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Quiz 58 Flashcards

1
Q

What is seen on EEG in CJD?

A

Periodic biphasic or triphasic sharp wave complexes

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2
Q

What disease do you seen intracellular alpha-synuclein inclusion bodies?

A

These are Lewy bodies; Parkinson disease

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3
Q

Loss of pigmented neurons in the substantia nigra is seen in what disease?

A

Parkinson disease

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4
Q

Sharp-bordered, eosinophilic inclusions (Negri bodeis) are seen in what disease?

A

Rabies encephalitis

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5
Q

What disease do you see intracellular accumulations of hyperphosphorylated tau?

A

Alzheimer disease

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6
Q

Arteriovenous fistula does what to the venous return curve and the cardiac output curve?

A

Venous return curve is shifted to the right; the cardiac output curve does not change (ie the contractility has not changed)

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7
Q

What is ingested in infant botulism?

A

Spores from Clostridium botulinum

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8
Q

Adult botulinum is due to ingestion of what?

A

Toxin

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9
Q

What is the gram stain and morphology of C. Botulinum?

A

Gram positive rod

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10
Q

Botulinum toxin inhibits the release of what?

A

Acetylcholine

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11
Q

What “cancers” are seen in MEN2A?

A

Pheochromocytoma, parathyroid hyperplasia, medullary thyroid carcinoma

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12
Q

What mutation is associated with MEN2A?

A

RET mutation - becomes a proto-oncogene

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13
Q

What does RET encode for? What diseases are seen when RET is mutation?

A

RET encodes for a tyrosine kinase; mutations are seen in MEN2A and MEN2B

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14
Q

What characteristics are seen in MEN2B?

A

Medullary thyroid carcinoma (RET mutation), pheochromocytoma, mucosal neuromas and marfanoid habitus

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15
Q

What cancer is associated with BCL-2 oncogene?

A

Follicular lymphoma

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16
Q

14;18 translocation results in what? What cancer is this seen in?

A

Results in BCL-2; seen in follicular lymphoma

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17
Q

What translocation is seen in follicular lymphoma?

A

14;18 resulting in BCL-2

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18
Q

What translocation is seen in Burkitt lymphoma?

A

8;14

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19
Q

What caners are associated with HER2 mutations?

A

Breast, ovarian and gastric

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20
Q

Mutation in HER2 results in what?

A

Gene amplification event

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21
Q

What cancer is L-myc associated with?

A

Small cell carcinoma of the lung

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22
Q

What characteristics are seen in MEN1?

A

Parathyroid tumor, pituitary tumor (prolactinoma or GH usually), pancreatic endocrine tumor

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23
Q

What mutation is seen in MEN1?

A

Mutation in MEN1 - menin; a tumor suppressor

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24
Q

Medullary thyroid cancer, pheochromocytoma, and mucosal neuromas with marfanoid habitus is seen in what disease?

