USMLE WORLD Flashcards

(372 cards)

1
Q

alkaptonuria is due to genetic deficiency of what?

A

homogentisate oxidase (you can’t take tyrosine and eventually make fumurate; has black urine if urine undergoes oxidation; causes ochronosis ( a blue-black pigmentation that is seen in ears, nose, & cheeks

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2
Q

what kind of BVs does nitroglycerin work on?

A

venodilator of mainly large veins

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3
Q

what is the name of the protein that is required for neutrophils to undergo diapedesis?

A

PECAM

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4
Q

what is the MC location for brain germinoma?

A

pineal region

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5
Q

what are the clinical features of brain germinoma?

A

tumor in pineal region, can cause preccocious puberty (may be seen in males due to increased beta-HCG prod.)
-aqueductal compression causing hydrocephalus, & parinaud syndrome (which is characterized by upward gaze and convergence, due to compression of the tectal area of the midbrain)

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6
Q

6-MP is metabolized by what enzyme?

A

xanthine oxidase (it is also activated by HGPRT)

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7
Q

what is the only GLUT transporter that is responsive to insulin?

A

GLUT4 (found in tissues like adipocytes and skeletal muscles)

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8
Q

what kind of cardiac abnormality is seen in turner’s syndrome?

A

coarctation of the aorta

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9
Q

which parts of the nephron are most susceptible to ischemic injury?

A

the proximal tubules and the thick ascending limb

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10
Q

what is contraction alkalosis?

A

can happen with thiazide & loop diuretics, both classes increase renal loss of Na, followed by loss of Cl-, there is reabsorption of bicarb to maintain electric neutrality in cells, the volume contraction stimulates aldosterone secretion (acts to resorb Na+ and water from distal tubule while excreting K+ & H+); overall Cl- concentration in body is low due to renal losses; giving saline improves acid-base status

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11
Q

the amount of coronary blood flow is determined by what?

A

duration of diastole

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12
Q

what drug do you use to treat essential tremor?

A

propanolol

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13
Q

which bacteria causes ecthyma gangrenosum?

A

pseudomonas aeruginosa: cutaneous necrotic dz w/ bacteremia & septicemia; pseudomonas invades perivascularly & releases tissue destructive exotoxins causing vascular destruction & decreased blood flow to skin which becomes edematous and necrotic

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14
Q

what are the 3 P’s of MEN 1 syndrome?

A
Pituitary tumors (prolactinoma, or GH secreting)
Pancreatic tumors (gastrinoma)
Parathyroid tumors
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15
Q

how do you calculate the number needed to harm?

A

NNH = 1 / attributable risk

attributable risk = event rate in treatment - event rate in placebo

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16
Q

in what part of the nephron would you see uric acid precipitate, like in the case of tumor lysis syndrome?

A

most likely to precipitate in collecting ducts b/c of the low pH

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17
Q

which types of molecules/cytokines stimulates neutrophil chemotaxis/

A
IL-8
C5a
LTB4
n-formylated peptides
5-HETE
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18
Q

what class represents the drugs of choice for heparin induced thrombocytopenia?

A

direct thrombin inhibitors (argatroban)

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19
Q

describe the innervation to the ear?

A

post. canal of the ear = CN X (small auricular branch)

Remainder of EAC & External part of TM = CN V3 (auriculotemporal branch)

Inner part of TM = CN IX (tympanic branch)

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20
Q

what is the mechanism of amatoxins (mushrooms)

A

potent inhibitors of RNA polymerase II, halting mRNA synthesis

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21
Q

which drug would you use on a lady with PCOS who wants to get pregnant?

A

CLOMIPHENE, an estrogen receptor modulator that decreases the negative feedback on the hypothalamus by the high amount of circulating estrogen

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22
Q

what is the enzyme deficiency in Tay-Sach’s dz?

A

AR deficiency of beta-hexosaminidase A, leads to built up of GM2 ganglioside

Features: progressive neurodegeneration, cherry red macula, weird startle response & no hepatosplenomegaly

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23
Q

bacillus anthracis has what unique component of its antiphagocytic capsule?

A

D-glutamate instead of polysaccharide

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24
Q

fenoldopam MOA

A

selective dopamine-1 receptor agonist

causes arteriolar dilation & natriuresis–> decreased PVR;

only drug that improves renal wolunction and decreases BP (can be used in pts w/ HTN & renal insufficiency)

