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Step 1 Endocrinology-First Aid 2017 > Pathology > Flashcards

Flashcards in Pathology Deck (141):
1

What is the mechanism of Cushing syndrome?

Increased cortisol due to a variety of causes

2

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

Exogenous steroid use

3

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

Primary adrenal adenoma, hyperplasia or carcinoma

4

What is Cushing disease?

An ACTH-secreting pituitary adenoma

5

What are some paraneoplastic causes of increased ACTH?

Small cell lung cancer or bronchial carcinoids

6

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

Adrenal hyperplasia

7

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

Cushing disease

8

What needs to be done if adrenal tumor is suspected?

Adrenal CT to confirm

9

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

High-dose dexamethasone test and CRH stimulation test

10

What test results are consistent with Cushing disease?

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

11

What test results are consistent with ectopic ACTH secretion?

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

12

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

MRI of pituitary

13

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

CT of the chest/abdomen/pelvis

14

Inability of the adrenal glands to generate enough glucocorticoids

Adrenal insufficiency

15

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

Waterhouse-Friderichsen syndrome

16

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

Primary adrenal insufficiency

17

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

Acute primary adrenal insufficiency

18

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

Autoimmune destruction

19

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

TB

20

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

Chronic primary adrenal insufficiency or Addison disease

21

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

Decreased cortisol
Increased ACTH

22

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

Decreased cortisol and ACTH

23

What is a common cause of tertiary adrenal insufficiency?

Exogenous steroid use

24

How is aldosterone synthesis affected in tertiary adrenal insufficiency?

Not affected

25

What prevents edema in hyperaldosteronism?

Aldosterone escape mechanism

26

What are lab findings in primary hyperaldosteronism?

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

27

What is the cause of primary hyperaldosteronism?

Adrenal adenoma

28

What is the cause of 2ndary hyperaldosteronism?

Renovascular HTN, juxtaglomerular cell tumors and edema

29

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

Neuroblastoma

30

Neuroblastomas originate from what cell line?

Neural crest cells

31

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

Neuroblastomas

32

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

Neuroblastoma

33

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

Wilms tumor

34

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

Neuroblastoma

35

What are common findings in urine in someone with Neuroblastoma?

Increased catecholamines (homovanillic and vanillylmandelic acid)

36

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

Neuroblastoma

37

What oncogene is associated with neuroblastomas?

N-myc oncogene

38

Most common tumor of the adrenal medulla in adults?

Pheochromocytoma

39

Pheochromocytoma is derived from what cells?

Chromaffin cells of the adrenal medulla

40

What germline mutations are associated with pheochromocytoma?

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

41

What is the rule for pheochromocytomas?

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

42

What are common symptoms of pheochromocytomas?

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

43

What are common findings in pheochromocytomas?

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

44

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

Tertiary adrenal insufficiency

45

What is the treatment for pheochromocytoma?

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

46

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

To avoid a hypertensive crisis

47

What are the cholesterol levels in hypothyroidism?

Hypercholesterolemia due to decreased LDL receptor expression

48

What are the CK levels in hypothyroidism?

Increased CK levels

49

What are the TSH levels in hypothyroidism?

Increased TSH levels if primary

50

What are the T3 and T4 levels in hypothyroidism?

Decreased T3 and T4

51

What are the CK levels in hyperthyroidism?

Normal CK levels

52

What is the TSH level in hyperthyroidism?

Decreased TSH levels

53

What are the free T3 and T4 levels in hyperthyroidism?

Increased T3 and T4 levels

54

What are the cholesterol levels in hyperthyroidism?

Hypocholesterolemia due to increased LDL receptor expression

55

Causes of nodular thyroid goiters

Toxic multi-nodular goiter
Thyroid adenoma
Thyroid cancer
Thyroid cyst

56

Causes of smooth/diffuse thyroid goiters

Graves disease
Hashimoto thyroiditis
Iodine deficiency
TSH-secreting pituitary adenoma

57

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

Hashimoto thyroiditis

58

What are antibodies are associated Hashimoto thyroiditis?

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

59

What malignancy is associated with Hashimoto thyroiditis?

Non-Hodgkin lymphoma (B-cell origin)

60

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

Thyrotoxicosis during parafollicular rupture

61

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

Hashimoto thyroiditis

62

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

Marginal zone lymphoma

63

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

Thyroid dysgenesis

64

What is the cause of congenital hypothyroidism?

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

65

What are the findings in congenital hypothyroidism?

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

66

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

de Quervain (subacute granulomatous thyroiditis)

67

What is the histology of subacute granulomatous thyroiditis?

Granulomatous inflammation

68

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

Hyperthyroid early on, followed by hypothyroid

69

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

Riedel thyroiditis

70

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

Riedel thyroiditis

71

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

Wolff-Chaikoff effect

72

What drugs can cause hypothyroidism and presents as goitrogens?

Amiodarone and lithium

73

What is the most common cause of hyperthyroidism?

Graves disease

74

What type of hypersensitivity reaction is Graves disease?

