STEP1 deck 3 Flashcards
gas gangrene bacterium, toxin
clostridium perfringens
lecithinase (alpha toxin/phospholipase C) => phospholipid splitting => cell lysis, tissue necrosis, and edema
newborn Turner syndrome findings
post. neck mass (cystic hygroma)
extremity edema
diminished femoral pulses (aortic coarctation)
SLE-specific Abs
anti-dsDNA Ab in 60%
anti-snRNP (anti-Smith) Ab in 20%
antimitochondrial Abs
primary biliary cirrhosis
rheumatoid factor
anti-IgG Fc IgM - RA an other collagen tissue disorders
anti-centromere Abs
CRESt syndrome
most common cause of community-acquired pneumonia
strep pneumoniae
medullary thyroid cancer association, origin, and histo
MEN2
parafollicular calcitonin-secreting C cells
sheets of polygonal cells with extracellular amyloid deposits (congo-red +)
chronic AV fistula
SNS + renal compensation for initially decreased TPR => increase contractility, vascular tone, and blood volume
free v. ER-attached ribosome protein product destinations
free - proteins bound for cytosol, nucleus, peroxisome, and mitochondria
attached - proteins bound for PM + nuclear membrane, ER, golgi, and lysosomes
causes of polyhydramnios
increased fetal urination (parvovirus, hemorrhage)
impaired fetal swallowing (obstruction, anencephaly)
anorexia nervosa tx
CBT, nutrition
Olanzapine (antipsychotic that => weight gain)
bulimia nervosa tx
CBT, nutrition
Fluoxetine (SSRI)
binge-eating disorder tx
CBT, weight loss therapy, SSRI, lisdexamfetamine, topiramate
imperforate hymen
at birth => mucocolpos => bulging itroitus
at menarch => primary amenorrhea + hematocolpos => pelvic pain
mechanism of hepatic encephalopathy
hyperammonemia => increased glutamate uptake and glutamine production within astrocytes => increased osmolarity and impaired glutamine release => decreased glutamate available to neurons and decreased excitatory neurotransmission
structures near parts of the duodenum
duodenum hugs pancreas
1st part: R gastroepiploic; intraperitoneal
(2-4 retroperitoneal)
2nd part: head of pancreas
3rd part: between abdominal aorta and SMA
4th part: becomes jejunum at ligament of Treitz
phenoxybenzamine
irreversible a1 and a2 adrenergic antagonist
used to block NE effects from pheochromocytoma
phentolamine
reversible, competitive a-antagonist
used to block NE effects from pheochromocytoma
Rett syndrome
X-linked dominant (mostly girls) MECP2 mut
=> normal development for 5-18mos, then regression of motor and language skills, slowed head growth, and hand twisting
mechanism for development of esophageal varices
cirrhosis => increased pressure in portal vein => L + R gastric veins, => esophageal veins
pulsus paradoxus
drop > 10 mmHg in BP during inspiration
tamponade, constrictive pericarditis
Initial cells infected by Shigella
M (microfold) cells at base of Peyer patch
cutaneous neurofibroma tissue type, origin, and association
benign nerve sheath neoplasm made of schwann cells
derived from neural crest
associated with NFT I (von Recklinghausen)
characteristics of drugs with low volume of distribution
(trapped in plasma compartment)
high molecular weight, binding to plasma proteins, high charge, hydrophilicity
components of LPS
O antigen core polysaccharide lipid A (responsible for toxic properties => sepsis
rate limiting step in acyclovir action
viruses it works on and doesn’t
monophosphorylation by viral thymidine kinase
more effective against HSV and VZV than EBV and CMV b/c make different thymidine kinases.
