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STEP 1 Studying > 5.5.2015 UWorld Notes > Flashcards

5.5.2015 UWorld Notes Flashcards

1
Q

Axtaxia telangiectasia: Characteristic features

A

Ataxia
Telangiectasia
Sinopulmonary infections

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2
Q

Chediak Higashi syndrome

A

Oculocutaneous albinism
Pyogenic infections
Progressive neurologic dysunction

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3
Q

Chronic granulomatous disease

A

Severe bacterial and fungal infections

Granuloma formation

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4
Q

DiGeorge syndrome

A 
Congential heart disease
Dysmorphic facies
Hypocalcemia from parathyroid hypoplasia
Conotruncal cardiac anomalies 
Hypoplastic thymus
Immunodeficiency like in SCID can occur but less than 1%
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5
Q

SCID

A

Severe bacterial and viral infections in infancy
Chronic diarrhea
Mucocutaneous candidiasis
Starts in infancy
Mutations in genes that impair T and B cell evelopment and function. Cell mediated and humoral immunity messed up.
Low or absent CD3+ T cells and hypogammaglobulinemia.
Thymic hypoplasia or aplasia is common finding due to severe T cell deficiency.

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6
Q

C5-C9 deficiency

A

Recurrent Neisseria infection

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7
Q

Wiskott Aldrich syndrome

A

Recurrent infections that worsen with age
Easy bleeding due to thrombocytopenia and eczema
Eczema
Abnormal cytoskeletal functioning=bad T and B cells.
Recurrent viral bacterial fungal infections.

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8
Q

CVID

A

B and T cell dysfunction
Hypogammaglobulinemia. Presents with recurrent infections in adults and kids after neonatal period.
Cell mediated immunity not as impaired as in SCID and thymic aplasia is unlikely.

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9
Q

X-linked agammaglobulinemia is ID syndrome caused by insufficiency production of mature B cells. What type of infections?

A

Encapsulated pyogenic bacteria (S. penumonia, H. influenzae, type B).
T cells are fine. NO absent thymic shadow.

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10
Q

Blotchy red muscle fibers on Gomori trichrome stain

A

Mitochondrial myopathies.

Abnormal mitochondira accumulate under sarcolemma of muscle fbiers. “REd ragged fiber diseases.

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11
Q

Mitochondrial myopathies

A

Myoclonic epilepsy with ragged red fibers
Leber optic neuropathy (blindess)
Mitochondrial encephalopathy with stroke like episdoes and lactic acidosis

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12
Q

Mitochondrial diseases have what type of inheritance?

A

Maternal

Male will not transmit to progeny

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13
Q

Common peroneal (fibular) nerve most commonly injured in leg why?

A

Superficial location laterally around neck of fibula

Lateral blows to knee and from plaster casts

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14
Q

Sciatic nerve branches into

A

Common peroneal nerve and tibial nerve in thigh posteriorly before popliteal fossa.

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15
Q

Common peroneal nerve divides into

A

Superficial and deep branches of peroneal just after neck of fibula.

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16
Q

Sensory of deep peroneal and superficial peroneal?

A

Deep peroneal: Sensory innervation to area between 1st and 2nd toes.
Superficial peroneal sensory to majority of foot.

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17
Q

What goes through popliteal fossa?

A

Popliteal artery and vein plus tibial nerve.

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18
Q

Tibial nerve

A

Plantar flexion and inversion.

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19
Q

Superficial branch peroneal motor innervates

A

lateral compartment of leg which everts foot.

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20
Q

Deep peroneal nerve motor innervates

A

anterior compartment of leg and this is dorsiflexion of foot and toes.

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21
Q

What is near medial malleolus?

A

Anteriorly: Great saphenous vein and saphenous nerve. Posteriorly: Posterior tibial artery, tibial nerve and tensons of flexor digotrum longus, flexor hallucis longus, and tibialis posterior.

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22
Q

Start codon?

A

AUG

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23
Q

Stop codon?

A

UAA, UAG, UGA

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24
Q

Splicing of introns in postranscription is done by?

A

snRNPs (small nuclear ribonucleoproteins

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25
Q

Uncharged tRNA (no aa) would not ineract with

A

mRNA and ribosomes during protein synthesis

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26
Q

Charged tRNA delivers aa to

A

Protein synthesis complex. Anticodon on tRNA molecule recognizes codon on mRNA and puts aa in sequence.

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27
Q

What binds to TATA promoter region?

