NBME Practice Tests

65 yo w/ painful gout requests a disabled parking permit. What does the physician say?

"I will authorize the permit, but i recommend that you continue w/ regular exercise and only use the permit when you are having severe pain" - DMV will need doctor to verify condition - usually for: Lung disease, Heart disease, impaired mobility, wheelchair, brace, or cane, limited ability walk or to use your legs, Documented vision problems, including low-vision or partial sightedness, Loss of one or both legs or loss of both hands, or limited use of these parts

A physician is sad as he has to inform a pt of terminal carcinoma. the pt comes in sees the physician's face and begins to cry and asks "is it bad news?" how should the physician respond?

"Yes it is." -research shows people want frank and empathetic disclosure of bad news - ABCDE - Advance preparation: arrange adequate time/privacy, facts, data - Building a therapeutic relationship: ID pt preferences for disclosure - Communicating: determine pt knowledge, avoid medical jargon, allow silence/tears, and answer ? - Dealing w/ pt + fam rxn: respond to emotional reactions and empathize - Encouraging/ validating emotions

78 yo has is on life support and neighbor who cooks for and cares for him tells you he wouldn't want to be kept on life support. pt has poor prognosis and has no relatives/children. What is the most appropriate thing to say at this time?

"thank you for telling me this. your friends previous statements to you can be helpful in making decisions for him" NOT "the hospital lawyer must be consulted since we do not know if he has advanced directives"

What can you tell a 13 yo boy with gynecomastia to reassure him?

"this will typically resolve in 12-18 months"

in albinism what do you expect the number of melanocytes and melanin to be decreased, normal, or increased?

# of melanocytes = norma melanin = decreased - normal melanocyte number, w/ decreased melanin production from decreased tyrosinase activity or defective tyrosine transport - phenylalanine -> tyrosine -> DOPA -> melanin (via tyrosinase) - increased risk of skin cancer

If the splenic artery is ligated, what organs will be affected?

- Body/tail of pancreas - fundus of stomach (short gastric arteries) - NOT the pyloris of the stomach as even though it gives off left gastroepiploic artery this anastomoses with the right gastroepiploic artery - spleen itself

if you were to compare a healthy 70 yo woman to her 20 year old self. Would you expect residual volume, arterial PO2, and alveolar-arterial PO2 difference to be increased/decreased/same?

- Residual volume: increased (increase in lung compliance of elderly from decreased elasticity) - Arterial PO2: decreased (from decrease in diffusing capacity) - Difference: increased (as less air is being diffused across into blood) - increased lung compliance, but decreased chest wall compliance (wall stiffness) -TLC stays the same

Girl with bed bug bites is at greatest risk for what infection?

staphylococcus aureus - scratching causes staph infection

45 yo w/ bariatric weight loss and diagnosed prediabetic. What foods should he avoid?

starchy foods

56 yo w/ chest pain. Pulse is 110, RR 20, BP 105/70 w/ cool clammy skin. pulmonary cath shows pulm artery pressure of 40/25, LA pressure of 25. Compared to a healthy person what will his pressure be for: capillary hydrostatic, interstitial hydrostatic, interstitial oncotic?

- Capillary hydrostatic: increased - interstitial hydrostatic: increased - interstitial oncotic: decreased - the inreased pressure in the capillary -> floods pleural space increasing interstitial pressure but it is transudative which is low protein and mostly liquid that will dilute the interstitial oncotic pressure

Woman with untreated graves disease has a baby w/ stridor, nasal flaring, and intercostal retractions. Why?

- Enlarged thyroid gland - Thyroid stimulating immunoglobulins can cross the placenta as it is IgG and IgG crosses placenta -IgA in breast milk

Why is there a hyperestrogenic state in liver cirrhosis?

- Failure of liver to degrade estrogen -> decreased metabolism - increased adrenal production of androstenedione -? peripheral conversion via aromatase -> estradiol - increases production of sex-binding globulin which bind testosterone and decrease testosterone/estrogen ratio

38 yo homeless man in ER for stomach cramps and appears drunk. He says he felt sick when he drank a homemade alcoholic drink. his respirations are 33/min, he has visual disturbances, and has metabolic acidosis w/ an anion gap. what is the mechanism of an antidote for this methanol poisoning?

- Fomepizole (alcohol dehydrogenase inhibitor) - inhibits alcohol dehydrogenase, blocking conversion of methanol -> formaldehyde - if not blocked, formaldehyde -> formic acid causing metabolic acidosis and tissue injury - used for overdose of methanol or ethylene glycol

50 kg animal receives 1L 5% saline IV. Will his intracellular volume/osmo and extraceullular volume/osmo be increased or decreased?

- Intracellular Volume: decreased - Intracellular Osm: increased - Extraceullular volume: increased - extracellular osmo: increased - giving a high salt fluid into the extracellular space via IV -> water will come out of the intracellular cells thus decreasing the volume and increasing osmo (relative to loss of water from cell) - Also dosing high salt to extravascular = high volume as water comes out of cell, and high osm as 5% is high salt that is not permeating the cell and have a salty environment pulling water from cells

a ten yo has an abrasion on his knee and one week has epidermal cells covering many areas of the skin. What kind of cell are epidermal cells where they can heal this way?

- Labile cells: never go in G0 and divide rapidly w/ a short G1 -> most affected in chemotherapy (bone marrow, gut epithelium, skin, hair follicles, germ cells) - Permanent cells: remain in G0 and regenerate from stem cells (neurons, skeletal and cardiac muscles, RBC) - Stable (quiescent) cells: Enter G1 from G0 when stimulated (hepatocytes, lymphocytes, PCT, periosteal cells of bone)

If taking ibuprofen, What will the levels of Leukotriene C4, Arachidonic acid, prostaglandin H2, prostaglandin E2 be?

- Leukotrien C4 and AA = increased - both PG decreased - NSAIDS block COX that turns AA -> PGH2 -> PGI2, E2, THXA2

23 yo w/ painless vaginal ulcer, dark field shows positive for spirochetes -> what is the histological change expected on bx of specimen?

- Obliterative endarteritis w/ lymphocytes and plasma cells - this disease is a vasculitis in sorts as the spirochete invades vessesl (hence destruction of vaso-vasorum in the heart -> aortic dissection as blood vessel weakens)

17 yo from party 30 mins after being found w/ blank stare/flat facial expression at party. pulse is 72, BP 104/68. Is sitting upright and is catatonic w/ rigidity. In exam he is hostile and tries to assault doctor. What drug is he on?

- PCP (Phencyclidine) - intoxication -> violence, impulsivity, psychomotor agitation, nystagmus, tachycardia, HTN, analgesia, psychosis - sedative-hypnotic and dissociative anesthetic that generally acts as a downer but can also cause incredible aggression coupled with pain insensitivity (the superman drug). Vertical nystagmus is a commonly mentioned physical exam finding.

How do you test for HIV in a normal person, and how would you test in a child of a mother with HIV?

- Presumptive diagnosis w/ HIV1/2 AG/AB immunoassay and test to detect P24 AG protein (if no AB formed yet, catches virus earlier) and IgG ABs -> high sen/spec - Then Do a HIV1/2 AB differentiation immunoassay -> if + AB/AG but negative HIV1 and 2 -> perform a HIV-1 NAT (nucleic acid test) to catch an acute infection) - Viral load determines RNA in plasma -> high viral load = poor prognosis - Need HIV genotyping to determine therapy - Western blots no longer recommended for confirmatory testing - HIV1/2 AG/AB not used in babies as maternal IgG AB is transferred -> use HIV viral load instead

10 month old w/ 4 day hx fever cough. Illness w/ low grade fever, copious clear nasal discharge + moist, nonproductive cough. He has all vaccines. He goes to daycare and has siblings. Temp is 100, pulse 101, RR 38, BP 85/60. He has intercostal retractions + b/l wheezes and expiratory rhonchi. The infection most likely has what properties?

- RSV bronchiolitis - RNA virus enters cell via fusion protein (target of the prophylactic monoclonal antibody drug Palivizumab = IgG rattle in slime and ghosts are pale) - Attaches to G protein to infect epithelial cells - most common cause of bronchiolitis, pneumonia in infants - virulence factors -> syncium = fusion protein that causes them to stick together (sticky hands of ghosts) - Ribavirion can be used to treat adults (Ghost babies in ribs)

a 65 yo comes to ER after 30 mins of SOB/chest discomfort, weakness. Pulse is 110, RR 22, BP 100/80. PE shows diaphoresis. ECG shows ST elevation in anterior leads. What will his SVR, Pulmonary Vascular Resistance, and PCWP be?

- SVR: increased - PVR: decreased - PCWP: increased - he is having a heart attack with low CO and low BP -> stretch receptors detect less stretch -> more sympathetic and less parasympathetic - increased symp = increased alpha 1 and epi/NE -> increase SVR systemically (greater than B2) - exception is in exercise bc of local metabolites - Pulm vascular resistance will increase if he is bleeding out but here his pulm vascular will drop because of increased PCWP -> inherent vasodilation and the beginning of pulm edema - PCWP increased = decreased pulmonary vascular resistance - PCWP: increased as there is decreased forward flow

Where are there columnar cells in the female reproductive tract?

- Stratified squamous epithelium: Vulva, vagina, ectocervix - squamocolumnar junction (cervical cancer) = transformation zone - simplex columnar epithelium = endocervix/cervical canal - columnar + glands (tubular and coiled depending on phase) = uterus - simple columnar ciliated = fallopian tube - simple cuboidal = ovary

In a mother with down syndrome what do you expect the fetal US nuchal translucency, pregnancy-associated plasma protein, and human chorionic gonadotropin to be?

- US: increased nuchal translucency (delayed pregnancy development in all aneuploidy) - pregnancy-associated plasma protein: decreased = protein that is a marker of fetal well-being -> comes from placenta - HCG: increased (lowered in trisomy 18, not reliable in 13) - low alpha fetoprotein - First screen: US + bHCG +PAP - quad screen: hCG, Alpha fetoprotein, estiol + inhibin A - bHCG: increased - low alpha feto - inhibin A: increased (made by placenta -> increased in sex-cord tumors) - estiol: decreased (pure measure of fetal well being) - Hideff: High HCG/Inhibin, and deficit of estriol and fetoproteins

What is the cause of osteogenesis imperfecta?

- defect in gene coding for procollagen, type I - glycosylation of pro-alpha chain hydroxylysine residue and formation of procollagen via hydrogen and disulfide bonds is interrupted - Can't form triple helix in the RER in cell

What is the metabolism of ethanol vs. ethylene glycol?

- ethanol (alcohol dehydrogenase) -> acetaldehyde (acetaldehyde dehydrogenase) -> Acetate - ethylene glycol (alcohol dehydrogenase) -> glycol aldehyde (aldyhyde dehydrogenase) -> glycolic acid -> -> calcium oxalate (in urine as envelopes) - can tx methanol, ethylene glycol, alcohol overdose with fomepizole (blocks alcohol dehydrogenase) - disulfiram blocks acetaldehyde dehydrogenase and increases acetaldehyde causing hangover

If given 500mg of a drug IV, and the serum is 12.5mg/L in a few minutes -> two hrs later it is 10 mg/L. If this is first order kinetics, what will be the serum concentration in 2 more hrs?

- first order = means time of t 1/2 is constant as serum concentration decreases - zero order: time of t 1/2 decreases as concentration decreases (not a constant 1/2 life disposal) - drug loses 2.5mg in 2 hrs (20% of drug) - 20% of ten = 2 - 2 mg more will be removed = 8

A 72-year-old woman 1-hour difficulty walking/speaking. type 2 DM well. speech. leftsided hemiparesis. tongue deviates to right when protruded. Sensation to pinprick and temperature normal, and proprioception + sensation to light touch are absent over left upper and lower extremities. What location in brain stem is most likely damaged?

- medial medullary syndrome or Dejerine syndrome - Stroke (left hemiparesis + right CN12 palsy) - Crossed findings = brainstem lesion - Right (ipsilateral) tongue - left-sided (contralateral) weakness = exiting right hypoglossal nerve has been affected (in right medulla) - injury to posterior pyramid where corticospinal tract runs to control muscles (prior to decussation) - from an Anterior spinal artery stroke/vertebral arteries - lateral coritcospinal tract: contralateral paralysis of upper and lower limbs - medial lemniscus: decreased contralateral proprioception - caudal medulla = hypoglossal nerve (tongue deviates ipsilaterally) - lateral medullary syndrome (PICA stroke) aka Wallenberg

A transcription factor responds to insulin by altering the transcription of metabolic genes. This TF can responds rapidly to changes in insulin signaling bc it is reversibly modified. Would this TF have nuclear/cytoplasmic shuttling, serine phosphorylation, and ubiquitin-mediated preoteolysis?

- nuclear/cytoplasmic shuttling: yes -> is the intracytoplasmic protein that allows a serum hormone insulin to have intranuclear activation of genetic information - serine phosphorylation: insulin binds tyrosine kinase -> phosphorylated for action - ubiquitin-mediated preoteolysis: rapid degradation of a protein in a proteasome -> this TF is reversibly modified and has rapid response implying that it does not need to be remade and re-transcribed/translated each time. Hence, it is most likely not broken down

Why are paclitaxel and vincristine different?

- paclitaxel: hypestabalize microtubules so mitotic spindle cannot break down (no anaphase in M cycle) cant disassemble - vincristine: bind tubulin and inhibit polymerization and prevent microtubule spindle formation (M-phase arrest) cant form

What does clopidogrel do?

- prevents ADP-induced expression of GpIIb/IIIa stimulated platelet activation by blocking P2Y12 receptor - same drugs = prasugrel, ticlopidine

if histamine is injected in left brachial artery, what will the arteriole resistance, capillary hydrostatic pressure, and capillary filtration rate be in the left arm?

- resistance = decreased - pressure = increased - filtration rate = increased - hypotension, vasodilation, edema - vasodilation causes relaxation of arteriolar smooth muscle -> increased blood flow - increase vascular permeability -> leakage of protein into interstitial space -> increases interstitial oncotic pressure - histamine: pre-capillary arterioles dilate -> increase flow into capillaries -> increase hydrostatic pressure -> increase filtration - increase permeability of post-capillary venules via increasing spacing btw endothelium -> increase capillary filtration - increase in interstitial fluid -> edema

young woman w/ UTI has E Coli. This strain has what virulent factor?

-Mannose-binding (type I) fimbria - gram negative - fimbriae are the cause of cystitis and pyelonephritis (think of them climbing up the tract via fimbriae) - don't confuse with gonorrhea pili

41 yo w/ numbness over right hand, sensation to pinprick is decreased in thumb and over portion of anterior forearm. Doctors thinks compression of C5/C6 spinal nerves as they exit spinal canal. Weakness of what movements will confirm this?

-abduction of the upper extremity - key to this question is that the injury is thought to be a proximal nerve injruy - this would include injury to the long thoracic nerve -> loss of serratus anterior -> cannot abduct arm above horizontal position - also early lesion may be referring to ERB Palsy (waiter tip) -> deltoid and supraspinatus injured-> arm hangs at side - thumb sensation = median nerve and anterior forearm = musculocutaneous nerve - median nerve: contributions from C5-T1 - musculocutaneous nerve - continuation of lateral cord -> just C5, 6, 7 - abduction of arm = suprasinatous -> suprascapular nerve (C5, C6 specifically same for infraspinatus) - deltoid (above 15) /teres minor: axillary nerve (C5, C6) - subscapularis: upper/lower subscapular nerve (C5, C6) - all rotator cuff msucles are C5,C6 - so the answer had to be a rotator cuff function -> internal/external rotation/abduction of arm

If a relative risk is 1.1 (95% CI: 0.8-1.4), What is the p-value calculated from a chi square test?

0.05 < p < 1.0 - 95% CI for a value including 1 in studies with RR and OR is including the null which is not significant - p should be > 0.05 as this is not significant - p values run through 0->1 - the chance a + value is a false positive (alpha) - p cant be 0 nor 1 as it must fall in this range

Someone with sepsis is anxious/confused. Treated w/ vancomycin/ceftriaxone, temp of 103, pulse 132, RR 28, BP 85/48, warm, flushed skin, no edema. What solution do you administer?

0.9% saline - IV fluids, pressors, epinephrine in anaphylaxis

How do you treat a woman with watery diarrhea and dry mucus membranes and an oxidase-positive, curved, gram-negative bacillus?

0.9% saline - vibrio cholera - toxin over-activates adenylate cyclase -> increases cAMP by permanently activating Gs, increases Cl- secretion in to gut and H2O efflux - grows in alkaline media (those taking PPI at increased risk -> same with c diff) acid labile (sensitive) - requires large inoculum - need oral rehydration solution - transmitted through contaminated water or uncooked shellfish

71 yo w/ 30 year hx of DM type II being treated with insulin. What will her endogenous insulin fasting levels be (normal = 5-20uU/mL)

1 uU/mL - in the early stages of DM type II you will have elevated insulin levels as your body is less sensitive to insulin and tries to combat the increased glucose levels - over time = beta cell exhaustion and insulin levels drop and pt needs replacement

What are the four phases of clinical trials?

1. small # of healthy volunteers/pt w/ disease -> is it Safe (asses toxicity, pharmocokinetics, and pharmocodynamics) 2. moderate # of pt w/ disease -> does it Work (treatment efficacy, optimal dosing, adverse effects 3. large # of pt in drug vs. placebo/standard of care -> Improvement? is it good or better of current standard of care 4. postmarketing survelience after approval -> Market (ling term effects/adverse effects can -> black box warning or withdrawal) SWIM

Spinal needle inserted posteriorly in L3/L4 arches will pass through what ligaments to reach spinal canal?

1. supraspinous ligament 2. interspinous ligament 3. ligamentum flavum 4. epidural space (epidural anasthesia stops here) 5. dura matter 6. arachnoid matter 7. subarachnoid space (where you collect CSF in LP)

80 yo cannot concentrate her urine above 450. She accumulated 450 mosm of solute a day which must be excreted by kidneys. She loses 900mL of water in urine and 100ML in sweat and feces. what is the minimum volume of water that she must ingest to prevent and increase in plasma osmolality?

2.0L - I kg water = I L - she can't lose 450 osm/ 1L she takes in - if she accumulates 450 osm she needs no less than a L of water to lose this - and she loses 900ml and 100 ml elsewhere (not in kidneys) - she needs 1L to rid herself of solutes, and one to replace the water lost elsewhere

80 yo is given 80mg dose of drug IV. 30 mins after infusion his serum concentration is 4ug/mL. Assuming distribution 1/2 life of 3 mins and ellimination 1/2 life of 24 hrs, the volume of distribution of this drug (in L) is what?

20 Vd = amount of drug in body/plasma drug concentration - here = 80mg/4ug/mL - turn 4ug/mL-> 4mg/L - 80/4 = 20

What is the inactive form of vitamin D?

24,25-dihydroxycholecalciferol - 25 hydroxycholecalciferol = liver storage form - 1,25 = after kidney = active form

In someone w/ CKD what substance in the vitamin D pathway can they not convert to their active substrate?

25-hydroxycholecalciferol - usually 1-alpha-hydroxylase (in PCT cells stimulated by PTH) converts: 25-hydroxycholecalciferol -> 1,25-dihydroxycholecalciferol

A child with a antibiotic clearance of 0.09L/hr/kg and steady state concentration is 12 ug/mL. What is the maintenance dose in mg/kg/day?

28.8 - maintenance dose = Cp x CL x t/F - Cp= target concentration at steady state - t = dosage interval (time between doses) - F = bioavailability - CL = clearance (12mg/L)(0.09L/hr/kg)(24hr) = 25.92?

25 yo wants to get pregnant but has a seizure disorder that is well-controlled on valproic acid. The fetus will be at greatest risk for drug-related adverse events in what stages of pregnancy?

3-8 weeks = embyonic period - neural tube formed by neuroectoderm and closed by 4th week - time of organogenesis - extremely susceptible to teratogens - causes neural tube defects - increases Na+ channel activation, and GABA concentration by inhibiting GABA transaminase - used for partial/focal, tonic-clonic, absence, and myoclonic seizures + bipolar disease, migraine prophylaxis - SE: GI distress, rare but fatal hepatotoxicity, pancreatitis, neural tube defects in fetus, tremor, weight gain, and not given in pregnancy

52 yo w/ carbon monoxide poisoning is given oxygen. how long will it take for the removal of all of the CO-carrying erythrocytes to clear form the circulation?

4 months - RBC life cycle is 120 days

2 days following an acute MI, a pt becomes short of breath and diaphoretic, high HR and pressure. What murmur would be heard?

4/6 holosystolic murmur heard best over the lower left sternal border and the cardiac apex - from posteromedial papillary muscle rupture (has single blood supply from posterior descending artery) -> severe mitral regurgitation occurs 2-7 days after an MI

7 month old boy no testicles in scrotum. Hernia palpated, and in OR a fallopian tube and uterus is in hernia sac. Testes show no abnormalities. What is the karyotype of this child?

46 XY - absence of sertoli cells or lack of Mullerian inhibitory factor -> develop both male and female internal genitalia and male external genitalia - male develops normally, but there was no inhibition of the paramesonephric duct _. internal female genitalia formed

22 yo man w/ infertility, bx showing scrotal tissue w/ increased hyalinization and fibrosis has what genetic abnormality?

47, XXY - Klinefelters Syndrome - meiotic non-disjunction - dysgenesis of seminiferous tubules -> small firm testes -> decreased inhibin B and increased FSH - abnormal leydig formation -> decreased testosterone, increased LH, increased estrogen (azoospermia)

A paper says " we chose the sample size to have an 80% power of detecting a 15% mean difference w/ a significance (2 sided) level of 5%" if there is no difference btw groups what is this error called, and what would be the chance they would find a statistically significant difference?

5%, type I error - type I error: (alpha) is saying there is a difference when none exists - aka a false positive - if p < 0.05 (or 5%), then this preset level is saying that 95% of the time they expected the study results. But because the study results were not true, they would actually only see these findings 5% of the time

What are the codons for the 5' end of a slice site and the 3' end?

5' = GU 3' = AG - if these are mutated or a new splice site is created in an intron -> retained intron in MRNA -> protein with impaired/altered function (some types of beta-thalassemia)

18 yo has schizophrenia what are the chance his identical twin brother also gets schizophrenia?

50% - same genetics: show a 30-50% correlation in identical twins - 15% correlation in fraternal twins

To decrease CV disease a 24 yo man wants to change diet. Wants to limit calories by 2000. Wants to maintain a 56g protein intake and have a balanced decrease in carbs and fats (fats:carb caloric ratio of 30:55). What describes the # of calories that she be provided from fat each day?

630 calories - 1g of carb/protein (whey) = 4 kcal - 1g alcohol = 7 kcal - 1g fatty acid = 9 kcal (# of letters = # of calories) - 56g protein = 56 x 4 = 224 calories of protein - which leaves 1776 calories - want a 30:55 ratio - 600:1200 - 1776 = 1X + 2X -> 3X - about 592 but the ratio is a little less than 1:2 so there really is more calories than estimates

in a study the recurrent stroke rates/1000 person years are 0.12 for standard treatment and 0.04 for a new anti-platelet drug. What is the relative risk reduction?

67% - RRR = 1-RR - RR = .04/.12 = 0.33 - 1-.33= .67 or 67%

A girl has pincer grasp, finger feeding, standing while holding onto table, and plays peek a boo. What is the patients age in months?

9 months - children stand by 10 months (crawls by 8 months) - pincer grasp by 10 months - orients to name and gestures (9 months) - object permanence by 9 months - says mama and dada by 10 months

What is the most specific finding in someone with pulmonary hypertrophy?

A P2 louder than A2 - The left-sided system is much higher pressure than right side, -> aortic valve usually louder than pulmonic valve - A P2 louder than A2 means pulmonary arterial pressure is significantly elevated

55 yo drinks methanol and then is given ethanol. To increase the Km of hepatic alcohol dehydrogenase for methanol in this patient the ethanol must act as what?

A competitive inhibitor - Methanol (alcohol dehydrogenase) -> formaldehyde (acetaldehyde dehydrogenase) -> formic acid (toxic metabolite) - toxicity: vomiting, gastric pain, dyspnea, bradycardia, permanent retinal damage, death - alcohol saturates alcohol dehydrogenase preventing conversion of methanol -> formic acid - the trick here is they say "to increase the Km" aka the amount of substrate needed to reach 1/2 vmax - this can only be done via competitive inhibition where the drug (alcohol) resembles the substrate and competes for binding sites

A patient in early stages of hemorrhagic shock is most likely to have what?

A weak pulse from decreased stroke volume - another answer was increased renal blood flow from sympathetic activation -> RAAS takes a little bit of time to compensate for drop in SVR

A woman is pregnant and bleeding out and brought in by her husband. Her pulse is 120, BP 90/60. Her closest OBGYN is 2 hours away, and she is muslim and refuses to be seen by a male. What do you do?

Ask the pt if she will allow examination if her husband is present at all times

63 yo with flank pain, tachycardia, hypotension while receiving a blood transfusion in ICU. Her urine is win-colored. What does she have?

ABO incompatibility - acute hemolytic transfusion reaction - type II hypersen rxn - intravascular hemolysis: hemoglobinuria (when ABO incompatibility) - or extravascular hemolysis: jaundice (host AB rxn against foreign AG on donor RBC) - both have fever, hypotension, tachypnea, tachycardia, flank pain - within 1 hour

16 yo has knife wound to left side of chest. X-ray shows air-fluid level in left side of chest, partial collapse of left lung, elevation of stomach bubble. Mediastinum is midline. What does he have?

AIR-FLUID LEVEL - hemopneumothroax, not under tension - there is both air and blood in the space, but the pressure is not pushing the diaphragm down, nor deviating the mediastinum away from injured side

following muscle twitches, ATP concentration does not fall appreciably. Why?

ATP is quickly regenerated by creatine phosphate

12 yo with 6 months of difficulty walking and leg pain. Atrophy of LE and hammer toes with a high stepping gait. What will a bc show?

Abnormal Myelin sheath - AD - pes cavus, hammer toe - CMT1A/PMP22

29 yo w/ pneumonia and positive test for cold agglutinins is given amoxicillin that doesn't cure his illness. Why?

Absence of peptidoglycan - mycobacteria pneumonia has sterols in cell wall and penicillins work by binding proteins (transpeptidases) and block cross-linking of peptidoglycan cell wall - this will not work on mycobacteria

30 mins after taking aspirin for a tension headache a 17 yo girl has difficulty breathing. What drug should she instead use to tc her headaches?

Acetominophen - sumatriptan used for cluster headaches and acute migraine not tension headaches - tension need NSAID used in acute treatment -> switch to central analgesic to avoid increased LOX from AA -> leukotrienes from blocking COX with every other NSAID - prophylaxis = amitriptyline, behavioral therapy

50 yo w/ abdominal pain -> antral ulcer. He also has knee pain. What drug can you use to treat his knee pain?

Acetominophen - inhibits COX 2 (icy spray on Coach cox 2) - acts as an antipyretic and analgesic (not anti-inflammatory) - good for mild-moderate pain in osteoarthritis/RA - not anti-inflammatory

35 yo has weakness and fever. When she was 25 she had Hodgkin disease tx w/ mechlorethamin, oncovin, procarbazine, and prednisone + splenectomy. She has no lymphadenopathy. Smear shows blast forms. What explains this?

Acute myelocytic leukemia - will see increased circulating blasts in peripheral smears - risk factor = prior exposure to alkylating agents, radiation, myeloproliferative disorders, down syndrome - t(15;17) responds to all-trans retinoic acid - DIC = common presentation - auer rods - procarbazine = treatment for Hodgkin lymphoma (cell cylce phase-nonspecific alkylating agent) -> causes bone marrow supression, pulmonary tox, leukemia, and disulfram reaction

38 yo after farm accident has high plasma fibrinogen what is causing this finding?

Acute-phase response - notably induced by IL-6 these are factors who's serum concentrations change significantly in response to inflammation - produced by liver in both acute and chronic inflammatory states - More FFiSH in the C - Ferritin: binds and sequesters iron to inhibit microbial iron scavenging - Fibrinogen: coagulation factor -> promotes endothelial repair, correlates w/ ESR - Serum amyloid A: prolonged elevation = amyloidosis - Hepcidin: decreased iron absorption (by degrading ferroportin) and decreasing iron release from macrophages -> anemia of chronic disease - C-reactive protein: opsonin -> fixes complement and facilitates phagocytosis. Measured as a non-specific sign of ongoing inflammation - Albumin (reduction conserves aa for positive reactants) and transferrin (internalized by macrophages to sequester iron) downregulated

In someone with IBD what monoclonal AB can you give them, and what is the target?

Adalimumab or infliximab - targets soluble TNF-alpha - used for IBD, RA, ankylosing spondylitis, psoriasis - TNF-alpha: activates endothelium, causes WBC recruitment, vascular leak - causes cachexia in malignancy, maintains granulomas in TB (always check pt for TB prior to AB initiation) - IL-1, IL-6, TNF-alpha = mediate fever and sepsis

40 yo from Kenya has 3 wk of diarrhea, disorientation, cachectic, poor memory, rash on upper face, neck and sun exposed areas. The deficient vitamin is part of a cofactor with which other component?

Adenine - Niacin (B3) is part of NAD+, NADP+ used in redox reactions - Nicotinamide adenine dinucleotide - from tryptophan - synthesis needs B2/B6 - used to lower levels of VLDL and raise HDL - has pellagra or Hartnups disease

65 yo w/ lesion at site of peripheral scar on lung. She had a pulmonary infarct 20 years ago and the lesion is newly present in the last 2 years. She has not smokes cigarettes for the last 20 years. What malignancy does she have?

Adenocarcinoma - chronic inflammation can lead to neoplastic transformation (think HCV -> HCC and h pylori -> chronic gastritis -> gastric adenocarcinoma) - on periphery - periphery and not related to active smoking = adenocarcinoma - more common at sites of previous injury

28 yo has pressure cuff placed around left arm, cuff is inflated to occlude blood flow for 2 minutes and then removed. Blood flow increases by 50% the next 3 mins, and returns to baseline. What humoral substance is responsible to this?

Adenosine - autoregulation = how blood flow remains constant over a wide range of perfusion pressures - hypoxia causes vasodilation (with the exception being the lungs = vasoconstriction) - adenosine increases blood flow - in heart: adenosine, NO, CO2, decreased O2 - Brain: CO2 -> vasodilation -> increased ICP - Kidney: tubuloglomerular FB - skeletal muscle: metabolites in exercise -> CO2, H+, Adenosine, lactate, K+ (at rest = sympathetic tone) - Skin: sympathetic tone most important for temperature control

Desmopressin activates what second messenger?

Adenyl cyclase - cAMP second messenger from Gs - FSH, LH, ACTH, TSH, CRH, hCG, ADH (V2-receptor), MSH, PTH, calcitonin, GHRH, glucagon, histamine (H2-receptor) - FLAT ChAMP - cGMP: BNP, ANP, EDRF (NO) -> BAD GraMPa - IP3: GnRH, Oxytocin, ADH (V1-receptor), TRH, Histamine (H1), Angio II, Gastrin (GOAT HAG) - Receptor tyrosine kinase: Insulin, IGF-1, FGF, PDGF, EGF (growth factors get found in the MAP kinase pathway) - JAK/STAT: Prolactin, immunomodulators (cytokines, IL-2, IL-6, IFN), GH, G-CSF, EPO, Thrombopeietin

35 yo w/ cocaine use daily comes to ER w/ 2 hrs of substernal chest pain, He used coke 6 hrs ago. Temp is 98.6, pulse is 110, RR 14, BP 160/100. What is next best step?

Admit pt to hospital for possible MI - cocaine can trigger prinzmental/vasospastic angina

In a study w/ 4000 people, 1000 smoke cigarettes and 3000 don't. In smokers and non-smokers, 30 were diagnosed w/ lung cancer. How many cases are attributable to smoking in a sample of 10,000 over the same time frame?

Asking about attributable risk - the difference of risk btw exposed and unexposed groups - can find RR and substract - or RR-1/RR X100 - here = 30/1000 = 0.03 - 30/3000 = 0.01 - 0.03-0.01 = 0.02 - 0.02 x 10,000 = 200

62 yo w/ muscle cramps and painful spasms in arms and legs. Past for months he can't open jars or use silverware. He has muscle weakness, exaggerated DTR. An appropriate tx for the cramps/spasms would be what?

Agonist of gamma-aminobutyric acid GABAb receptors in spinal cord - Baclofen - Treats muscle spasticity in MS (autoimmune demyelination of CNS), muscle spasticity, dystonia - acts on the BACK

25 yo has glucose of 100 after 20 hr fast. What precursor molecule is contributing to the normal glycemia?

Alanine - carries aa acid groups to the liver from muscle - 8-12hrs after fasting -> astored glycogen exhausted - gluneogenesis takes over around 12 hrs in liver (after 3/4 days also in kidney) - main precursors: pyruvate, alanine, glutamine, lactate, glycerol - after about 1 week of stravation = ketones (which will not maintain glucose levels in blood) - not acetoacetate/b-hydroxybutarate (ketone body) - valine will only be released once you start breaking down protein (far away from that)

26 yo brought to ER 3 hrs after taking 50 tablets of aspirin. She is nauseated, confused, sleepy. pulse is 130, RR 30, BP 100/60. What are her serum HCO3-, arterial PH, PCO2?

All decreased - Phase I: hyperventilation from respiratory center stimulation, ->respiratory alkalosis + compensatory alkaluria -> K+ and bicarb excreted in urine -> may last 12 hours - Phase II: paradoxic aciduria in presence of respiratory alkalosis when sufficient K+ is lost from kidneys. Begins in hours -> 12-24 hours - Phase III: dehydration, hypokalemia, progressive metabolic acidosis. Begins 4-6 hrs after ingestion in a young infant or 24 hours in adult

2 yo boy w/ Fanconi syndrome. What do you expect the fractional reabsorption of amino acids, glucose, phosphate, and bicarb to be?

All decreased - cause can be genetic, or from cisplatin, expired tetracyclines, tenofovir - generalized reabsorption defect in PCT -> increased excretion of amino acids, glucose, bicarb, and phosphate and decreased reabsorption - may lead to metabolic acidosis (proximal RTA) from decrease bicarb, hypophosphatemia, and osteopenia - form hereditary defects (wilsons disease, glycogen storage disease), ischemia, multiple myeloma, lead poisoning - in PCT: PTH hormone inhibits Na/Phos cotransport -> phosphate excretion, decrease reabsorption

In a woman who has a mass pressing on the trachea just above the carina, will the FVC, FEV1/FVC ratio, Peak expiratory flow rate, peak inspiratory flow rate be increased, normal, or decreased?

All decreased - constant airway limitation on inspiration and expiration - will appear almost as a perfect circle on PFT (both decrease in flow) - increased negative pressure space will not move mass, nor will pushing chest down creating positive pressure - this is a fixed intra-thoracic tumor - Variable extrathoracic: above sternal notch -> inspiring = vaccuum pulls air inward above sternal notch - drop in airway pressure narrows extrathoracic airway, increases resistance, which reduces flow - Ex) - Variable intrathoracic: inspiration flow limitation is worse in expiration from decrease in intrathoracic pressure causes the obstruction to cause greater obstruction -> increased block of flow - Ex) - fixed obstruction will effect both (Ex: post intubation tracheal stenosis/tumors) -> area wont change in inspiration or expiration - fixed extrathoracic: paralyzed vocal chord, laryngeal web, tracheal web, retropharyngeal absess, epiglottitis, aspiration - fixed intrathoracic: tracheal stenosis, tracheal ring, foreign body lower down, tumors - variable: pharyngeal malasia (soft larynx) - noise in inspiration (extrathoracic) obstructs in inspiration - expiration noise: expirational problems in bronchioles - decreased flow rate

Man with hypertension has thickening of LV wall. Will his transcription actor c-jun, b-myosin heavy chain, endothelin be increased or decreased?

All increased - increased B-myosin: hypertrophic cardiomyopathy (primary or from HTN chronic afterload) 45% of mutations are in b-myosin chain (disordered myosin fibrils) or cardiac myosin binding protein C (all AD) if sporatic its B-myosin gene - endothelin: two receptors A and B - A on pulmonary artery: major driving force to pulm HTN and limit NO -> more contrictors than dialotors. meds block endothelin at this receptor "sartan" "sentan" Bosentan for tx - also sildanifil for PD5 inhibitors and dilated vascular in pulm artery - prostocyclins also: epoprastonil - B receptor: CV disease and atherosclerosis and HTN increases plt binding to endothelial cells and associated with hypertrophic changes in chronic HTN - c-jun + fos = two TF dimerize and make active TF called AP1 -> regulate cell cycle progression specifically Cyclin D1 (G1->S transition) causes cell proliferation in all cells in body - c-jun suppresses P53 (to drive G1->S transition and also indirectly inhibits apoptosis) - c-jun most common overexpressions in a lot of cancers (non-small cell lung cancers, and estrogen driven breast cancers)

25 yo w/ 6 months irregular period and acne. Her BMI is 35, voice is deep, and dark hairs over lip and chin, open/closed comedones over face, chest, back. Serum studies will show what levels of fasting insulin, testosterone, and LH?

All three values will be high - Polycystic ovarian syndrome (Stein-Leventhal syndrome) - hyperinsulinemia/insulin resistance alters hypothalamic feedback -> increased LH/FSH, increased androgens (testosterone) from theca interna cells, and decreased rate of follicular maturation -> unruptured follicular cysts and anovulation - increased risk of endometrial cancer from unopposed estrogen from repeated anovulatory cycles - give OCP, clomiphene, metformin and spironolactone/ketoconazole (antiadrogens)

If someone is put on positive pressure ventillation w/ 10 cm H2O positive end expiratory pressure, what will there Peak inspiratory PA, end-tidal PA, Peak inspiratory Pip (intrapleural), and end-tidal ip be (+, -, or zero)?

All will be positive - normal breaths are negative pressure while intracellular space is always positive - in positive pressure ventilation, you are pushing air into lung, while chest pressure remains positive

Five nerves sit in the cavernous sinus. List the nerves from top to bottom along the wall and which sole nerve sits in closest proximity to the internal carotid artery?

Along wall from top to bottom: - CN III (occulomotor) - CN IV (trochlear) - CN V1 (ophthalmic) - CN V2 (maxillary) - CN VI (abducens -> closest to internal carotid) - cavernous sinus = collection of venous drainage, on either side of pituitary, drains blood from eye/superficial cortex -> internal jugular vein - Cavernous sinus syndrome: eye issues, decreased corneal sensation, horner syndrome, decreased maxillary sensation, from pit. mass affect/thrombosis -> CN V1 most susceptible to injury

What medication could be used for watery nasal decongestion during an allergy attack?

Alpha-adrenergic agonist (phenylephrine alpha 1 > alpha 2) in Sudafed and most OTC decongestants

What protein inclusion do you expect to see in parkinsons?

Alpha-synuclein - can have amyloid B, tau as well - alzheimers: amyloid or tau - Creutzfeldt-jakob: tau or prions - neurodegenerative disorder (reversible causes of this = hypothyroidism/vita B12/neurosyphilis) - MPTP a contaminant in illegal dugs is toxic to substantia nigra - lewy bodies are made of alpha-synuclein (intracellular eosinophilic inclusions)

25 yo and husband come to counseling for conception. They are both Vietnamese and previous screening shows a MCV of <80 um, and hemoglobin electrophoresis showed no abnormalities. What is the offspring at greatest risk for?

Alpha-thalassemia trait - alpha-globin gene deletions - cis deletion (on same chromosome) prevalent in Asian populations, trans (deletion on separate chrom) in African populations - normal is aa/aa - because their electrophoresis what normal they do not have HbH (one cis mutation, and one other missing --/a-) nor HbgBarts (non viable fetus (--/--) - They are both carriers (aa/--) as both have microcytic anemia - if one was aa/a- they would not show up as having anemia at all, and if one was a-/-- they would have an abnormal electrophoresis showing HbH - Their possible outcomes are: aa/aa, aa/--, aa/--, --/-- - the question asks the greatest risk = being a carrier aa/-- is the highest % chance

In a person with asbestos with hx of ferrugonous bodies, the interaction of these foreign bodies and what cells cause the fibrosis causing interstitial fibrosis?

Alveolar macrophages - in chronic inflammation -> prolonged infiltration of mononuclear cells (macrophages, lymphocytes, and plasma cells) -> tissue destruction and repair (angiogenesis/fibrosis) - stimuli like persistant infections (TB), autoimmunity, prolong exposure to foreign material (asbestos, silica) - macrophages = main cell -> chronic inflammation from their interaction w/ T cells (Th1 -> INF gamma -> macro activation/inflammation vs. Th2 -> IL-4/IL-13-> repair/antiinflammatory) - outcomes of chronic inflammation -> scarring, amyloidosis, neoplastic transformation

18 yo w/ anxiety, confusion, appears cachectic and older than his age. High pulse, normal respirations, diaphoresis, pupillary dilation, and muscle weakness. he gets charcoal in his stomach. What will be positive on toxicology?

Amphetamines - dilation, euphoria, wakefulness and attention, HTN, tachycardia, anorexia - Tx: benzodiazepine for agitation and seizures

47 w/ aplastic anemia treated for disseminated aspergillosis w/ antifungal that binds specific sterols in plasma membrane. What drug is this?

Amphotericin B - Binds ergosterol (unique to fungus) in cell membrane and disrupts membrane integrity (like nystatin) and forms pores -> leaks electrolytes - used for systemic mycosis (cryptococcus = Amph B + flucytosine) - supplement K+ and Mg+ bc altered renal tubule permeability - SE: fever/chills, hypotension, nephrotoxic, arrythmia, anemia - fungus has cell wall and cell membrane - cell wall drugs: echinocandins (caspoFUNGIN) block B-glucan in cells wall - cell membrane: amphotericin B, nystatin, terbinafine (blocks squalene epoxidase -> ergosterol), AZOLES (block 14-alpha-demethylase -> ergosterol)

What is the term that describes the HER2/neu receptor overexpression w/ greater than 10 receptors/cell?

Amplification

17 yo football player uniform soaked with sweat. The odor associated with the sweat is from secretion of what glands?

Apocrine - mixed sweat glands = apoeccrine glands in axillary and perianal areas - develop in puberty from eccrine precursors - Both eccrine and apocrine sweat glands innervated by SNS - eccrine glands = acetylcholine, for apocrine glands = catecholamines - Apocrine: axilla (armpits) and perianal areas. mediated by hormones/puberty, and provides nutrients for bacteria on skin - active in stress/sexual excitement - pheromones - Eccrine sweat glands: all over body, highest density in palms and soles, then on the head, but much less on the trunk and the extremities = water-based (and NA+/Cl) secretion for cooling in humans

A normal appearing 17 yo girl has never has menstrual period. Vagina ends blindly and no uterus or ovaries are palpated. Buccal smear shows chromatin-negative nuclei. What is diagnosis?

Androgen insensitivity syndrome - 46 XY, AR - sertoli still makes MIF -> no female organs, but no androgens -> no male organs, but do have crytochord testes (in abdomen/labia majora) - NOT Tuners syndrome - testes present but external genitalia are female/ambiguous -> testicular feminization - defect in androgen receptor -> normal appearing female w/ 46 XY - female external genitalia w/ scant axillary/pubic hair, rudimentary vagina, uterus/fallopian tubes absent - pt develop normal testes usually in labia majora and need to remove to prevent cancer - increased testosterone, estrogen (from aromatization of testosterone), LH but no body hair from no androgens - chromatin negative nuclei = no chromatin present -> no barr body present = haploid in respect to an X chromosome ( if you are a male you are chromatin negative, if you have XXY you are chromatin positive) meaning 1 fewer X chromosome - Turner's also has chromatin negative

Blood gas values: Arterial PO2 96 Arterial PCO2 40 Arterial O2 content 12.0 (N=20) Mixed venous PO2 36 Mixed venous O2 8.0 (N=15) What do they have?

Anemia Hb concentration = decreased % O2 saturation of Hb = normal Dissolved O2 (PaO2) = normal total O2 content = decreased - O2 content (1.34 x Hb x SaO2) + 0.003 xPaO2) - The only time the PaO2 will be decreased is in high altitudes

What is an anti-idiotypic AB and why cant it be used to treat Multiple myeloma?

Anti-idiotypic AB: binds AG hypervariable region of AB/ or AG binding site/or outside of binding region - can inhibit any AB from binding to its AG and bind the AG as well - rogam: is an example of this - ELISA are an example of this -> AB targeting a system - MM: makes IgG/ IgA and secretes these into the environment - these cells do not have these AB on their surfaces and hence why you cant bind this to them - Anti-allotopic (constant region) Ex) Rheumatoid Arthritis = the IgM that binds IgG Fc region

26 yo woman receives 1 unit of packed RBC and develops hypotension and hemoglobinuria. What is causing this?

Antibodies and complement C5-9 - this is acute hemolytic transfusion reaction = type II hypersensitivity -> intravascular hemolysis (ABO incompatibility) or extravascular (AB against foreign AG on donor RBC) - fever, hypotension, flank pain, hemoglobinuria from lysed RBC - within an hour of transfusion - other transfusion issues: allergic (type I HS rx) against plasma proteins in those with IgA deff, febrile nonhemolytic rxn (cytokines made in storage of blood products or a type II HS rxn w/ host AB directed against donor HLA -> rever, chills, flushing prevented by irradiating WBC, and transufsion acute lung injury (donor anti-leukocyte AB against recipient nutrophils/pulm endothelial cells -> ARDS in 6 hours)

5 yo w/ recurrent pancreatitis since 2. He has xanthomas, hepatosplenomegaly. Fasting plasma = turbid. total cholesterol = 235, TG = 1875. After receiving injection of heparin, lipoprotein lipase activity is in normal range. What deficiency does he have?

ApoC-II - they are saying that the lipoprotein lipase is normal - hence the issue must be in Apo CII which is a cofactor for lipoprotein lipase that catalyzes cleavage of chylomicron (CCC) - Type I hyper-chylomicronemia (AR) either a lipoprotein lipase or Apo CII deficiency - increased chylomicrons, TG, Chol in blood - leads to pancreatitis, hepatosplenomegaly, xanthomas, creamy layer in supernatant, no increased risk for atherosclerosis From Diet: - when chylomicron enters veinous blood from lymphatic of enterocytes it has Apo48 - HDL places Apo CII and ApoE onto chylomicron - Apo CII activates lipoprotein lipase to allow uptake of TG rich chylomicron into adipocytes - remaining chylomicron remnant -> liver via Apo E From Liver: - VLDL is released w/ Apo B-100 - HDL tags liver TG + Chol rich VLDL w/ Apo CII and Apo E - VLDL uptake by adipocytes via Apo CII - remnant IDL taken up by liver via Apo E - Liver also releases LDL w/ Apo B100 (more chol less TG) to blood for take-up by peripheral cells for lipid synthesis

A mother w/ toxoplasmosis has an asymptomatic female. What test will indicate that the newborn has congenital toxoplasma gondii?

