test #35 4.26 Flashcards Preview

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Flashcards in test #35 4.26 Deck (124)
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1
Q
calcineurin inhibitors
A
cyclosporin and tacrolimus
2
Q
germline mutations in RET proto-onco gene affect cells of what origin
A
neural crest cells!

men2a: medullary thyroid carcinoma (parafollicular C cells of thyroid), pheo, and parathyroid gland

men2b: medullary thyroid carcinoma, pheo, and mucosal neuromas
3
Q
thyroid parafollicular cells arise from..
A
4th and 5th pharyngeal pouches
4
Q
name development of pharyngeal pouches (5)
A
1. ears (middle ear, mastoid air cells, eustachian tube = endoderm lined)
2. tonsils
3. inferior parathyroid, thymus
4. superior parathyroid, medullary thyroid parafollicular cell
5. medullary thyroid parafollicular cells

5
Q
development of thyroid
A
thyroid follicular cells = outpouching of pharyngeal epithelium, migrate to lower neck & fuse with parafollicular C cells from 4/5th pharyngeal pouch.
6
Q
pathophysiology of mutation in VHL
A
VHL gene regulates transcription of HIF-1 (hypoxia inducible factor).

unregulated HIF --> increased VEGF & EPO

= hemangiomas

also associated w/ pheos & RCC
7
Q
projection
A
misattributing one's own unconscious, undesired thoughts, feelings, impulses onto another person, who does NOT actually have them

occurs when a person ins unable to express difficult thoughts themselves.

transplant thoughts to another --> relieves difficult feelings without causing internal confluct of self-expression.

angry w/ parents, think parents mad at you.

lack insign and acknowledgement of their own motivations & feelings
8
Q
identification
A
modeling one's behavior after someone perceived to be more powerful / prestigious.

may be admirable or non admirable.

child of abusive father becomes child abuser
9
Q
acting out
A
avoiding unacceptable feelings by behaving badly

expressing unwanted thoughts/impulses through action instead of reflection and mature discourse
10
Q
most severe trisomy
A
trisomy 13 -- patau
usu die in first week
only 5% survive 6 months
11
Q
describe trisomy 13-patau
A
associated w/ early defect in prechordal mesoderm

midface, eye, forebrain most affected.

1. head/neck: severe cleft lip/palate, microopthalmia or anopthalmis, coloboma, cylops, malformed/absent nose, deafness, scalp defects

2. CNS: severe MR, microcephaly, holoprosencephaly, absent olfactory nerve or bulb, NT defects

3. extremities: polydactyly, rocker-bottom feet

4. renal: polycystic kidney disease

5. GI: abdominal wall defects: omphalocele or umbilica hernia, pyloric stenosis
12
Q
47 XXY
A
klinefelter; tall male w/ gynecomastia, small testes, and infertility
13
Q
47 XXX
A
clinically silent, maybe slightly decreased IQ
14
Q
47 XYY
A
tall stature, acne, delays in motor and language
15
Q
k-ras mutations common in..
A
pancreatic malignancy
16
Q
when is opening snap of mitral stenosis head
A
really really close to closure of S2

due to tensing of MV leaflet after cusps completed their opening excursion

17
Q
relationship between time of A2-opening snap in mitral stenosis and severity
A
inverse. shorter interval, more severe.

bc higher steady state left atrial pressure.
18
Q
control symptoms of a neonate in opiod withdrawal (leaky baby)
A
tincture of opium or paregoric
19
Q
what maintains blood brain barrier
A
tight junctions [claudins & occludins]

between endothelial cells in capillary beds of CNS.

materials can only move transcellularly or carrier mediated.
20
Q
why use naficillin usu
A
penicillinase-resistant penicillin.

use often against s. aureus.

skin infections and soft tissue infection -- folliculitis, abscesses.
21
Q
rx for lung abscesses
A
usu clindamycin

'above the belt' anaerobes'
22
Q
petechia, purpura, ecchymoses? blanch?
A
petechia 1cm

do not blanch, bc RBC are not in vasculature. RBC leaked in skin/subcutaneous tissue

PPE
23
Q
which blanch. telangietcasia or petechiae/purpura/ecchymoses?
A
teleangiectasia, bc in a vessel
24
Q
intranasal glucocortoids localization
A
unlikely to cause systemic immunosuppression -- very localized
25
Q
causes of candida vaginitis
A
OFTEN:
1. ANTIBIOTIC use! suppress normal flora, facilitates candida overgrowth.