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25
what is the drug of choice for mucormycosis?
amphotericin B
26
name the type of cell: pyknotic superficial or immature squamous cells w/ dense irregulary staining cytoplasm and PERINUCLEAR CLEARING
koilocyte (HPV)
27
name the drug: opioid narcotic designed to produce analgesia w/ no abuse; it has partial agonist & weak antagonist activity at mu receptors; so it can produce withdrawal in opioid addicts
pentazosine
28
what is the mechanism of disease in vitiligo?
caused by loss of EPIDERMAL MELANOCYTES
29
name the term: changes seen in body of neuron after axon has been cut, there are enlarged rounded cells with peripherally located nuclei and dispersed granular Nissle substance (this reflects increased protein synthesis that is supposed to help in repair)
axonal reaction
30
what is the MCC of retinitis in HIV pts?
CMV (treat with ganciclovir)
31
what is trousseau's syndrome?
migratory superficial thrombophlebitis; can be seen as paraneoplastic syndrome in visceral cancers
32
what is the most common site of injury in aortic rupture?
aortic isthmus (area b/w the ascending and descending aorta after the place where L. subclavian artery branches off the aorta) note: MC due to motor vehicle accidents
33
what is the enzyme deficiency in Lesch-Nyhan syndrome?
x linked disorder of HGPRT deficiency results in failure of purine salvage pathway leads to increased amounts of purine bases like hypoxanthine & guanine that get degraded to uric acid end results is you lose a lot of purine bases so now you have to increase de novo purine synth. to replace the lost purine bases (PRPP increases)
34
Drugs of choice for treating GC & chlaydia together
GC - Ceftriaxone | Chlamydia - Azithromycin
35
niacin moa
used to treat dyslipidemia; lowers levels of VLDL & raises levels of HDL
36
what is the atopic triad?
atopic dermatitis (eczema) allergic rhinitis asthma
37
what is the chi square test used for?
used to test the assoc. b/w 2 categorical variables
38
what is the mechanism of the genetic defect in fragile x syndrome?
there is increased number of CGG repeats that leads to hypermethylation of cytosine bases and subsequent gene inactivation
39
what are two drugs used to treat CMV retinitis?
Ganciclovir (DOC) | Foscarnet
40
how do you treat postop urinary retention?
the problem is decreased micturition reflex and decreased contractility of the bladder (holding on to too much urine) so the treatment is bethanechol or an alpha-1 blocking drug
41
which vitamin can be used to treat measles?
vitamin A
42
which 2 beta blockers are nonselective blockers of both alpha & beta receptors?
carvedilol | labetalol
43
describe the murmur of hypertrophic cardiomyopathy
systolic ejection murmur decreases with maneuvers that increase the afterload and preload -this is b/c increasing these parameters decreases the L. ventricular outflow tract obstruction by increasing L. ventricular volume
44
describe the clinical presentation of congenital hypothyroidism
hypotonia, poor feeding, jaundice, macroglossia, constipation, and UMBILICAL HERNIA
45
describe the MOA of atrial natriuretic peptide
causes peripheral vasodilation and increased urinary excretion of sodium & water
46
MOA and clinical use of buspirone
moa = stimulates 5-HT1A receptors clinical use = Gen. anxiety disorder, doesn't cause sedation, addiction or tolerance, so good for use in pts prone to abusing BNZs
47
what is Kussmaul sign?
seen with paradoxical rise in JVP during inspiration, seen in constrictive pericarditis
48
which renal disease is associated with IgG4 antibodies to the phospholipase A2 receptor?
membranous nephropathy
49
what is cheyne-stokes respiration?
cyclic breathing where there is apnea, then gradually increasing tidal volume, then decreasing tidal volumes until the next apneic period; seen in heart failure
50
what is Kussmaul breathing?
deep and labored breathing pattern seen in DKA
51
how do you treat TCA cardiotoxicity?
treat with sodium bicarbonate (b/c TCAs can block fast sodium channels and cause QRS prolongation)
52
what is the defect in essential fructosuria?
defective fructokinase (AR), can't trap fructose in cells, generally asymptomatic w/ finding of fructose in blood and urine
53
what is the t-test used for?
used to compare the means of 2 groups of subjects
54
which viral vaccines are live attenuated?
``` Smallpox Yellow Fever Chickenpox (VZV) Sabin Polio virus MMR Influenza (intranasal) ``` "Live! One night only! See Yellow Chickens get vaccinated w/ Sabin & MMR. It's INcredible"
55
which viral vaccines are killed?
Rabies Influenza (injection) Salk Polio HAV
56
which viral vaccines are recombinant?
HBV (antigen = recombinant HBsAg) HPV (6, 11, 16, 18)
57
which atypical antipsychotic can cause hyperprolactinemia and subsequently amennorhea?
risperidone
58
midshaft fracture of the humerus is most likely to damage what 2 structures?
radial nerve deep brachial artery
59
hematogenous osteomyelitis is most common in children and usually affects which part of the long bones?
metaphysis (due to slow blood flow and capillary fenestrae in the area)
60
which two bacteria both make toxins that work by ribosylating & inactivating EF-2, thus inhibiting protein synthesis?
1. Diptheria toxin | 2. Exotoxin A (pseudomonas)
61
what is the mechanisms of the AE of angioedema with ACE inhibitors?
build up of bradykinin (which is a vasodilator and promotes edema)
62
Vitamin A toxicity presentation
papilledema & seizures -due to increased ICP Hepatitis bone pain (periosteal proliferation)
63
what kind of drugs can cause pulmonary htn?
taking appetite suppressants (fenfluramine or phentermine) for about 3 months, presents with dyspnea on exertion and leads to RVH, possibly sudden cardiac death
64
name the disorder: AR, resulting from defective DNA-repair genes, pts are hypersensitive to ionizing radiation w/ features like cerebellar atxia, oculocutaneous telangiectasias, repeated sinopulmonary infections & increased risk of malignancy
ataxia-telangiectasia
65
how can you treat the opthalmopathy in graves dz?
use glucocorticoids to reduce severity of inflammation and decrease the extraocular volume
66
how can you treat urge incontinence?
the problem is too much bladder contraction treat with oxybutynin (M3 receptor) that will prevent overcontraction of the bladder
67
how do we treat listeriosis?
AMPICILLIN
68
MOA of phenoxybenzamine
irreversible alpha-1 & alpha-2 antagonist, reduces the # of receptors available for NE binding, so you can't overcome the action of the drug by adding more NE
69
how does carotid massage cause increased parasympathetic tone on the heart?
baroreceptors sense too much pressure--> INCREASED FIRING via CN IX, the CNS responds by causing vagal nerve to prolong AV nodal refractory period this can be used to stop re-entrant tachycardias
70
what are the 2 most common causes of eugonadotropic amenorrhea?
1. mullerian agenesis | 2. imperforate hymen
71
what is the arteriovenous concentration of an inhaled anesthetic mean?
reflects overall tissue solubility of an anesthetic if there is high tissue solubility then there will be large arteriovenous concentration gradient and a slower onset of action
72
what is the blood supply to the proximal 1/3 of the ureter?
comes from branches of the renal artery, this is the reason that the prox. 1/3 of donor ureter can stay after renal transplant
73
which part of the anal canal is damaged with anal fissures?
in the posterior midline distal to the dentate line (this happens b/c there is poor perfusion to this area, and this makes the area sensitive to trauma by hard fecal masses)
74
