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zz - step 3 - uworld > uWorld 14 > Flashcards

uWorld 14 Flashcards

1
Q

what secondary changes can be seen in minimal change disease (and other nephrotic syndromes) that compensate for decreased plasma albumin concentration

A

liver increases synthesis of proteins, including LIPOPROTEINS (which is followed by lipiduria)

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2
Q

what happen to plasma aldosterone levels in nephrotic syndrome

A

increase (and thus sodium and water retention happen)

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3
Q

what is the most important source of protection in an Influenza A vaccine

A

humoral response- antibodies against hemagglutinin

can neutralize the virus and primarily block its binding to the host cells

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4
Q

what are the classical physical features of fragile X

A 
mental retardation (MCC of inherited intellectual disability)
macrocephaly, long narrow face, prominent forehead, jaw, chin ears, machroorchidism

due to CGG repeat in the fragile X mental retardation 1 (FMR1) gene on the long arm of ch X

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5
Q

what is the structure of fetal hemoglobin

A

alpha2gamma2

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6
Q

what is given to patients with aspirin allergies to prevent cardiovascular events

A

clopidogrel (irreversilby blocks P2Y12 component of ADP receptors on platelet surface)

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7
Q

what is cilostazol

A

PDE inhibitor that is occasionally used in pts with symptomatic peripheral vascular disease (claudication)

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8
Q

what is eptifibatide

A

platelet glycoprotein IIb/IIIa inhibitor that inhibits the final common pathway of lately aggregation
occasionally used in some patients with acute coronary syndrome, but it is not used for patients with stable CHD

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9
Q

what drugs can induce parkinsonism

A

antipsychotics (first generation more than second)

antiemetics/gastric motility agents (prochlorperazine, metoclopramide)

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10
Q

verapamil can be used for its vasodilatory properties and also affects cardiac contractility but has minimal effect on skeletal muscle. Which of the following properties of skeletal muscle is responsible for this resistance of the medication?

A

No dependence on extracellular calcium influx

cardiac myocytes depolarization causes L-type calcium channels on the plasma membrane to open and allow influx of extracellular calcium- this then ind to and activates RyR2 channels inducing release of calcium from the SR (CALCIUM-INDUCED CALCIUM RELEASE)
-smooth muscle is similar however calcium-calmodulin rather than calcium-troponin (like in cardiac) complex facilitates the interaction b.w actin and myosin

in skeletal muscle L-type calcium channels directly interact with RyR1 calcium channels to cause release of calcium from SR- there is NO significant influx of calcium across the plasma membrane L-type channel- PHYSICAL INTERACTION b/w the 2 channels that triggers sarcoplasmic calcium release

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11
Q

an elaborate T-tuble system is found in what types of muscle

A

skeletal and cardiac muscle but NOT smooth
T-tubles are a communication of the extracellular space that permits a more paid spread of depolarization throughout skeletal and cardiac muscles

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12
Q

what muscle cells depend on extracellular calcium influx into the cells via L-type calcium channels for excitation-contraction coupling

A

cardiac and smooth muscle NOT skeletal

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13
Q

why is skeletal muscle resistant to the effect of calcium channel blockers

A

does not require an initial influx of extracellular calcium for excitation-contraction coupling

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14
Q

pulmonary capillary wedge pressure is a measure of what and an isolated increase in it represents what

A

left atrial pressure and left end-diastolic pressure
increase in PCWP can represent MITRAL STENOSIS from increase in left atrial pressure that is reflected to the pulmonary veins

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15
Q

where is majority of water absorbed in the kidneys no matter what the hydration status is

A

PROXIMAL TUBULES REABSORB over 60% of water filtered by the glomeruli

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16
Q

how is the release of prolactin controlled

A

inhibitory control by dopamine secretion from the HYPOTHALAMUS
disruption of dopaminergic pathways or blockade of dopamine D2 receptors can cause hyperprolactinemia

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17
Q

what two branches of the external iliac artery branch before the inguinal ligament