2. contraception, high estrogen
3. systemic corticosteroids
4. uncontrolled diabetes
5. immunosuppression
26
Q
normal pH of vagina? gardnerella infection? trichomonas? candida vulvovaginitis?
A
normal: 3.8-4.2

gardnerella: pH > 4.2 (more basic)

trichomonas: pH >4.5 (more basic)

candida: pH normal, acidic
27
Q
major bacterial flora of vagina
A
gram + lactobacilli

also: corynebacterium, fungi (candida), group B strep, e. coli

usu pH ~4
28
Q
decrease thickening of vaginal epithelium and decreased glycogen associated with..
A
postmenopausal & lactating women
29
Q
elevation of vaginal secretion pH suggests (pH > 4.5)
A
gardnerella or trichomonas
30
Q
how are many carcinogens activated in humans
A
via microsomal monooxygenase (cytochrome p450 monooxygenease)

often convert procarcinogen -> carcinogen

like benzopyrene-induced lung cancer
31
Q
which part of renal tubules is absolutely impermeable to water? which is low, but technically possible w/ ADH
A
impermeable: thick ascending loop

early DCT: very low permeability
late DCT: vary based on ADH
32
Q
where are parietal cells located in gastric mucosa?
A
superficial gastric glands
33
Q
where are chief cells located in gastric mucosa?
A
deep gastric glands
34
Q
5 layers of gastric mucosa
A
1. simple columnar epithlieum
- secrete mucus to protect

2. superficial lamina propria
- contains gastric pits
(mult glands connect to single pit)

3. superficial gastric gland
- has parietal cells (oxyntic, pale, round, plate-like

4. deep gastric gland
- chief cells (pepsinogen) granular basophillic

5. muscularis mucosa
- separates lamina propria from submucosa

submucosa -- vascularized CT
35
Q
fusobacterium, peptostreptococcus, bacteroides in lung..
A
aspiration pneumonia

alcoholic, seizure, severe neuro disease
36
Q
3 ways to get lung abscess
A
1. aspiration of oropharyngeal contents: fusobacterium, peptostreptococcus, bacteroides

2. complication of bacterial pneumonia: local immunosuppression, old oage, underlying chronic disease, necrotizing: s. aureus, e.coli, k. pneumonia, or s. pneumo type 3

3. specticemia or infective endocarditis: hematogenous spread of infection -- usu multiple. most common: staph or strep, e. coli, fungi.
37
Q
where does blood accumulate in a subdural hematoma?
A
between dura mater and arachnoid. usu venous

gradual onset of symptoms.
crescent shaped
cortical briding veins
38
Q
where does 2,3 DPG bind?
A
picket formed by 2 beta chains (+ charged amino acid, histidine, which attracts - charged phosphates on 2,3 DPG

(histidine absent in HbF, so it doesn't bind)
39
Q
HgbA1c binding 2,3 DPG?
A
does not affect binding to 2,3 DPG
40
Q
chlorpromazine associated w/
A
corneal deposits
41
Q
haloperidol associated w/
A
extrapyramidal symptoms
42
Q
ziprasidone associated w/
A
long QT
43
Q
olanzepine associated w/
A
weight gain
44
Q
clozapine associated w/
A
agranulocytosis and seizures
45
Q
thioridazine associated with
A
retinal deposits that resemble retinitis pigmentosa
46
Q
leading cause of blindness in industrial nations
A
macular degeneration
47
Q
dry vs. wet age related macula degeneration?
A
dry: subretinal drusen or pigment changes

wet: abnormal blood vessels with subretinal fluid/hemorrhage, grey subretinal membrane, or neovascularization
48
Q
progression of macular degeneration
A
DRY (gradual vision loss in one or both eyes) usu progresses to WET (more common, acute vision loss over period of days-weeks)
49
Q
rx for dry age related macular degeneration?
A
antioxidant vitamins and zinc
50
Q
rx for wet age related macular degeneration
A
anti-VEGF, laser or phototherapy

