Ch 24 - Endo Path Flashcards
1
Q
hormones from lactotroph cells
A
prolactin
2
Q
hormones from somatotroph cells
A
GH
3
Q
hormones from mammosomatotroph cells
A
prolactin, GH
4
Q
hormones from corticotroph cells
A
ATCH, POMC, MSHj
5
Q
hormones from thyrotroph cells
A
TSH
6
Q
hormones from gonadotroph cells
A
FSH, LH
7
Q
oxytocin and ADH are made in
A
the hypothalamus
8
Q
oxytocin and ADH are stored in
A
axon terminas of the posterior pituitary
9
Q
acute hemorrhage into an adenoma causing rapid enlargement of the lesion
A
pituitary apoplexy
10
Q
thyroiditis that is triggered by viral infection, usually post URI
A
granulomatous thyroiditis
11
Q
MCC of thyroid pain
A
granulomatous thyroiditis
12
Q
progressive destruction of thyroid parenchyma, hurthle cell change, mononuclear infiltrates with germinal centers w/ or w/out extensive fibrosis
A
hashimoto thyroiditis
13
Q
typically painless, lymphocytic inflammation in the thyroid, postpartum thyroiditis
A
subacute lymphocytic thyroiditis
14
Q
autoimmune disorders characterized by the production of autoantibodies against multiple thyroid proteins, esp the TSH receptor
A
graves dz
15
Q
thyroid stimulating immunoglobulin antibody
A
seen in 90% of graves dz pts
16
Q
4 causes of exopthalmos
A
gravez dz: 1) mononuclear cell infiltration of retroorbital space 2) EOM inflammaiton and edema 3) accumulation of hydrophilic GAGs 4) increased adipocytes
17
Q
pale follicular lumen with scalloped edges
A
graves dz
18
Q
lab findings of Graves dz
A
inc T3/T4, dec TSH
19
Q
thyrotoxicosis, opthalmopathy, dermopathy
A
graves dz
20
Q
Occurs in geographic areas where soil, water, and food supply contain low iodine levels and the condition is present in >10% of the population
A
Endemic goiter
21
Q
MC areas of endemic goiter
A
Mountains; Andes and Himalayas
22
Q
Substances that interfere wit thyroid hormone synthesis and examples
A
Goiter opens - cabbage, cauliflower, brussle sprouts, turnips, cassava
23
Q
Peak incidence of sporadic goiter occurring
A
Puberty or YA life
24
Q
Inheritance pattern of genetic causes of sporadic goiter
A
AR
25
Diffuse, symmetrical enlargement of the thyroid gland. Follicles lines by crowded columnar cells that may pile up to form projections. Follicles can be distended or small.
Hyperplastic phase of nontoxic goiter
26
Follicular epithelium involutes to form colloid-rich glands in thyroid
Colloid involution phase of nontoxic goiter
27
Goiter growing behind sternum or clavicles
Intrathoracic or plunging goiter
28
Long standing goiter with autonomous nodule that produces hyperthyroidism
Plummer syndrome (toxic multinodular goiter)
29
Radio iodine scan of multinodular goiter will show
Uneven iodine uptake - even an occasional hot nodule
30
somatic mutations of TSH receptor signaling pathway are found in
toxic adenomas and toxic multinodular goiters
31
MC mutations causing thyroid autonomy in
GOF mutations in TSHR and GNAS
32
hallmark of all follicular adenomas
presence of an intact, well-formed capsule encircling the tumor
33
nonfuxnl adenomas appar as __________ on radionuclide scanning
cold nodules
34
most papillary carcinomas have GOD mutations involving
RET, NKRT1, or BRAF
35
RET/PTC rearrangements are more frequent in papillary cancers arising from
radiation exposure
36
BRAF mutations in papillary carcinomas correlate with
adverse prognostic factors - mets and extrathyroid extension
37
acquired mutations that activate RAS or PI-3K/AKT of the RTK pathway
follicular carcinoma
38
(2;3)(q13;p25) translocation
follicular carcinoma
39
germline RET mutations
medullary thyroid carcinomas
40
MC form of thyroid cancer
papillary carcinoma
41
branching papillae w/ dense fibrovascular stalk covered by single or multiple layers of cuboidal epi cells
papillary thyroid carcinoma morphology
42
optically clear or empty appearance of nuclei with invaginations of cytoplasm ("pseudo-inclusions")
nuceli morphology of papillary thyroid carcinoma
43
concentrically calcified structures within cores of papillae
psammoma bodies - seen in papillary carcinoma of the thyroid
44
papillary carcinomas are _____ masses on scintisacans
cold
45
distinguish btwn follicular adenomas and minimally invasive carcinomas with
aval of the tumor-capsule-tyroid interface
46
common route of dissemination of follicular carcinomas and MC met sites
hematogenously - bone, brain, lungs, liver
47
large, pleomorphic giant cells w/ occasional multinucleate giant cells, spindle cells with sarcomatous appearance, and mixed spindle and giant cells
