UWorld Mock blocks week 1/28-1/3

Question 1

Fabry inheritance

Answer 1

X-linked recessive

Question 2

alpha-galactosidase A deficiency –> accumulation of globotriaosylceramide

Sx present in adolescence.

Neuropathic pain, hypohydrosis (decreased sweating), angikeratomas, telangiectasis, glomerular disease, cerebrovascular disease, cardiac disease

Answer 2

Fabry

Question 3

Shizotypal

Answer 3

odd-type of behavior, odd beliefs, social anxiety, and perceptual distortions - eccentric

and odd-type LIKE schizophrenia

Question 4

Schizoid

Answer 4

Socially detached - loners

Question 5

dofetilide

Answer 5

Class III anti-arrhythmic (amiodoraone and sotalol)

K+ blocker

Inhibit outward potassium current during repolarization - phase 3 –> prolong repolarization and prolong action potential duration

Question 6

Ureteric bud derivatives

Answer 6

collecting system: collecting tubules and ducts, major/minor calyces, renal pelvis, ureters

Question 7

Metanephric mesoderm derivatives

Answer 7

glomeruli, bowman’s space, PCT, loop of hence, DCT

Question 8

Leukamoid reaction

Answer 8

benign leukocytosis in response to an underlying condition such as were infection/hemorhage, malignancy, or acute hemolysis.

Alkaline pos levels are normal or increased (would be low in CML)

increased bands, myelocytes, and granules (dohle bodies) in neutrophils

Question 9

Isotype switching

Answer 9

Germinal centers of lymph nodes

CD40-CD40L

Question 10

MHC I

Answer 10

Heavy chain and a beta-2 micro globulin

On all cells

Presents antigen to cytotoxic CD8+ cells

Results in apoptosis by CD8

Question 11

MHC II

Answer 11

Alpha and beta chains

Found on APCs

Presents antigen to CD4+

Results in TH cell activation to stimulate cell mediated and humoral immune response

Question 12

aminolevulinate synthase

Answer 12

rate-limiting step of heme synthesis

Requires pyridoxine as co-factor

Isoniazid or B6 deficiency –> impaired function –> impaired heme synthesis –> sideroblastic anemia (microcytic)

Question 13

C. diff treatment

Answer 13

Fidaxomicin, metronidazole, or vancomycin

Question 14

Latanoprost

Answer 14

Topical prostaglandins to treat open angle glaucoma

Increase OUTFLOW of aqueous humor

Question 15

Ulnar nerve compression

Answer 15

Medial epicondyle OR Guyon’s canal (hook of hamate)

Question 16

Isoniazid

Answer 16

Inhibits mycotic acid synthesis

Question 17

Rabies treatment

Answer 17

prophylactic inactivated rabies vaccination

Question 18

S3

Answer 18

Normal in <40and pregnancy

Systolic heart failure, mitral regurg, high output states

Best heard in left lateral decubitus at end-expiration

Question 19

S4

Answer 19

Found in older adults

Stiff ventricle - always pathologic

Abnormal in children, indicates diastolic dysfunction - LVH

Question 20

Dandy walker malformation

Answer 20

Absence of cerebellar vermis, cystic dilate of fourth ventricle, posterior fossa enlargement

Developmental delay and progressive skull enlargement

Question 21

Rickets

Answer 21

Excess of unmineralized osteoid matrix and epiphyseal cartilage d/t Fit D deficiency

Frontal bossing, craniotabes, costochondral junction deformity, bowed legs

Question 22

Thiazides

Answer 22

Indirectly cause hypercalcemia –> suppressed PTH

Question 23

Between keratinocytes

Answer 23

Desmosomes - destruction = pemphigus vulgaris

Acantholysis - intraepithelial cleavage BETWEEN keratinocytes

Question 24

Lithium

Answer 24

Nephorgenic DI

Question 25

Lynch syndrome

Answer 25

Mismatch repair gene defect Colorectal cancer Ovarian cancer Endometrial cancer

Question 26

Tacriolomus and cyclosporine - immunosuppressants used to prevent transplant rejection and inflammatory disorders

Answer 26

Calcineurin inhibitors Nephrotoxic

Question 27

Metoclopramide

Answer 27

Contraindicated in parkinson's - antagonist on dopamine receptors

Question 28

Tissue ischemia

Answer 28

Dysfunction on Na/K ATPase --> K+ leakage from affected cells Increase Na+ in cells --> swelling and explosion

Question 29

Homocysteinuria

Answer 29

Marfanoid with intellectual disability, hypercoagulability, and downward lens dislocation

Question 30

Water soluble vs. lipid soluble in placenta

Answer 30

Water soluble DON'T cross placenta - Heparin Lipid soluble DO cross placenta - Warfarin

Question 31

Hyperkalemia

Answer 31

Peaked T waves on ECG

Question 32

Hypocalcemia

Answer 32

QT prolongation

Question 33

Hypercalcemia

Answer 33

QT shortening, T-wave flattening or inversion in severe cases

Question 34

Hypokalemia

Answer 34

QT prolongation, U-waves, and ST depression Severe hypokalemia causes torsades

Question 35

Tardive dyskinesia

Answer 35

up regulation of dopamine receptors

Question 36

Dopamine beta hydroxylase

Answer 36

Converts dopamine to norepinephrine - necessary for sympathetic adrenergic activity

Question 37

Fructose-2,6-biphosphate

Answer 37

Increases when insulin is released --> activates phosphofructokinase-1 --> glycolysis can proceed Decreases when glucagon is released --> decreased inhibition of Fructose-1,6-biphosphatase --> gluconeogenesis can occur

Question 38

CMV RETINITIS

Answer 38

CD4<50 Treat w/ ganciclovir

Question 39

CKD

Answer 39

decreased phosphate excretion and vitamin D conversion --> hypocalcemia --> secondary hyperparathyroidism

Question 40

CD18

Answer 40

Leukocyte adhesion deficiency

Question 41

Anti-jo-1

Answer 41

Dermatomyositis Polymyositis + skin findings

Question 42

Dubin-johnson syndrome

Answer 42

conjugates chronic hyperbilirubinemia - no hemolysis

Question 43

Zidovudine

Answer 43

nucleoside reverse transcriptase inhibitor to treat HIV infection Competitively binds reverse transcriptase and is incorporates as thymidine analog w/o a 3'-OH group - prevents formation of 3'-5' phosphodiester bond

Question 44

Sheehan syndrome

Answer 44

ischemic necrosis of pituitary - pituitary grows w/o increased blood supply.

Question 45

Zollinger-ellison

Answer 45

Ulcers, diarrhea, abdominal pain, acid reflux MEN 1

Question 46

Milrinone

Answer 46

Used in refractory heart failure PDE-3 inhibitory --> increase cAMP degradation --> Ca2+ influx --> positive isotropy PDE-3 inhibitor in vascular smooth muscle --> arterial and venous dilation

Question 47

Class IA antiarrhythmics

Answer 47

Quinidine, procainamide, disopyramide Intermediate phase 0 depolarization Prolonger AP

Question 48

Class IB antiarrhythmics

Answer 48

Lidocaine, mexelitine Weak phase 0 depoloraztion Shortened AP duration

Question 49

Class IC antiarrhythmics

Answer 49

Flecainide, propafenone Strong phase 0 depoloraztaion No change in AP duration

Question 50

Elastase

Answer 50

Released by neutrophils or alveolar macrophages --> emphysema

Question 51

Xeroderma pigementosa

Answer 51

AR defective nucleotide excision repar - UV-specific endonuclease