uworld assessment block #4 4.15 Flashcards Preview

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Flashcards in uworld assessment block #4 4.15 Deck (109):
1

infectious arthritis causes? presentation

most common (1) n. gonorrhea (disseminated). can also be (2) s. aureus (2) streptococcus

STD triad: synovitis (arthralgia, knee), tenosynovitis (hand), dermatitis

(note, no eye symptoms. compared to reiter's -- conjunctivitis)

2

differentiate infectious arthritis from reactive arthritis 'reiter's'

infectious agents:

infectious arthritis: gonorrhea, s. aureus, strep
reactive arthritis: nongonococcual urethritis (chlamydia) and GI bugs (campylobacter, salmonella, shigella, yersinia)

eye involvement:
infectious: tenosynovitis, synovitis, dermatitis
reactive: conjunctivitis, seroneg arthritis, urethritis

3

viral arthritis

symmetric polyarticular disease. associated w/ parvovirus, hep B, hep C, rubella, alphavirus

4

prader-willi presentation

neonatal hypotonia, extreme hyperphagia, morbid obesity, ultimately DM type II, short, small hands & feet, hypogonadism, characteristic facies.

5

pathophysiology of prader-willi

imprinting.
normal: mom's gene 15q11 region SILENT. express ONLY PAPA.

disease:
(1) delete papa's gene
(2) 25% uniparental disomy (only inherit mom's imprinted gene)

would detect microdeltion of papa's chr 15 on FISH. if FISH is normal = uniparental disomy.

6

pathophysiology of angelman's syndrome

imprinting
normal: papa's gene is normally SILENT. express ONLY MOM.

disease:
(1) delete mom's gene
(2) 5% uniparental disomy. (only inherit papa's imprinted gene)

7

presentation of angelman's syndrome

inappropriate laughter "happy puppet", seizure, ataxia, severe intellectual disability

8

paracentric inversions

inversions of chromosomal DNA in one arm of chromosome not including centromere

9

GI presentation of celiac's disease

(1) blunting of villi (2) lymphocytes in lamina propria (3) hyperplasia of crypts

CD8+ T cells infiltrate surface epithelilum, while CD4+ in lamina propria.

distal duodenum

10

Ab found in celiac's disease

anti-transglutaminase, anti-gliadin, anti-endomysial

11

presentation of dermatitis herpatiformis? pathophysiology?

PRUITIC erythematous urticarial plaques on extensor surfaces of elbow, knee, buttocks, back.

caused by CROSS-REACTION of gliadin IgG and IgA with RETICULIN

--> component of fibrils that anchor the epidermal basement membrane to superficial dermis.

neutrophils at dermal tips --> microabscesses --> subepidermal blisters

12

normal thickness of right ventricle? left ventricle?

right: 3-4mm
left: 1 cm

13

layers of epidermis

corneum
lucidium
granulosum
spinosum
basale

14

describe stratum corneum

15-20 layers of dead squamous cells that lack nuclei.

generally thickest in regions exposed to friction (soles of feet)

15

callus

painless thickening of stratum corneum, at locations of repeated of external pressure / friction

16

essential characterestics of PCOS

amenorrhea, obesity, hirsuitism, virilization, peripheral insulin resistance, dyslipidemia

17

ovaries in PCOS

bilateral enlargement and smooth thickened capsules.

subcapsular follicles in diff stages of atresia.

hyperplastic theca stroma cells rim arrested follicles

18

hormone imbalance in PCOS

elevation in everything EXCEPT FSH

think: high LH makes theca cells produce more androgens.

FSH is diminished, so granulosa cells can't make as much estrogen.

19

lesch-nyhan inheritance? presentation?

X-linked recessive.

self-mutilating behavior, mild mental retardation, involuntary movement, delayed motor development, nephrolithiasis, gout

FROST on skin under diaper --> hyperuricuria

rx: allopurinol

20

HGPRT fxn

purine salvage.
convert hypoxanthine and guanine to inosine monophosphate (IMP) and guanine mono phosphate (GMP).

absence: increased purine synthesis, hyperuricemia, hyperuricuria

21

ADA deficiency

impt for purine catabolism. deficiency: build up dATP, inhibit ribonucleotide redutase, inhibit DNA synthesis.

22

adenine phosphoribosyltransferase (APRT)

involved in purine salvage. converts adenine to AMP.

deficiency: hyperuricemia and adenine stone urolithiasis.

autosomal recessive. no neurological symptoms

23

erythromyocin drug effect

block translocation.

prevent tRNA release from donor site after peptide bond formation. 50S.

