Pathology Flashcards
(141 cards)
1
Q
What is the mechanism of Cushing syndrome?
A
Increased cortisol due to a variety of causes
2
Q
What causes bilateral adrenal atrophy and is the most common cause of Cushing syndrome?
A
Exogenous steroid use
3
Q
Atrophy of uninvolved adrenal gland in Cushing syndrome points to what cause?
A
Primary adrenal adenoma, hyperplasia or carcinoma
4
Q
What is Cushing disease?
A
An ACTH-secreting pituitary adenoma
5
Q
What are some paraneoplastic causes of increased ACTH?
A
Small cell lung cancer or bronchial carcinoids
6
Q
What is the affect of increased endogenous ACTH on adrenal glands?
A
Adrenal hyperplasia
7
Q
What is responsible for the majority of endogenous cases of Cushing syndrome?
A
Cushing disease
8
Q
What needs to be done if adrenal tumor is suspected?
A
Adrenal CT to confirm
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
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
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
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
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
14
Q
Inability of the adrenal glands to generate enough glucocorticoids
A
Adrenal insufficiency
15
Q
Acute primary adrenal insufficiency due to hemorrhage associated with septicemia form N. meningitidis, DIC or endotoxic shock
A
Waterhouse-Friderichsen syndrome
16
Q
Hypotension, hyperkalemia, metabolic acidosis with skin and mucosal hyperpigmentation points to what disease?
A
Primary adrenal insufficiency
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
18
Q
What is the most common cause of chronic primary adrenal insufficiency in the Western world?
A
Autoimmune destruction
19
Q
What is the most common cause of chronic primary adrenal insufficiency in the developing world?
A
TB
20
Q
Adrenal atrophy or destruction by disease either from autoimmune or TB is called what?
A
Chronic primary adrenal insufficiency or Addison disease
21
Q
What cortisol and ADH levels are seen in primary adrenal insufficiency?
A
Decreased cortisol
Increased ACTH
22
Q
What cortisol and ADH levels are seen in secondary adrenal insufficiency?
A
Decreased cortisol and ACTH
23
Q
What is a common cause of tertiary adrenal insufficiency?
A
Exogenous steroid use
24
Q
How is aldosterone synthesis affected in tertiary adrenal insufficiency?
A
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