which metabolite accumulates in galactokinase deficiency?
galactitol accumulates galactose can't be phosphorylated so it is made into galactitol by aldose reductase
75
what is the hawthorne effect?
the tendency of a study population to affect an outcome due to the knowledge of being studied
76
what is the MCC of primary amenorrhea in a women with fully developed secondary sex traits?
MC = imperforate hymen other is mullerian duct anomaly
77
which drug can you give to people exposed to radioactive iodine isotopes?
give potassium iodide, which can prevent the uptake of the radioactive iodine by competitively inhibiting the transporter
78
what is the enzyme deficiency in maple syrup urine dz?
defect in alpha-ketoacid dehydrogenase can't degrade branched chain amino acids beyond their deaminated alpha-ketoacid state
79
how do you treat a lung abscess?
use clindamycin which has activity against oral anaerobes & also covers aerobic gram-positive bacteria like streptococcus
80
which enzyme is missing in hereditary fructose intolerance?
aldolase B fructose-1-phosphate accumulates leading to decreased availability of PO4, this leads to inhibition of glycogenolysis & gluconeogenesis Symptoms: hypoglycemia, jaundice, cirrhosis vomiting
81
what can precipitate biotin deficiency?
caused by antibiotic use or excessive ingestion of raw egg whites Features: dermatitis, alopecia, enteritis note: biotin is a cofactor for several carboxylase enzymes
82
naloxone has greatest affinity for which opioid receptors?
Mu - which when activated would normally cause euphoria, respiratory & cardiac depression, and decreased GI Motility
83
what clinical maneuver can you do to better hear an S3 heart sound?
have pt lie down on their left side and have them fully exhale
84
what are some of the clinical features of friedreich's ataxia?
cerebellar ataxia (w/ degeneration of the spinocerebellar tracts), loss of proprioception & vibration, kyphoscoliosis, hypertrophic cardiomyopathy, and high foot arches, also DM
85
how can you prevent the nephrotoxicity assoc. with cisplatin?
use amifostine, which is a free radical scavenger
86
what are the two MCC of aseptic meningitis?
enteroviruses like coxsackievirus & echovirus
87
what is meconium ileus?
distal small bowel obstruction in CF neonate due to abnormally dehydrated meconium
88
what is hartnup disease?
AR disorder that has deficiency of neutral amino acid (e.g. tryptophan) transporters in proximal renal tubular cells and on enterocytes leads to neutral aminoaciduria and can cause pellagra-like symptoms note: hartnup dz can lead to niacin deficiency
89
which enzyme is deficient in classic galactosemia?
deficiency of galactose-1-phosphate uridyl transferase Features: vomiting, lethargy & failure to thrive right after breastfeeding is started "Fructose is to Aldolase B as Galactose is to UridylTransferase -FAB GUT"
90
which pancreatic bud forms the accessory pancreatic duct?
dorsal pancreatic bud note: this forms body, tail, isthmus and accessory pancreatic structure
91
which pancreatic bud forms the main pancreatic duct?
ventral pancreatic bud remember: this forms pancreatic head and main pancreatic duct, and uncinate process is only formed from ventral pancreatic bud
92
what are all the functions of thyroid peroxidase?
catalysis iodide oxidation, formation of mono & diiodotyrosine, and coupling that forms T3 & T4
93
which drug can you use to prevent perinatal transmission of HIV?
zidovudine-retroviral reverse transcriptase inhibitor
94
renal cell carcinoma results from neoplastic transformation of what type of cells?
proximal tubule cells (they are polygonal clear cells that are filled with lipids & carbs)
95
which two types of cells can't use ketones for energy?
RBC's (they have no mitochondria) hepatocytes: have mitochondria but they can't turn acetoacetate into acetyl CoA
96
what is a crossover study?
where subjects are randomly allocated to sequence of 2 or more treatments given consecutively, a washout period is given b/w the first and 2nd treatment
97
what is primidone?
anticonvulsant; drug is metab. to phenobarbital and phenylethylmalonamide (PEMA), all 3 parts are active anticonvulsants
98
which anticholinesterase drug can be used to reverse both CNS & peripheral effects of atropine toxicity?
physostigmine-this is the only anticholinesterase that has tertiary amine structure and so it can cross the BBB
99
what drug do you use to treat low HDL levels?
niacin
100
what is orotic aciduria?
inability to convert orotic acid to UMP (de novo pyrimidine synth. pathway) b/c of defect in UMP synthase, autosomal recessive
101
how do pts with orotic aciduria present?
hypochromic megaloblastic anemia, neurologic abnormalities, growth retardation & excretion of orotic acid in the urine
102
how do you treat orotic aciduria?
treat w/ uridine supplementation which improves symptoms by inhibiting CPSII (which is the regulatory step in this pathway)
103
describe the normal aging of the heart
decreased left ventricular volume develop. of sigmoid-shaped ventricular septum myocyte atrophy w/ interstitial fibrosis & accumulation of cytoplasmic lipofuscin
104
what is apple-peel atresia?
intestinal atresia distal to the duodenum & is due to vascular accident in utero, happens where there is obstruction of the SMA & this leads to blind-ending proximal jejunum w/ absence of long length of small bowel & dorsal mesentery, the terminal ileum distal to the atresia assumes a spiral configuration around an ileocolic vessel
105
what is the MCC of impaired FA beta oxidation?
MC due to Acyl-CoA dehydrogenase deficiency, the enzyme that catalyzes the first step in the beta-oxidation pathway features: hypoglycemia after prolonged fasting with INAPPROPRIATELY LOW LEVELS OF KETONE BODIES
106
which classes of drugs are proven to improve survival and reduce hospitalizations in chronic CHF?
ACEIs (e.g. captopril) Angiotensin II Receptor blockers (e.g. -artans) Aldosterone antagonist (e.g. spironolactone) Certain Beta-blockers (Metoprolol, carvedilol, Bisoprolol)
107
which drugs provide symptomatic relief in CHF ?
Diuretics (loops, thiazides) Digoxin Vasodilators (nitrates)
108
hit me with the mnemonic for the CYP-450 inhibitors?
``` C-cimetidine R-ritonavir (protease inhibitors A-amiodarone C-ciprofloxacin K-ketoconazole A-acute alcohol use M-macrolides I-isoniazid G-grapefruit juice O-omeprazole S-sulfonamides ```
109
hit me with the mnemonic for the CYP-450 inducers?
"Guiness, Coronas & PBRS induce Chronic alcoholism" ``` G-griseofulvin C-carbamazepine P-phenytoin B-barbiturates R-rifampin S-st. john's wort Chronic Alcoholism ```
110
describe the defect in type I dyslipidemia- hyperchylomicronemia
AR, deficiency of lipoprotein lipase or altered apolipoprotein C-II Causes pancreatitis, hepatosplenomegaly, & eruptive/pruritic xanthoma; there is no increased risk for atherosclerosis increased blood levels of chylomicrons, TG, cholesterol
111
describe the defect in Type II dyslipidemia-familial hypercholesterolemia
AD, absent or decreased LDL receptors causes accelerated atherosclerosis, tendon xanthomas, & corneal arcus increased blood levels of LDL & cholesterol
112
describe the defect in type IV dyslipidemia-hypertriglyceridemia
AD, hepatic overprod. of VLDL, causes pancreatitis increased blood levels of VLDL & triglycerides
113
describe the genetic defect in abetalipoproteinemia?
AR, defect in MTP gene leading to decreased B-48 & b-100--> decreased chylomicron & VLDL synthesis and secretion symptoms appear in first few months of life findings: failure to thrive, steatorrhea, acanthocytosis, ataxia, night blindness
114
describe type I glycogen storage disorder-Von Gierke's dz