Type II; IgG thyroid stimulating immunoglobulin

75

What HLA subtypes are associated with Graves disease?

HLA-DR3 and HLA-B8

76

What histological findings are seen in Graves disease?

Tall crowded follicular cells and scalloped colloid

77

What lymphocytes are associated with Graves disease?

T-cells

78

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

Exophthalmos

79

What symptoms are seen in Graves disease?

Exophthalmos, pretibial myxedema

80

What causes pretibial myxedema in Graves disease?

Dermal fibroblasts

81

What mediates exophthalmos?

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

82

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

Toxic multinodular goiter

83

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

TSH receptor mutations

84

What are T3 and T4 levels in toxic multinodular goiter?

Increased T3 and T4 levels

85

What is usual cause of toxic multinodular goiters?

Iodine deficiency

86

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

Thyroid storm

87

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

Thyroid storm

88

What lab findings are typically seen in thyroid storm?

Increased LFTs

89

What is the treatment for thyroid storm?

4 Ps:
Propranolol, PTU, Prednisone, and Potassium iodine

90

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

Jod-Basedow phenomenon

91

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

Thyroid adenoma

92

What is the histology or thyroid adenoma?

Follicular with absence of capsular or vascular invasion

93

What is the treatment for thyroid cancer?

Thyroidectomy

94

How is thyroid cancer diagnosed?

Fine needle aspiration

95

What nerve can be damaged in thyroidectomy?

Recurrent laryngeal nerve

96

Hypocalcemia after thyroidectomy is cause by damage to what structure?

Removal of parathyroid glands

97

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

Recurrent laryngeal nerve

98

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

Superior laryngeal nerve

99

What is the most common thyroid cancer?

Papillary carcinoma

100

What is the histology of papillary carcinoma?

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

101

What mutations increase risk of papillary carcinoma?

RET and BRAF mutations

102

What is a major cause of papillary carcinoma in childhood?

Irradiation

103

What is the prognosis of papillary carcinoma?

Excellent prognosis

104

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

Follicular carcinoma of the thyroid

105

What mutation is associated with follicular carcinoma?

RAS mutation

106

How does follicular carcinoma spread?

Hematogenous spread

107

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

Medullary carcinoma

108

What stain is used in Medullary carcinoma?

Congo red stain for amyloid stroma

109

What mutations are associated with medullary carcinoma?

MEN 2A and 2B RET mutations

110

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

Has medullary carcinoma so prophylactic thyroidectomy

111

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?

Undifferentiated/anaplastic carcinoma

112

What is lymphoma of the thyroid associated with?

Hashimoto thyroiditis

113

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

Hypoparathyroidism

114

What are common findings in Hypoparathyroidism?

Tetany, hypocalcemia and hyperphosphatemia

115

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

Chvostek sign

116

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

Trousseau sign

117

Common procedures done to test for Hypoparathyroidism are what?

Chvostek and Trousseau sign

118

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

Pseudohypoparathyroidism type 1A or Albright hereditary osteodystrophy

119

What are common findings in Albright hereditary osteodystrophy?

Shortened 4th/5th digits and short stature

120

What is the inheritance pattern of Albright hereditary osteodystrophy?

Autosomal dominant

121

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

Defective Gs protein a-subunit

122

How is the defect inherited in Albright hereditary osteodystrophy?

From mother due to imprinting

123

How is the defect inherited in pseudopseudohypoparathryroidism?

Inherited from father due to imprinting

124

What is the mechanism of pseudopseudohypoparathryroidism?

Defective Gs protein without end-organ resistance

125

What are the PTH levels in pseudopseudohypoparathryroidism?

Normal

126

What are the causes of primary hyperparathyroidism?

Carcinoma, adenoma, hyperplasia

127

What are lab findings seen in primary hyperparathyroidism?

Hypercalcemia, hypercalciuria, hypophosphatemia
Increased PTH, ALP, cAMP in urine

128

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

Primary hyperparathyroidism

129

What is the mnemonic for primary hyperparathyroidism?

Stones, thrones, bones, groans and psychiatric overtones

130

What is the cause of osteitis fibrosa cystica?

Increased PTH

131

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

Osteitis fibrosa cystica

132

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

Osteoclasts and deposited hemosiderin from hemorrhages

133

What is the most common cause of 2ndary hyperparathyroidism

Chronic renal disease

134

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

2ndary hyperparathyroidism

135

What are common findings in 2ndary hyperparathyroidism?

Hypovitaminosis D, Hypocalcemia, Hypophosphatemia
Increased ALP, PTH

136

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

Hypovitaminosis D, Hypocalcemia
Hyperphosphatemia
Increased ALP, PTH

137

What is the cause of tertiary hyperparathyroidism?

Refractory hyperparathyroidism from chronic renal failure

138

What are common lab findings in tertiary hyperparathyroidism?

Markedly increased PTH and increased calcium

139

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

Familial hypocalciuric hypercalcemia

140

What lab findings are seen in familial hypocalciuric hypercalcemia?

Hypercalcemia, low urine calcium, normal to increased PTH

141

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

Increased renal calcium uptake