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Step I > Barone > Flashcards

Barone Flashcards

1
Q

Micro vs macro cirrhosis of liver

A

3mm

alcohol is MCC of micro

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2
Q

UC

A

toxic megacolon -> sepsis -> death
continuous lesions
pseudopolyps (healing regions)

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3
Q

UC histo

A

superficial inflammation

crypt abscesses

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4
Q

UC markers

A

HLAB27
pANCA
anti-myeloperoxidase

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5
Q

Chrons

A

transumural inflammation -> strictures - string sign
skip lesions- cobblestone
creeping fat

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6
Q

Chrons histo

A

transmurral granulomatous inflammation

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7
Q

markers

A

NOD2
ASCA
Anti-saccharomyces cerevisiae Ab (brewers yeast - corona)

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8
Q

purple cyto

A

neutros
bacteria
Ca

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9
Q

pink cyto

A

proteins

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10
Q

cardiac myocytes

A

intercalated discs w/gap jnxs made up of kinexins

looks like little circles in a big cirle

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11
Q

Hemochromatosis

A

increased Fe
mutation in HFE
C282Y (Cys-> tyr)

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12
Q

what is toxic about Fe and Cu

A

Fenton Rx

tuns H202 -> OH radical

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13
Q

Tx for hemochromatosis

A

phlebotomy
chelation:
1st line- deferasirox (PO)
2nd line- deferoxamine (IV)

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14
Q

Tx for excess Cu

A

depencilimin

pennies are made of Cu

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15
Q

Prussian blue stain

A

Fe

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16
Q

congo red stain

A

amyloid

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17
Q

acid fast aka

A

kineyou
siehl-nelson
Fite

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18
Q

acid fast stain

A

TB
MAI
weak- nocardia

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19
Q

PAS stain

A 
stains sugar
glycogen storage diseases
glycoproteins (alpha 1 anti-trypsan def)
bacteria (whipples disease)
fungi (silver better)
pagets disease of nipple (mucus stains)
AML-M6
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20
Q

AML-M6

A

red cell form
googly elmo
erythroid leukemia
stains PAS

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21
Q

herpes viruses

A

all DNA
all double stranded (except parvo)
encapsulated by nuclear membrane like HPs invisibility cloak -> herpes is forever!

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22
Q

rabies infectious process

A

binds AChRs

Dynin retrograde transport

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23
Q

cyclophosphamide

A

Nitrogen mustard via p450 -> alkylates DNA -> kills cells

build up of acroline

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24
Q

acroline

A

hemorrhagic cystitis and transitional cell carcinoma

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25
Q

how do you prevent hemorrhagic cystitis

A

mesna should always be given w/cyclophosphamide

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26
Q

repetitive catalase + infections in kid

A

think chronic granulomatous disease

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27
Q

troponin

A

most specific marker of cell death

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28
Q

myoglobin

A

most sensitive marker of cell death

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29
Q

amylase

A

most sensitive marker of pancreatits

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30
Q

lypase

A

most specific marker of pancreatitis

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31
Q

how do you know if ALP is elevated d/t liver or bone?

A

if GGT elevated biliary

if GGT normal bone

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32
Q

cytotoxic T cells kill how

A

FasL binds FasR/CD95 on infected cell -> death domain -> caspase 8 -> caspases 3,6,7

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33
Q

p53

A

G1->S checkpoint by keeping RB hypophosphorylated

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34
Q

DNA strand breaks… what happens?

A

Sensed by ATM -> tell p53 to stop cycle and BRACA to fix it

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35
Q

ataxia telangetasia

A

mutated ATM

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36
Q

Lynch syndrome

A 
mutated MLH1/MSH2
CEO
colon
endometrial 
ovarian
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37
Q

excision endonuclease repair

A

sun damage

broken in xeroderma pigmentosa -> skin CA

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38
Q

if mutation cannot be fined

A

increased p53 -> Bax punches holes in outer membrane -> cytochrome C into cyto -> APAF1 -> caspase 9 -> caspase 3

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39
Q

BCL2

A

anti-apoptotic
plugs up BAX channels
follicular NHL turns on BCL2

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40
Q

follicular NHL

A 
b cell tumor (B cell markers)
14,18
IgH, BCL2
low grade, divides slowly
Px 10yrs
chemo or radiation do nothing
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41
Q

infliximab

A

Anti TNF

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42
Q

rituximab

A

anti CD20

check serology for HBV and HBC

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43
Q

kid born w/o thymic shadow

A

DiGeorge
SCIDs
HIV

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44
Q

adult w/thymic shadow

A

Thymoma
MG
pure red cell aplasia

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45
Q

viral hepatits

A

councilman bodies

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46
Q

concentric left ventricular hypertrophy

A

HTN

S4 gallop

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47
Q

PTEN mutation

A

chrom 10
prostate
follicular thyroid CA
endometrial CA

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48
Q

serous adenocarcinoma of endometrium

A

older women
usually papillary -> bad bc break off and seed
usually in ovary
worst ovarian CA
present w/adnexal mass w/ascites -> death sentence