ADH effect on urea and secondary benefit
(vasopressin)
activates urea transporters in the medullary collecting duct, which transport urea down gradient (now that more H2O reabsorbed) => decreased urea excretion
secondary benefit is more urea in interstitium allows more H2O absorption in loop of Henle
aortic regurg murmur and location
early diastolic murmur best heard at left sternal border
medications that => immediate rash and mechanism
opioids, contrast, and vancomycin
IgE- independent mast cell degranulation by activation of PKA and PI3 kinase
hyperacute transplant rejection timing, mech, gross appearance, and histo
min-hrs
preformed anti-graft Abs
mottling and cyanosis
arterial fibrinoid necrosis and capillary thrombotic occlusion
acute transplant rejection timing, mech, and histo
<6 mos
donor ag => activation of naive immune cells
C4d, neutrophilic infiltrate, and necrotizing vasculitis, or
lymphocytic interstitial infiltrate and endotheliitis
chronic transplant rejection timing, mech, and histo
mos-yrs
chronic, low-grade response
vasculat wall thickening, luminal narrowing, interstitial fibrosis and parenchymal atrophy
cancers associated with Down syndrome
ALL, AML
elevated alk phos follow-up test
GGTP to confirm from liver, not bone
chronic kidney disease effect on PTH and Ca
decreased GFR => decreased phosphate clearance => increased Ca binding => hypocalcemia
decreased vitamin D activation in kidneys also => hypocacemia
hypocalcemia => secondary hyperparathyroidism
lidocaine mech and use
class IB antiarrhythmic that binds inactivated Na channels and rapidly dissociates
preferentially works in depolarized, ischemic, tissue; no QRS prolongation
used for ventricular arrythmias post-MI
fibrate function and side effect
(fenofibrate, gemfibrozil)
upregulate LPL
inhibit cholesterol 7a-hydroxylase => decreased bile acid synth => cholesterol gallstones
mitral valve opening on pressure tracing
when LV pressure falls below LA pressure
sulfonylureas and meglitinides MOA and SEs
(glypizide, glyburide, blimepiride) increase insulin secretion by blocking pancreatic B-cell potassium channels
hypoglycemia (less with glypizide b/c short-acting), weight gain
metformin MOA and SEs
stimulates AMPK, decreasing glucose production and insulin resistance
lactic acidosis
thiazolidinediones, MOA, and SEs
pioglitazone and rosiglitazone
activate PPAR-y (TF) => decreased insulin resistance
fluid retention, weight gain
exenatide, liraglutide MOA and SEs
GLP-1 agonists
increased glucose-dependent insulin secretion, decrease glucagon secretion, delay gastric emptying
pancreatitis
sitagliptin and saxagliptin MOA and SEs
inhibit DPP4 => increased endogenous GLP-1 and GIP levels
nasopharyngitis
acarbose and miglitol MOA and SEs
a-glucosidase inhibitors => reduced intestinal disaccharide absorption
diarrhea, flatulence
canagliflozin and dapagliflozin MOA and SEs
inhibit SGLT2 => increased renal glucose exretion
UTIs, hypotension
1st and 2nd most common causes of meningitis in adults
strep pneumoniae, then neisseria meningitidis
PSGN histo, EM, and IF
enlarged, hypercellular glomeruli (leukocyte infiltration and cell proliferation)
epithelial humps
starry sky IgG and C3 deposits
things below the left sternal border at the 2nd and 4th intercostal spaces
2nd: pulmonary trunk
4th: right ventricle
mech of zenker diverticulum formation
cricopharyngeal motor dysfunction => dysphagia => increased pressure => herniation => diverticulum (false)
why is pus green?
neutrophil myeloperoxidase (catalyzes HClO production during respiratory burst)
pO2 at altitude
can be in the 50s
porcelain gallbladder cancer association
gallbladder adenocarcinoma
strawberry v. cherry hemangioma
strawberry is the most common benign vascular tumor in children (bright red, sharply demarcated borders, regress by age 5)
cherry is the most common benign vascular proliferation in adults (small bright red cutaneous papules; don’t regress)
nerve at risk curing thyroidectomy and muscle it innervates
external branch of the superior laryngeal n.
