UWorld Mock blocks week 1/28-1/3 Flashcards
Fabry inheritance
X-linked recessive
alpha-galactosidase A deficiency –> accumulation of globotriaosylceramide
Sx present in adolescence.
Neuropathic pain, hypohydrosis (decreased sweating), angikeratomas, telangiectasis, glomerular disease, cerebrovascular disease, cardiac disease
Fabry
Shizotypal
odd-type of behavior, odd beliefs, social anxiety, and perceptual distortions - eccentric
and odd-type LIKE schizophrenia
Schizoid
Socially detached - loners
dofetilide
Class III anti-arrhythmic (amiodoraone and sotalol)
K+ blocker
Inhibit outward potassium current during repolarization - phase 3 –> prolong repolarization and prolong action potential duration
Ureteric bud derivatives
collecting system: collecting tubules and ducts, major/minor calyces, renal pelvis, ureters
Metanephric mesoderm derivatives
glomeruli, bowman’s space, PCT, loop of hence, DCT
Leukamoid reaction
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
Isotype switching
Germinal centers of lymph nodes
CD40-CD40L
MHC I
Heavy chain and a beta-2 micro globulin
On all cells
Presents antigen to cytotoxic CD8+ cells
Results in apoptosis by CD8
MHC II
Alpha and beta chains
Found on APCs
Presents antigen to CD4+
Results in TH cell activation to stimulate cell mediated and humoral immune response
aminolevulinate synthase
rate-limiting step of heme synthesis
Requires pyridoxine as co-factor
Isoniazid or B6 deficiency –> impaired function –> impaired heme synthesis –> sideroblastic anemia (microcytic)
C. diff treatment
Fidaxomicin, metronidazole, or vancomycin
Latanoprost
Topical prostaglandins to treat open angle glaucoma
Increase OUTFLOW of aqueous humor
Ulnar nerve compression
Medial epicondyle OR Guyon’s canal (hook of hamate)
Isoniazid
Inhibits mycotic acid synthesis
Rabies treatment
prophylactic inactivated rabies vaccination
S3
Normal in <40and pregnancy
Systolic heart failure, mitral regurg, high output states
Best heard in left lateral decubitus at end-expiration
S4
Found in older adults
Stiff ventricle - always pathologic
Abnormal in children, indicates diastolic dysfunction - LVH
Dandy walker malformation
Absence of cerebellar vermis, cystic dilate of fourth ventricle, posterior fossa enlargement
Developmental delay and progressive skull enlargement
Rickets
Excess of unmineralized osteoid matrix and epiphyseal cartilage d/t Fit D deficiency
Frontal bossing, craniotabes, costochondral junction deformity, bowed legs
Thiazides
Indirectly cause hypercalcemia –> suppressed PTH
Between keratinocytes
Desmosomes - destruction = pemphigus vulgaris
Acantholysis - intraepithelial cleavage BETWEEN keratinocytes
Lithium
Nephorgenic DI
Lynch syndrome
Mismatch repair gene defect
Colorectal cancer
Ovarian cancer
Endometrial cancer
Tacriolomus and cyclosporine - immunosuppressants used to prevent transplant rejection and inflammatory disorders
Calcineurin inhibitors
Nephrotoxic
Metoclopramide
Contraindicated in parkinson’s - antagonist on dopamine receptors
Tissue ischemia
Dysfunction on Na/K ATPase –> K+ leakage from affected cells
Increase Na+ in cells –> swelling and explosion
Homocysteinuria
Marfanoid with intellectual disability, hypercoagulability, and downward lens dislocation
Water soluble vs. lipid soluble in placenta
Water soluble DON’T cross placenta - Heparin
Lipid soluble DO cross placenta - Warfarin
Hyperkalemia
Peaked T waves on ECG
Hypocalcemia
QT prolongation
Hypercalcemia
QT shortening, T-wave flattening or inversion in severe cases
Hypokalemia
QT prolongation, U-waves, and ST depression
Severe hypokalemia causes torsades
Tardive dyskinesia
up regulation of dopamine receptors
Dopamine beta hydroxylase
Converts dopamine to norepinephrine - necessary for sympathetic adrenergic activity
Fructose-2,6-biphosphate
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
CMV RETINITIS
CD4<50
Treat w/ ganciclovir
CKD
decreased phosphate excretion and vitamin D conversion –> hypocalcemia –> secondary hyperparathyroidism
CD18
Leukocyte adhesion deficiency
Anti-jo-1
Dermatomyositis
Polymyositis + skin findings
Dubin-johnson syndrome
conjugates chronic hyperbilirubinemia - no hemolysis
Zidovudine
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
Sheehan syndrome
ischemic necrosis of pituitary - pituitary grows w/o increased blood supply.
Zollinger-ellison
Ulcers, diarrhea, abdominal pain, acid reflux
MEN 1
Milrinone
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
Class IA antiarrhythmics
Quinidine, procainamide, disopyramide
Intermediate phase 0 depolarization
Prolonger AP
Class IB antiarrhythmics
Lidocaine, mexelitine
Weak phase 0 depoloraztion
Shortened AP duration
Class IC antiarrhythmics
Flecainide, propafenone
Strong phase 0 depoloraztaion
No change in AP duration
Elastase
Released by neutrophils or alveolar macrophages –> emphysema
Xeroderma pigementosa
AR
defective nucleotide excision repar - UV-specific endonuclease