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Flashcards in test #35 4.26 Deck (124):
1

calcineurin inhibitors

cyclosporin and tacrolimus

2

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

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

thyroid parafollicular cells arise from..

4th and 5th pharyngeal pouches

4

name development of pharyngeal pouches (5)

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

development of thyroid

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

6

pathophysiology of mutation in VHL

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

unregulated HIF --> increased VEGF & EPO

= hemangiomas

also associated w/ pheos & RCC

7

projection

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

identification

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

acting out

avoiding unacceptable feelings by behaving badly

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

10

most severe trisomy

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

11

describe trisomy 13-patau

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

47 XXY

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

13

47 XXX

clinically silent, maybe slightly decreased IQ

14

47 XYY

tall stature, acne, delays in motor and language

15

k-ras mutations common in..

pancreatic malignancy

16

when is opening snap of mitral stenosis head

really really close to closure of S2

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

17

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

inverse. shorter interval, more severe.

bc higher steady state left atrial pressure.

18

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

tincture of opium or paregoric

19

what maintains blood brain barrier

tight junctions [claudins & occludins]

between endothelial cells in capillary beds of CNS.

materials can only move transcellularly or carrier mediated.

20

why use naficillin usu

penicillinase-resistant penicillin.

use often against s. aureus.

skin infections and soft tissue infection -- folliculitis, abscesses.

21

rx for lung abscesses

usu clindamycin

'above the belt' anaerobes'

22

petechia, purpura, ecchymoses? blanch?

petechia 1cm

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

PPE

23

which blanch. telangietcasia or petechiae/purpura/ecchymoses?

teleangiectasia, bc in a vessel

24

intranasal glucocortoids localization

unlikely to cause systemic immunosuppression -- very localized

25

causes of candida vaginitis

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

2. contraception, high estrogen
3. systemic corticosteroids
4. uncontrolled diabetes
5. immunosuppression

26

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

normal: 3.8-4.2

gardnerella: pH > 4.2 (more basic)

trichomonas: pH >4.5 (more basic)

candida: pH normal, acidic

27

major bacterial flora of vagina

gram + lactobacilli

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

usu pH ~4

28

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

postmenopausal & lactating women

29

elevation of vaginal secretion pH suggests (pH > 4.5)

gardnerella or trichomonas

30

how are many carcinogens activated in humans

via microsomal monooxygenase (cytochrome p450 monooxygenease)

often convert procarcinogen -> carcinogen

like benzopyrene-induced lung cancer

31

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

impermeable: thick ascending loop

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

32

where are parietal cells located in gastric mucosa?

superficial gastric glands

33

where are chief cells located in gastric mucosa?

deep gastric glands

34

5 layers of gastric mucosa

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

fusobacterium, peptostreptococcus, bacteroides in lung..

aspiration pneumonia

alcoholic, seizure, severe neuro disease

36

3 ways to get lung abscess

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

where does blood accumulate in a subdural hematoma?

between dura mater and arachnoid. usu venous

gradual onset of symptoms.
crescent shaped
cortical briding veins

38

where does 2,3 DPG bind?

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

HgbA1c binding 2,3 DPG?

does not affect binding to 2,3 DPG

40

chlorpromazine associated w/

corneal deposits

41

haloperidol associated w/

extrapyramidal symptoms

42

ziprasidone associated w/

long QT

43

olanzepine associated w/

weight gain

44

clozapine associated w/

agranulocytosis and seizures

45

thioridazine associated with

retinal deposits that resemble retinitis pigmentosa

46

leading cause of blindness in industrial nations

macular degeneration

47

dry vs. wet age related macula degeneration?

dry: subretinal drusen or pigment changes

wet: abnormal blood vessels with subretinal fluid/hemorrhage, grey subretinal membrane, or neovascularization

48

progression of macular degeneration

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

49

rx for dry age related macular degeneration?

antioxidant vitamins and zinc

50

rx for wet age related macular degeneration

anti-VEGF, laser or phototherapy

(limit development of choroidal neovascular membranes)

intra-vitreous ranibizumab and pegaptanib

51

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

non small cell lung cancer

52

anti-IL2 for..

use for immunosupression for GVHD and organ transplant

53

anti-TNF-alpha for..

inflammatory autoimmune conditions: RA, IBD, seronegative spondyloarthropathies

54

infertility, sinusitis, bronchiectasis

kartageners (mutation in dyenin arm)

55

hypocalcemic tetany & recurrent viral and fungal infxn

digeorge 22q11.2 deletion

56

3 cardiac issues associated w/ marfans

all related to cystic medial necrosis

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

autosomal dominant, fibrillin

57

2 possible cardiac issues in digeorge

tetrology of fallot & aortic arch abnormalities

58

cardiac abnormality in friedrich's ataxia

hypertrophic cardiomyopathy

autosomal recessive:

59

mutation in friedrich's ataxia

autosomal recessive.

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

60

VIPoma

WDHA. "pancreatic cholera"

watery diarrhea, hypokalemia, achlorhydria

rx: somatostatin

61

VIP made by..

pancreatic islet cells, neurons of GI mucosa

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

62

who makes gastrin

G cells in stomach mucosa.
located in antrum.