24

chloramphenicol drug effect

bind to 50S

block peptidyltransferase action

25

puromycin

aminonucleoside, structure analogus to aminoacyl-tRNA.

leads to premature release of unfinished polypeptide chains and polypeptidyl-puromycin derivatives.

26

differentiation of a cell primarily depends on

transcription factor: favors expression of only those genes required for cell to perform function.

specific for each individual tissue.

27

cytokine vs. growth factor

growth factor = type of cytokine. stimulate GROWTH and DIFFERENTIATION of various lines. regulates synthesis of transcription factor.

28

timolol for glaucoma?

block b2 receptor mediated secretion of aqueous humor from ciliary epithelium

29

what GI hormone would be elevated in a patient with pernicious anemia?

GASTRIN. autoimmune destruction of parietal cells.

parietal cells secrete instrinic factor (bind to b12) AND HCl!

low HCl will prompt Gastrin secretion

30

pernicious anemia

results in atrophic gastritis w/ profound hypochloridia and increased serum gastrin levels

31

what leads to varicoele (left)

compression of renal vein between SMA and aorta

recumbent position allows blood to drain fine

32

spermatocele

cystic masses, arise from caput of epididymis. measuring between 2-5cm in diameter. usu do not cause symptoms

33

transillumination of varicoele

NEGATIVE. will not.

34

where does fluid accumulate in hydrocoele?

between parietal and visceral layers of tunica vaginalis

35

Peutz-Jegher's

rare, autosomal dominant. pigmented mucocutaneous macules and hamartomatous polyps in GI tract.

36

acanthos nigricans suggests (2)

underlying GI malignancy (gastric) and / or insulin resistance

37

neurofibromas that undergo malignant degeneration?

malignant peripheral nerve sheath tumors

38

rx after mechanical valve surgery (chronically)

warfarin (antagonize vitamin K-dependent gamma-carboxylation)

antiplatelets are not enough!

39

when are gbIIb/IIIa blockers used?

acute coronary syndrome

40

what origin is melanocytes

neural crest cell

41

at what stage to oocytes arrest in development

primary oocytes in prophase

42

cardiac problem seen in diGeorge?

truncus arteriosus, tetrology of fallot, interrupted aortic arch

43

3rd and 4th pharyngeal pouch important for

parathyroids, thymus, and ultimobranchial body

44

sinus venosus in utero

receives blood from vitelline vein, umbilical vein, and common cardinal vein

eventually forms coronary sinus, oblique vein of left atrium, and sinus venarum of right atrium

cardinal veins return blood from abdominal walls and wolffian bodies

45

which nucleotide is methylated in hypermethylated DNA

cytosine-guanine dinucleotide repeat sequences.

46

gaucher's disease presentation

hepatosplenomegaly. anemia, thrombocytopenia, bleeding, BONE PAIN and FRACTURES.

macrophages with crinkled-tissue paper appearance

47

enzyme deficiency in gaucher's disease

glucocerebrosidase.

increase in glucocerebroside.

48

most prevalent genetic disorder among ashkenazi jews

gaucher's disease

49

wrinkled-tissue paper in macrophage

gaucher's disease

50

built up product in fabry's

x-linked recessive. defect alpha-galactosidase. retention of ceramide trihexose

51

build up of ceramide trihexose in..

fabry's, defect in alpha-galactosidase

52

defect in arylsulfatase

metachromatic leukodystrophy

53

accumulation in metachromatic leukodystrophy

defect in arylsulfatase
cerebroside sulfate accumulates

peripheral neuropathy and motor skill difficulties

54

what is S-adenosylmethionine

SAM. produced by condensation of methionine with ATP.

functions as methyl group donor. involved in several reactions.

in adrenergic system: SAM important for conversion of NE to E by PNMT

55

rx against TNF-alpha (etanercept and infliximab) used for..

autoimmune processes like rheumatoid arthritis, crohn's, ankylosing spondylitis, psoriasis

56

tacrolimus

suppress T-lymphocyte activation by binding to FKBP-12. used in transplants

57

acute coronary syndrome characterized by...

occlusion of coronary vasculature, typically by thrombus, which arises secondary to increased platelet deposition & clotting factor activity at the site of a disrupted atherosclerotic plaque.

