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Step 1 Endocrinology-First Aid 2017 > Pathology > Flashcards

Pathology Flashcards

1
Q

What is the mechanism of Cushing syndrome?

A

Increased cortisol due to a variety of causes

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2
Q

What causes bilateral adrenal atrophy and is the most common cause of Cushing syndrome?

A

Exogenous steroid use

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3
Q

Atrophy of uninvolved adrenal gland in Cushing syndrome points to what cause?

A

Primary adrenal adenoma, hyperplasia or carcinoma

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4
Q

What is Cushing disease?

A

An ACTH-secreting pituitary adenoma

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5
Q

What are some paraneoplastic causes of increased ACTH?

A

Small cell lung cancer or bronchial carcinoids

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6
Q

What is the affect of increased endogenous ACTH on adrenal glands?

A

Adrenal hyperplasia

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7
Q

What is responsible for the majority of endogenous cases of Cushing syndrome?

A

Cushing disease

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8
Q

What needs to be done if adrenal tumor is suspected?

A

Adrenal CT to confirm

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9
Q

What is next step if ACTH levels are elevated on low-dose dexamethasone test?

A

High-dose dexamethasone test and CRH stimulation test

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10
Q

What test results are consistent with Cushing disease?

A

Adequate suppression with high-dose dexamethasone and increased ACTH and cortisol with CRH stimulation

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11
Q

What test results are consistent with ectopic ACTH secretion?

A

No suppression with high-dose dexamethasone and no increase in ACTH and cortisol with CRH stimulation

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12
Q

What is next step with adequate suppression with high-dose dexamethasone and increased ACTH and cortisol with CRH stimulation?

A

MRI of pituitary

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13
Q

What is next step with no suppression with high-dose dexamethasone and no increase in ACTH and cortisol with CRH stimulation?

A

CT of the chest/abdomen/pelvis

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14
Q

Inability of the adrenal glands to generate enough glucocorticoids

A

Adrenal insufficiency

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15
Q

Acute primary adrenal insufficiency due to hemorrhage associated with septicemia form N. meningitidis, DIC or endotoxic shock

A

Waterhouse-Friderichsen syndrome

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16
Q

Hypotension, hyperkalemia, metabolic acidosis with skin and mucosal hyperpigmentation points to what disease?

A

Primary adrenal insufficiency

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17
Q

Shock or hemorrhage causing hypovolemia leading to ischemia of the adrenal glands causes what type of adrenal insufficiency

A

Acute primary adrenal insufficiency

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18
Q

What is the most common cause of chronic primary adrenal insufficiency in the Western world?

A

Autoimmune destruction

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19
Q

What is the most common cause of chronic primary adrenal insufficiency in the developing world?

A

TB

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20
Q

Adrenal atrophy or destruction by disease either from autoimmune or TB is called what?

A

Chronic primary adrenal insufficiency or Addison disease

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21
Q

What cortisol and ADH levels are seen in primary adrenal insufficiency?

A

Decreased cortisol

Increased ACTH

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22
Q

What cortisol and ADH levels are seen in secondary adrenal insufficiency?

A

Decreased cortisol and ACTH

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23
Q

What is a common cause of tertiary adrenal insufficiency?

A

Exogenous steroid use

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24
Q

How is aldosterone synthesis affected in tertiary adrenal insufficiency?

A

Not affected

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25
Q

What prevents edema in hyperaldosteronism?

A

Aldosterone escape mechanism

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26
Q

What are lab findings in primary hyperaldosteronism?

A

Increased aldosterone, sodium reabsorption and potassium excretion
Decreased renin
Metabolic alkalosis

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27
Q

What is the cause of primary hyperaldosteronism?

A

Adrenal adenoma

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28
Q

What is the cause of 2ndary hyperaldosteronism?

A

Renovascular HTN, juxtaglomerular cell tumors and edema

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29
Q

What is the most common tumor of the adrenal medulla in children?

A

Neuroblastoma

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30
Q

Neuroblastomas originate from what cell line?

A

Neural crest cells

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31
Q

What type of tumors can occur anywhere along the sympathetic chain?

A

Neuroblastomas

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32
Q

Abdominal distention and a firm, irregular mass that can cross the midline points to what disease?

A

Neuroblastoma

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33
Q

Abdominal distention with a smooth mass that is unilateral, points to what disease?

A

Wilms tumor

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34
Q

Child with history of “dancing eyes-dancing feet” and an abdominal mass that crosses the midline with normal BP points to what disease?

A

Neuroblastoma

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35
Q

What are common findings in urine in someone with Neuroblastoma?

