test #35 4.26 Flashcards Preview

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1
Q

calcineurin inhibitors

A

cyclosporin and tacrolimus

2
Q

germline mutations in RET proto-onco gene affect cells of what origin

A

neural crest cells!

men2a: medullary thyroid carcinoma (parafollicular C cells of thyroid), pheo, and parathyroid gland
men2b: medullary thyroid carcinoma, pheo, and mucosal neuromas

3
Q

thyroid parafollicular cells arise from..

A

4th and 5th pharyngeal pouches

4
Q

name development of pharyngeal pouches (5)

A
  1. ears (middle ear, mastoid air cells, eustachian tube = endoderm lined)
  2. tonsils
  3. inferior parathyroid, thymus
  4. superior parathyroid, medullary thyroid parafollicular cell
  5. medullary thyroid parafollicular cells
5
Q

development of thyroid

A

thyroid follicular cells = outpouching of pharyngeal epithelium, migrate to lower neck & fuse with parafollicular C cells from 4/5th pharyngeal pouch.

6
Q

pathophysiology of mutation in VHL

A

VHL gene regulates transcription of HIF-1 (hypoxia inducible factor).

unregulated HIF –> increased VEGF & EPO

= hemangiomas

also associated w/ pheos & RCC

7
Q

projection

A

misattributing one’s own unconscious, undesired thoughts, feelings, impulses onto another person, who does NOT actually have them

occurs when a person ins unable to express difficult thoughts themselves.

transplant thoughts to another –> relieves difficult feelings without causing internal confluct of self-expression.

angry w/ parents, think parents mad at you.

lack insign and acknowledgement of their own motivations & feelings

8
Q

identification

A

modeling one’s behavior after someone perceived to be more powerful / prestigious.

may be admirable or non admirable.

child of abusive father becomes child abuser

9
Q

acting out

A

avoiding unacceptable feelings by behaving badly

expressing unwanted thoughts/impulses through action instead of reflection and mature discourse

10
Q

most severe trisomy

A

trisomy 13 – patau
usu die in first week
only 5% survive 6 months

11
Q

describe trisomy 13-patau

A

associated w/ early defect in prechordal mesoderm

midface, eye, forebrain most affected.

  1. head/neck: severe cleft lip/palate, microopthalmia or anopthalmis, coloboma, cylops, malformed/absent nose, deafness, scalp defects
  2. CNS: severe MR, microcephaly, holoprosencephaly, absent olfactory nerve or bulb, NT defects
  3. extremities: polydactyly, rocker-bottom feet
  4. renal: polycystic kidney disease
  5. GI: abdominal wall defects: omphalocele or umbilica hernia, pyloric stenosis
12
Q

47 XXY

A

klinefelter; tall male w/ gynecomastia, small testes, and infertility

13
Q

47 XXX

A

clinically silent, maybe slightly decreased IQ

14
Q

47 XYY

A

tall stature, acne, delays in motor and language

15
Q

k-ras mutations common in..

A

pancreatic malignancy

16
Q

when is opening snap of mitral stenosis head

A

really really close to closure of S2

due to tensing of MV leaflet after cusps completed their opening excursion

17
Q

relationship between time of A2-opening snap in mitral stenosis and severity

A

inverse. shorter interval, more severe.

bc higher steady state left atrial pressure.

18
Q

control symptoms of a neonate in opiod withdrawal (leaky baby)

A

tincture of opium or paregoric

19
Q

what maintains blood brain barrier

A

tight junctions [claudins & occludins]

between endothelial cells in capillary beds of CNS.

materials can only move transcellularly or carrier mediated.

20
Q

why use naficillin usu

A

penicillinase-resistant penicillin.

use often against s. aureus.

skin infections and soft tissue infection – folliculitis, abscesses.

21
Q

rx for lung abscesses

A

usu clindamycin

‘above the belt’ anaerobes’

22
Q

petechia, purpura, ecchymoses? blanch?

A

petechia 1cm

do not blanch, bc RBC are not in vasculature. RBC leaked in skin/subcutaneous tissue

PPE

23
Q

which blanch. telangietcasia or petechiae/purpura/ecchymoses?

A

teleangiectasia, bc in a vessel

24
Q

intranasal glucocortoids localization

A

unlikely to cause systemic immunosuppression – very localized

25
Q

causes of candida vaginitis

A

OFTEN:
1. ANTIBIOTIC use! suppress normal flora, facilitates candida overgrowth.