A

Transcription factor II

Facilitates tRNA binding and translocation steps of protein synthesis.

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28
Q

Stage refers to how much tumor?

A

Spreads into adjacent tissues and distant organs

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29
Q

Grade refers to degree of tumor?

A

Differentiation

Low grade=Well differentiated

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30
Q

Thymidine and brain tumors. Why?

A

Thymidine is nt used for DNA syntehsis. Uptake occurs in S phase of cell cycle. High uptake=large number of tumor cells in S phase. Cells in S phase will soon proceed to mitosis and proliferate. Indicates high grade tumor. Poor prognosis.

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31
Q

High grade tumors are radiosensitive because?

A

Rapidly dividing cells

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32
Q

Signs of marijuana intoxication?

A

Conjunctivial injection, tachyardia, increased appetite, dry mouth. Metabolized in liver. Stored in lipophilic tissues.

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33
Q

Miosis (constricted) seen in what intoxication?

A

Opiate

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34
Q

Mydriasis (dilated) seen in?

A

Cocaine intoxication and opiate withdrawal

35
Q

Nystagmus seen in?

A

PCP intoxication

36
Q

Early MI changes (total myocardial ischemia)

A

Cessation of aerobic glycolysis and initiation of anaerobic glycolysis. Within 60 seconds, there is no cardiomycocyte contractility. Low ATP production and accumulation of bad metabolites (lactate).

Ischemia less than 30 minutes=>Restoring blood flow reversible contractile dysfunction (myocardial stunning). Returns to normal in hours to days. After 30 minutes, irreversible injury.

37
Q

Rubeola/Measles

A

Infection with measles virus (enveloped, nonsegmented, negative sense RNA virus). Koplik spots (white/blue specks on buccal mucosa adjacent to second molars).

CCCK; Cough, Coryza, conjunctivitis, Koplik spots. MEASLES. Maculopapular rash in 1-2 days after this.

38
Q

Age related macular degeneration

A

Age, genetics, enviro factors, smoking=Inc risk
Dry AMD= Gradual vision loss in one or both eyes. Chronic oxidative damage to retinal pigment epithelium and choriocapillaris->subrentinal inflammation->abnormal extracellular matrix formation. Subretinal drusen deposits.

Wet AMD: Acute vision loss with metamorphopsia (distrotion of straight lines). Gray green subretinal discolaration with adjacent fluid/hemorrhage. Hypozia stimulates local VEGF and causes neovascularization with leaky vessels.

Treatment: Smoking cessation. Wet AMD=treat with VEGF inhibitors (ranibizumab and bevacizumab)

39
Q

Rituximab

A

Against CD20 glycoprotein on B cells (lymphoma and RA)

40
Q

Epidermal growth factor receptor inhibitors to treat non-small cell lung cancer

A

Erlotinib and gefitinib

41
Q

Anti-interleukin 2 therapy for

A

Immunosuppression in organ transplant patients (graft vs host disease).

42
Q

TNF alpha inhibitors

A

Inflammatory atuoimmune conditions (RA, IBD, spondyloarthropathies).

43
Q

Excessive bleeding common in patients with renal dysfunction due to

A

accumulation of uremic toxins in circulation. These impair aggregation and adhesion of platelets. Prolonged bleeding time with normal platelet count, prothrombin time,and activated partial thromboplastin time.

Uremic bleeding can be improved with dialysis.

vWF=bridge between glycoprotein 1b receptors and subendothelial collagen allowing platelets to bind to damaged vessels. Prolonged bleeding time and normal platelet count. Decreased factor VIII and prolonged aPTT because vWF protects factor VIII from degradation.

44
Q

Hemophilia A

A

Isolated aPTT prolongation. Caused by factor VIII deficiency.

45
Q

Heparin prolongs aPTT via

A

Antithrombin associated inhibition of factors II and X. PT is normal due to addition of heparin neutralizers in PT reagent.

46
Q

DIC would cause

A

Decreased platelet count. Increased bleeding time, aPTT, and PT.

47
Q

Warfarin inhibits

A

vit K dep clotting factors (II, VII, IX, X)

Prolongs PT and aPTT but PT is increased more than aPTT. Platelet count and bleeding time are normal.

48
Q

Treating thyrotoxicosis

A

Beta blocker (propranolol) decreases peripheral conversion of T4 to T3 by inhibiting iodothyronine deiodinase and decreases TH action on target tissues. Reduces heart rate and anxiety.