Antitoxoplasma IgM antibody concentrations - IgG may be mother's - and can also do PCR

30 yo w/ Marfans had tearing chest pain, w/ hx of ECHO showing aortic root dilation. No longer took propranolol, pulse 120, BP 80/40, PE w/ JVD that does not decrease w/ inspiration. No heart sounds heard. What will her Right atrial pressure, PCWP, SVR, and cardiac index be?

Aortic dissection (of ascending aorta -> cardiac tamponade) - Cardiac index: how much blood is being pumped out/body surface area -> decreased as heart can't pump out anymore blood (can't fill properly with blood surrounding heart -> decrease in CO -Increased right atrial pressure (equilibrates with other chambers) making the pulmonary capillary wedge pressure the SAME (about 18) - markedly increased SVR as she is in obstructive shock (cold, clammy, very decreased CO) - equilibration of diastolic pressure in all four chambers as blood surrounds heart uniformly - Beck's triad: hypotension, distended neck veins, distant heart sounds - ECG = electrical alterans (from swinging of the heart in large effusion) - increased HR, pulsus paradoxus (decrease in amplitude of systolic BP by more than 10 in inspiration)

64 man dies while playing tennis he has no known cardiac risk factors and no CAD. Autopsy shows cardiac valve defect and concentric Left Ventricular Hypertrophy. What valve abnormalities was involved in sudden death?

Aortic stenosis

24 yo has a 3/6 systolic murmur. Cardiac catheterization shows pressure, % O2 saturation of the RA (6, 75), RV (30/5, 75), Pulm artery (30/20, 75), LA (10, 98), LV (180/10, 98), Aorta (110/70), 98) What does he have?

Aortic stenosis - The LV systolic BP is markedly higher than the decreased aortic pressures showing an increased pressure to get blood to flow across stenotic valve - normal cardiac pressures: - RA <5 - RV 25/5 - PA 25/10 - PCWP 4-12 - LA <12 - LV 130/10 - Aorta 130/90

Drug X and Drug Y have same mechanism of action, but drug X has greater bioavailability vs. Drug Y. What will be greater in drug X than Y if both are given at the same dose?

Area under the curve - definite integral in a plot of drug concentration in blood plasma vs. time - or the amount of drug in body aka bioavailability -the peaks and troughs of this graph = max concentration, followed by drug half life (ellimination)

A newborn male has a limb deformity. The woman's mother, sister, and husband are in the room. How d you deliver the news?

Ask mother and sister to leave room and inform the parents of the abnormality

50 yo man found dead, his family said he had chest pain for 3 weeks prior. Autopsy shows severe atherosclerosis of all three major coronary arteries w/ myocardial softening and mottling on anterolateral wall. Bx shows many neutrophils infiltrated. What caused his death?

Arrhythmia - gross: first day = dark mottling - occurs in first few days of MI (plus HF, cardiogenic shock) important cause of death before reaching the hospital in the first 24 hrs post-MI -> early coagulative necrosis, wavy fibers, neutrophils appear + reperfusion injury - in 1-3 days (coagulative necrosis, acute inflammation w/ neutrophils) = friction rub + fibrinous pericarditits - 2-7: posteromedial papillary rupture (single blood supply from PDA) -> severe mitral regurge - 3-5 days: macrophage-mediated degradation -> VSD (increased O2 sat and pressure in RV) - 3-14 days (macrophages and granulation tissue at margins weakens tissue): free wall rupture contained by scar tissue -> decreased CO and risk of arrhythmia/embolus - 5-14: free wall rupture -> cardiac temponade -> LV hypertrophy/prior MI protects against this - weeks: autoimmune phenomenon (Dresslers) = fibrinous pericarditis contracted scar complete - mottling 12-24 hrs - 24-74 hrs neutrophils - arrhythmia = biggest cause of death - at any point in time the greatest cause of death is ventricular arrhythmia -thats why you begin beta-blockers immediately following MI (for cardioprotective effects and to prevent arrhythmia)

in exercise what do you expect the following values to be: arteriolar diameter, tissue adenosine concentration, and vascular conductance?

Arteriole diameter: increased from vasodilation adenosine: increased vasodilation of smooth muscles conductance: increased as it is 1/resistance -> as diameter of blood vessel increases, conductance increases

64 yo has surgical repair of abdominal aortic aneurysm. In repair the testicular artery is ligated. Anastomotic supply from what artery maintains adequate blood supply to the left testis?

Artery of the ductus deferens - aka artery to vas deferens/deferential artery - contents of spermatic cord ("protection doesn't contribute to a good sex life" P: pampiniform plexus (drains to testicular vein/internal iliac v) D: ductus deferens (vas deferens) C: cremasteric artery T: testicular artery A: artery of the ductus deferens (vas deferens) G: genital branch of genitofemoral nerve S: sympathetic nerve fibers L: lymphatic vessels

A 28 yo has hypertriglyceridemia and is started on Nicotinic acid after not responding to gemfibrozil. What drug should she take to avoid the most common adverse reaction?

Aspirin - Niacin (B3) decreases LDL, increased HDL (most effective at increasing this), and slightly decreases TG - inhibits lipolysis (hormone-sensitive lipase) in adipose tissue and reduces hepatic VLDL synthesis - SE: Red flushed face which is decreased by NSAID or long term use of drug - from prostaglandin = mediator of vasodilation and inflammation -> decreased by NSAIDS (not by acetaminophen though as this is only centrally acting) - can also cause hyperglycemia/hyperuricemia - can also increase LFT and cause hepatotoxicity

65 yo w/ well-controlled DM had no abnormalities on labs 1 year ago. Today, she has pulse 82, BP 135/80, RR 18, Hb 11.2, Hct 33%, Urea nitrogen 30, Creatinine 2.1. Abdominal US shows decreased size of both kidneys and MR aortography shows b/l proximal renal artery stenosis. What is the diagnosis?

Atherosclerosis - renal impairment from renal artery stenosis -> decreased perfusion of one or both kidneys -> increases renin -> increases angio -> increases HTN - two main causes: 1. Atherosclerotic plaques: proximal 1/3 of renal artery, usually in older males, smokers 2. fibromuscular dysplasia: distal 2/3 of renal artery or segmental branches, usually young or middle aged females - causes atrophy of affected kidney - most common cause of secondary HTN in adults - other large vessels often involved

infant eats insecticide and is in respiratory distress, respirations are 18/min and shallow, 82% sat, copious oral secretions. after securing airway how do you treat?

Atropine - organophos binds acetylcholine estrase and prevents breakdown at the synaptic cleft - presents with too much parasympathetic symptoms - atropine is a reversible antagonist of muscarinic acetylcholine receptors

30 yo man with hemoptysis, dyspnea, weakness and hematuria. Bronchoscopy shows diffuse pulmonary hemorrhages. Renal Bx shows glomerular necrosis w/ crescents and linear deposition of IgG and C3. What mechanism is causing this disease?

Autoantibodies against host cell basement membranes - Goodpastures - type II hypersensitivity rxn - treat with plasmapheresis

what AB are indicative of a diagnosis of rheumatoid arthritis?

Autoantibody (IgM) reactive with IgG - Rheumatoid factor = IgM AB against IgG Fc region (in 80%) - anti-Cyclic Citrullinated Peptide more specific

In someone w/ restrictive cardiomyopathy and renal failure w/ hx of RA, renal Bx shows eosinophilic hyaline material w/ congo red stain showing birefringent pattern under polarized light. Why can this material stain this way?

B-pleated sheet structure - amyloid = abnormal aggregation of proteins (or fragments) into B-pleated linear sheets making them insoluble fibrils -> cellular damage and apoptosis - here as she has RA -> secondary amyloidosis w/ deposition of "AA" = serum amyloid A -> seen in chronic inflammatory conditions (RA< IBD, familial mediterraanean fever, infection) - leads to restrictive cardiomyopathy, arrythmia, hepatomegaly, nephrotic syndrome, neuropathy, carpal tunnel

78 yo fever, chills, fatigue, SOB, 12lb weight loss over past month. had urinary cath for prostatic obstruction, has a 101 temp, pulse 100, BP 160/80. Soft S1, normal S2. A 2./6 diastolic murmur best in second left IC space -> accentuated when patient leans forward and holds breath. What does he have

Bacterial endocarditis - murmur - fever - recent procesdure w/cath

Protein interferes with normal structure of desmosomes. What cell junctions in the skin are most likely to be affected by presence of this protein?

Basal keratinocytes and suprabasal keratinocytes - in the stratum spinosum - think like pephigus vulgaris: causes separation of basal layer and stratum spinosum right above basal karitinocytes -> gives tombstone appearance = suprabasal blisters - thin blisters - vs detachment of basal cells from basement membrane -> bullous pemphigoid

23 yo has hx of breast cancer, and read that gamma radiation is used to sterilize meat and is scared this will cause cancer. What do you tell her?

Because radiated food itself is not radioactive, you will not be exposed to ionizing radiation by consuming it - Irradiation kills bacteria that cause food poisoning (salmonella, campylobacter, E. Coli), preserves food and reduce food waste - food is exposed to electron beams, X-rays or gamma rays - Irradiated food has been exposed to radiation but does not become radioactive itself.

26 yo and 25 couple want genetic counseling prior to conception. both have microcytic anemia and increased HbA2. genetic analysis of the man = heterozygous null mutation of B Hb gene. Women is heterozygous for mutation that causes 50% decrease in B-globulin on one allele. What are the genetic outcomes for this pt?

Beta-thalassemia - dad = B+/B- - mom = B+/B50% - children: B+B+ (100% beta), B+B50% (75% beta), B+B- (50% beta), B50%B- (25% of beta) - 1/4 = 25% b-globin function and may require occasional transfusions - can have B-thalassemia minor (heterozygote): underproduction of beta chain, usually assymptomatic. dx by increased HbA2 on electrophoresis (>3.5%) - B-thal major: homozygote, no beta chain at all -> severe microcytic anemia w/ target cells and need transfusions (secondary hemochromatosis) - marrow expansion, crew cut skull on x-ray, chipmunk facies, extramedullary hematopoiesis -> hepatosplenomegaly - increased risk fo parvovirus aplastic crisis - increased HbF (alpha/gamma) and HbA2 - HbF is protective in infancy

69 yo male with BPH has urinary retention 1 day after removal of a melanoma. A drug is given that increases phosphoinositide turnover in smooth muscle cells of bladder fundus but has no effect on nicotinic synapses. what is the drug?

Bethanechol - direct cholinergic/muscarinic agonist - activates bladder smooth muscle - no nicotinic activation

Someone in ESRD missed dialysis and is coming in confused. He has bilateral crackles on auscultation. Serum studies will show a decrease in what?

Bicarbonate - renal failure -> metabolic acidosis - hyperkalemia - dyslipidemia (TG) - uremia (confusion here) - Na+/H2O retention - EPO failure/anemia - renal osteodystrophy (hypocalcemia, hyperphos

In translation, when the 60s ribosome has a tRNA loaded w/ aa in the P position, and E and A positions are empty what is the next step?

Binding of aminoacyl-tRNA to the A site - CCA sequence 3' end of the tRNA (Can Carry amino acid) - T (w/ ribothymidine, pseudouridine, cytidine) arm tethers the tRNA to ribosome - D arm has dihydrouridine for tRNA recognition by aminoacyl tRNA synthase that then analyzes the exact aa for the unique tRNA - eLFS recognize 5' cap of MRNA and assemble the correct tRNA for initiation - elongation: aminoacyl-tRNA binds A site (except for initiator MET code) and rRNA makes peptide bond to grow peptide in A site - ribosome advances and moves peptidyl tRNA to P site - eukaryotic release factors recognize stop codon and stop translation -> released A: incoming aminoacyl-tRNA P: for growing Peptide E: holds empty tRNA as it Exits

Why does a woman's oxytocin concentrations increase in labor?

Birth canal reflex - oxytocin causes uterine contractions during labor and after to expel placenta and causes vessel contractions to stop bleeding, and milk letdown w/ suckling - analogs used to induce labor, strengthen contractions, and control postpartum hemorrhage - IP3 signaling pathway (contractions) - carried by neurophysins down axons to posterior pituitary - birth canal stretch -> stretch receptors release oxytocin from posterior pituitary - and mechanoreceptors in nipples from suckling

48 yo w/ urinary frequency and urgency. Medication to help her will act how?

Blockade of parasympathetic innervation of the detrusor muscle - oxybutynin or tolterodine(M3 muscarinic antagonist) - treat incontinence/bladder spasms after urological surgery or urge incontinence (overactive bladder = detrusor overactivity -> leak w/ urge to void immediately) - associated w/ UTI

What role does Nicotinic acid play in lipid metabolism?

Blocks releases VLD from hepatocytes - nicotinic acid = niacin - reduces the amount of triglycerides/VLDLs made from the liver - B3 - big decrease in LDL, Big increase in HDL, decrease in TG - inhibits lipolysis (hormone-sensitive lipase) in adipose tissue - reduces hepatic VLDL synthesis - SE: red, flushed face (decreased by NSAID/long term use), hyperglycemia, hyperuricemia - Niacin deficiency -> pellagra (diarrhea, dermatitis, dementia, death)

In someone with sleep apnea, what will decrease after tx?

Blood pressure - pulmonary HTN from hypoxemia from decreased breathing in sleep causes increase BP - decrease once hypoxia is relieved

30 yo migrant farm worker presents with fever, chills, headache. temp is 104, pulse is 101, RR 20, BP 105/62, supply neck. Some blood and urobilinogen in blood. Blood smear shows RBC with red granules. What is unique about this organism?

Blood smear of trophozoite ring form in RBC + schizont containing merozoites + red granules (schuffner stippling = eosinophilic stippling in RBC) seen in p vivax/ovale - these uniquely form hypnozoites in liver - need tx with chloroquine + primaquin to kill hypnozoites in liver (but test for G6PD) - have tertian fever - vs. malariae: quartan fever and falciparum = irregular fever pattern, neuro, renal, brain occlusion from parasitic RBC (are banana shaped) - all from anopheles mosquito

Group X is given isoflurane at 1.5X the minimal alveolar concentration. Group Y is given isoflurane and nitrous oxide at 0.75X the MAC for each. Group Y has a faster onset of anesthesia. This is due to what differentiating factor btw the two drugs?

Blood:gas partition coefficient - N2O has a lower blood:gas PC (0.47) meaning that less of the drug is dissolved in the blood at equilibrium allowing for a quicker onset of action, a quicker recovery, and lower potency (potency = w/MAC) - Isoflurane has a higher Bloos:gas coefficient (1.4) meaning that 1.4X as much of the drug is dissolved in the blood, more soluble in lipid/blood, high potency and slow induction/recovery.

33 yo has radical thyroidectomy and has vessels on left side of his neck ligated. Postoperative calcium and PTH is low. What vessels were damaged?

Branch of the thyrocervical trunk - the blood supply to the parathyroid glands is from the inferior thyroid artery -thyroid is from superior thyroid artery off of external carotid (ligating risk = external laryngeal nerve_. crycothyroid) - also inferior off of thyrocervical (injury to recurrent laryngeal -> dysphonia/dysphagia) - and both inferior thyroid veins drain into left brachiocephalic vein - also have thyroid ima artery off of common carotid

18 yo previously healthy male who was hiding in closet from intruders "from the other place," no hx of drugs/alcohol, afraid of doctor, heard male voices for past 2 wks, no abnormalities on labs. What does he have?

Brief psychotic disorder - more than one positive symptom (hallucinations, delusions, unusual thought processes, disorganized speech, bizarre behavior) lasting less than 1 month - usually stress related

62 yo smoker w/ 3 year hx of cough and purulent sputum for 2 weeks. She has swelling of metatarsals, metacarpals, and proximal phalanges. X-ray of the bone shows new bone formation and arthritis of the joints and clubbing of the digits/ What is the underlying disease?

Bronchogenic carcinoma - adenocarcinoma, most common primary lung cancer - more common in women than men can arise in nonsmokers - Activating mutations in KRAS, EGFR, ALK - Paraneoplastic syndrome: hypertrophic osteoarthropathy = abnormal proliferation of skin and bone at distal extremities -> clubbing, arthralgia, joint effusions, periostosis of tubular bones - stains + for mucin

a 3 lb newborn baby is born at 28 weeks. He has severe repiratory sdistress and is given intubation with 100% O2 w/ PEEP. One month later a CXR = persistant increased densities in all lung fields. He gets pneumonia and dies. At autopsy lungs are firm and poorly aerated. Bx: interstitial fibrosis surrounding irregular, dilated airspaces w/ bronchiolar metaplasia. What is the dx?

Bronchopulomonary dysplasia - in intubation and reoxygenation you can have free radical damage of cells via membrane lipid peroxidation, protein modification, DNA breaks - complications: retinopathy of prematurity (abnormal vascularization), Bronchopulomonary dysplasia , reperfusion injruy after thrombolytic therapy - more common in infants with low birth weight - follows treatment of respiratory distress syndrome - also worry about intraventricular hemorrhage from poorly matured germinal matrix

2 yo boy has had 2 episodes of strep pneumo bactermia and multiple ear and sinus infections since birth. He has no tonsillar tissue what immunodeficiency syndrome does he have?

Bruton agammaglobulinemia - X-linked recessive (more in boys) - defect BTK a tyrosine kinase gene -> no B-cell maturation - recurrent bacterial and enteroviral infections after 6 months (no more maternal IgG) - absent B cells in periphery, decreased Ig of ALL CLASSES - ABSENT/scant lymph nodes and tonsils (no primary follicles and germinal centers) - Do not give live vaccines

32 comes in w/ pain and swelling of right cheek. She has swollen/tender area overlying the parotid gland. Sialolithiasis is suspected. The calculus is in a duct that passes through what muscle prior to entering oral cavity?

Buccinator - the parotid duct arises from anterior border of parotid - runs over the masseter muscle below the zygomatic arch - it then pierces through the buccal fat pad and buccinator muscle - opens in the second upper molar region - sialolithiasis: stones in the salivary gland (parotid, submandibular, sublingual) - from dehydration/trauma - tx w/ NSAIDS, gland massage, warm compress, sour candies (promotes salivary flow)

83 yo w/ 3 days of painful blistering on torso. He has numerous 0.5-1cm, clear-fluid-filled, tense blisters. Hx show subepidermal blisters. Production of autoantibodies directed against which structures?

Bullous pemphigoid Antigen - Less severe than pemphigus vulgaris - Type II rxn - IgG AB against hemidesmosomes (BULLow the epidermis) connect keratin in basal cell to BM. - tense blisters, no oral mucosa - IF: linear pattern at epidermal-dermal junction - Negative Nikolsky sign (Skin will not slough with light pressure)

6 month girl. drooping smile, left sided facial weakness, and decreased hearing in left ear. CT shows stenosis of internal acoustic meatus on left. What cranial nerves is abnormal?

Facial and vestibular as they both traverse the internal acoustic meatus as they leave the skull

18 yo injured his hand. tried to tag a player in flag football and snagged the tip of his ring finger on a players jersey. He felt a pop and pain. He has pain on palmer tip of finger, he cannot flex distal interphalangeal joint of right ring finger, but he can flex MCP and PIP joints. He injured a tendon whose muscle is supplied by fibers from what nerve roots?

C8 - Flexor digitorum profundus lies deep to superficialis, but it attaches more distally and tendons go through tendons of superficialis, and end up attaching to distal phalanx for flexion of DIP - innervated by ulnar nerve C8, T1

What is normal urine ph?

about 6.0 - will be alkaline in struvite stones (about 7.3) from urease producing organisms (proteus, staph saprophyticus, klebsiella) - urea -> ammonia -> increases Ph - all stones radiopaque except uric acid (radio lUcent)

22 yo w/ frequent episodes of cutaneous urticaria and occasional laryngospasm over the past year. An allergy evaluation fails to ID cause. What is deficient in his serum?

C1 esterase inhibitor (binds C1r, C1s) - causes hereditary angioedema from unregulated activation of kallikrein -> increases bradykinin - decreased C4 levels -ACE inhibitors contraindicated as they also increase bradykinin

20 yo woman w/ low grade fever, fatigue, joint pain for a year. A week ago these got worse at the beach and got rash on areas not even in sun. temp is 101. PE shows malar/trunk rash, oral ulcers, swollen lymph nodes. ANA AB 1:200. Gene encoding what protein is mutated?

C1q - SLE: autoimmune disease from type II and III rxns - associated w/ deficiency of early complement proteins (C1q, C4, C2) -> decreased clearance of immune complexes

30 yo given penicillin and gets serum sickness. What mediator stimulates release of histamine from mast cells?

C3a -> histamine release, hypotension and edema - C3a, C4a, C5a = Anaphylaxis - C5A = neutrophils chemotaxis - C3b = opsonization (binds lipopolysaccharides on bacteria) - mast cells activated by tissue trauma, C3a, C5a, surface IgE cross-linking by AG - prevented by cromolyn sodium - IgE independent mast cell degranulation from vancomycin, opioids, and radiocontrast dye

35 yo has pain in right arm, tingling in fingers on right hand. Weakness in extension and pronation of forearm. The triceps muscle stretch reflex is decreased. What nerve is injured?

C7 - triceps reflex = C6, C7 - but C7 = elbow extension, wrist flexion

21 yo w/ 10 day hx of difficulty walking. 2 years ago has vision loss in left eye that improved over months. Neuro exam shows decreased visual acuity in left eye/pallor of optic disc. She has broad based gait + lesions in white matter on cerebellum on MRI. What describes the pathogenesis of this disease?

CD4+ T lymphocytes are activated by myelin basic protein - She has multiples sclerosis - autoimmune inflammation/demyelination of CNS (brain/spinal cord) w/ axonal damage - optic neuritis (vision loss + marcus gunn pupil) - brain stem/cerebellar symptoms (diplopia, ataxia) - pyramidal tract demyelination (weakness/spasticity) - spinal cord syndromes (shock sensation, neurogenic bladder) - increased symptoms with increased body temp (exercise/hot bath) - women>men living far from equator - increased IgG and myelin basic protein in CSF - oligoclonal bands = diagnostic, and MRI is gold standard - periventricular plaques (oligodendrocyte loss) - white lesions disseminated in time and space - can give interferon B, glatiramer, natalizumab - acute flares = IV steroids - neurogenic bladder = cath/muscarinic antagonist - spasticity = baclofen (Gaba receptor agonist) - pain = TCA, anticonvulsants

person w/ HIV has mycobacterium avium w/ enlarged intra-abdominal lymph node on CT. Temp is 102, pulse 104, BP 11/84, and further enlargement of lymph nodes noted. What cells improved their function cause these findings?

CD4+ T-cells - Th1 cells secrete IFN-gamma and IL-2 to activate more macrophages and cytotoxin T-cells to kill phagocytosed microbes -> form granuloma via macrophages secreting cytokine (TNF-alpha, test for TB prior to starting anti-tnf) - inhibited by IL-4, IL-10 from Th2 cells - deficiency = increased mycobacterial diseases - can also have IL-12 receptor deficiency -> decrease Th1 response (AR), -> disseminated mycobacterial and fungal infections -> decreased INF-gamma - Because this person has HIV, they inherently have a low CD4+ count (as the CD8+ are killing viral infected cells), this is giving him a high susceptibility to mycobacterial infections -> no CD4+ = no activated macrophages - treat person w/ prophylactiv azithromycin/clarithromycin when CD4+ <50 to prevent mycobacterium

What B/T lymphocyte interaction allows for class switching?

CD40 (B)-CD40 ligand (helper T cells) - defective in hyper IgM syndrome - defect in CD40L on Th cells -> class switching defect - X-linked recessive - severe pyogenic (pus) infections early in life - opportunistic infection w/ pneumocystis, cryptosporidium, CMV - normal or increased IgM, decreased IgG, IgA, IgE - failure to make germinal centers

What cells are responsible for a low CD4+ T-cell count in HIV?

CD8+ T-lymphocyte-mediated killing - directly kill virus infected cells

A doctor wants to give a drug over 24 hrs (continuous infusion) to maintain a steady state drug concentration (Css) in plasma. to maintain a Css the appropriate rate of infusion is determined by what calculation (CL = drug clearance, Vd = volume of distribution, t1/2 = half life)

CL x Css - a drug infused at constant rate ned 4/5 half lives to reach steady state - clearance = volume of plasma cleared of drug/ unit time -> may be impaired in renal, liver heart failure - Maintenance dose = target plasma concentration at steady state x CL X dosage interval time / bioavailability - because this is a continuous infusion = no need to calculate dosage time - because this is IV F = 100% absorbed -> maintenance dose = CL x Desired steady state concentration = amount of drug that must be given

investigator studying pt with west nile. Over 5 years data is collected on 25 pt w/ west nile (demographic information on patients reported -> age, gender, ethnicity) and info on source of infection. What kind of study is this?

Case series - generally small descriptive studies, tracking pts derived by a health care setting (registered cases) with known exposure or tx and examine outcome - describes characteristics or outcomes in a particular group of people, but cannot determine how they compare w/ people who are treated differently or who do not have condition

66 yo has 2 day fever, flank pain, pain w/ urination, and nausea. 10 days ago she was admitted to hospital for similar symptoms, dx w/ acute pyelonephritis. She was discharged home on ciprofloxacin after a 3 day course of IV cipro started to improve symptoms. She also has HTN, HLD, and osteoporosis. She is taking alendronate, calcium carbonate, ezetimibe, hydrochlorothiazide, and simvastatin. Temp 102, BP 130/80, what drug interaction is occurring with the AB?

Calcium carbonate - divalent/trivalent cations (calcium, magnesium) decrease absorption of flouroquinolones and tetracyclines - don't take with milk, calcium, mg, antacids - "floxacin" - inhibit prokaryotic topoisomerase II (DNA gyrase) - treat gram - rods of urinary and GI tracts (including pseudomonas), same gram +, otitis externa - hurt tendons, bones and myalgia, teratogen, don't give to those <18 due to damage to cartilage - tendonitis/tendon rupture in those >60 - ciprofloxacin inhibits cyp450

70 yo woman w/ fever despite broad spectrum AB after surgery on pelvic organs for carcinoma. She has a central venous cath, well healing surgical site. Samples from central cath grow on sheep agar, gram stain shows 4um elliptical purple budding organisms. What is the organism?

Candida albicans - On sheeps agar will look like white, creamy mounds with "feet" or clear circumferential surrounding of clear growth - can cause disseminated candidiasis in neutropenic patients

If a compound is taken up by bacterial cells, no energy is required, and the compound is not concentrated in the cells what kind of transport is this?

Carrier-mediated diffusion - transport divided between active and passive transport: 1. Active: requires energy (ATP) to move against concentration gradient 2. Passive: - Simple diffusion: flows from high->low until concentrated in cell - channel mediated: specific pore for unique ion -> unique ion will flow in until concentrated or equilibrated - carrier mediated: need carrier that can transport in but will not freely flow can saturate carrier

35 yo disoriented and hallucinating and is alcoholic. He has seizure, and BP is 180/100. Serum K+ is 2.5 and urine K+ is 40. Alcohol withdrawal is suspected. Why is he hypokalemic?

Catecholamine-mediated intracellular shifts of K+ - He has delirium tremens - B1 causes potassium to be shifted into cells - alcohol withdrawal = release catecholamines

What specific part of the arachidonic acid pathway is prevented by aspirin?

Cell membrane phospholipids -> arachidonic acid (aspirin blocks this step) -> Prostaglandin G2 -> prostaglandin H2 etc etc

16 yo w/ pain and tingling in her left hand when she is backpacking. Symptoms resolve 5 minutes after she takes off her backpack. What abnormality causes this?

Cervical rib - thoracic outlet syndrome - compression of lower trunk and subclavian vessels, most commonly in the scalene triangle (space btw collar bone and first rib) - from a cervical rib, pancoast tumor - muscle defect (presents similar to Klumpke palsy) in intrinsic hand muscles, lumbricals, interossei, thenar, hypothenar - atrophy of instrinsic hand muscles, ischemia, pain and edema due to vascular compression - subclavian artery and brachial plexus exit btw the middle/anterior scalene together, just over the first rib and below the clavicle - subclavian vein returns in front of anterior scalene between rib and clavicle - increase pressure if pt has cervical rib (extra rib coming off of C7)

What is the natural ligand for the CCR5-receptor that HIV has tropism for?

Chemokines (chemokine receptor) - HIV binds CCR5 in early infection - CXCR4 in late infection - gp 120 allows entry by binding to CCR5/CXCR4 - miravoroc binds CCR5 and blocks entry - gp41 then -> fusion that is inhibited by enfuvirtide - both surface proteins come from env gene -> mutations in env gene = resistance to miravoroc and enfuvirtide

A study is assessing HTN in caucasian people vs. AA. 500 white and 500 AA and BP taken over 3 months. the prevalence in white = 10%, the prevalence in AA = 16%. What statistical test can you use to determine if the difference is statistical?

Chi-square test - checks differences between 2+ percentages or protportions of categorical outcomes (not mean values) - ex) comparing % of members of 3 diff ethnic groups w/ essential HTN - if you were comparing the mean HTN of the two groups = t-test - mean of more than 2 groups (3+) = ANOVA

25 yo man returned from peace corps work in Africa and begins oral chloroquine for p vivax. He had a good response, but recurs 2 months later why?

Chloroquine is ineffective on the exoerythrocytic malarial tissue stages - Vivax/ovale: 48 hr cycle w/ dormant form (hypnozoite) in liver - because it was initially receptive to chloroquine -> not resistant tot he drug, but rather had parasite in liver still that needed to be killed via primaquine

25 yo wants prenatal diagnosis at 12 weeks gestation. Her karyotype is 45, XX, t(14q21q) what prenatal test is now most appropriate?

Chorionic villi sampling - used to detect birth defects, genetic diseases - small sample of cells (chorionic villi) taken from placenta where it attaches to wall of uterus - taken between 11-13 weeks to look at fetal cells (amniocentesis done at 15 weeks -> may be more difficult to have abortion and takes sample of placenta which will have shed cells of baby)

Pt w/ HCV has a liver bx w/ lymphocytic infiltrations and collagen deposition. What inflammatory process is this?

Chronic inflammation - infiltration of mononuclear cells (macrophages, lymphocytes, plasma cells) - tissue destruction and repair w/ angiogenesis and fibrosis (collagen deposition) - from persistant infections HCV, TB, type IV autoimmune disease - macrophage are dominant cell - scarring, and possibly leads to HCC)

71 yo w/ 6 mo hx of calf pain and discomfort, can't walk more than 3 blocks. Has HTN and DM. Weak dorsalis pedis pulse bilaterally. What is a drug that will improve the pt symptoms and walking distance by altering platelet function and providing direct arterial vasodilation?

Cilostazol, dipyridamole - antiplatelet phosphodiesterase inhibitors - increases cAMP in platelets -> inhibits platelet aggregation and are vasodilators - good for intermitted claudication and vasodilation/preventing strokes or TIA when combined with aspirin - can create coronary steal ( dilating all coronary arteries decreases blood flow to ischemic areas may worsen ischemia) - dipyridamole used in coronary testing

70 yo woman unresponsive, has GAD taking diazepam and also is taking OTC heartburn meds. What interaction caused this?

Cimetidine - is a potent inhibitor of CYP450 - Diazepam (metabolized in liver)

49 yo overdose after 12hr from ferrous sulfate. Serum sodium and potassium are normal. What are his Cl- and HCO3-?

Cl- 96, HCO3- 8 - Iron poisoning: - Acute: high mortality rate from formation of free radicals and peroxidation of membrane lipids -> abdominal pain, vomiting, GI bleed, radioopaque pill seen in xray -> anion gap acidosis and multiorgan failure -> scarring with GI obstruction - give chelation = deferoxamine, deferasirox and gastric levage - chronic: from hemochromatosis, or chronic transufsions (thalessemia) -> arthropathy, cirrhosis, cardiomyopathy, DM, hypogonadism -> treat w/ phlebotomy

What can you give a 27 yo w/ irregular periods, and no pelvic abnormalities, and normal hormone levels?

Clomiphene - antagonist at estrogen receptor in hypothalamus -> prevents normal feedback inhibition - and increases release of LH and FSH from pituitary -> ovulation

8 yo w/ ventricular systolic overload. ECG shows severe left axis deviation of the main QRS vector (upward in lead I, down in lead II). What explains this?

Coarctation of the aorta - aortic narrowing near inertion of ductus arteriosus (juxtaductal) - HTN in UE and weak, delayed pulse in LW (brachial-femoral delay) - w/ age, intercostal arteries enlarge due to collateral circulation -> arteries erodes ribs -> notched appearance on CXR - May lead to HF, increase risk of cerebral hemorrhage (berry aneurysm), aortic rupture, and endocarditis "left axis deviation" = electrical axis pulled to the left from left ventricular hypertrophy from coartation and systolic overload and increased afterload for prolonged time increased SVR

52 yo has chronic pancreatitis. Deficiency in what enzyme is most likely preventing him from digesting triglycerides?

Colipase - trypsin/chymotrypsin = digest proteins -> polypeptides/dipeptides - carboxypolypeptidase = peptides into individual amino acids - elastases/nucleases - amylase = digestion of carbohydrates (glycogen/startch) -> di/trisaccharides - lipase = break down fats -> FA/monoglycerides - phospholipases A1/A2 = split FA from phospholipids - esterase = hydrolyzes cholesterol esters - enterokinase cleaves trypsinogen -> trypsin (in duodenum) to activate these enzymes

43 yo w/ 6 months altered consciousness. In interview she stops talking, turns head to right, extends and stiffens upper extremity, has blank look, and does not respond to questions. She then has repetative lip smaking and picking movements of hands -> 30 seconds. She slowly returns to normal in 4/5 mins. What kind fo seizure is this?

Complex partial - focal seizure - loss of consciousness = complex - usually has automatisms (involuntary actions)

24 yo in coma, intubated. He receives a tidal volume of 500 mL, and pt end expiratory pressure = +5, end inspiratory pressure = +25 an esophageal balloon inserted and end inspiratory pleural pressure = +20. What is the respiratory system's compliance?

Compliance = change in lung pressure over change in pressure (inversely proportional to wall stiffness) - here total volume = 500 mL - change in pressure = 25-5 = 20 - 500 mL/20 = 25 mL/cm

If a diabetic states "i know insulin would help control my blood sugar, but a lot of people in my family have DM and insulin made them sick at times." what stage of change are they at?

Contemplation as he is acknowledging problem, but unwilling to change - precontemplation: denying problem - preparation/determination: preparing for behavioral changes - action/willpower: changing behaviors - maintenance: maintaining changes - relapse: returning to old behavoirs and abandoning changes

Newborn w/ petechial rash, microcephaly, hepatosplenomegaly has CMV IgG, as does mother and IgM for CMV. What does this mean?

Congenital cytomegalovirus - member of the herpes family - HHV-5 - congenital transfusion, sexual contact, saliva, urine, transplant - can cause mononucleosis (- monospot test) - pneumonia in tx pt - esophagitis - in AIDS worry about retinitis (hemorrhage, cotton-wool exudates, vision loss) - latent in mononuclear cells (inclusions)

full-term newborn w/ respiratory distress. PE = cyanosis, decreased breath sounds b/l and bowel sound over hemithorax. Xray shows multiple cystic areas on left and decreased aeration on right. What is the diagnosis?

Congenital diaphragmatic hernia - usually have a left sided pulmonary hypoplasia (poorly developed bronchial tree w/ abnormal histology as the left is usually where the hernia occurs - hear bowel sounds as the gut is either fully in the thoracic cavity or blebing through a thin membrane

52 yo woman with breast cancer. Has 4-weeks of radiation 6 months ago. Her respirations are 26. PE shows no cancer recurrence. CT scan of chest shows b/l atelectasis in upper lung. What pathological process caused this?

Contraction - atelectasis = alveolar collapse which can be from four different mechanisms: 1. obstruction: of airway prevents new air from reaching airways -> old air reabsorbed and lung collapses (foreign body, mucous plug, tumor) 2. compressive: something pressing on lung squishing it down (space-occupying lesion, pleural effusion 3. contraction: scarring of lung parenchyma that distorts alveoli (sarcoidosis, radiation fibrosis, necrotizing pneumonia) 4: adhesive: lack of surfactant (neonatal respiratory distress syndrome in premies) - atelectasis happens in intubation: not intubation that causes this, but rather the procedure itself, as they have decreased tidal volume that causes collapses small alveoli - after intubation it is from misplaced tube or from mucus plugging (increase secretions which should be suctioned) same reason you get it in COPD

12 yo can't walk for 5 days, she is unconcerned about this issue. Exam is totally normal with normal reflexes, and variable sesation to light touch/pain. What is dx?

Conversion disorder - functional neurologic symptom disorder - loss of sensory or motor function (paralysis, blindness, mute) after acute stressor - patient aware but indifferent to symptoms (la belle indifference) - more common in females, adolescents, and young adults

68 yo w/ 10 months SOB and swelling of feet. He has fam hx of CV disease. He smoked 2 packs of cigs daily for 50 years. Pulse is 80, RR 24, BP 150/80, PE shows edema + diffuse scattered wheezes b/l, 2/6 pansystolic murmur on lower left sternal border that increases with inspiration. PMI is in sub-xiphoid area, distant S1/S2. Liver is enlarged. What is the diagnosis?

Cor pulmonale - prolonged hypoxemic state from COPD -> vasconstriction of pulm arterioles -> secondary pulmonary HTN -> right sided heart failure and back up of system - secondary from cor pulmonale, right sided heart failure - primary is from genetic mutation inactivating BMPR2 (AD, middle aged women)

A virus isolated from a 4 yo girl with fever, cough. Experiments shows that the virus forms plaques on human laryngeal cells but rapidly loses its ability to form plaques when dried or exposed to pH 5.0. What virus is this?

Coronavirus - Enveloped SS + RNA virus - common cold, SARS (asia), MERS - helical virus - infects human laryngeal cells - need respiratory secretions and can only replicate in respiratory epithelium - coxsackie can live in heart, hand, foot unlike this - EBV can effect only B cells in mucosa in oral but not laryngeal cells - this is the only virus that can replicate in laryngeal cells

27 yo 3 week hx of left sided chest pain/tenderness. pain radiates to back/exacerbated when reaching overhead. BP 130/86, no abnormal PE findings. tenderness to palpation 2cm lateral to midline and 10cm below clavicle on left. No xray abnormalities. What is the diagnosis?

Costochondritis - inflammation of where upper ribs join with cartilage that holds them to the sternum. - localized chest pain you can reproduce by pushing on cartilage in front of ribcage

Newborn baby with machine-like murmur along sternal border, increased precordial activity, and bounding peripheral pulses. What enzyme does the treatment block?

Cyclooxygenase - treat with indomethacin (NSAID)

If a virus can be killed by heating to 60 degrees C for 30 minutes, the virus most likely has what characteristic?

it is enveloped - enclosed in a lipid bilayer membrane and is sensitive to heat - non-enveloped virus's have no membrane layer and are not sensitive to heat

How do you treat strongyloides?

ivermectin or bendazoles

Where is the SA node located?

junction of superior vena cava and right atrium

38 yo w/ 3 sons w/ sickle cell and no support from her family. She used to feel angry and abondoned, but now takes kickboxing and feels happier. What coping mechanism is this?

Displacement - redirection of emotions or impulses to a neutral person or object - yelling at dog instead of yelling at husband who you're angry at - sounds more like sublimation (aggression taken out in a different channel = mature defense mech)

What are side effects of Metoclopramide?

D2 receptor antagonist, increases resting ton, contractility, LES tone, motility, promotes gastric emptying - used for diabetic and post-op gastroparesis, antiemetic, and persistant GERD - SE: parkinsonian effects, tardive dyskinesia, restlessness, drowsiness, fatigue - GALACTORRHEA and hyperprolactinemia via dopa blockade - increase release of PL from adenohypophysis

A 2-year-old w/ severe pain, swelling, redness of thumb. no trauma. eating poorly, of temperature is 37.7°C (99.8°F). PE shows an oral vesicle, cervical lymphadenopathy, and the findings in the photograph. Which of the following types of infectious agents is the most likely cause of the findings in this patient's finger?

DNA Virus - Oral vesicle + blistering vesicular lesion on hand - No fever, not toxic-appearing = Herpes (herpetic whitlow) - Most get HSV1 as children, not all are symptomatic. HSV is a large double-stranded, linear DNA virus - cant treat adults with acyclovir/valcyclovir

In a child with neonatal respiratory distress syndrome what do you expect their functional residual capacity to be?

Decreased - Functional residual capacity = volume of gas in lungs after normal inspiration (RV + Expiratory reserve volume) - decreased surfactant -> increases surface tension -> alveolar collapse (air rushes out of alveoli on expiration) - ground glass appearance on x-ray - risks: prematurity, maternal DM, c-section (no stress = no steroids) - test for by lecithin:sphingomyelin ratio (more than 2 is healthy)

Following an orchiectomy for prostate cancer, what cellular changes will be increased in the prostate?

DNA fragmentation - atrophy of the gland due to decreased testosterone feeding the tumor - ATP dependent programmed cell death -> activate caspases -> cell shrinkage, chromatin condensation, membrane blebbing, and apoptotic bodies that are phagocytosed - deeply eosinophilic cytoplasm, basophilic nucleus, pyknosis (condensation of chromatin) + karyorrhexis (fragmentation of nucleus) - intact cell membrane, no inflammation - DNA laddering: fragments of multiples of 180 bp) indicates apoptosis

59 yo man comes into ER w/ 2 day history of fever and headache, he is oriented to time and place, and PCR of CSF shows herpes simplex virus. The drug you give inhibits what viral enzyme?

DNA polymerase - acyclovir, famcyclovir, valacyclovir - gaunosine analogs - only phosphorylated by HSV/VZV thymidine kinase in affected cells - inhibit DNA polymerase by chain termination

32 yo has fecal incontinence since giving birth to her first child what is causing this?

Damage to the anal sphincter - in child birth you may have a stretch injury to the pudendal nerve (S2-4) = sensory to perineum and motor to external urethral and anal sphincter - may lead to decrease sensation in perineum/genital area and fecal/urinary incontinence - can be blocked w/ local anesthetic in childbirth using ischial spine as landmark for injection

80 yo gravida 4, para 4 has 6 month hx of decreased frequency of bowel movements -> only 1 or 2 weekly with no relief w/ fiber/fluid intake. she feels incomplete evacuation of bowels and manually supports posterior vaginal wall to defecate. DRE shows no bleed, fissure, mass, hemorrhoids, good rectal tone/reflexes. What is causing these symptoms?

Damage to the rectovaginal septum - rectocele: posterior vaginal wall prolapses as the rectum herniates towards it -> childbirth/weakening of pelvic floor muscles or episiotomy - episiotomy = incision of posterior vaginal wall to prevent tearing - or tearing of septum in child birth (separation of vagina and rectum) - mild = pressure in vagina and feeling of incomplete rectum - moderate: cant pass stool -> goes into ectocele and not down towards rectum/anus need to manually push on vaginal wall to push stool out of rectum - really bad: obstruction of distal colon

35 yo being studied for steroid hormones. She ovulates on day 14. What day will her estradiol be high and progesterone be low?

Day 12 - estrogen grows uterine lining (bricks - if unopposed-> cancer) - progesterone maintained endometrium to support fetus (mortar)

If a mutant form of the voltage-gated sodium channel is inactivated more rapidly than normal, what effect will this have on the electrical properties of the neuron?

Decrease the amplitude of the action potential

12 yo w/ 2 months of yellowing of eyes and skin. PE just shows jaundice. Her total bili is 3 and her direct is 1. Haptoglobin, AST, ALT are normal. What does she have?

Decreased activity of UDG glucuronosyltranferase (mild decrease, absent = Crigler-Najjar) - Gilber's Syndrome: benign congenital unconjugated bilirubin - AR - usually w/ stress, illness, fasting - common -Phototherapy -> increases excretion, doesn't conjugate bili

A person w/ Li-Fraumeni has a mutation in his P53 that converts arginine -> proline and alters hydrogen bonding btw mutant P53 gene and thymidine oxygen and adenine ring nitrogen of DNA. What is the result of this mutation on the transcription of genes that inhibit cell division and contain the TATA sequence.

Decreased binding of RNA polymerase - TATA and CAAT boxes are where RNA polymerase II and other TF bind DNA upstream from gene locus -> mutation decreases gene transcription dramatically - if P53 is mutated and cannot bind adenosine an thymidine aka the TATA box, then this TF cannot regulate the transcription of genes that will inhibit (tumor suppress) cell division - so no binding of p53 to induce these genes = no RNA polymerase to make genes to halt cell proliferation

25 has facial flushing and rapid heart rate when he consumes even a little alcohol. He has a variant (K487, oriental variant) compared to normal (E487, native variant) in aldehyde dehydrogenase. Kinetics are: E487: Km = 37, Kcat = 180 K487: Km = 5600, Kcat = 9.5 - What is the most likely cause of this condition?

Decreased catalytic efficiency of K487 - Km = inversely related to the affinity of the enzyme for its substrate (here, E487 has small Km = higher affinity, whereas K487 has high Km = low affinity) -> the amount fo substrate needed to reach 1/2 Vmax (when enzyme is completely saturated) - kcat = turnover #, the # of times each enzyme site converts substrate to product per unit time - Kcat/Km = catalytic efficiency - for K487 = 9.5/5600 = very small compared to non-mutated enzyme

In mouse model where thymocytes are made to constitutively express bcl-2, what do you expect to see vs. control mice?

Decreased cell death in thymic cortex - cortex is where b-cells mature - similar to follicular cell carcinoma t(14:18) - waxing and waning lymphadenopathy

82 yo w/ fecal incontinence for 2 months. PE shows no abnormalities. What is causing this?

Decreased function of puborectalis muscle - comprise pelvic floor and plays an important role in both fecal continence and defecation - tonically contracted and maintains anorectal angle at rest - this is the anal sling muscle that prevents bowels from being linear - wraps around rectum and attaches to pubic bone - innervated by perineal branches of S3, 4 (PNS) - Contraction of internal and external anal sphincters contributes to continence - internal sphincter = tonically contracted via Sympathetic fibers from superior rectal and hypogastric plexuses stimulate and maintain internal anal sphincter contraction -> inhibited by parasympathetic fiber stimulation (pelvic splancnic) - external sphincter: puedenal

What causes Niemann-Pick disease?

Decreased lysosomal hydrolase activity - progressive neurodegeneration, hepatosplenomegaly (vs. tay-sachs w/ no hepatosplenomegaly) - foam cells (lipid laden macrophages) - cherry-red spot on macula - sphingomyelinase deficiency - increased sphingomyelin

48 w/ fatigue, headache, BP 180/110, serums shows low potassium, and increased ALDO, CT scan shows tumor in adrenal gland. What additional findings would support aldo secreting tumor?