deficiency in glucose-6-phosphatase, AR findings: severe fasting hypoglycemia, way too much glycogen in liver, increased blood lactate, hepatomegaly
115
Name the dz: lysosomal alpha-1,4-glucosidase (acid maltase) deficiency
Type II Glycogen storage dz-Pompe's Dz findings: cardiomegaly & systemic findings leading to early death
116
name the dz: deficiency in debranching enzyme (alpha-1,6-glucosidase)
Type III glycogen storage dz-Cori's dz, AR Gluconeogenesis is intact findings: milder form of type I w/ normal blood lactate levels
117
name the dz: deficiency in skeletal muscle glycogen phosphorylase
Type V glycogen storage dz- McArdle's Dz, AR findings: increased glycogen in muscle, can't break it down, leads to painful muscle cramps, myoglobinuria with strenuous exercise
118
name the dz: deficient in alpha-galactosidase A
Fabry's dz (lysosomal storage dz), X-linked recessive findings: increased ceramide trihexoside buildup, peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal dz
119
name the dz: deficiency in glucocerebrosidase
Gaucher's disease (MC lysosomal storage dz) features: hepatosplenomegaly, aseptic necrosis of femur, bone crises, Gaucher's cells (mphages that look like crumpled tissue paper) accumulate substrate = glucocerebroside
120
Niemann-Pick dz has a deficiency in what enzyme?
sphingomyelinase accumulated substrate = sphingomyelin features: prog. neurodegeneration, hepatosplenomegaly, cherry-red spot on macula, foam cells
121
Tay-Sach's dz has a deficiency in what enzyme?
Hexosaminidase A Lysosomal storage dz Accumulated substrate = GM2 Ganglioside Features: prog. neurodegen., developmental delay, cherry-red spot on macula, lysosomes w/ onion skin, NO HEPATOSPLENOMEGALY
122
what enzyme is deficient in Krabbe's dz?
Galactocerebrosidase Lysosomal Storage Dz accumulated substrate = Galactocerebroside features: peripheral neuropathy, developmental delay, optic atrophy, globoid cells
123
what is the genetic defect in metachromatic leukodystrophy?
arylsulfatase A deficiency Lysosomal storage dz accumulated substrate = cerebroside sulfate features: central & peripheral demyelination w/ ataxia, dementia
124
name the dz: deficiency in alpha-L-iduronidase
Hurler's syndrome Mucopolysaccharidoses-Hurler's syndrome accumulated substrate = heparan sulfate, dermatan sulfate features: developmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly
125
name the dz: deficiency of iduronate sulfatase
Mucopolysaccharidoses-Hunter's syndrome (X-linked recessive) accumulated substrate = heparan sulfate, dermatan sulfate features: mild hurler's + aggressive behavior, no corneal clouding
126
what is your mnemonic for NON-ENVELOPED viruses?
"Give PAPP smears & CPR to a naked Heppy" (DNA = PAPP, RNA = CPR & hepevirus) Papillomavirus (HPV) Adenovirus Parvovirus Polyomavirus (JC virus) Calicivirus (Norwalk) Picornavirus Reovirus (Rotavirus & Coltivirus) Hepevirus (HEV)
127
what is your mnemonic for the negative strand viruses?
"Always Bring Polymerase Or Fail Replication" Arenaviruses Bunyaviruses (bunyavirus & Hantavirus) Paramyxoviruses Orthomyxoviruses (influenza virus) Filoviruses (Ebola) Rhabdoviruses (rabies virus)
128
what is your mnemonic for the segmented viruses?
BOAR (all are RNA viruses) Bunyaviruses (Bunyavirus & hantavirus) Orthomyxoviruses (influenza) Arenaviruses Reoviruses (rotavirus & coltivirus)
129
what is the main lab marker used to confirm menopause?
FSH; note LH goes up but this happens later
130
what type of amyloid is found in cardiac amyloidosis?
derived from ANP
131
how does tetrodotoxin (pufferfish toxin) work?
it is a neurotoxin that binds voltage-gated sodium channels in nerve & cardiac tissue, preventing Na+ influx & depolarization
132
what are 3 major congenital cyanotic heart dzs that result from a failure of neural crest migration?
Tetrology of fallot Transposition of the Great Vessels Truncus Arteriosus
133
which two classes of drugs are assoc. w/ fat redistribution syndrome?
HIV protease inhibitors glucocorticoids
134
what is the only bacterial DNA polymerase w/ 5' to 3' exonuclease activity?
DNA polymerase I (has same functions as DNA polymerase III but also excises RNA primer w/ 5' to 3' exonuclease)
135
what effect does myelination have on the length constant and time constant of a nerve?
increases the length constant decreases the time constant
136
what is the major virulence factor for strep pyogenes, it is responsible for inhibiting phagocytosis and complement activation, it also activates bacterial adherence & is the target of type-specific humoral immunity to strep pyogenes?
Protein M
137
parasite that causes terminal hematuria and bladder cancer
Schistosoma haematobium; caused by contact w/ freshwater snails
138
what is the usual cause of neonatal tetanus?
colonization of C. tetani in the umbilical stump (can be prevented by proper immunization)
139
name the dz: defect in ATM gene that codes for a protein involved in DNA break repair; immune deficiency has IgA deficiency and predisposes to infections of the upper and lower airways; has increased risk of sinopulmonary infections w/ cerebellar ataxia, & telangiectasias
ataxia-telangiectasia (AR)
140
name the dz: subtype of lung adenocarcinoma, uncommon, arises in non-smokers, arises from alveolar epithelium, often peripheral & multifocal; histology shows tall, columnar cells that line the alveolar septa w/o evidence of vascular or stroma invasion
bronchoalveolar carcinoma
141
40-60% of pts with melanoma have what mutation?
BRAF V600E
142
name the defense mechanism: immature defense mechanism that substitutes an imaginary, less disturbing view of the world to avoid awareness of painful feelings
fantasy
143
name the dz: has saddle anesthesia, loss of anocutaneous reflex; assoc. w/ S2-S4 damage
cauda equina syndrome
144
name the dz: lymphatic malignancy assoc. w/ persistent lymphedema like in a post-radical mastectomy
lymphangiosarcoma
145
what are the lab markers of Ca2+, PO4, & PTH in osteoporosis?
NORMAL
146
name the dz: defect in cystathionine beta synthetase (converts homocystine to cystathionine), and subsequently cysteine becomes an essential amino acid
homocystinuria
147
name the dz: AD dz caused by abnormal nucleotide mismatch repair; genes include MSH2 & MLH1, which codes for components of human MutS & MutL homologs
HNPCC (Lynch syndrome) the mutations in MSH2 & MLH1 account for ~90% of cases of lynch syndrome
148
which type of colonic polyp can cause symptoms of secretory diarrhea?
villous adenomas -tend to be larger, sessile, and more severely dysplastic than tublar, can also cause bleeding, secretory diarrhea is due to secretion of large amounts of mucus; can also cause partial intestinal obstruction
149
which mood stabilizer drug has side effects which include hypothyroidism and polyuria (nephrogenic diabetes insipidus)?
Lithium
150
what is Parakeratosis?
hyperkeratosis with retention of nuclei in the stratum corneum (seen in psoriasis)
151
what is hyperkeratosis?
increased thickness of stratum corneum (seen in psoriasis, calluses)
152
what is spongiosis?
epidermal accumulation of edematous fluid in intercellular spaces (seen in eczematous dermatitis)
153
what is acantholysis?
separation of epidermal cells (seen in pemphigus vulgaris)
154
what is acanthosis?
epidermal hyperplasia (increased stratum spinosum) e.g. acanthosis nigricans
155
what is the most likely location for colon adenocarcinoma?
rectosigmoid colon
156
Lacunar infarcts are most often due to what?
hypertensive arteriolosclerosis of small, penetrating arterioles
157
ZES causes recurrent ulcers in what parts of the GI tract?
distal duodenum and jejunum
158