(limit development of choroidal neovascular membranes)

intra-vitreous ranibizumab and pegaptanib
51
Q
epidermal growth factor receptor inhibitors (erlotinib & gefitinib) rx for?
A
non small cell lung cancer
52
Q
anti-IL2 for..
A
use for immunosupression for GVHD and organ transplant
53
Q
anti-TNF-alpha for..
A
inflammatory autoimmune conditions: RA, IBD, seronegative spondyloarthropathies
54
Q
infertility, sinusitis, bronchiectasis
A
kartageners (mutation in dyenin arm)
55
Q
hypocalcemic tetany & recurrent viral and fungal infxn
A
digeorge 22q11.2 deletion
56
Q
3 cardiac issues associated w/ marfans
A
all related to cystic medial necrosis

1. dissection aortic aneurysm
2. aortic valve incompetence
3. mitral valve prolapse

autosomal dominant, fibrillin
57
Q
2 possible cardiac issues in digeorge
A
tetrology of fallot & aortic arch abnormalities
58
Q
cardiac abnormality in friedrich's ataxia
A
hypertrophic cardiomyopathy

autosomal recessive:
59
Q
mutation in friedrich's ataxia
A
autosomal recessive.

mutation in frataxin, mitochondrial protein impt in iron homeostasis & respiratory fxn
60
Q
VIPoma
A
WDHA. "pancreatic cholera"

watery diarrhea, hypokalemia, achlorhydria

rx: somatostatin

61
Q
VIP made by..
A
pancreatic islet cells, neurons of GI mucosa

relaxes smooth muscle, inhibits H+ secretion, stimulates pancreatic bicarb and Cl- secretion
62
Q
who makes gastrin
A
G cells in stomach mucosa.
located in antrum.
63
Q
glucagonoma may present w/
A
(1) secondary diabetes mellitus
(2) necrolytic migratory erythema of skin
64
Q
optic tract fibers project to.. (4)
A
mainly 1. LGN
2. superior colliculus (reflex haze)
3. pretectal area (light reflex)
4. suprachiasmatic nucleus (circadian rhythm)
65
Q
meyer's loop connects
A
LGN, through temporal lobe, LINGUAL gyrus of striate (info form lower retina, upper world)
66
Q
UPPER fibers of optic radiation carry
A
LGN, through parietal to CUNEUS gyrus of striate (info from upper retina, lower world)
67
Q
what can stimulate glycogen utilization in muscles?
A
1. sustained Ca2+ ITSELF during contraction --> direct activation of phosphorylase kinase (activates glycogen phosphorylase)

2. B1/glucagon-dependent increase in cAMP -> PKA ->
- phosphorylates 'phosphorylase kinase' [activates it to phosphorylate glycogen phosphorylase]
- & phosphorylates glycogen synthase to inactivate it.
68
Q
toxicity of amphotericin B
A
renal toxicity, hypomagnesium, and hypokalemia

(require daily supplementation)

renal toxicity: due to reduced GFR (renal vasoconstriction), directly toxic to epithelial cells, causing ATN, electrolyte disturbances

can lead to reduced EPO
69
Q
osler-weber-rendu
A
hereditary hemorrhagic telangiectasia

autosomal dominant

70
Q
telengiectasia on skin & mucus membranes w. rupture --> epistaxis, GI bleed, hematuria?
A
hereditary hemorrhagic telangiectasia

(osler-weber-rendu)
71
Q
serum marker for heptocellular carcinoma
A
alpha-fetoprotein

does NOT correlate well w/ size, stage, or prognosis.

also associated w/ pregnancy, gonadal tumors, chronic liver disease (viral hepatitis)
72
Q
CEA (carcinoembryonic antigen) is a marker for
A
colorectal cancer.

aid in staging, planning treatment, determining prognosis.

NOT a good screening tool, as it can be elevated w/ benign disease
73
Q
CA-125 marker..
A
marker for ovarian cancer

also elevated in: endometrial, lung, breast, pancreatic cancer

also benign: endometriosis, cirrhosis, PID

NOT a good screening tool.
74
Q
PSA is a marker for..
A
extent of prostate cancer & evaluating response to treatment

commonly used to screen, but controversial
75
Q
acid phosphatase marker for..
A
secondary prostate tumor marker antigen.

also elevated in patients w/ active osteoclast-indiated bone resorption
76
Q
hCG is a marker for
A
gestational trophoblastic disease
77
Q
sudden cardiac death defined by..
A
cardiac arrest that begins 1hr of a precipitating event that ultimately proves fatal.