Thyroid storm (thyrotoxicosis) increases sensitivity to catecholamines via TH mediated upregulation of beta adrenergic receptors. B blockers decrease effect of sympathetic adrenergic impulses on target organs.

49
Q

What decreases TH synthesis?

A

Propylthioruracil and methimazole (inhibit thyroid peroxidase)

50
Q

What reduces TH release in thyrotoxicosis?

A

Iodine

51
Q

What can cause reperfusion arrhythmia on arterial reopening?

A

Fibrinolytics like tPA. Most effective if used in 6 hours.
These arrhythmias are usually benign.

tPA increases formation of plasmin from plasminogen and increases clot lysis. tPa, reteplase, and tenecteplase act only on fibrin attached to recently formed clot without systemic activation.

Contraindications: Hemorrhagic stroke, ischemic stroke in past year, active internal bleeding, bp of > 180/110 mm Hg, dissecting aneurysm.

52
Q

Vasopressin (ADH) affects

A

Late distal convoluted tubule water permeability varies based on ADH
Urinary pH regulated through H+ secretion by intercalated cells in late distal and collecting tubules.

Thick and thin ascending limbis of loop of Henle are impermeable to water. Osmolarity of tubular fluid decreases due to passive adsorption of NaCL and active transport of leectrolytes out of lumen by Na/K/2Cl cotransporter.

Descending limb of loop of Henle permeable to water. Most ions stay in lumen and water moves into intersititum. Lumen fluid becomes hypertonic.

PCT: Reabsorbs electrolytes, glucose and aa. Water reabsoprtion occurs passively with transport of solutes. Tubular fluid is isotonic.

DCT: Water permeability is low.

53
Q

Foamy urine

A

Bile salts or proteinuria in urine.
URI and ankle edema=Nephrotic syndrome associated with low serum albumin. Hypoalbuminemia lowers plasma oncotic pressure and causes intersitial edema formation due to net plasma filtration into interstitum. Reduction of intravascular volume stimulates increase in RAAS.

54
Q

Left sided heart failure b/c poorly controlled HTN

A

LV hypterophy and decreased LV diastolic compliance. Higher diastolic filling pressures needed to maintain SV and CO. Increase in pressure is transmitted backward to LA and pulmonary veins where it can cause pulmonary HTN.

Increase in pressure leads to pulmonary venous congestion. Endothelial damage and capillary leakage of serum proteins into intersititum. Decreased production of NO (vasodilator) and increased production of endothelin (vasoconstrictor) by dysfunctional epithelium results in increased vascular tone. Over time, remodeling of pulmonary vasculature with increased smooth muscle cell proliferation and collagen/elasatse deposition. This is usually reversible when LV dysfunction is corrected.

COPD: Pulm HTN happens because hypoxia induced vasoconstriction and emphysematous oblieration of vasculature.

Large ventricular septal defect will cause PH at young age if increased pulmonary artery flow volume. ASD take longer to produce PH because less shunting and pressure overload. In CHF, pulmonary arterial flow will decrease or remain same.

55
Q

Beta blockers given to pts with stable angina and HTN becuase

A

Inhibit sympathetic stimulation of heart reducing HR and CTY.

Atenolol is selective B1 adrenergic antagonist. B1 receptors are found in cardiac tissue and renal jxtaglomerular cells but not on vascular smooth muscle. B1 is GPCR which increases cAMP levels.

Propranolol is nonselective B blocker. B2 and B2 adrenergic receptors blocked. Results in decreased cAMP in cardiac myocytes, renal juxtaglomerular cells, and vascular smooth muscle.

Diltiazem is Ca channel blocker and doesn’t affect cAMP.

56
Q

Klebsiella pneumonia

A

Gram neg lactose fermenting thick capsule clear zone on Gram stain mucoid growth in culture.

Affects alcoholics. Aspiration while asleep from colonies in oropharynx. Upper lobes affected. Blood tinged sputum and lung tissue necrosis with early abscess formation.

57
Q

PCR you need…

A

Nucleotide sequence of regions flanking target DNA
DNA polyermase Taq polymerase bc not denatured by high temps required for the denaturation phase of PCR. Eliminates need to replenish enzyme with repeated cycling. Elongates in 5 to 3 direction starting with 3 end of primer.

Deoxynucleotide triphosphates: Required to build new DNA strands.