name the vitamin deficiency: causes degeneration of spinocerebellar tracts, dorsal column of spinal cord and peripheral nerves, also hemolytic anemia, acanthocytosis, and muscle weakness
Vitamin E deficiency note: this presentation is similar to Friedreich's ataxia
159
22q11 syndromes are associated with which cardiac defects?
truncus arteriosus & Tetralogy of Fallot
160
Which dz may cause these congenital cardiac defects: ASD, VSD, AV septal defect (endocardial cushion defect)
Down syndrome
161
Which congenital cardiac defects are assoc. w/ congenital rubella?
Septal defects PDA pulmonary artery stenosis
162
which dz is assoc. w/ these congenital heart defects: bicuspid aortic valve (MC in this disorder), coarctation of the aorta (preductal)
turner syndrome
163
Marfan syndrome is assoc. with which congenital heart defects?
MVP thoracic aortic aneurysm & dissection aortic regurgitation
164
which cardiac defect is associated with infants of diabetic mothers?
transposition of the great vessels
165
name the drug: PDE inhibitor used for intermittent claudication, inhibits platelet aggregation & is a direct arterial vasodilator, has been shown to be superior to aspirin in treatment of peripheral arterial dz
cilostazol
166
name the class of antiarrhythmics: potent Na channel blockers that exhibit strong-use dependence by prolonging the QRS duration to a greater extent at higher heart rates
Class IC antiarrythmics Flecainide & Propafenone
167
what is your mnemonic for retroperitoneal structures?
SAD PUCKER S-suprarenal (adrenal glands) A-Aorta & IVC D-duodenum (2nd-4th parts) ``` P-Pancreas (except tail) U-Ureters C-Colon (ascending & descending) K-kidneys E-Esophagus (lower 2/3) R-rectum (partially) ```
168
name the dz: have small jaws, small eyes, and malformed low set ears, rocker-bottom feet; note: presence of CLENCHED HANDS w/ OVERLAPPING FINGERS is distinguishing feature
Edwards syndrome (trisomy 18)
169
name the dz: triad of tinnitus, vertigo, sensorineural hearing loss; pathogenesis is related to increased volume & pressure of endolymph in the vestibular apparatus
Meniere's dz
170
these are adverse effects for which drug: changes in color vision and anorexia, can cause ventricular dysrhythmias, headache, fatigue, and confusion
digoxin
171
what's the diagnosis: sudden onset abdominal/flank pain, hematuria & left sided-varicocele in a pt with membranous glomerulopathy
renal vein thrombosis nephrotic syndrome is a hypercoagulable state and renal vein thrombosis is a common complication of nephrotic syndrome
172
how does C. diff toxin A work?
attracts neutrophils causing mucosal inflammation, loss of water into the gut lumen and diarrhea, as well as mucosal death
173
how does C. diff toxin B work?
cytotoxin that causes actin depolymerization, loss of cellular cytoskeleton integrity cell death and mucosal necrosis
174
treatment of choice in arsenic poisoning?
dimercaprol
175
what is the MC benign liver tumor?
cavernous hemangioma note: don't biopsy b/c this can cause fatal bleeding and doesn't help with the diagnosis
176
what is the drug of choice in trigeminal neuralgia?
carbamazepine
177
the vaccine for haemophilus influenzae type b is composed of what?
polyriboysl-ribitol-phosphate, which is a component of the Hib capsule and it is conjugated to diptheria or tetanus toxoid
178
what is the formula for power?
1-beta power is the probability of finding a true relationship
179
name the tumor: benign capillary hemangioma of infancy; appears in first few wks of life, grows rapidly & regresses spontaneously at ~5-8 y/o
strawberry hemangioma
180
name the tumor: benign capillary hemangioma of the elderly, does not regress, increased frequency w/ age
cherry hemangioma
181
name the tumor: polypoid capillary hemangioma that can ulcerate & bleed, assoc. w/ trauma and pregnancy
pyogenic granuloma
182
name the tumor: cavernous lymphangioma of the neck, assoc. w/ turner's syndrome
cystic hygroma
183
name the tumor: benign painful, red-blue tumor under the fingernails, arises from modified smooth muscle cells of the glomus body
glomus tumor
184
name the tumor: rare BV cancer occurring in the head, neck, and breast areas, usually in elderly, on sun-exposed areas, assoc. w/ radiation therapy & arsenic exposure, very aggressive and difficult to resect due to delay in diagnosis
angiosarcoma
185
name the tumor: lymphatic malignancy assoc. w/ persistent lymphedema (e.g. post-radical mastectomy)
lymphangiosarcoma
186
name the dz: chronic musculoskeletal pain disorder affecting women 20-50 y/o, pain is diffuse and found at multiple symmetrical tenderspots, other features include insomnia & emotional disturbances
fibromyalgia
187
name the dz: pain and stiffness in shoulders and hips, often w/ fever, malaise, & weight loss, doesn't cause muscular weakness, more common in women over 50 y/o
polymyalgia rheumatica labs: increased ESR, CRP, with a normal CK
188
what is the MCC of croup (viral laryngotracheitis)?
parainfluenza virus
189
what is the MCC of viral bronchiolitis?
RSV
190
what is a common complication of prematurity that can lead to long-term neurodevelopmental impairment characterized by a hemorrhage into the fragile germinal matrix?
neonatal intraventricular hemorrhage increases in freq. w/ decreasing age & birthweight common complication of prematurity
191
name the drug: requires an acidic environment (like in the mphage phagolysosomes) to exert antimicrobial effects
pyrazinamide
192
Pts w/ Fabry dz (deficiency in alpha-galactosidase A) who don't receive enzyme replacement will die of what?
renal failure
193
name the dz: due to deficient Porphobilinogen deaminase (aka HMB synthase, converts PBG --> HMB), features: abd. pain & neuro manifestations, no photosensitivity, urine darkens upon standing, increased urinary ALA & PBG; give glucose which will decrease activity of ALA synthase and thus alleviating the abd. pain & neuropsych manifestations
acute intermittent porphyria
194
what are the 3 stop codons?
UGA UAA UAG
195
name the dz: characterized by sacral agenesis causing lower extremity paralysis & urinary incontinence, COMMONLY ASSOC. W/ MATERNAL DM
Caudal regression syndrome
196
taking which two classes of drugs used to treat hyperlipidemia will increase your risk of getting myopathy?
statins + fibrates
197
the combination of taking fibrates and bile acid-binding resins increases your risk for what adverse effect?
cholesterol gallstones
198
what kind of insulin do you use when treating DKA?
regular insulin
199
name the dz: caused by cystathionine synthetase deficiency; pts present with symptoms that resemble marfans, like a ectopia lentis (dislocated lens) and some have developmental delay; they have high risk of thromboembolism; about half of these pts respond to Vitamin B6 (pyridoxine) which is a cofactor for the deficient enzyme
homocystinuria
200
breast milk lacks what two important fat soluble enzymes?
Vitamin D (black kids at higher risk of deficiency) Vitamin K (given parenterally to prevent hemorrhagic dz of newborn)
201
what is the drug of choice for hairy cell leukemia?
cladribine this is a purine analog that achieves high intracellular concentrations b/c it is resistant to degradation by adenosine deaminase
202
name the drug: COMT inhibitor that serves to increase the bioavailability of levodopa by inhibiting peripheral methylation
entacapone used in Parkinson's
203
name the glcyogen storage disorder: leads to incomplete glycogen degradation. Alpha-1,6-glucosidic branch points can't be degraded, so small chain dextrin-like material accumulates w/I the cytosol of hepatocytes; Present w/: hypoglycemia hypertriglyceridemia, ketoacidosis, and hepatomegaly
