test #35 4.26 Flashcards
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
- ears (middle ear, mastoid air cells, eustachian tube = endoderm lined)
- tonsils
- inferior parathyroid, thymus
- superior parathyroid, medullary thyroid parafollicular cell
- 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.
- head/neck: severe cleft lip/palate, microopthalmia or anopthalmis, coloboma, cylops, malformed/absent nose, deafness, scalp defects
- CNS: severe MR, microcephaly, holoprosencephaly, absent olfactory nerve or bulb, NT defects
- extremities: polydactyly, rocker-bottom feet
- renal: polycystic kidney disease
- 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
causes of candida vaginitis
OFTEN:
1. ANTIBIOTIC use! suppress normal flora, facilitates candida overgrowth.
2. contraception, high estrogen
3. systemic corticosteroids
4. uncontrolled diabetes
5. immunosuppression
26
normal pH of vagina? gardnerella infection? trichomonas? candida vulvovaginitis?
normal: 3.8-4.2
gardnerella: pH > 4.2 (more basic)
trichomonas: pH >4.5 (more basic)
candida: pH normal, acidic
27
major bacterial flora of vagina
gram + lactobacilli
also: corynebacterium, fungi (candida), group B strep, e. coli
usu pH ~4
28
decrease thickening of vaginal epithelium and decreased glycogen associated with..
postmenopausal & lactating women
29
elevation of vaginal secretion pH suggests (pH > 4.5)
gardnerella or trichomonas
30
how are many carcinogens activated in humans
via microsomal monooxygenase (cytochrome p450 monooxygenease)
often convert procarcinogen -> carcinogen
like benzopyrene-induced lung cancer
31
which part of renal tubules is absolutely impermeable to water? which is low, but technically possible w/ ADH
impermeable: thick ascending loop
early DCT: very low permeability
late DCT: vary based on ADH
32
where are parietal cells located in gastric mucosa?
superficial gastric glands
33
where are chief cells located in gastric mucosa?
deep gastric glands
34
5 layers of gastric mucosa
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
fusobacterium, peptostreptococcus, bacteroides in lung..
aspiration pneumonia
alcoholic, seizure, severe neuro disease
36
3 ways to get lung abscess
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
where does blood accumulate in a subdural hematoma?
between dura mater and arachnoid. usu venous
gradual onset of symptoms.
crescent shaped
cortical briding veins
38
where does 2,3 DPG bind?
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
HgbA1c binding 2,3 DPG?
does not affect binding to 2,3 DPG
40
chlorpromazine associated w/
corneal deposits
41
haloperidol associated w/
extrapyramidal symptoms
42
ziprasidone associated w/
long QT
43
olanzepine associated w/
weight gain
44
clozapine associated w/
agranulocytosis and seizures
45
thioridazine associated with
retinal deposits that resemble retinitis pigmentosa
46
leading cause of blindness in industrial nations
macular degeneration
47
dry vs. wet age related macula degeneration?
dry: subretinal drusen or pigment changes
wet: abnormal blood vessels with subretinal fluid/hemorrhage, grey subretinal membrane, or neovascularization
48
progression of macular degeneration
DRY (gradual vision loss in one or both eyes) usu progresses to WET (more common, acute vision loss over period of days-weeks)
49
rx for dry age related macular degeneration?
antioxidant vitamins and zinc
50
rx for wet age related macular degeneration
anti-VEGF, laser or phototherapy
(limit development of choroidal neovascular membranes)
intra-vitreous ranibizumab and pegaptanib
51
epidermal growth factor receptor inhibitors (erlotinib & gefitinib) rx for?
non small cell lung cancer
52
anti-IL2 for..
use for immunosupression for GVHD and organ transplant
53
anti-TNF-alpha for..
