test #35 4.26 Flashcards
calcineurin inhibitors
cyclosporin and tacrolimus
germline mutations in RET proto-onco gene affect cells of what origin
neural crest cells!
men2a: medullary thyroid carcinoma (parafollicular C cells of thyroid), pheo, and parathyroid gland
men2b: medullary thyroid carcinoma, pheo, and mucosal neuromas
thyroid parafollicular cells arise from..
4th and 5th pharyngeal pouches
name development of pharyngeal pouches (5)
- ears (middle ear, mastoid air cells, eustachian tube = endoderm lined)
- tonsils
- inferior parathyroid, thymus
- superior parathyroid, medullary thyroid parafollicular cell
- medullary thyroid parafollicular cells
development of thyroid
thyroid follicular cells = outpouching of pharyngeal epithelium, migrate to lower neck & fuse with parafollicular C cells from 4/5th pharyngeal pouch.
pathophysiology of mutation in VHL
VHL gene regulates transcription of HIF-1 (hypoxia inducible factor).
unregulated HIF –> increased VEGF & EPO
= hemangiomas
also associated w/ pheos & RCC
projection
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
identification
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
acting out
avoiding unacceptable feelings by behaving badly
expressing unwanted thoughts/impulses through action instead of reflection and mature discourse
most severe trisomy
trisomy 13 – patau
usu die in first week
only 5% survive 6 months
describe trisomy 13-patau
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
47 XXY
klinefelter; tall male w/ gynecomastia, small testes, and infertility
47 XXX
clinically silent, maybe slightly decreased IQ
47 XYY
tall stature, acne, delays in motor and language
k-ras mutations common in..
pancreatic malignancy
when is opening snap of mitral stenosis head
really really close to closure of S2
due to tensing of MV leaflet after cusps completed their opening excursion
relationship between time of A2-opening snap in mitral stenosis and severity
inverse. shorter interval, more severe.
bc higher steady state left atrial pressure.
control symptoms of a neonate in opiod withdrawal (leaky baby)
tincture of opium or paregoric
what maintains blood brain barrier
tight junctions [claudins & occludins]
between endothelial cells in capillary beds of CNS.
materials can only move transcellularly or carrier mediated.
why use naficillin usu
penicillinase-resistant penicillin.
use often against s. aureus.
skin infections and soft tissue infection – folliculitis, abscesses.
rx for lung abscesses
usu clindamycin
‘above the belt’ anaerobes’
petechia, purpura, ecchymoses? blanch?
petechia 1cm
do not blanch, bc RBC are not in vasculature. RBC leaked in skin/subcutaneous tissue
PPE
which blanch. telangietcasia or petechiae/purpura/ecchymoses?
teleangiectasia, bc in a vessel
intranasal glucocortoids localization
unlikely to cause systemic immunosuppression – very localized
causes of candida vaginitis
OFTEN:
1. ANTIBIOTIC use! suppress normal flora, facilitates candida overgrowth.
- contraception, high estrogen
- systemic corticosteroids
- uncontrolled diabetes
- immunosuppression
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
major bacterial flora of vagina
gram + lactobacilli
also: corynebacterium, fungi (candida), group B strep, e. coli
usu pH ~4
decrease thickening of vaginal epithelium and decreased glycogen associated with..
postmenopausal & lactating women
elevation of vaginal secretion pH suggests (pH > 4.5)
gardnerella or trichomonas
how are many carcinogens activated in humans
via microsomal monooxygenase (cytochrome p450 monooxygenease)
often convert procarcinogen -> carcinogen
like benzopyrene-induced lung cancer
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
where are parietal cells located in gastric mucosa?
superficial gastric glands
where are chief cells located in gastric mucosa?
deep gastric glands
5 layers of gastric mucosa
- simple columnar epithlieum
- secrete mucus to protect - superficial lamina propria
- contains gastric pits
(mult glands connect to single pit) - superficial gastric gland
- has parietal cells (oxyntic, pale, round, plate-like - deep gastric gland
- chief cells (pepsinogen) granular basophillic - muscularis mucosa
- separates lamina propria from submucosa
submucosa – vascularized CT
fusobacterium, peptostreptococcus, bacteroides in lung..