Cori Dz (AR debranching enzyme deficiency)
204
name the dz: cerebellar hemangioblastoma w/ congenital cysts of the kidneys, liver, and/or pancreas, AD
von hippel lindau dz
205
what is the most common location for colonic diverticula?
sigmoid colon
206
what is the cofactor for amino acid transamination and decarboxylation reactions?
pyridoxal phosphate (B6)
207
what anatomical structure is damaged in nursemaid's elbow?
annular ligament results from sudden traction on the outstretched and pronated arm of a kid; kids don't hurt until they move the arm
208
what is your mnemonic for encapsulated bacteria?
"Even Some Pretty Nasty Killers Have Shiny Bodies" ``` E-E. coli (some strains) S-Strep pneumoniae P-Pseudomonas N-Neisseria meningitidis K-Klebsiella H-Haemophilus influenzae S-Salmonella B-group B strep ```
209
Name the lymph node cluster: drains head and neck
cervical
210
Name the lymph node cluster: drains lungs
hilar
211
Name the lymph node cluster: drains trachea and esophagus
mediastinal
212
Name the lymph node cluster: drains upper limb, breast, skin above umbilicus
axillary
213
Name the lymph node cluster: drains liver, stomach, spleen, pancreas, upper duodenum
celiac
214
Name the lymph node cluster: drains lower duodenum, jejunum, ileum, colon to splenic flexure
superior mesenteric
215
Name the lymph node cluster: drains colon from splenic flexure to upper rectum
inferior mesenteric
216
Name the lymph node cluster: drains lower rectum to anal canal (above pectinate line), bladder, vagina (middle third), prostate
internal iliac
217
Name the lymph node cluster: testes, ovaries, kidneys, uterus
Para-aortic
218
Name the lymph node cluster: drains the anal canal (below pectinate line), skin below umbilicus (except popliteal territory)
superficial inguinal
219
Name the lymph node cluster: drains the dorsolateral foot, posterior calf
popliteal
220
What is the rate limiting step in glycolysis?
PFK1
221
What is the rate limiting step in gluconeogenesis?
Fructose-1,6-bisphosphatase
222
What is the rate limiting step in TCA cycle?
isocitrate dehydrogenase
223
What is the rate limiting step in glycogen synthesis?
glycogen synthase
224
What is the rate limiting step in glycogenolysis?
glycogen phosphorylase
225
What is the rate limiting step in HMP shunt?
G6PD
226
What is the rate limiting step in de novo pyrimidine synthesis?
CPS II
227
What is the rate limiting step in de novo purine synthesis?
Glutamine-PRPP amidotransferase
228
What is the rate limiting step in urea cycle?
CPS I
229
What is the rate limiting step in fatty acid synthesis?
Acetyl-CoA carboxylase
230
What is the rate limiting step in fatty acid oxidation?
carnitine acyltransferase I
231
What is the rate limiting step in ketogenesis?
HMG-CoA Synthase
232
What is the rate limiting step in cholesterol synthesis?
HMG-CoA reductase
233
what are the regulators of PFK1 (RLS of glycolysis)?
stimulates: AMP, fructose-2,6-BP inhibits: ATP, citrate
234
what stimulates Fructose-1,6-bisphosphatase (RLS of gluconeogenesis)?
ATP
235
What inhibits fructose-1,6-bisphosphatase (RLS of gluconeogenesis)?
AMP | Fructose-2,6-BP
236
what stimulates isocitrate dehydrogenase (RLS of TCA cycle)?
ADP
237
what inhibits isocitrate dehydrogenase (RLS of TCA cycle)?
ATP | NADH
238
What stimulates glycogen synthase (RLS of glycogen synthesis)?
Glucose | Insulin
239
What inhibits glycogen synthase (RLS of glycogen synthesis)?
epinephrine | glucagon
240
what stimulates glycogen phosphorylase (RLS of glycogenolysis)?
AMP epinephrine glucagon
241
what inhibits glycogen phosphorylase (RLS of glycogenolysis)?
insulin | ATP
242
What stimulates G6PD (RLS of HMP shunt)?
NADP+
243
What inhibits G6PD (RLS of HMP shunt)?
NADPH
244
What molecules inhibit glutamine-PRPP amidotransferase (RLS of de novo purine synthesis)?
AMP IMP GMP
245
What molecule stimulates CPS I (RLS of urea cycle)?
N-acetylglutamate
246
what stimulates Acetyl-CoA Carboxylase (RLS of fatty acid synthesis)?
insulin | citrate
247
what inhibits acetyl-CoA carboxylase (RLS of fatty acid synthesis)?
glucagon | palmitoyl-CoA
248
what molecule inhibits carnitine acyltransferase I (RLS of fatty acid oxidation)?
malonyl-CoA
249
What stimulates HMG-CoA reductase (RLS in cholesterol synthesis)?
Insulin | Thyroxine
250
What inhibits HMG-CoA reductase (RLS of cholesterol synthesis)?
Glucagon | Cholesterol
251
name the drug: short acting balanced venous and arterial vasodilator that decreases both preload and afterload; this has balanced changes, so SV stays the same
nitroprusside
252
which types of vasculitis are considered granulomatous?
Large vessel: Temporal arteritis & Takayasu's arteritis Small vessel: Wegener's, Churg-Strauss
253
Which types of vasculitis are considered immune complex mediated?
Medium vessel: PAN | small vessel: HSP (IgA deposition)
254
why do we use beta blockers in thyrotoxicosis?
decreases the effect of sympathetic adrenergic impulses reaching target organs & DECREASES THE RATE OF PERIPHERAL CONVERSION OF T4 to T3
255
name the drug: this is a lipopeptide antibiotic w/ activity against MRSA, causes depolarization of bacterial cell membrane and inhibition of DNA, RNA, and protein synthesis; AE = INCREASED CPK LEVELS & INCREASED INCIDENCE OF MYOPATHY
daptomycin
256
which antiarrhythmic has Class III activity but does not predispose to torsades de pointes like other class III drugs?
amiodarone
257
what are two reasons that pregnant women and women taking OCPs are at an increased risk for gallstones?
1. estrogen induces cholesterol hypersecretion | 2. Progesterone induces gallbladder hypomobility
258
name the pathology: Pharyngoesophageal false diverticulum A Herniation of mucosal tissue at Killian triangle between the thyropharyngeal and cricopharyngeal parts of the inferior pharyngeal constrictor. Presenting symptoms: dysphagia, obstruction, foul breath from trapped food particles (halitosis). Most common in elderly males.
Zenker diverticulum
259
Name the bacterial virulence factor:binds to the Fc part of IgG at the complement-binding site and so prevents activation of complement; this leads to decreased production of C3b and thus impaired opsonization
Protein A (Staph aureus)
260
Name the alzheimer's drug: NMDA receptor antagonist; helps prevent excitotoxicity (mediated by Ca2+)
memantine
261
what is donepizil used for?
Cholinesterase inhibitor used in Alzheimer's dz; this works b/c there is decreased ACh in Alzheimer's
262
stimulating what sympathetic receptor inhibits insulin release?
alpha2-adrenergic receptors
263
Stimulating what sympathetic receptor causes insulin to be released?
beta2-adrenergic receptors
264
how do you diagnose von Willebrand dz?
prolonged bleeding time prolonged PTT decreased response to RISTOCETIN (which activates GP Ib-IX receptors on platelets and makes them available for vWF binding)
265
name the childhood leukemia: presents w/ large anterior mediastinal mass that can compress the great vessels causing SVC syndrome and possible compressing the esophagus causing dysphagia & stridor
T-cell ALL (only ~15% of all ALL)
266
Name the pathological finding: interstitial myocardial granulomas; seen in rheumatic carditis
Aschoff Bodies
267
name the pathology: plump macrophages w/ abundant cytoplasm & central, round-to-ovoid nuclei w/ central slender ribbons of chromatin; seen in Aschoff bodies
Anitschkow cells
268
name the pathology: has NON-CASEATING GRANULOMAS, cobblestone appearance to mucosa, transmural inflammatory infiltrate
Crohn's dz
269
``` name the dz: AD Inherited disorder of blood vessels. Findings: telangiectasia, recurrent epistaxis, skin discolorations, arteriovenous malformations (AVMs), GI bleeding, hematuria ```