most common: ventricular fibrillation
78
Q
most common cause of death in patients suffering from MI during prehospital phase (prior to arriving in ED)
A
ventricular fibrillation
79
Q
when do ventricular mural thrombosis occur post MI
A
after at least 48 hrs in
80
Q
when do ventricular rupture most commonly occur post MI?
A
3-7 days in
81
Q
most common tumor in HIV+ patients
A
kaposi sarcoma

(HHV8 infects vascular & lymphatic endothelial cells)
82
Q
presentation of kaposi sarcoma
A
multiple blue-violet or brownish dermal plaques, first appear on feet & legs before spreading proximally.

can develop on mucosal membranes of face and genitals.

can spread to lungs and GI tract
83
Q
histology of kaposi sarcoma
A
spindle and endothelial cell proliferation, red blood cell extravasation, inflammation
84
Q
CMV in HIV+ patient causes (3)
A
1. esophagitis
2. colitis
3. retinitis
85
Q
bilateral retinal hemorrhage & acute subdural in a baby?
A
shaken baby syndrome, child abuse

infant has: larger head, larger subarachnoid space, higher brain water content, decreased cervical muscle tone

rupture of congested retinal veins
86
Q
ristocetin test
A
ristocetin activates GpIb receptors on platelets, makes available for vWF binding.

if vWF levels low, there is poor platelet aggregation in response to ristocetin
87
Q
half life of factor VIII w/ vWF? without?
A
w/ vWF: 12 hours
w/o vWF: 2 hours
88
Q
desmopressin in heme
A
releases vWF from endothelial cells
89
Q
glanzmann thrombasthenia
A
hereditary deficiency of GbIIbIIIa.

mucocutaneous bleeding and increased bleeding time.

platelet aggregation in response to ristocetin is fine (GbIb is fine). but decreased w/ addn of ADP (GbIIbIIIa messed up)
90
Q
tardive dyskinesia
A
involuntary perioral movements: biting, chewing, grimacing, tongue protrusions

involuntary choreoathetoid movt can also be seen

usu between 4 months-4years of treatment. usu irreversible
91
Q
atypical antipsychotic most likely to cause extrapyramidal symptoms? least?
A
most likely: risperidone
(also galactorhea and amenorrhea)

least: clozapine
92
Q
acute dystonia. time, symptoms, rx?
A
between 4hr-4days of antipsychotic:

muscle spasm / stiffness, tongue protrusions, opisthonus, oculogyric crisis (forced sustained elevation of eyes in upward position)

rx: antihistamine or anticholingerics

diphenhydramine, benztropine, trihyxyphenidyl
93
Q
parkinsonism (drug-induced)? rx?
A
between 4 days-4months of treatment.

cogwheel rigidity, masked facies, bradykinesis, pill-rolling, tremor, shuffling gait

rx: benztropine anticholinergic
94
Q
akathisia
A
subjective feeling of restlessness that compels patient to constantly move around.
95
Q
pH urease test for h. pylori
A
urea --> NH3 + CO2, pH increase, color change in phenol red.
96
Q
E. coli that does NOT ferment sorbitol and does NOT produce glucoronidase?
A
EHEC, which has shiga-like toxin that inactivates 60S ribosomal subunit --> prevents tRNA binding
97
Q
many e. coli (except EHEC) are able to..
A
(1) ferment sorbitol
(2) produce glucoronidase

EXCEPT EHEC
98
Q
which bacteria can activate adenylate cyclase (6)
A
-b. pertussis (pertussis toxin)
-b. anthracis (edema factor)
-ETEC (heat labile toxin)
-camplyobacteri jejuni (enterotoxin)
-bacillus cereus (heat labile toxin
-v. cholerae (choleragen toxin)

increase cAMP, decreased absorption, increased secretion
99
Q
which toxins activate guanylate cyclase (2)
A
-ETEC (heat stabile toxin)
-yersinia enerocolitica (y. enterocolitica enterotoxin)

increase cGMP, watery diarrhea, electrolyte loss
100
Q
which toxins inactivate EF-2 (2)
A
-corynebacterium diptheriae (diptheria toxin)
-pseudomonas aeruginosa (exotoxin A)