inflammatory autoimmune conditions: RA, IBD, seronegative spondyloarthropathies
54
infertility, sinusitis, bronchiectasis
kartageners (mutation in dyenin arm)
55
hypocalcemic tetany & recurrent viral and fungal infxn
digeorge 22q11.2 deletion
56
3 cardiac issues associated w/ marfans
all related to cystic medial necrosis
1. dissection aortic aneurysm
2. aortic valve incompetence
3. mitral valve prolapse
autosomal dominant, fibrillin
57
2 possible cardiac issues in digeorge
tetrology of fallot & aortic arch abnormalities
58
cardiac abnormality in friedrich's ataxia
hypertrophic cardiomyopathy
autosomal recessive:
59
mutation in friedrich's ataxia
autosomal recessive.
mutation in frataxin, mitochondrial protein impt in iron homeostasis & respiratory fxn
60
VIPoma
WDHA. "pancreatic cholera"
watery diarrhea, hypokalemia, achlorhydria
rx: somatostatin
61
VIP made by..
pancreatic islet cells, neurons of GI mucosa
relaxes smooth muscle, inhibits H+ secretion, stimulates pancreatic bicarb and Cl- secretion
62
who makes gastrin
G cells in stomach mucosa.
| located in antrum.
63
glucagonoma may present w/
(1) secondary diabetes mellitus
| (2) necrolytic migratory erythema of skin
64
optic tract fibers project to.. (4)
mainly 1. LGN
2. superior colliculus (reflex haze)
3. pretectal area (light reflex)
4. suprachiasmatic nucleus (circadian rhythm)
65
meyer's loop connects
LGN, through temporal lobe, LINGUAL gyrus of striate (info form lower retina, upper world)
66
UPPER fibers of optic radiation carry
LGN, through parietal to CUNEUS gyrus of striate (info from upper retina, lower world)
67
what can stimulate glycogen utilization in muscles?
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
toxicity of amphotericin B
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
osler-weber-rendu
hereditary hemorrhagic telangiectasia
autosomal dominant
70
telengiectasia on skin & mucus membranes w. rupture --> epistaxis, GI bleed, hematuria?
hereditary hemorrhagic telangiectasia
| osler-weber-rendu
71
serum marker for heptocellular carcinoma
alpha-fetoprotein
does NOT correlate well w/ size, stage, or prognosis.
also associated w/ pregnancy, gonadal tumors, chronic liver disease (viral hepatitis)
72
CEA (carcinoembryonic antigen) is a marker for
colorectal cancer.
aid in staging, planning treatment, determining prognosis.
NOT a good screening tool, as it can be elevated w/ benign disease
73
CA-125 marker..
marker for ovarian cancer
also elevated in: endometrial, lung, breast, pancreatic cancer
also benign: endometriosis, cirrhosis, PID
NOT a good screening tool.
74
PSA is a marker for..
extent of prostate cancer & evaluating response to treatment
commonly used to screen, but controversial
75
acid phosphatase marker for..
secondary prostate tumor marker antigen.
also elevated in patients w/ active osteoclast-indiated bone resorption
76
hCG is a marker for
gestational trophoblastic disease
77
sudden cardiac death defined by..
cardiac arrest that begins 1hr of a precipitating event that ultimately proves fatal.
most common: ventricular fibrillation
78
most common cause of death in patients suffering from MI during prehospital phase (prior to arriving in ED)
ventricular fibrillation
79
when do ventricular mural thrombosis occur post MI
after at least 48 hrs in
80
when do ventricular rupture most commonly occur post MI?
3-7 days in
81
most common tumor in HIV+ patients
kaposi sarcoma
| HHV8 infects vascular & lymphatic endothelial cells
82
presentation of kaposi sarcoma
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
histology of kaposi sarcoma
spindle and endothelial cell proliferation, red blood cell extravasation, inflammation
84
CMV in HIV+ patient causes (3)
1. esophagitis
2. colitis
3. retinitis
85
bilateral retinal hemorrhage & acute subdural in a baby?