58

desired aspirin effect in stable angina?

block acute coronary syndrome (thrombus formation). block cox-1 (low dose aspirin)

limits platelet aggregation! (also affects GI system)

59

desired aspirin effect in inflammatory conditions (like rheumatoid arthritis)

block inflammation, cox-2!

selective cox-2 were generated b/c high dose also blocks cox-1 --> GI bleeds

60

aspergillus fungus histology

methenamine silver stain. septate hyphae out of which asexual fruiting structures known as conidiophores project.

conidiophores -- each have terminal vesicle w/ outwardly radiating phialides and condiospores attached -- giving 'broom like appearance'

generally, pts w/ history of TB may develop aspergillus in lung cavities previous formed by TB. fungus balls.

61

pneumocystis jiroveci on silver stain

cup or disc-shaped organisms.

62

examples of competent bacteria (able to undergo transformation)

haemophilus, streptococcus, bacillus, neisseria

63

FSGS associated w/ what demographic?

HIV infection, heroin addiction, sickle cell (black & hispanic)

64

in FSGC, describe the focally sclerosed parts (3)

basement membrane collapse, increased matrix production, hyalinosis

65

FSGS can progress to.. (esp with HIV-associated nephropathy)

collapsing glomerulonephropathy. usual FSGS lesions with collapse and sclerosis of whole glomerular tuft.

glomerular epithelial cells proliferate & hypertrophy. marked tubular injury (accompanying microcyst formation)

66

goodpasture's nephropathy is a form of...

RPGN

67

describe deposits in membranous glomerulonephropathy

diffuse glomerular capillary wall thickening w/ spike subepithelial IgG deposits

68

describe membranoproliferative glomerulonephritis

enlarged, hypercellular glomeruli w/ PMNs & monocytes.

increased mesangial cellularity --> capillary basement membrane thickening.

granular IgG and/or C3 deposits

69

postinfectious glomerulonephritis

diffuse hypercellularity (PMNs and monocytes)

diffuse granular deposits of IgG and C3 along glomerular capillary wall & mesangium.

70

IgA nephropathy

diffuse mesangian proliferation & expansion of ECM. granular IgA in mesangium & capillary walls

71

see collapsed lung w/

absence of lung markings along peripheral edge.

white visceral pleural line on chest radiograph.

heart & trachea moved away from affected side.

72

rx for collapsed lung (tension pneumothorax)

needle aspiration or chest tube placement (remove air from chest space)

73

homeobox genes

highly conserved set of genes (animals, fungi, plants).

code for protein TRANSCRIPTION FACTORS (homeodomain proteins).

GUIDE morphogenesis.
(mutation = structural defects)

modulate expression of other genes in cells. guide development from earliest stages of embryogenesis to final differentiation of cells.

74

HoxA-13 mutation

hand-foot-genital syndrome.

characterized by clinodactyly (curavture of finger), shortened thumbs, small feet, short great toes, urinary tract abnormalities. GI abnormalities: duplications in reproductive tact of women. hyposadias in men.

75

Pax-3 mutations

Waardenburg syndrome

76

Waardenburg syndrome

mutation in Pax-3.

heterochromia irides, poliosis, dystopia canthorum, sensorineural DEAFNESS.

dystopia canthorum: lateral displacement of inner canthorum of eye -- widened nasal bridge.

77

VHL mutation

predisposition to retinal, cerebellar, spinal hemangioblastomas.

renal cell carcinoma, pheochromocytoma, pancreatic tumor.

78

major effects of active vitamin D (1,25 dihydroxycholecaliferol)

- increased intestinal absorption of orally ingested Ca2+ and PO4-

- this lowers PTH

- enhanced bone resorption and release of bone Ca2+ and PO4- when serum levels of one of these are low

79

what contributes to serum anion gap? what is normal?

Na - (Cl + HCO3) = 12-16meq/ml

>30 = unaccounted acidic anions

80

sudden onset back pain, hematuria, oliguria, and anion gap metabolic acidosis

ethylene glycol posioning --> causes calcium oxalate crystals in renal tubules --> renal failure

81

basic steps of thermal cycling PCR

heating - DNA strand denature
cooling - primer hybridization
rewarming - primer extension & DNA synthesis

82

which facial sinus is most commonly affected during URI?

maxillary, bc drainage is located SUPERIOR to the floor of the sinus (gravity doesn't help)

83

drainage of ethmoid air cells

superior meatus

84

Virchow's node

left supraclavicular (between clavicle and trapezius).

receives drainage from thoracic duct -- samples lymph from
- all abdominal viscera
- viscera of left hemithorax
- all tissues inferior to umbilicus
- all left-sided tissues superior to umbilicus.

can be enlarged in abdominal malignancy, breast cancer, lung cancer, lymphoproliferative disorders

85

deep cervical lymph nodes drain

head & neck

86

axillary lymph nodes involved in..

classical metastatic breast cancer (could also go to parasternal nodes)

87

deltopectoral (infraclavicular) lymph nodes

participates in draining lymph from arm. groove between deltoid and pectoralis.