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49
Q

pagets carcinoma

A

keratin +

PAS +

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50
Q

Melanoma

A

S100 (so are pheos and schwanomas)

HMB-45

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51
Q

Nieman pick

A

chrom 11

sphingomyelin -> zebra body lysosomes -> hepatosplenomegaly

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52
Q

lipofusion

A

accumulates in aging heart, brain, liver
perioxidized undigestible lipids
normal

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53
Q

BRAF mutations

A

nevi and melanoma

RAS pathway

54
Q

type of melanoma

A

nodular
acrolentiginous
superficial spreading
lentigo

55
Q

nodular melanoma

A

vertical growth
worst Px
breslow thickness

56
Q

acrolentiginous

A 
flat
2nd worse Px
dark skin pts (bob marley)
mutations in C-kit (tyrosine kinase)
Tx w/imatinib
57
Q

superficial spreading

A

MC

eventually becomes vertical growth and is too late to cure

58
Q

lentigo maligna

A 
freckle on steroids
hutchinson freckle
old 
face
best Px
59
Q

psamomma bodies

A

meningiomas
thyroid papillomas
papillary serous adeoma in ovaries
mesothelioma (shipbuilders)

60
Q

CML

A

9,22
BCR-ABL
TK, so Tx w/imatinib

61
Q

GIST

A

Tx with imatinib

62
Q

Dx of papillary thyroid CA

A

orphan annie eyes

63
Q

what chemo drugs are CI in restrictive lung disease

A

bleomycin

busulfan

64
Q

what antiarrhythmic is CI in restrictive lung dis

A

amioderone (Class III/K)

can also cause hypo/hyper thyroid and blue skin

65
Q

lymphedema

A

Tx w/compression

@ risk for lymphangiosarcoma

66
Q

non-pitting edema

A

myxedema from thyroid

lymphedema

67
Q

nutmeg liver

A

d/t right sided heart failure
Budd-chiari d/t blocked outflow of hepatic v
zone III

68
Q

what damages zone I of liver

A

HTN

viruses

69
Q

palpable purpura

A

small vessel vasculitis
henlock scholein purpura
microscopic angiopathic

70
Q

ITP

A 
spleen makes Abs against GpIb and/or GpIIb/IIIa on platelets
type II hypersensitivity 
spleen sequestered opsonized platelets
NO splenomeglay
Healthy young women or kids post viral 
secondary to SLE, HIV
high BT, normal pT/pTT
bone marrow lots of megakarycytes
71
Q

Tx of ITP

A

steroids
active bleeding -> IV IgG then platelets
rituximab (anti CD20)
splenectomy (almost everyone eventually)

72
Q

TTP

A

young female presents w/stroke
usually triggered by virus
pentad: CNS, fever, low platelets (stuck in brain), anemia, increased BUN/Cr

73
Q

Abs in TPP

A

ADAMTS13

usually inactivates vWF

74
Q

other signs of TTP

A

microangiopathic hemolytic anemia (schytocytes/helmet cells)
elevated BT
pT/pTT normal (unless in DIC)

75
Q

Tx of TTP

A

plasma exchange

76
Q

vWD

A 
superficial bleeding
MC familial bleeding disorder
platelet count normal
elevated BT
pT normal, pTT may be prolonged d/t concurrent factor VIII def
\+ ristocetin (adhesion disorder)
77
Q

how do you differentiate vWD from hemophilia

A

hemophilia normal BT
hemophilia males
low vWF Ag in vWD

78
Q

Tx of vWD

A

avoid aspirin

give desmopressin

79
Q

hemophilia A

A 
XR
missing factor 8
normal pT
elevated pTT
normal BT
normal platelet count
Male w/just an increased pTT
5x > then hemophilia B
often bleed at circumcision
easy bruising at 6mo+
bleeding in joints
80
Q

mixing studies

A

determines whether deficiency or inhibitor

81
Q

Tx of hemophilia A

A

mild bleeding- desmopressin (release factor 8 and vWF)

severe- give 8

82
Q

hemophilia B

A 
aka christmas disease/kings disease
deficiency in factor 9
prolonged pTT
normal count, pT, BT
mixing corrects
usually severe
XR
83
Q

hemophilia C

A 
factor 11 deficiency
very mild bleeding
AR
increased pTT
Tx with factor 11 or FFP
84
Q

factor 7 deficiency

A

normal pTT

abnormal pT

85
Q

vit k deficiency

A

new borns
C. diff
rat poison/warfarin overdoese

86
Q

liver disease and bleeding

A

stops making factors
7 effected first -> pT
looks like vit K deficiency

87
Q

warfarin

A

blocks vit K epoxide reductase
knocks out proteind C and S first -> hypercoag -> skin necrosis in those w/protein C deficiency
monitor w/pT

88
Q

heparin

A

activates anti thrombin III (factor 2)
also knocks out 12, 10, 9, 10
monitor w/pTT

89
Q

what if you Tx w/heparin and nothing happens

A

anti thrombin III deficiency

90
Q

which CA most likely to cause DIC

A

AML M3 (15,17)