Decreased plasma renin activity - elevated ALDO will have NFB on renin cause a decrease in activity - can't use Na+ as measure

Woman on propranolol has BP 90/50, and pulse of 45. PE shows no abnormalities. normal ECG. Propranolol is decreased and she returns with a pulse of 80. Why was her pulse so low while taking a higher dose of propranolol?

Decreased slope of diastolic depolarization - block sympathetic input to SA, AV nodes (decrease cAMP -> closure of Ca+ channels preventing upstroke of calcium AP -> in ventricular cells this would be Na+) - Pacemaker current: phase 4 -> Na+ funny channel (slow depolarization = spontaneous depolarization -> voltage dependent L type Ca+ channels open -> phase 0 upstroke dictated by Ca+ -> phase 3 (K+ channels b-> repolarization) -> phase 4 again - beta blockers prolong phase 4 = prolonged conduction time/refractory period - used to treat suprqventricular arrhythmia (blocks AV node), afib, flutter - prolong PR interval (atria contracts but signal takes a long time to get to ventricle for QRS contraction) - B1: Gs-proteins -> activate adenylyl cyclase -> cAMP -> activates protein kinase A -> phosphorylates L-type Ca+ channels -> increased Ca+ into cell -> enhanced release of Ca+ by SR -> increase inotropy (contractility) and HR (chronotropy) - B1: increases SA node firing, and increases AV conduction by Gs by increasing cAMP - unique tissue as it has a slow upstroke phase 4 (HCN channels) open and allow Na+ to cause depolarization -> threshold to open voltage ca+ channels hence why slope is low in phase 0 = as they are slower channels vs. fast Na+ channels - open via hyperpolarization and cyclic nucleotides (increase when B1 binds -> increases cAMP) which will increase firing rate - adenosine is Gi on the SA/AV and decreases cAMP and slows heart (it has Gs on coronary arteries, and Gi on bronchiole smooth muscles) - M2 has Gi has activity on SA/AV which act as beta blockers (no activity on ventricular cells) -> parasympathomemetic (digoxin) could slow the heart down

histone acetyltransferase catalyzes the acetylation of lysine residues in the amino-terminal tails of histones. What is the effect of this modification?

Decreases the affinity of the histones for DNA - histone acetylation removes the positive charge of histones -> relaxed DNA coiling -> increases transcription - histone acetylation makes DNA active - by decreasing affinity of histones to DNA you decrease supercoiling making DNA more available - histone deacetylation = more silent DNA - histone methylation = reversible transcription suppression makes DNA mute

48 yo on furosemide for edema associated with biventricular failure and HTN. 5 days later edema is not fully resolved, and potassium is low. What is the mechanism of action of a drug that should be added?

Decreases the luminal permeability to Na+ in the collecting duct - Amiloride and tramterene = K+ sparing diuretics block the luminal Na+ channel preventing sodium re-uptake -> lead to decreased K excretion - Also have spironolactone and eplerenone: block ALDO receptors on basalateral side

What is the outcome of having a hep C infection? can you cure it?

Lifelong persistent infection - Hep C + HIV are both associated with IV drug use - most patients w/ Hep A will clear the virus after acute illness, Hep C causes chronic infection in 80% of patients -> cirrhosis over time (~20 years) and possible HCC

10 yo girl w/ fatigue, is pale. Her father had a splenectomy for "low blood" and a cholecystectomy. Her CBC: Hb 6.4, MCV 90, reticulocyte of 9%. Smear shows round darkened RBC with no central pallor. What abnormality is underlying this disease?

Deficiency of erythrocyte spectrin - AD inheritance - defect in RBC membrane skeleton/plasma membrane - increased MCHC - spleen removes blebs of damaged membrane -> small, round RBC w/ no central pallor - aplastic crisis in parvovirus B19 - increased fragility in osmotic fragility test (no space for water to be absorbed in cell-> lysis)

12 yo w/ fatigue, weakness, erythematous maculopapular rash on elbows and proximal muscle weakness. Can't lift her legs, but can move fingers. increased creatine kinase, serum anti-nuclear antibody + what does she have?

Dermatomyositis - increased creatine kinase + muscle weakness will differentiate this from SLE - Also may have interstitial lung disease (SOB/cough) unlike SLE - "shawl and face" rash - gattron papules on knuckles - increased risk of ovarian malignancy

What cough medicine can you use to avoid constipation?

Dextromethorphan - an opioid anti-tussive - antagonizes NMDA receptor (NoMAD camel) - SE: serotonin syndrome, and opioid effects when used in excess - mu opioid agonist -> less likely to act on GI constipation - not apure opioid agonist, also NMDA antagonism - people get high off it as a dissociative drug - combined with quinidine (prevents degradation of dextromethorphane as a cyp inhibitor) = pseudobulbar affect treatment -

55 yo has constipation after starting an OTC medication for chronic persistent cough and sinus congestion. What ingredients is causing this?

Dextromethorphan - antitussive (antagonizes NMDA glutamate receptors) - synthetic codeine analog has mild opioid effects when used in excess - naloxone can be given in overdose - mild abuse potential - may cause serotonin syndrome if combined with other serotonergic agents - constipation - other decongestants: pseudoephedrine and phenylephrine (alpha agonists): reduce hyperemia (blood flow to body), edema in nose, and open obstructed eustachian tubes - may cause HTN, rebound congestion when used for more than 4-6 days, or CNS anxiety

55 yo w/ constipation after taking OTC cough, congestion meds. what ingredient is causing this symptom?

Dextromethorphan (robitussin) - anti-tussive (antagonist to NMDA glutamate receptors) - synthetic codeine analog w/ mild opioid effect in excess (can give naloxone for OD)

62 yo w/ new dx of Alzheimers. Retired and lives at home w/ husband. What will help her to maintain her functional level?

keep notes and lists to help her memory

4 yo is yellow as his family does not eat milk, meat, or any animal products. No changes in stool/urine color. No travel, and no sick contacts. His sclerae are nonicteric. What intervention will improve the appearance?

Dietary change - B12 deficiency can cause yellowing of skin - adding this back into diet = relieve symptoms

66 yo w./ 6 months decreased exercise intolerance/SOB on exertion. Pulse 92, BP 120/80. Bilateral basilar crackles, S3 gallop, 2/6 holosystolic murmur at apex. apical impulse at anterior axillary line in 6th intercostal space. Echo shows left and right ventricular enlargement with decreased systolic function. What does he have?

Dilated cardiomyopathy - S3 = increase ventricular filling pressure (in mitral regurge, HF, dilated ventricles) - holosystolic murmur at apex: mitral regure from large LV (stretches annulus wide) - PMI is usually at midclavicular line in IC space 5 -> here it is anterior axillary -> large and displaced

What describes the onset of sleep in a narcoleptic at night?

Direct transition from wakefulness to REM sleep - excessive daytime sleepiness (despite awakening well rested) w/ rapid onset overwhelming sleepiness >3X/week for 3 months - have decrease orexin (hypocretin) in lateral hypothalamus - hypnagogic (just before GOing to sleep) or hypnopompic (before awakening, get POMPed in the morning) hallucinations - Sleep episodes start with REM sleep (sleep paralysis) - cataplexy: loss of all muscle tone after strong emotional stimuli like laughter - Treat w/ sleep hygiene, and stimulants (amphetamine, modafanil (increase DOPA in synapse)) or nighttime sodium oxybate (GHB)

physician is trying newly marketed drugIt has good therapeutic effect but increases liver enzymes to 3X normal in 5/45 patients. The doctor can't find data linking the new drug to liver dysfunction. What should they do?

Discontinue the new drug in affected patients and file MedWatch reports on the FDS's website - MedWatch = Food + Drug Administration's "Safety Information and Adverse Event Reporting Program." - used for reporting adverse event or sentinel event via voluntary reporting that is shared w/ medical community/general public - also disseminates medical product safety alerts - such as recalls and other safety communications

28 yo w/ 30 mins of sudden SOB 3 year hx of cocaine abuse. Temp is 100, pulse 100, BP 150/45, he has diminished pulses in left upper extremity. Crackles heard over all lung fields. Grade 2/6 diastolic murmur on left sternal border. Chest x-ray = widened aortic arch. What is the dx?

Dissecting aneurysm - longitudinal intimal tear = false lumen - associated w/ HTN, bicuspid aorta, connective tissue disorder - tearing sudden onset pain radiating to back w/ unequal BP in arms - chest x-ray shows widened mediastinum - type A: Ascending aorta -> aortic regurgitation or cardiac temponade tx w. surgery - type B: descending aorta (below left subclavian artery) tx w/ beta=blockers then vasodilators

9 yo girl w/ poor growth, bitemporal hemianopia. Labs show decreased growth hormone. MRI shows calcified cystic mass in suprasellar region. This tumor is derived from what origin?

Diverticulum of the roof of the embryonic oral cavity (ectoderm) - craniopharyngioma -> benign Rathke pouch tumor w/ cholesterol crystals in "motor oil" like fluid filled tumor and calcifications - most common childhood supratentorial tumo - don't confuse w/ pituitary adenoma (both cause bitemporal hemianopia)

What medications can you give to help with alzheimer's progression?

Donepezil, Rivastigmine, galantamine = indirect agonists (anticholinesterases) - first line therapy for Alzheimer's - Dona Riva dances at the Gala - can also give memantine = NMDA receptor antagonist (helps prevent excitotoxicity mediated by Ca+) in mod-advanced dementia

27 yo being treated for hodgkin lymphoma receives bone marrow tx from his cousin. 2 weeks after, he has general erythematous, maculopapular rash, diarrhea, and increased liver enzymes and bilirubin. What is the mechanism causing this reaction?

Donor T lymphocytes reacting against host cell - Graft vs. Host disease - grafted immunocompetent T-cells proliferate in immunocompromised host rejecting host cells -> severe organ dysfunction

16 yo w/ 2 year hx of fainting while standing, BP 110/80 while supine, and 60/40 while standing. Plasma shows no NE and marked increase in DOPA when rising from supine to standing. What is she deficient in?

Dopamine B-hydroxylase - production of DOPA/NE: phenylalanine (phenylalanine hydroxylase, BH4 = PKU) -> tyrosine (tyrosine hydroxylase, BH4) -> DOPA (DOPA decarboxylase) -> Dopamine (dopamine B-hydroxylase) -> NE (phenylethanolamine-N-methyltransferase) -> Epi via cortisol in adrenal glands

50 yo woman w/ discharge from both breasts. Has HTN, and DM type II. She takes hydrochlorothiazide. She has milky discharge from both breasts what is causing this?

Drug effect

32 yo has sister who died from melanoma, and family w/ melanoma and large pigmented skin lesions. What lesions is most likely on both sun-exposed and non sun-exposed areas?

Dysplastic nevi - xeroderma pigmentosum - autosomal recessive disease - defective nucleotide excision repair (endonuclease release oligonucleotides w/ damaged bases, and DNA polymerase and ligase fill/reseal gap -> occurs in G1 phase of cell cycle) - sun causes pyrimidine dimers that are unable to undergo excision repair

16 yo w/ abdominal pain, vomiting has perforated appendix. What bacteria is most likely present?

E Coli - appendicitis = acute inflammation of appendix from obstruction from fecalith (adults) or lymphoid hyperplasia (children) - obstruction of appendix -> close loop obstruction in structure -> bacteria (E. coli/bacteroides multiple/inflammation ensues) -> increases pressure - Stimulates visceral afferent nerve fibers of T8-T10 -> initial diffuse periumbilical pain (irritates pleura) -> localizes to RLQ (Mcburneys point = ASIS -> umbilicus, closer to ASIS) - may perforate -> peritonitis - psoas, obturator, rovsing signs (pressing on left illicit pain on right), guarding on rebound tenderness

78 yo sudden pain in leg, cool and pale leg with no pulses. An ECG shows irregularly irregular rhythm. What treatment will provide effective relief from these symptoms?

Embolectomy - need to lyse the clot - thromboembolic event - has an active, acute ischemia from emboli and needs to be removed surgically

16 yo pregnant girl w/ generalized tonic-clonic seizures, pulse 120, RR 24, bP 170/109, Hct 42%, Plt 90,000, high AST/ALT has what?

Eclampsia and HELLP Sydrome - Hemolysis - Elevated Liver enzymes - Low Platelets - from severe pre-eclampsia - will have schistocytes and maybe DIC and hapatic subscapular hematomas (rupture -> severe hypotension)

2 week old full-term female w/ pale stool and jaundice that progresses and no excretion of bile. Bx shows inflammation of liver and proliferation of small bile ducts and increased portal fibrosis. If not treated what will develop?

End-stage cirrhosis - biliary atresia = most common reason for pediatric liver transplant - fibro-obliterative destruction of extrahepatic bile ducts -> cholestasis - newborn after persistent jaundice after 2 weeks of life, darkening urine, acholic stools (pale), hepatomegaly - labs with increased direct bili

48 yo w/ uterine bleeding for 3 months during and in between menses @ irregular intervals. nonpalpable adnexa, curettage -> abundant tissue. whats causing this?

Endometrial hyperplasia

What antiretroviral prevents entry into a CD4+ t-cell

Enfuvirtide

64 yo has bronchospastic pulmonary disease still has wheezing after increasing his B-adrenergic agonist. He is given systemic prednisone. What is the action of prednisone on enhancing the B-adrenergic agonist?

Enhanced action of the agonist at B-adrenergic receptors - glucocorticoids enhances β receptor responses = bronchial smooth muscle relaxation - regulates coupling of β receptors to G proteins -glucocorticoids upregulate downregulated β receptor function after chronic β agonist exposure - increase in rate of gene transcription and hence of receptor numbers - cortisol will increase downstream proteins that help increase B1 stimulation (also increased by thyroid hormone)

40 yo w/ 1 wk of abdominal pain, watery/bloody diarrhea after 2 week vacation in Kenya 1 month ago. Distended and tender abdomen. Stool shows 12-um trophozoites w/ erythropagocytosis. What organism?

Entamoeba Histolytica - Amebiasis - may have dysentery, liver abscess (anchovy paste), RUQ pain, colon bx with flask-shaped ulcers - from fecal-oral transmission of cysts in water - tx: metronidazole or iodoquinol/ paromomycin for asymptomatic cyst passers

75 yo w/ fever, back pain, pain on urination. Has hx of prostatitis. Gram stain shows gram positive cocci in chains. What bacteria is causing this illness?

Enterococcus faecalis - cystitis: dysuria, urgency, and WBC (not casts!) in urine - even though strep A also is gram + growing in chains, it does not cause UTI - UTI causing bugs: E coli, stap saprophyticus, klebsiella pneumoniae, serratia, proteus, pseudomonas - of all of this this is the only gram + that grows in chains - treat with first generation cephalosporines, or fluroquinolones - cause of UTI: E coli and urinary bacteria, klebsiella - can be in pairs and chains, more generally in chains = a type of strep (gamma hemolytic) and therefore in chains - UTI tx: general cystitis (non pregnant): nitropherantoin, bactrim for 5 days - pyelonephritis: flouroquinolone, in pregnancy you use ceftriaxoine, gentamicin, or amoxicillin - pregnant: nitropherantoin - complicated: flouriquinolone

Dysfunction of what cells cause the symptoms of celiac disease?

Enterocytes - associated with DQ2/DQ8 (I 8 2 much gluten at Diary queen) - AB: IgA anti-endomysial, IgA anti-tissue transglutaminase, IgA and IgG deaminated gliadin peptide - intolerance of gliadin - malabsorption/steatorrhea - northern european descent - dermatitis herpetiformis - villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis - moderate increase of malignancy - d-xylose test: passively absorbed in proximal small intestine -> blood/urine decrease with mucosa defects/bacterial overgrowth - no absorption of gluten via enterocytes

A substance is made in GI tract that causes activation of pancreatic trypsinogen to trypsin and activation of proteolytic zymogens. What is the substance?

Enteropeptidase - trypsinogen converts to active trypsin to activate other proenzymes and cleave more trypsinogen ->trypsin in pancreases via enterokinase/enteropeptidase a brush border enzyme in duododenal/jejunal mucosa

In diabetic ketoacidosis, what stimulates hormone-sensitive lipase in adipocytes leading to accumulation of free fatty acids for ketone formation?

Epinephrine - HSL stimulated by catecholamines and ACTH in times where the body would need the breakdown of stores for extra energy in "stress" - this is inhibited by insulin - in light of "starvation in the face of plenty" the body releases catecholamines that break down stores to "feed" body causing ketoacidosis

13 yo has severe rejection 6 months after cadaveric renal tx. 2 weeks later she gets lymphadenopathy aand hepatosplenomagaly. Lymph node bx shows monomorphous population of B lymphocytes. What virus is causing this?

Epstein-Barr virus - associated with lymphoproliferative disease in tx pt - If you have had EBV in past, it might flare up when your immune system is suppressed - If you have not had EBV before, a tx may introduce EBV-infected cells from donor - also proliferation of B cells = CD21 is how EBV attacks into B cells

Someone with AIDS is at a large risk for developing what malignancy?

Epstein-Barr virus-induced brain lymphoma - aka primary central nervous system lymphoma - considered an AIDS defining illness - confusion, memory loss, seizures, mass lesion, may have ring enhancing lesions on MRI - need to distinguish from toxo - need bx or if failure after treatment of toxo or via CSF

60 yo w/ bacterial bronchopneumonia in ICU responds to AB but 3 days later gets a maculopapular rash over extremities w/ central vesicles and bullae w/ targetoid appearance. Bx shows subepidermal blisters and degeneration of basal cell layer, keratinocyte necrosis, lymphocytic infiltration @ dermal/epidermal junction. What is dx?

Erythema multiforme - associated w. infections (mycoplasma pneumo, HSV), drugs (sulfa, b-lactams, phenytoin) - presents w/ multiple types of lesions -> macules, papules, vesicles, target lesions)

2 yo boy has fever, sore throat, and rash. temp 101, w/ widespread red, sandpaper-like rash over extremities. Purulent exudate seen over tonsils. He has a strep pyogenes (A) infection. What component of strep caused this?

Erythogenic toxin - strep pyogenes has capsule made out of hyaluronic acid - toxins cause: scarlett fever (strawberry tongue, pharyngitis, widespread rash), toxic shock (super AG SpeA< SpeC), and necrotizing fasciitis (SpeB) - M protein causes: virulence factor for rheumatic fever via molecular mimicry (interferes with opsonization), is antiphagocytic -> strep throat causes RF only, not impetigo - RF: JONES (Joints, heart problems, nodules on extensor surface, erythema marginatum (target like rash), sydenhams chorea) - PSGN: from strep throat or impetigo

What organisms causes prostatitis in older men?

Escherichia coli - in younger men think gonorrhea or chlamydia

70 yo w/ recurrent pneumonia, fatigue, and weight loss for 3 months. T4 and TSH are normal, but T3 is decreased. TSH increases after TRH administration. What does she have?

Euthyroid sick syndrome - pt with chronic illness - signs of hypothyroidism decreased T3 and normal T4/TSH - decreased peripheral conversion of T4->T3 - IL-6 causes free T3 to be diminished - tx underlying condition

11 yo w/ pain in thigh, warmth and tenderness and ill-defined mass. X-ray = solitary mid-diaphyseal osteolytic lesion w/ permeative margins. has conentric layers of reactive bone. Bx shows small, uniform, hyperchromatic cells w. a high nuclear:cytoplasm ratio and scant stroma w/ no matrix. What does he have?

Ewing Sarcoma - most common in white males <15 - in diaphysis of long bones (femur), pelvic flat bones - anaplastic small blue cells of neuroectoderm origin (look like lymphocytes) - Differentiate by testing for t(11:22) - Onion skin periosteal reaction - aggressive w/ early met and responds to chemo well - Permeative = moth eaten appearance

18 yo w/ pain on right elbow. She is a tennis player practicing a lot. There is tenderness over lateral right elbow. What muscle originates in this area of inflammation?

Extensor carpi radialis brevis - lateral epicondylitis - tennis elbow - repetitive extension (backhand shots) or idiopathic - injured extensor tendon where extensor muscles insert - extensor carpi radialis longus, extensor carpi radials brevis, extensor carpi ulnaris, extensor digitorum communis

Woman who has episiotomy (incision made posteriorly from posterior vaginal commissure) is at greatest risk for what injury if this is torn further in delivery?

External anal sphincter

in a study where hyperglycemia and hyperinsulinemia is induced. Muscle bx show increased malonyl-CoA. This directly inhibits what process?

Fatty acid oxidation - in a state of being well fed, the body wants to store FA -> TG, and not break them down for fuel - FA ox in mitochondria - rate limiting enzyme = carnitine acyltransferase I and inhibited by Malonyl-CoA from FA synthesis - in presence of glucose you don't need to break down FA

23 yo drinks heavily on a weekend what cellular changes are most likely to occur in his liver?

Fatty change - Alcoholic liver disease: - Hepatic steatosis: macrovesiclar fatty change that may be reversible with alcohol cessation - alcoholic hepatitis: requires sustained, long-term consumption -> swollen and necrotic hepatocytes with neutrophilic infiltration. Mallory bodies (intracytoplasmic eosinophilic inclusions of damaged keratin filaments). Think AST.ALT by 2:1 ratio - Alcoholic cirrhosis: final and irreversible form. Sclerosis around central vein may be seen in early disease. Regenerative nodules surrounded by fibrous bands in response to chronic liver injury -> portal HTN and end-stage liver disease - acute hepatocyte swelling in hepatitis

What places 70 yo person at greatest risk of having vertical compression fractures and decreased bone mineral density?

Female gender - estrogen inhibits apoptosis in bone-forming osteoblasts and induces apoptosis in bone-resorbing osteoclasts - in estrogen decrease in menopause -> increases cycles of remodeling and bone resorption -> increase osteoporosis

43 yo had forcible eversion of his left foot while jogging on uneven ground. X-ray shows fracture of fibula above distal tibiofibular articulation. Contraction of what muscle caused this pt pain at the fracture.

Fibularis (peroneus) brevis - muscle along fibula that inserts on the talus - motor innervation via superficial peroneal nerve (peroneus longus/brevis) -> eversion

4 day old baby w/ respiratory distress, cyanosis was found in crib not breathing until mom picked him up. baby has narrow thorax, ear w/ periauricular tags, micrognathia, glossoptosis, mandibular cleft, short palate. What pharyngeal arch was defected?

First - He has Pierre Robin sequence: micrognathia (small jaw), glossoptosis (downward displaced tongue), cleft palate, airway obstruction First Arch derivatives: - cartilage: Maxillary process (maxilla, zygomatic bone), mandibular process (Meckel cartilage, mandible), Malleus, incus and sphenomandibular ligament - muscles: mastication, mylohyoid, anterior belly of digastric, tensor tympani, anterior 2/3 of tongue, tensor veli palatini - nerve: CN V3 (mandibular of trigeminal)

15 yo w/ 1 day of red and painful skin after sunbathing w/ no blisters. What kind of burn is this?

First-degree burn - acute cutaneous inflammation reaction from excessive UV radiation -> DNA mutation inducing apoptosis of keratinocytes - UVB = dominant for sun Burn - UVA = for tAnning and photoAging - exposure to both increases risk of skin cancer

investigator studying vaccine against capsular polysaccarides of Neisseria meningitis. Carbohydrates are conjugated to compounds and injected into mice. Titers if anti-capsular IgG AB are measured. What compound is most likely to increase titers of these AB when conjugated to polysaccharides?

Flagellin - the only way to make IgG AB is via CD4+ which will only occur in the face of a protein that is engulfed and presented via MHC II - polysaccharide vaccine: just vaccinated against that subunit - MHC II can only present protein to CD4+ via proteins which helps to cause class switching of b-cells to get an IgG response - a strong T-cell dependent response needs conjugation to protein (diphtheria) - Pneumonia conjugated -> t-cell response with IgG cells and memory cells result hence why they are given to infants who have weaker AB response

Cross sections area of an aneurysm is 2cm^2, and the velocity of blood flow is 20cm/sec. What is the flow rate (L/min) through the aneurysm?

Flow rate (Q) = Flow velocity X cross sectional area - 2X20 = 40cm^3/sec - 40X60s = 24000 mm/sec (1cm^3 = 1 mm) - 2.4L/min

66 yo w/ cavitary lesion in right lower lobe -> klebsiella pneumoniae. His symptoms resolve w/ AB what will be found in the tissue 6 months later?

Focal pulmonary fibrosis - not normal parenchyma - the cavitary lesion most likely destroys the lung parenchyma -> no more type II pneumocytes, no ability to regenerate

59 yo progressively can't swallow, had 15 lb weight loss, smoked cigs, and consumes a lot a beer a day. He is cachectic and wheezes on lungs. CXR shows enlargement of paratracheal and hilar lymph nodes. Endoscopy = 6cm mass in mid esophagus that is centrally ulcerated w/ elevated mucosal rim. What will bx show?

Foci of keratinization - esophageal cancer -> progressive dysphagia (solids and then liquids) + weight loss - poor prognosis usually late presentation of disease - Squamous cell carcinoma: upper 2/3: from alcohol, hot liquids, caustic strictures, smoking, achalasia -> most common worldwide - Adenocarcinoma: lower 1/3 from chronic GERD, Barrett esophagus, obestiy, smoking, achalasia -> more common in America

in methanol overdose causing a severe metabolic acidosis and visual symptoms (dilated pupils and diminished light reflex) what is building up causing acidosis?

Formic acid - an ethanol - methanol -> formaldehyde (via alcohol dehydrogenase) -> formic acid (via formaldehyde dehydrogenase) that causes optic nerve damage - treat w/ fomepizole or ethanol = competitive inhibitor of alchohol dehydrogenase that will block break down of methanol into formic acid

28 yo at 18 wks gestation has palpitations, and increased serum total thyroxine (T4). What is the best test to confirm hyperthyroidism?

Free T4 - in pregnancy thyroxine-binding globulin increases (estrogen causes an increase in TBG -> can also be seen in OCP) - increased TBG causes an increase in bound T3/T4 = inactive hormone - true hyperthyroidism would need to evaluate free T4 to ensure the active hormone production is increased

28 yo woman with chronic renal failure (GFR less than 20) needs treatment for volume overload and pulmonary edema. What is the best therapy?

Furosemide, bumetanide, torsemide - cause maximum amount if diuresis in shortest amount of time - currently most efficacious diuretic for edematous states (HF, cirrhosis, nephrotic syndrome, pulmonary edema), HTN, hypercalemia - sulfanamide, inhibit NA+/K+/2Cl- in thick ascending limb of loop of henle - abolishes hypertonicity in medulla -> no more ability to concentrate urine - stimulates PGE release (vasodilatory on afferent arteriole) inhibited by NSAIDS - increases Ca+ excretion (loops lose Ca+) - SE: ototoxicity, hypokalemia, hypomagnesemia, dehydration, allergy (sulfa -> use ethacrynic acid = more ototoxicity), metabolic alkalosis, interstitial nephritis, gout

In what stage of the cell cylce of mitotic cyclins synthesized?

G2 - cyclins are proteins that control progression of cells through cell cycles by activating cyclin dependent kinases - concentration varies in cell cycle - generally made prior to the cycle it works upon, here mitosis needs these cyclins to move into the M phase, and G2 is just prior when the proteins are beginning to be made - is made and then ready to activate the M phase CDK-cyclin complex to undergo mitosis - degraded after mitosis - CDK2 and cyclin B G2->M - DEAB: the progression of cyclins in sequence - make Cyclin D and A + CDK 4 and CDK2 respectively + CE and CDK2 -> G1->S regulation - S: Cyclin A + CDK 2 remains active S->G2 - G2-> M: cyclin B and CDK1 = mitotic cyclins and drop off in mitosis

35 yo w/ 6 months of burning abdominal pain 1-2 hrs after meals, black stools for 2 days, no relief w/ antacids or histamine 2 blockers. He is sweating and lightheaded, low BP. he has 1cm mass in pancreas. Antibodies to what substance will help to ID this tumor?

Gastrin - Zollinger-Ellison Syndrome - gastrin-secreting tumor in pancreas or duodenum - causes acid hypersecretion - gastrin levels remain elevated even after secretin test (should lower it) - may be associate with MEN 1

A 2 mo old male w/ generalized tonic clonic seizures, myoclonus, hiccuping. He has been floppy since birth. He has decreased deep tendon reflexes/hypotonia. A signaling defect in an inhibitory NT is suspected. What NT receptors contains the defect?

Glycine receptor - glycine and GABA are inhibitory NT - child has rare myoclonus disorder - main point = glycine is inhibitory - without inhibitory NT = myoclonus (quick jerky movements), hiccuping etc

Cyclophasphamide kill mitotic cells. What cells are most rapidly depleted? B lymphocytes, granulocytes, T lymphocytes?

Granulocytes - turnover faster than bone marrow cell types - most likely to have neutropenia in chemo -> most pronounced - total leukocyte count will be low with neutropenia is most prominent

81 yo woman w/ massive PE from DVT is in hospital. Her platelet count is 160,000. Medication is started and a week later her platelet count is 55,000. Thrombocytopenia from a drug w/ what mechanism?

Heparin -> potentiates the action of antithrombin III - heparin induces thrombocytopenia -> IgG antibodies against heparin bound platelet factor 4 (PF4) - antibody-heparin-PF4 complex activates platelets -> thrombosis and thrombocytopenia

Why are there larger amounts of CO2 in venous blood samples?

HCO3- transported in the plasma - CO2 transported in blood in three forms 1. (70%) HCO3- 2. (21-25%) carbaminohemoglibin or HbCO2: CO2 bound to Hb at N-terminus of globin, not heme -> favors deoxygenated form of hb 3. (5-9%) dissolved CO2 - most is via bicarb and very little from dissolved CO2 in plasma/RBC

What does ritonavir/lopinavir do?

HIV medication that hinders protein processing - protease inhibitors - all end in "navir" (Navir tease a protease) - assembly of HIV-1 proteaase (pol gene) needs to cleave polyprotein products - prevents maturation of new viruses

2 month old baby vaccinated w/ vaccine that was manufactured to convert t-independent antigens -> T-dependent forms to enhance protection. What vaccine is this?

Haemophilus influenzae type B - polysaccharide conjugate vaccine, inactivated bacterial vaccine. join polysaccharide capsule that surrounds Hib -> protein carrier = conjugation Four kinds of vaccines: - Live attenuated: no pathogenicity, but can replicate organism -> cellular/humoral response (lifelong immunity, may cause infection in immunocomped) -> MMR, smallpox, BCG, intranasal flue, yellow fever, rotavirus, adenovirus, vericella, polio (sabin A life) - killed/inactivated: inactivated by heat, same structure -> humoral response, safer than live vax, need boosters.rabies, hep A, flu, Salk polio (sulking dead) - Subunit: AG from organism that stimulates best immune response -> expensive/weak response -> HBV (HBsAG), HPV, Acellular pertussis (part of TDaP), neisseria meningitis, strep pneumo, hflu - Toxoid: denatured toxin from bacteria -> makes AB against toxin of organism -> may need booster = tetanus and diphtheria

pale 62 yo w/ fatigue and indigestion, decreased appetite, mild splenomegaly, Hb 9, Hct 27%, Leukocyte 3100 w/ 75% lyphocytes, 20% neutrophils, 5% monocytes, Plt 75,000. Lymphocytes have cytoplasmic projections and + for acid phosphatase even in presence of tartrate. What is the dx?

Hairy cell Leukemia - adult males - mature B-cell tumor w/ hair like projections - no peripheral lymphadenopathy - causes marrow fibrosis -> dry tap - splenomegaly (of red pulp) and pancytopenia - Stains TRAP (tartrate-resistant acid phosphatase) - treat w/ cladribine, pentostatin

33 yo was diagnosed w/ epilepsy at age 10. His most recent seizure was a generalized tonic-clonic 5 years ago. His medication at that time was adjusted, he is on carbamazepine. He has never crash a car. What is his status regarding driving?

He is medically qualified to drive - In U.S. can drive if their seizures are controlled w/ medication and they meet licensing requirements in their state - seizure free for three months and a year. - 44 of the 50 states, burden is placed on pt to report - Six states place burden of reporting on patient's physician - licensing agency decides to revoke or restrict a driver's license - Exceptions are made when a physician determines that seizures were caused by medication experimentation or were temporary - Those who have been seizure-free for a year without medication need not report the condition

electrical stimulation of the stellate ganglion results in increased what?

Heart rate - aka the cervicalthoracic ganglion (sympathetic chain) is a sympathetic ganglion formed by fusion of inferior cervical ganglion and first thoracic ganglion - may be cut in order to decrease Raynaud's phenomenon and hyperhydrosis of the hands - injury leads to horner's syndrome - in HR: decrease in blood flood decreases signaling -> CN IX -> solitary nucleus -> sympathetic chain -> slow HR and constrict blood vessels

25 yo man lives 10,000 feet above sea level for 4 weeks, after returning home, his ventilation returns to normal after 2 weeks but oxygen delivery to his muscles is greater than before acclimatization. An increase in what describes this?

Hematocrit - you make more EPO -> makes more RBC that then live for 120 days (more than two weeks) allowing increased oxygen delivery -

How does colon cancer spread to the liver?

Hematogenous spread of colonic tumor via portal venous system

39 yo has hair growth on face, no menstrual period, CT of pelvis shows ovarian tumor and high testosterone. What increases from testosterone?

Hemoglobin - Testosterone and other androgens have an erythropoietic stimulating effect that can cause polycythemia, which manifests as an increase in hemoglobin, hematocrit, or red blood cell count - hence why men have higher Hct, plt, and hb

26 yo shot in leg, he is pale, unconscious, pulse 120, RR 16, BP 80/60. Is his arterial baroreceptor firing rate, SVRA, pulmonary vascular resistance increased/decreased and will his systemic capillary fluid transfer be absorptive or filtrating?

Hemorrhagic shock - arterial baroreceptor rate: decreased (to activate sympathetic response and dampen PNS -> increase HR) - SVRL Increased from increase in sympathetic NS/RAAS activation from blood loss -> increase vascular resistance in lungs (pulmonary resistance is the same as SVR, not as PCWP (measures LA pressure) which would be decreased in hypovolemic shock) - increase pulmonary vascular resistance - systemic capillary fluid transfer: absorptive as hydrostatic pressure decreases -> fluid fills in and kidney is reabsorbing salt/water

6 month old well-child exam from China and had poor health care. No hx of illness, and no meds. Screening for what is an appropriate step?

Hepatits B - serious global public health problem - transmitted via sex, parenteral contact or infected mother ->baby - if contracted early in life -> chronic liver disease (cirrhosis + HCC) - high endemicity countries (China) = most common route of infection = vertical transmission from mother to child - Screening of all pregnant women + passive immunization w/ human hepatitis B IgG are not affordable for many - availability of vaccine = most effective means of prevention - highest incidence in Southeast Asian and Sub-Saharan African (up to 90% of infants infected during first year of life and 30%-50% infected btw 1-4) -> chronic infections and about 25% of adults who become chronically infected during childhood die from HBV-related liver cancer or cirrhosis

2 yo boy w/. developmental delay. History of hearing loss in mother, and delayed speech in sister. Maternal uncle had stroke like episodes at age of 25. on PE he has ophthalmoplegia (weakness of eyes) and hypotonia and his serum lactic acid concentration is increased. What genetic term describes these findings?

Heteroplasmy - variation in phenotypic presentation of disease as it is passed via mitochondria - Here = mitochondirial myopathies: - myopathy, lactic acidosis, CNS disease, stroke-like episodes - muscle bx: ragged red fibers

52 yo w/ chest pain, took three doses of nitroglycerin w/ no relief pulse is 90/min, BP 114/70, and sweating. lungs are clear. He has an S4, and ST-segment elevation in inferior leads. He is given morphine and gets pruritus, warmth, pulse is now 120, and BP 90/50 w/ facial flushing. Release of what is causing this presentation?

Histamine - the other options wereL - epinephrine (vasoconstriction, and increases BP) - NO - serotonin - thromboxane

50 yo w/ smoking hx has increased SOB, cough, wheezing, increased chest diameter, decreased breath sounds. What will his bicarb be?

High - COPD: Increase air trapping, CO2 retention, hypoxia and secondary polycythemia - state of elevated CO2 -> respiratory acidosis with compensatory renal retention of bicarb

bx from calcified lesion on breast mammogram: poorly cohesive cells growing in sheets, N:C ratio ~ 1:1, prominent nucleoli. Microscopic evidence of invasion, but no adenopathy nor metastasis. What is her grade and her stage?

High grade, low-stage neoplasm - grade: the way the cells look -> poor or well differentiated (do the look or not look like the tissue of origin = differentiation) stage: are the cells invading surrounding tissue

How does smoking affect the mucus production and secretion, activity of cilia, and alveolar macrophage function?

Mucus production and secretion increases (increase in goblet size/function) -> this mucus remains stagnant as the mucociliary escalator is slowed down and eventually depleted with prolonged smoking. - Alveolar macrophage function decreases

37 yo w/ HTN of 188/131 despite therapy. exam shows flame hemorrhages w/ arteriolar narrowing. What disorder of blood vessels will most likely be caused by this condition?

Hyperplastic arteriosclerosis - not berry aneurysm - this pt already shows sings of end-organ damage (in eye) - onion skinning in malignant HTN w/ proliferation of smooth muscle cells - increase risk of berry (saccular) aneurysm in: coarctation of aorta, ADPKD, Ehlers-Danlos syndrome

17 yo wrestler collpases as he tried to take long steam bath and used laxatives to fit into weight class. What will hi lab studies be?

Hypokalemia - in dehydration: kidneys decrease GFR, Decrease RPF (increases FF) - increased ALDO will cause sodium retention and potassium depletion to restore blood volume - hypovolemic and high pulse pressure -> increased RAAS - contraction alkalosis = alkalosis in volume contraction - diarrhea/vomiting: lose Na+, K+, Cl- but vomiting (alkalosis) diarrhea (acidosis) - in metabolic acidosis you lose potassium -> lose H+ protons to compensate along with potassium and become hypokalemic (even thought the H+ causes K+ to come out of cells)

64 yo man w/ cough, dyspnea, chest pain, afebrile. X-ray of chest has density in hilar region. Bx has round cells w/ little cytoplasm that are twice the size of lymphocytes arranged in infiltrating sheets w/ neither glandular nor squamous organization. What is abnormal in their serum?

Hyponatremia - Small cell (oat cell) lung cancer - Undifferentiated (high N:C ratio) and aggressive - S = Central, Smoker, Sydromes (many paraneoplastic syndromes) - ACTH -> Cushings - SIADH -> hyponatremia - AB against Ca+ channels -> Lambert Eaton/ or against neurons -> paraneoplastic mylitis, encephalitis, cerebellar dengeneration - chromogranin A +, neuron specific enolase +, synaptophysin +

What cytokines cause sepsis?

IL-1 and tumor necrosis factor-alpha

3 yo w/ bacterial colitis from salmonella enterica. What factors recruits neutrophils to inflammatory site by intestinal epithelial cells?

IL-8 - major chemotactic factor for neutrophils "clean up on aisle 8" neutrophils needed to clean up - neutrophil chemotactic factors: IL-8, C5a, LTB4

nurse comes in for TB screening. Sample of her whole blood is incubated with pathogen. Three days later, what cytokine's presence will indicate a positive finding?

INF gamma - interferon gamma release assay mixes blood with TB AG and WBC will release INF gamma if person has or has ever had TB (Latent infections = positive) - less likely to have false positives if person had prior BCG vaccine - APC will carry TB to lymph -> naive t-cell -> IL12 (turns CD4+ to TH1)-> TH1 produces INF gamma-> activates macrophages -> epitheliod histocyte/giant cells/granuloma

woman w/ rapid HR. she is 55kg w/ ventricular tachy. IV dose wants to be given to obtain peak serum concentration of 10mg/L. Volume of distribution of drug is 1.81 L/kg. What loading dose is needed (mg)?

IV = 100% bioavailability Vd = volume of distribution of 1.81 L/kg, and woman weighs 55kg -> (1.81 x 55 = 99.55) Loading dose = target plasma concentration x volume of distribution x weight LD = 10 mg/L (99.55) = 1000mg loading dose

A study wants to detect a mean difference of 0.4 in an asthma treatment. there is 80% power, and significance level of 5%. What does 80% power mean?

If the treatment really change the mean asthma score by 0.4, there is an 80% chance a study of this size will find a p value <0.05 - power = 1-B -> statistical power = rejecting the null hypothesis when it is false - B = type II error (False Negative) or study saying there is no significant findings when really there are - alpha = type I error -> saying there is a difference when really there is not (usually 0.05) if P <0.05 the probability of obtaining the result by chance is less than 5%

Scientist wants to know if CD44v10 variant protein is expressed in a pt w/ hodgkin disease. He has fresh frozen tissue, and bx from pt. What is the best lab technique to do so?

Immunohistochemistry (IHC) - immunostaining - involves process of identifying antigens (proteins) in cells of a tissue section by exploiting principle of antibodies binding specifically to antigens in biological tissues

30 yo woman with Li-Fraumeni syndrome has adenocarcinoma of the breast. What mechanism underlies this condition?

Impaired regulation of apoptosis - P53 = tumor supressor genes, the traffic cops of the cell cycle - prevent progression from G1-> S phase until DNA is either repaired (if possible), or they call in factors (BAX) to undergo apoptosis - activates P21 - Li-Fraumeni: AD, multiple malignancies at early age -> SBLA (sarcoma, breast, leukemia, adrenal gland)

39 yo with PKD has blood in urine, pulse 100, RR 24, BP 160/90, BUN 100, creatine 8, What will his pH, PCO2, HCO3- be?

In CKD/Failure -> metabolic acidosis and retained nitrogenous waste products - decreased bicarb (PCT can't reabsorb it), decreased PCO2 (respiratory compensation for acidosis), and low pH - dyslipidemia, high K+, uremia, Na+/H2O retention, growth retardation, EPO failure and anemia, renal osteodystrophy (hypocalcemia, hyperphos, and failure of vita D hydroxylation -> high PTH -> thinning of bones)

Woman after birth has severe vaginal bleeding. A branch of what major artery must be ligated?

Internal iliac - both uterine and vaginal arteries come off of the internal iliac vs. ovarian that stems directly from aorta bilaterally

What do statins do to the levels of HDL and Triglycerides?

Increase HDL and decrease TG - HMG-Coa reductase inhibitor - REALLY increase LDL - mild increase in HDL and TG - Inhibit conversion of HMG CoA to mevalonate (cholesterol precursor and decrease mortality in CAS pts - SE: hepatotoxicity (Increase IFT), myopathy (especially w/ fibrates or niacin)

48 yo woman in ER w/ palpitations. Pulse 180, BP 104/68 w? tachycardia. A carotid sinus massage is performed, and sinus rhythm is restored. Why?

Increase cardiac parasympathetic activity - increased pressure on carotid sinus -> makes body feel like there is high blood pressure via carotid baroreceptor (sinus) not chemoreceptor (carotid body) - increased stretch -> increased afferent signaling -> increased in parasympathetic firing (this increases AV node refractory period and decreases HR and SVR)

34 yo ran 12 miles on a hot day. Pulse is 130, BP is 80/60. What changes in his autonomic nervous system occurred?

Increase in sympathetic efferent activity and decrease in parasympathetic activity

73 yo w/ mid-back pain, discomfort and can walk without problems. Pain in percussion over thoracic spine. X-ray of spine shows vertebral cortical thickening of the end-plates and "picture frame" vertebrae. What do you expect the labs to be?

Increased Alkaline phosphate - Paget Disease causes cortical thickening - common localized disorder of bone remodeling from increased osteoclastic activity followed by increased osteoblastic activity -> poor quality thickened bone - all lab values normal except elevated alk phos from hyperactive osteoblasts - mosaic pattern of woven and lamellar bone - long bone chalk stick fractures - increase blood flow from ateriovenous shunts -> high output heart failure - increased risk of osteosarcoma from overactive unregulated osteoblast growth - tx w/ bisphosphanates to prevent lytic phase bone resorption from osteoclasts

65 yo w/ osteoarthritis has pain radiating dow distal anterior thigh, knee, medial leg and foot. Bony outgrowth of vertebrae compressing what nerve root is suspected in which intervertebral foramina?

L3-L4 - L4 rediculopathy

What are changes in the CV system w/ normal aging?

Increased basal systolic blood pressure - Thickening/stiffening of arteries from collagen + Ca+ deposition + loss of elastic fibers in medial layer -> rise in systolic blood pressure and diastolic BP declines - In LV = modest concentric wall thickening from cellular hypertrophy, cavity size does not change - Reduced peak HR + peripheral O2 utilization but no decline in SV

30 yo infertility, weight gain, irregular period has a progestin challenge test and bleeds after withdrawal. What is the most likely cause of her oligomenorrhea?

Increased estrogen from adipose tissue - PCOS

32 yo has ruptured membrane at 39 weeks and umbilical cord compression is suspected. What would explain the decrease in fetal heart rate due to compression?

Increased fetal systemic vascular resistance - increase in BP felt by babies carotid body -> increases firing to tell PNS vagus nerve to kick on and slow HR - Compression of babies umbilical veins/arteries are part of their circulation and will be felt as a increase in SVR

19 yo burns 35% of her body surface severely. What will occur over the next 7 days?

Increased metabolic rate - widespread loss of skin = inability to regulate temperature properly - metabolic rate increases to increase the heat/water being lost to the environment - profound hypermetabolic response persists up to 24 months - elevations in catecholamines, cortisol, inflammatory cells = whole body catabolism, elevated resting energy expenditures and multi-organ dysfunction - prevent full rehabilitation - need early excision/grafting of burn wounds, thermoregulation, early + continuous enteral feeding w/ high protein-high carb feedings Also: - wide spread inflammatory response - edema: excess outflow into interstitium cause by increase capillary permeability - may also cause hypovolemic shock (cold clammy skin, decreased PCWP, decreased CO, increased SVR -> treat w/ IV fluid) - Relative polycythemia (from a decrease in plasma volume which makes RBC Mass appear larger)

60 yo man w/ sudden onset substernal chest pain radiating to arm and neck + weakness, nausea, sweating which started while jogging. He has a hx of HTN and S4. He is given sublingual medication that works in 2 minutes. What is the mechanism of action causing relaxation of vascular smooth muscle?

Increasing cGMP - Nitrates (Nitroglycerine sublingual tablets - vasodilate by increasing NO in smooth muscle - dialates veins > arteries -> decreases preload (decreases left ventricular end diastolic volume, decreases LV stress)

80 yo woman w/ SOB and fatigue for 6 months and aortic stenosis and has valve replacement. She is given dicumarol w/ prolongation of PT. How does this medication work?

Induces a vitamin K deficiency-like state - competitive inhibitor of vitamin K epoxide reductase - inhibits vitamin K recycling -> depletion of active vitamin K - prevents formation of active prothrombin + other coagulants and inhibits blood clotting - recognize that PT measures factor VII a major marker for measuring warfarin that also acts to inhibit vitamin K - Factor VII = shortest half life, and most quickly inhibited by warfarin hence why it is used for monitoring

20 yo male, 4 hr of abdominal pain, nausea, vomiting. He was drinking ethanol all weekend, and took three doses of acetaminophen in 2 hrs. why is he at increased risk fo liver injury?