aspiration pneumonia
alcoholic, seizure, severe neuro disease
3 ways to get lung abscess
- aspiration of oropharyngeal contents: fusobacterium, peptostreptococcus, bacteroides
- complication of bacterial pneumonia: local immunosuppression, old oage, underlying chronic disease, necrotizing: s. aureus, e.coli, k. pneumonia, or s. pneumo type 3
- specticemia or infective endocarditis: hematogenous spread of infection – usu multiple. most common: staph or strep, e. coli, fungi.
where does blood accumulate in a subdural hematoma?
between dura mater and arachnoid. usu venous
gradual onset of symptoms.
crescent shaped
cortical briding veins
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)
HgbA1c binding 2,3 DPG?
does not affect binding to 2,3 DPG
chlorpromazine associated w/
corneal deposits
haloperidol associated w/
extrapyramidal symptoms
ziprasidone associated w/
long QT
olanzepine associated w/
weight gain
clozapine associated w/
agranulocytosis and seizures
thioridazine associated with
retinal deposits that resemble retinitis pigmentosa
leading cause of blindness in industrial nations
macular degeneration
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
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)
rx for dry age related macular degeneration?
antioxidant vitamins and zinc
rx for wet age related macular degeneration
anti-VEGF, laser or phototherapy
(limit development of choroidal neovascular membranes)
intra-vitreous ranibizumab and pegaptanib
epidermal growth factor receptor inhibitors (erlotinib & gefitinib) rx for?
non small cell lung cancer
anti-IL2 for..
use for immunosupression for GVHD and organ transplant
anti-TNF-alpha for..
inflammatory autoimmune conditions: RA, IBD, seronegative spondyloarthropathies
infertility, sinusitis, bronchiectasis
kartageners (mutation in dyenin arm)
hypocalcemic tetany & recurrent viral and fungal infxn
digeorge 22q11.2 deletion
3 cardiac issues associated w/ marfans
all related to cystic medial necrosis
- dissection aortic aneurysm
- aortic valve incompetence
- mitral valve prolapse
autosomal dominant, fibrillin
2 possible cardiac issues in digeorge
tetrology of fallot & aortic arch abnormalities
cardiac abnormality in friedrich’s ataxia
hypertrophic cardiomyopathy
autosomal recessive:
mutation in friedrich’s ataxia
autosomal recessive.
mutation in frataxin, mitochondrial protein impt in iron homeostasis & respiratory fxn
VIPoma
WDHA. “pancreatic cholera”
watery diarrhea, hypokalemia, achlorhydria
rx: somatostatin
VIP made by..
pancreatic islet cells, neurons of GI mucosa
relaxes smooth muscle, inhibits H+ secretion, stimulates pancreatic bicarb and Cl- secretion
who makes gastrin
G cells in stomach mucosa.
located in antrum.
glucagonoma may present w/
(1) secondary diabetes mellitus
(2) necrolytic migratory erythema of skin
optic tract fibers project to.. (4)
mainly 1. LGN
- superior colliculus (reflex haze)
- pretectal area (light reflex)
- suprachiasmatic nucleus (circadian rhythm)
meyer’s loop connects
LGN, through temporal lobe, LINGUAL gyrus of striate (info form lower retina, upper world)
UPPER fibers of optic radiation carry
LGN, through parietal to CUNEUS gyrus of striate (info from upper retina, lower world)
what can stimulate glycogen utilization in muscles?
- sustained Ca2+ ITSELF during contraction –> direct activation of phosphorylase kinase (activates glycogen phosphorylase)
- B1/glucagon-dependent increase in cAMP -> PKA ->
- phosphorylates ‘phosphorylase kinase’ [activates it to phosphorylate glycogen phosphorylase]
- & phosphorylates glycogen synthase to inactivate it.
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
osler-weber-rendu
hereditary hemorrhagic telangiectasia
autosomal dominant
telengiectasia on skin & mucus membranes w. rupture –> epistaxis, GI bleed, hematuria?
hereditary hemorrhagic telangiectasia
osler-weber-rendu
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)
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
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.