cricothyroid muscle
thyroid hormone receptor type
intracellular zinc finger TF
arteries commonly affected by spontaneous deep intracerebral hemorrhage and cause
penetrating branches (eg lenticulostriate arteries)
from hypertensive vasculopathy
renal vein thrombosis pathophys and presentation
nephrotic syndrome => excretion of antithrombin II => renal vein thrombosis (flank pain, hematuria, new left-sided varicocele, and elevated LDH)
most common urea cycle disorder, labs, and mech
ornithine transcarbamylase deficiency
=> carbamoyl phosphate buildup => increased pyrimidine synth => orotic aciduria
loss of urea cycle function also => hyperammonemia
wegener’s granulomatosis Abs and target
C-ANCA
neutrophil proteinase 3
pip-tazo mechanism
piperacillin - beta lactam
tazobactam - beta lactamase inhibitor
beta lactamase inhibitors
tazobactam, clavulanate, sulbactam
tetralogy of fallot development
abnl neural crest cell migration => deviation of the infundibular septum (between aorta and pulmonary artery) => VSD and overriding aorta
glycerol kinase
liver enzyme that catalyzes conversion of glycerol from TGs to Glycerol 3-phosphate, which can then => glucose
short acting benzos
<6 hrs triazolam, oxazepam, midazolam
intermediate acting benzos
6-24 hrs
ATOM
alprazolam, temazepam, oxazepam, lorazepam
long acting benzos
> 24 hrs
diazepam, chlordiazepoxide, flurazepam
age 3 milestones
play imaginatively in parallel, speak in 3 word sentences, copy a circle, use utensils, and ride a tricycle
anti-pseudomonal drugs
cefepime, ceftazidime, pip-tazo, ciprofloxacin, levofloxacin, aminoglycosides, carbapenems
effects of hyperventilation in cerebral edema
drop in PaCO2 => vasoconstiction => decreased cerebral blood volume => decreased ICP
cystic medial degeneration
myxomatous degeneration with pooling of proteoglycans (ECM) and basket weave pattern in the media of large arteries => aortic aneurysm
brown kidney stones
bilirubin stones from biliary tract infection
black kidney stones
bilirubin stones from hemolysis
irreversible complication of ASD
pulmonary vascular sclerosis from pulmonary HTN => Eisenmenger
mech of radiation for Ca
dsDNA breaks + free radical formation => DNA damage
side effects of inhaled anesthetics
decreased CO, increased internal pressures
respiratory depression
decreased CNS vascular resistance and increased cerebral blood flow => increased ICP
decreased RPF and GFR
ventricular response in AFib
depends on AV node refractory period => rate of 90-170
purkinje pacemaker rate
<40 bpm
matrix metalloproteinases and overactivation
degrade collagen other ECM proteins -> wound contraction
increased MMP activity => contracture
arachnoid granulations, route of damage, and consequences
(villi) - allow CSF to enter the sinuses => bloodstream
damaged 2/2 meningeal infection (eg TB meningitis) or subarachnoid hemorrhage
=> communicating hydrocephalus
mech for neuro sx of B12 deficiency
B12 is cofactor for methylmalonyl-CoA mutase and methionine synthase, so def => elevated methylmalonic acid and homocysteine => dysruption on myelin synthesis => subacute combined degeneration
primary location of nitro action
large veins
PAH
paraaminohippuric acid
freely filtered and secreted by carriers in the PCT
peau d’orange
itchy, erythematous rash with firm, coarse skin and brease edema
inflammatory breast cancer => lymphatic obstruction
poor prognosis
poor prognostic factors in PSGN
increased age, pre-existing kidney disease
electrolyte abnormality in babies with CF
hyponatremia from excessive salt wasting in sweat
GI sx of CREST sy
Calcinosis, Raynaud, Esophageal dysmotility, Sclerodactyly, Telangiectasia
Esophageal dysmotility is from atrophy and fibrous replacement of th emuscularis in the lower esophagus => GERD
targets of deep brain stimulation in Parkinson
globus pallidus internus, subthalamic nucleus (indirect pathway)
electrolyte abnormality associated with ganulomas
(sarcoid, TB)
macrophages make activated vitD => hypercalcemia
also in some lymphomas
diptheria treatment
in order of importance:
diptheria antitoxin (passive immunization)
penicillin or erythromycin