ADP ribosylation
101
Q
which toxins disrupt cytoskeleton?
A
-c difficile
102
Q
polyarteritis nodosa associated with..
A
hepatitis B
103
Q
giant cell (temporal) arteritis associated w.
A
polymyalgia rheumatica
104
Q
what is the most common vasculitis from antbiotic use?
A
microscopic polyangiitis / leukoclastic angiitis / hypersensitvity angiitis

type III immune rxn, often post penicillin
105
Q
which vitamin enhances Fe2+ absorption
A
vitamin C / ascorbic acid!

bc keeps it in reduced form!
106
Q
suppositories and first-pass metabolism?
A
rectum drained by superior, middle, and inferior rectal veins..

superior --> portal circulation via IMP

middle & inferior --> darin to systemic circulation via internal iliac and internal pudendal (respectively)

2/3 of suppositories --> systemic circulation
107
Q
major determinant of bioavailability after oral intake?
A
liver blood flow (first pass metabolism)
108
Q
what constitutes first pass metabolism
A
reduction, oxidation, and hydrolysis.

in large part: cytochrome P450 system
109
Q
what constitutes second pass metabolism
A
glucoronidation, acetylation, and sulfation
110
Q
most common benign tumor in lung?
A
hamartoma aka pulmonary chondroma

has mature hyaline cartilage, fat, smooth muscle & clefts lined by respiratory epithelium.

-- excessive growth of a tissue type native to the organ of involvement
111
Q
well defined coin lesion w/ popcorn calcifications on x-rsy?
A
hamartoma aka pulmonary chondroma
112
Q
bronchioloalveolar carcinoma on x-ray
A
variant of adenocarcinoma.

almost always at lung periphery. characterestic distribution along alveolar septae w/o vascular or stroma invasion.

xray: peripheral mass or pneumonia-like consolidation
113
Q
myelofibrosis is a type of
A
chronic myeloproliferative disease
114
Q
describe basis of myelofibrosis
A
chronic myeloproliferation and atypical megakaryocyte hyperplasia.

clonally expanded megakaryocyte --> activate fibroblast--> progresive replacement of marrow space w/ collagen deposition.

early: marrow hypercellularity. later: fibrosis -> pancytopenia.

MASSIVE splenomegaly, because pt compensates w/ extramedullary hematopoesis
115
Q
myelofibrosis vs. aplastic anemia
A
myelofibrosis = massive splenomegaly, bc of extramedullary hematopoesis compensation
116
Q
rx for myeleofibrosis
A
ruxolitinib, jak 2 inhibtor
117
Q
myelodysplastic syndrome
A
stem cell disorders characterized by INEFFECTIVE HEMATOPOESIS and cell maturation defects.

may be associated pancytopenia.

bone marrow: disordered differentiation affected ALL NON-LYMPHOID (erythroid, granulocytic, monocytic, megakaryocytic) lines

findings in bone marrow: ringed sideroblasts and megalobastoid maturation.

could be: de novo mutation, environmental exposure to radiation, benzene, chemo)

risk of progression to AML

118
Q
myelopthisic anemia
A
anemia caused by space-occupying lesions in bone marrow. all hematopoetic series affected, pancytopenia expected.

most common infiltrator: metastatic carcinomas w/ associated fibrosis
119
Q
aplastic anemia (3 characterestics)? bone marrow? histopatholgoy?
A
1. pancyotpenia
2. low retic count
3. absent splenomegaly

bone marrow aspirate: dry

histopath:marrow replacement w/ fat cells and fibrous stroma

120
Q
fanconi defective DNA repair causes..
A
aplastic anemia
121
Q
cryoprecipitate can be used to treat..
A
coagulation deficiency of fibrinogen and factor VIII (hemophilia 8)

has: fibrinogen, factor VIII, factor XIII, vWF and fibronectin
122
Q
risks of blood transfusions (5)
A
-infection transmission (low)
-iron overload
-transfusion reaction (i.e. IgA deficiency, ABO mismatch)
-hypocalcemia (due to citrate = calcium chelator)
-hyperkalemia (RBC may lyse in old blood units
123
Q
pseudo-pelger-huet anomaly
A
with myelodysplastic syndrome:

neutrophils w/ bilobed nuclei (two nuclear masses) connected w/ a thin filament of chromatin.

typically seen after chemotherapy
124
Q
hairy cell leukemia can often present with? rx?
A
pancytopenia and splenomegaly

rx: cladribine (adenosine analogue resistant to adenosine deaminase)