  1. contraception, high estrogen
  2. systemic corticosteroids
  3. uncontrolled diabetes
  4. immunosuppression
26
Q

normal pH of vagina? gardnerella infection? trichomonas? candida vulvovaginitis?

A

normal: 3.8-4.2
gardnerella: pH > 4.2 (more basic)
trichomonas: pH >4.5 (more basic)
candida: pH normal, acidic

27
Q

major bacterial flora of vagina

A

gram + lactobacilli

also: corynebacterium, fungi (candida), group B strep, e. coli

usu pH ~4

28
Q

decrease thickening of vaginal epithelium and decreased glycogen associated with..

A

postmenopausal & lactating women

29
Q

elevation of vaginal secretion pH suggests (pH > 4.5)

A

gardnerella or trichomonas

30
Q

how are many carcinogens activated in humans

A

via microsomal monooxygenase (cytochrome p450 monooxygenease)

often convert procarcinogen -> carcinogen

like benzopyrene-induced lung cancer

31
Q

which part of renal tubules is absolutely impermeable to water? which is low, but technically possible w/ ADH

A

impermeable: thick ascending loop

early DCT: very low permeability
late DCT: vary based on ADH

32
Q

where are parietal cells located in gastric mucosa?

A

superficial gastric glands

33
Q

where are chief cells located in gastric mucosa?

A

deep gastric glands

34
Q

5 layers of gastric mucosa

A
  1. simple columnar epithlieum
    - secrete mucus to protect
  2. superficial lamina propria
    - contains gastric pits
    (mult glands connect to single pit)
  3. superficial gastric gland
    - has parietal cells (oxyntic, pale, round, plate-like
  4. deep gastric gland
    - chief cells (pepsinogen) granular basophillic
  5. muscularis mucosa
    - separates lamina propria from submucosa

submucosa – vascularized CT

35
Q

fusobacterium, peptostreptococcus, bacteroides in lung..

A

aspiration pneumonia

alcoholic, seizure, severe neuro disease

36
Q

3 ways to get lung abscess

A
  1. aspiration of oropharyngeal contents: fusobacterium, peptostreptococcus, bacteroides
  2. complication of bacterial pneumonia: local immunosuppression, old oage, underlying chronic disease, necrotizing: s. aureus, e.coli, k. pneumonia, or s. pneumo type 3
  3. specticemia or infective endocarditis: hematogenous spread of infection – usu multiple. most common: staph or strep, e. coli, fungi.
37
Q

where does blood accumulate in a subdural hematoma?

A

between dura mater and arachnoid. usu venous

gradual onset of symptoms.
crescent shaped
cortical briding veins

38
Q

where does 2,3 DPG bind?

A

picket formed by 2 beta chains (+ charged amino acid, histidine, which attracts - charged phosphates on 2,3 DPG

(histidine absent in HbF, so it doesn’t bind)

39
Q

HgbA1c binding 2,3 DPG?

A

does not affect binding to 2,3 DPG

40
Q

chlorpromazine associated w/

A

corneal deposits

41
Q

haloperidol associated w/

A

extrapyramidal symptoms

42
Q

ziprasidone associated w/

A

long QT

43
Q

olanzepine associated w/

A

weight gain

44
Q

clozapine associated w/

A

agranulocytosis and seizures

45
Q

thioridazine associated with

A

retinal deposits that resemble retinitis pigmentosa

46
Q

leading cause of blindness in industrial nations

A

macular degeneration

47
Q

dry vs. wet age related macula degeneration?

A

dry: subretinal drusen or pigment changes
wet: abnormal blood vessels with subretinal fluid/hemorrhage, grey subretinal membrane, or neovascularization

48
Q

progression of macular degeneration

A

DRY (gradual vision loss in one or both eyes) usu progresses to WET (more common, acute vision loss over period of days-weeks)

49
Q

rx for dry age related macular degeneration?

A

antioxidant vitamins and zinc

50
Q

rx for wet age related macular degeneration

A

anti-VEGF, laser or phototherapy

(limit development of choroidal neovascular membranes)

intra-vitreous ranibizumab and pegaptanib

51
Q

epidermal growth factor receptor inhibitors (erlotinib & gefitinib) rx for?

A

non small cell lung cancer

52
Q

anti-IL2 for..