Induction of cytochrome P450 enzymes that activate acetaminophen -> hepatotoxic metabolite - acetaminophen can be conjugated to non-toxic metabolites (sulfate/glucuronide). - In presence of ethanol -> CYP2E1 is induced, increasing the breakdown of acetaminophen -> NAPQ1 (TOXIC to liver) - Overrides ability of liver to breakdown NAPQ1 to cysteine and mercapturic acid (non-toxic) via glutathione

In a severe lower jaw fracture, what nerve can be injured?

Inferior alveolar nerve (branch of V3 -> mandibular nerve of the trigeminal that provides sensation to the lower teeth) - runs along jaw

35 yo w/ 4 days of fever, muscle aches, malaise, loss of appetite, non-productive cough, wife and children also have illness. temp is 102, normal Xray, the virus replicates in nucleus. What virus is it?

Influenza virus - orthomyxovirus, enveloped, negative sense ss RNA w/ 8 segmented genome - has hemmagglutinin (binds sialic acid and promotes viral entry) and neuraminidase (promotes progeny virion release) - can have fatal bacterial superinfection w/ S aureua/pneumo/hflu)

68 yo giving informed consent. Pt says he understands what he has been told, that he is willing to have operation, and that his family can discuss it later. What does the pt have tp fulfill the criteria for informed consent?

Information, Competence, and voluntariness - Consent: disclosure (of info), understanding (comprehension), capacity (ability to reason/make own decision -> not competence which is legal), voluntariness (freedom from coercion/manipulation) - pt must know all risk/benefits, including the option of no treatment - pt can revoke consent, even ORALLY

woman w/ candida albicans treated with caspofungin. What is the mechanism of this drug?

Inhibit fungal cell wall synthesis by inhibiting B-glucan carbohydrates in the cell wall - an Echinocandin along w/ anidulagungin, micafungin - treats invasice aspergillosis and candida - SE: GI upset, flushing (by histamine release) - don't confuse with cell MEMBRANE inhibitors (terbinafine blocks squalene epoxidase and "azoles" block 14-alpha-demethylase in ergosterol synthesis in cell membrane)

67 yo w/ urinary urgency after urinary bladder catheter in prostate surgery. What is the mechanism of action of the drug you use for treatment?

Inhibition of muscarinic receptors - oxybutynin, solifenacin, tolterodine (turtle, Ox) - genitourinary and reduce bladder spasms and urge urinary incontinence (overactive bladder)

27 yo is infertile. He has poor libido, and is unable to maintain erection. He is on TH and corticosteroid after removal of a pituitary adenoma 2 years ago. Serum testosterone is 0.05 (N: 10-35), azoospermia. What can you give him to restore fertility?

Injections of gonadotropins - He has no pituitary adenoma function -> can't give him GnRH as there is no organ to act on - give him instead LH/FSH to initiate testosterone production

79 yo w/ third degree atrioventricular block. What is the appropriate next step in management?

Insertion of a transvenous pacemaker

37 in MVA trauma, multiple rib fractures, RR 30 and labored, hyperresonance in right thorax, decreased tactile fremmitus, no adventitious sounds. What is the afferent pathway of this pateints chest pain is carried by what nerves?

Intercostal - Unlike autonomic nervous system innervating visceral pleura, intercostal nerves are somatic nervous system - enables them to control contraction of muscles and specific sensory information regarding skin and parietal pleura - damage to internal wall of thoracic cavity felt as sharp pain in injured region - Damage to visceral pleura = un-localized ache

5 yo has pain in left leg, imi=migrated from iceland and has had three fractures since birth. She is vegan with no dairy products. She has fracture of fibula. She has a vitamin deficiency that directly affects what processes?

Intestinal calcium absorption - Deficiency in vitamin D - Moved from Iceland -> minimal sun exposure -> minimal D3 (cholecalciferol) from exposure of skin (stratum basale) to sun. ingestion of fish, milk, plants - D2 (ergocalciferol) from eating plants, fungi, yeasts - Both converted to 25-OH D3 (storage in liver) and to activate form 1,25-OH D3 (calcitriol) in kidney - it increases calcium + phos absorption in intestine - increases bone mineralization at low levels and bone resorption at high levels - high PTH, low Ca+ and low phos -> increases calcitriol production (NFB on itself) - lack of vitamin D = Rickets in children from malabsorption, decreased sun exposure, poor diet, CKD, liver disease - give oral vita D to breastfed infants

54 yo w/ rheumatic heart disease come to ER w/ 45 mins of SOB. Pulse is 115, RR 24, BP 110/80, crackles over lower lungs bilaterally. has 2/6 systolic ejection murmur radiating to right side of neck and diastolic murmur at apex. ECG shows afib, and LA enlargement. No ischemia. What is causing her pulmonary symptoms?

Intra-alveolar transudates - increase pulmonary veinous pressure -> pulmonary veinous distention, and transudation of fluid into alveoli - presence of hemosiderin-laden macrophages (heart failure cells) in lung alveoli

What would increase the pulmonary lympatic flow rate?

Intravenous infusion of 0.9% saline for 5 minutes - increasing pulmonary capillaries pressure -> transudative leak of fluid -> increased flow of lymphatics that are closely intertwined with pulmonary capillary beds

45 yo lifelong smoker quit 3 years prior has white patch on lateral border of tongue. Bx shows carcinoma in situ. If this is left untreated what will occur next?

Invasion of submucosa - not dissemination to distant sites -> this would happen second - tongue structure: stratified squamous epithelial layer, basement membrane, lamina propria -> sumucosa w/ glands -> muscle core

2 wk old has asymmetric left pupil and a coloboma of left eye. Incomplete closure of the embryonic fissure limited to what structure spares this pt vision?

Iris - coloboma: hole in one of the structures of the eye, such as the iris, retina, choroid, or optic disc - from a gap (choroid/optic fissure) that fails to close up completely before a child is born - if only a small part of the iris is missing = vision is normal - if a large part of retina or optic nerve is missing, vision = poor and a large part of the visual field may be missing - common in fetal alcohol syndrome

45 yo w/ CHF w/ two first degree relatives dying in their 40s w/ dilated cardiomyopathy and cirrhosis. He has cardiac enlargement, and generalized hyperpigmentation. His serum glucose is 320. Hx of his endocardium will show excess of what?

Iron - Hemochromatosis - AR - C282Y mutation on HFE gene on chromosome 6 - HLA-A3 -Abnormal hepsidin -> tells enterocytes to increase uptake of iron - High serum iron, high ferritin, low Total Iron Binding Capacity, High saturation - "Bronze diabetes" as iron deposits in skin, pancreas, liver, heart - increased risk for HCC - need phlebotomy, chelation w/ deferasirox ("fera")

30 yo Hb 8.2, Hct 25%, MCV 69, reticulocyte 0.8% - what is the diagnosis?

Iron deficiency anemia - microcytic, hypochromic anemia - hemoglobin defect as = heme + globin and heme cannot be made as iron is low (GI bleed, increase demand i.e. pregnancy, menorrhagia) - microcytic think: iron def, lead poisoning, sideroblastic anemia, anemia of chronic disease (body does not release iron), defective globin chain (thalassemia))

Why is rintonavir given with another protease inhibitor in the treatment of HIV?

It increases plasma concentrations of other PIs also metabolized by CYP450 - it itself inhibits CYP450 causing decrease metabolism of other PIs

What is the inheritance pattern of Fragile X? How does transmission change if the mother has fragile X vs. the father?

It is X linked dominant (like hypophosphatemic rickets and Alports) Mothers w/ disease: give it to 50% of daughters and sons Fathers w/ disease: give it to all daughters and no sons

A pt w/ fever from IL-6 induced by IL-1 has activation of the NF-kB signal transduction pathway. What is the role of IkB in this pathway?

It releases NF-kB after undergoing phosphorylation - NF-kB sits bound to IkB in the cytoplasm. An extracellular signal activates IKB kinase (IKK) -> phosphorylates IkB which dissociates from NF-kB allowing its entry into nucleus -> binding of DNA for production of inflammatory cytokine genes - extracellular signal for fever = IL-6

50 yo from Minnesota, 3 day hx of fever, malaise. temp 102, pulse 84, hb 13.6, leukocyte 3500, plt 90,000. He has Anaplasma phagocytophilum. What organism that gave him this infection also can give two other infections?

Ixodes tick (live on deer/mice) -> babesia microti and borrelia burgdorferi - anaplasma: has no rigid cell walls and is primarily intracellular - cause anaplasmosis - will see granulocytes w/ morulae (speckled round inclusion) in them

What kind of protein is deficient in leukocyte adhesion deficiency type 1

LFA-1 integrin (CD18) - impaired migration/chemotaxis - AR - recurrent skin/mucosal bacterial infections that are slow to respond to antibiotics - absent pus/neutrophils - delayed wound healing and >30 day separation of the umbilical cord - increased neutrophils in blood, none at infection site

30 yo woman w/ 20lb weight loss after low-carb diet, low-fat diet. What should she include in her diet to maintain good nutrition?

Linoleic acid - biosynthesis of arachidonic acid (AA), prostaglandins, leukotrienes, thromboxane (TXA) - in lipids of cell membranes - in nuts, fatty seeds (flax seeds, hemp seeds, poppy seeds, sesame seeds, etc.) and vegetable oils - consumption of linoleic acid vital to health = essential fatty acid - Essential amino acids: cannot be made in body = histidine, isoleucine, leucine, lysine, methionine, phenylalanine, threonine, tryptophan, and valine

16 yo w/ bulimia brought in by mom from nonbloody diarrhea. Mom thinks she is using laxatives. RR 30, BP 89/61, pulse 120. What are her potassium, chloride, and bicarb levels?

K+ = decreased Cl- = increased Bicarb = decreased - Fluid loss w/ dehydration, electrolyte loss (sodium, potassium, magnesium), and vascular collapse sometimes occur - Bicarbonate loss can cause metabolic acidosis - Hypokalemia can occur when patients have severe or chronic diarrhea or if the stool contains excess mucus - Hypomagnesemia after prolonged diarrhea can cause tetany -Patients with diarrhea often develop potassium depletion owing to large faecal losses of this ion; these losses are greatest malnourished who are potassium-deficient before diarrhea starts - diarrhea: normal anion gap acidosis and lose na+ and potassium in diarrhea, and secrete H+ and potassium as they are acidodic

60 yo man with no hx of bleeding problems has elevated PTT what molecule in the kinin cascade can be deficient?

Kallikrein -> leads to a build up of high molecular weight kininogen that activates the intrinsic pathway -> elevated PTT

Male who smokes a pack of cigs, with polycythemia, hypercalcemia, microscopic hematuria has a brain mass bx. Staining is positive for epithelial membrane AG, and appears as round white, space filled cells. What is the site of origin for this met?

Kidney - polycythemia from increased EPO - clear round cells - hematuria - will produce: PTHrP (hypercalcemia), ectopic EPO, ACTH, Renin = PEAR - aldesluekin (IL-2 analog) = treatment

What organ besides the liver can release glucose in times are starvation?

Kidney - gluconeogenesis occurs mainly in liver to maintain euglycemia, but kidney and intestinal epithelium also have the same enzymes (glucose-6-phosphatase, fructose 1,6, bisphosphatase, phosphoenolpyruvate caroboxylase, pyruvate carboxylase)

64 yo man with hx of alcoholism, disoriented, jaundice, spider angiomata, flapping of hands, abdominal distention, dullness in flanks to percussion. Neomycin is begun. What does this do?

Kills bacteria in the gut that generate ammonia - also rifaximin does same thing - lactulose: increases NH4+ Production - Hepatic encephalopathy: cirrhosis -> portosystemic shunts (blood no longer flows through liver) -> decreased NH3- metabolism -> neuropsych issues (asterixis -> coma). - caused from: increased NH3- production/absorption (dietary protein, GI bleed, constipation, infection) - Decreased NH3- removal: (renal failure, diuretics, TIPS shunting)

If given the concentrations of a substrate [S] and the velocity of which an enzyme acts on that substrate (V), how can you determine the Km if the enzyme follows Michaelis-Menten kinetics? ex) An enzyme is saturated at 2.0 (vmax) when [S] = 20, and even at [S] = 200. At V= 1.0, [S] = 0.6. What is the Km

Km = [S] at 1/2 Vmax - meaning look at the velocity at which the enzyme is saturated (Vmax) and find half of that value. The concentration of the substrate at 1/2 Vmax = the Km - in the example the Km would be 0.6 as this is the substrate concentration at 1/2 Vmax

65 yo woman w/ hypertension -> ER w/ headache, chest pain, SOB, on aspirin, furosemide, lisinopril. Respiration 20, BP 230/110. Wants to treat w/ nonselective a1/B antagonist. What can they use?

Labetalol - Carvedilol also non-selective

in a 62 yo male w/ BP 220/160 wants a drug to decrease HR and SVR. What can you us?

Labetalol - alpha and beta antagonist can be used in HTN emergency - reduces afterload via vasodilation - also can be used in pregnancy - used IV

a study on cough medication studied 120 pt under age 12 getting dextromethorphan or no tx. parents rated their child's cough after receiving the medicine or nothing. findings were significant. what limits the internal validity of this study?

Lack of blinding between groups - creates bias and ruins internal validity/ quality of the experiment

56 yo man with rotator cuff injury underwent operation. to strengthen his infraspinatus and teres minor what movements should he perform against resistance?

Lateral (external) rotation - infraspinatus -> suprascapular nerve - teres minor -> axillary nerve Medial (internal) rotation and adduction - subscapularis -> subscapular nerves abducts arm first 15 degrees - supraspinatus -> suprascapular nerve - most common rotator cuff injury

following a stroke a pt is hoarse and cannot detect pinprick or cold on left side of face or right side of body. Damage likely occurred in what area of the brain?

Lateral medulla - posterior inferior cerebellar artery stroke - nucleus ambiguus (CN IX, X, XI) = dysphagia, hoarseness, decreased gag reflex, hiccups (Don't PICA hoarse that can't eat) - vestibular nuclei = vomiting, vertigo, nystagmus - lateral spinothalamic tract, spinal trigeminal nucleus = decreased pain + temp from controlateral body and same side face - sympathetic fibers = horner syndrome (ptosis, anhidrosis, miosis) all from lack of sympathetic innervation - inferior cerebellar peduncle = ipsilateral ataxia, dysmetria

a 29 yo has bilateral jaw pain after trying to each a double cheeseburger. PE shows drooling, and dislocation of temporomandibular joints. Reduction is suggested. relaxation of what muscles will facilitate this procedure?

Lateral pterygoid - the only muscle that opens the mouth (lateral lowers jaw) - 3 close jack (masseter, temporalis, medial pterygoid) - all innervated by trigeminal V3 - it takes more muscle to keep your mouth shut - TMJ = too tight fibrous capsule/thinning of disc - reduce pain/irritation via losening fibrous capsule surrounds TMJ joint which also surrounds lateral pterygoid that makes up this joint

Prior to screening test, the overall 5-year survival rate was 70%. After initiating a new screening tool, the 5 year survival is 85%. In this time there were no changes in treatment of prostate cancer. The apparent decrease in mortality is from what bias?

Lead-time bias - early detection is confused with increased survival - early detection makes it seem like survival has increased, but the disease's natural course is unchanged - don't confuse w/ length-time bias: screening detects diseases with long-latency periods while those with shorter latency periods are symptomatic earlier (slowly progressing cancer is more likely detected by screening test than rapid/aggressive cancer)

a 19 yo in car crash has wound in R cerebral cortex -> complete paralysis of left lower extremity, fracture of the right mid-humerus w/ severed radial nerve, and fracture of the right tibia. Which deep tendon reflex is going to be strongest?

Left achilles tendon - The upper motor nerve injury to the right cerebral cortex causes an UMN injury which inherently -> hyperreflexia in the injured leg that would be seen in an achilles tendon test (S2) on the left side (contralateral to injury)

Full term newborn has respiratory distress and herniation of abdominal contents into left pleural cavity. Maldevelopment of what structures is causing this defect?

Left pleuroperitoneal membrane - usually on left side due to protection of right hemidiaphragm by liver - NOT left diaphragmatic crus (central structure

52 yo woman w/ right dominant heart had angioplasty of circumflex artery, which structure will now have increased blood flow?

Left ventricle - Left circumflex supplies lateral and posterior wall of LV and anterolateral papillary muscle - LAD: anterior 2/3 interventricular septum and anterolateral papillary muscle and anterior surface of LV - PDA supplies posteriomedial papillary muscle -> increased risk of posteriomedial papillary rupture of mitral valved = mitral valve prolapse in acute MI

51 yo farmer had a cut on his foot, and DM type II. elevated temp, erythema to the mid-calf, edema, and tenderness of the ankle and around laceration with elevated leukocyte count. What kind of response is this?

Leukemoid reaction - must differentiate this from chronic myelogenous leukemia that also has elevated WBC - CML will have very low LAP (leukocyte alkaline phosphatase) vs. high LAP in bening neutrophilia aka leukomoid rxn

66 yo w/ 2 month hx of inability to maintain erection. He has fatigue, difficulty sleeping, and concentrating. 3 months ago he has cerebral infarction w/ right hemiparesis that resolved. PE shows no abnormalities. Will his libido/nocturnal erections be normal, decreased, increased?

Libido = decreased nocturnal erections = normal

In a man w/ fever after transurethral resection of prostate, low BP, and lactose-positive, gram-negative rods, what describes the event that develops hypotension?

Lipopolysaccharide stimulation of toll-like receptor - innate immunity: toll-like receptors: pattern recognition that recognize PAMPS (pathogen-associated molecular patterns) that lead to activation of NF-KB - examples of PAMPS: LPS from gram neg bacteria, flagellin from bacteria, and nucleic acids from viruses - mediator of acute inflammation - LPS endotoxin on E. coli -> septic shock - leading cause of UTI

24 yo man 8 hr hx of abdominal pain, nausea, vomiting. Has fam hx of pancreatitis and HLD. Serum shows glucose of 90, TG of 3500, and lipase of 400 (N=14-280), and serum looks milky. What is deficient?

Lipoprotein lipase - degrades TG in circulating chylomicrons - pancreatic lipase degrades TG in small intestine - hormone sensitive lipase: degrades TG in stored adipocytes - Familial hyperchylomicronemia (Type I dyslipidemia, AR) - no lipoprotein lipase or apoC-II - increased chylomicrons, TG, cholesterol - pancreatitis, hepatosplenomegaly, eruptive/pruritic xanthomas (no increase risk for atherosclerosis), creamy layer in supernatant

35 yo w/ IV drug use has not used for 10 years. He consumes 6 beers daily. He is not sexually active and has no interest in relationships. He has gynecomastia, hypogonadism and spider angiomata. No hepatosplenomegaly, ascites, or edema. What organ has dysfunction?

Liver - cirrhosis -> due to estrogenic state gives you the findings listed - can get cirrhosis from chronic hepatitis that he may have from being IV drug user/drinking alcohol - just has not worsened to portal HTN - hyperestrogen in cirrhosis: decrease testosterone: estrogen ratio (spider angiomata from estrogen), lack of impotence (from too little testosterone), gynecomastia (from decreased testosterone or too much estrogen) in adolescence get gynecomastia too much estrogen and why newborns can lactate from top much estrogen from mom - liver dyfuntionc: too much estrogen -> can't break down estrogen, and cant make Sex binding globulin which makes more free estrogen

2 pt -> 54 yo man (GFR 60), and 76 yo woman (GFR 20) are going to receive IV vancomycin. Both weigh 70kg. What about this drug administration will be the same?

Loading dose - loading dose = target plasma concentration in steady state X Vd/ bioavailability - in renal/liver disease maintenance dose decreases but loading dose remains the same - time to steady state depends on half life and is independent of dose/dosing frequency

Severely weakened girl, decreased muscle strength, and muscle biopsy shows abnormal mitochondria in subsarcolemmal regions. What do you expect her Max O2 consumption, venous blood lactate, and energy production by glycolysis to be?

Mac O2 consumption = decreased Venous lactate = increased glycolysis = increased - her mitochondria are deficient -> this means no aerobic respiration -> increased glycolysis and no need for O2 and profound weakness - mitochondrial disease = MERRF

What drugs interfere with DNA synthesis by crosslinking?

Lomustine and cyclophsphamide - lomustine: Nitrosoureas also have carmustine -> require bioacitvation, cross BBB-> CNS cross links DNA - tx brain tumors (glioblastoma) and can cause CNS toxicity (convulsion, dizziness, ataxia) - cyclophosphamide: cross-links at guanine, need bioactivation by liver, a mustard gas - tx solid tumors, leukemia, lymphoma, rheumatic disease, SLE, granulomatosis w/ polyangitis - SE: myelosupression, SIADH, fanconi syndrome (ifosfamide), hemorrhagic cystitis + bladder cancer (prevent w. mesna = sulfa group binds toxic metabolites) and hydration - other alkylating agents: busulfan (pulm firbosis), procarbazine (causes leukemia)

50 yo has PE and tx is started with heparin. 24 hrs later warfarin is added. On day 2 his PTT is increased and PT and INR is normal. What explains the normal PT time and INR in this pt?

Long half-life of factor II (prothrombin) - factor two has the longest half life and thus takes longest to degrade - this measures VII (extrinsic pathway), and X, V, II, I of common pathway - his PTT is already being prolonged by warfarin, but would most likely also be normal if no heparin was bring given - hence the warfarin just hasn't kicked in yet - onset of action = 8-12 hours, full effect takes 3 days

scuba diver ascends to fast and gets decompression sickness w/ small gas bubbles in vessesl supplying dorsal white matter of upper thoracic spinal cord. What is the outcome of this injury?

Loss of discriminative sensation in the lower extremities - Damage to dorsal column = pressure, vibration, fine touch, and proprioception - fasciculus gracilus (lower body, legs) - Fasciculus cuneatus (upper body, arms)

18 yo has sepsis after abortion, and becomes dyspneic, oliguric, and has petechiae, ecchmoses, and bleeding from IV sites. What will her levels of fibrinogen be?

Low - DIC: widespread activation of clotting factors, decrease clotting factors -> bleeding state - from sepsis (gram neg), trauma, OBGYN, pancreatitis, malignancy, nephrotic syndrome, transfusions - Labs: schistocytes, increased fibrin degredation products (d-dimer elevated), decreased fibrinogen, decreased factors V/VIII

Woman wants to get stool for occult blood to see if she has cancer -> doctor prefers a colonoscopy as they are worried that the stool occult test has what?

Low sensitivity - Many people will have blood in stool but for various reasons, no just because of cancer - So think: many people with cancer will not have positive occult blood and still have cancer, and few will have blood + cancer (true positives/total cancer cases = low, not the most sensitive test)

13 yo boy to get chemo for leukemia, has malignant lymphocytes. The cells do not express CD4, CD8, surface IgM or IgG, cytoplasmic IgM, u-heavy chain, cytoplasmic IgG, and y-heavy chain. The do express MHC class I and have rearrangement of T-lymphocyte receptor B-chain gene D and J. What is the normal counterpart of these malignant lymphocytes?

T-lymphocyte thymocytes localized in the thymic cortex

65 yo woman is vomiting bright red blood, BP 70/30, in hypovolemic shock. Placement of a catheter in internal jugular vein for blood transfusion is planned. Improper insertion of cath will damage what underlying structures?

Lung and common carotid artery - internal jugular sits right next to common carotid - central veinous lines: can cause a lot of damage -> can entirely misplace it (carotid/vertebral artery) - can also cause a pneumothorax - with subclavian and internal jugular line placement

11 yo w/ pain in knee. X-ray shows osteolytic mass eroded through the cortex and elevated periosteum. Mass surrounded by reactive bone. Bx shows atypical cells w/ hyperchromatic pleomorphic nuclei surrounded by eosinophilic matrix, some of which is calcified. Where will this tumor spread first?

Lungs - osteosarcoma - most commonly spreads to lungs

If elastin has a decreased number of desmosine cross-links, what amino acid is most likely abnormal?

Lysine - elastin is made of glysine, valine, alanine, and lysine and the crosslinks use lysine to form cross-links - Similar to collagen that has hydroxylation of proline and lysine residues - Collagen synthesis: hydroxylation of proline/lysine residues on preprocollagen -> hydrogen/disulfide bonds procollagen (triple helix = issues = osteogenic imperfecta) -> exocytosis of procollagen into extracellular space -> cleave disulfide terminal regions -> insoluble tropocollagen (no cleavage = ehlers danlos) -> crosslinking of tropocollagen via lysl oxidase (covalent lysine-hydroxylysine cross-linkage via copper containing lysyl oxidase) = collagen fibrils - no cross linking = ehlers-danlos syndrome, menkes disease

5 yo with recurrent middle ear infection and URIs. Impaired motor and cognitive function, hepatosplenomegaly, inclusion bodies in fibroblasts, and deficiency of N-acetylglucosamine-1-phosphotransferase. Cells make a lot of acid hydrolases, adn are unable to target what organelle?

Lysosomes - I-cell disease (inclusion cell disease) - lysosomal storage disorder - golgi cannot phosphorylate mannose residues (decreased mannose-6-phosphate) and proteins are excreted extracellular rather than -> lysosomes - coarse facial features, gingival hyperplasia, clouded corneas, restricted joint mvmnt, claw hand deformity, kyphoscoliosis

20 yo has acute onset confusion after an all-night dance party. Pulse is 140. BP 180/110, PE shows pale, cold extremities, no diaphoresis. ECG shows sinus tachycardia. What drug did he take?

MDMA - 3,4 methylenedioxymethamphetamine - intoxication = hallucinogenic stimulant -> euphoria, disinhibition, hyperactivity, distorted sensory and time perception, bruxism, life threatening effects of HTN, tachycardia, hyperthermia (no sweating), hyponatremia, serotonin syndrome - dance party = MDMA - amphetamine sympathomometic + anticholinergic (dry mouth, and no sweating) - or volume depleted and not sweating

30 yo woman with long standing PID had surgical removal of tube. What inflammatory cells will most likely be in this tissue?

Macrophages - chronic inflammation - characterized by mononuclear infiltration (macrophages, lymphocytes, plasma cells) -> tissue destruction and repair (with angiogenesis/fibrosis) - macrophages are the dominant cell from result of their interaction with T lymphocytes - acute = neutrophils, eosinophils, basophils, complement, mast cells -> 2/3 days later macrophages to clean up debris or call in more mediators if more work needs to be done

What allows for complete healing of the lung tissue following pneumonia?

Maintenance of the basement membrane integrity

45 yo has yellow skin for 4 days. has been drinking excessively for ten days. liver is tender to palpation. Bili is high, AST 110, ALT 69. Liver bx will show what?

Mallory hyaline (bodies) - alcoholic hepatitis - sustained, long-term consumption - swollen, necrotic hepatocytes w/ neutrophilic infiltration - mallory bodies (intracytoplasmic eosinophilic inclusions of damaged keratin filaments) - AST>ALT (about 2X as much 2:1 ratio)

3 month old w/ facial abnormalities, weakness since birth. coarse facial features, hypotonia, urinary excretion of mucopolysaccharides are normal, concentration of lysosomal enzymes increased in serum, and decreased in cultures skin fibroblasts. What molecular marker is decreased in the pt lysosomal enzymes?

Mannose 6-phosphate - I-cell disease - lysosomal storage disorder, defect in N-acetylglucosaminyl-1-phosphotransferase -> failure of golgi to phosphorylate M6P on glycoproteins, and protiens are secreted extracellularly rather then delivered to lysosomes - course facial features, gingival hyperplasia, clouded corneas, restricted joints, claw hand deformities. Often fatal

Boy w/ mental retardation and obese with Prader-Willi syndrome has karyotyping and fluorescent in situ hybridization. There is no usual deletion in chrom 15. What is another way this child may have this disorder?

Maternal origin of both chromosomes 15 - 75% is from a paternal gene that is inappropriately silenced - 25% is from inheriting two maternal chrom that are not meant to be imprinted (silenced) so the child is left with no chrom 15

In a baby with both a cleft lip, what two structures failed to fuse?

Maxillary and medial nasal prominences - Cleft lip: failure of fusion of the maxillary and merged medial nasal processes (formation of primary palate) - Cleft palate: failure of fusion of the two lateral palatine shelves or failure of fusion of lateral palatine shelf w/ nasal septum and/or median palatine shelf - distinct multifactorial etiologies but often do occur together

22 yo w/ slash transversely over the palmar aspect of distal forearm 2 cm proximal to distal palmar wrist crease. He can't abduct the left thumb, and sensation decreased over palmar aspect of the thumb, index, middle finger. What nerve is injured?

Median nerve

What cells are you hoping to suppress by giving prednisone to someone with Crohn's?

T-lymphocytes - forms granulomas and lymphoid aggregates via TH1 mediated disease

40 yo w/ mole on back increased in size in 4 months. irregular lesion w/ variegated black-tan pigmentation and ill-defined margins. Tissue shows pleomorphic, hyperchromatic cells in clear islands that tend to coalesce and are present at all levels of epidermis w/ extension into papillary dermis. What is it?

Melanoma

When having a heart attack, what is happening to the cells that allow myocardial creatine kinase (CK-MB) to be elevated in serum?

Membrane lipid peroxidation - increased free radicals -> steal electrons from lipids in cell membranes -> cell permeability/damage (decreased ATP, increase ROS as antioxidants can no longer work as well) - prolonged decrease in ATP -> in cell injury -> irreversible cell injury -> rupture of lysosomes and autolysis, plasma membrane damage (degradation of membrane phospholipid) -> leakage of cytosolic enzymes into serum, adn influx of Ca++ activating lysosomal enzymes -> necrotic cells death

what will be the intracellular concentrations of Na+, K+, Ca+ in a myocardiocyte that just had an MI?

Na+ = increased K+ = decreased Ca+ = increased -decrease ATP -> decrease activity of Ca+ and Na+/K+ pumps -> cellular swelling

12 yo girl w/ seizure, mental retardation, scoliosis. Had repair for dislocation of lenses in both eyes 3 years ago. Her plasma cystathionine is decreased, CT of head shows small infarction in right cerebral hemisphere. What amino acid is increased in this patient?

Methioine - she has homocystinuria - she has a cystathionine synthase deficiency (turns homocysteine -> cystathionine -> cysteine) - shunts homocysteine -> methionine via methionine synthase - increased methionine - tx: decrease methionine, increase cysteine/B6, B12, folate - other mutations: decreased affinity of cystathionine synthase for B6 (give more B6) - methionine synthase deff (increase methionine in diet) - MTHFR (methylenetetrahydrofolate reductase) deff (tx w/ increased folate in diet) - all forms: excess homocysteine - homocystinuria, osteoporosis, marfanois habitus, ocular changes (down and in), thrombosis, atherosclerosis, stroke, MI, kyphosis, intellectual disability, fair complexion

7 yo from developing country with lack of protein. What protein is most critical for her diet at this time?

Methionine - essential amino acid (not made in body and must be obtained from diet) - other essentials: PVT TIM HaLL = phenylalanine, valine, tryptophan, threonine, isoleucine, methionine, histidine, leucine, lysine - Glucogenic: Methionine, histidine, valine (I MET HIS VALentine, shes so SWEET) - gluconogenic/ketogenic: isoleucine, phenylalanine, threonine, tryptophan - ketogenic: leucinem lysine (pureLy ketogenic aa) - acidic: aspartic acid, glutamic acid (- charge in body) - basic: arginine, histidine, lysine (HIS LYS ARG basic)

Isolated genomic DNA from a mutant strain of E coli can now be digested into small fragments by restriction endonuclease that recognizes GATC sequence. Wild type E Coli cannot be digested. The mutation that causes this type of phenotype occurs in gene encoding for what activity?

Methylase - deoxyadenosine methylase: adds a methyl group to adenine of sequence 5'-GATC-3' in newly synthesized DNA. Immediately after DNA synthesis, the daughter strand remains unmethylated for a short time - This prevents the restriction endonuclease described in this question from accessing the GATC zone. If this enzyme is mutated, then the enzyme can access this area for DNA cleavage into fragments - restriction endonucleases = cleave DNA and are found in bacteria as protection against foreign DNA (virus) - used in molecular cloning

22 yo w/ foul smelling diarrhea and farts. 10 lb weight loss after trip to Indonesia, where he did not have access to clean drinking water. What is the mechanism of the drug used to treat him?

Metronisdazole - forms toxic free radical metabolites in bacterial cells that damage DNA - bactericidal and antiprotozoal - treats giardia here, and entamoeba, trichomonas, gardnerella, anaerobes (bacteroides, c. diff) and can be used in place of amoxicillin in H Pylori "triple therapy" if they have penicillin allergy - anaerobes below the diaphragm (intrabdominal infection) vs. clindamycin (above diaphragm) - can cause disulfuram like reaction = flushing, tachycardia, nausea, vomiting after alcohol

4 yo back from trip in Louisiana where he was barefoot. conjunctiva are pale, and eosinophils 15%. stool prep shows a parasite egg. What is causing fatigue?

Microcytic anemia - ancylostoma and necator americanus hookworms - suck blood from intestine wall - have cutaneous larva migrans (pruritic, serpinginou rash from walking barefoot on contaminated beach) - larvae penetrate skin - albendazole or pyrantel pamoate

14 yo w/ migraine headaches, vomiting, and focal seizures. Hearing loss since age 11, and mother/grandmother have tone deafness. She has loss of vision in one half of visual field, and weakness in RUE/LE. Increased lactic acid in CSF. What mutation does she have?

Mitochondrial tRNAleu - transmitted through mother w/ variable expression from heteroplasmy (mito are divided up into cells differently -> some may get a lot fo bad mito and some may not get any) - Mitochondrial myopathies: rare -> myopathy, lactic acidosis, and CNS disease (MELAS syndrome = mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes) from failure in oxidative phosphorylation - muscle bx shows ragged red fibers (from accumulation of diseased mito in subsarcolemma of muscle fiber)

47 yo severe chest pain for 2 hrs, pulse 110 and thready, BP 90/70, ox sat 85%, cold, clammy skin. Crackles heard bilaterally. ECG = 4mm ST-elevation in V4-V6. CO 2L/min. What is decreased in this pt?

Mixed venous oxygen tension - this is measured by a sample from pulmonary artery catheter and tell us venous O2 content - the O2 remaining in blood after tissue extraction of O2 - sepsis/shock -> increased oxygen extraction from tissues as less blood is present for oxygenation -> more hypoxic tissue increased unloading of O2 from Hb -> less O2 in returning venous blood - V4-V6 = LAD infarct (V3-V4 = anteroapical/distal LAD, and V5-V6 = anterolateral LAD or LCX) - here he is going into cardiogenic shock from acute MI -> cold clammy skin from increased SVR, increased PCWP, very decreased CO - needs ionotropes/diuresis

85 yo woman with broken femur started on morphine. Three days later has low respirations (6/min), pinpoint pupils, and serum creatinine is 1.8. Why?

Morphine is metabolized to active metabolites that accumulate and are excreted by the kidneys causing toxicity

What are the major differences between Hep A and Hep E

Mortality among pregnant women - both are RNA viruses - fecal-oral transmission - no chronic state - short duration - Hep E = poor prognosis in pregnant women - NO HCC risk

40 yo w/ orthostatic hypotension and loose stools for 1 year. He has 26 year hx of type I DM, and stool studies are normal. What is the cause of this diarrhea?

Motility Disorder - diabetic neuropathy = motor, sensory, and autonomic degeneration - here, he has autonomic degeneration (too much parasympathetic response)

3 month old can lift her head while laying prone. She is eating well, and is sleeping well. She has not yet smiled in response to human faces and has not started to coo or gurgle w/ attention. Are her motor, social, and verbal/cognitive milestones normal or delayed?

Motor: normal social: delayed verbal/cognitive: delayed - at 3 months: moro reflex disappears (arms spreading when falling) - social smile by 2 months - lifts head up prone by 1 month - babbling

30 yo in bike accident hit shoulder hard on ground. He cannot flex right elbow, and no sensation to pinprick on right lateral forearm. What portion of the brachial plexus is injured?

Musculocutaneous nerve (C5-C7) - upper trunk compression - decreased biceps (C5-6) or triceps (C7) reflex - weakness of forearm flexion and supination - loss of sensation over lateral forearm

24 yo w/ 3 day hx of numbness in both feet now has ascended to his thighs. He has numbness/tingling in hands, ataxic gait. DTR gone in UE/LE. Sensation to vibration and joint position is absent in fingers/feet b/l. A defect in what structure explains these findings?

Myelinated primary afferents - Acute inflammatory demyelinating polyradiculopathy aka Guillain-Barre syndrome - autoimmune destroys Schwann cells via inflammatio/demyelination of motor/sensory fibers and peripheral nerves. - from molecular mimicry/triggered by stress - even though it occurs with capylobacter there is no real association with viruses - symmetric ascending weakness/paralysis and decreased DTR beginign in LE - may see autonomic irregularities - can recover completely - need respiratory support - plasmapheresis /IV IG - immune medited polyneuropathy - symmetric, ascending muscle weekness and absent DTR - someitmes autonomic and sometimes peripheral CN invovled - Miller fischer: opthalmoplegia, areflexia, and ataxia - immune responce that cross rexn with perihperal nerves and damaged schwan cells - enahncement in thickening in intrathecal nerve roots and cauda equina - LP: albunocytological disassociation -> same as MS increased albumin, and normal WBC - slow nerve conduction velocity, and reduce amplitude in muscle AP - outcomes are better with plasmophoresis and IVIG - concern -> AV block or respiratory failure - 90% recover spontaneously - post-viral infection and bacterial (campylobacter/mycoplasma/EBV/Zika)

63 yo w/ left upper quadrant fullness, lethargy, SOB, 20lb weight loss, pallor, palpable spleen tip. Hg 9, Hct 27%, leukocyte count 4000, serum uric acid 15. Blood smear shows erythrocytes w/ abnormal shapes/sizes, nucleated erythrocytes, myelocytes. Aspiration of bone = dry tap. Bone bx = thickened bony trabeculae and increase retinaculum. What does he have?

Myelofibrosis - proliferation of mature myeloid cells especially megakaryocytes - 50% JAK2 kinase mutation - megakaryocytes make excess PDGF -> marrow fibrosis - splenomegaly from extramedullary hematopoiesis (bone marrow fibrosed) and stem cells go back to where they used to make cells in fetus (spleen/liver) - smear: tear drop cells (some RBC still trying to squeeze out of fibrosed bone marrow), nucleated RBC, immature granulocytes (usually reticulin gates in bone marrow prohibit immature cells from leaving bone marrow) - risk of infection, thrombosis, bleeding as spleen cannot make enough cells to compensate for the large # of cells the bone used to make

17 yo w/ 1 day hx of SOB, weakness, muscle tenderness after triathlon day prior. High temp, RR 20, BP 150/90, crackles in lungs, serum creatinine is 4, UA: 3+ protein, 4+ hemoglobin. What substance is causing this?

Myoglobin - in extreme states of exercise under hot conditions, severe rhabdomyolysis can occur causes breakdown of type I muscle fibers -> slow twitch red fibers, w/ increase mito/myoglobin and increased oxidative phosphorylation - increased myoglobin can cause AKI - need aggressive hydration

40 yo goes horseback riding 3-4 times weekly and develops swollen mass on left inner thigh. Over the next 2 weeks the mass becomes circumscribed and very firm. What is this?

Myositis ossificans - the formation of bone within muscle after trauma - a form of metaplasia - reprogramming of stem cells and full replacement of one cell type by another that get better adapt to a new stress/irritant - a metaplasia of connective tissue - bone tissue forms inside muscle or soft tissue after an injury - young adults + athletes - occurs in the large muscles of the arms or the legs

54 yo w/ sudden loss of vision in left eye, most of vision returned in a day + 3 month hx of SOB w/ exertion. ECHO shows mass in LA. What is this?

Myxoma - systemic emoboli can cause blindness - SOB as the pedunculate mass can block mitral valve -> obstruction cause syncopal episodes or SOB - they allow stasis of blood and can even emoblize pieces of themselves.

19 yo man w/ SOB and dizziness. w/ and ECHO showing a heterogeneous pedunculate mass in the left atrium. What is it?

Myxoma - 90% in left atrium - "ball valve" - most common primary tumor in adults - most common tumor = metastasis (melanoma) - obstruction of the LA (have multiple episodes of syncope) - may have early diastolic "tumor plop" - Hx: gelatinous material, myxoma cells in glycosaminoglycans

2 yo girl w/ strabismus. If not treated she will have deficits in depth perception from lack of competitive interactions in visual cortex. calcium entry through which receptor mediates the outcome of this competitive process?

N-methyl-D-aspartate (NMDA) - receptor antagonist that helps prevent excitotoxicity (mediated by Ca+) - drug target of memantine - used for alzheimers - glycine and GABA: use chloride currentness = inhibitory receptors in CNS (mediates inhibitory NT in the spinal cord and brainstem) - glutamate = excitotoxicity (G-protein) - serotonin = G-protein excitatory/inhibitory NMDA: channels: needs glutamate and glycine to open and magnesium blocks channesl - Once open: sodium but more calcium flows in - Needs to reverse potential before opening - Help in learning and memory by leading to calcium signaling that upregulate receptors that are both glutamate receptors -> and now the NMDA receptors are more readily open in response to glutamate -> the more you stimulate a pathway the more responsive you are to this new information hence why memantanine is used in NMDA are used in alzheimers = antagonist - NMDA will only open at 0 mV - PCP, ketamine are all NMDA antagonists -> dissociative drugs as this is also associated with arousal

50 yo w/ fever, SOB, and sputum grows gram positive diplococci. What enzyme kills the diplococci?

NADPH oxidase - when bacteria are phagocytosed -> rapid release of reactive oxygen species -> kills bacteria - deficiency in NADPH oxidase = chronic granulomatous disease -> at risk for infection with catalase positive species like s. aureus, aspergillus (catalase breaks down bacteria H2O2 that could otherwise be used by deficiency cells to make ROS)

Baby with pyloric stenosis and vomiting will have what lab values for Na+, K+, Cl-, HCo3-?

Na+ 132 (low) K+ 3.2 (normal low) Cl- 90 (low) HCO3- 37 (high) - pyloric stenosis - most common outlet obstruction - palpable olive mass, visible peristalsis, non-bilious projectile vomit at 2-6 weeks - hypokalemic hypochloremic metabolic alkalosis (from vomiting gastric acid and volume contraction -> dehydration -> increased ALDO and decrease K+)

What transporter does oral fluid therapy act on in someone infected with vibrio cholerae?

Na+/Glu co transporter - gram neg, comma shaped flagellated (attach to intestine wall), oxidase positive - grows in alkaline media - rice water diarrhea - endotoxin activates Gs -> Increased cAMP - sensitive to stomach acid (acid labile) and needs high inoculation # unless pt on PPI (decreases stomach acidity) - uncooked shellfish/water -> fecal-oral non invasive - oral rehydration solution: replenishes h2o and solutes lost in diarrhea -> solution has water, chloride, K+, Na+, glucose - Na+ and glu are absorbed and help water be reabsorbed as well

55 yo found unconscious w/ bottles of albuterol, ampicillin, codeine, theophylline. Temp is 99, pulse 112, RR 6, BP 95/60. Acute tx includes what?

Naloxone - opioid receptor antagonist - works immediately to reverse the respiratory depression

Studying strep pneumo. several strains express a capsular type on present on strep. mitis. They incubate non-encapsulated S. pneumo w. the lysate from heat-killed s. mitis and new colonies of s.pneumo express a new capsular type. no colonies expressing this capsule are noted after adding DNase to the lysate. What is causing this gene transfer?

Natural transformation - the killed environmental naked DNA of the capsuled s. mitis is in environment after cell lysis - incorporates the scattered DNA into cell and adds it to cell's DNA - DNA fragments are ruined when DNase is added -> destroys lose naked DNA for transformation - transduction: phages infect bacterium destroys cell DNA, repackages new cell DNA into capsid, and infects another cell transferring DNA

A person who is starved for 2 weeks with water lost weight. What is the balance of his nitrogen?

Negative nitrogen balance -> in catabolic state with no consumption of proteins vs. anabolic which would be positive balance

What is absolute risk reduction?

the difference in risk attributed to the intervention compared to control (if 8% get flu w/ placebo vaccine vs. 2% with vaccine = 0.08-0.02 = 0.06 or 6% difference ex) drug preventing stroke vs. standard treatment: standard tx has 0.12 strokes new drug has 0.04 strokes -> ARR = 0.08 or 8%

46 yo retired boxer w/ sleep disturbances, mask-like features, stooped posture slow/rigid movements, shuffling gait, resting tremor. What is causing this?

Neuronal degeneration - early onset parkinson's (like Muhammed Ali) from repetative TBI leading to increased neurodegeneration

Poliovirus mRNA lacks a 5' m7G cap but is translated efficiently by cellular ribosomes. Which of the following additional structural features of poliovirus mRNA is the most likely cause of its ability to be translated in the absence of a cap?

Presence of an internal ribosome entry site - the 7-methylgaunosine cap prevents degradation of the mRNA molecule and assists in binding to the ribosome for translation - without it they will need to have an alternate way to bind to the ribosome for translation

52 yo w/ blindness in left eye. funduscopy -> pale, opaque fundus and bright red fovea centralis. Visual field testing = dense scotoma over entire visual field of left eye. nothing wrong w/ right eye. If the left eye is illuminated what reaction will the right eye have?

No constriction bc the retinal ganglion cells in the left eye have been destroyed - central retinal artery occlusion - acute, painless monocular vision loss. Retina is cloudy w/ thin vessels and cherry red fovea - evaluate for embolic source

26 month old boy, 3 day cough, SOB, fever. He has many cutaneous abscesses from staph. aureus in the past 9 months. His oxygen sat is 93%, crackles are heard over lungs, chest x-ray show cavitary lesions in lingula. cultures grow filamentous gram-positive rod. what is causing this?

Nocardia asteroides - He most likely has chronic granulomatous disease - deficiency of NADPH oxidase means no ROS made in oxidative burst to kill organisms - If organisms have catalase -> they degrade their own H2O2 so the cell can use this to make ROS - X-linked - dx: abnormal dihydrorhodamine test (less green flourescence) - and Nitroblue tetrazolium dye fails to turn blue

12 yo boy is beginning puberty. What causes the physical changes of puberty?

Nocturnal luteinizing hormone pulses - pulsatile GnRH leads to puberty - also increase in growth hormone - begins spermatogenesis

14 yo boy w/ breast tissue is embarrassed. He is normal height/weight. PE shows b/l 1.5cm masses, normal penis/testes, tanner stage 3. Serum gonadotropins, estrogen, testosterone are normal. What is causing this?