A

Increased catecholamines (homovanillic and vanillylmandelic acid)

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36
Q

Histology with Homer-Wright rosettes and Bombesin and NSE positive indicates to what disease?

A

Neuroblastoma

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37
Q

What oncogene is associated with neuroblastomas?

A

N-myc oncogene

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38
Q

Most common tumor of the adrenal medulla in adults?

A

Pheochromocytoma

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39
Q

Pheochromocytoma is derived from what cells?

A

Chromaffin cells of the adrenal medulla

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40
Q

What germline mutations are associated with pheochromocytoma?

A

NF-1, VHL, RET (MEN2A and MEN2B)

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41
Q

What is the rule for pheochromocytomas?

A 
Rule of 10s:
10% malignant
10% bilateral
10% extra-adrenal (bladder wall, organ of Zuckerandl)
10% calcify
10% kids
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42
Q

What are common symptoms of pheochromocytomas?

A

Relapsing and remitting symptoms of 5 Ps:
Pressure (increased BP) - increased catecholamines
Pain (headache)
Perspiration
Palpitations
Pallor

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43
Q

What are common findings in pheochromocytomas?

A

Increased catecholamines: Epi, NE, dopamine in urine and plasma

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44
Q

What is a worry in someone with history of chronic exogenous steroid use who stops using immediately?

A

Tertiary adrenal insufficiency

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45
Q

What is the treatment for pheochromocytoma?

A

Irreversible a-antagonists (phenoxybenzamine) and B-blockers prior to tumor resection

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46
Q

Why is a-blockade needed before giving B-blockers?

A

To avoid a hypertensive crisis

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47
Q

What are the cholesterol levels in hypothyroidism?

A

Hypercholesterolemia due to decreased LDL receptor expression

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48
Q

What are the CK levels in hypothyroidism?

A

Increased CK levels

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49
Q

What are the TSH levels in hypothyroidism?

A

Increased TSH levels if primary

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50
Q

What are the T3 and T4 levels in hypothyroidism?

A

Decreased T3 and T4

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51
Q

What are the CK levels in hyperthyroidism?

A

Normal CK levels

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52
Q

What is the TSH level in hyperthyroidism?

A

Decreased TSH levels

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53
Q

What are the free T3 and T4 levels in hyperthyroidism?

A

Increased T3 and T4 levels

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54
Q

What are the cholesterol levels in hyperthyroidism?

A

Hypocholesterolemia due to increased LDL receptor expression

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55
Q

Causes of nodular thyroid goiters

A

Toxic multi-nodular goiter
Thyroid adenoma
Thyroid cancer
Thyroid cyst

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56
Q

Causes of smooth/diffuse thyroid goiters

A

Graves disease
Hashimoto thyroiditis
Iodine deficiency
TSH-secreting pituitary adenoma

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57
Q

What is the most common cause of hypothyroidism in iodine-sufficient regions?

A

Hashimoto thyroiditis

58
Q

What are antibodies are associated Hashimoto thyroiditis?

A

Anti-thyroid (anti-microsomal) peroxidase and anti-thyroglobulin antibodies

59
Q

What malignancy is associated with Hashimoto thyroiditis?

A

Non-Hodgkin lymphoma (B-cell origin)

60
Q

Hashimoto thyroiditis may present as hyperthyroid early in disease course - why?

A

Thyrotoxicosis during parafollicular rupture

61
Q

Presents as a non-tender, moderately enlarged thyroid with decreased T3 and T4 and Hurthle cells with lymphoid aggregates and germinal centers on histology.

A

Hashimoto thyroiditis

62
Q

What malignancy can present as an enlarging gland in Hashimoto thyroiditis?

A

Marginal zone lymphoma

63
Q

What is the most common cause of congenital hypothyroidism in the U.S?

A

Thyroid dysgenesis

64
Q

What is the cause of congenital hypothyroidism?

A

Severe fetal hypothyroidism due to maternal hypothyroidism, thyroid agenesis, thyroid dysgenesis, iodine deficiency, dyshormonogenetic goiter

65
Q

What are the findings in congenital hypothyroidism?

A

6 Ps:

Pot-bellied, Pale, Puffy, Protruding umbilicus, Protuberant tongue, and Poor brain development

66
Q

Self-limited disease often following a flu-like illness presenting with increased ESR, jaw pain, and very tender thyroid

A

de Quervain (subacute granulomatous thyroiditis)

67
Q

What is the histology of subacute granulomatous thyroiditis?

A

Granulomatous inflammation

68
Q

How does subacute granulomatous thyroiditis present early on in disease course?