Hereditary Hemorrhagic Telangiectasia (aka Osler-Weber-Rendu syndrome)
270
Name the dz: Neurocutaneous disorder characterized by café-au-lait spots and cutaneous neurofibromas. Autosomal dominant, 100% penetrance, variable expression
NF 1 (aka von Recklinghausen dz) AD, caused by mutations in the NF1 gene on chromosome 17 mnemonic: 17 letters in von Recklinghausen
271
name the dz: Findings: bilateral acoustic schwannomas, juvenile cataracts, meningiomas, and ependymomas
Neurofibromatosis type 2 NF2 gene on chromosome 22
272
name the dz: Neurocutaneous disorder with multi-organ system involvement, characterized by numerous benign hamartomas, angiofibromas, mitral regurg, ash-leaf spots, cardiac rhabdomyoma, mental retardation, renal angiomyolipoma, seizures, shagreen patches, increased incidence of subependylmal astrocytomas & ungual fibromas
Tuberous sclerosis, AD
273
Name the dz: features cavernous hemangiomas in skin, mucosa, organs; bilateral renal cell carcnomas; hemangioblastoma in retina, brainstem, and cerebellum and pheochromocytomas
von Hippel-Lindau dz AD mutation in VHL tumor suppressor gene on chromosome 3, which results in constitutive expression of HIF
274
name the dz: features port-wine stain of the face, ipsilateral leptomeningeal angiomas that cause seizures/epilepsy, intellectual disability and episcleral hemangioma --> increased IOP --> early onset glaucoma
Sturge-Weber Syndrome Mnemonic: "STURGE" ``` S-sporadic, port-wine Stain T-tram track Ca2+ (opposing gyri) U-unilateral R-retardation G-Glaucoma, GNAQ gene E-epilepsy ```
275
what is the genetic defect in Sturge-weber syndrome?
congenital, non-inherited (somatic), developmental anomaly of neural crest derivatives due to activating mutation of GNAQ gene Mnemonic: "STURGE" ``` S-sporadic, port-wine Stain T-tram track Ca2+ (opposing gyri) U-unilateral R-retardation G-Glaucoma, GNAQ gene E-epilepsy ```
276
what is the genetic defect in von Hippel-Lindau disease?
AD mutation in VHL tumor suppressor gene on chromosome 3, which results in constitutive expression of HIF
277
The skin tumors of NF-1 have what embryological derivative?
neural crest cells
278
what is the most important risk factor for pancreatic cancer?
smoking
279
when you see an elderly female who presents with painless jaundice, what should you start thinking about?
pancreatic cancer (compressing the common bile duct)
280
what are the two main drugs used to treat narcolepsy?
Amphetamines Modafinil (psychostimulant)
281
how can you prevent acyclovir induced crystalline nephropathy?
GIVE ADEQUATE HYDRATION
282
pure red cell aplasia is associated with what other dzs?
Thymoma Lymphocytic leukemias Parvovirus B19
283
what is the drug of choice for the prevention and treatment of post-MI Arrhythmias?
lidocaine b/c lidocaine binds rapidly depolarizing and depolarized cells (like ischemic myocardium)
284
what is your mnemonic for the glycogen storage diseases?
"Very Poor Carbohydrate Metabolism" V-von Gierke's-Type I, deficiency = Glucose-6-phosphatase P-Pompe's-Type II, deficiency = Lysosomal alpha-1,4-glucosidase aka acid maltase C-Cori's-Type III, deficiency = debranching enzyme (alpha-1,6-glucosidase) M-Mcardle's-Type V, deficiency = skeletal muscle glycogen phosphorylase
285
what does the LAD supply?
anterior 2/3 of interventricular septum anterior papillary muscle anterior surface of L. ventricle
286
what does the posterior descending/interventricular artery supply?
posterior 1/3 of interventricular septum Posterior walls of ventricles
287
what is the ECG triad of WPW syndrome?
ECG triad: shortened PR-interval, delta wave at start of QRS, widened QRS interval
288
What are the hyper problems of thiazides?
Hyperuricemia Hypercalcemia Hyperglycemia Hyperlipidemia
289
what is the MCC of increased AFP?
underestimation of gestational age
290
which types of insulin are long-acting?
Glargine | Detemir
291
Which types of insulin are rapid-acting?
Lispro Aspart Glulisine
292
which type of insulin is considered short-acting?
regular insulin
293
which type of insulin is considered intermediate acting?
NPH (used in DKA)
294
what is the most common tumor of the adrenal medulla in children, usually < 4 y/o?
Neuroblastoma
295
name the pathology: presents in young child w/ abdominal distension & a firm, irregular mass that can cross the midline
neuroblastoma Note: derived from neural crest cells, can occur anywhere along the sympathetic chain
296
what lab tests could help you diagnose a neuroblastoma?
HVA (breakdown product of dopamine) in urine Bombesin +
297
neuroblastomas are associated with overexpression of what oncogene?
N-myc
298
Which leads will show Q waves with an infarct in the anterior wall (LAD)?
V1-V4
299
Which leads will show Q waves with an anteroseptal (LAD) infarct?
V1-V2
300
Which leads will show Q waves with an anterolateral (LAD or LCX) infarct?
V4-V6
301
which leads will show Q waves in a lateral wall (LCX) infarct?
I, aVL
302
Which leads will show Q waves in an inferior wall (RCA) infarct?
II, III, aVF
303
Name the murmur: in early diastole during rapid ventricular filling phase. Associated with  filling pressures (e.g., mitral regurgitation, CHF) and more common in dilated ventricles (but normal in children and pregnant women).
S3
304
Name the murmur: in late diastole. High atrial pressure. Associated with ventricular hypertrophy. Left atrium must push against stiff LV wall
S4 "atrial kick"
305
What is your mnemonic for ring enhancing lesions in the brain?
"MAGIC DR L" ``` M-mets A-abscess G-GBM I-infarct C-contusion ``` D-demyelinating dz R-Radiation necrosis L-lymphoma don't forget toxoplasmosis
306
which kinds of endocrine hormones and cytokines are receptor-associated tyrosine kinase (JAK/STAT pathway?
Prolactin Immunomodulators (e.g. cytokines-IL-2, IL-6, IL-8, IFN) GH Mnemonic: JAK PIGs
307
which type of endocrine hormones and molecules have intrinsic tyrosine kinase activity?
``` Insulin IGF-1 FGF PDGF EGF ``` MAP Kinase, think growth factors
308
which hormones use steroid receptors?
``` Vitamin D Estrogen Testosterone T3 & T4 Cortisol Aldosterone Progesterone ```
309
which hormones & molecules use IP3 signaling pathways?
``` GnRH Oxytocin ADH (V1-receptor) TRH Histamine (H1-receptor) Angiotensin II Gastrin ```
310
which two vasodilators use the cGMP signaling pathway?
ANP | NO
311
Which hormones use the cAMP signaling pathway?
FLAT ChAMP FSH LH ACTH TSH ``` CRH hCG ADH (V2 receptor) MSH PTH ``` others: calcitonin, GHRH, glucagon
312
name the pathology: assoc. w/ IgG4 abs to the phospholipase A2 reeptor
membranous nephropathy
313
what are the three gram negative bacteria that are oxidase + ?
``` Pseudomonas aeruginosa Vibrio cholerae (also glucose +) Moraxella catarrhalis (diplococcus) ```
314
what is your mnemonic for the adult brain tumors?
"MGM Studios" Mets GBM Meningioma Schwannoma
315
prolonged exposure to loud noises causes hearing loss due to damage of what?
stereociliated hair cells of the organ of corti
316
term for: the proliferation of astrocytes in an area of neuron degeneration, leads to the formation of a glial scar which compensates for the volume loss that occurs after neuronal death
gliosis
317
what are the 3 drugs of choice for tonic clonic seizures?
phenytoin carbamazepine valproate
318
what is the DOC for myoclonic seizures?
valproic acid
319
what is the DOC for absence sezirues?
ethosuximide 2nd choice is valproate
320
what is the DOC for simple partial seizures?
carbamazepine
321
what is the DOC for complex partial seizures?
carbamazepine
322
name the pathology: features myxomatous changes in media of large arteries, this predisposes to development of aortic dissections & aortic aneurysms; frequently seen in marfan's