Normal development - Pubertal gynecomastia in males is normal and generally goes away on its own - transient relative imbalance between estrogen and testosterone - will resolve by 18 when adult ratios of androgens are achieved - need to reassure pt - normal in newborns (maternal estrogen), pubertal, and elderly males (decreased test) - also seen w/ spironolactone, hormones, cimetidine, finasteride, ketoconazole (Some Hormones Create Funny Knockers)

53 yo alcoholic, coughing, temperature, clubbing of digits, halitosis, missing teeth, dental caries. X ray w/ cavitation on right lower lobe. Sputum cultures will grow what organisms

Normal oral flora - aspiration pneumonia w/ klebsiella (intestinal flora) - but will also find oral flora i.e streptococcus (gram+ cocci in chains)

32 yo moves to a mountainous region for 2 months. After 2 weeks his SOB is better, and his exercise tolerance has increased. What cell type has increased in the bone marrow?

Normoblasts - increased erythropoietin increases erythropoiesis - hemocytoblast (stem cell) -> proerythroblast (committed) -> early erythroblast -> late erythroblast -> NORMOBLAST -> reticulocyte -> erythrocyte

pt w/ HIV on treatment had hx of 3 week severe pain in legs, and numbness/tingling in feet. What can you give him for relief?

Nortriptyline - NRTI (Abacavir, Didanosine, emtricitabine, lamivudine, stavudine, tenofovir, zidovudine) SE: bone marrow suppression (can give G-CSF and EPO), peripheral neuropathy, lactic acidosis, anemia (ZDV), and pancreatitis (Didanosine), central adiposity - Treat with tricyclic antidepressants, ("triptyline/ipramine), anti-seizure (gapapentin, pregabalin), capsaicin, lidocaine patches, and SSRI (venlafaxine, duloxetine)

47 yo w/ jaundice. Labs shows increased serum bilirubin and a positive urine bilirubin test. What is the the most likely cause?

Obstruction of the bile duct - conjugated (direct) hyperbilirubinemia: from biliary tract obstruction: gallstones, cholangiocarcinoma, pancreatic or liver cancer, liver fluke, biliary tract disease (primary sclerosing cholangitis, primary biliary cholangitis) or excretion defect (Dubin-Johnson (black liver) and Rotor syndrome - water soluble and excreted in urine - unconjugated hyperbilirubinemia (indirect): lipophilic hence why babies get kernicterus -> immature UDP-glucuronosyltransferase -> decrease conjugation -> accumulation of unconjugated bili in basal ganglia (Tx w/ non UV phototherapy to change to water soluble form for excretion - bleeding ulcer, hemolysis, hemophilia, phsiologic (newborns), Criger-Najjar, Gilbert syndrome

In someone with schizophrenia, what is the treatment?

Olanzapine - first line is atypical antipsychotics - "apine" or "idone" - mostly serotonin/dopamine antagonists - all prolong QT, extrapyramidal symptoms - reperidone: high porlactin -"apines" metabolic syndrome (weight gain, DM, HLD)

20 yo lost sense of flavor for food after he fell down stairs and hit his head 1 week ago. Physical exam is normal, no abnormalities. What cranial nerve was damaged?

Olfactory - avulsion of cribriform plate from trauma can cause loss of taste - no facial drooping = no facial nerve injury

30 yo woman w/ eye pain, blurred vision, and weakness in right arm and both legs. Ophthalmologic exam -> optic disc swelling. PE shows spasticity/hyperreflexia, MRI shows T2 weighted bright signal abnormalities. CSF shows increased mononuclear cells and oligoclonal banding. cytokines released by lymphocytes in CNS destroy what?

Oligodendrocytes - Multiple sclerosis - high incidence in Caucasians living far from equator - HLA-DRB1, decreased vitamin D, smoking - autoimmune demyelination of CNS (brain/spinal cord) with axonal damage

50 yo w/ cough and weight loss had begun taking itraconazole for histo infection. He is on hydrochlorothiazide, enalapril, atenolol, omeprazole, metoclopramide. An interaction between itraconazole and which drug are preventing itrazonazole from working?

Omeprazole - is a CYP450 inhibitor - azoles are also CYP450 inhibitors - interaction prevents the function of the azole and lower the amount fo drug in the body

53 yo w/ GERD and epigastric tenderness. What drug will relieve symptoms and promote healing of esophageal mucosa?

Omeprazole - irreversibly inhibits H+/K+ ATPase in stomach parietal cells - treats peptic ulcers, gastritis, reflux, zollinger-ellison syndrome, and use in h pylori infection/ stress ulcer prophylaxis - SE: increase risk of c diff, pneumonia, interstitial neph, decreased reabsorption of mg and ca+ -> osteoporosis - CYP450 inhibitor

Which bone mets are osteoblastic vs. osteolytic?

Osteoblastic: Prostate Osteolytic: multiple myeloma, lung, thyroid, kidney Mixed: breast Prostate, Breast > Kidney, Thyroid, lung - Lead (PB) KeTtLe - Via Batson's valveless plexus

in a 65 yo woman w/ decreased bone density what do you expect her osteoblast, osteoclast, and RANKL levels to be?

Osteoblasts = decreased osteoclasts = increased RANKL = increased - Osteoblasts make RANK ligand -> stimulates RANK receptors in osteoclasts and increases osteoclast activity - osteoprotegerin binds RANKL to prevent RANK-RANKL interaction and decrease osteoclast activity (denosumab mimics osteoprotegerin)

72 yo w/ muscle pain/weakness for 6 months exacerbated by activity. She has proximal muscle weakness and tenderness over the surface of her shins. Her PTH is elevated and her calcium is low. What does she have?

Osteomalacia - defective mineralization of osteoid - usually from vitamin D deficiency - Vita D deficiency causes secondary hyperparathyroidism

78 yo man has progressive hearing loss in right ear. Bone conduction is better than air conduction in both ears. what is the cause of his hearing loss?

Otosclerosis - when your stapes gets stuck in place - prevents sound travel from middle ear to inner ear - bone is louder than ear in conductive hearing loss dont confuse w/ - loss of hair cells - sensorineural hearing loss (presbycusis) - noise induced hearing loss = damage to sterociliated cells in organ of corti (loss of high frequency hearing first) - two tests: - Weber: lateralization: base of fork on forehead and asl them to tell which ear sound is louder -> equal = noromal hearing or bilateral hearing loss - lateralization: bony conduction better in one ear better than the other - conductive hearing loss (less interference from ear canal and just hear bone sounds) - bone sounds = nerve sounds - sensineural: hearing loss at level of nerve - conduction: issue with conductal system - Rinne: air and bone conduction. base of fork on mastoid process, when tone no longer heard, move fork to ear to see if they can still hear it - can still hear it = no conductive hearing loss = + test - cant hear it = conductive hearing loss - he has conductive hearing loss in both ears and the only option was otosclerosis (similar to pagets disease)

10 yo girl with increased hair growth on face, and increased muscle mass. Breast tanner stage 1, pubic hair tanner stage 5 + clitorimegaly, normal vagina. Labs: low LH, high testosterone. What cell types is causing this hirsutism?

Ovarian sertoli-leydig cells - sex cord stromal tumor (benign) - sertoli-leydig cell tumor: small, grey-yellow brown mass, resembles testicular hx w/ tubules/cords lined by pink sertoli cells - makes androgens -> virilization (hirsutism, male pattern baldness, breast atrophy, clitoral enlargement, ammenhorrhia) - mass is actually in ovary and has Reinke crystals (pink cells w/ crystals)

52 yo has onset of chest pain, SOB. played tennis all day and cannot remember how much fluid he had. Pulse 122, RR 28, BP 90/50. Dry skin + decreased capillary refill. No abnormal ECG/cardiac enzymes. Will the tubular osmolality be isotonic, hypotonic, or hypertonic in the PCT, Macula Densa, Medullary collecting duct?

PCT: isotonic Macula densa: hypotonic Medullar collecting duct: hypertonic - decreased BF to kidneys - decreased BP = renal baroreceptors - decreased NaCl (hypotonic) delivery to macula densa -> activates RAAS - increased RAAS = increased GFR -> more solutes + water absorbed by PCT in isotonic way - Activation of ADH/ALDO: water resorption (no solutes) in CD = hypertonic solution remains in tubule) concentrates urine to have high osmolality and retain water - PCT: stays constant entire (if kidneys are functioning) -> midpoint of DCT isotonic in any state (hypovolemic/hypervolemic states it will be isotonic to serum) -> active transport of ions and water is reabsorbed 1:1 - descending loop: passive resorption of water via vasa recta - base of loop of henel = hypertonic compared to serum - thin limb (passively reabsorbs 1:1) + thick (concentrating DCT more solutes absorbed than water): isotonic again as you passively absorb ions again. responds to ADH -> if on it will return to isotonic urine or no ADH = hypotonic - thick loop: actively resorbs again -> junction of DCT/thick limb you are hypotonic again (Na/Cl) where ADH works - thick whole path is just diluting urine, while reabsorbing water and more salt making it hypotonic - macula dense: in this hypotonic region as it is after the main - CD: no ADH -> hypotonic, active = hypertonic (low as 70-80 osm -1500 osm) - here, no abnormalities in her kidneys and shes dehydrated and will make concentrated filtrate

How does sildenafil work?

PDE-5 inhibitor decreases cGMP breakdown - erection: parasympathetic NS (pelvic splanchnic nerves S2-4 -> pudendal) - NO -> increases cGMP -> smooth muscle relaxation -> vasodilation -> proerectile (point = parasympathetic) - NE -> increases Ca+ -> smooth muscle contraction -> vasoconstriction -> antierectile (Squeeze = emission: sympathetic via hypogastric nerve, T11-L2 and Shoot = ejaculation: visceral and somatic nerves pudendal)

55 yo w/ 3 months of SOB, crackles over lung bases, Xray of enlarged hilar lymph nodes and reticulonodular infiltrates. bx of enlarged lymph node = noncaseating granuloma. Labs: Ca+ 12, phos 4.2, creat 1, nitrogen 10. What are her PTH and 1,25 dihydroxycholecalciferol levels?

PTH low, high 1,25 dihydroxycholecalciferol = calcitriol aka active vitamin D is increased via epithilioid histiocytes in granuloma of sarcoidosis - presents with hypercalcemia

what would you give a 45 yo w/ chronic pancreatitis from alcohol abuse with diarrhea that has decreased fecal elastase?

Pancrelipase - combination of three enzymes: lipase, protease, amylase normally made in pancreas and are important in digestion of fats, proteins, sugars

Woman with sharp chest pain, SOB after a nonpenetrating injury in rugby. RR are 22, she has decreased breath sounds, decreased tactile fremitus, and increased tympany to percussion on right. What is she at risk for?

respiratory acidosis - traumatic pneumothorax - decreased alveolar space and lung surface area -> decreased gas exchange and hypercarbia -> respiratory acidosis

25 yo at 12 weeks gestation passes tissue vaginally w/ an obvious fetus, chorionic villi w/ focal edema and trophoblastic proliferation. What is the diagnosis?

Partial hydatidiform mole - 69 XXX, XXY, XYY - 2 sperm+ 1 egg - partial fetal parts - normal hCG levels complete mole = 46 XX, 46 XY -> usually an enucleated egg and a single sperm -> duplicates paternal DNA - no fetal parts -very high hCG - cluster of grapes/honeycomb uterus, snowstorm on US - higher risk of malignancy

55 yo w. 2 months of decreases appetite and 20lb weight loss and rash. He has necrolytic migratory erythema over axillae and groin. Labs show glucose of 280 and glucagon of 1500 (N=20-100). CT shows mass in head of pancreas. What is occurring in this persons liver?

Partial oxidation of fatty acids - glucagon -> releases glucose into blood - negative effect on acetyl-coa carboxylase (fatty acid synthesis) - negative effect on HMG-CoA reductase (no cholesterol formation) - positive effect on glycogen phosphorylase (breaks down glycogen -> glucose = glycogenolysis) - negative effect on glycogen synthase (glycogenesis) - increase in fasting state: increase cAMP via Gs, increases kinase A -> Increases Fructose bisphosphatase 2 -> decreases PFK-2 -> less glycolysis and more gluconeogenesis

55 yo woman can't recognize picture of her own mother. she cannot recognize objects unless she can touch them or hears a sound they make. What blood supply was injured?

Posterior cerebral - occipital lobe - can cause contralateral hemianopia without macular sparing (blindness over half the vision field) - alexia without agraphia (cannot read with preservation of written and spoken language) - prosopagnosia = inability to recognize faces

30 yo w/ urinary incontinence after tx of pelvis fracture. He has distended bladder, and no micturition reflex. After bladder fills, overflow urine flows out of urethra a few drops at a time. What nerves are injured?

Pelvic nerves - Overflow incontinence -> incomplete emptying (detrusor under-activity/outlet obstruction) - leak with overfilling - damage to parasympathetic nerves involved in micturition reflex (parasympathetic pelvic nerves) - pudendal nerve = somatic control of external sphincter/external anal sphincter (and sensation of perineum/genitals)

In a prostatectomy what are you worried of injuring?

Pelvic parasympathetic nerves - risk is impotence

A diabetic with constipation with no relief from laxatives has dysfunction of what nerves?

Pelvic splanchnic - enteric nervous system slows and has neuropathy - these nerves are the parasympathetic nerves to the enteric NS that allow for "rest and digest"

25 yo w/ 2 hr of nausea, vomiting, abdominal cramping, difficulty farting. He has brown speckled spots on his lips, fingers and toes. Labs show hypochromic microcytic anemia, and stool occult blood is positive. What does he have?

Peutz-Jeghers syndrome - hamartomatous polyps (abnormal lesions, no risk of carcinoma, also in juvenile polylposis) - AD -Numerous hamartomas in GI tract - hyperpigmented macules - increased risk of breast, and GI cancers

ten healthy subjects given new oral drug, and monitored for drug effect and toxicity. Blood samples taken and analyzed to define pharmokinetics of drug. What phase of dug trial is this?

Phase 1 Does it SWIM? - Phase 1: small # of healthy volunteers/patient w/ disease of interest (if not safe for healthy people) -> assesses Safety/toxicities/pharmokinetics/dynamics - Phase 2: moderate # of pt w/ disease: does it Work? efficacy, optimal dosing, adverse effects - Phase 3: Large # of pt randomly assigned to tx or placebo: is it good or better -> compares new tx to standard of care (is there any Improvement) - Phase 4: post-Marketing surveillance of pt after approval -> can it stay? any long-term effects, may be withdrawn from market/black box warnings

51 yo w/ pheochromocytoma given propranolol to decrease tachycardia. What should be given before starting propranolol in this pt?

Phenoxybenzamine - Prevents catecholamine hypertensicve crisis - give a before b - Unopposed alpha stimulation -> HTN

24 yo has toxic goiter (Grave's) undergoes partial thryoidectomy and a few months later gets muscle cramps, tetany, hypocalcemia. What will her phosphate, PTH, and vitamin D levels be?

Phosphate: high PTH: low Vita D: normal - hypoparathyroidism from injury to parathyroid glands - can see chvostek sign (tapping facial nerve -> twitch - trousseau sign -> occlusion of brachial artery with BP cuff -> carpal spasm - vitamin D: skin 7-dihydrocholesterol in basal level of skin -> most D3 synthesis in basalis and corneum -> UV light -> pre-vita D3 (pre-cholicalciferol) -> isomerize immediately -> vita D3 (cholicalciferol) = dependent on UV radiation -> liver (25 alpha hydroxylase = cytocrome enzyme that can be affected) -> 25 hydroxycholicalciferol = calcidiol (D3) -> pertubular capillaries of kidney (1-alpha hydroxylase upregulated by PTH) -> 25 hydroxycolicalciferol -> 1,25 dihydroxy D3) = calcitriol - two forms of vita D3 cholicalciferol (milk and dairy), plants make D2 (ergocalciferol) - calcidiol = can mean Vita D2 or D3 - decreased 25 hydroxyvita D = liver disease, no sunlight, any limited vita D, limit dietary intake

25 yo 42 weeks pregnant fails to begin labor and comes to hospital. Given oxytocin. Activation of what signaling pathway will mediate therapeutic effect of drug?

Phosphoinositide hydrolysis - releases Ca+ and causes synchronized uterine contractions - binds Gq -> IP3 pathway - other hormones that bind Gq/IP3 = GnRH, ADH (V1 receptor), TRH, Histamine (H1), Angio II, Gastrin, Alpha 1, M1, M3

How do you tx dry mouth, flushing, disorientation from jimsonweed overdose? This drug must be carefully titrated to avoid cardiac side effects?

Physostigmine - jimsonweed = naturally occurring atropine - crosses BBB (tertiary amine) can help with CNS effects - increase Ach

25 yo woman w/ flu-like illness (myalgia, fatigue), SOB when lying down, and myocardial bx shows viral infection what is the cause?

Picornavirus - + sense ssRNA, naked virus, feca-oral - makes long protein that uses viral proteases to cleave - all RNA replicates in cytoplasm - coxsackie virus type B - dilated cardiomyopathy, devils grip/Bornholms disease (extreme sharp pain in chest = pleurodynia) - tx w/ supportive care

42 yo with sudden severe headache, BP 163/90, right pupil is 6mm and non-reactive to light. Adduction of right eye is impaired and nuchal rigidity is noted and blood in subarachnoid space. What arteries is most likely effected?

Posterior communicating artery - this is a subarachnoid hemorrhage which are usually bleeding from truama, or rupture of an aneurysm (saccular aneurysm) which would be in the circle of willis) - Posterior cerebral artery/ Pcomm will -> CN III palsy presented here

56 yo w/ SOB after coronary artery bypass grafting. RR 26. Dullness to percussion at left base, X-ray shows fluid in pleural cavity. thoracentesis yields 1200 ml pale, milky fluid = chyle. What procedure can produce this chylothorax?

Placement of the central line via left internal jugular vein - chylothorax are due to thoracic duct injury from trauma or malignancy - thoracic duct ascends from cysterna chyli w/ aorta through the diaphragm - enters in the junction of the left subclavian and internal jugular veins - in central line placement can rupture this duct causing chylothorax - the right lymphatic duct is not the major cause of chylothorax, the thoracic duct is - increased triglycerides on fluid analysis

62 yo w/ CKD has anemia. You give her EPO and then monitor her by measuring what lab value?

Plasma hemoglobin concentration - as this is a measure of anemia

Study of a vancomycin-resistant strain of E faecalis is cultured. Ten generations grow in medium lacking vancomycin. The resulting bacteria are screened for vancomycin resistance. There are one per 100 cells that have vancomycin-sensitivity. What explains the decreased resistance in these?

Plasmid loss - conjugation: F+ plasmid contains genes required for sex pilus and conjugation - Bacteria without plasmid are F- - Sex pilus on F+ contacts F- bacteria - single strand plasmid DNA is transferred across conjugal bridge (mating bridge) and F- becomes F+ - no transfer of chromosomal DNA - in high frequency recombination: F+ plasmid is incorporated into bacterial chrom DNA (Hfr cell) and can transfer plasmid + flanking chromosomal genes to F- recombinant cell that remains F- but has new bacterial genes in chromosome

60 yo w/ 1 month of SOB w/ exertion. right lung base has decreased breath sounds, dullness to percussion, decreased tactile fremitus, and no adventitious sounds. Left lung base has crackles. What does he have?

Pleural effusion - pneumonia would have bronchial breath sounds, inspiratory crackles, egophony, dull percussion, but INCREASED tactile fremitus - increase in tactile fremitus means dense/inflammed tissue - a decrease in tactile fremitus suggest air/fluid, or decrease lung tissue (emphysema) in lung - This is because sound waves (pt saying "99" travels faster trough solids, and slower in air/liquids) the more close the molecules of the substance are (solids) the quicker sound waves can travel through them

32 yo w/ absent tactile fremitus, hyperresonance on percussion, and absent breath sounds at lung base. What is the dx?

Pneumothorax on the left - fremitus is only increased in consolidation (sounds travel faster in solids) - all have decreased breath sounds except bronchial breath sounds or crackles in pneumonia - pneumonia only one w/ egophony, whispered pectoriloquy - percussion: dull in effusion, atelectasis and hyperresonant in pneumothroax (echo) - hyperresonant = pneumothorax (tension pneumo = deviation of trachea away from injury side)

7 yo from africa just moved and has 2 day hx of headache and fever + back pain. In village, another child had similar symptoms. Pt has 102 fever, weakness of left lower extremity, knee reflex is weaker on left, fasciculations on lower extremities. What does he have?

Poliomyelitis - picornovirus (ss+, naked, RNA) - RNA translated into a large polypeptide and cleaved by virus encoded proteases -> functional viral proteins - from poliovirus (feca-oral transmission) replicates in oropharynx before spreading to blood -> CNS -> destruction of anterior horn of spinal cord (LMN death) - malaise, headache, fever, nausea - LMN: asymmetric weakness, hypotonia, flaccid paralysis, fasciculations, hyporeflexia, muscle atrophy - respiratory muscle involvement -> respiratory failure - CSF: increased WBC, (lymphocytic pleocytosis), slight increased protein, no change in glucose

What is the mutation in PKD?

Polycystin - numerous cysts in kidney cortex/medulla -> ESRD in 50% of pt - mutation in PKD1 (chrom 16) or PKD2 (chrom 4) - associated with berry aneurysm, mitral valve prolapse, benign haptic cysts, diverticulosis - can use ACEI/ARBS for HTN

23 yo with SOB for 2 weeks, RR 28, PE no findings. Labs: Serum = Na+ 135, K+ 4.0, Cl- 110, HCO3- 15, pH 7.25, Urine = sodium 20, Cl- 30, K+ 15. What is the diagnosis?

Renal tubular acidosis - she has a normal anion gap (135-(110+115) = 10) and all other options were anion gap acidosis - RTA may be from lack of reabsorption of bicarb (type II), inability of alpha intercalated cells to secrete H+ (type I), or hypoALDO/ALDO resistance -> hyperkalemia (type 4)

10 yo girl, school nurse thinks she has Marfan's. She is 95% for height and 25th for weight. Palate is narrow, she has long extremities, and joint laxity. Her mother is 5' 4" but she has the same mutation as her daughter. What describes the single nucleotide change in the patient and her mother?

Polymorphism - Marfans may not have complete penetrance - a polymorphism = genetic variation in a particular nucleotide -discontinuous genetic variation resulting in occurrence of different forms or types of individuals among members of a single species - In Marfan's the parents may be unaffected, yet may pass the gene on to their children due to differences in penetrance

19 yo has weakness of muscles of his neck and extremities for past week. Bladder and bowel function is normal, position sense is impaired. What is the dx?

Polyneuropathy - damage or disease affecting peripheral nerves in roughly the same areas on both sides of the body, featuring weakness, numbness, and burning pain - usually from vitamin B deficiency, trauma, infections, charcot marie tooth, dry beri beri, vitamin B6 deff, diabetes, Krabbe, Fabrys, cediak hagashi, acute intermitted porphyria, chemo agents - syringomyelia will never touch dorsal column -> never have issues with position sense (chiari type I) or post-traumatic or viral - hereditary: scoliosis, hammertoe - can be distal (diabetes, acohol, B12 deff) moves towards cell bodies from perimeter + sensory motor issues - can affect large and small fibers - ALS: degeneration of corticospinal tract (spasticity, UMN) and anterior horns (atrophy, fasciculations, loss of DTR) -> UMN/LMN sings - sporadic or SOD mutation (super oxidase dysmutase -> more free radicals) (familial) - paralysis -> respiratory issues and death - he has a melinopathy -> slow demyelination that affects motor and sensory smilar to guillain barre post viral

2 yo w/ 12 hr headache, no appetite, vomiting. temp fo 103, pulse 120, RR 40, BP 90/50. PE shows nuchal rigidity, LP = increased protein, decreased glucose, increase nutrophils, gram + diplococci. Immunization with what kind of vaccine would have prevented this?

Polysaccharide protein conjugate vaccine pneumococcal conjugate vaccination for (PCV13): enhance immunogenicity by promoting a T-cell activation and class switching (protein taken up via phagocytosis -> MHC II -> CD4+ -> IgM -> IgG (usually) - babies + children < 2 years old - adults 65 years or older (as immunity wanes) - 2 - 64 years old with asplenic states (removal/sickle cell/etc) pneumococcal polysaccharide vaccination for (PPSV23): - adults 65 years or older - 2 - 64 years old w/ certain medical conditions - 19 - 64 years old who smoke cigarettes

62 yo cannot move arms/legs. Her BP 173/95, horizontal eye movements are impaired bilaterally, vertical eye movement is fine. Her speech is dysarthric. Where is the lesion?

Pons - Basilar Artery stroke - pons, medulla, lower midbrain: RAS spared = preserved consciousness - corticospinal and corticobulbar tracts = quadriplegia, loss of voluntary facial, mouth, and tongue movements - ocular cranial nerve nuclei, paramedian pontine reticular formation = loss of horizontal, but not vertical eye movemets - "Locked in" syndrome (think locked- in the BASement)

75 yo w/ horner syndrome on right, difficulty speaking, pain and temperature sensations decreased on right side of face, and left side of body. What artery is occluded?

Posterior inferior cerebellar - lateral medulla - nucleus ambiguus (CN IX, X, XI0 -> dysphagia, hoarseness, decreased gag reflex, hiccups - vestibular nuclei: vomiting, vertigo, nystagmus - lateral spinothalamic tract, spinal trigeminal nucleus: decreased pain and temo sensation from contralateral body, ipsilateral face - sympathetic fibers: ipsilateral horner syndrome - inferior cerebellar peduncle: ipsilateral ataxia, dysmetria - Lateral Medullar (Wallenberg syndrome): Nucleus ambiguus effects specific to PICA lesions - AICA (anterior inferior cerebellar artery) has same presentation but + FACIAL DROOP means AICA's pooped - this is a lower motor lesion (UMN lesion in cortical stroke) decreased lacrimation, salivation, taste from 2/3 tongue Cerebellar lesions - Follicular nodular = ocular dysmetria Cerebellar hemisphere - limb ataxia -> finger dysmetria Vermis = truncal ataxia -> usually cute through vermis which is in the middle

12 yo girl from central africa, abdominal pain, vomiting blood, temo of 100, hepatosplenomegaly, and abdominal ascites. Lab: high eosinophils. Stool shows egg w/ lateral spine. How do you treat her?

Praziquantel - She has schistosoma mansoni -> liver/spleen enlargement, fibrosis/inflammation and portal HTN - Snails are intermediate host - cercariae penetrate skin of humans who swim - Schistosoma Haematobium = egg w/ terminal spine -> squamous cell carcinoma pf bladder, hematuria, pulmonary HTN

32 yo has diastolic BP that ranges from 100-110. otherwise healthy with normal labs. Urine contains trace protein, and no albumin. He is unlikely to develop edema because of what regulatory adjustments?

Precapillary resistance - systolic: function of stroke volume (increased by hyperthryoidism/b1 agonism) - diostolic: blood volume and SVR - edema required more capillary flow from the precapillary resistance - typical in elderly from calcification/hardening of the arterioles this prevents blood flow through -> no edema

the prevalence of high grade cervical intraepithelial neoplasia in unscreened population is 5%. The prevalence in a screened population is 0.2%. As prevalence decreases what will also decrease?

Predictive value of a positive test - as prevalence increases, PPV increases, and NPV decreases - as prevalence decreases, PPV decreases, and NPV increases

What does an increase in disease prevalence increase?

Predictive value of a positive test - decreases predictive value of a negative test (more people with disease = more like a positive test means the person actually has the disease) - sensitivity/spec remain the same = qualifiers of a test itself

45 yo with joint pain from RA has had no success with over-the-counter agents. The physician wants to place her on disease modifying antirheumatic drug (DMARD). Due to delayed effects of these drugs, he also gives her another agent to use until the DMARD is effective. What is the agent?

Prednisone - reduces inflammation/pain and reduce joint damage - NSAIDS + glucocorticoids f - DMARDS include: methotrexate, sulfasalazine, hydroxychloroquine, leflonomide - Biologics: TNF-alpha inhibitors (infliximab, adalimumab, certolizumab, golimumab)

Difference between prerenal and postrenal azotemia?

Prerenal: - urine osmolality: >500 (concentrated urine) - urine Na+: <20 (retaining it from loss of fluid and functioning tubules) - FENa: <1% (very little Na+ excreted as most is being reabsorbed in attempt to regulate fluid loss) - BUN/Cr: >20 (urea reabsorbed in attempt to maintain volume, creatine not reabsorbed) Intrinsic renal: - urine osmolality: <350 (dilute and cannot concentrate from broken tubules) - urine Na+: >40 (dumping and not retaining anymore) - FENa: >2% - BUN/Cr: <15 same idea

26 yo with infertility has 47 XXY karyotype. Genetics show that one of the X chromosomes was inherited from the father. An error of chromosome segregation occurred during anaphase at which stages of spermatogenesis?

Primary Spermatocyte - spermatogenesis begins at puberty, full maturation = 2 months - gonium is going to be a sperm, Zoon is zooming to egg - N = diploidy, C = chromatid - Spermatogonium (diploid, 2N, 2C, 46chrom, Sex XY) -> out of tight junctions -> - primary spermatocyte (diploid, 2N, 4C, 46 sister chrom, XXYY)-> meiosis I -> - secondary spermatocyte (haploid, IN, 2C, 23 chrom XX or YY) -> meiosis II - spermatid (haploid, IN, 2C, 23 chrom, x or Y) -> - spermiogenesis -> mature spermatozoon w/ acrosome, taile etc - failed separation of an X and a Y occurs in going from primary -> secondary spermatocyte

In sterilization, a system has 15-20 psi pressure and generated temperature of 121 C- 126 degrees C. What infectious agents will most likely still be infective after this?

Prions - conversion of normal (alpha helical) protein (PrPc prion protein) -> beta-pleated (PrPsc) - transmissible via CNS-related tissue (iatrogenic corneal tx) or food - PrPsc resists protease degradation and converts more PRPsc - resistant to sterilizing procedures (standard autoclaving described) - causes spongiform encephalopathy and dementia, ataxia, death

3 yo with constant fractures of bones, and blue sclera and normal teeth. What biochemical process is altered?

Procollagen synthesis - Osteogenesis imperfecta - AD - glycosylation of residues and then forms disulfide/hydrogen bonds -> triple helix (will not occur in this disease) - no normal type ONE collagen (bone) -treat with bisphosphanates to prevents fractures

38 yo 2 years of intense menstrual pain and bleeding. She has one daughter but is no longer fertile. family hx of infertility. Enlarged ovary and retroflexed uterus, has ovarian cysts and peritoneal cyst. A bc of a cyst will show what?

Proliferative endometrial tissue - When in ovaries, frequently bilateral, can be in peritoneum - blood filled chocolate cysts - cyclic pelvic pain, bleeding, dysmenorrhea (period pain), infertility - ovarian cancer generally in women over age 55 and most are epithelial

10 yo boy has bruised easily since swallowing his grandfathers pills 2 days ago. aspirin and dicumarol were in his medicine cabinet. What lab finding can help you determine that he actually took dicumarol?

Prolonged prothrombin time - it is a competitive inhibitor of vitamin K epoxide reductase - acts in similar fashion to warfarin (this is the natural compound that inspired warfarin) - will want to monitor via PT but as this was two days prior the coagulation factors will be decreased as well - he also has bruising (think similar to hemophilia)

scurvy decreases what enzyme activity?

Prolyl hydroxylase - pro alpha chain (backbone gly-X-Y) -> hydroxylation of proline and lysine residues needs vitamin C (add -OH) -> glycosylation - leads to swollen gums, easy bruising, petechiae, hemarthrosis, anemia, poor wound healing, perifollicular hemorrhages, corkscrew hair, weakened immune response

9 yo has cystic fibrosis. A defect in what is causing his bronchial epithelium symptoms?

Protein structure - NOT membrane receptors - CFTR gene on chrom 7 -> deletion of Phe508 - codes for an ATP-gated Cl- channel that secretes Cl- in lungs and GI tract and reabsorbs Cl- in sweat - misfolded protein -> protein retained in RER and not transported to membrane -> degraded -> decreased Cl- and H2O secretion -> Na+ reabsorption in epithelial cells -> increase H2O absorption -> thick mucus in lungs/GI/vaginal - increase Na+ = Increased transepithelial potential difference - lumacaftor = corrects misfolding -> improves transport to membrane - ivacaftor = opens Cl- channels - in sweat duct: Cl- and Na+ normally reabsorbed, in CF = No Cl-/Na+ absorption - in Lung/tissue: Cl- usually excreted, H2O and Na+ mildly reabsorbed, in CF = no Cl- excretion pulls more H2O and Na+ into cell = dehydrated endothelium

29 yo has PCOS and the doctor recommends behaviors changes including dietary changes and exercise. What will result in patient adherence to this plan?

Provide follow-up appointments to assess the patients progress in attaining her goals - compliance will always be to call them back to follow-up to set achievable monthly/weekly goals

Acetominophen and NSAIDS reduce fever by decreasing the synthesis of what factors in the hypothalamus?

Prostoglandin E2 - Fever is cause by IL-1, IL-6 and TNF-alpha go to hypothalamus -> activate PG E2 -> resets hypothalamus at a higher level (set point) and body heats up - can directly block PGE2 by NSAIDS/Tylenol - tylenol acts centrally while other NSAIDS act peripherally - Tylenol has no anti-inflammatory effect - hence why its okay to give Tylenol to someone who has peptic ulcer disease/CKD

What cells in the kidney are the first to show anoxic injury?

Proximal tubule

4 yo had surgery and unable to breath spontaneously for 3 hours after. She was under sevoflurane. Succinylcholine was administered for intubation. What is the cause of her prolonged apnea?

Pseudocholinesterase deficiency - succinycholine activates Ach receptors without being broken down by cholinesterase - Pseudocholinesterase deficiency = increased sensitivity to muscle relaxant drugs in anesthesia, choline esters - drugs broken down in a few minutes and muscles can move again - here, not be able to move or breathe on own for a few hours after the drugs are administered - in theory cholinestrase should be able to reverese phase 2 of succinycholine (when ach receptors are available but desensitized) - pseudocholinestrase breaks down succinocholine (and pilocarpine adn other drugs as well) just like cholinestrase breaks down Ach - will prolong paralysis

58 yo has 4-year history of recurrent cough with increased sputum. cough supressants have not helped. he has smoked 2 packs of cigs for 35 years. No temp, pulse 72, RR 18. PE shows cyanosis and diffuse wheezing. What cell type are abnormal in this pt?

Pseudostratified columnar epithelial cells - he has chronic bronchitis - Presents with wheezing, crackles, cyanosis (hypoxemia from shunting), CO2 retention and secondary polycythemia (increased EPO in states of hypoxemia) - Hx: hypertrophy and hyperplasia of mucus-secreting glands in bronchi -> Reid index increased - DLCO normal - dx: productive cough for >3 months in a year for >2 consecutive years - chronic bronchitis: sputum production and stasis from hypertrophic goblets cells and damage to the cilia on the pseudostratified cells - in small bronchioli

6 week old boy w/ 2 week hx of projectile vomiting after feeding. no change in formula helps. His vomit has no bile, he is dehydrated, and decreased stools. He has metabolic alkalosis. What is the dx?

Pyloric stenosis - Most common cause of gastric outlet obtruction - palpable olive shaped mass in epigastric region, visible peristalsis, nonbilious projectile vomit at 2-6wks old - common in males/exposure to macrolides - hypokalemic hypochloremic metabolic acidosis (from vomiting gastric acid and volume contraction) - thickened pylorus - need surgical incision

18 yo 2 week of fatigue, scleral icterus, and labs: low Hb, high erythrocyte 2,3 bisphosphoglycerate, high billi (mostly indirect). What erythrocyte enzyme is deficient?

Pyruvate Kinase Deficiency - will see burr cells (echinocytes) -> uniform, small projections - anemia: normocytic, hemolytic (reticulocyte index >2%), intrinsic hemolysis - AR, decreases ATP -> rigid RBC (bc RBC undergo glycolysis for engery, they can no logner produce ATP) -> extravascular hemolysis - Increased levels fo 2,3-BPG to promote unloading of O2 and decrease Hb affinity for O2 - hemolytic anemia in newborn - AR, glycolysis slows down from build up of pyruvate -> increased 2,3 BPG (increases as there is not enough energy) - pyruvate is inhibited by ATP and alanine and activated by fructose 1,6 BP that will build up as pyruvate kinase is not working + build up of 1,3 BPG which is transformed to 2,3 BPG -> also upregulated by HIF (hypoxia induced factor) to increase O2 release - RBC then cannot make ATP and undergo extravascular hemolysis

a study shows a transcription factor is synthesized as a larger precursor protein with two membrane-spaning domains. The TF is by proteolysis when it is needed. Where is this initially produced?

RER

18 yo w/ mono. She has atypical lymphocytes. What cells account for the atypical lymphocytes?

Reactive T lymphocytes - not virus-infected B lymphocytes - from Epstein-Barr virus (HHV-4) -> mono - associated w/ lymphoma (burkitt), nasopharyngeal carcinoma (asians), and lymphoproliferative disease in transplanted patients - CD21 - atypical cells are reactive cytotoxic T cells responding to virus infected B cells - positive monospot test = heterophile antibodies detected by agglutination of sheep or horse RBC - amoxicillin in mono = rash - B cells will be normal - T-cells will appear abnormal -> hence why this is the answer and these cells are also more abundant in Mono, not B cells

After being severely beaten and sustaining a gunshot wound to abdomen, a 42-year-old woman undergoes resection of a perforated small bowel. During operation, plastic reconstruction of facial fractures, and open reduction and internal fixation of left femur. 36 hrs postoperatively, she is awake but not completely alert. She is receiving IV morphine via a patient-controlled pump. She says that she needs morphine to treat her pain, but she is worried that she is becoming addicted. She has no hx of substance use disorder. She drinks one to two glasses of wine weekly. Which of the following initial actions by the physician is most appropriate?

Reassure the patient that her chance of becoming addicted to narcotics is minuscule - Narcotic use for acutely painful conditions is both reasonable and important. (Very) short-term use (immediately post-surgical) does not lead to long-term dependence - drugs addicts should also receive narcotics to control pain.

25 yo with hx of splenectomy now has target erythrocytes. This finding is due to loss of function of what portion of the spleen?

Red pulp - disproportional increase in ratio of surface membrane area to volume from increased red cell surface area, or decreased Hb (thalassemia) -> decrease in cell volume not affecting membrane area) - decreased osmotic fragility - usually cleared by splenic macrophages - red pulp = connective tissue cords of Billroth and sinusoids engorged w/ blood = red color - filter blood of AG, microorganisms, defective or worn-out RBC - monocytes = greater than total # in circulation -> can mobilize to leave spleen and assist in tackling ongoing infections - White pulp: - periarteriolar lymphoid sheaths (PALS) = arteriole supply of spleen, contain T lymphocytes. - Lymph follicles w/ B lymphocytes btw PALS and marginal zone -> make IgM and IgG2 for opsonization of organisms -> mostly encapsulated bacteria (H flu, strep pneumo, niesseria meningitis) - marginal zone btw white pulp and red pulp - antigen-presenting cells (dendritic cells and macrophages)

Hox genes play a role in embyrogenesis by what process?

Regulation of transcription - HOX or homeobox genes are involved in segmental organization of embryo in a craniocaudal direction - they code for transcription factors - mutations = appendages in the wrong locations - these are end points of signal transduction

6 month old has 2 month hx of failure to thrive and intractable diarrhea worse after feeds. Eczematous dermatitis and mild lymphadenopathy. Heelstick shows elevated glucose, and genetic testing shows mutation in gene encoding FOXP3. Mutation causes a decrease in what t-cell population?

Regulatory T lymphocytes - IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked): defect in FOXP3 -> autoimmunity w/ enteropathy, endocrinpathy, nail dystrophy, dermatitis, other autoimmune dermatitis, and diabetes in male infants - Regulatory T cells help maintain specific immune tolerance by suppressing CD4 and CD8 T-cell effector functions (express CD3, CD4, CD25, FOXP3) - produce anti-inflammatory cytokines (IL-10, TGF-B)

55 yo w/ left flank pain, gross hematuria and mass palpable in left upper quadrant of abdomen. US shows 12cm mass in pole of left kidney, angiogram shows hyper vascular mass. What is the diagnosis?

Renal cell adenocarcinoma -> same as RCC

5 yo boy w/ pain in right eye -> found to have mass. No hx of neoplasms, and physician says he will have no future risk of any other neoplasms. He has strabismus and tenderness of eye. Left eye is normal. The first mutation occurred where?

Retinal cells - non-hereditary form of RB, only one eye is affected and no family hx - born with two normal RB1 gene. Then, in early childhood, both copies of RB1 gene in certain retinal cells -> mutations - not at risk of passing RB1 gene mutations to children

32 yo had 2 week hx of thrush and is on zidovudine for HIV, but with poor compliance. Labs shows CD4+ 100, and high viral load. She has zidovudine resistance. A mutation in what viral genes codes for this?

Reverse transcriptase - not thymidine kinase (this would be for an anti-retroviral that needs viral kinase to work = acyclovir) - NRTI's competitively inhibit nucleotide binding to reverse transcriptase and terminate the DNA chain w/ a lack of a 3' OH - all nucleosides except tonofovir (nucleotide) - need to be phosphorylated to be active (except tenofovir) - cause bone marrow suppression (give GCSF), peripheraly neuropathy, lactic acidosis, anemia, pancreatitis (didanosine), agranulocytosis (zidovudine), and lipodystrophy - test for HLA-B5701 mut prior to abacavir

25 yo w/ 6 month hx of joint pain non-responsive to aspirin. bilateral swelling of proximal interphalangeal joints, metacarpophalangeal joints, and wrists + weakness of grasp. A few small nodules beneath skin around finger joints. What does she have?

Rheumatoid arthritis - the little nodules felt are rheumatoid nodules = fibrinoid necrosis w/ palisading histiocytes) - not SLE because no rash, kidney, lung, heart issues

2 month old with vomiting, diarrhea, and dehydration. Stool shows wheel-like shape viral particles. What type of nucleic acid does this virus have, does it have an envelope, and what is its capsid symmetry?

Rotavirus - Double-stranded RNA, segmented with NO envelope and Icosahedral symmetry - segments dsRNA virus (reovirus) - most important global cause of infantile gastroenteritis - major cause of acute diarrhea in US in winter especially in day care centers/kindergartens - destroys villi -> decreased absorption of Na+ and K+ - routine vaccine (live attenuated) give before 3 months -> may increase risk of intususeption - DS linear virus with 10-12 segments - NSP4 causes secretory diarrhea - Cl- permeability increased - number one cause of diarrhea in young children - give oral rehydration

5 yo w/ sudden onset of uncontrollable irregular movements of left side of body. What nuclei is damaged?

Right Subthalamic - Hemiballismus - sudden, wild flailing of one arm +/- ipsilateral leg - from injury to contralateral subthalamic nucleus from a lacunar stroke - lacunar strokes are the most common type of brain ischemia from occlusion of small penetrating arteries from hyaline arteriosclerosis from HTN -> damage to deep structures of brain usually present w/ movement issues - Not caudate: helps store memories, and regulate emotion (as seen in Huntingtons) - subthalamic nucleus uses excitatory glutamate -> normal motor function

woman w/ weakness in left leg, smoker for 40 years. BP 140/100. Left leg shows 1/5 muscle strength, and DTR of 3+. When her eyes are closed she cannot tell if her great tow is raised or depressed. What artery has an embolism?

Right anterior cerebral - motor and sensory cortices -Lower limb - contralateral paralysis and sensory loss in lower limb, and urinary incontinence

A girl chips her tooth and inhales it. Where will it most likely aspirate it?

Right lower lobe

19 yo, withdrawn/isolated for 1 year. stays in bedroom, not interested in friends, radio broadcasts only she can hear, thinks about government schemes. no alcohol or drugs. She is distracted, sad, anxious, blunted affect. What medication does she need?

Risperidone - She has schizophrenia: she has more than two positive symptoms (auditory hallucinations, delusions, unusual thought processes) and negative symptoms (blunted affect, withdrawal, anhedonia) - treat with atypical anti-psychotics - may cause hyperprolactinemia (amenorrhia, galactorrhea, gynecomastia)

42 to w/ 3 year hx of intermittent facial rash on forehead, eyelids, nose, cheeks, she said its getting worse from moving from NY -> FLA. She always blushed easily. Spicy foods make her flush. Her face will sometimes burn/sting/feel dry. she has erythema over nose and cheeks w/ scattered telangiectasias and papules. what does she have?

Rosacea - inflammatory skin disorder w/ papules/pustules/comedones. May have flushing in response to external stimuli alcohol/heat - phymatous rosacea can cause rhinophyma (bulbous deformation of the nose)

a healthy 61 yo come to the physician and a split S1 heart sound is heard over the left sternal border. What explains the first component of the sound?

S1 = Mitral and tricuspid valve closure S2 = aortic and pulmonic valve closure - closure of mitral valve = M1 - closure of tricuspid valve = T1 - M1 sound is much louder than T1 from higher pressures in left side of the heart; thus - M1 radiates to all cardiac listening posts (loudest at apex), T1 is only heard at left lower sternal border. This makes the M1 sound the main component of S1 - M1 physiologically occurs before T1 - Inspiration delays closure of tricuspid valve in a normal person, from increased venous return, enhancing splitting of S1 - An S1 split can signify a RBBB in which the M1 will be markedly before the T1 (and A2 well before P2) - LBBB causes no splitting at all between the two - Similar to S2, A2 is always before P2 and the splitting is increased in breathing

What medication can you give someone who is bulemic?

SSRI - Fluoxetine, fluvoxamine, paroxetine, sertraline, escitalopram, citalopram - inhibit 5-HT reuptake - takes 4-8 weeks to show effects

75 yo w/ ringing in ears, nausea, fatigue, pulse 100, RR 24, BP 140/85, mild epigastric tenderness. pH 7.42, PCO2 30, PO2 95, HCO3- 19. What is causing this?

Salicylate poisoning - ASA initially activates the respiratory center in the brainstem causing hyperventilation and a respiratory alkylosis (low CO2) - after 12 hrs, the increased ASA uncouples oxidative phosphorylation -> build up of lactic acid -> metabolic acidosis (low bicarb) - combined states = normal or altered pH - toxic to CN VIII = tinnitus

Pregnant woman at 34 wks, has nausea/vomiting, abdominal pain for 12 hrs. prior labs are completely normal, now BP is 164/102 w/ RUQ tenderness. Her labs show low Hb/Hct/Plt, with elevates bili and liver enzymes. Peripheral blood smear will show what?

Schistocytes - HELLP syndrome - Hemolysis - Elevated Liver enzymes - Low Platelets - a manifestation of severe preeclampsia - Blood smear shows shistocytes - Can lead to DIC and hepatic subcapsular hematoma -> rupture -> severe hypotension - need to immediately deliver baby

24 yo w/ sickle cell w/ malaise and fatigue for a week. Her hct is usually 25-30% now is 13% w/ MCV 105 and reticulocyte of <1% what diagnostic test will explain the pt symptoms and decreased hct?