A

INFERIOR EPIGASTRIC ARTERY

deep circumflex iliac

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18
Q

what is pantoprazole

A

proton pump inhibitor

used to great GERD

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19
Q

what is seen histologically in GERD

A

basal zone hyperplasia
lamina propria papillae
scattered eosinophilia and neutrophils
NOCTURNAL COUGH

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20
Q

eosinophilic esophagitis

A

an immune/antigen-mediated disease that typically presents with dysphagia and good impaction in atopic adults
characterized histologically by NUMEROUS superficially located intraepithelial eosinophils, which and hep differentiate it from reflux esophagitis
PPIs usually doe not work

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21
Q

what causes pill-induced esophagitis

A

tetracylcine antibiotics
potassium chloride
bisphosphonates

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22
Q

what is the most common malignant brain tumor of childhood

A

medulloblastoma (typically arises in cerebellum)

23
Q

patients with what are at increased risk of developing ALL

A

Down syndrome
ataxia-telangiectasia
NF1

24
Q

because the pentose phosphate pathways is the MAIN SOURCE of NADPH, the pathway is particularly active in:

A

cells experiencing OXIDATIVE STRESS (erythrocytes), where NADPH is used to REGENERATE GLUTATHIONE
organs (liver, adrenals) involved in REDUCTIVE BIOSYNTHESIS (FATTY ACIDS, CHOLESTEROL, STEROIDS) and cytochrome P450 metabolism
phagocytic cells generating a respiratory burst (NADPH oxidase)

25
Q

an increase in mean corpuscular hemoglobin concentration (MCHC) and loss of central pallor is seen in what disease

A

hereditary spherocytosis

acquired spherocytosis- MCC is autoimmune hemolytic anemia

26
Q

what is haptoglobin

A

acute phase reactant that combines with free hemoglobin in the citation to preserve body iron stores and prevent tissue damage
haptoglobin levels decrease in any form of hemolytic anemia (even with extravascular hemolysis, as some free hemoglobin will spill into the blood stream)

27
Q

what are the skeletal findings in hyperparathyroidism

A

most commonly involve the CORTICAL (COMPACT) BONE) in the appendicular skeleton (the pectoral girdle, pelvic girdle, and limbs)
SUBPERIOSTEAL THINNING is a characteristic feature and appears radiologically as MUBPEROSTEAL EROSIONS in flanges of the hand, and a granular “SALT AND PEPPER” SKULL, and osteolytic cysts in the long bones (OSTEITIS FIBROSIS CYSTICA)

28
Q

trabecular thinning with fewer interconnections is characteristic of what bone disease

A

OSTEOPOROSIS

29
Q

osteoid matrix accumulation around trabeulae is seen in what bone disease

A

vitamin D DEFICIECNY
also see excessive un-mineralized osteoid with widened osteoid seams
patients typically have low urine calcium

30
Q

persistence of the primary spongiosa in the medullary cavity with no mature trabeculae is a classic finding int hat

A

OSTEOPETROSIS (“marble bone disease”)
caused by decreased osteoclastic bone resorption, which resulting in accumulation of woven bone and diffuse skeletal thickening

31
Q

what is the most common skeletal manifestation of primary hyperparathyroidism

A

OSTEITIS FIBROSIS CYSITCA
bone pain, subperiosteal erosions affecting the phalanges of the hand, and a “salt and pepper” skull, and BROWN TUMOR bone CYSTS

32
Q

intimal streaks are the earlier lesion of what

A

atherosclerosis

33
Q

aortic regurgitation in the presence of mediastinal widening suggests what

A

thoracic aortic aneurysm

34
Q

what ANTIOXIDANT ENZYMES convert ROS to oxygen and water, neutralizing their capacity for cellular damage

A

SUERPOXIDE DISMUTASE
GLUTATHIONE PEROXIDASE
CATALASE

35
Q

what is myeloperoxidase

A

found in neutrophils
converts hydrogen peroxide to hypochlorous acid (a bactericidal compound that also causes oxidative damage to host cells)

