 110

Children - pineoblastoma, Adults - pineocytoma

Front 111

Location of adrenal gland

Back 111

Sella turcica

Front 112

Organ located between left and right thalamic pulvinar regions, inferior to copus callosum and superior to collicular plate

Back 112

Pineal gland

Front 113

Relative amount of islets left when Type 1 DM becomes clinically evident

Back 113

Pretty much none

Front 114

Necrolytic migrating erythema (a skin rash) is found with what endocrine tumor?

Back 114

Glucagonoma

Front 115

Hormone production reduced in 17 alpha hydroxylase deficiency

Back 115

Cortisol and DHEA

Front 116

Hormone production reduced in 21 hydroxylase deficiency

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Aldosterone and cortisol

Front 117

Hormone production reduced in 11 hydroxylase deficiency

Back 117

Aldosterone and cortisol

Front 118

How to differentiate between 21 vs 11 hydroxylase deficiency

Back 118

Glucocorticoid activity. Neither can make cortisol, but with 11 hydroxylase deficiency, some glucocorticoid active intermediates are produced

Front 119

Alternate names for MEN IIA and IIB

Back 119

IIA - Sipple syndrome; IIB - Williams syndrome

Front 120

How to differentiate between MEN 2A versus 2B

Back 120

2B does not have hyperparathyroidism, instead has neuromas/ganglioneuromas at multiple sites and Marfanoid appearance

Front 121

MEN with Marfanoid appearance

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MEN 2B

Front 122

Neoplasm present in nearly all MEN 2A and 2B

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Thyroid medullary carcinoma

Front 123

Lesions in MEN IIA

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Thyroid medullary carcinoma, pheochromocytoma (40-50%) parathyroid hyperplasia/adenoma (10-20%)

Front 124

Oncogene mutated in MEN 2A and B

Back 124

RET

Front 125

Bilateral pheochromocytomas means?

Back 125

It's familial (check MEN II)

Front 126

What causes granulomatous destruction of the adrenals?

Back 126

TB

Front 127

How to differentiate between toxic multinodular goiter versus toxic follicular adenoma

Back 127

Look at the rest of the gland. Will be enlarged with goiter, atrophied with adenoma

Front 128

Initial thyroid enlargement and hyperfunction followed by progressive atrophy. Dx?

Back 128

Hashimoto Thyroiditis

Front 129

Cause of permanent neonatal diabetes

Back 129

Gain of function mutation in ATP-sensitive potassium channels