Serum parvovirus B19 IgM AB test - DNA virus -> all DNA are double stranded except parvo (part-of-a-virus) = smallest virus - naked virus - leads to aplastic anemia in sickle cell pt - infects RBC precursors and endothelial cells

42 yo w/ numbness and tingling in both legs for 2 weeks. Sensation to vibration is decreased in lower extremities and feet. DTR are decrease in knees/ankles. A romberg sign is +. What is causing this?

Sensory neuropathy - aka Charcot-Marie-tooth disease or hereditary motor and sensory neuropathy - progressive hereditary nerve disorders related to the defective peripheral nerves/myelin sheath - usually AD - Foot deformities (pes cavus, hammer toe) - lower extremity weakness (foot drop) - sensory defects - most common = CMT1A from PMP22 gene duplication - or from DM - the decreased ability to sense the world from issues with peripheral LMN nerves

1 week old baby has possible defect in fatty acid oxidation. Physical exam has no abnormalities. What lab serum level will you want to measure?

Serum acylcarnitine - medium-chain acyl-CoA dehydrogenase deficiency - decreased ability to break down fatty acids into acetyl-CoA -> accumulation fatty acyl carnitines in blood w/ hypoketotic hypoglycemia - missing acyl-CoA dehydrogenase to breakdown fatty acyl-CoA after coming out of carnitine shuttle

Rheumatoid arthritis is most similar to what other kind of immune reaction?

Serum sickness - both are type III hypersensitivity rxn = immune complexes (AG-AB mostly IgG) activate complement -> attracts neutrophils that release lysosomal enzymes - three things stuck together: AG-AB-complement - others: SLE, polyarteritis nodosa, PSGN, some drugs (penicillin = haptens), and hep B - RA: HLA-DR4 (4 walled rheum) - rheumatoid factor = IgM AB that targets IgG FC region - most specific AB = anti-cyclic citrullinated peptide

healthy 67 yo woman w/ 3 day hx of fever, SOB, non-productive cough, headache, muscle pain, temp of 102. What vaccine would have prevented this?

She has the FLU - influenza virus vaccine

5 yo girl w/ AIDS has vesicular rash. Antiviral therapy is ineffective and lesions worsen. Genetic mutation of what cell component is causing this resistance?

She probably has shingles/HSV - treat with acyclovir, famciclovir, valacyclovir = guanosine analogs that are monophosphorylated by viral thymidine kinase in infected cells only (less SE) - Then triphosphate formed by cellular ensymes - inhibit DNA polymerase by chain termination - mutated thymidine kinase = resistance - valacyclovir = prodrug of acyclovir w/ better bioavailability

29 yo woman wanting to lose weight takes thyroxine exogenously from a friend with a prescription. Her thyroid function is normal. What will her thyroid gland look like after several months?

She will have follicular atrophy - as you are providing the end product of the HPA axis, the T3,4 will provide NFB on the hypothalamus (no more TRH) and anterior pituitary (no more TSH) gland which will decrease production of endogenous T3,4 in the follicular cells of the thyroid

4 yo w/ fever, abdominal cramping, bloody mucoid stools and tenesmus for three days. Other children from daycare also have it. Micro exam shows methylene blue-stained fecal smear w/ numerous neutrophils. What is the organism?

Shigella sonnei - Not E. coli - both have shiga-like toxin that inactivates 60S ribosome by removing adenine from rRNA - Shigella = GI mucosal damage, dysentery, enhances cytokine release -> can cause HUS - E coli = enhances cytokine release and causes HUS, but unlike shigella, does not invade host cells - neutrophils in stool = dysentery or invasive cytotoxic = inflammatory diarrhea - bloody/mucoid stools and tenesmus are specific for shigella - methylene blue = also a specific stain for shigella -> will stain - also shigella is mainly fecal-oral while E coli you would mostly get while eating hamburger

37 yo farmworker comes in w/ 12 hrs of severe pain in abdomen/legs and spasms in jaw. PW shows spasms of masseter, and abdomen muscles. This presentation involves binding of a toxin to what?

Synaptobrevin - part of the SNARE complex -Synaptobrevin is degraded by tetanospasmin, a protein derived from bacterium Clostridium tetani (tetanus) - Clostridium botulinum toxin also cleave SNARE proteins, and synaptobrevin is target protein for several of the serotypes

58 yo AA with CHF, S3, ECHO w/ LV ejection fraction of 30%. Tx initiated w/ hydralazine and isosorbide dinitrate. What med is contraindicated for her?

Sildenafil - both hydralazine (vasodilate arterioles>veins -> afterload reduction) and nitrates (isosorbide dinitrate, veins > arteries -> decreases preload) increase cGMP - Adding sildenafil (phosphodiesterase inhibitors -> increases cGMP even more -> severe hypotension from vasodilation)

56 yo w/ lethargy has labs that show Na+ serum of 125, cortisol 20, Plasma osmolality of 275 and urine osmo of 450, urine Na+ of 30. What neoplasm does he have?

Small cell carcinoma of the lung - May be secreting ACTH (higher cortisol) and SIADH via production of atopic ADH

A drug that increases action of cGMP in the penis occurs in which cell type?

Smooth muscle cells of the deep artery - increased cGMP increases vasodilation of deep arteries (two at center of corpus cavernosa) -> proerectile - veins narrow -> traps blood in corpus cavernosa as this tissue expands it presses up on the deep dorsal vein preventing the blood to return -> erection via parasympathetic innervation from pelvic splanchnic (S2-4)

40 yo w/ 1 year of crampy abdominal pain, diarrhea, rectal bleeding w/ mucus. Abdomen is diffusely tender, and exam shows diffuse ulcers. What is the most appropriate initial therapy?

Sulfasalazine - a combination of sulfapyridine (antibacterial) and 5-aminosalicyclic acid (anti-inflammatory) - activated by colonic bacteria (okay to give NSAID type medication as it passes the gastric mucosa, and stays in the lumen) - Tx for UC, Crohn disease - malaise, nausea, sulfa allergen, reversible oligospermia - other tx for UC: other 5' aminosalicylic preps (mesalamine), 6-mercaptopurine, infliximab (targets soluble TNF-alpha), colectomy

17 yo girl is found to have osteosarcoma while with her parents in the ER. How will you tell her the news?

Speak to the patient and her parents about the findings together - don't speak to her alone - don't speak to parents alone

43 yo w/ immune thrombocytopenia purpura. Corticosteroids don't work, will undergo operation to improve condition. Removal of what structure is this?

Spleen - develop anti-GpIIa/IIIb AB -> splenic macrophage consumption of platelet-AB complex - primary or secondary to SLE, HIV< HCV, CLL - Increased Megakaryocytes in bone marrow - decreased platelet count - increased bleeding time - Tx: IVIG, rituximab, splenectomy

A woman has a large bump on the back of her wrist that transilluminates, is firm, and nontender. What is most likely to occur to this bump?

Spontaneous regression - Ganglion cysts = most common mass or lump in hand - not cancerous, harmless - most frequently develop on back of the wrist - fluid-filled cysts quickly appear, disappear, change size - ganglion rises out of a joint, like a balloon on a stalk - fluid, similar to fluid that lubricates your joints - can also get mucuous cysts on fingers - associated with use such as in gymnastics

In a cohort study of elderly women, relative risk ratio for hip fractures among those who exercise = 1.2 (95% confidence interval of 1.1 to 1.8). Which is the most appropriate conclusion about effect of regular exercise on risk for hip fracture?

Statistically significant overall increase in risk - odds ratio > 1 = increases odds/risk/likelihood - If 95% confidence interval range does not include 1, then the difference is statistically significant (not necessarily clinically meaningful)

25 yo woman w/ mitral valve dysfunction had dental work and took amoxicillin prophylaxis. She now has a new murmur, fever, and culture shows alpha hemolysis, w/ gram + cocci in chains. What is the organism?

Streptococcus mitis - a member of the viridans family - includes S. Mitis, S. mutans, S sanguinis - resistant to optochin (live in the mouth, not afraid O the chin) - S Sanguinis: maes dextrans that bind to fibrin-platelet aggregates on damaged heart valves -> subacute endocarditis (sanguinis = blood)

6 wk old has sickle cell anemia, daily tx w/ penicillin recommended -> this will decrease the risk of what?

Streptococcus pneumoniae - increase risk of infection w/ encapsulated organisms - penicillin treats mainly organisms w/o b -lactamase - h. flu/salmonells = gram -(not what they are using penicillin to treat for)

What can you use to treat irritable bowel syndrome?

Stress-related bowel issues w/ diarrhea + constipation = irritable bowel syndrome (IBS) - Lubiprostone = fatty acid that induces stool-softening intestinal secretions used for idiopathic constipation in IBS or opiate use

60 yo woman has right shoulder pain when raising arm or laying on right side. She has tenderness to palpation to right of deltoid and lateral to acromioclavicular joint. She can abduct her arm to 90 degrees, but additional abduction is prevented by pain. She has no impingement sign. What is the dx?

Subacromial bursitis - bursa that sits right under the acromion and right above the supraspinatus - helps motion of the supraspinatus tendon of rotator cuff in activities such as overhead work - from repetitive overhead movement - rotator cuff tendinitis = impingement sign is positive -> can be seen on MRI and pain in front and lateral side (painful on abduction) and atrophy of supraspinatous and infraspinatus muscles

28 yo 4 day palpitations, sever, neck pain, fatigue, malaise. pulse is 120 and regular. PE shows diffusely tender, mildly enlarged thyroid gland. No exopthalmos. Serum shows low TSH. What is the diagnosis?

Subacute granulomatous thyroiditis (de Quervain) - self-limited disease following a viral infection/flu-like illness - presents as hyperthyroid early in course -> hypothyroid (permanent in 15%) - granulomatous inflammation - increased ESR, jaw pain, very tender thyroid (de Quervain = in pain)

A 51-year-old man w/ lump on his tongue interfering w/ his speech + eating; 6.8-kg (15-lb) weight loss, smoked 1 pack of cigarettes daily and consumed six 12-oz bottles of beer on weekend nights during the past 30 years. evaluation confirms squamous cell carcinoma. It is most appropriate to evaluate which of the following lymph nodes first for evidence of metastasis in this patient?

Submental - Many oral cavity lesions, especially anteriorly such as the tip of the tongue, drain first to submental nodes

23 yo with sjogren and burning pain in feet is relieved with capsaicin. what neurotransmitter is causing pain?

Substance P

How does enterococcus faecalis develop resistance from vancomycin?

Substitution of d-lactate for d-alanine in peptidoglycan wall

60 yo has prolonged apnea after general anesthesia. Plasma cholinesterase activity is abnormal. What agent is prolonging apnea?

Succinylcholine

Female w/ isoflurane anesthesia develops temperature and muscle rigidity. Simultaneous administration of what compound caused this?

Succinylcholine - Malignant hyperthermia - skeletal muscle hypersensitivity to anesthetics and succinylcholine - related to defect in ryanodine receptor in sarcoplasmic reticulum (necessary in muscle contraction and calcium levels) - if abnormal the channel releases an abnormal amount ca+ into cytoplasm w/ anesthetics -> heat production, fever, muscle rigidity - treat with dantrolene (muscle relaxant -> blocks ryanodine receptors)

A 53-year-old man has 6-month hx of blood in stool. He has had no pain w/ defecation. PE = 1-cm, visible anal mass below dentate line. If mass is malignant, it is most appropriate to evaluate which of the following lymph nodes for possible metastasis?

Superficial inguinal - Below the dentate line: anal cancer drainage is superficial inguinal LN, Somatic innervation (painful external hemorrhoids), inferior rectal artery (branch of internal pooodendal), inferior rectal vein -> internal pud ->internal iliac -> IVC, location of squamous cell carcinoma - Above dentate line: superior rectal (then internal iliac), visceral nerves, superial rectal artery (branch of IMA), Superior rectal veins -> IMV -> splenic vein -> portal vein, non-painful internal hemorrhoids, location of adenocarcinoma

Severe pain after meals (better when she doesn't eat), unintentional weight loss, hx of atherosclerosis + abdominal bruit and absent pedal pulses. What artery could be atherosclerotic?

Superior Mesenteric

2 day old has bilious emesis, abdominal tenderness, and hemodynamic deterioration. An upper GI barium shows ligament of treitz on right of abdomen and duodenal obstruction. What artery is occluded?

Superior mesenteric artery - ligament of treitz: marks the division of upper and lower GI tract (important for bleeding) - connects the duodenum to connective tissue surrounding SMA - point of fixation for rotation of gut -> here = malrotation

56 yo woman frequently burns herself, loss of pain/temperature in both upper extremities. Touch, vibratory sensation and proprioception are normal. What will be on an MRI?

Syrinx of the central region of the spinal cord from c4-T5 - cystic cavity blocks anterior white commisures (decussation of the spinothalamic tracts) - "cape like" loss of pain and temperature - associated with chiari I malformations or from trauma

woman with invasive squamous cell carcinoma of cervix from HPV 16. What cells recognize and kill these virus infected tumor cells?

T lymphocytes

6 month old w/ recurrent URI, hypocalcemia, repair of coarctation of aorta, broad nasal bridge, dysplastic pinnae. What is causing his recurrent infection?

T-lymphocyte deficiency - DiGeorge Syndrome -22q11 deletion - failure of 3 and 4th pharyngeal pouch (no thymus and parathyroid) - hypocalcemia - tetany (involuntary muscle cramps and contractions) - CATCH 22: Cardiac defects, Abnormal facies, Thymic hypoplasia, Cleft palate, Hypocalcemia - velocardiofacial syndrome: palate, facial, cardiac defects

20 yo w/ appendicitis and periumbilical pain that localizes to Mcburney's point. This is bc the periumbilical region body wall and appendix are both supplied by afferent fibers w/ sensory cell bodies in dorsal root ganglia of what spinal nerve level?

T10 - acute inflammation of appendix from obstruction from fecalith (adult) or lymphoid hyperplasia (children) - obstruction increases intraluminal pressure -> stimulates visceral afferent nerve fibers (T8-T10) -> initial diffuse periumbilical pain - inflammation then extends to serosa and irritates parietal peritoneum = localized pain to RLQ at Mcburneys point - can perforate = peritonitis - psoas (hip extension), obturator(crossing leg over), and rovsing sign (pressing on LLQ -> pain in RLQ), guarding adn rebound tenderness on exam

15 yo from India w/ hypopigemented, hypoesthetic lesions on her neck for 2 weeks. Bx shows acid-fast bacilli. What do these organisms result in dermal vs. visceral infections?

Temperature sensitivity - mycobacterium leprae likes cool temperatures (skin/superficial nerves -> glove and stocking loss of sensation) - cannot be grown in vitro - dx via skin bx PCR - reservoir in US = armadillos - Two forms most people are in between: 1. Lepromatous: diffuse, leonine like facies, communicable w/ high bacterial load, from low-cell mediated immunity w/ Th2 response (L = lethal) 2. Tuberculoid: a few hypoesthetic (decreased sensation), hairless skin plaques, high cell-mediated immunity w/ Th1 response, low bacterial load. tx: dapsone, rifampin for tuberculoid and clofazamine added for L form

42 yo had generalized tonic-clonic seizure. She tells the physician she smelled a bad smell right before. What region of her brain did the seizure arise?

Temporal lobe - Frontal lobe: coordinates information other area and controls behaviors. Skeletal muscle movement (in front of central sulcus) -> motor association area, and primary motor cortex, Broca's area, frontal eye field (in pre-frontal cortex) - Parietal lobe: behind central sulcus -> primary somatic sensory cortex and sensory association area = sensory info from skin, msk, organs, taste buds - occipital: visual association cortex, visual cortex - temporal: auditory cortex/association area, gustatory cortex, olfactory cortex, Wernicke area (comprehends language -> broca's for verbalization via arcuate fasciculus = damage = pt can't repeat words)

4 month old boy w/ rare autosomal recessive skeletal dysplasia + abnormal bone formation has mutation in gene that controls traffic vesicles into the golgi complex. Electron microscopy will show what?

dilated rough endoplasmic reticulum - If ER could not move proteins to golgi via COPII -> ER would have build up of proteins - two steps forward (anterograde motion of COPII), one step back (retrograde motion of COPI)

On colonoscopy you see a large pendunculated mass in the transverse colon, about the size of a strawberry. What is this?

Tubular adenoma - the growth of a polyp -> normal colon -> loss of APC gene (decreases intracellular adhesion, increased proliferation) -> colon at risk -> KRAS mutation (unregulated intracellular signaling) -> adenoma -> Loss of tumor supressor genes (TP53, DCC) -> increased tumor genesis -> carcinoma - Overexpression of COX-2 implicated in CC -> NSAIDS may be chemopreventive

person being treated for rhinitis with chlorpheniramine for 4 weeks will most likely develop what side effect?

drowsiness - 1st generation H1 blocker - weight gain, dizziness, hypotension - cognitive impairment in elderly - treat extrapyramidal side effects of antipsychotics (rebalance Ach/dopa balance)

woman w/ RA and treatment w/ prednisone and methotrexate have not been effective. The next step in therapy will block which cytokine?

Tumor necrosis factor alpha - adalimumab (humira) - certolizumab, golimumab, infliximab target soluble TNF alpha -Enteracept is a decoy TNF alpha receptor, not a monoclonal AB (binds excessive TNF alpha w/ a receptor itself)

32 w/ 3 month hx of swelling and tenderness of breasts. He has been receiving thyroid hormone and steroid replacement after removing pituitary adenoma. He began hCG replacement 4 months ago. What is the binding site and action of this hormone causing the gynecomastia in this pt?

The hCG has the same alpha subunit at LH -> Binds to testicles -> increases the amount fo testosterone made which is peripherally converted to estradiol -> enlarges breast tissue (similar idea of taking exogenous testosterone -> too much will be converted peripherally)

meta-analysis of three genome-wide studies of crohn disease. Have 3200 cases and 4800 control. Initial studies show 11 loci w/ OR >1.3-1.5. The combined metanalysis ID 21 more loci w/ OR 1.1-1.3. Estimated 32 loci explain 10% of overall variance in disease risk w/ two loci = 2% of the variance. Previous studies show 50% heritability rate for Crohn disease. What explains these results?

The identified loci account for a relatively small part of the variance - 50% heritability and there is 12% identified in these studies - this implies that these variance are not explaining the total variance represented by the 50% heritability in the disease itself

35 yo felt down for past 2 years, and not fully enjoyed life, unmotivated/pessimistic, little energy for relationships/work. no changes in appetite or sleep, and is able to work. has had thoughts life is not worth living, but no suicidal thinking/planning. What does he have?

dysthymic disorder

25 yo man with high temp after running a race. His temp will cool back down because of what?

evaporation of sweat

healthy 2 day old has jaundice due to reduction in the rate of what?

decreased conjugation of bilirubin to glucoronic acid - at birth immature UDP-glucuronosyl transferase -> unconjugated hyperbilirubinemia -> jaundice/kernicterus (deposition of unconjugated bili, lipid soluble bili in brain specifically basal ganglia) - in first 24 hrs of life, resolves w/o tx in 1-2 weeks - can give phototherapy to isomerize unconjugated bili to its water soluble form

23-year-old for genetic counseling prior to conception. Her brother + maternal uncle = Duchenne muscular dystrophy (DMD) + died at 28/17. Serum studies show a muscle creatine kinase of 120 U/L (N=22- 198). patient's 50-year-old mother has creatine kinase of 300 U/L. Which is patient's carrier status?

The patients DMD carrier status is uncertain bc of random X inactivation - DMD is X-linked - mom is a carrier (brother had it) and bc lab testing - mom has 2 X chrom, only one is mutated - no way to know which her daughter received + expresses by her phenotype (if she is a carrier or not) - Just because her CK is normal doesn't mean she isn't a carrier-the phenotype of the X-linked carrier depends on X-inactivation

Newborn baby with hypothyroidism (no thyroid) has no symptoms. What can the mom transfer to the baby allowing for normal development?

Thyroxine (T4) is transferred in late gestation through the placenta preventing the initial symptoms of congenital hypothyroidism

severe bleed from gunshot wound to abdomen -> 3L blood in peritoneum. Cells in liver/kidney = centrilobular pallor and swelling. Why is this occurring?

decreased function of Na+/K+ ATPase - reversible injury from blood loss

A 30-year-old woman w/ 2-day abdominal pain, hx of recurrent URI, sinusitis, pancreatitis. Has thick nasal secretions. She says that her sweat is salty and crystallizes on her skin. Genetic testing for 36 most common mutations shows a detectable mutation (G551D) in one allele of CFTR gene. Which of the following best explains this patient's clinical phenotype?

The second CFTR mutation was not detected by the testing obtained - Cystic fibrosis = AR of CFTR, you need a double hit to express disease. If test only picked up one, then it must have missed other

Male born w/ right sided aortic arch. He has full cheeks, low set ears, and a small chin. He has a decreased calcium concentration. What is the site of the malformations in this child?

Third and fourth branchial pouches - This child has DiGeorge's syndrome - 22q11 deletion: poor development of the 3/4 pharyngeal pouches -> thymic aplasia, hypocalcemia (no PTH), cardiac defects - 3rd pouch: inferior parathyroids, thymus (end up below 4th pouch structures - 4th pouch: superior parathyroid glands, parafollicular C cells

76 yo man w/ pulsatile abdominal mass -> aortic aneurysm and horseshoe kidney. Surgical repair of the aneurysm will be challenging because of the presence of what?

anomalous origins of multiple renal arteries to each kidney - as kidney sit in the pelvis they receive certain blood supply, as they ascend into the abdomen in embryology this blood supply dissipates and reforms as the renal arteries in the abdominal aorta. - in horseshoe kidney may have as many as 3/4 arteries from various parts of the aorta providing a complication during surgery

what is slipped-strand mispairing?

This is when a DNA sequence has two regions along the strand where there are analogous complementary regions - The wrong portions of the DNA match up and bind -> a loop of DNA is left unbound - the loop is degraded -> shorter DNA strand and an early stop codon

An asymptomatic 30 yo comes in for routine exam and PE shows nontender adnexal mass on left side -> 6cm fluid filled cyst. What caused this ovarian cyst?

anovulation - follicular cyst: distention of unruptured graafian follicle (ovum developing) - associated w/ hyperestrogenism, andometrial hyperplasia, most common ovarian mass in young woman

in wound healing, 2 wks after burn tissue shows increased fibroblast migration and proliferation, increased collagen and fibronectin, and decreased degradation of ECM by metalloproteinases. This is caused by production of what?

Transforming growth factor B - three phases: 1. inflammatory: (3 days): plts, neutrophils, macrophages -> clot formation, increase vessel permeability, cells migrate to injury and macro clear debri 2 days later 2. Proliferative (day 3-wks): fibroblasts, myofibroblasts, keratinocytes, macro -> granulation tissue, type III collagen, angiogenesis, epithelial cell proliferation, wound contraction via myofibroblasts - TGF-B angiogenesis and fibrosis 3. Remodeling (1wk-6+months): fibroblasts -> type III collagen replaced by type I collagen (increases tensile strength of tissue). need collagenase to break down type III collagen needs zinc (no zinc = no wound healing)

What paraneoplastic syndrome can be caused by large cell lung cancer?

bHCG - may cause gynecomastia in men -

33 yo w/ splenic laceration in MVA. three units of packed RBC given. 5 hrs later -> shortness of breath, RR 29, cackles in lung fields, CXR b/l lung infiltrates. Arterial PO2 = 55 mm hg. What happened?

Transfusion-related acute lung injury - rare but serious syndrome - sudden acute respiratory distress within 6 hrs following transfusion - activation of recipient neutrophils by donor-derived AB targeting HLA or human neutrophil antigen (HNA) - activation of neutrophils causes neutrophil-mediated endothelial damage and capillary leak

48 woman in early menopause and thinning of vaginal tissue. what serum concentration is increased?

follicle-stumulating hormone - specific for menopause as you lose the negative feedback from decrease estrogen - LH will no longer have a surge - increased GnRH - HAVOCS: hot flashes, atrophy of vagina, osteoporosis, coronary artery disease, sleep disturbances

An increase in the amount of iron in hepatocytes -> increased ferritin gene products in Western blotting. it does not change the amount of ferritin detected by Northern blotting or PCR. Iron increases the frequency of what cellular process?

Translation of ferritin mRNA - Western blotting: protein - Northern: RNA - Southern: DNA - SNoW DRoP - no more RNA is made, only an increase in proteins = increased translation

40 yo woman w/ hx of low-grade follicular B-cell lymphoma. 1 week ago she completed a 4-month course of rituximab and is in complete remission. She appears well. What is she at increased risk for?

bacterial pneumonia - also has risk of PML from JC virus (multifocal leukoencephalopathy) - this drug causes immunosuppression with increased risk of infection - initial risks (not after 4 months like this woman) = infusion reaction (headache, fever, dyspnea, pruritis, hypotension and serum sickness (7-10 days after initiation, again not 4 months after)

57 yo man w/ 6 months of constipation and rectal fullness, DRE no palpable lesion. Stool occult blood = positive and colonoscopy shows mass in sigmoid colon w/ adenocarcinoma on Bx. Which lymph nodes will this initially spread to?

inferior mesenteric - follow vasculature

17 yo needs emergent operation and parents cannot be contacted for permission. The physician decides to proceed without permission. What ethical principal is this?

beneficence - doctors acting in best interest of pt - may conflict with autonomy or what is best for society (mandatory TB tx) - pt interest supersedes - vs. nonmaleficence = do no harm. only conducitng procedures where the positive outcomes outweighs the risks (most surgeries/medicines) - common good principle is not a real thing

What will a liver bx of HCV show?

infiltration of lymphocytes, plasma cells, and macrophages - lymphoid aggregated w/ focal areas of macro-vesicular steatosis - signs of chronic inflammation = lymphocytes + plasma cells (not edema and neutrophils)

Drug X is being studied and shows an increase in phrenic nerve activity upon administration and a decrease in abdominal pressure/no more breathing what drug is this?

Tubocurarine - competitive Ach antagonist - can reverse blockade w/ neostigmine (give w/ atropine to prevent muscarinic effects of bradycardia)

24 yo w/ 8 year hx of seizure disorder refractory to meds, flesh colored papules in nasolabial folds w/ hypopigmented ash-leaf macules. MRI of brain shows subependymal nodules. What's the dx?

Tuberous sclerosis - TSC1 mut on chrom 9 -TSC2 on chrom 16 (Both = tumor suppressor genes) - AD w/ variable expression - Hemartomas in CNS - Angiofibroma (flesh colored papules in nasolabial folds) - Mitral regurge - Ash-leaf spots - Rhabdomyoma -Mental retardation -Angiolipomas (blood vessel/fat tumors) - Seizures - shagreen patches (plaque-like irregular soft skin lesions)

9 yo w/ mental retardation, seizures, systolic murmur, pink/yellow papules on bridge of nose and along nasolabial folds. CT shows calcified nodules on gyri in brain, and subvalvular mass in heart septum. What is the diagnosis?

Tuberous sclerosis - AD - TSC1 (chrom 9), TSC2 (chrom 16) mutations = tumor supressor genes - hamartomas in CNS/skin - angiofibromas (little dots on nose) - mitral regurge - ash leaf spots (pale spots on skin) - mental retardation - seizures - shagreen patches (cluster of hive-like bumps) - angiomyolipoma (benign renal neoplasm composed of fat, vascular, and smooth muscle elements)

18 month old boy w/ seizures, mild developmental delay. No family hx of seizure disorder or illness. PE = six hypopigmented macules on torso and raised, flesh-colored lesions on back. An MRI of brain shows multiple lesions in cerebral cortex. What is dx?

Tuberous sclerosis complex -AD, variable expression - TSC1 mut on chrom 9, or TSC2 on chrom 16 - tumor suppressor genes - ash-leaf spots, mental retardation, seizures - flesh colored lesion = angiofibromas - multiple lesions on cerebral cortex -> will not see in brain - will not see cortical lesions in brain either - NF in brain: neurofibromas on periphery or around any nerve sheath (anywhere in body from neural crest cells), hemartomas in iris, and gliomas of optic nerve - NOT NF1 bc that is AD with 100% penetrance mutation of NF1

What will the levels of type I/type II pneumocytes and fibroblast be in a pt with chronic inflammatory interstitial pneumonitis?

Type I: decreased as disease is ruining basement membrane Type II: increased and the progenitor cells try to rebuild lung tissue Fibroblasts: Increase as fibrosis is expected after long-term inflammation - pneumonitis: mixed type III/IV hypersensitivity to environmental AG -> dyspnea, cough, tightness, headache. In farmers, bird fanciers lung. can be reversed if stimulus is removed

39 yo w/ 1 week of red spots on shins, joint pain, and fatigue. He has purpura on LE. Liver edge is palpated 4cm below right costal margin. labs show: leukocyte 10,000, AST 142, ALT 154, Hep C RNA +, Anti-Hep C AB+, cryoglobulins +, C4 low, urine shows protein and RBC/ What type of hypersensitivity is this?

Type III immune complex mediated (AB-AG-complement) - mixed cryoglobulinemia - from viral infections like HCV - triad of palpable purpura, weakness, arthralgia, and may also have peripheral neuropathy and renal disease (MPGN>MN) - cryoglobulins are IG that precipitate in the col - vasculitis due to mixed IgG and IgA immune complex deposition - also MPGN and MN are both immune complex mediated, type III rxn

HPV has E6 causes P53 degradation by targeting these proteins to which cellular enzyme?

Ubiquitin ligase - ubiquitin-tagged proteins are sent to the proteasome that degrades damaged/ubiquitin tagged proteins - defects implicated in parkinsons

26 yo with tenesmus and bloody diarrhea, normal terminal ileum. Bx has microabscesses and crypts and a depletion of mucus from goblet cells. What is the diagnosis?

Ulcerative colitis -continuous lesions -always involved the rectum - mucosal/submucosal inflammation only - lead pipe appearance on imaging - crypt abscess and ulcers

72 yo with multiple myeloma's B and T lymphocytes are isolated and undergo southern blot analysis of lymphoid specific genes. A single B lymphocyte J-region probe is used and a B lymph shows 1.5Kb band. The T-lymph DNA w/ same band shows 6 Kb. Why?

Unrearranged immunoglobulin gene - in B cells - Generation of antibody diversity (AG independent): via random recombination of VJ (light chain) or V(D)J (heavy chain) genes - random addition of nucleotides to DNA in recombination by TdT enzyme - random combo of heavy chains w/ light chains - generation of AB specificity: AG dependent is via somatic hypermutation and affinity maturation (variable region) - isotype switching (constant region) - B cell: from bone -> secrete IgG on surface -> circulation adn are activated w/ CD4+ t-cells via TH2 dependent, or independent activation (immediate responds to LPS/polysaccharide) -> IgM response with no memory = weak but immediate response hence why IgM increases after response to novel AG - or B-cells recognize protein they eat and process to MHCII -> presents after lysosomal degredation -> t-cell in lypmh, spleen, MALT and is activated by T cell - activated = - isotype switchig: b-cell makes IgM-> IgG/E/A (via CD40 biding to CD40L on t-cell) still in lymph tissue - this also = somatic hypermutation: induces by activated T-cell causes b-cell to increase mutation rate of Ig gene -> decrease specificity of idiotypic region for AG and sometimes will increase the specificity and these cells will outcompete the other b-cells, and others will die - and affinity maturation: this is the outcome -> when b-cell make more specific AB to AG due to somatic hypermutation followed by clonal selection leading to this affinity maturation and outcome of higher binding - separate topic: random assortment V(D)J: in bone marrow for b cell and in thymus for t cell - b-cell has one Ig heavy on chrom 14 -> have various V, D, J regions and light segments - heavy chain chooses one V and D segment and combines them then those are combined to J segment -> random combination - light chain will chose btw V and J - t-cells will also chose these combinations -> both heavy and light have VDJ recombination - RAG: gene that makes these in both - tdt: randomly inserts nucleotides into the hypervariable region of AB (can use to tell ALL from AML) - leads to AB diversity

In paraneoplastic syndrome of SIADH. What do you expect the urine Na+ and the serum osmolality to be?

Urine Na+ increased Serum Osmolality decreased - may also have increased BP (increased fluid), decreased renin, decreased ALDO - hyponatremia - increased ADH - hyponatremia w/ continued Na+ excretion - urine osmolality > than serum osmolality - body responds to H2O retention by increasing ANP/BNP, decreasing ALDO - the increased ANP/BNP excretes more Na+ trying to excrete more water - may -> cerebral edema, seizures, correct slowly to avoid osmotic demyelination - first line tx: fluid restriction and let body balance itself - ADH antagonists: conivaptan, tolvaptan, demeclocyline (tetracycline)

35 yo w/ 2 days blistering lesions in sun-exposed areas of face, arms, hands that she has had before. On oral contraceptive pills. Her AST, ALT, total porphyrin, and urine uroporphyrin III is increased. What enzyme is deficient?

Uroporphyrinogen decarboxylase - uroporphyrinogen III -> coproporphyrinogen III - This is porphyria cutanea tarda which is AD mutation - uroporphyrin (tea colored urine) excess - most common, exacerbated by alcohol, assocaited with hep C - tx: phlebotomy, avoid sun, and antimalarias (hydroxychloroquine) - other diseases: - In mito: ALA synthase (rate limiting step) -> sideroblastic anemia (x-linked) - In cyto: ALA acid -> Poprphobilinogen (via ALA dehydratase) = lead poisoning - poprphobilinogen -> hydroxymethylbilane (via porphobilinogen deaminase aka uroporphyrinogen synthase) = acute intermittent porphyria - and ferrocholetase in mito = lead poisoning (protoporphyrin -> heme)

50 yo w/ progressive dyspnea over 6 months, now even at rest w/ persistant dry cough since having a respiratory infection a year ago. Lung tissue shows chronic inflammation w/ fibrous thickening of the alveolar septa. What is his dx?

Usual interstitial pneumonitis - restrictive lung disease (decrease lung volumes, decrease in FVC, and TLC) - PFT: increase FEV/FVC ratio -> short shallow breaths - poor breathing mechanics (normal DLco and normal A-a gradient - muscle weakness/obesity/scoliosis) vs. Interstitial lung disease (decrease DLco and increase A-a gradient) - pneumoconiosis (coal), sarcoidosis, (ACE, Ca+), idiopathic fibrosis, goodpasture syndrome, granulomatosis w. polyangitis, hypersensitivity pneumonitis (mixed type III/IV hypersensitivity reaction to environmental antigen -> dyspnea, cough, chest tightness, headache. usually in farmers or bird fanciers lung -> reversible if stimulus avoided in early stages)

50 yo w/ azotemia, renal US shows b/l hydroureters and hydronephrosis. What cancer does she have?

Uterine cervix - blocks the entry of the ureters at the trigone/insertion site causing back-up

Which clotting factors is first to be decreased by 50% after initiation of warfarin?

VII (proconvertin) - shortest half life of all of the clotting factors - Warfarin will only work once the already made clotting factors are depleted from blood, and then the blocking of epoxide reductase which prevents gamma carboxylation of vitamin K dependent clotting factors II, VII, IX, X can work

5 yo boy on chemo for ALL has fever, cough, RR 46, temp 101, PE shows vesicular rash, crackles in both lungs, and CXR shows nodular infiltrates in both lung fields. What is causing this?

Varicella-zoster - herpes family, dsDNA virus, enveloped (zeus) - can cause pneumonia - spreads via respiratory droplets - multinucleated giant cells - may develop encephalitis in adults - treat w/ acyclovir in adults -in immunocompromised it is easier to get complications such as penumonia - there is a live vaccine (wouldn't give to immunocomped) - torches infection: can cause limb hypoplasia, scaring in dermatomal pattern, blindess

24 yo had a burn and examination of the wound shows a 7cm, pink, granular, edematous wound. Formation of this tissue was caused by increased activity of what?

Vascular endothelial growth factor Phases of wound healing: 1. inflammatory (up to 3 days): platelets, neutrophils, macrophages -> clot formation, increase vessel permeability, and neutrophil migration into tissue. Macrophages clear debris 2 days later 2. Proliferative (3-weeks): fibroblasts, myofibroblasts, endothelial cells, keratonocytes, macrophages -> Deposition of granular tissue and type II collagen, angiogenesis (via VEGF), epithelial cell proliferation, dissolution of clot, and wound contraction (myofibroblasts) 3. Remodeling (1 wk-6+ months): fibroblasts -> type III collagen replaced by type I to increase tensile strength. Collagenase (requires zinc -> deficient = delayed healing) break down type III

Pulmonary embolism causes what findings on a V/Q scan?

Ventilation perfusion relationship will show increase in dead space

18 month old who has not eaten in 24 hrs has high pulse, increase respirations, hepatomegaly. His glucose, ketones, and carnitine are decreased in serum, and urine ketones are decreased, and dicarboxylic acids are present. He is given medium chain triglycerides for 6 months which helps. What is deficient?

Very long-chain acyl-Coa dehydrogenase deficiency - hypoketonic hypoglycemia - fat deposits in liver = hepatomegaly - cardiorespiratory distress

49 yo female w/ bc has numbness in both hands and feet during chemo. Sensation and pinprick/fine touch is decreased over hands/feet. Adverse effect from chemo. Why?

Vincristine - Microtubules polymerization is inhibited -> nerves unable to use microtubules for vesicular transport on MT - think dynein and kinesin

35 yo has fever and sharp chest pain, temp of 101. Friction rub is heard on chest, and all secondary causes of pericarditis are ruled out. What is the most likely cause of primary pericarditis in this patient?

Virus - causes are idiopathic (most common -> presumed viral) - confirmed infection (coxsackievirus B), neoplasia, autoimmune (SLE, RA), uremia from CKD, CV (acute STEMI or dressler syndrome), radiation therapy - inflammation of the pericardium - sharp pain made worse on inspiration and relieved by sitting up and leaning forward - complicated by pericardial effusion - friction rub and ECG changes with widespread ST-segment elevation and PR depression - tx: NSAID, colchicine, and/or glucocorticoids

Overdose on vitamin D (15 yo thought she could kill herself). one month later, labs show high calcium. Why is her calcium so elevated?

Vitamin D -> increased absorption of calcium in the GI tract - PTH usually -> turns 25-OH D3-> 1 alpha-OHD3 - activated vita D goes to bone to increase calcium and phosphate release from bone and to intestine to increase absorption of Ca+ and Phos

9 yo boy adopted from African country. Has difficulty walking and fatigue. Normal pulses, neuro exam shows weakness in plantar dorsiflexion and foot intrinsics. He has broad based, ataxic gait. Sensation to light touch is decreased over lower extremeties and no vibration sense. Labs: Hb/hct decreased,MCV normal, Increased lactate dehydrogenase. What vitamin is he deficient in?

Vitamin E - antioxidant (protects RBC and membrane from free radical damage) - deficient: hemolytic anemia, acanthocytosis (spikey RBC), muscle weakness, demylination of posterior columns (decreased position and vibration sensation) and spinocerebellar tract (ataxia) - similar to B12 but without megaloblastic anemia, hypersegmented neutrophils and no increase in methylmalonic acid - increased vita E = enhanced anticoagulant effect of warfarin (also inactivates vita K) - two cells types = acinocytes (burr cells = vita E deff or pyruvate kinase deff, or uremic/on dialysis) and akanthocytes (spurr cells) -> made in abatelipoproteinemia

16 yo girl with cystic fibrosis, weakness, numbness, and tingling of arms and legs, difficulty walking with ataxic gait. Non-adherent medication. What is she deficient in?

Vitamin E - CF -> dysfunction of exocrine pancreas -> malabsorption of fat soluble vitamins (DEAK) - looks like vitamin B12 deficiency, but no megaloblastic anemia, hypersegmented neutrophils or increased serum methylmalonic acid (which is normal in folate acid def too)

55 yo w/ fever, chills nausea, diarrhea and no drinking or eating, w/ little urine in the past 12 hrs. His BP goes from 110 supine -> 80 standing. He has high urea nitrogen, creatinine, and uric acid in serum. Urine: specific gravity 1.030, no RBC or protein, Na of 10, and creat of 19. What is causing these findings?

Volume depletion - AKI: abrupt decline in renal function - Prerenal azotemia: due to decreased RBF (hypotension), decreased in GFR, sodium, water, and urea retained by kidney in attempt to conserve volume - high BUN/creatinine ratio (>20) -urine Na <20 -urine osmolality >500 (concentrated urine)

Newborn w/ bulging, fluid filled mass in midline over lumbosacral region and no spontaneous movements of the lower limbs. In which timeframe did this anomaly occur in embryonic development?

between day 15-40 - notocord induces overlying ectoderm -> to become neuroectoderm and form neural plate that becomes neural tube + neural crest cells - notochord -> nucleus pulposus - folic acid supplement needed

28 yo woman 1 wk fever, chills and 10lb weight loss. she has 5 year hx of chronic sinusitis, and has had many operations for sinus obstruction. Temp is 102, she has diminished nasal septa, chest x-ray w/ pulmonary nodules and serologic studies of AB against proteinase 3. What does she have?

Wegener granulomatosis - small vessel vasculitis - Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis - hemoptysis, cough, dyspnea - renal hematuria/red cell casts - TRIAD: focal necrotizing vasculitis, necrotizing granulomas of the lung and upper airway, necrotizing glomerulonephritis - PR3-ANCA/c-ANCA (anti-proteinase 3) - CXR: large nodular densities - give cyclophosphamide/corticosteroids

Monoclonality of neoplastic cells in endometrial cancer is best determines by analysis of what?

X chromosome-linked isoenzymes - monoclonality: tells us the neoplastic cells or from single mother cell - historically use G6PD enzyme isoforms on X chrom - inherit one from each parent and they are X-linked - one isoform is randomly inactivated on the x-chromosome via lyonization - normal ration of G6PDA and G6PDB is usually about 50% as one of each is randomly inactivated - 1:1 ratio is maintain in hyperplasia - In neoplasia you will only see G6PDA in excess as one cell is deriving all of the new cells - can also use adrogen receptor isoforms on X chrom as well - same idea in B-cells -> either kapp or lamba (usually a 3:1 ratio) but in cancer = 20:1 ratio -

23 yo w/ ALL going to get treatment w/ intense chemo. What do you want to give her prior to treatment to prevent kidney failure?

Xanthine Oxidase inhibitor (allopurinol) - Tumor lysis syndrome: oncologic emergency from massive tumor cell lysis most often in lymphoma/leukemias - release of K+, Phos = hyperkalemia (muscle weakness, arrythmia)/hyperphosphatemia (calcium phos crystal -> stones) - hypocalcemia from Ca+ sequestration by increased phos binding of free Ca+ (seizures, tetany) - hyperuricemia -> AKI, stones - tx: aggressive hydration, allopurinol, and rasburicase (catalyzes uric acid -> allantoin)

5 yo who lives on a farm has diarrhea, cramping, abdominal pain, and fever for 2 days. He drinks unpasteurized milk. Organism grows at 4 degrees celcius. What is it?