A

use for immunosupression for GVHD and organ transplant

53
Q

anti-TNF-alpha for..

A

inflammatory autoimmune conditions: RA, IBD, seronegative spondyloarthropathies

54
Q

infertility, sinusitis, bronchiectasis

A

kartageners (mutation in dyenin arm)

55
Q

hypocalcemic tetany & recurrent viral and fungal infxn

A

digeorge 22q11.2 deletion

56
Q

3 cardiac issues associated w/ marfans

A

all related to cystic medial necrosis

  1. dissection aortic aneurysm
  2. aortic valve incompetence
  3. mitral valve prolapse

autosomal dominant, fibrillin

57
Q

2 possible cardiac issues in digeorge

A

tetrology of fallot & aortic arch abnormalities

58
Q

cardiac abnormality in friedrich’s ataxia

A

hypertrophic cardiomyopathy

autosomal recessive:

59
Q

mutation in friedrich’s ataxia

A

autosomal recessive.

mutation in frataxin, mitochondrial protein impt in iron homeostasis & respiratory fxn

60
Q

VIPoma

A

WDHA. “pancreatic cholera”

watery diarrhea, hypokalemia, achlorhydria

rx: somatostatin

61
Q

VIP made by..

A

pancreatic islet cells, neurons of GI mucosa

relaxes smooth muscle, inhibits H+ secretion, stimulates pancreatic bicarb and Cl- secretion

62
Q

who makes gastrin

A

G cells in stomach mucosa.

located in antrum.

63
Q

glucagonoma may present w/

A

(1) secondary diabetes mellitus

(2) necrolytic migratory erythema of skin

64
Q

optic tract fibers project to.. (4)

A

mainly 1. LGN

  1. superior colliculus (reflex haze)
  2. pretectal area (light reflex)
  3. suprachiasmatic nucleus (circadian rhythm)
65
Q

meyer’s loop connects

A

LGN, through temporal lobe, LINGUAL gyrus of striate (info form lower retina, upper world)

66
Q

UPPER fibers of optic radiation carry

A

LGN, through parietal to CUNEUS gyrus of striate (info from upper retina, lower world)

67
Q

what can stimulate glycogen utilization in muscles?

A
  1. sustained Ca2+ ITSELF during contraction –> direct activation of phosphorylase kinase (activates glycogen phosphorylase)
  2. B1/glucagon-dependent increase in cAMP -> PKA ->
    - phosphorylates ‘phosphorylase kinase’ [activates it to phosphorylate glycogen phosphorylase]
    - & phosphorylates glycogen synthase to inactivate it.
68
Q

toxicity of amphotericin B

A

renal toxicity, hypomagnesium, and hypokalemia

(require daily supplementation)

renal toxicity: due to reduced GFR (renal vasoconstriction), directly toxic to epithelial cells, causing ATN, electrolyte disturbances

can lead to reduced EPO

69
Q

osler-weber-rendu

A

hereditary hemorrhagic telangiectasia

autosomal dominant

70
Q

telengiectasia on skin & mucus membranes w. rupture –> epistaxis, GI bleed, hematuria?

A

hereditary hemorrhagic telangiectasia

osler-weber-rendu

71
Q

serum marker for heptocellular carcinoma

A

alpha-fetoprotein

does NOT correlate well w/ size, stage, or prognosis.

also associated w/ pregnancy, gonadal tumors, chronic liver disease (viral hepatitis)

72
Q

CEA (carcinoembryonic antigen) is a marker for

A

colorectal cancer.

aid in staging, planning treatment, determining prognosis.

NOT a good screening tool, as it can be elevated w/ benign disease

73
Q

CA-125 marker..

A

marker for ovarian cancer

also elevated in: endometrial, lung, breast, pancreatic cancer

also benign: endometriosis, cirrhosis, PID

NOT a good screening tool.

74
Q

PSA is a marker for..

A

extent of prostate cancer & evaluating response to treatment

commonly used to screen, but controversial

75
Q

acid phosphatase marker for..

A

secondary prostate tumor marker antigen.

also elevated in patients w/ active osteoclast-indiated bone resorption

76
Q

hCG is a marker for

A

gestational trophoblastic disease

77
Q

sudden cardiac death defined by..