91
Q

AML M3

A

(15,17)
PML,RAR
Tx/all trans retinoic acid
‘sexy young girl (15-17) driving sexy BMW M3, looking for sexy DIC to give her the (auer) rod, but she is in the wrong part of town and can only find ALL TRANS (ATRA) which will only give her the (zinc) finger

92
Q

auer rods

A

lysosomes -> TF -> coag

93
Q

brutons agammaglobulinemia

A 
x-linked
BTK (burtons tyrosine kinase) mutations
normal until 6mo 
histo- no germinal centers 
no gamma bumb
IVIG every month
94
Q

digeorger

A

3rd and 4th pharyngeal pouch
chacostek and trousous sign
no paraCx in nodes
TBX1 gene

95
Q

SCIDs

A 
IL-7 matures T cells, IL2 activates
SCIDS have ILR mutation on T cells
X linked
never give live vaccine 
BM transplant
96
Q

wiscott-aldrich

A 
X-linked
TIE:
Thrombocytopenia
infections
eczema
death- bleeding, infections, lymphoma
IgM low
IgA and E high
eventually become mentally challenged
97
Q

how do you tell TB and MAC appart

A

TB makes niacin

TB has porin factor and sulfatides

98
Q

what advantage does foscarnet have over gancyclovir?

A

it does not need to be activated by viral kinase

99
Q

periventricular calcifications

A

congenital CMV

100
Q

basal ganglia calcification

A

congenital toxo

101
Q

anti arrythmics

A 
No Better Pot then in Cali
Na
BB
K
Ca
102
Q

when do you shock an arrythmia

A

decreased BP
change in mental status
chest pain
severe SOB

103
Q

myocarditis w/eos

A

loefflers endomyocarditis

104
Q

right sided cardiac fibrosis

A

tricuspid regurg, pulmonic valve stenosis d/t carcinoid syndrome

105
Q

what vit is deficient in carcinoid syndrome

A

niacin, bc tryptophan used up making serotonin

DO NOT supplement tryptophan (restrict it) and supplement niacin

106
Q

Tx of carcinoid syndrome

A

surgery

somatostatin analog octreotide (tumor will take it up to ID tumor)

107
Q

tumor in heart that stains S100 and HMB-45

A

metastatic melanoma

108
Q

tumor in heart that stains cytokeratin (CK) 7

A

metastatic lung CA

109
Q

tumor in heart that stains CK 2D

A

metastatic colon CA

110
Q

increased unconjugated/indirect bilirubin

A

hemolytic anemai
physiologic jaundice of newborn
gilberts (very common)
crigler-najjar

111
Q

crigler-najjar tyep 1

A

fatal, w/o liver transplant
no UGT
phenobarbital -> nothing
confirm w/genetic testing UGT promoter

112
Q

crigler najjar type 2

A

too little UGT,
phenobarbital -> corrects
uncommon
UGT coding region

113
Q

increased conjugated/direct bilirubin

A

dubin-johnson
rotors
obstructive

114
Q

dubin-johnson

A

black liver
not bilirubin pigment from catecholamine build up
cannot transport conjugated bili out of liver

115
Q

rotors

A

normal color of liver

116
Q

which parasite causes obstructive jaundice

A

clonorchis sinensis

117
Q

primary biliary cirrhosis

A 
granulomas cause obstruction
only destroys bile ducts inside liver
xanthomas
fatigue
chirrosis 
common in SLE, RA
AMA abs (mito)
118
Q

primary sclerosing cholangitis

A 
unknown etiology
autoimmune
segmental inflammation and fibrosing of bile ducts inside and out of liver 
males 20-30
70% have UC
progresses to cholangiocarcinoma 
string of beads
119
Q

what cells lay down fibrosis in liver?

A

ITO cells, aka hepatic stellate cell

usually fat and vit A

120
Q

what signals fibrosis

A

TGF-beta

121
Q

trichome stain

A

think fibrosis
blue-fibrosis
red- regenerative nodules

122
Q

how do you Tx esophageal varices

A 
ocreotide to decrease portal HTN 
surgery
lactulose to bind proteins and pull them out to prevent hepatic encephalopathy 
give broad spectrum abx
prevent another w/BB
123
Q

hepatic encephalopathy

A

d/t ammonia build up

cannot eat protein

124
Q

HBV

A

ground glass cytoplasm

125
Q

HCV

A

fatty change

lymphoid aggregates

126
Q

amebic liver abscess

A

brown fluid, looks like chocolate

Tx w/metronidasole

127
Q

mallory bodies

A

swollen hepatocytes w/pink ropey stuff in alcoholic hepatitis

128
Q

wilsons disease

A

mutated ATP7B
Cu accumulates
atrophy of lenticular nucleus of basal ganglia
doesn’t present until 10+
chirrotic liver
Cu collects in descemets memebrane-> kayser fleischer rings
cogwheel ridgitiy, resting tremor, shuffling gate
mis Dx as parkinsons or ASHD

129
Q

Dx of wilsons

A

ceroplasmin low
high urine Cu
liver Bx shows Cu

130
Q

what level to horseshoe kidneys get stuck at?

A

IMA at L3

131
Q

ARPCKD

A

PKHD1- codes for fibrocystin
fatal near birth
radiating cysts

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