58
Q

Nucleolus contains

A

Ribosomal DNA coding for 28S, 5.8S, and 18S ribosomal RNA parts and newly transcribed rRNA in associated with ribosomal proteins translated in cytoplasm from mRNA. Primary function is synthesis and assembly of ribosomal subunits 60s and 40s.

59
Q

Minute ventilation

A

Total volume of new air that enters respiratory pathways per minute.
Min vent=Tidal volume * breaths/min

60
Q

Alveolar ventilation is

A

Volume of new air reaching gas exchange areas per minute. Does not include dead space volume.
Alveolar vent=(Tidal volume-dead space) volume*breaths/min

61
Q

FRC

A

Volume of air remaining in lungs after normal exhalation. Sum of residual volume and expiratory reserve volume.

62
Q

Residual volume

A

Volume of air remaining in lungs after max exhalation.

63
Q

Tidal volume is

A

Volume of new air that enters lungs with normal breath.

64
Q

Vit A overdose

Acute, chronic, teratogenic

A

Acute: N/V, vertigo, blurred vision.
Chronic: Alopecia, dry skin, hyperlipidemia, hepatotoxicity, hepatosplenomegaly, visual difficulties. Papilledema suggestion of cerebral edema in setting of benign intracranial HTN. Pseudotumor cerebri.
Teratogenic: Microcephaly, cardiac anomalies, fetal death.

Thiamine deficiency: Berberi and Wernicke Korsakoff in alcoholic. Peripheral neuropaty, high output cardiac failure. Traid of ataxia, confusion, ophthalmoplegia.

Niacin deficiency is 3D’s of pellagra: Dementia, dermatitis, diarrhea.

Vitamin B12 deficiency (cobalamin) is pernicious anemia. Older, mentally slow woman with is lemon colored (anemic and icteric) with smooth shiny tongue atrophic glossitis and shuffling broad based gait.

Vitamin B2 deficiency: Cheilosis, stomatits, glossitis, dermatitis, corneal vascularization, ariboflavinosis.

Vitamin C overdose: False neg stool guaiac results. Diarrhea and abdominal bloating. Calcium oxalate nephrolithiasis maybe.

Vitamin E overdose: Hemorrhagic stroke in adults and necrotizing enterocolitis in infants.

65
Q

Alpha and beta interferons suppress viral replication by

A

Induces apoptosis of infected cells, limiting viral protein synthesis.

66
Q

Interferon gamma

A

produced by NK and T cells. Functions to promote TH1 differentiation and induce class II MHC expression and activate macrophages.

67
Q

Shigella

A

Fever, abdominal cramps, blood diarrhea (dysentery).
Transmitted via fecal oral route.
Non lactose fermenting organisms that produce acid during glucose fermentation. Do not produce H2S and are not mobile.
Shigella invades GI mucosa esp M cells over Peyer’s patches. Lyse its vacuole and enter cytosolic compartment.

Can induce apoptosis of hot cell and spread to adjacent cells via protrusions created through host cell actin polymerization. Shigella invasion triggers robust host inflammatory response that is mediated by neutrophils.

Some strains do produce shiga toxin and halt protein synthesis but plays a minor role in disease process.

Very pathogenic and easy to spread (10-100 organisms).

68
Q

DNA damage

A
  1. Depurination of DNA and base excision repair (spontaneous or chemical)
  2. Formation of thymine dimers (UV rays)
  3. Breaks in DNA chains and oxidative damage (ionizing radiation)
  4. Cross linkage, intercalation and alkylation (chemical and pharm agents)
69
Q

Base excision repair used to correct defects in single bases induced by chemicals or spontaneous. Process?

A

Glycosylases remove defective base, 5” end removed by endonuclease, sugar-phosphate is cleaved on 3” by lyase. DNA polymerase replaced missing nucleotides and ligase reconnects DNA strand.

70
Q

Nafcillin resistance is mediated by alterations in penicillin binding protein structures. PBPs?

A

MRSA resistant to all B lactam antibiotics because of altered PBP that doesn’t bind beta lactam effectively. PBPs are enzymes involved in cell wall synthesis. Altered PBPs have reduced affinity for all beta-lactam antimcirobial agents.

B lactamase inactivates B lactam antibiotics (penicillins) by breaking B lactam ring.

Rifampin resistance: Mutation in RNA polymerase
Tetracycline and sulfonamides: Decrease in levels of drug bc uptake and/or increased efflux.
Quinolone: Mutations in DNA gyrase.