63

glucagonoma may present w/

(1) secondary diabetes mellitus
(2) necrolytic migratory erythema of skin

64

optic tract fibers project to.. (4)

mainly 1. LGN
2. superior colliculus (reflex haze)
3. pretectal area (light reflex)
4. suprachiasmatic nucleus (circadian rhythm)

65

meyer's loop connects

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

66

UPPER fibers of optic radiation carry

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

67

what can stimulate glycogen utilization in muscles?

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

toxicity of amphotericin B

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

osler-weber-rendu

hereditary hemorrhagic telangiectasia

autosomal dominant

70

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

hereditary hemorrhagic telangiectasia

(osler-weber-rendu)

71

serum marker for heptocellular carcinoma

alpha-fetoprotein

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

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

72

CEA (carcinoembryonic antigen) is a marker for

colorectal cancer.

aid in staging, planning treatment, determining prognosis.

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

73

CA-125 marker..

marker for ovarian cancer

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

also benign: endometriosis, cirrhosis, PID

NOT a good screening tool.

74

PSA is a marker for..

extent of prostate cancer & evaluating response to treatment

commonly used to screen, but controversial

75

acid phosphatase marker for..

secondary prostate tumor marker antigen.

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

76

hCG is a marker for

gestational trophoblastic disease

77

sudden cardiac death defined by..

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

most common: ventricular fibrillation

78

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

ventricular fibrillation

79

when do ventricular mural thrombosis occur post MI

after at least 48 hrs in

80

when do ventricular rupture most commonly occur post MI?

3-7 days in

81

most common tumor in HIV+ patients

kaposi sarcoma

(HHV8 infects vascular & lymphatic endothelial cells)

82

presentation of kaposi sarcoma

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

histology of kaposi sarcoma

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

84

CMV in HIV+ patient causes (3)

1. esophagitis
2. colitis
3. retinitis

85

bilateral retinal hemorrhage & acute subdural in a baby?

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

ristocetin test

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

half life of factor VIII w/ vWF? without?

w/ vWF: 12 hours
w/o vWF: 2 hours

88

desmopressin in heme

releases vWF from endothelial cells

89

glanzmann thrombasthenia

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

tardive dyskinesia

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

atypical antipsychotic most likely to cause extrapyramidal symptoms? least?

most likely: risperidone
(also galactorhea and amenorrhea)

least: clozapine

92

acute dystonia. time, symptoms, rx?

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

parkinsonism (drug-induced)? rx?

between 4 days-4months of treatment.

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

rx: benztropine anticholinergic

94

akathisia

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

95

pH urease test for h. pylori

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

96

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

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

97

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

(1) ferment sorbitol
(2) produce glucoronidase

EXCEPT EHEC

98

which bacteria can activate adenylate cyclase (6)

-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

which toxins activate guanylate cyclase (2)

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

increase cGMP, watery diarrhea, electrolyte loss

100

which toxins inactivate EF-2 (2)

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

ADP ribosylation

101

which toxins disrupt cytoskeleton?

-c difficile

102

polyarteritis nodosa associated with..

hepatitis B

103

giant cell (temporal) arteritis associated w.

polymyalgia rheumatica

104

what is the most common vasculitis from antbiotic use?

microscopic polyangiitis / leukoclastic angiitis / hypersensitvity angiitis

type III immune rxn, often post penicillin

105

which vitamin enhances Fe2+ absorption

vitamin C / ascorbic acid!

bc keeps it in reduced form!

106

suppositories and first-pass metabolism?

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

major determinant of bioavailability after oral intake?

liver blood flow (first pass metabolism)

108

what constitutes first pass metabolism

reduction, oxidation, and hydrolysis.

in large part: cytochrome P450 system

109

what constitutes second pass metabolism

glucoronidation, acetylation, and sulfation

110

most common benign tumor in lung?

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

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

hamartoma aka pulmonary chondroma

112

bronchioloalveolar carcinoma on x-ray

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

myelofibrosis is a type of

chronic myeloproliferative disease

114

describe basis of myelofibrosis

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

myelofibrosis vs. aplastic anemia

myelofibrosis = massive splenomegaly, bc of extramedullary hematopoesis compensation

116

rx for myeleofibrosis

ruxolitinib, jak 2 inhibtor

117

myelodysplastic syndrome

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

myelopthisic anemia

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

most common infiltrator: metastatic carcinomas w/ associated fibrosis

119

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

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

bone marrow aspirate: dry

histopath:marrow replacement w/ fat cells and fibrous stroma

120

fanconi defective DNA repair causes..

aplastic anemia

121

cryoprecipitate can be used to treat..

coagulation deficiency of fibrinogen and factor VIII (hemophilia 8)

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

122

risks of blood transfusions (5)

-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

pseudo-pelger-huet anomaly

with myelodysplastic syndrome:

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

typically seen after chemotherapy

124

hairy cell leukemia can often present with? rx?

pancytopenia and splenomegaly

rx: cladribine (adenosine analogue resistant to adenosine deaminase)