cystic medial degeneration
323
these are the only kidney stones that are RADIOLUCENT; can be detected on ultrasound or CT
uric acid stones
324
what is the MC complication of SAH hemorrhage?
vasospasm ~ 4-12 days after initial insult; you can prescribe nimodipine to prevent this vasospasm
325
happens most commonly in pts < 2 y/o around the ileocecal valve; manifests w/ severe, colicky abdominal pain, currant jelly stools and sometimes a palpable mass in the RLQ
intussusception
326
Name the drugs: anion inhibitors block iodide absorption by the thyroid gland via COMPETITIVE INHIBITION
perchlorate, pertechnetate
327
assoc. w/ sickle cell dz or trait, DM, analgesic nephropathy or severe obstructive pyelonephritis; features acute colicky flank pain, gross hematuria and passage of tissue fragments in urine
papillary necrosis
328
what is the main source of glucose from 12-18 hrs of fasting?
glycogenolysis
329
what is the main source of glucose after 18 or more hours of fasting?
gluconeogensis
330
exposure to: arsenic, thorotrast, PVC, tumor cells express CD31 an endothelial cell marker
hepatic angiosarcoma
331
which sympathetic receptors inhibit uterine contraction (tocolysis) when stimulated?
beta-2
332
which sympathetic receptors cause contraction of the ocular papillary dilator muscle resulting in mydriasis?
alpha-1
333
DOC for paroxysmal supraventricular tachycardia?
adenosine
334
name the drug: slows conduction through the AV node by hyperpolarizing the nodal pacemaker and conducting cells; AE include flushing, chest burning, hypotension and high grade AV block
adenosine
335
what is the greatest risk factor for aortic dissection?
HTN
336
what is the first step in the development of aortic dissection?
intimal tearing of the artery
337
this is an acute inflamm. of the gallbladder that is INITIATED 90% OF THE TIME BY OBSTRUCTION OF THE GALLBLADDER NECK OR CYSTIC DUCT
acute calcuous cholecystitis
338
cofactor for alpha-ketoglutarate DH, transketolase, and pyruvate dehydrogenase
thiamine
339
name the murmur: murmur features wide, fixed splitting of S2; can produce irreversible pulmonary htn as a result of left to right shunting, eventually there is late onset reversal from left to right to RIGHT TO LEFT (eisenmanger syndrome) and this causes cyanosis
atrial septal defect
340
moa = inhibits synthesis of polysaccharide glucan, which is part of fungal CELL WALL
echinocandins (caspofungin & micafungin)
341
name the drug: AE: hirsutism, coarse facial features, acneiform skin rash, gingival hypertrophy, also causes GENERALIZED LYMPHADENOPATHY (pseudolymphoma)
phenytoin
342
how do you distinguish b/w the similar presentations of rubella and rubeola (measles?
both feature a rash that starts on the face and spreads to the trunk Only rubella has additional finding of postauricular lymphadenopathy
343
what lab test can you perform on a pt's urine to determine if there are cystine stones present?
SODIUM CYANIDE-NITROPRUSSIDE TEST detect's cystine's sulfhydryl groups, turns the urine purple after several mins
344
what is cystinuria?
inborn defect of transporter of cystine, ornithine, arginine, and lysine, AR; presents as: nephrolithiasis in pts in their teens and 20's, UA shows pathognomonic hexagonal cystine crystals
345
what is the PAS stain used to diagnose?
stains glycogen & mucopolysaccharides used for: Whipple's dz, AAT in liver, some adenocarcinomas that secrete mucins
346
which nucleotide analog used to treat HIV does not have to be activated?
Tenofovir
347
What are the 3 non-nucleoside reverse transcriptase inhibitors?
Nevirapine Efavirenz Delavirdine Mnemonic "NED's not a nucleoside" note: don't require phosphorylation to be active
348
what is the name of the HIV drug that inhibits HIV genome integration into host cell chromosome by reversibly inhibiting HIV integrase?
Raltegravir Mnemonic: "INTEGRAVIR"
349
Name the drug: moa = slows conduction through the AV node by hyperpolarizing the nodal pacemaker and conducting cells; AE = flushing, chest burning (due to bronchospasm), hypotension & high grade AV block; note this is the DOC for PSVT; very short half life ~10 seconds
Adenosine
350
DOC in hypertensive emergecy
nitroprusside
351
What's the diagnosis? presents as: escalating fever w/ initial diarrhea or constipation followed by hepatosplenomegaly, formation of rose spots on abdomen and possible hemorrhagic enteritis w/ bowel perf
typhoid fever
352
name the drug: increases the peripheral metabolism of levodopa which decreases its effectivenes
vitamin b6 (pyridoxine)
353
Pts w/ an imperforate anus may have what other congenital malformations?
urogenital tract anomalies
354
chronic rejection of a lung transplant usually affects which part of the lungs?
small airways, causing bronchiolitis obliterans syndrome (inflammation and fibrosis of the bronchiolar walls leading to narrowing and obstruction of the bronchioli); symptoms include dyspnea and wheezing
355
name the drug: MOA = inhibits carbohydrate polymerization, specifically inhibits arabinosyl transferase (enzyme that makes a constituent of the mycobacterial cell wall); AE include OPTIC NEURITIS which typically presents in conjunction with decreased visual acuity, central scotoma and color blindness
ethambutol
356
Name the pathology: results from the passage of a large gallstone through a cholecyst-enteric fistula into the small bowel where it ultimately causes obstruction at the ILEOCECAL VALVE; GAS IS SEEN w/I the gallbladder & biliary tree on abdominal x-ray due to the presence of the fistula, and pts presents w/ signs of small bowel obstruction
gallstone ileus
357
What is the virulence factor that allows the e. coli to survive hematogenous spread and to establish meningeal infection?
K-1 antigen
358
name the types of short acting insulin
Glulisine, Aspart, Lispro; menmonic "Short GALs are Sweet"
359
What is formed by the union of the right and left common iliac veins at the level of L4-L5?
IVC
360
What is formed from the joining of the superior mesenteric vein with the splenic vein?
portal vein
361
Name the murmur: asssoc. w/ a low-pitched, holosystolic murmur that accentuates during maneuvers that increase afterload (e.g. handgrip maneuver)
VSD
362
which drugs are first line treatment for hypertriglyceridemia?
fibrates (gemfibrozil & fenofibrate)
363
Name the drug: an integrase inhibitor that disrupts the ability of HIV to integrate its genome into the host cell's chromosomes's thus preventing host cell machinery from being used to synthesize HIV mRNA.
Raltegravir
364
What is the MC cardiac abnormality predisposing to native valve bacterial endocarditis (15 -60 y/o)?
mitral valve prolapse
365
what is the marker for monocyte-macrophage lineage?
CD14
366
Which vessel was occluded? transmural ischemia of inferior left ventricular wall, producing ST elevation in leads II, III, & aVF & possibly SA node dysfunction with bradycardia
RCA
367
Which vessel was occluded? anteroseptal transmural ischemia w/ ST elevations in leads V1-V4
Proximal LAD
368
Which vessel was occluded? transmural ischemia of lateral wall of left ventricle, w/ ST elevations in V5 & V6
LCX
369
Name the pathology: features: diffuse increased thickness of glomerular BM on light microscopy, nephrotic syndrome, spike and dome appearance on silver stain and granular deposits on immunofluorescence are diagnostic
membranous nephropathy
370
what are the two main AE of statins?
myopathy | hepatotoxicity
371
which antineoplastic drug inhibits ribonucleotide reductase?
hydroxyurea
372
which antineoplastic drug blocks de novo purine synthesis?
6-Mercaptopurine