A

MEN2B

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25
Parathyroid tumors, medullary thyroid carcinoma and pheochromocytomas are seen in what disease?
MEN2A
26
What is a leading cause of myocarditis in latin American?
Trypanosoma cruzi
27
What is Chagas disease?
Dilated cardiomyopathy, megacolon and megaesophagus due to Trypanosoma cruzi
28
Trypanosoma cruzi causes what disease?
Chagas disease
29
How is trypanosomoa cruzi transmitted?
Reduviid bug
30
What does the reduviid bug transmit?
T. Cruzi
31
Patient from Latin America presents with SOB, palpitations and pulmonary congestion and cardiomegaly on CXR, what do you think is the cause?
Infection with T. Cruzi
32
What is an early (acute) sign of infection with T cruzi?
Romana sign - unilateral periorbital swelling
33
What transmits Yersinia pestis?
Flea
34
What does yersinia pestis cause?
Bubonic plague
35
What transmits Rickettsia prowazekii?
Louse
36
What does Rickettsia prowazekii cause?
Epidemic typhus
37
What does the rash from Rickettsia prowazekii look like?
Rash on trunk that spreads out, sparing the palms and and soles
38
In VW disease, what two lab findings stay normal, what two increase?
Platelet count and PT stay normal; PTT and bleeding time increase
39
Abnormal response to ristocetin that corrects with normal plasma is seen in what disease?
VW disease
40
What factor is decreased in Hemophilia A?
Factor VIII
41
Factor VIII deficiency results in what abnormal lab(s) finding?
Increased PTT; PT, bleeding time, and plt count all normal
42
What do labs show in DIC?
Low platelets and fibrinogen, increased BT, PT, PTT
43
D-dimer is a specific test for what?
Fibrin degradation products
44
What symptoms are seen in Zenker diverticulum?
Symptoms of halitosis, dysphagia, change in voice, obstruction of the esophagus
45
What is Zenker diverticulum?
False diverticulum; herniation of mucosa at a point of weakness at the junction of the pharynx and esophagus
46
What is seen in the urine in a patient with a pheochromocytoma?
Vanillylmandelic acid and homovanillic acid
47
Pheochromocytomas can be a part of what hereditary syndrome that also can cause renal cell carcinoma?
Van Hippel-Lindau syndrome
48
What characteristics are seen in von Hippel-Lindau disease?
Hemangioblastomas, angiomatosis, bilateral RCC, pheochromocytoma
49
What is the inheritance pattern for vHL?
AD
50
What is the mutation that causes vHL disease?
Mutation in VHL - Tumor suppressor gene
51
What function does the VHL gene serve?
Inhibits hypoxia inducible factor 1a
52
What is reaction formation?
Unconscious adoption of behavior opposite to that which would reflect true feelings and intentions
53
If someone is manifesting the opposite of how they feel - eg feel love but show hate ("girls have cooties"), this is an example of what defense mechanism?
Reaction formation
54
Obsessive-compulsive behaviors often exhibit what type of defense mechanism?
Undoing - reversing the unacceptable
55
Taking an unacceptable impulse and making it into an acceptable channel (eg art, literature, mentoring) is an example of what type of defense mechanism?
Sublimation
56
A man who wants to cheat on his wife accuses his wife of being unfaithful is an example of what defense?
Projection
57
Cafe-au-lait spots are seen in what disease?
NF type 1
58
What is the inheritance pattern of NF1?
AD
59
What chromosome is responsible for NF1?
17
60
What characteristics are seen in NF type 1?
Cafe-au-lait spots, lisch nodules, pheochromocytoma, cutaneous neurofibromas, optic gliomas
61
What chromosome is responsible for NF2?
22
62
What is the inheritance pattern for NF2?
AD
63
What findings are associated with NF2?
Bilateral acoustic schwannomas, juvenile cataracts, meningiomas, ependymomas
64
What connects the left pulmonary artery to the aortic arch?
Ductus arteriosus
65
Patent ductus arteriosus causes blood to flow in what direction?
Left to right - from aorta into left pulmonary artery
66
What type of murmur is heard with PDA? Where is it best heard?
Machine like, LUSB
67
During fetal circulation, what direction is blood flowing through the ductus arteriosus?
From left pulm. Artery to aorta
68
Treatment with tPA can cause a change in what lab(s)?
PT and PTT will be prolonged
69
What is the most common cause of neonatal meningitis?
GBS - strep agalactiae
70
What is the second most common cause of neonatal meningitis?
E. Coli
71
What is the third most common cause of neonatal meningitis?
Listeria
72
Why should a mother avoid delicatessen foods?
Help prevent neonate from being infected with Listeria monocytogenes
73
What is the gram stain and morphology of listeria?
Gram positive rod
74
Mutation in CDH1 results in what type of cancer?
Gastric adenocarcinoma; infiltrating lobular breast cancer
75
What does the gene CDH1 code for?
E-cadherin
76
Mosaic pattern of newly formed woven bone is specific for what disease?
Pagent disease of bone
77
What affect does Paget's disease have on the heart?
Can cause high output cardiac failure
78
What cancer are patients with Paget's disease predisposed to?
Osteosarcoma
79
What region of the bone does Ewing sarcoma occur?
Mainly diaphysis of long bones
80
What is seen on xray of a Ewing sarcoma?
"Onion skin effect"
81
What translocation is characteristic of Ewing sarcoma?
11:22
82
What type of cells are seen in Ewing sarcoma? (Describe appearance)
Anaplastic small blue malignant tumor cells
83
Intracytoplasmic granules of eosinophils contain what?
Crystalline cores that have major basic protein
84
What function does major basic protein serve?
Destroy parasites and contributes to epithelial damage during asthma
85
What anticonvulsants induce the CYP450 system?
Phenytoin, carbamazepine, barbiturate
86
What antibiotic induces the CYP450 system?
Rifampin
87
What virus(es) can reverse transcribe?
Retroviruses (HIV) and hepadnaviruses (HBV)
88
What arteries make up the circle of willis?
Anterior cerebral, anterior communicating, internal carotid, posterior cerebral, posterior communicating
89
Opening snap can be heard on what heart murmur?
Mitral stenosis
90
Exposure to vinyl chloride increases risk for wha?
Hepatic angiosarcoma
91
What predisposing factor is seen in hepatic angiosarcoma?
Exposure to vinyl chloride; also exposure to arsenic is another risk factor
92
Exposure to benzene increases risk for what cancer?
Leukemia
93
What type of HSR is rheumatic fever?
Type II
94
What region of the stomach is affected by H. Pylori infection?
Antrum
95
What is seen on biopsy of the stomach in H. Pylori infection?
Chronic inflammation with lymphocytic infiltration of the mucosa
96
Type B gastritis is caused from what?
H. Pylori
97
Type A gastritis is caused from what?
Autoimmunity to parietal cells
98
Autoimmune gastritis affects what region of the stomach?
Fundus and the body
99
Chronic alcohol use is associated with what type of gastritis?
Acute erosive
100
Cushing ulcer causes what?
Acute erosive gastritis
101
What is HbS?
Type of Hgb seen in sickle cell anemia - point mutation of 6th aa of beta-globin chain
102
A substitution of glutamic acid with valine results in what type of Hgb? What disease?
HbS; sickle cell anemia
103
How many alpha globin chains are there?
4
104
Two alpha globin gene mutations causes what?
Thalassemia trait
105
Three alpha globin gene mutations result in what?
Hb H disease
106
Four alpha globin gene mutations results in what?
Hgb Bart disease
107
What is the result of Hb Barts?
Incompatible with life - causes hydrops fetalis
108
What muscles are affected first in DMD?
LE proximal muscles - glutes and quads
109
What anterior pituitary hormones are inhibited by somatostatin?
Growth hormone and TSH
110
What cells release testosterone in the male? What hormone acts on these cells to promote testosterone release?
LH acts on Leydig cells
111
Hyperplastic gastropathy characterized by enlarged rugal folds is seen in what disease?
Menetrier disease
112
Menetrier disease leads to loss of what macromolecule?
Protein; "protein-losing gastroenteropathy"
113
What hormones are most important to replace in pituitary apoplexy?
Glucocorticoids an thyroid hormone

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