DPT vaccine
gallstone ileus formation and sx
large gallstone => fistula between gallbladder and duodenum, then gets lodged in the terminal ileum => small bowel obstruction, pneumobilia (gas in biliary tree and gallbladder)
papilledema mech
increased ICP compresses optic nerves => impaired axoplasmic flow => optic disc edema
isoproterenol
(iso-pro-tunnel)
B1 + B2 agonist
=> increased CO (HR and contractility) and decreased SVR (vasodilation)
Li-Fraumeni sy
AD p53 mut
=> leukemia, sarcomas, breast, brain, and adrenal cancer
idiopathic /familial pulmonary arterial hypertension
EMPR2 mut => endothelial smooth muscle proliferation => luminal narrowing, intimal fibrosis
tx: lung transplant, vasodilators, bosentan
bosentan
endothelin-receptor antagonist
used in idiopathic pulmonary arterial hypertension
lung transplant chronic rejection
mos-yrs
major cause of mortality
inflammation of small bronchioles (bronchiolitis obliterans)
diff from vascular rejection in kindey/abdominal transplants
blotchy red muscle fibers on Gomori trichrome stain
(ragged red fibers)
mitochondrial myopathy
maternally inherited
3 common mitochondrial myopathies
myoclonic epilepsy with ragged red fibers (MERRF)
leber optic neuropathy (blindness)
mitochondrial encephalopathy with stroke-like episodes and lactic acidosis (MELAS)
mycobacterium avium complex sx, distinguishing features, tx, and prophylaxis
CD4 <50
more anemia, hepatosplenomegaly, elevated alk phos and LDH than in TB
grows best at 41 C
tx with macrolide + rafabutin or ethambutol
prophylax with azithromycin
reticulocyte appearce
large, blue on Wright-Giemsa stain b/c of ribosomal RNA
auer rods
stain positively for myeloperoxidase
found in APL
campylobacter transmission
fecal-oral
can be spread by domestic animals and contaminated food
optimal site for femoral vascular access
common femoral artery below the inguinal ligament
if above, increase risk of retroperitoneal hemorrhage which can’t be controlled via compression
common 5a-reductase deficiency presentation
small phallus, hypospadias
left frontal lobe lesion sx
apathy, depression
right frontal lobe lesion sx
disinhibition
non-dominant parietal lobe lesion (right) sx
hemi-neglect, apraxia
dominant parietal lobe lesion (left) sx
Gerstmann syndrome (right/left confusion, difficulty with writing and math)
metyrapone stimulation test
blocks 11-B-hydroxylase => reduced cortisol => increased pituitary ACTH secretion => increased 17-hydroxycortisone production
carrier regenerated by anaerobic lactate formation
NAD+, needed for glycolysis
congenital hypothyroidism
initially normal, then maternal T4 wanes
lethargy, enlarged fontanelle, protruding tongue, umbilical hernia, poor feeding, constipation, dry skin, and jaundice
colchicine indications and SEs
used for gout in people who can’t take NSAIDS (ulcers)
=> diarrhea, nausea, vomiting, and AP
Wilson’s disease
AR Cu retention
=> liver accumulation, eventually liver damage => cu escape => Kayser-Fleischer rings, basal ganglia atrophy
krukenberg tumor and histo
primary gastric cancer that metastasizes to ovaries
signet ring cells from mucin displacing the nucleus
snRNP function
spliceosome
spinal muscular atropy
mutation in SMN1 gene => imapired assembly of snRNPs in lower motor neurons => loss of splicing => flaccid paralysis, degeneration of anterior horn cells in spinal cord
location of most deoxygenated blood
coronary sinus because myocardial oxygen extraction is very high
most common cause of extramedullary hematopoiesis
severe chronic hemolytic anemia (B-thalassemia)
imaging to diagnose toxic megacolon and causes of same
abdominal XR
from ulcerative colitis and c. diff.
rotator cuff muscles and functions
supraspinatus - abduction
infraspinatus - external rotation
teres minor - adduction, external rotation
subscapularis - adduction, internal rotation
causes of hyaline arteriolosclerosis
chronic poorly controlled HTN
diabetes mellitus
developmental source of female internal genitalia
paramesonephric ducts (mullerian)
sotalol
class III antiarrhythmic (K channel blocker) used to treat AFib
prolongs QRS => long AT and torsades