A

cardiac arrest that begins 1hr of a precipitating event that ultimately proves fatal.

most common: ventricular fibrillation

78
Q

most common cause of death in patients suffering from MI during prehospital phase (prior to arriving in ED)

A

ventricular fibrillation

79
Q

when do ventricular mural thrombosis occur post MI

A

after at least 48 hrs in

80
Q

when do ventricular rupture most commonly occur post MI?

A

3-7 days in

81
Q

most common tumor in HIV+ patients

A

kaposi sarcoma

HHV8 infects vascular & lymphatic endothelial cells

82
Q

presentation of kaposi sarcoma

A

multiple blue-violet or brownish dermal plaques, first appear on feet & legs before spreading proximally.

can develop on mucosal membranes of face and genitals.

can spread to lungs and GI tract

83
Q

histology of kaposi sarcoma

A

spindle and endothelial cell proliferation, red blood cell extravasation, inflammation

84
Q

CMV in HIV+ patient causes (3)

A
  1. esophagitis
  2. colitis
  3. retinitis
85
Q

bilateral retinal hemorrhage & acute subdural in a baby?

A

shaken baby syndrome, child abuse

infant has: larger head, larger subarachnoid space, higher brain water content, decreased cervical muscle tone

rupture of congested retinal veins

86
Q

ristocetin test

A

ristocetin activates GpIb receptors on platelets, makes available for vWF binding.

if vWF levels low, there is poor platelet aggregation in response to ristocetin

87
Q

half life of factor VIII w/ vWF? without?

A

w/ vWF: 12 hours

w/o vWF: 2 hours

88
Q

desmopressin in heme

A

releases vWF from endothelial cells

89
Q

glanzmann thrombasthenia

A

hereditary deficiency of GbIIbIIIa.

mucocutaneous bleeding and increased bleeding time.

platelet aggregation in response to ristocetin is fine (GbIb is fine). but decreased w/ addn of ADP (GbIIbIIIa messed up)

90
Q

tardive dyskinesia

A

involuntary perioral movements: biting, chewing, grimacing, tongue protrusions

involuntary choreoathetoid movt can also be seen

usu between 4 months-4years of treatment. usu irreversible

91
Q

atypical antipsychotic most likely to cause extrapyramidal symptoms? least?

A

most likely: risperidone
(also galactorhea and amenorrhea)

least: clozapine

92
Q

acute dystonia. time, symptoms, rx?

A

between 4hr-4days of antipsychotic:

muscle spasm / stiffness, tongue protrusions, opisthonus, oculogyric crisis (forced sustained elevation of eyes in upward position)

rx: antihistamine or anticholingerics

diphenhydramine, benztropine, trihyxyphenidyl

93
Q

parkinsonism (drug-induced)? rx?

A

between 4 days-4months of treatment.

cogwheel rigidity, masked facies, bradykinesis, pill-rolling, tremor, shuffling gait

rx: benztropine anticholinergic

94
Q

akathisia

A

subjective feeling of restlessness that compels patient to constantly move around.

95
Q

pH urease test for h. pylori

A

urea –> NH3 + CO2, pH increase, color change in phenol red.

96
Q

E. coli that does NOT ferment sorbitol and does NOT produce glucoronidase?

A

EHEC, which has shiga-like toxin that inactivates 60S ribosomal subunit –> prevents tRNA binding

97
Q

many e. coli (except EHEC) are able to..

A

(1) ferment sorbitol
(2) produce glucoronidase

EXCEPT EHEC

98
Q

which bacteria can activate adenylate cyclase (6)

A
  • b. pertussis (pertussis toxin)
  • b. anthracis (edema factor)
  • ETEC (heat labile toxin)
  • camplyobacteri jejuni (enterotoxin)
  • bacillus cereus (heat labile toxin
  • v. cholerae (choleragen toxin)

increase cAMP, decreased absorption, increased secretion

99
Q

which toxins activate guanylate cyclase (2)

A
  • ETEC (heat stabile toxin)
  • yersinia enerocolitica (y. enterocolitica enterotoxin)

increase cGMP, watery diarrhea, electrolyte loss

100
Q

which toxins inactivate EF-2 (2)

A
  • corynebacterium diptheriae (diptheria toxin)
  • pseudomonas aeruginosa (exotoxin A)

ADP ribosylation

101
Q

which toxins disrupt cytoskeleton?

A

-c difficile

102
Q

polyarteritis nodosa associated with..