Yersinia enterocolitica - gram negative, transmitted through puppy feces - mostly in children - transmitted through contaminated milk products - resistant to cold temperatures (ice on building outside of puppy store, baby drinking milk from bottle) - stains heavily on two ends (bipolar) - encapsulated and causes bloody diarrhea - mimics appendicitis

3 yo short for her age and small w/ coarse facial features, contractures of large joints. X-ray shows dysostosis multiplex. Lysosomal enzyme analysis shows B-hexosaminidase, B-glucoronidase, B-galactosidase, and alpha-fucosidase. What is causing this?

abnormal targeting of these enzymes to lysosomes - Mucolipidosis II (ML-II) = fatal inherited metabolic disease - deficiency of GlcNAc-phosphotransferase, -> generates mannose 6-phosphate - lysosomal acid hydrolases are mistargeted, lysosomes filled w/ undigested substrates

17 yo has septic shock that is unresponsive to IV ADH (vasopressin). High does Dopamine is given. Stimulation of what receptors will benefit this patient?

a1-adronoreceptors - Dopamine works by stimulating the following D1=D2>B>a - used in unstable bradycardia, heart failure, shock - LOW doses act on D1 receptors to increase renal blood flow - inotropic and chronotropic effects at MEDIUM doses due to B effects - vasoconstriction at HIGH doses due to alpha effects - given after failed NE in shock

If a mother with hypothyroidism has a TSH that increases during pregnancy, what is her child at risk for?

abnormal brain development - growth from TH stimulates growth after pregnancy - TH more important for brain growth in utero, as well as after + bodily growth after birth as well - no TH in utero = mental retardation

25 yo w/ 2 years of intermittent diffuse cramping lower abdominal pain + 2-6 loose, watery stools relieved by defecation. Btw episodes her stools are normal. PE is normal. The drug you can use has what mechanism?

accentuation of U-opioid myenteric plexus receptor - loperamide - U-opioid receptor agonist, slows gut motility, poor CNS penetration (low addictive potential) - use = diarrhea - SE: constipation/nausea - she has irritable bowel syndrome: recurrent abdominal pain associated with >2: pain with defecation, change in stool frequency, change in form of stool - no structural abnormalities, common in middle aged women - may have diarrhea/constipation or both - first line: lifestyle changes/dietary changes

24 yo has purple spots over legs. three months ago she has splenectomy for immune thrombocytopenic purpura. Labs shows Hb 13.5, hct 40%, leukocyte 10,000, platelet 25,000, PTT 29 sec. Her plt after operation were 20,000 -> 100,000 and she had howell-jolly bodies. Today she has no howell-jolly bodies. What explains this?

accessory spleen - small nodule of splenic tissue found apart from main body of the spleen - in 10% of the population and are 1 centimetre in diameter. They may resemble a lymph node or a small spleen - hence why she no longer has howel-jowell bodies, and why plts are still being removed

Immediate therapy for a DVT has what mechanism?

activation fo antithrombin III -Heparin

70 yo had repair of fractured femur. She weighs 143, what is the most important predictor of success in rehabilitation of this patient?

activity levels before fracture

What mediator can increase coronary blood flow?

adenosine and NO

35 yo male w/ acromegaly and increased insulin-like growth factor 1. Tumor w/ dense granulated somatotroph adenoma w/ Gs subunit that lack GTPase activity. the tumor cell has an increase in what enzyme?

adenyl cyclase - tumors with constitutively active G proteins can overstimulate adenyl cyclase and increase CAMP

30 yo w/ tx of hodgkin 2 months ago calls his physician frequently. He takes his temp very often, and contacts the office when it is elevated. Evaluation shows no evidence of recurrence but he is scare that it may not be detected rapidly enough. What does he have?

adjustment disorder w/ anxiety - emotional symptoms (anxiety, depression) that occurs in 3 months of an identifiable psychosocial stressor (divorce, illness) lasting < 6 months once stressor has ended. If symptoms persist = GAD - symptoms cannot meet criteria for MDD - tx: CBT, SSRI

chinese peanut farmer with 10cm hypodense lesion in liver shows dysplastic hepatocytes in small clusters w/ no normal architecture. Exposure to what toxin cause this?

aflatoxin (from aspergillus) in stored grains and nuts - hepatocellular carcinoma

1 yo boy w/ fine, pale hair and blue eyes. what is causing this pale color?

albinism - inability to produce melanin - decrease in tyrosinase (DOPA->melanin from dopamine pathway) - Melasma = hyperpigmentation in pregnancy - vitiligo = patches of depigmentation from autoimmune destruction of melanocytes

51 yo has diaphoresis, tachycardia, and BP 155/100 24 hours after an abdominal operation. 2 hours later = generalized tonic-clonic seizure. What happened?

alcohol withdrawal - delirium tremens = 2-4 days after last drink - classically seen in inpatient setting when they cannot drink - altered mental status, hallucinations, autonomic hyperactivity, anxiety, seizures, tremors, physchomotor agitation, insomnia, nausea - treat with benzodiaepines (chloridiazepoxide, lorazepam, diazepam - long acting) - uniquely = LOT benzos for those who drink a lot = lorazepam, oxazepam, temazepam -> have minimal first pass metabolism in liver - use in liver disease - Time from last drink: - 3-36hr: tremors, insomnia, GI upset, diaphoresis, mild agitation - 6-48 hrs: withdrawal seizures - 12-48hrs: alcoholic hallucinations (visual) - 48-96hrs: delirium tremens

Young boy with fanconi syndrome. Will his reabsorption be increased/decreased of the following: amino acids, glucose, phosphate, HCO3-

all decreased - generalized reabsorption defect in PCT

75 yo w/ stress urinary incontinence w/ sphincter incompetence. What drug can you use to treat her?

alpha 1 agonist = causes urethral sphincter and prostatic smooth muscle contraction - causes urinary retention - no real drug exists

31 yo w/ type II DM w/ oozing-foul smelling wound on foot. PE shows necrotizing wound w/ purple black discoloration over heel. Crepitant bullae producing serious drainage are present. Gram stain shows gram + rods. What virulence factor is made?

alpha toxin - clostridium perfringes - deep penetrating wounds exposed to dirt/dust - forms spores found in soil - gas gangrene in wound -> necrosis -> crepitus - alpha toxin (phospholipase/lecithinase) lyses RBC by ripping lipid bilayer - double zone of hemoylsis - obligate anaerobe - slow onset diarrhea - treat w/ penicillin

4 month old w/ 1 day hx of respiratory distress, weakness, and difficulty feeding. RR 50, he has enlarged tongue, hypotonia, and cardiomegaly. Bx of skeletal muscle shows increased glycogen. What enzyme is deficient?

alpha-1,4-glucosidase - Pompe Disease Type II - cardiomegaly, hepertrophic cardiomyopathy, hypotonia, exercise intolerance, and systemic findings leading to early death - deficient lysosomal acid alpha-1,4-glucosidase (acid maltase)

17 yo with significant blood loss after being injured. Blood loss stimulates bone marrow to make more of what?

aminolevulinate - to initiate production of more heme - glycine + succinyl Coa -> AA via aminolevulinic acid synthase (rate limiting step) - negative FB via glucose, hemin - occurs in mitochondria - decreased heme increases ALA synthase activaty - decreased RBC -> more heme production - needs B6 pyridoxine - deficiency = X-linked sideroblastic anemia

18 yo w/ ALL on chemo and low neutrophil count has lung infiltrate with PAS stain of budding fungus. How do you treat him?

amphotericin B - most likely cryptoccocus neoformans

35 yo male collapses in factory after exposure to unknown compound. He is dyspneic, noncyanotic, and smells of burnt almonds. what is the antidote?

amyl nitrite - cyanide poisoning - from combustion, or ingestion of amygdalin (in apricot seeds) - breath has bitter almond odor - CV collapse - tx: hydroxcobalmin (forms cyanocobalamin) or induce methoglobinemia w/ nitrites and sodium thiosulfates

What drug can be used to treat male-pattern baldness?

androgenic alopecia - hair thinning over vertex - block synthesis of dihydrotestosterone via finasteride (5 alpha-reductase inhibitor) decreases conversion to DHT (more potent testosterone) - use for BPH and baldness - SE: gynecomastia, sexual dysfunction - Rogaine = minoxidil: direct arteriole vasodilator for severe refractory HTN and baldness by unknown mech

67 yo w/ double vision, drooping of left eyelid, eye is fixed in a down an out position, and pupil is dilated. what caused this?

aneurysm of the posterior communicating artery - CN III sits right at the junction of the posterior communicating and posterior cerebral artery

68 yo has 1 month hx of light-headedness and tightness in chest with exertion. Pain is worse after arguing with wife and resolve w/ rest. Has hx of lower GI bleed (neg on upper and lower endoscopy/colonoscopy). temp 98.6, pulse 85, RR 15, BP 110/75, PE is normal, Hb 8.2, Hct 24%. Stool + for occult blood. ECG normal. Colonoscopy shows no abnormalities. What is cause these GI symptoms?

angiodysplasia - tortuous dilation of vessels (small vascular malformation of gut) leading to hematochezia (bright blood in stool) - usually on right side of colon - more common in older patients - confirmed by angiography - associated with aortic stenosis and von willibrand disease - he has coronary angina - generally cause of otherwise unexplained GI bleeding/anemia

37 yo w/ excision of lump on neck. 7 days later the sutured incision is erythematous and warm. No pain/drainage. What will be on hx at this time?

angiogenesis - 7 day = proliferative w/ fibroblasts, myofibroblasts, endothelial cells, keritonocytes, macrophages - laying down granulation tissue and collagen III

10 yo boy has 4 day of nosebleeds. and easy bruising. 3 wks ago = URI. PE shows ecchymoses over upper and lower extremities. Labs: normal Hb, leukocytes, and decreased plt. Bone marrow smear = increased megakaryocytes. What platelet abnormalities does he most likely have?

antibodies against glycoprotein (Gp) IIb/IIIa complex = immune thrombocytopenia - splenic macrophages eat plt - all plt disorders have increased bleeding time, mucous membrane bleeding, petechiae, epistaxis - platelet count will be normal in Glanzman (decreased GpIIb/IIIa, no plt clumping, no aggregation) and normal/decreased in Bernar-soulier (decreased GpIb, abnormal ristocetin, large plt) - key: increased megakaryocytes on BM bx and decreased plt count = ITT - prior infection - tx with steroids, IVIG, rituximab, TPO receptor agonist or splenectomy

68 yo 3 day hx of severe deep chest pain, SOB, stridor, hoarseness, difficulty swallowing, and nonproductive cough. He has long-standing hypertension and smoked 2 ppd of cigs for 45 years. temp is 99, pulse 80, RR 15, and BP 160/94, lungs are clear. PE shows pulsation above manubrium of sternum and displacement of trachea to the right. Murmur is hear in 2nd right intercostal space. What is the dx?

aortic aneurysm - males older males and trauma, connective tissue disorders, and HTN are increased risk for thoracic aneurysm -> peak at age 60-70 - less common than aortic aneurysms - tertiary syphilis, takayasu, vasculitis can also lead to TA - ascending > aortic > descending (can become abdominal) - ascending: cystic medial necrosis (breakdown of elastic fibers and accumulation of ECM in media of large blood vessels) can lead to dissection _> highest RF HTN - descending/AAA: smoking and atherosclerosis - can cause turbulent flow -> bruit - peripheral emboli may form from turbulence - thoracic: usually found incidentally -> will have pressure in chest, thoracic back pain, compression of mediastinum (wheeze, stridor, issues swallowing, and compress esophagus causing dysphagia, or SVC syndrome -> fullnes in head, facial plethora, edema of UE worse with lifting), recurrent laryngeal nerve compression = hoarseness - bulging of aortic knob in CXR and wide mediastinum (also in dissecting aneruysm) and tracheal deviation away from mass - CT confirms if it is ruptured or contained -> if ruptured (severe chest pain looks like MI and will die quickly) can treat by lowering BP - asymptomatic repair on size - symptomatic decide of risk of rupture outweighs risk of surgery

15 yo boy fell on wrist and has tenderness on palpation of radial wrist between abductor pollicis longus and extensor pollicis brevis tendons. What is he at increased risk for?

avascular necrosis of the proximal aspect of the scaphoid bone - this is the anatomical snuff box - radial artery sits in this region and feed a portion of the bone through one entry site. If a portion of the bone fully fractures, the bone will separate itself from the blood supply -> may result in a piece of avascular dead scaphoid in the wrist

29 yo in MVA is given exploratory surgery and surgeon slides his hand behind liver causing massive hemorrhage and woman's BP becomes undetectable in a minute. What lesion was associated with the hemorrhage?

avulsion of hepatic veins from the IVC - not hepatic artery or vein which are anterior (enter together on inferior surface) enter in a common ligament (porta hepatitis in the hepatoduodenal ligmanent) - pringels meneuvar: can clamp ligament and everything in ligament is shut off -> decrease blood flow in liver surgery - only structures that are behind the liver

52 yo male w/ no past hx come in w/ progressive issues swallowing, double vision, slurred speech, ptosis, and waddling gait. He has fatigable weakness. What is the mechanism of disease?

binding of autoantibodies to the acetylcholine receptor

10 yo from Democratic Republic of Congo has four 0.5mm firm nontender nodules on iliac crest/left thigh/left knee. Exam from skin snip = microfilariae. What vector caused this infection?

black fly - skin snip is done in Onchocerca volvulus - from a bite from a female black fly - skin changes, loss of elastin fibers, and river blindness (black flies, black skin nodules, black sight) - tx with ivermectin - will see eosinophilia

68 yo is not able to sustain an erection for sexual intercourse, but has no difficulty masturbating. His wife died 2 years ago and he recently began dating. What will his libido and nocturnal erections be?

both will be normal

32 yo w/ 3 months of epigastric pain that wakes him at night and relieved by eating. smokes 1 pack of cigs a day and drinks coffee. He has H. pylori and is receiving AB. what lifestyle change will decrease recurrence of symptoms?

decrease smoking

in immunocompromised patients a positive PPD was changed from 10mm -> 5mm. What effect will this have on incidence and prevalence of a positive PPD skin test?

both will increase - incidence = # new cases/those at risk - prevalence = # of existing cases/total # of people - the prevelence = incidence for disease with short duration (common cold) - the prevalence > incidence in a chronic disease (DM) as many have the disease - increased survival time: increased P, same I - increased mortality: decreased P, same I - therapy: decreased P, same I - faster recovery time: decreased P, same I - vaccine: decrease P and I - decrease risk factors: decrease both

how would someone describe the pain of diabetic neuropathy?

burning pain - maybe a tingling sensation as well

How does aspirin effect platelet function to decrease risk of thrombosis?

decreased adherence - irreversibly inhibits COX1/2 reducing platelet activation and aggregation - inhibits Thromboxane A2 that increases platelet aggregation - effects last until new platelets are produced (increase bleeding time = risk of bleeding) - decreases platelet aggregation at low dose (<300mg), antipyretic and analgesic at medium dose,a nd high dose = anti-inflammatory

55 yo w/ SOB and CKD on hemodialysis and HTN. He is confused and EKG shows low voltage w/ electrical alternans. What will his BP, pulse, jugular venous pressure, and pulsus paradoxus be?

cardiac tamponade: compression of heart by fluid (blood, effusions), decreased CO, equilibration in all four chambers -> complication of acute pericarditis that can occur from uremia in CKD - BP low - pulse: high - jugular pressure: high - increased pulsus paradoxus (decreased amplitude of systolic BP by more than 10 in inspiration) - Beck's triad: hypotension, distended neck veins, distant heart sounds

What portion of the kidneys are at risk in the use of aminoglycosides?

cause acute tubular necrosis - will see granular casts - three stages: 1. inciting event 2. maintenance phase: oliguric lasts 1-3 weeks (risk of hyperkalemia, metabolic acidosis, uremia 3. recovery phase: polyuric, BUN and creatinine fall, risk of hypokalemia and renal wasting of electrolytes and minerals - from ischemia or nephrotoxicity - from aminoglycosides, radiocontrast, lead, cisplatin, ethylene glycol, crush injury (myoglobinuria -> or working out in dehydrating heat), hemoglobinuria

What does TGF-beta do?

cell growth, differentiation, proliferation and apoptosis - some colorectal cancers have mutated TGF-beta receptors thus preventing apoptosis and allowing for overgrowth and tumor spread

12 yo swimming in stream up to his neck in 60F H2O for 20 mins. Do you expect his central blood volume, ADH, and atrial natriuretic peptide to be increased, decreased or same?

central blood volume: increased ADH: decreased ANP: increased - thought: increased pressure on body -> increase pressure felt by baroreceptors -> want to decrease BP -> release of ANP that prevents reabsorption of Na+, body no longer wants to reabsorb water -> ADH decreases and more urine output (ew), and the cold water -> vasoconstriction -> decreased peripheral blood flow, increased central blood

5 yo who was given aspirin for the flu presents with 16 hrs of severe vomiting, increased sleepiness, prolonged prothrombin time, high ammonia, transaminase, and lactate and goes into a coma. What caused this coma?

cerebral edema from Reye's syndrome - never give child aspirin except in kawasaki

10 day old comes in with 1 day hx of red eyes with discharge. Mother has no prenatal care. What infectious agent is causing this?

chlamydia trachomatis - 5-14 days postpartum - gonorrhea: 2-5 days after birth from mother's infection - HSV: days-6wks + vesicular lesions

55 yo woman never is in the sun, but her dietary vitamin D is good. She has decreased 25-hydroxycholecalciferol. Decrease production of what precursors in the skin is most likely?

cholecalciferol - sun takes 7-dehydrocholesterol -> cholecalciferol -> liver to become 25-hydroxyvitamin -> 1,25 dihydroxyvitamin D3 - diet vitamin D: ergocalciferol and cholecalciferol

vibrio cholera has what toxin?

cholera toxin: over-activates adenyl cyclase -> increases CAMP by permanently activating Gs -> increases Cl- secretion into the gut lumen -> water efflux follows -

20 yo w/ BP 140/100, urea nitrogen of 50 and UA: numerous WBC and WBC casts, with protein of 3000 (n<150). GFR is estimated at 20%. She has small asymmetric kidneys w/ scars, and blunted calyces + VUR. What does she have?

chronic pyelonephritis - coarse blunted calyx - from recurrent infection of acute pyelonephritis - thyroidization of kidney (tubules w/ eosinophilic casts) - WBC + WNBC casts = infection - not hydronephrosis -> this would show dilation of renal pelvis and calyces

a study began in the 1950s to determine the cause of lung cancer. 40,000 physicians in the UK were asked if they smoked cigs. the frequency of lung cancer was then assessed ten years later and smokers vs. non-smokers compared. what is this study design?

cohort study - compares a group w/ a given exposure of risk - tells us if exposure is related to later development of disease - can be prospective/retrospective (who will develop disease, or who did develop disease) - can measure relative risk - follow over time to see if outcome is based on exposure

after surgery, what ECM component is required to increase the tensile strength of a postoperative abdominal incision?

collagen - strengthens ECM - elastin: allows elasticity - firbonectin: anchoring protein-> connects to laminin, and blot clots (fibrinogen-> fibrin) - hyaluronan: in synovial tissue/fluid - laminin: anchoring proteins and connect cell organelles to membrane

What does IgM do?

complement activation

40 yo has chorionic villus sampling at 11 weeks. Chromosome analysis shows 46, XY in 14 cells, 47, XY+15 in additional 25 cells, and subsequent chromosome studies of amniotic fluid shows 46 XY in 50 cells. What explains this?

confined placental mosaicism - chorionic villus sampling takes cells from placenta - second test described was an amniocentesis showing all normal cells -> confirmed that this defect was only present in placental cells from a CVS - Most chromosoms in the fetus are also present in placenta as expected as both = from same zygote - 2% of pregnancies w/ chorionic villus sampling (CVS) at 9-11 wks have cytogenetic abnormality (usually trisomy) in placenta - tissue specific chromosomal mosaicism affecting placenta only - Dx of CPM = after dx of chromosomal mosaicism in a CVS, second prenatal testing (amniotic fluid culture or fetal blood culture analysis) shows a normal diploid karyotype

70 yo does a valsalva maneuver that causes a bulge of the anterior vaginal wall. What is causing this finding?

cystocele - aka anterior prolapse: when supportive tissue btw a woman's bladder + vaginal wall weakens and stretches, allowing bladder to bulge into vagina - some may have trouble starting urination, urinary incontinence, or frequent urination - recurrent UTI and urinary retention - Cystocele and prolapsed urethra often occur together and is called a cystourethrocele - Causes: childbirth, constipation, chronic cough, heavy lifting, hysterectomy, genetics, overweight

4 yo boy w/ progressive weakness, loss if muscle function. He has difficulty running, climbing stairs, and rising from a sitting position. He has hypertrophy of calf muscles, and increased creatine kinase. What is the defective protein?

cytoskeleton - Duchenne muscular dystrophy - X-linked disorder - deleted dystrophin gene: helpes anchor muscle fibers in skeletal and cardiac muscle. It connects intracellular cytoskeleton (actin) to transmembrane proteins (alpa/beta dystroglycan) that are connected to ECM - no dystrophin = myonecrosis - increased CK and aldolase - onset before 5 years - fibrofatty replacement of muscle -> pseudohypertrophy

30 yo 2 wk flank pain. BP 168/98. PE shows 7cm mass palpated on abdomen. CT scan shows mass in retroperitoneal space compressing renal artery. If perfusion pressure to affected kidney is decreased, but GFR and RPF are unchanged, what mechanism mediates autoregulation in renal arterioles?

decreased afferent arteriolar resistance - RAAS is helping her flow - angio II: more efferent than afferent constriction - and prostacyclin are made in afferent arteriole (when renal afferent flow is decreased - via stretch receptors -> activate increases renin and prostacyclin) -> more dilation of afferent and angio constricts downstream - increase hydrostatic pressure in the glomeruli -> increases GFR - slows RBF down - FF = GFR/RBF -> will increase FF in theory - NSAIDS can decrease FF via blocking the prostacyclin - hence why acetominiophen is ok to use -> acts centrally

A healthy 35 yo has fractures pelvis from MVA and is on 3 week bedrest. What physiologic change will occur?

decreased blood volume - cardiovascular deconditioned - a large part of this is blood volume - in bed = no gravity -> fluid goes away from legs -> thorax and head -> increases blood flow to kidneys -> will encourage water excretion - more stretch in heart increases ANP/BNP -> free water secretion - increased urine output decreases blood volume - more blood to carotid sinus -> decreases ADH/ALDO initially increase in urine output - increased HR - skeletal atrophy - cardiac muscle atrophy

44 yo w/ headache and papilledema. LP shows elevated opening pressure, and she is placed on acetazolamide. How does this improve the condition?

decrease CSF production by choroid plexus in this case of pseudotumor cerebri - carbonic anhydrase inhibitor - causes HCO3- diuresis and decreases total bicarb - used for glaucoma (decreases aqueous humor synthesis), metabolic alkalosis, altitude sickness, idiopathic intracranial HTN (like this ?), alkalinizes urine - SE: proximal renal tubular acidosis (type II), NH3 toxicity, sulfa allergy, hypokalemia, and can cause calcium stone formation but can prevent uric acid stone - hypokalemia as Na+ and bicarb are no longer reabsorbed causing diuresis -> NA+ has compensatory reabsorption in CD in exchange for K+ - Mannitol will also pull water out of brain -> used in urgent ICP settings -> increased extracellular volume and may cause pulm edema/hyponatremia

a 54 yo man with MI is hypotensive, what causes swelling of parenchymal cells in vital organs in minutes?

decrease in intracellular ATP concentrations - stops all ATP pumps -> increase calcium in cell, increased Na+ in cell, decreased K+ in cell - other reversible injuries: ribosomal/polysomal detachment (decreased protein synthesis) - plasma membrane blebbing - nuclear chromatin clumping - rapid loss of function (myocardial cells won't contract after 1-2 mins of ischemia) Irreversible injury: - breakdown of plasma membrane -> leak of enzymes (troponin), influx of Ca+ which will further activate degradative enzymes (think pancreatitis) - mitochondrial damage/dysfunction -> loss of ETC from decreased ATP - ruptured lysosomes -> autolysis - nuclear degradation -> pyknosis (nuclear condensation) -> karyorrhexis (nuclear fragmentation via endonucleases) -> karyolysis (nuclear dissolution)

A male newborn born at 28 weeks is tachypneic, hypoxemic. What structures are altered in his type II pneumocytes?

decrease number of lamellar bodies - surfactant maturation begins at 20 weeks and does not complete until 35 weeks - relieves collapsing pressure = 2 (surface tension)/radius - increased tendency to collapse as radius decreases - corticosteroids in birthing process important (vaginal birth = stressful vs. c-section) - measure lecithin/sphingomyelin ratio (more or = 2 is healthy, low = Neonatal respiratory distress syndrome)

18 hr male newborn weights 12lb. His mother has DM and his glucose is 20. What fetal conditions right before birth precipitate the post-natal hypoglycemia?

decreased gluconeogenesis - high states of insulin due to high maternal glucose prevents gluconeogenesis - in states of high glucose no gluconeogenesis will occur as no glucose is needed - once child is born and no more mother's glucose present, the insulin made by the child is still present and causes hypoglycemia - NOT decreased glycogen synthetase activity as this is for glycogenesis and is activated by insulin - glycogenolysis via glycogen phosphorylase negatively impacted by insulin

alcoholic w/ skin color change has been taking two extra strength acetominophen tablets every 4-6 hours for the past 3 days for headache. He has jaundice and increased PT and AST. Alteration in what metabolite in hepatocytes is causing this illness?

decreased glutathione - acetominophen metabolite NAPQI depletes glutathion and forms toxic tissue byproducts in liver - N-actetylcystein is antidote (regenerates glutathione) - paired w/ alcohol = ethanol is an inducer of Cyp2E1 cause increased production of NAPQI further depleting the glutathione stores

63 yo has operative repair of leaking berry aneurism in circle of willis. 2 days later she has widening of subarachnoid space. What changes in CSF is causing these findings?

decreased movement through the arachnoid villi - communicating hydrocephalus - from decreased absorption by arachnoid granulations (arachnoid scaring post-meningitis/surgery) - increase ICP and papilledema and herniation

45 yo undergoing surgical procedure. What maneuver would result in intracranial pressure?

decreasing respiratory rate - increases CO2 -> CO2 regulates cerebral perfusion - decreased CO2 -> vasoconstriction -> decreases cerebral blood flow -> decreases ICP (used to treat acute cerebral edema - and vice versa

22 yo hit by car while crossing street on lateral side of leg. He cannot dorsiflex his left foot. Inversion and eversion are normal. What nerve is damaged?

deep fibular (peroneal) nerve - foot drop - usually from a fracture of the neck of the fibula

33 yo w/ 2 months of easy fatigability/dark urine in the mornings. no fever or weight loss, nor travel, no meds. Temp 98.4, pulse 107, RR 20, BP 121/73. PE shows scleral icterus. Labs show: Hb 9.2, Hct 35%, total bili 3.5, direct bili 0.2, indirect 3.3, urine blood 2+, protein 1+. Direct antiglobulin (coombs) test is negative, and acidified serum test is positive. What does she have?

defect in a cell membrane anchor protein - paroxysmal nocturnal hemoglobinuria - Red blood cells in patients with PNH will hemolyze w/ acidified normal and acidified patient's serum, whereas normal RBC's will not hemolyze - now just dx via flow cytometry - increase complement-mediated intravascular RBC lysis - mutation in PIGA gene -> impaired of GPI anchoring for decay-accelerating factor (DAF/CD55) and membrane inhibitor of reactive lysis (MIRL/CD59) that protects RBC from complement - acquired mutation in hematopoietic stem cell - increased incidence of acute leukemia - associated w/ aplstic anemia - pancytopenia, venous thrombosis (budd chiari) - red/pink urine - CD55/CD59 - RBC on flow cytometry - tx: eculizumab (targets terminal complement protein C5) - NOT hereditary spherocytosis bc that is extravascular hemolysis -> no hemoglobinuria/hemosiderinuria

What is the defect in Chediak-higashi synrome?

defect in lysosomal trafficking gene (LYST), microtubule dysfunction in phagosome-lysosome fusion - AR - neurodegeneration - lymphohistiocytosis - albinism - recurrent staph/strep infections - peripheral neuropathy - giant granules in granulocytes - pancytopenia

43 yo woman with difficulty walking in past 3 months, weakness and decreased bulk in LE. Patellar and achilles tendon reflexes are diminished. Sensation of joint position, pain, temp are normal. what is causing these findings?

degeneration of motor neurons of the lumbar cord - This is a LMN injury -> everything is lowered = less muscle mass, decreased muscle tone, decreased reflexes

What are langerhan cells a derivative of?

dendritic cells -> they are the APC of the skin - contain Birbeck granules - in all layers of epidermis - most prominent in stratum spinosum - from bone marrow moncytes - langerhan histiocytosis: neoplastic proliferation of langerhan cells -> cells are CD1+ and s-100+ - presents with rashes, bone lesions in children can be fatal -> usually in young child and involves kin and bone +/- eosinophilia

Why does deoxygenated blood carry more carbon dioxide for a given Pco2 than oxygenated blood?

deoxyhemoglobin is a better buffer of hydrogen ions than oxyhemoglobin

83 yo has repair of abdominal aortic aneurysm. Graft from femoral artery is threaded through aorta and extends from below the renal arteries -> bifurcation of aorta. What organ will lose its blood supply and rely on collateral circulation?

descending colon - inferior mesenteric artery is blocked -> SMA will extend its anastomosis via marginal artery of drummond at splenic flexure - also gonadal artery is blocked but have the artery to the vas deferens/ductus deferens

25 yo in crush injury to chest w/ burns develops tachypnea and dyspnea + b/l interstitial and alveolar infiltrates. He is intubated/mechanically ventilated. Damage to what will preclude restoration of normal tissue architecture and pulmonary function?

destruction of basement membranes preclude = prevent

an obese woman begins taking orlistat. What is she at increased risk for developing?

diarrhea - inhibits gastric and pancreatic lipase -> decreased breakdown and absorption of dietary fats - used for weight loss - SE: Abdominal pain, flatulence, bowel uregency/frequent bowel movements, steatorrhea, decrease absorption of fat soluble vitamins - modest weight los of 7.5lb/year

83 yo found confused/bedridden. temp is 96, pulse 100, BP 85/50, and unchanged after 1L infusion w/ normal saline. CO is high, Pulm capillary wedge pressure is low, and SVR is low. What does he have?

early septic shock - Distributive shock (sepsis, anaphylaxis, CNS injury) will be the only kind of shock with decreased SVR as a rule - all other states of hypovolemia (bleeds, cardiogenic, obstructive) will have systemic vasoconstriction due to RAAS etc)

68 yo w/ 9 month abdominal pain after meals and 24lb weight loss (avoids eating for fear of pain). she had coronary bypass 6 years ago. On aspirin, atenolol,s simvastatin. CT abdomen shows 3cm ectatic aorta. What pairs of arteries are involved in this condition?

ectatic = more than 2 cm in diameter -> dilated - chronic mesenteric ischemia: intestinal angina - atherosclerosis of celiac artery, SMA, or IMA -> intestinal hypoperfusion -> postprandial epigastric pain -> food aversion/weight loss - watershed area in inferior mesenteric/SMA = marginal artery of drummond -> increased risk for mucosal/ischemic injury - three similar condition: acute mesenteric, chronic mesenteric, and ischemic colitis = similar - acute mesenteric = acute + severe = embolus/thombus or mesenteric veinous thrombus (afib) or are hypercoaguable or hypotensive or medications via pressor effects (cocaine) - pain out of proportion with findings need quick workup and resection of bowel or thrombectomy - chronic: pain after eating, lose weight, dont want to eat -> angioplasty with stent - ischemic colitis: LLQ pain and bloody diarrhea -> colonoscopy with ulceration "thumb print sign" - bost = most common place is at splenic flecture most sensitive to lack of O2

9 month old boy w/ respiratory distress for 1 hr and cyanosis causing death. autopsy shows ventricular septal defect and persistant truncus arteriosus. What was absent in fetal cardiac development?

ectodermal neural crest cells failed to migrate

A newborn has esophageal atresia. After surgery the tissue shows a fibrotic esophagus that is fibrotic w/ no lumen. What is the embryonic origin of the cells that fill the lumen?

endoderm - from the endodermal primitive gut tube

What is the embryological origin of pancreatic islet cells?

endodermal cells - Endoderm: (ENternal layer) - gut tube epithelium -> anal canal above dentate line) - urethra, lower vagina (urogenital sinus) - luminal epithelium: lungs, liver, gallbladder, pancreas, eustachian tube, thymus, parathyroid, parafollicular C cells

15 yo girl with CF has a mutation in the CFTR gene -> deletion of phenylalanine 508. This mutation does not prevent synthesis of the CFTR, but prevents it from folding properly. The protein will accumulate in what cellular compartment?

endoplasmic reticulum - defect in CFTR on chrom 7 - encodes an ATP-gated Cl- channel (secretes Cl- in lungs/GI and absorbs in sweat glands) - most commonly = misfolded protein retained in RER and not transported to cell membrane -> decreased Cl-/H2O secretion -> increased Cl- + Na+ absorption dehydrates mucus - presents with contraction alkalosis + hypokalemia (like loop diuretic) as extra cellular fluid loses water and salt and ALDO will try to reclaim it at expense of K+/H+ - test newborns with trysinogen - pulmonary infections s aureus in young -> pseudomonas in adults - misfolded proteins can be degraded by proteasomes via ubiquination

32 has 7 days of sneezing, nasal stuffiness, and watery eyes. She has similar symptoms each spring while gardening. What cell types will be increased in her nasal secretions?

eosinophils - not mast cells - allergies = TH2 cellular response -> IL-4,4,6,10,13 - acitivates eosinophils and promotes production of IgE - type 1 hypersensitivity - elevated in allergic processes - mast cells wouldn't be there as they are in tissues - eosinophils will proliferate in allergies, but mast cells will degranulate but not prolfierate - basophils also elevated but in serum

a 66 yo dies 7 days after an MI from an interventricular septal rupture. What is most likely on hx of the tissue?

erythrocytes, cellular debris, macrophages, and early granulation tissue - in 3-14 days = hear appears hyperemic border with central yellow-brown softening - macrophages and granulation tissues at margins - risk of free wall rupture -> temponade, papillary muscle rupture -> mitral regurge, interventricular rupture all from macrophage-mediated structural degradation. - LC pseudoaneurysm (risk of rupture)

xray of newborn shows ribs associated w/ seventh cervical vertebra. This transformation of the 7th cervical segment to a thoracic identity may have resulted from what alteration in HOX gene at the 7th cervical level?

expression of a HOX gene normally expressed only caudal to C7 - homeobox gene -> involved in segmental organization of embryo in a craniocaudal direction that codes for transcription factors that regulate transcirption - mutations = appendages in wrong places

why do elderly people have excessive wrinkling with scaly patches and diffuse bruises on forearms?

extensive solar elastosis - aka Actinic elastosis = accumulation of abnormal elastin (elastic tissue) in dermis of skin, or in conjunctiva of eye, from cumulative effects of prolonged and excessive sun exposure = photoaging from UVA - appears as thickened, dry, wrinkled skin -

In a thyroidectomy, a branch of the thyrocervical trunk is ligated. A branch of what other artery should be ligated?

external carotid - thyrocervical trunk off of the subclavian branches to provide the inferior thyroid artery - small thyroid ima artery comes off of the common carotid - superior thyroid artery comes off of the external carotid

25 yo woman overdoses on barbiturates and is placed on mechanical ventilator and has poor prognoses. She has a living will signed and consistent with directive, the husband requests mechanical ventilation be discontinued. what are the next best steps?

extubate the pt and discontinue ventilation. Make no attempt to do cardiopulmonary resuscitation in case of cardiac or respiratory failure - Do not intubate (DNI) = DNR - no code - no cardiopulmonary resuscitation

Why does a newborn has spinobifida oculta?

failure of fusion of the sclerotomes

52 yo w/ 3 wk increased thirst and urinary frequency, 10lb weight loss. He has HTN/HLD on pravastatin/metoprolol and appears ill. Hes overweight. What is increased in this patient?

fasting serum glucose concentration - He has diabetes melitis

3 yo boy w/ 1 week of cough, wheezing, nausea, coarse b/l crackles on chest. Stool: 1.6cm roundworm larva w/ b/l infiltrates on chest x-ray. What was ingested by this child?

feces-contaminated soil - Ascaris lumbricoides - migrates to lung (from intestine -> lymph -> lungs)

In a hypertensive emergency, a man has BUN of 70, and creatinine of 7.0. 24 hr shows 300mg protein in urine. What is the pathologic finding in the kidneys?

fibromuscular hyperplasia of arterioles - in malignant HTN -> arteriolosclerosis via hyperplastic arteriolosclerosis = thickening of vessel wall by hyperplasia of smooth muscle (onion skin appearance) which decrease BF in glomerulus as afferent arteriole scars - leads to end-organ ischemia or fibrinoid necrosis of vessel wall w/ hemorrhage ->AKI (flea bitten appearance from little hemorrhages) - vs. hayline arteriolosclerosis: proteins leaking into vessel wall -> vascular thickening showing pink hyaline on H and E - from long-standing HTN or DM (non-enzymatic glycosylation) -> glomerular scaring -> chronic renal failure

What does fish oil do?

fish oil/omega 3 fatty acids antagonize VLDL-cholesterol secretion - increase LDL/HDL slightly - decrease FFA delivery to liver

How can you test a nerve injury to the flexor digitorum profundus?

flexion at the proximal interphalangeal joint - the flexor digitorum profundus acting on the proximal and distal joints, and the flexor digitorum superficialis acting on the proximal joints

43 yo male w/ 6 week hx of sharp, stabbing pain on left side of face that hurts when he touches it. Touching face lateral to the left nasal ala reproduces pain. the nerve supplying this area of the face exits the skull through which foramina?

formen rotundum

25 yo after MVA has low BP from ruptured spleen has surgery -> episodes of hypotension. in next 24 hrs he has azotemia, and is fully colume restored, but urine remains less than 20ml/h. What will his UA show?

fractional excretion of sodium greater than 2% - intrinsic renal azotemia -> decreased RBF = decreased GFR - causes acute tubular necrosis as repleted water and he still has decreased urine output -> no improvement of symptoms with hydration (prerenal azotemia will improve with repletion of water) - from prolonged ischemia - urea absorption is impaired as tubules are no longer functioning -> decreased BUN (<15) - kidney can no longer concentrate urine = <350 osm - no longer reabsorbing sodium (>40 excreted) making the FENa >2%

What is a cross sectional study?

frequency of disease and frequency of risk-related factors assessed in the present -> snapshot in time - can show risk factor association w/ disease but not establish causality ex) ambient noise and hearing loss in manufacturing plant (take samples from diff locations in factory and measure hearing loss + how loud the noise is)

25 yo has an ovarian mass that is determined to be a teratoma. What is the source of this neoplasm?

germ cells - most common ovarian tumor in females (10-30 yo) - contains elements from all three germ layers - pain from ovarian enlargement/torsion - can have a monodermal form of thyroid tissue (struma ovarii) -> hyperthyroidism - rare transformation to squamous CC

23 yo female in DKA given insulin w/ improvement. the activity of which enzyme has increased in hepatocytes?

glucokinase - this catalyzes glucose -> glucose-6-phosphate - this is a kinase as it phosphorylates glucose - glucokinase in liver/ B cells of pancreas - hexokinase in other tissues - glucokinase has a lower affinity for glucose uptake (liver only stores glucose in high glucose states) whereas hexokinase has a higher affinity and will take up glucose in low states - hexokinase: has no effect from insulin, but has NFB from G6P - glucokinase: controlled by insulin but has no NFB from G6P

a 3 month old has URI and ate poorly for 3 days. Temp is 98.6, RR 30, PE mild hepatomegaly, labs show hypoglycemia, lactic acedemia, ketonemia, and metabolic acidosis. Following IV glycerol + fructose on different days, his glucose levels do not increase. They do increase w/ galactose. A defect in what liver metabolic pathways is causing this?

gluconeogenesis - gluconeogenic pathway will take fructose and glycerol -> glucose - vs. galactose that can go straight to -> glucose - glucose 6 phostphatase mutation = Von Gierke's disease - this effects the ability to release glucose from glycogen - cannot take G6p -> glucose - he is having a fasting hypoglycemia as he cant release glucose from glycogen or from other sources

boy with diarrhea appears dehydrated. oral rehydration therapy will have what ingredients in it?

glucose and sodium - activate the sodium/glu intestinal absorption to reabsorb electrolytes and water

What molecule is required for the synthesis of glucosamine from fructose 6-phosphate?

glutamine - glucosamine = glycosylated protein in polysaccharides (N-acetylclucosamine -> GAG makes up ECM/ground substance in bone + type 1 collagen + articular cartilage) - glucose with an amino group - takes amino group from glutamine -> glumate - only arginine and glutamate can donate amines - glutamine shuttles amino acids often and gives them up more than arginine

6 yo girl w/ renal tubular acidosis on potassium citrate. They suspect defect in renal ammoniagenesis. What substrate is the source of the ammonia production?

glutamine - glutamine -> glutamate -> alpha ketoglutarate - produces NH3 to act as a buffer in urine to capture H+

33 yo with muscle weakness, cramping beginning after exercise. He has high serum creatine kinase. After exercising forearm, vein sample shows lactate concentrations do not increase. What deficiency does he have?

glycogen phosphorylase - McArdle Disease = muscle disease - increased glycogen in muscle cannot break it down = painful muscle cramps - blood glucose levels unaffected

healthy 22 yo w/ normal glucose levels eats 800 calorie meal of protein, fat, carbs. He then sleep through the night and fasts. 12 hrs later, his serum glucose concentrations are in reference range. What mechanism maintains the serum glucose concentration?

glycogenolysis in the liver - post-absorptive phase (6-24 hours after a meal) dominated by glycogenolysis - Gluconeogenesis from 24 hours to 2 days - Then ketosis - glycogenolysis rate limiting enzyme = glycogen phosphorylase -> g-6-p and needs glucose-6-phosphatase to release glucose (defect = Van Gierke disease) - increased by epinephrine, glucagon, AMP - inhibited by g-6-p, insulin, ATP (no need to break down glycogen)

52 yo has massive heart attack and is treated with O2, B-blocker, aspirin, and reperfusion therapy. 18 days later he gets vfib and dies. A bx from site of infarction shows round bright areas of bloody appearing tissue speckled with pink collage, and macrophage. What kind of tissue is this most likely?

granulation tissue - 3-14 days after MI tissue will have yellow-brown softening - macrophages and granulation tissue at margins as scar begins to heal permanent cells - NOT coagulative necrosis as this is an early event of MI - 1-3 days = coagulative necrosis and acute inflammation with neutrophils

10 yo w/ painful 2mm nodules on her chin after sutures. on hx you see giant cells. What is this?

granuloma - pattern of chronic inflammation to foreign body - t-cell response - wall off foreign body without degrading it - giant cell from fusion of activated macrophages - secretion of IL-12 -> differentiate CD4+ into TH1 cells - TH1 secretes INF-gamma -> macrophage activation -> increase cytokines (TNF) -> forms giant cells

52 yo w/ stable angina treated w/ medication that decreases CO and increases coronary artery blood flow. What SE will he have?

headache - Nitrate: venodilation and dilate coronary arteries as well - vasodilation of cerebral vessels -> headache - also cause hypertension - nitroprusside -> cyanide as a product - nitrates -> degraded to nitrites -> myethoglobinemia (Fe2+ -> fe3+ doesn't bind O2 as effectively) - esophageal spasms also respond to this - isosorbide - long acting - nitroglycerin - short acting

31 yo with pregnancy of 69, XXY, HTN, US showing vesicular structures but no embryo. What will indicate that an embryo has been formed in this conceptus?

hematopoietic stem cells - Partial moles has 1 egg + 2 sperms -> will have stem cells and partial fetus parts - only some villi are hydropic, w/ focal/minimal trophoblastic proliferation - will express P57 (maternally expressed marked) as there is maternal genetic material here - will have normal increase in uterine size - low risk for invasive mole and choriocarcinoma - both moles are made of primary stem villi and trophoblastic lacunae

3 yo w/ pale skin, European decent, no personal or fam hx of illness. Has low Hb, hct, and normal mean corpuscular volume with spherocytes on smear. What are his genetics going to show?

heterozygous mutation in ankyrin gene - normocytic hemolytic (intrinsic - due to damaged RBC) anemia - extravascular hemolysis from splenic macrophages - defect in proteins interacting w/ RBC membrane and plasma membrane (ankyrin, band 3, protein 4.2, spectrin) - AUTOSOMAL DOMINANT - increased fragility in osmotic fragility test - tx: splenectomy - spontaneous somatic mutation in structural defect

2 week old newborn with patent ductus arteriosus has what cardiovascular findings?

higher than normal left ventricular output - in fetal period shunt is right -> left - high pressure from aorta will reverse the flow of blood (left->right shunt) in this connection once baby is born and pulm has lower pressure - blood will flow back from aorta high pressure -> pulm artery -> pulm vein -> LA -> LV giving a newborn a higher CO - may lead to RV or LV hypertrophy and heart failure - continuous machine-like murmur best at left infraclavicular area - from congenital rubella/prematurity - patency maintained by PGE and low O2 tension (can maintain life in transposition of the great vessels) - late PDA = cyanosis in lower extremities - indomethacin ends patency

A man accidentally touch a stove and burns himself -> blister formation 20 mins later. At this time the fluid will show presence of what mediator?

histamine - following insult (burn, cut, irritation) -> redness, warmth, swelling, pain - vasodilation -> increases blood flow (mediated by histamine, pG, Bradykinin, NO) - swelling: endothelial contraction from tissue damage increases vascular permeability -> leakage of protein-rich fluid from postcapillary venules -> interstitial space (exudate) -> increases interstitial oncotic pressure (from leukotrienes, histamine, serotonin) - Pain: sensitization of nerve endings (bradykinin, PGE2, histmaine) - histamine in all three

36 yo has AML w/ translocation of 15 and 17. Once treated with all-trans retinoic acid, the fusion protein will most likely attract what proteins to form a pre-transcriptional complex?

histone acetylase - induce differentiation of promyelocytes - acetylation removes histones + charge and allows DNA to relax and increase transcription - general TF (bind at promotor) needed for RNA polymerase to bind TATA for transcription - specific TF (all steroid hormones vita DEAK): enhancers or silencers - cis regulatory elements at any location from gene - enhancer = activator - silencer binding = supressor - co- activators HATs: histosone acetyltransferase -> at terminal lysine residue (terminal amino group that is + charged -> becomes neutral and relaxes/losens DNA for transcription = chromatin remodeling (heterochrom (hard to transcribe) ->euchrom (easy to transcribe) - repressor binding silencer: Histone deacetylases -> promotes euchrom -> heterochrom - this here is progrowth - APL has less binding affinity to ATRA -> but can give high doses to saturate receptor to create force maturation of this prolmyelocytic line - large amount may exceed bone marrow -> rupture of cell lines, and release of auer rods into circulation (made of myeloperoxidase) causes DIC which is the primary concern of AML

31 pregnant woman w/ malaria. temp, low BP, RR 24. What complications is she at risk for?

hypoglycemia - a frequently encountered complication in falciparum malaria due to increased glucose use and impaired glucose production caused by inhibition of gluconeogenesis - malaria also effects liver -> hypoglycemia - can also cause ARD, renal failure - seizures and strokes from cerbral malaria - kids more than adults - hypoglycemia

5 yo has genetic defect preventing reabsorption in PCT. What are his labs going to show?

hypophosphatemia - phos filtred and reabsorbed in PCT -> PTH inhibits Na+/Phos co-transport to prevent re-uptake of phos in PCT

What causes babies to have spit-up?

immature lower esophageal sphincter -> reflux of normal formula and air that is simultaneously swallowed - want to keep baby upright, and burp them

5 yo just recovered from URI and has easy bruising, petechiae on lower extremeties and labs shows Hb 12, Hct 36%, leukocytes 8000, plt 18,000. RBC are normochromic and normocytic. What is the cause of thombocytopenia?

immune destruction of platelets - may be idiopathic or from autoimmune reasons, or viral infections - will see increased megakaryocytes on BM bx (shown in stem), and decreased plt - not henoch-scholein as there is no hematuria, no GI upset, and RBC are normocytic (no schistocytes)

Alcoholic pt is seen with a wound from 6 weeks ago that has dehisced (re-opened). She has few teeth, and hemorrhagic macule around her hair follicles. Why does she have poor wound healing?

inadequate hydroxylation of collage polypeptides - preprocollagen (gly-proline-lysine) -> hydroxylation of the pro/lys residues to aid in later cross-linking -> glycosylation -> procollagen via hydrogen/disulfide bonds -> extracellular space -cleave disulfide rich procollagen -> tropocollagen -> covalent bonds of hydroxylated lysine -> collagen fibrils - this step in collagen synthesis requires vitamin C - vitamin C deficiency = scurvy

screening program for chlamydia in college 1st yr students. initial screening -> 500 of 2500 students had it. One year later and additional 200 students had it. What is the annual incidence of this infection?

incidence: # of new cases/# of people at risk (New cases) Prevalence: # of existing cases/total population (all current cases) it increases PPV and deceases NPV - 2500-500 = 2000 still at risk - 200/2000 = 10%

What does demyelinating an axon do to an action potential?

increase axonal capacitance - myelin speeds transmission of electrical impulses along axons by insulating axon and reducing axonal membrane capacitance - conductance: increased area (larger axons) transmit signal faster than small ones (there is less resistance) - capacitance = number of Na+ ions that can be stored in cell membrane - at myelinated membrane, the capacitance (or the capacity to store charges) is decreased, while it is increased at the nodes or ranvier - takes more time to depolarize nodes of ranvier (slow) than at the areas of myelin (fast) as there are less charges that must move to change membrane potential and carry signal down axon - less ions flow in to cell, and less Na+ need to be pumped out of cell via Na/K+ pump to repolarize myelinated cells - but AP recharges at nodes of ranvier as they lose their size a little in myelin

24 yo receives injection of histamine in brachial artery. What changes occur in his forearm?

increased lymphatic flow - not decreased oncotic flow as this causes vasodilation and filtration of both fluid and proteins maintaining oncotic pressure -> exudate - histamine increases permeability -> increase filtration -> increases hydrostatic pressure - protein and fluid is washed out together in lymph - transudative would decrease oncotic pressure - lymph fluid will increase as fluid goes up in interstitial space - anything that increases edema -> increases lymph flow

what allows increased maximal expiratory flow rate in someone with interstitial pulmonary fibrosis?

increased radial traction on airways - Small bronchi and bronchioles depend on radial stretching by elastic lung parenhcyma to maintain their patency - elastic recoil (radial traction) of the lungs increases as lung is expanded - lower lung volumes, reduced radial traction = narrowing of smaller airways. - excessive resistance to airflow from disease = breathe at large lung volumes to enhance radial traction - in restrictive disease = increased radial traction from fibrotic connective tissue

treatment with propranolol causes an increase/decrease in peripheral resistance/CO?