A

Hyperthyroid early on, followed by hypothyroid

69
Q

Thyroid replaced by fibrous tissue with inflammatory infiltrate and may extend to surrounding structures mimicking anaplastic carcinoma

A

Riedel thyroiditis

70
Q

Young female presents with fixed, hard, rock-like painless goiter points to what disease?

A

Riedel thyroiditis

71
Q

Thyroid gland down-regulation in response to increased iodine seen in hypothyroid diseases - what effect is this?

A

Wolff-Chaikoff effect

72
Q

What drugs can cause hypothyroidism and presents as goitrogens?

A

Amiodarone and lithium

73
Q

What is the most common cause of hyperthyroidism?

A

Graves disease

74
Q

What type of hypersensitivity reaction is Graves disease?

A

Type II; IgG thyroid stimulating immunoglobulin

75
Q

What HLA subtypes are associated with Graves disease?

A

HLA-DR3 and HLA-B8

76
Q

What histological findings are seen in Graves disease?

A

Tall crowded follicular cells and scalloped colloid

77
Q

What lymphocytes are associated with Graves disease?

A

T-cells

78
Q

Increased osmotic swelling, muscle inflammation and adipocyte count in retro-orbital space causes what in Graves disease?

A

Exophthalmos

79
Q

What symptoms are seen in Graves disease?

A

Exophthalmos, pretibial myxedema

80
Q

What causes pretibial myxedema in Graves disease?

A

Dermal fibroblasts

81
Q

What mediates exophthalmos?

A

Infiltration of activated T-cells in the retro-orbital space leading to increased cytokines like TNF-a and IFN-y

82
Q

Focal patches of hyperfunctioning follicular cells distended with colloid and working independently of each TSH

A

Toxic multinodular goiter

83
Q

What is the cause of toxic multinodular goiters working independently of TSH?

A

TSH receptor mutations

84
Q

What are T3 and T4 levels in toxic multinodular goiter?

A

Increased T3 and T4 levels

85
Q

What is usual cause of toxic multinodular goiters?

A

Iodine deficiency

86
Q

Uncommon but serious complication that occurs when hyperthyroidism is incompletely treated or undertreated, and then significantly worsens in the setting of acute stress such as infection, trauma or surgery

A

Thyroid storm

87
Q

Commonly presents with agitation, delirium, fever, diarrhea, coma and tachyarrhythmias from incomplete treatment of hyperthyroidism after acute illness

A

Thyroid storm

88
Q

What lab findings are typically seen in thyroid storm?

A

Increased LFTs

89
Q

What is the treatment for thyroid storm?

A

4 Ps:

Propranolol, PTU, Prednisone, and Potassium iodine

90
Q

A patient with an autonomous nodule who is iodine deficient is given iodine and develops thyrotoxicosis

A

Jod-Basedow phenomenon

91
Q

Asymptomatic benign solitary growth of the thyroid that is non-functional rarely causing hyperthyroidism

A

Thyroid adenoma

92
Q

What is the histology or thyroid adenoma?

A

Follicular with absence of capsular or vascular invasion

93
Q

What is the treatment for thyroid cancer?

A

Thyroidectomy

94
Q

How is thyroid cancer diagnosed?

A

Fine needle aspiration

95
Q

What nerve can be damaged in thyroidectomy?

A

Recurrent laryngeal nerve

96
Q

Hypocalcemia after thyroidectomy is cause by damage to what structure?

A

Removal of parathyroid glands

97
Q

What nerve can be damaged during ligation of inferior thyroid artery in thyroidectomy?

A

Recurrent laryngeal nerve

98
Q

What nerve can be damaged during ligation of superior laryngeal artery in thyroidectomy?

A

Superior laryngeal nerve

99
Q

What is the most common thyroid cancer?

A

Papillary carcinoma

100
Q

What is the histology of papillary carcinoma?

A

Empty-appearing nuclei with central clearing (Orphan Annie eyes), psammoma bodies, and nuclear grooves

101
Q

What mutations increase risk of papillary carcinoma?

A

RET and BRAF mutations

102
Q

What is a major cause of papillary carcinoma in childhood?

A

Irradiation

103
Q

What is the prognosis of papillary carcinoma?

A

Excellent prognosis

104
Q

Presents with uniform follicles on histology, invades thyroid capsule and vasculature with a good prognosis?

A

Follicular carcinoma of the thyroid

105
Q

What mutation is associated with follicular carcinoma?

A

RAS mutation

106
Q

How does follicular carcinoma spread?

A

Hematogenous spread

107
Q

Thyroid cancer from parafollicular cells that produces calcitonin in sheets of cells in an amyloid stroma

A

Medullary carcinoma

108
Q

What stain is used in Medullary carcinoma?