88

epitrochlear nodes

near elbow. drain lymph from ulnar aspect of forearm and hand.

enlarge w/ skin or soft-tissue infection distal to elbow.

89

circadian sleep rhythm sleep disorder

intrinsic abnormal suprachiasmatic nucleus. sleep/wake cycles off sync with time. i.e. want to go to bed at 3am and wake much later.

present: excessive daytime sleepiness during the school week in an otherwise health child (due to inability to attain needed amount of sleep when social demands require premature waking)

90

narcolepsy (3 features)

(1) daytime REM attacks and (2) cataplexy (sudden loss in antigravity muscle tone)

auditory or visual hallucinations of a hynagogic (upon falling asleep, more common) or hypnopompic (upon wakeing, less common) may occur

91

pathway of hormone secretion from suprachiasmatic nucleus

SCN --> NE --> pineal gland --> melatonin.

SCN regulated by light.

92

progression of EEG waves
awake (eyes open)
awake (eyes closed)
non-REM 1 (5%):
non-REM 2 (45%):
non-REM 3 (25%):

REM (25%):

BATS Drink Blood

awake (eyes open): beta
awake (eyes closed) alpha

non-REM 1: theta
non-REM 2: sleep spindles & k complex
non-REM 3: delta waves

REM: beta waves

93

in which stage of sleep does bed-wetting occur?

stage 3: delta waves. deepest

94

in which stage does teeth clenching (bruxism) occur?

stage 2: sleep spindles & k complexes. longest sleep

95

rx for narcolepsy

- daytime stimulants (amphetamines and modafinil)

- nightime sodium oxybate (GHB) GABA-b agonist

96

fatal familial insomnia

autosomal dominant prion disease. severe intractable insomnia and death

97

preload on contraction velocity

initial muscle fiber length prior to contractin.
amount of stretch placed on fiber before contraction occurs.

increasing preload increases contractile velocity up to a maximum value. beyond which --> actin/myosin becomes ineffient.

98

passive tension exerted by skeletal myocyte positively correlated with

resting length.

decrease in passive tension implies decrease in fiber length (decrease in maximal contraction velocity)

99

afterload on skeletal muscle contraction velocity

maximal velocity of contraction increases as afterload decreases

100

how does giardia look in GI system? what does it cause?

binucleate flagellated trophozite (looks like it's looking at you)

kite-like, owl's eyes

attaches to small bowel mucosa w/ ventral sucking disk.

does NOT damage or invade mucosa.

greasy, frothy, foul-smelling, nonbloody diarrhea. excessive flatulence.

malabsorption due to dysfunction of brush border enzymes.

usu camping / travel abroad

101

identification of entamoeba histolytica

serology or stool:

(1) trophozites w. RBCs in cytoplasm
(2) cysts w/ up to 4 nuclei

102

acute leukemia presents w/ (3)

PANCYTOPENIA:
anemia: fatigue
leukopenia: opportunistic infxn
thrombocytopenia: bleeding

103

3 cases where basophilic stipling is seem

ribosomal aggregates
1. alcohol abuse
2. lead poisoning
3. thalasemia

104

virulence factor for aspergillus

vascular invasion

105

aspergillus is associated w/

aflatoxin -- carcinogen related to hepatocellular carcinoma

106

felty syndrome

triad of (1) rheumatoid arthritis (2) splenomegaly (3) neutropenia

107

rx for esophageal variceal hemorrhage?

ocretotide (somatostatin analogue)

reduce splanchnic blood flow & divert blood from portal system to systemic circulation

indirectly induces splanchnic vasoconstriction via inhibition of glucagon and VIP (which vasodilate splanchnic vasculature)

also good bc doesn't cause systemic vasoconstriction

108

use of pentagastrin (2)

synthethetic gastric analogue:
(1) screen for carcinoid syndrome (stimulate 5HT secretion)
(2) screen for medullary carcinoma of thyroid (stimulate calcitonin secretion)

109

glucagon & VIP on splanchnic vasculature

vasodilation