PSA is a marker for..
extent of prostate cancer & evaluating response to treatment
commonly used to screen, but controversial
acid phosphatase marker for..
secondary prostate tumor marker antigen.
also elevated in patients w/ active osteoclast-indiated bone resorption
hCG is a marker for
gestational trophoblastic disease
sudden cardiac death defined by..
cardiac arrest that begins 1hr of a precipitating event that ultimately proves fatal.
most common: ventricular fibrillation
most common cause of death in patients suffering from MI during prehospital phase (prior to arriving in ED)
ventricular fibrillation
when do ventricular mural thrombosis occur post MI
after at least 48 hrs in
when do ventricular rupture most commonly occur post MI?
3-7 days in
most common tumor in HIV+ patients
kaposi sarcoma
HHV8 infects vascular & lymphatic endothelial cells
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
histology of kaposi sarcoma
spindle and endothelial cell proliferation, red blood cell extravasation, inflammation
CMV in HIV+ patient causes (3)
- esophagitis
- colitis
- retinitis
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
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
half life of factor VIII w/ vWF? without?
w/ vWF: 12 hours
w/o vWF: 2 hours
desmopressin in heme
releases vWF from endothelial cells
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)
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
atypical antipsychotic most likely to cause extrapyramidal symptoms? least?
most likely: risperidone
(also galactorhea and amenorrhea)
least: clozapine
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
parkinsonism (drug-induced)? rx?
between 4 days-4months of treatment.
cogwheel rigidity, masked facies, bradykinesis, pill-rolling, tremor, shuffling gait
rx: benztropine anticholinergic
akathisia
subjective feeling of restlessness that compels patient to constantly move around.
pH urease test for h. pylori
urea –> NH3 + CO2, pH increase, color change in phenol red.
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
many e. coli (except EHEC) are able to..
(1) ferment sorbitol
(2) produce glucoronidase
EXCEPT EHEC
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
which toxins activate guanylate cyclase (2)
- ETEC (heat stabile toxin)
- yersinia enerocolitica (y. enterocolitica enterotoxin)
increase cGMP, watery diarrhea, electrolyte loss
which toxins inactivate EF-2 (2)
- corynebacterium diptheriae (diptheria toxin)
- pseudomonas aeruginosa (exotoxin A)
ADP ribosylation
which toxins disrupt cytoskeleton?
-c difficile
polyarteritis nodosa associated with..
hepatitis B
giant cell (temporal) arteritis associated w.
polymyalgia rheumatica
what is the most common vasculitis from antbiotic use?
microscopic polyangiitis / leukoclastic angiitis / hypersensitvity angiitis
type III immune rxn, often post penicillin
which vitamin enhances Fe2+ absorption
vitamin C / ascorbic acid!
bc keeps it in reduced form!
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
major determinant of bioavailability after oral intake?
liver blood flow (first pass metabolism)
what constitutes first pass metabolism
reduction, oxidation, and hydrolysis.
in large part: cytochrome P450 system
what constitutes second pass metabolism
glucoronidation, acetylation, and sulfation
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
well defined coin lesion w/ popcorn calcifications on x-rsy?
hamartoma aka pulmonary chondroma
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
myelofibrosis is a type of
chronic myeloproliferative disease
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
myelofibrosis vs. aplastic anemia
myelofibrosis = massive splenomegaly, bc of extramedullary hematopoesis compensation
rx for myeleofibrosis
ruxolitinib, jak 2 inhibtor
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
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
aplastic anemia (3 characterestics)? bone marrow? histopatholgoy?
- pancyotpenia
- low retic count
- absent splenomegaly
bone marrow aspirate: dry
histopath:marrow replacement w/ fat cells and fibrous stroma
fanconi defective DNA repair causes..
aplastic anemia
cryoprecipitate can be used to treat..
coagulation deficiency of fibrinogen and factor VIII (hemophilia 8)
has: fibrinogen, factor VIII, factor XIII, vWF and fibronectin
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
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
hairy cell leukemia can often present with? rx?
pancytopenia and splenomegaly
rx: cladribine (adenosine analogue resistant to adenosine deaminase)