anaplastic thyroid carcinoma morphology
48
large bulky neck mass that spreads to adjacent neck structures and/or mets to the lung
anaplastic thyroid carcinoma
49
neuroendocrine neoplasm derived from parafollicular cells or C cells
medullary thyroid carcinoma
50
important marker for diagnosis and postop follow-up in pts with medullary carcinoma of the thyroid
calcitonin
51
morphological indicative of inherited predisposition to medullary carcinoma of the thyroid
multiple prominent clusters of C cell scattered throughout parenchyma of the thyroid tumor
52
polygonal ot simple-shaped cells that form nests, trabuecular, and possible follicles, acellular amyloid deposits, calcitonin demonstrable in cytoplasm
medullary carcinoma of the thyroid morphology
53
cases of medullary carcinoma of the thyroid are more agressive if ass'd with
MEN-2B
54
asymptomatic MEN-2 pts with RET mutations are offered
prophylactic throidectomy
55
MC b9 neoplasm of thyroid
follicular adenoma
56
genetic abnormality that is farily unique to thyroid cancer
PAX8/PPARG fusion, RAS, PI-3K, or BRAF mutations
57
midline neck mass, anterior to trachea
thyroglossal duct cyst
58
polygonal cells with central round uniform nuclei and light to dark pink cytoplasm
cheif cells in parathyroid gland
59
autonomous production of PTH usually d/t adenoma or hyperplasia
primary hyperthyroidism
60
compensatory hypersecretion of PTH usually from chronic renal failure
secondary hyperparathyroidism
61
MCC of primary hyperparathyroidism
solitary parathyroid adenoma
62
pericentromeric inversion of ch 11
cyclin D1 gene inversion
63
location of sporadic mutations found in tumor suppressor gene of MEN1
ch11q13
64
rare AR mutation in the CASR gene
familial hypocalciuric hypercalcemia
65
increased osteoclast activity, peritrabecular dribrosis, cystic brown tumors
von Recklinghausen dz of the bone - generalized osteitis fibrosa cystric d/t severe hyperparathyroidism
66
MC areas of sites of metastatic calcification
stomach, lungs, myocardium, and blood vessels
67
hypercalcemia is MCly seen secondarily to these neoplasias
solid - lung, breask, head, neck; hematologic - multiple myeloma
68
painful bones, renal stone, abdominal groans, psychic moans
s/s of primary hyperparathyroidism
69
hypoparathyroidism, mucocutaneous candidiasis, primary renal insufficiency
autoimmune polyendocrine sydrome type 1 (APS1)
70
gene mutation found in APS1
AIRE gene
71
AD hypoparathyroidism is caused by
GOF mutation in CASR gene
72
hallmark of hypocalcemia
tetany
73
beta islet cells
insulin
74
alpha islet cells
glucacon
75
delta islet cells
somatostatin
76
PP cells
pancreatic polypeptide
77
D1 cells
VIP
78
enterochromaffin cells
serotonin
79
secretes GIP into the proximal small bowel
enterendocrine K cells
80
secrets GLP-1 into the distal ileum and colon
L cells
81
2 most important incretins for regulation of insulin release from pancreas
GIP, GLP-1
82
enzymes that break down incretins
DPP4
83
two new agents from tx of type 2 diabetes
GLP-1 agonists, DPP-4 inhibitors
84
principal metabolic function of insulin
increase rate of glucose transport into certain cells in the body
85
mediators of GLUT-4 transporter to the plasma membrane
AKT, CBL
86
locus that has greatest contribution in genetic susceptibility to type 1 DM
HAL gene cluster on 6p21
87
genetic profile of individuals with highest risk of inheriting T1 DM
DR3 or DR4 haplotype with a DQ8 haplotype
88
islet autoantigen targets
insulin, GAD, ICA512
89
most important environmental risk factor in development of T2 DM
obesity
90
2 cardinal metabolic defects characterized by type 2 DM
1) decreased response of peripheral tissue to insulin (insulin resistance) 2) inadequate insulin secretion
91
major tissues where insulin resistance manifests
liver, skeletal mm, and adipose tissue
92
characteristic found in islet cells of long standing T2 DM pts
amyloid deposition
93
MC mutations seen in MODY pts w/ beta cell function defects
GCK gene mutations
94
insulin resistance, diabetes, hypertriglyceridemia, acanthosis nigricans, abnormal fat depotsition in the liver
lipoatrophic diabetes - impaired response to insulin
95
classic triad of diabetes
polydipsia, polyphagia, polyuria
96
severe acute metabolic complication seen MCly in T1 but can also be seen in T2 DM
DKA
97
fatigue, N/V, severe abd pain, fruity breath, and deep, labored breathing
DKA s/s
98