A

hepatitis B

103
Q

giant cell (temporal) arteritis associated w.

A

polymyalgia rheumatica

104
Q

what is the most common vasculitis from antbiotic use?

A

microscopic polyangiitis / leukoclastic angiitis / hypersensitvity angiitis

type III immune rxn, often post penicillin

105
Q

which vitamin enhances Fe2+ absorption

A

vitamin C / ascorbic acid!

bc keeps it in reduced form!

106
Q

suppositories and first-pass metabolism?

A

rectum drained by superior, middle, and inferior rectal veins..

superior –> portal circulation via IMP

middle & inferior –> darin to systemic circulation via internal iliac and internal pudendal (respectively)

2/3 of suppositories –> systemic circulation

107
Q

major determinant of bioavailability after oral intake?

A

liver blood flow (first pass metabolism)

108
Q

what constitutes first pass metabolism

A

reduction, oxidation, and hydrolysis.

in large part: cytochrome P450 system

109
Q

what constitutes second pass metabolism

A

glucoronidation, acetylation, and sulfation

110
Q

most common benign tumor in lung?

A

hamartoma aka pulmonary chondroma

has mature hyaline cartilage, fat, smooth muscle & clefts lined by respiratory epithelium.

– excessive growth of a tissue type native to the organ of involvement

111
Q

well defined coin lesion w/ popcorn calcifications on x-rsy?

A

hamartoma aka pulmonary chondroma

112
Q

bronchioloalveolar carcinoma on x-ray

A

variant of adenocarcinoma.

almost always at lung periphery. characterestic distribution along alveolar septae w/o vascular or stroma invasion.

xray: peripheral mass or pneumonia-like consolidation

113
Q

myelofibrosis is a type of

A

chronic myeloproliferative disease

114
Q

describe basis of myelofibrosis

A

chronic myeloproliferation and atypical megakaryocyte hyperplasia.

clonally expanded megakaryocyte –> activate fibroblast–> progresive replacement of marrow space w/ collagen deposition.

early: marrow hypercellularity. later: fibrosis -> pancytopenia.

MASSIVE splenomegaly, because pt compensates w/ extramedullary hematopoesis

115
Q

myelofibrosis vs. aplastic anemia

A

myelofibrosis = massive splenomegaly, bc of extramedullary hematopoesis compensation

116
Q

rx for myeleofibrosis

A

ruxolitinib, jak 2 inhibtor

117
Q

myelodysplastic syndrome

A

stem cell disorders characterized by INEFFECTIVE HEMATOPOESIS and cell maturation defects.

may be associated pancytopenia.

bone marrow: disordered differentiation affected ALL NON-LYMPHOID (erythroid, granulocytic, monocytic, megakaryocytic) lines

findings in bone marrow: ringed sideroblasts and megalobastoid maturation.

could be: de novo mutation, environmental exposure to radiation, benzene, chemo)

risk of progression to AML

118
Q

myelopthisic anemia

A

anemia caused by space-occupying lesions in bone marrow. all hematopoetic series affected, pancytopenia expected.

most common infiltrator: metastatic carcinomas w/ associated fibrosis

119
Q

aplastic anemia (3 characterestics)? bone marrow? histopatholgoy?

A
  1. pancyotpenia
  2. low retic count
  3. absent splenomegaly

bone marrow aspirate: dry

histopath:marrow replacement w/ fat cells and fibrous stroma

120
Q

fanconi defective DNA repair causes..

A

aplastic anemia

121
Q

cryoprecipitate can be used to treat..

A

coagulation deficiency of fibrinogen and factor VIII (hemophilia 8)

has: fibrinogen, factor VIII, factor XIII, vWF and fibronectin

122
Q

risks of blood transfusions (5)

A
  • infection transmission (low)
  • iron overload
  • transfusion reaction (i.e. IgA deficiency, ABO mismatch)
  • hypocalcemia (due to citrate = calcium chelator)
  • hyperkalemia (RBC may lyse in old blood units
123
Q

pseudo-pelger-huet anomaly

A

with myelodysplastic syndrome:

neutrophils w/ bilobed nuclei (two nuclear masses) connected w/ a thin filament of chromatin.

typically seen after chemotherapy

124
Q

hairy cell leukemia can often present with? rx?

A

pancytopenia and splenomegaly

rx: cladribine (adenosine analogue resistant to adenosine deaminase)