71
Q

Intercostal VAn lie in subcostal groove on…

A

Lower border of rib.

72
Q

Salicylate intoxiation

A

Respiratory alkalosis (salicylicates stimulate medullary respiratory center. Resulting increase in ventilation leads to increased loss of CO2 in expired air).

AG metabolic acidosis (Lipolysis; inhibits citric acid cycle; ketoacids, lactate, pyruvate in blood).

pH in normal range as acid base disturbances shift pH in opposite directions.
PaCO2 on ABG is low (respiratory alkalosis and compensation for metabolic acidosis). Low HCO3 due to metabolic acidosis.

73
Q

Primary hyperaldosteronism: Conn’s syndrome

A

HTN, hypokalemia, metabolic alkalosis, decreased plasma renin activity. Aldosteorne causes resorption of Na and H20 and wasting of K and H ions.

High aldosterone suppresses renin.

Treatment is surgical resection of adenoma or aldosterone antagonists. Spironolactone or eplerenone (few side effects).
Side effect of both: Gynecomastia.

74
Q

1 SD, 2SD, 3SD

A

1 SD holds 68% of values.
95% in 2 SD 2.5% above 2SD and 2.5% below 2SD
99.7% in 3 SD

75
Q

H pylori infection of gastric mucosa

A

Higher risk of MALT lymphoma (low grade B cell lymphoma) and gastric adenocarcinoma. H pylori affects gastric antrum.

Overproduction of gastrin by pancreatic islet cell tumor=hypersecretion of gastric acid leading to peptic ulcers, abdominal pain, GERD, diarrhea. ZE sndrome.

Crohn’s disease: Fevers, RLQ abdominal pain (terminal ileitis), diarrhea.

Gluten intolerance: Lymphocytic infiltrations of small intestinal mucosa and blunting of intestinal villi in small intestine.

Megaloblastic anemia in Type A chronic gastritis (autoimmune). Affects body of stomach rather than antrum. Autoimmune destruction of parietal cells causes decreased IF and failure of vitamin B12 absorption.

76
Q

Warfarin inhibits anti-coagulants

A

Protein C and S
Without these, hyperoagulable state. Can cause kin necrosis because Protein C declines yet Factor II, IX, and X decline at slower rate. Can cause thrombosis in patients with congenital deficiency of protein C and S in first week of therapy.

77
Q

Friedreich’s ataxia and Vitamin E

A

Degneration of spinocerbellar tracts, dorsla column of spinal cord and peripheral nerves.
Ataxia, dysarthria, loss of positoin and vibration sensation.

78
Q

Phenobarbital and other barbiturates can induce hepatic metabolism and clearance of warfarin. Can also increase hepatic synthesis of clotting factors.

A

This can lead to left atrial mural thrombus and thromboembolic stroke.

79
Q

CYP 450 inducers

A 
Carbamazepine
Phenobarbital
Phenytoin
Rifampin
Griseofulvin
80
Q

CYP 450 inhibitors

A 
Cimetidine
Ciprofloxacin
Erythromycin
Azole antifungals
Grapefruit juice
Isoniazid
Ritonavir
81
Q

C difficile infections treated with oral metronidazole, vancomycin, or fidaxomicin.

A

Metronidazole (mild-to-moderate cases) and vancomycin (severe cases) are first line options with fidaxomicin used for patients with recurrent colitis or at increased risk of recurrence. Fidaxomicin has minimal systemic absorption and narrow spectrum (recurrent CDI and bacteriocidal). Less effect on normal colonic flora than metronidazole or vacomycin.

Doxycycline effective in clostridial skin infections (C perfringens, C septicum)
Metronidazole can be used for initial treatment of CDI and first recurrence.
Neomycin used to treat diarrhea due to E coli and hepatic encephalopathy. Surgical prophylaxis for GI procedures.

Oral vancomycin is recommended for treatment of initial or recurrent CDI. Minimal systemic absorption and bacteriostatic against C difficle.

82
Q

High levels of prolactin

A

Suppress GnRH leading to hypogonadism, milk discharge from breasts (galactorrhea).

Hyperprolactinemia causes hypogonadism (low estrogen in females) which causes accelerated bone loss.

Risk of enlargement of microadenoma is low.

83
Q

MEN 1

A

Parathyroidism (hyper)
Peptic ulcer due to gastrin secreting tumor (pancrease)
Pituitary adenoma

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