condition seen in T2 DM pts with severe dehdration and blood glucose levels that can be as high as 600-1200mg.dL
hyperosmolar hyperosmotic syndrome (HHS)
99
MC acute metabolic complication in either type of DM
hypoglycemia
100
recommended matinence of HbA1c in diabetics
101
two factors that highly contribute to diabetic microangiopathy
AGEs and activated PKC
102
leads to cataract formation in diabetics
accumulation of sorbitol
103
decreased levels of NADPH compromises
GSH regeneration > decreased ROS defense
104
MCC of death in diabetics
MI from atherosclerosis of the coronary aa
105
PAS + ovoid or spherical, laminated nodules of matrix situated in the periphery of the glomerulus
Kimmelstiel-Wilson dz aka nodular glomerulosclerosis seen in DM pts
106
pattern of acute pyelonephritis seen much MCly in diabetics than nondiabetics
necrotizing papillitis
107
inhibits fibrinolysis and acts as a procoagulant in the formation of atherosclerotic plaques, levels are elevated in diabetics
PAI-1
108
earliest manifestation of diabetic nephropathy
microalbuminuria
109
LOF mutations leading to mTOR oncogenic signaling pathway in pancreatic endocrine neoplasms
PTEN, 2TSC
110
nearly half of PanNETs have somatic mutations in either of these two genes
ATRX, DAXX
111
pancreatic islet cell lesions, hypersecretion of gastric acid, severe peptic ulcerations
Zollinger-Ellison syndrome
112
skin rash, anemia, increased levels of glucagon
alpha-cell tumors
113
DM, cholelithiasis, steatorrhea, hypochlorhydria
delta-cell tumors
114
watery diarrhea, hypokalemia, acholhydria
WDHA syndrome - VIPoma
115
zona fasiculata
glucocorticoids (cortisol)
116
zona glomerulosa
mineralocroticoids (aldosterone)
117
zona reticularis
sex steroids (estrogens and androgens)
118
elevated serum cortisol, low serum ACTH
ACTH-independent Cushing syndrome
119
hormones that regulate sporadic macronodular adrenal hyperplasia
gastric inhibitory peptide, LH, ADH
120
ACTH levels elevated and are not suppressed by admin of low dose dexamethasone, reduces ACTH with high dose
pituitary cushings
121
ACTH levels elevated, ACTH secretion is insensitive to low or high dose dexamethazone
ectopic ACTH excretion
122
ACTH levels low, ACTH secretion is insensitive to low or high dose dexamethazone
cushing's d/t adrenal tumor
123
MC manifestation of primary hyperaldosteronism
high blood pressure
124
MCC underlying cause of primary hyperaldosteronism
b/l idiopathic hyperaldosteronism (IHA)
125
hyperaldosteronism cause by a solitary adosterone-secreting adenoma
Conn syndrome
126
secondary hyperaldosteronism activates RAAS and is characterized by
increased levels of plasma renin
127
eosinophilic, laminated cytoplasmic inclusions found in aldosterone-producing adenomas
spironolactone bodies
128
21-hydroxlase def is caused by mutations in
CYP21A2
129
should be suspected in any neonate with ambiguous genitalia
CAH
130
overwhelming nisseria infection, rapidly progessive hypotension, DIC ass'd with widespread purpura, rapidly developing adreocortical insufficiency
waterhouse friederichen syndrome
131
MCC of primary adrenal insufficiency in developed countries
autoimmune adrenalitis
132
chronic mucocutaneous candidiasis and abnormalities f the skn, dental enamal, and nails ass'd with combinations of organ-specific autoimmune disorders (ex: autoimmune adrenalitis)
APS1
133
APS1 is ass'd with mutations of
AIRE
134
adrenal insufficiency and autoimmune thyroiditis or DM T1
APS2
135
inheritance pattern of congenital adrenal hypoplasia
x-linked
136
autoantibodiees against IL-17 and IL-22
APS1
137
MC adrenal neoplasm in children
carcinoma
138
common carcinoma that mets to the adrenals
bronchogenic
139
turns brown when incubated with potassium dichromate solution
pehochromocytoma
140
definitive sis of malignancy in pehochromocytomas is based exclusively on
metastases
141
lab diagnosis of pheo is based on increased urinary excretion of
free catecholamines + metabolites (VMA, metanephrines)
142
MC manifestation of MEN-1
primary hyperparathyroidism
143
leading cause of morbidity and morality in MEN-1
endocrine pancreatic tumors
144
MC anterior pituitary tumor seen in MEN-1
prolactinoma
145
MC site of gastrinomas in MEN-1 pts
duodenum
146
pheo, medullary carcinoma of the thyroid, parathyroid hyperplasia
sipple syndrome, MEN2A
147
MEN2A is clinically distinct from MEN1 d/t
GOF mutations in RET (ch10q11.2)
148
pheo, multifocal thyroid carcinoma, ganglioneuromas, marfanoid habitus
MEN2B