A

Congo red stain for amyloid stroma

109
Q

What mutations are associated with medullary carcinoma?

A

MEN 2A and 2B RET mutations

110
Q

What is done if RET mutations detected in someone with thyroid cancer?

A

Has medullary carcinoma so prophylactic thyroidectomy

111
Q

Thyroid cancer that is common in older patients presenting with dysphagia and respiratory compromise with invasion of local structures and has a very poor prognosis?

A

Undifferentiated/anaplastic carcinoma

112
Q

What is lymphoma of the thyroid associated with?

A

Hashimoto thyroiditis

113
Q

Accidental surgical excision of parathyroid glands, autoimmune destruction, or DiGeorge syndrome can all cause what condition?

A

Hypoparathyroidism

114
Q

What are common findings in Hypoparathyroidism?

A

Tetany, hypocalcemia and hyperphosphatemia

115
Q

Contraction of facial muscles with tapping of facial nerve on the check is called what?

A

Chvostek sign

116
Q

Occlusion of brachial artery with BP cuff causing carpal spasm is called what?

A

Trousseau sign

117
Q

Common procedures done to test for Hypoparathyroidism are what?

A

Chvostek and Trousseau sign

118
Q

Unresponsiveness of kidney to PTH causing hypocalcemia despite increased PTH level is called what?

A

Pseudohypoparathyroidism type 1A or Albright hereditary osteodystrophy

119
Q

What are common findings in Albright hereditary osteodystrophy?

A

Shortened 4th/5th digits and short stature

120
Q

What is the inheritance pattern of Albright hereditary osteodystrophy?

A

Autosomal dominant

121
Q

What is the mechanism of end-organ resistance to PTH in Albright hereditary osteodystrophy?

A

Defective Gs protein a-subunit

122
Q

How is the defect inherited in Albright hereditary osteodystrophy?

A

From mother due to imprinting

123
Q

How is the defect inherited in pseudopseudohypoparathryroidism?

A

Inherited from father due to imprinting

124
Q

What is the mechanism of pseudopseudohypoparathryroidism?

A

Defective Gs protein without end-organ resistance

125
Q

What are the PTH levels in pseudopseudohypoparathryroidism?

A

Normal

126
Q

What are the causes of primary hyperparathyroidism?

A

Carcinoma, adenoma, hyperplasia

127
Q

What are lab findings seen in primary hyperparathyroidism?

A

Hypercalcemia, hypercalciuria, hypophosphatemia

Increased PTH, ALP, cAMP in urine

128
Q

Disease that presents with weakness and constipation, abdominal/flank pain, and depression

A

Primary hyperparathyroidism

129
Q

What is the mnemonic for primary hyperparathyroidism?

A

Stones, thrones, bones, groans and psychiatric overtones

130
Q

What is the cause of osteitis fibrosa cystica?

A

Increased PTH

131
Q

Cystic bone spaces filled with brown fibrous tissue with increased PTH levels?

A

Osteitis fibrosa cystica

132
Q

What is contained in the cysts seen in osteitis fibrosa cystica?

A

Osteoclasts and deposited hemosiderin from hemorrhages

133
Q

What is the most common cause of 2ndary hyperparathyroidism

A

Chronic renal disease

134
Q

2ndary hyperplasia due to decreased calcium absorption and/or increased phosphate

A

2ndary hyperparathyroidism

135
Q

What are common findings in 2ndary hyperparathyroidism?

A

Hypovitaminosis D, Hypocalcemia, Hypophosphatemia

Increased ALP, PTH

136
Q

What are common findings in 2ndary hyperparathyroidism due to chronic renal failure?

A

Hypovitaminosis D, Hypocalcemia
Hyperphosphatemia
Increased ALP, PTH

137
Q

What is the cause of tertiary hyperparathyroidism?

A

Refractory hyperparathyroidism from chronic renal failure

138
Q

What are common lab findings in tertiary hyperparathyroidism?

A

Markedly increased PTH and increased calcium

139
Q

What disease is caused by a defective G-coupled sensing receptor in multiple tissues requiring higher than normal calcium levels to suppress PTH

A

Familial hypocalciuric hypercalcemia

140
Q

What lab findings are seen in familial hypocalciuric hypercalcemia?

A

Hypercalcemia, low urine calcium, normal to increased PTH

141
Q

What is the cause of hypocalciuria and hypercalcemia seen in familial hypocalciuric hypercalcemia?

A

Increased renal calcium uptake

Step 1 Endocrinology-First Aid 2017 (4 decks)

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