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
ristocetin test
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
half life of factor VIII w/ vWF? without?
w/ vWF: 12 hours
| w/o vWF: 2 hours
88
desmopressin in heme
releases vWF from endothelial cells
89
glanzmann thrombasthenia
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
tardive dyskinesia
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
atypical antipsychotic most likely to cause extrapyramidal symptoms? least?
most likely: risperidone
(also galactorhea and amenorrhea)
least: clozapine
92
acute dystonia. time, symptoms, rx?
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
parkinsonism (drug-induced)? rx?
between 4 days-4months of treatment.
cogwheel rigidity, masked facies, bradykinesis, pill-rolling, tremor, shuffling gait
rx: benztropine anticholinergic
94
akathisia
subjective feeling of restlessness that compels patient to constantly move around.
95
pH urease test for h. pylori
urea --> NH3 + CO2, pH increase, color change in phenol red.
96
E. coli that does NOT ferment sorbitol and does NOT produce glucoronidase?
EHEC, which has shiga-like toxin that inactivates 60S ribosomal subunit --> prevents tRNA binding
97
many e. coli (except EHEC) are able to..
(1) ferment sorbitol
(2) produce glucoronidase
EXCEPT EHEC
98
which bacteria can activate adenylate cyclase (6)
- 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
which toxins activate guanylate cyclase (2)
- ETEC (heat stabile toxin)
- yersinia enerocolitica (y. enterocolitica enterotoxin)
increase cGMP, watery diarrhea, electrolyte loss
100
which toxins inactivate EF-2 (2)
- corynebacterium diptheriae (diptheria toxin)
- pseudomonas aeruginosa (exotoxin A)
ADP ribosylation
101
which toxins disrupt cytoskeleton?
-c difficile
102
polyarteritis nodosa associated with..
hepatitis B
103
giant cell (temporal) arteritis associated w.
polymyalgia rheumatica
104
what is the most common vasculitis from antbiotic use?
microscopic polyangiitis / leukoclastic angiitis / hypersensitvity angiitis
type III immune rxn, often post penicillin
105
which vitamin enhances Fe2+ absorption
vitamin C / ascorbic acid!
bc keeps it in reduced form!
106
suppositories and first-pass metabolism?
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
major determinant of bioavailability after oral intake?
liver blood flow (first pass metabolism)
108
what constitutes first pass metabolism
reduction, oxidation, and hydrolysis.
in large part: cytochrome P450 system
109
what constitutes second pass metabolism
glucoronidation, acetylation, and sulfation
110
most common benign tumor in lung?
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
well defined coin lesion w/ popcorn calcifications on x-rsy?
hamartoma aka pulmonary chondroma
112
bronchioloalveolar carcinoma on x-ray
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
myelofibrosis is a type of
chronic myeloproliferative disease
114
describe basis of myelofibrosis
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
myelofibrosis vs. aplastic anemia
myelofibrosis = massive splenomegaly, bc of extramedullary hematopoesis compensation
116
rx for myeleofibrosis
ruxolitinib, jak 2 inhibtor
117
myelodysplastic syndrome
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
myelopthisic anemia
anemia caused by space-occupying lesions in bone marrow. all hematopoetic series affected, pancytopenia expected.
most common infiltrator: metastatic carcinomas w/ associated fibrosis
119
aplastic anemia (3 characterestics)? bone marrow? histopatholgoy?
1. pancyotpenia
2. low retic count
3. absent splenomegaly
bone marrow aspirate: dry
histopath:marrow replacement w/ fat cells and fibrous stroma
120
fanconi defective DNA repair causes..
aplastic anemia
121
cryoprecipitate can be used to treat..
coagulation deficiency of fibrinogen and factor VIII (hemophilia 8)
has: fibrinogen, factor VIII, factor XIII, vWF and fibronectin
122
risks of blood transfusions (5)
- 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
pseudo-pelger-huet anomaly
with myelodysplastic syndrome:
neutrophils w/ bilobed nuclei (two nuclear masses) connected w/ a thin filament of chromatin.
typically seen after chemotherapy
124
hairy cell leukemia can often present with? rx?
pancytopenia and splenomegaly
rx: cladribine (adenosine analogue resistant to adenosine deaminase)