36
Q

what is cytochrome oxidase

A

final component of the ETC and functions to cover molecular oxygen to water while establishing the proton gradient necessary for synthesis of ATP

37
Q

what is the inheritance of PKU

A

AR

38
Q

GPIIb/IIIa is either deficient or defective in patients with

A

Glanzmann thrombasthenia

39
Q

what is seen on EM and immunoflourescen of PSGN

A

her cellular glomeruli fue to leukocyte infiltration (neutrophils and monocytes) and mesangial and endothelial cell proliferation
granular deposits of IgG, IgM, and C3
SUBEPITHELIAL HUMPS on EM

40
Q

sub endothelial C1q deposits are characteristic of what renal disease

A

type 1 membranoproliferative GN

41
Q

IgE deposits are sometimes seen in what renal disease

A

lupus nephritis
confined to the capillary wall
associated with a poorer prognosis

42
Q

what are the clinical features of restless leg syndrome

A

uncomfortable urge to move the legs:
unpleasant sensation in the legs
onset with inactivity or at NIGHT
RELEIF with MOVEMENT (walking, stretching)

43
Q

what are the causes of restless leg syndrome

A 
idiopathic
IRON DEFICIENCY 
uremia
diabetes (esp w/ neuropathy)
multiple sclerosis, Parkinson disease
Drugs (antidepressants, metoclopramide)
44
Q

what is the treatment of restless leg syndrome

A 
avoidance of aggravating facts (alcohol, sleep deprivation)
supportive measures: leg massage, exercise, heating pads
DOPAMINE AGONISTS (PRAMIPEXOLE)
45
Q

what drug is used to treat restless leg syndrome

A

PRAMIPEXOLE (dopamine agonist) or ropinirole

46
Q

patients who have overdosed on beta blockers should be treated with what

A

glucagon (increases heart rate and contractility independent of adrenergic receptors)

47
Q

what is Osgod-Schlatter disease (OSD)

A

an overuse injury of the SECONDARY OSSIFICATION CENTER (APOPHYSIS) of the TIBIAL TUBERCLE
very common cause of knee pain in young ADOLESCENTATHLETES after a recent growth spurt

presents as in and selling at the TIBIAL TUBERCLE, the insertion of the PATELLAR LIGAMENT (connects the tibia to the patella)

REPETATIVE QUADRICEPTS CONTRACTION (jumping) and CHRONIC AVULSION cause the proximal patellar tendon to separate fro the TIBIAL TUBERCLE

48
Q

what does the COPPER REDUCTION TEST detect

A

presence of a REDUCING SUGAR (like FRUCTOSE, glucose, and galactose)

urine dipstick only positive if glucose present (not galactose or fructose)

49
Q

a deficiency in acid alpha-glucosidase (aka acid maltase) causes what

A

glycogen storage disease type II (POMPE DISEASE)

affected infants have cardiomyopathy, muscle weakness, and hypotonia

50
Q

what is essential in making the diagnosis of THROMBOTIC THROMBOCYTOPENIA PURPURA

A

microangiopathic HEMOLYTIC ANEMIA (↑ LDH, ↓ haptoglobin) with SCHISTOCYTES
THROMBOCYTOPENIA (↑ bleeding time, normal PT/PTT)

Tx: PLASMA EXCHANGE if life-saving and

51
Q

in pregnancy DIC is mediated by what

A

TISSUE FACTOR (THOMBOPLASTIN)

52
Q

what is seen on light microscopy of craniopharyngioma

A

cysts lined by cords/nests of stratified squamous epithelium with peripheral palisading and internal areas of lamellar “WET” KERATIN

53
Q

what is seen in a cranipharyngioma

A

cystic or partially cystic with solid areas
cysts are filled with a brownish-yellow, viscous fluid that resembles machine oil due to the presence f protein and CHOLESTEROL CRYSTALS
DYSTROPHIC CALCIFICATION of the cysts is highly characteristic and may be detected on neuroimaging

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