increased total peripheral resistance and decreased cardiac output - Non-selective B antagonist - B1 blockade -> decreased HR\, decreased contractility, decreased renin release, decrease lipolysis - B2 blockade -> blocks vasodilation (causing and increase in SVR), bronchoconstriction (caution in those with asthma), decreases glycogenolysis (hypoglycemia), increased uterine tone, decreased K+ cell uptake

When started on lovastatin, what adaptive/compensatory response will happen at a cellular level?

increased transcription of HMG-CoA reductase

What causes numerous neutrophils and crystals in a joint to have severe swelling?

increased vascular permeability - Gout: acute inflammatory monoarthritis - from underexcretion (most pt): idiopathic, renal failure, thiazide diuretics - overproduction (10%): Lesch-Nyhan, PRPP, increased cell turnover (TLS), von gierkes disease - uptake of urate crystals by neutrophils -> ROS -> cytokines -> inflammation and vasodilation - hence why you treat with NSAID (anti-inflammatory) or glucocorticoids (inhibit phospholipase A2 = no conversion of membrane phospholipids -> arachidonic acid)

20 yo has had 12 hrs of contractions, temp is 102, and pulse is 115. PE shows tenderness of uterus, closed cervix. Fetal heart rate is 210. What is the primary stimulus for uterine muscle contractions?

inflamed maternal decidua release of prostaglandin - similar to misoprostol a PGE1 analog used to induce labor by ripening cervix/facilitate labor - can also be used as an abortifacient - Stretch reflex in uterus -> oxytocin from posterior pituitary -> Gq comped receptor -> uterine contractions and this is self sustaining -> places more pressure on cervix/vaginal wall -> more oxytocin (from periventricular/supraoptic nucleus) - progesterin downregulates sensitivity to oxytocin - estrogen up-regulates sensistivity - rupture of membrane (premature) -> increases myometrial contractility - ruptures amniotic fluid interacts with decidua -> causes inflammation/irritation and this releases cytokines -> PGE2 (contracts) in pre-term rupture -> triggers normal oxytocin reflex - Preventing labor: "tocolytics" - relax uterine smooth muscle: B2 agonist (Gs on smooth muscle) terbutaline (24-48hr delay), retotradine or calcium channel blockers (nifedipine, up to 2-7 days), atosabin (oxytocin receptor antagonist), magnesium sulfate (in pre-eclampsia and reduces uterine contractility -> myosin light chain inhibitor) helps to give steroids in 24-48 days to babies - inducing labor: - misoprostol (PGE1 = uterine SM constrictor and contraction of cervix -> effacement and thinning and ripening of cervix) - deneprostone: PGE2 same idea -> keeps a PDA opening as well (given vaginally and no effect on PDA in child)

14 yo w/ daily headache for 2 months, bilateral aching temples, not acting himself, and is confused and forgetting names, dates, places and is clumsy with falls. School performance declined. He has broad based gait, alert and oriented to PPT, slow to answer questions. Chronic abuse of what substance is causing this?

inhaled glue

Zanamivir

inhibits influenza neuraminidase - neuraminidase cleave progeny virion from cell allowing release - prevents both flu A and B and may shorten duration of illness

A 18 yo champion wrestler/football player is concerned about enlarged breast tissue and is embarrassed. He is not obese, and has sexual development. What is the next best step in treatment?

inquiry about anabolic steroid use - inhibits hypothalamic-pituitary gonadal axis -> decreased intra-testicular testosterone -> decrease testicular size/sperm count

57 yo w/ breast cancer in upper inner quadrant. What vein would provide the most direct pathway for malignant cells to travel to the vertebral column?

intercostal - drainage of breast: axillary, lateral thoracic (outer quadrants), and internal thoracic and intercostal veins medially - Connections btw intercostal veins and vertebral plexus allow metastasis to bones and to nervous system

Where does the cervix lymph drain aka where would malignant cancer cells drain first in cervical squamous cancer?

internal iliac nodes

What are the ling findings in someone with scleroderma?

interstitial fibrosis, pulmonary HTN

18 yo w/ fever, malaise, blood in urine since oral AB tx for UTI + 3 day rash. temp is 100, UA: Blood 3+, protein 1+, leukocytes 150/hpf, eosinophils 30%. What does she have?

interstitial nephritis - aka tubulointerstitial nephritis - acute interstitial inflammation - w/ pyuria (Urine w/ WBC/pus) primarily of eosinophils and azotemia after given drugs that act as haptens -> hypersensitivity - usually drug related or from systemic disease (SLE, sjogren, sarcoidosis, mycoplasma infection) - from drugs (P) - Pee (diuretics), Pain-free (NSAID), Penicillin and cephalosporines, PPI, rifamPin

The breakdown of dipeptides and tripeptides -> free amino acids takes place primarily in which of the following area in the GI tract?

intestinal mucosa - small intestine mucosa can only transport single AA or short polymers of two to three amino acids (dipeptides/ tripeptides) - large proteins enzymatically digested prior to absorption - stomach secretes pepsinogen -> converted to the active protease pepsin by the action of H+ - pancreas secretes proteases: trypsin (activated by eneterokinase/enteropeptidase), chymotrypsin, carboxypeptidases -> break down proteins for absorption in small intestines -brush border of small intestine has membrane-bound peptidases for complete digestion of oligopeptides -> single amino acids or dipeptides and tripeptides

11 yo with DKA is unconscious. Why?

intracellular and extracellular dehydration - severe osmotic diuresis from increased glucose - potassium levels will be high as you measure serum levels (extracellular shift out of cells with no insulin) - intracellular levels will be low and total potassium will be decreased as you are also excreting it in urine

pregnant 28 yo has enlargement of the pituitary gland on MRI. What cells accounts for this enlargement?

lactotrope - The normal pituitary gland enlarges gradually during pregnancy because of estrogen-stimulated hyperplasia and hypertrophy of prolactin cells

What is the fibrous protein that forms the two dimensional network on the inner surface of the nuclear membrane?

lamins - a kind of intermediate filament that is part of the cytoskeletal elements that maintain and support cell structure - other IF: vimentin, desmin, cytokeratin, glial fibrillary acidic protein (GFAP), neurofilaments - hold together shape, helps in mitosis, and apoptosis (allow for DNA condensation -> targeted by caspases)

indirect inguinal hernias are located where?

lateral to the inferior epigastric and superior to the inguinal ligament (as they travel through the inguinal canal that is above the ligament)

75 yo w/ enlarging lesion (1.5cm) brown-black mottled, scaly lesion w/ irregular borders. Hx: atypical melanocytes spread along basilar layer of epidermis. What caused these findings?

lentigo maligna - melanoma in situ w/ no invasive growth - progresses slowly, non-invasive form for years - transition to melanoma marked by bumpy surface (vertical growth/ invasion) = lentigo maligna melanoma - normally in elderly on face/ forearms - thought of as melanoma precursor - Incidence of evolution to lentigo maligna melanoma is low in elderly pt

Previously healthy woman has 6 hour history of fever/shaking chills. 4 hours ago she took four 325 mg aspirin tablets for fever. Her temp is 103, pulse 96, RR 18, BP 102/60. PE shows marked tenderness b/l in costovertebral areas. Labs: Hb 13, Hct 39%, Leukocytes count 32,000, urine ph 6, nitrites 4+, leukocytes esterase 4+. Blood smear shows leukocytosis. What is causing this?

leukemoid reaction - This woman has acute pyelonephritis (CV tenderness and fevers and chills) - leukomoid rxn occurs in response to infections, medications, severe hemorrhage - differentiate from CML via high leukocyte alkaline phosphatase (LAP = high activity of neutrophils, in malignancy = many non-functioning cells) - labs: +leukocyte esterase, + nitrites -> indicates gram - organism, sterile pyuria (pus/WBC in urine) (pyuria with negative urine cultures = neisseria gonnorhea/chlamydia) - NOT an allergic rxn to aspirin = can induce asthma (COX inhibition), chronic sinusitis, nasal polyps - aspirin can also = AKI (would see muddy brown casts), and interstitial nephritis but interstitial neph would have = fever, rash, hematuria, pyuria, and costovertebral angle tenderness and can be asymptomatic, but wouldn't have nitrites or leukocyte esterase - reaction to acute infection with left shift and increase WBC -> most will be immature neutrophils (vc. CML = non- leukomoid as there will be more mature cells than leukemoid rxn)

young girl with SOB, hx of similar episodes, with diffuse wheezes, sputum shows many eosinophils, and charcot leyden cyrstals. What cytokines are involved?

leukotriene B4, C4, D4 - Asthma

57 yo dies of klebsiella pneumoniae and has abscess cavities w/ purulent exudate. What kind of necrosis is this?

liquefactive necrosis - bacterial abscesses, lung abscess/cavitations, brain infarctions - neutrophils release lysosomal enzymes that digest tissue - early: cellular debris/ macrophages - late: cystic spaces and cavitation - neutrophils and cell debris found in bacterial infection

21 yo male in ER from stab wound has repair of small intestine, and puncture wound on liver. 6 months later he has adhesions and in surgery they see complete healing without scaring. What tissue is this?

liver - - NOT the small intestine: bowel causes bands of scar tissue aka adhesions after being handled in operations - small bowel obstructions usually caused by scar tissue, hernia, or cancer - The more surgeries that involve the bowel, the more scars are likely to form

35 yo w/ chagas disease comes to the ER with 2 hr history of chest pain, 4 lb weight loss. Pulse 100, RR 25, and BP 135/90. ECG normal, and barium swallow showed esophagus with beak-like narrowing at level of the LES. A bx will show what?

loss of neurons in the myenteric plexus - He has achalasia due to Chagas disease (mega-esophagus) - Failure of LES to relax due to loss of myenteric (aurbach) plexus from loss of postganglionic inhibitory neurons = NO and VIP that usually allow for relaxation, and coordinated esophageal peristalsis - uninhibited Ach and substance P = excitatory and cause contractions - manometry: uncoordinated/absent peristalsis with high LES resting pressure - progressive dysphagia from solids -> liquids - barium shows bird beak appearance - increase risk of esophageal cancer - Achalasia = means absence of relaxation

What property about local anesthetics explains why it may have a rapid onset of action?

low blood solubility - nitrous oxide (N2O) has low blood solubility, and thus fast induction and low potency (a HIGH MAC -> more gas is needed to induce anesthesia in 50% of pt)

33 yo falls on outstretched hand. Swelling/pain central of palmar side of right wrist, palpation in anatomical snuff box -> no pain. What bone is injrued?

lunate - from scaphoid in clockwise direction: So Long Pinky, Here, Comes The Thumb

35 yo w/ asthma treated w/ corticosteroids dies of complications of histoplasmosis. Exam shows lungs are 1.5X normal weight w/ focal areas of fibrosis a 2-5mm nodules. Examination of nodules will show what cells infiltrating?

lymphocytes and monocytes - systemic mycoses can cause pneumonia and may disseminate - from dimorphic fungi (mold in the cold, yeast in the heat except cocidioides = spherule) - Can form granulomas (via CD4+ activation of macrophages) - cannot be transmitted from person to person - Tx local infection w/ flucanzole/itraconazole - amphotericin B for spread - Histo infect macrophages and presents w/ tongue ulcers, splenomegaly, pancytopenia - from bird/bat droppings in mississippi/ohia river valleys - dx via urine/serum AG

Someone diagnosed w/ pancreatic cancer is most at risk for developing what?

major depressive disorder

what part of bone is removed in a laminectomy?

make cut at the lamina in between transverse process and spinous process - remove just the top half with spinous process to relieve pressure

60 yo w/ involuntary rhythmic jerking of right leg and foot and increased ankle jerk reflex. Babinski sign present on right. MRI shows 2cm round enhancing lesion in interhemispheric fissure in region of central sulcus what is the diangosis?

meningioma - common, typically benign w/ females > males - occurs near surfaces of brain and parasigittal region and extra-axial (external to brain parenchyma) and may have dural attachment (dural tail) - usually asymptomatic, or w/ seizure/focal neurologic signs - arachnoid cell origin, spindle cells in whorled pattern w. psammoma bodies - question described the mass being in the dura of the brain and not in the parenchyma

48 yo has back pain for 2 weeks. No smoking, drugs, alcohol. Serologic studies and UA are normal. X-ray of spine shows two lytic lesions in T10/L1. What is the most likely diagnosis?

metastatic carcinoma of the breast - prostate, breast > kidney, thyroid, lung - lytic: thyroid, kidney, non-small cell lung cancer - blastic: prostate, small cell lung cancer - mixed = breast - bone metastasis >> than primary tumor (multiple myeloma) - NOT thyroid: no hypocalcemia, no PE findings of nodules (follicular carcinoma usually metastasizes) - lung is most common site in thyroid and doesn't metastasize often at all - breast mostly goes to bone and brain and commonly metastasizes

Man has an UMN stroke and CT initially shows no abnormalities, but a week later shows a hypo-dense region -> loss of tissue. What brain cell clears necrotic tissue?

microglial cells

85 yo w/ fracture of femur and is on morphine w/ patient controlled analgesic pump. Three days later her RR 6, PE shows pinpoint pupil, creatinine is 1.8. What about morphine is causing this?

morphine is metabolized to active metabolites that accumulate - kidneys are dysfunctional -> not excreting an active metabolite of morphine - liver goes through phase 2 -> glucoronydation to morphine 6 which is slightly active are not excreted - this leads to accumulation of morphine 6 -> opioid overdose - patient controlled pumps = could not have overdosed herself -> limits the amount of drug available to pt

a healthy 3 yo boy has a cleft lip and palate w/ no other birth defects. What is the mode of inheritance that best explains these abnormalities?

multifactorial - but usually occur together -

57 yo woman has dullness to percussion on right, and large pleural effusion. Thoracentesis planned. She is in seated position, what intercostal space will you place the needle in?

ninth - need to do it pretty low

pt wants to commit suicide if her terminal pancreatic pain becomes unbearable. The physician refuses, but tells the pt he will do everything to manage her pain. What ethical principal?

nonmaleficence - "do no harm" - must be balanced against beneficence - if benefits outweigh risk a pt can make an informed decision to proceed (most surgeries and medications)

What kind of receptor does T3/T4 bind?

nuclear/retinoid X binding to DNA

52 yo woman begins tx w/ cisplatin for non-small cell carcinoma of lung. What other drug do you want to prescribe?

ondansetron - 5-HT3 antagonist -> decreases vagal stimulation -> powerful central-acting antiemetic - controls vomiting postoperatively and in pt w/ chemo - SE: headache, constipation, QT interval prolongation, serotonin syndrome - also can give amifostine (free amethyst) to prevent renal toxicity -> scavenges free radicals made by cisplatin in kidney

1 week old with poor feeding, vomiting, and lethargy. Born at term, she has healthy brother and sister who died at 10 days. She has decreased muscle tone and poor responsiveness. Bicarb is 8, pH is 7.15, and plasma ammonia is 10X upper limit. What is the cause?

organic acid metabolism disorder - organic acids (thymidine, uracil, valine, isoleucine, methionine, threonine, cholesterol, odd-chain fatty acids -> propionyl-CoA (proprionyl CoA carboxylase that needs biotin) -> methylmalonyl-CoA (methymaloynl CoA isomerase/mutase that uses B12) -> succinyl-CoA -> TCA cycle - can have propionic acidemia is propionyl CoA carboxylase is blocked, or methylmalonic acidemia is methylmalonyl coa isomerase/mutase is blocked or B12 deff

gram stain shows pink rods and blue cocci. What is a characteristic of just the pink rods?

outer membrane - in gram negative only - contains endotoxins (Lipopolysaccharide -LPS/LOS) -> Lipid A induces TNF and IL-1 - contains porins and outer membrane proteins that are antigenic - also only gram neg has periplasmic space (between outer membrane and cytoplasmic membrane that houses B-lactamase that inactivates AB) - Gram Positive only have lipoteichoic acid (drives immune response -> AG)

1/2 hour after delivery, 30 yo woman has heavy blood loss from vagina. pulse is 120 and BP is 90/60. uterus is soft and contractions stopped. What medicine will help>

oxytocin - promotes uterine contractions - control post-partum hemorrhage

50 yo overdosed on a drug w/ sodium salt with CNS effects. Alkalinization of urine will cause the greatest absolute increase of ellmination is the drug has what pKa, protein binding plasma (%), and Vd?

pKa: 6.0 (acidic) Protein binding in plasma: 20% (low) Vd: 1L/Kg (low) - making urine basic -> want a weak acid to have ion trapping in urine, and if it is not bound to plasma protein it will be freely filtered and trapped in urine - also the Vd is saying that the medication is confined to the plasma as it is only occupying a small space. This implies the drug will be filtered by the kidney and not trapped in the tissue (high Vd)

35 yo w/ chronic atrophic gastritis, pale, dizzy, palpable liver edge, no occult blood in stool, decreased vibratory sensation, 27% Hct, MCV of 112, normal plt. Absence of which cells is causing this?

parietal cells - not producing intrinsic factor (complexes with Vita B12 to be absorbed in terminal ileum - autoantibodies to parietal cells in body/fundus

6 yo girl has severely itchy scalp similar to other kids in her class. Exam shows hair shafts with 1-2mm white globular protuberances. What is this?

pediculus humanus capitis - aka lice aka phthirus pubis (pubic lice) - blood sucking lice that cause pruritus in scalp, neck and waistband/axilla (body lice), pubic and perianal (pubic lice) - body lice can transmit Rickettsia prowazekii (epidemic typhus), Borrelia recurrentis (relapsing fever), and Bartonella quintana (trench fever) - tx: pyrethroids, malathion or ivermectin lotion and nit combing

2 yo boy w/ chronic bacterial infections. normal PE, and T and B lymphocytes are normal. Natural killer cells are normal. He has absent MHC I and dx is bare lymphocyte syndrome type I. What protein is mutated?

peptide transporter (TAP) - MHC I include: HLA-A,B, and C - they bind TCR and CD8+ t-cells - has 1 long and 1 short chain - is expressed on all nucleated cells, APC, platelets, except RBC - function is to present endogenous AG (viral or cytosolic proteins) to CD8+ cytotoxic T-cells - loaded AG in RER after delivery via TAP (transporter associated with AG processing) aka peptide transporter - associated with B2-microglobulin protein

26 yo in MVA has BP 90/60, systolic BP decreases 20 mmhg in inspiration, PE shows jugular veinous distention and there are distant heart sounds. What does he have?

pericardial tamponade - Becks triad: distant heart sounds, distended neck veins, hypotension

3 month old baby w/ yellow eyes and skin and weakness since birth. PE shows jaundice, large fontanels, flat midfacial area, hypotonia, and hepatomegaly. labs: increased VLCFA. Liver bx shows foamy, lipid-filled hepatocytes, necrosis, and absence of a specific organelle. What organelle is this?

peroxisomes - Zellweger syndrome = peroxisomal disorders, early demise - very long chain fatty acids and branched-chain fatty acids (phytanic acid) = cant undergo mitochondrial beta-oxidation - instead undergo special form of beta-oxidation in peroxisomes and alpa oxidation unique to peroxisomes - neurological defects from improper neuronal migration/myelination - X-linked adrenoleukodystrophy = defective transport of VLCFA into peroxisomes - peroxisomes also make cholesterol, bile acids, plasmalogens (for white matter of brain)

2 month old boy w/ hypotonia, poor feeding, large fontanels, midface hypoplasia, hepatomegaly, cryptorchidism. Serum = high very-long-chain fatty acids, phytanic acid, and pipecolic acid. What organelle is absent in his hepatocytes?

peroxisomes - organelle responsible for beta-oxidization of very-long-chain fatty acids - alpha-oxidation - break down of branched chain FA, AA, ethanol - synthesis of cholesterol, bile acids Refsum disease: - AR disorder fo alpha oxidation - phytanic acid not metabolized to pristanic acid - scaly skin, ataxia, cataracts, night blindness, shortening of 4th toe

55 yo w/ DM type I has burning pain in his feet for 4 months b/l. Sensation to pinprick is decreased. What is causing this pain?

persistent activation of voltage-gated Na+ channels in nociceptor - peripheral neuropathy - glucose (aldose reductase) -> sorbitol and usually is -> fructose by sorbitol dehydrogenase - some tissues don't have sorbitol dehydrogenase -> accumulation of sorbitol -> causes osmotic damage - occurs in retina, kidney, schwann cells - also caused by microvascular damage -> narrowing -> nerve ischemia - inappropriate activation of neurons in stem, and pain is the inappropriate activation - had to be increased firing of nociceptor

1 yo boy unvaccinated has meningitis and cultures from nasopharynx and CSF show gram-neg coccobacilli. EM shows pili on culture from nose, but no pili from CSF. What shuts off expression of pili in this bacteria?

phase variation - h flu -> infantile meningitis in those that are unvaccinated (gram neg coccobacilli) - phase variation = dealing w/ rapidly varying environments without requiring random mutation. It involves variation of protein expression, frequently in an on-off fashion - bacteria change phase in diff environments - ex) salmonellas switch expression of flagella or not -> once you get an human immune response it can switch flagella on or off - you either express the protein or not - not like antigenic variation which you will express different forms of the same protein - most gram neg bacteria do this

25 yo w/ fever, malaise, rash, painless lesion on her labia 3 weeks ago, no condoms. Rash is on palms and soles. What is a sensitive but nonspecific rapid serologic screening test used to diagnose?

phosphlipid (cardiolipin) on charcoal particles - VDRL detects nonspecific AB that reacts w/ beef cardiolipin - quantitative, inexpensive, and widely available test for syphilis (sensitive, not specific) - False positives: Pregnancy, Viral infection (EBV, Hepatitis), Drugs, Rheumatic fever, Lupus and leprosy (false Positive results on VDRL) - can also do dark field microscopy to directly see organism sphirochetes - confirmatory test: FTA ABs (fluorescent treponema antibody absorption) -> specific test to confirm screening

22 yo basektball player using cocaine develops SOB in practice. His respirations are 25/min and lung is hyper-resonant and diminished breath sounds. What is diagnosis?

pneumothorax - decrease breath sounds, hyper-resonant in percussion, decreased tactile fremitus

girl in a case from tibia fracture has cast removed. left calf is smaller than right calf. What process in myocytes are causing this atrophy?

polyubiquitination - Atrophy: decrease in tissue mass and size from increased cytoskeleton degradation via ubiquitin-proteasome pathway -> breaks down proteins

30 yo w/ irrational behavior and abdominal pain. Started tx w/ trimethoprim-sulfamethoxazole for UTI 3 days ago and urine turned burgundy. His mother/maternal GF have same symptoms. He has elevated 5-aminolevulinic acid and porphobilinogen. What enzyme is deficient?

porphobilinogen deaminase - Acute intermittent porphyria - 5 "Ps": painful abdomen, port wine-colored urine, polyneuropathy, psych issues, precipitated by drugs (cyto p450 inducers, alcohol, starvation) - tx: hemin and glucose -> inhibit ALA synthase

experiment examines pro-opiomelanocortin gene. The gene encodes an mRNA from which protein products are generated. The protein is a precursor of adrenocorticotropic hormon and B- lipotropin. What processes is necessary to generate these hormones?

post-translational modification - the formed protein itself makes the two separate proteins meaning you need something to happen after production of protein to form them - not alternative splicing - post-translational modification = - trimming: removal of N or C terminal propeptides from zymogen to generate mature protein (trypsinogen ->trypsin) - covalent alterations: phosphorylation glycosylation, hydroxylation, methylation, acetylation, and ubiquination

34 yo scientist was exposed to aerosolized 125 iodine. She has no symptoms. What can you give her?

potassium iodide - saturates the thyroid with stable, non-radioactive iodine

A direct inguinal hernia pushed through the superficial ring. If the hernia gut compresses the nerve that lies on the external surface of the spermatic cord at the superficial ring, what will be impaired?

sensation from anterior surface of scrotum - genitofemoral nerve genital branch (skin of labia majora/scrotum and cremasteric reflex) - ilioinguinal nerve = root of penis and upper scrotum - direct inguinal hernia -> through hesselbachs plexus -> exits through external surface of superficial inguinal ring where genitofemoral nerve is to be transmitted via cord w/ inguinal nerve as well

what does cocaine place a pregnant mother/child at risk for in pregnancy?

prematurity - also low birth weight, preterm birth, IUGR, placental abruption

How does prostate cancer spread to bone?

prostatic venous plexus to vertebral venous plexus directly

in a patient with a paraesophageal hernia what do you expect to see on imaging?

protrusion of the fundus into the chest above the level of T10 - gastroesophageal junction will be in normal position, but stomach fundus will protrude through the phrenoesophageal ligament (not the diaphragm)

24 yo african american male, red bumps on face, chin, neck that are itchy and painful. What does he have?

pseudofolliculitis barbae - "razor bumps" to the extreme

80 woman w/ cloudy urine after knee replacement, w/ temp, indwelling foley catheter. urine shows 12 WBC/hpf, and gram neg rods that are oxidase positive with a diffusible green pigment. What is the causal organism?

pseudomonas aeruginosa

45 yo w/ 6 month hx of SOB with exertion, hx of frequent nose bleeds since adolescence, has red spots on tongue, and clubbing of his fingers. Albuterol does not releive his symptoms. What is causing his symptoms?

pulmonary arteriovenous shunting - he has hereditary hemarrhagic telangiactasia (osler-weber-renu syndrome) = epistaxis, GI telangiactasia, and pulmonary AVms and cerebral AVms - GI bleeding, iron deficiency, pulmonary AVm -> cyanosis as they can cause a R->L shunt, dyspnea, pulmonary bleeds -> clubbing - increased risk of embolic stroke and cerebral absess - cerebal AVm can cause hemorrhagic stroke - nosebleeds = telangiactasia in nose

largest risk factor for sarcoidosis?

race -> African american

What is gene rearrangement?

recombination of gene segments to produce a functional variable region sequence - What is normally one gene configuration related to immune globulins in most tissues -> multiple bands in bone marrow = gene rearrangement -> how we create new antibodies - aka V(D)J recombination in lymphocytes in early stages of T and B cell maturation = somatic recombination -> highly diverse AB/immunoglobulins (Igs) and T cell receptors (TCRs) in adaptive immune system - V(D)J recombination in the primary lymphoid organs (bone marrow for B cells + thymus for T cells and in a nearly random fashion - literally rearranges actual gene segments in this unique location to gain new aa -> new immunoglobulin proteins

2 days after admission for sickle cell disease crisis, a 24 yo man has blood in urine, and flank pain. Labs at admission show decreased Hct 11% from normal 25%. UA shows gross blood, rare WBC, and no WBC casts. What is causing this?

renal papillary necrosis - Common in SAAD PAPaillaries: - Sickle cell, Acute pyelonephritis, Analgesics (NSAIDS), Diabetes Melitus - sloughing of necrotic renal papillae - gross hematuria and proteinuria - will present as kidney stone-like as patient passes the tissue

Clostridium perfringens alpha toxin creates gas gangrene via what mechanism?

splitting lecithin to phosphorylcholine and diglyceride - it is a phosholipase (lecithinase) that degrades tissue and cell membranes - degradation of phospholipids -> myonecrosis "gas gangrene" and hemolysis (double zone of hemolysis on blood agar)

How do you calculate reticulocyte index?

reticulocyte count X pt Hct/45 (normal Hct)

68 yo woman has increased urination and pain in low back. She had cervical cancer 15 years ago, and had hysterectomy w/ b/l salpingo-oophorectomy and had external beam radiation to pelvis. PE shows no abdominal masses. Labs shows increased urea and creatinine and decreased urine osmolality. US shows b/l hydronephrosis and hydroureter w/ distal ureteral narrowing b/l. What explains this?

retroperitoneal fibrosis - rare inflammatory disorder in which abnormal formation of fiber-like tissue (fibrosis) occurs behind membrane that lines cavity of abdomen (peritoneum) - abnormal tissue growth spreads to affect ureters from kidney -> bladder - similar to Reidel's thyroiditis = thyroid replaces by fibrous tissue w/ inflammatory infiltrate -> fibrosis may extend to local structures (trachea/esophagus) mimicking anaplastic carcinoma - considered a manifestation of IgG related systemic disease (autoimmune pancreatitis, retroperitoneal fibrosis, nonifectious aortitis) - b/l hydro -> increased creatinine - causes: 1) infections TB, histo, and actinomycosis 2) drugs: methysergide, pergolide, bromocriptine, ergotomines, methyldopa, hydralazine and beta blockers 3) external beam radiation for cancer 4) recent abdominal or pelvic surgery; or recent abdominal/pelvic trauma.

in patients w/ adenosine deaminase deficiency, there is a 50-100 fold increase in dATP concentration in T-lymphocytes. This increase inhibits what enzyme, thus compromising DNA synthesis?

ribonucleotide reductase - also inhibited by hydroxyurea (decreases DNA synthesis in S phase) makes pyrimidine bases - used in myeloproliferative disorders (CML, polycythemia vera, Sickle cell to increase HbF) - makes ribonucleotides for formation of DNA - adenosine deaminase deficiency = SCID (AR) - ADA is required for degradation of adenosine and deoxyadenosine. increased dATP causes lymphotoxicity and no T-cells - UDP is the precursor to pyrimidine nucleosides - DUP is converted to deoxy-UDP by ribonucleotide reductase - so any increase in ATP (adenosine triphosphate) or DNA = no more DNA needed - ribonucletides -> reduces them to the "dATP" - ATP negatively regulates this production specifically dATP

72 yo has mass in posterior mediastinum and in procedure the thoracic duct is damaged. Injury will impair drainage of what structure?

right kidney - the thoracic duct drains all of the organs below the diaphragm (extension of cysterna chyli) - is then courses behind the mediastinum (enters thorax w/ aorta at T12) -> this was the site of injury so any organ below diaphragm could have decreased drainage) - once it course towards entry site into veinous drainage at angle of left subclavian and left internal jugular, it joins left bronchomediastinal branch and left subclavian lymph trunk

40 yo woman w/ breast cancer has 1 week of loss of left peripheral vision in both eyes. where may metastatic tumor have spread?

right occipital lobe - or lesion/pressure on right optic tract

68 yo w/ 2 hr episode of loss vision in right eye 2 days ago. He had left-femoral-popliteal bypass 3 months ago for peripheral vascular disease. He has cholesterol emobli on funduscopic exam. What artery is affected?

right ophthalmic - Sole artery to the eye "end organ" in nature

30 yo woman w/ MS. Lights are off and both pupils are 6mm (usually 4mm in ambient light), light in left eye = both eyes constrict to 2mm. Light in Right eye = both dilate to 4mm. Where is the lesion in this patient?

right optic nerve - Marcus gunn pupil - aka relative afferent pupillary defect (RAPD) - light into normal eye -> constriction of ipsilateral and contralateral eye (direct/consensual reflex) - light in affected eye = both pupils dilate instead of constrict due to impaired conduction of light signaling along injured optic nerve - relative relaxation of pupils noted

25 yo man with low back pain radiating down his leg from lifting 200lb weights from ground over his head. whats causing this pain?

rupture of intervertebral disc - herniated lumbar disk that presses on the sciatic nerve (L4-S3)

What spinal reflex arch allows a male to maintain an erection?

sacral parasympathetic nerves and pudendal nerves (S2,3,4 -> external genetalia sensation)

60 yo man with prostate adenocarcinoma -> bilateral orchiectomy. After death the microscopic changes on his prostate will be what?

scattered shrunken cells - Prostate adenocarcinomas are receptive to growth from testosterone, so without it, the cells will become atrophied - can also give leuprolide (GnRH agonist that acts as an antagonist when given continuously -> decreases LH/FSH and testosterone production)

32 yo who likes to work at home with his 15 cats who "are his best friends and doesn't have to waste time talking to anyone" His boss wants him to join teams, but he refuses. what does he have?

schizoid personality disorder - cluster A personality disorder - voluntary social withdrawal (aloof) - limited emotional expression - content with social isolation

51 yo w/ HBV and antigen negative polyarteritis nodosa has muscle weakness/myalgia. Bx of muscle would show what?

segmental ischemic necrosis - Polyartiritis nodosa: inflammation of medium vessels (vasculitis) preventing blood flow/O2 delivery to organs - middle age men - HBV infection in 30% - fever, weight loss, malaise, headache - abdominal pain, melena, HTN, ernal damage - transmural inflammation of the artery wall w/ fibrinoid necrosis - diff stages of inflammation in diff vessels - renal micro-aneurysm - corticosteroids, cyclophosphamide

After starting someone on PPI w/ history of renal calculi, what lab test would you want to monitor?

serum calcium - decreased magnesium and calcium reabsorption may lead to fracture risk

75 yo woman w/ 16 hours left arm/leg weakness. Exam shows hemiplegia. CT scan of head shows an edematous right hemisphere. No evidence of hemorrhage but sparing of right occipital + midline frontal cortices. What will bx of brain show?

shrunken eosinophilic (red) neurons - Ischemic stroke or TIA (less than 24 hours) - ischemic stroke for 16 hours - just like heart, lack of O2 leads to timely change in tissue via liquefactive necrosis - 12-24 hours (here): eosinophilic change in cytoplasm of neurons (red neurons) - 24 hr: necrosis - day 1-3: infiltration of neutrophils - 4-7: microglial cells (macrophages), granulation - 2-3 weeks: gliosis (reactive astrocytes that line space of necrotic tissue)

3 week old has had vomiting after feeding since birth. He is eager to feed after vomiting. Abdomen shows mobile mass in epigastrum to the right of the midline. What explains this condition?

single primary developmental defect - wrong answers described - amniotic band: fibrous obstruction in amnion -> rings around digits/arms and legs and constricts them -> swelling and congenital lymphedema or amputation + club foot/hands and clefts of lip and palate, and hemangioma. from partial rupture of amnion (not chorion) can do fetal surgery - VACTER association: Vertebral abnormalities (hypoplastic vertebrae), ano-rectal defects (atresis/imperforate), cardiac defects (truncoarteriosus, transposition), Tracheesophageal fistual, Esophageal atresia, Renal defects (horseshoe, reflux), Radial defects (hypoplastic thumb, extra digit, radial aplasia/underdevelopment)

What doe the SMA supply?

small intestine, ascending colon, and part of transverse colon

52 yo w/ hypercholesterolemia on lovastatin and gemfibrozil is added. He then develops renal failure. Why?

statins have increased risk of myopathy especially when used with a fibrate or niacin - fibrates (gemfibrozil, bezafibrate, fenofibrate): decrease triglycerides and increase HDL slightly -> upregulate LDL and increase TG clearance and activate PPAR alpha to induce HDL synthesis - also have increased risk fo gallstones via inhibition of cholesterol 7 alpha hydroxylase (prevents formation of bile acid synthesis -> over saturation of cholesterol)

50 yo w/ SOB has BP 135/95, clear lungs, and ECHO shows hypokinesis of posterior LV w/ increasing activity levels. What is the cause of this?

stenosis of the right coronary artery - less blood is being sent to posterior descending artery that supplies posterior 2/3 of ventricles

woman with type 1 DM, recurrent episodes of fainting when she is hypoglycemic. What is the mechanism of an injection drug that can be used to help these episodes?

stimulation of hepatic glucose production

in a study on ADHD a researcher wants to look at a new behavioral program vs. standard therapy. Bc ADHD is more common in boys than girls, they are randomized separately. What is this called?

stratification - making populations homogenous and then randomizing from the homogenous groups

In a man who fell and can't breath, what is most suggestive that he fractured his ribs which is causing respiratory problems?

subcutaneous crepitus - pneumomediastinum - presence of gas (air) in mediastinum from trauma - crepitus from broken ribs

35 yo with discharge from right sphenoethmoidal recess. Where is this structure located?

superior to the superior concha - space above - drains the sphenoid sinus - superior meatus: drains posterior ethmoid, - middle meatus: anterior ethmoid, frontal, maxillary sinus - inferior meatus: drains nasolacrimal duct

55 w/ right shoulder pain after lifting weights above her head, an 8/10. point tenderness lateral to the acromion, over the humeral head. She has full passive motion of shoulder. when the shoulder is abducted 90 degrees and arm is given thumbs down sign -> pain against resistance. All sensation intact. what tendon is injured?

supraspinatus - most common rotator cuff injury - the muscle sits above and lateral to the acromion - Job's test/empty can test (internal rotation w/ thumb pointing to the floor)

44 yo w/ 2 month abdominal pain/diarrhea. relief after eating and antacids. Epigastric tenderness, and gastrin of 500 (N=50-100), and gastric acid secretions of 80 (N=6-40). what definitive trreatment?

surgical removal of suspected tumor - relief with meals = duodenal ulcers (gastric ulcers are worse with eating) - gastrin is much higher in ZE than h. pylori -

When food irritates your larynx and causes you to cough, are the vocal cords open or closed while swallowing, after irritation, and during coughing?

swallowing: closed After irritation: closed Coughing: open

a population of vegetarians surveyed to invstigate association between garlic/onion consumption and cholesterol. Results shows those with high consumption has mean serum cholesterol of 159. Those w/ low consumption have mean of 208. What test can yuo use to see if these are statistically different?

t-test - checks differences between means of 2 groups - tea is MEANt for 2 - ex) comparing mean BP btw men and women - ANOVA: checks differences btw 3+ groups (3 words analysis of variance) -> comparing BP btw three ethnic groups - Chi-squared: checks difference between 2+ percentages or proportions of categorical outcomes (not mean numerical values) think CHI-tegorical -> ex) comparing the percentage of 3 difference ethnic groups who have HTN

Production of what hormone in puberty causes increased acne?

testosterone - androgens increase acne production - testosterone, dihydrotestosterone, androstenedione are androgens - NOT estrogen, androstanediol

17 yo in ER on hot day collapses from being outside all day. When he was 4 he has many skin grafts for full thickness burns on 30% of body. He os hyperthermic. Why did this happen?

the burn scars and skin grafts lack eccrine glands, limiting his ability to sweat.

20 yo's period is one week late, and her period is a regular 28 day cycle. She had unprotected sex w/ her BF 20 days ago, and she is pregnant. What stage of development is the embryo at?

the neural plate is present, but the neural tube is not yet complete - week one: hCG secretion begins at time of implantation of blastocyst (day 6) - week two: bilaminar disk (2wks = 2 layers) - week three: gastrulation -> trilaminar disc (3wk=3 layers), notochord arises from mesoderm and overlying ectoderm = neural plate - week 3-8: neural tube formed by neuroectoderm -> closes at week 4 + organogenesis (teratogens most dangerous) - week 4: heart beat, upper/lower limb buds (4 wks, 4 chambers, 4 limbs) - week 6 fetal cardiac activity visible by US - week 8: fetal movements - week 10: genitalia (tenatalia)

why would a cholera vaccine of whole killed bacterial cell has little effect?

the vaccine cannot elicit secretory antibody at the epithelial surface

25 yo w/ malaise and facial rash for a week, 10 year hx of arthritic pain in peripheral joints. She has positive cardiolipin AB, anti-dsDNA, and anti-smith AB. what hemotological abnormality is most likely?

thrombocytopenia - SLE causes immune thrombocytopenia from anti-nuclear antibodies

What is the strongest risk factor of pancreatic cancer?

tobacco use, cigarette smoking, chronic pancreatitis, diabetes, age >50, jewish/AA males

30 yo pregnant at 20 wks has fundal height greater than expected for gestational age and increased amniotic fluid. What is causing this?

tracheoesophageal atresia - error in embryonic development at week 4-7 (embryonic stage of lung development) - presents as polyhydramnios in utero bc fetus unable to swallow amniotic fluid - polyhydramnios also in anencephaly (failure of rostral neuropore to close -> no forbrain, open calvarium, and increase fluid (no swallowing center in brain) - also seen in maternal diabetes, fetal anemia, multiple gestations

Valproic acid used for tx of bipolar disorder inhibits histone deacetylase. What does this affect in the cell?

transcription - histone deacetylation = removal of acetyl groups -> tightened DNA coiling -> decreases transcription - DNA methylation makes DNA mute (occurs in imprinting usually on CG islands) - histone acetylation makes DNA active (removes histones + charge -> relaxed DNA, increased transcription)

27 yo has cesarean delivery 2 months ago. She has firm subcutaneous nodule adjacent to surgical incision. Microscopic examination shows fibrous connective tissue, macrophages, multinucleated giant cells, fibroblasts, and few lymphocytes and foreign material. What factor promotes fibroblast migration and proliferation that led to the development of this lesion?

transforming growth factor B - responsible for angiogenesis and fibrosis - chronic inflammation of wound healing -> fibroblast proliferation and TGF B brings these in - fibroblasts make VEGF and makes new vessels - TGF B = profibrotic and downregulates acute inflammatory response (anti-inflammatory) - up regulated in cancer via matrix matalloproteinases which assists in basement memrabane migration - signals via SMAD pathway or induce apoptosis via SMADS -> activates MAPK -> P53

24 yo with prolonged asthma comes to ER and is given B2 agonist. What is a common effect that will be seen after?

tremor - albuterol relaxes bronchial smooth muscle (short acting B2 agonist) - for acute exacerbations - can cause tremor, arrhythmia - increases insulin release -> hypokalemia (increased cell uptake), lypolysis, increase glycogenolysis, aqueous humor production, decreases uterine tone (tocolysis -> suppress premature labor)

52 yo w/ abdominal pain for 6 years, bulky, foul-smelling stools that float. 20lb weight loss, and 20 yr hx of alcoholism. Pain in LUQ. What will be found in excessive amounts in the feces?

triglycerides - pancreatitis = hypercalcemia/hypertriglyceridemia - inability to absorb fats from diet - triglycerides are the most common fat in the human diet and in the human body - cholesterol is found in animal fats - inability to absorb fats from diet -> increased TG in stool

45 yo with MDD started on fluoxetine. He is at increased risk for serotonin syndrome with use of what substance found in food/drugs?

tryptophan

man w/ non-small cell carcinoma given vincristine. What is the intracellular target of this drug?

tubulin - vinka alkyloid that binds B-tubulin and prevents polymerization and mitotic spindle formation arresting the cell in M stage

46-year-old woman w/ ankylosing spondylitis tried NSAID, Sulfasalazine w/ no improvement. The most appropriate next step in treatment is administration of a drug that inhibits what?

tumor necrosis factor alpha - etanercept, infliximab, adalimumba etc - also used for RA (etanercept) and the rest for psoriasis, IBD - drug induces lupus and screen for TB prior to drug initiation - HLA-B27 - symmetric involvement of spine and SI joints -> ankylosis (joint fusion), uveitis, aortic regurgitation - bamboo spine (vertebral fusion), can cause restrictive disease - monitor reduces chest wall expansion to assess disease severity - more common in males

in a study designed to test a new drug to treat endometriosis, 100 woman were randomly assigned to one of two groups. 48 woman receive the new drug and 52 receive standard therapy. The primary purpose of this method of assigning patients to diff groups is to create what?

two groups with similar underlying characteristics - avoids selection bias aka making study populations that are not representative of target population - to ensure all groups in study are as similar as possible and to reduce any bias or lurking variables that can influence the outcome of the study

An woman dies and on autopsy what cell will take a majority of the alveolar endothelial surface area?

type I pneumocytes - 97% of alveolar surfaces -> line alveoli - squamous and thin for gas diffusion - collapsing pressure = 2 (surface tension)/radius

32 yo woman delivers baby w/ dysmorphic features and organ anomalies. Mother has had many spontaneous abortions. What explains the abnormalities in this baby?

unbalanced chromosome rearrangement - fetal aneuploidy (trisomy etc) are major causes for these abnormalities and reason for recurrent miscarriages - Has either Edward syndrome (18) or Patau (13)

What is normal jugular venous pressure?

under 8mm hg - 12 mm hg -> high and tells us that the person is in right heart failure most likely from left heart failure

mass in ovaries (CA 125 positive cancer) needs to be removed. What structure passing inferior to ovary is at risk of injury?

ureter - passes behind ovary, round ligament, and ovarian vein and artery

35 yo has left oophorectomy due to a mass. What structure is at greatest risk for injury when dividing the suspensory ligament?

ureter - suspensory ligament (aka infindibulopelvic ligament) contains ovarian vessels - ligated in oophorectomy to avoid bleeding - ureters course retroperitoneally, close to the vessels -> risk for injury during ligation - ovarian artery and vein come down from aorta and travel directly over ureter - ureter also under uterine vessels in cardinal/transverse cervical ligament

Saying ah and watching elevation of the uvula is by what nerve?

vagus

28 yo pregnant woman has anal itching, pain with defecation, and bright red blood with wiping. She has constipation. What is the underlying cause of this issue?

venous hypertension -

38 yo shot, and placed on broad spectrum AB. His usual dose of warfarin over the next 6 weeks has to be decreased. Why?

vitamin K deficiency caused by depletion of normal gut flora from AB use - Unlike vitamins A/D, vitamin K not stored in body in significant amounts can become deficient in a week - lower vitamin K means there is less for warfarin to block (epoxide reductase activation of vitamin K blocked by warfarin)

A food handler has a furuncle and prepares meatloaf that stands at room temperature for 12 hours that people eat and get sick from. What symptoms will they get?

vomiting - a furuncle = a boil most commonly cause by staph aureus - staph can cause rapid onset food poisoning from enterotoxins - due to ingestion of preformed toxin - short incubation period (2-4hrs) -> non-bloody diarrhea and emesis - enterotoxin is heat stable and not destroyed by cooking

20 yo has intense menstrual bleeding that is regular, and she takes OCP. She cut her finger and it took longer to clot. normal platelets. Pale conjunctiva. Anemia on labs, normal PT and PTT. What does she have?

von willibrand disease - AD disorder - common to have heavy menses - OCP increases clotting factors most likely normalizing her PTT which could be decreased due to no VWF maintaining factor 8 - no platelet coagulation with ristocetin - treat w/ desmopressin that releases vWF from endothelium

What causes cachexia?

weight loss, muscle atrophy, and fatigue in chronic disease (cancer, AIDS, HF, COPD) - mediated by TNF-alpha, INF-gamma, IL-1, and IL-6 - all cytokine mediated

32 yo w/ 6 months of low back pain and stiffness worse in morning and improve during day. Pain radiates to buttock and not down his legs. He gets stiff when sitting for too long. He cannot bend forward completely. How can you confirm the dx

x-ray of sacroiliac joints - symmetric involvement of spine and sacroiliac joints -> ankylosis (joint fusion), uveitis, aortic regurgitation - Bamboo spine (from vertebral fusion) - costovertebral and costosternal ankylosis -> restrictive lung disease - monitor degree of reduced chest wall expansion to asses disease severity -more common in males - A seronegative spondyloarthritis (no rheumatoid factor IgG AB) - association w/ HLA-B27 (MHC I) - all have back pain w/ morning stiffness improves w/ exercise, peripheral arthritis, inflamed insertion of tendons, dactylitits (sausage fingers) uveitis

Why do you not want to give someone w/ salmonella enterica antibiotics?

you want to avoid prolonged fecal excretion of the organism