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Flashcards in Barone Deck (131):
1

Micro vs macro cirrhosis of liver

3mm
alcohol is MCC of micro

2

UC

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

3

UC histo

superficial inflammation
crypt abscesses

4

UC markers

HLAB27
pANCA
anti-myeloperoxidase

5

Chrons

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

6

Chrons histo

transmurral granulomatous inflammation

7

markers

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

8

purple cyto

neutros
bacteria
Ca

9

pink cyto

proteins

10

cardiac myocytes

intercalated discs w/gap jnxs made up of kinexins
looks like little circles in a big cirle

11

Hemochromatosis

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

12

what is toxic about Fe and Cu

Fenton Rx
tuns H202 -> OH radical

13

Tx for hemochromatosis

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

14

Tx for excess Cu

depencilimin
pennies are made of Cu

15

Prussian blue stain

Fe

16

congo red stain

amyloid

17

acid fast aka

kineyou
siehl-nelson
Fite

18

acid fast stain

TB
MAI
weak- nocardia

19

PAS stain

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

20

AML-M6

red cell form
googly elmo
erythroid leukemia
stains PAS

21

herpes viruses

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

22

rabies infectious process

binds AChRs
Dynin retrograde transport

23

cyclophosphamide

Nitrogen mustard via p450 -> alkylates DNA -> kills cells
build up of acroline

24

acroline

hemorrhagic cystitis and transitional cell carcinoma

25

how do you prevent hemorrhagic cystitis

mesna should always be given w/cyclophosphamide

26

repetitive catalase + infections in kid

think chronic granulomatous disease

27

troponin

most specific marker of cell death

28

myoglobin

most sensitive marker of cell death

29

amylase

most sensitive marker of pancreatits

30

lypase

most specific marker of pancreatitis

31

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

if GGT elevated biliary
if GGT normal bone

32

cytotoxic T cells kill how

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

33

p53

G1->S checkpoint by keeping RB hypophosphorylated

34

DNA strand breaks... what happens?

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

35

ataxia telangetasia

mutated ATM

36

Lynch syndrome

mutated MLH1/MSH2
CEO
colon
endometrial
ovarian

37

excision endonuclease repair

sun damage
broken in xeroderma pigmentosa -> skin CA

38

if mutation cannot be fined

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

39

BCL2

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

40

follicular NHL

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

41

infliximab

Anti TNF

42

rituximab

anti CD20
check serology for HBV and HBC

43

kid born w/o thymic shadow

DiGeorge
SCIDs
HIV

44

adult w/thymic shadow

Thymoma
MG
pure red cell aplasia

45

viral hepatits

councilman bodies

46

concentric left ventricular hypertrophy

HTN
S4 gallop

47

PTEN mutation

chrom 10
prostate
follicular thyroid CA
endometrial CA

48

serous adenocarcinoma of endometrium

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

49

pagets carcinoma

keratin +
PAS +

50

Melanoma

S100 (so are pheos and schwanomas)
HMB-45

51

Nieman pick

chrom 11
sphingomyelin -> zebra body lysosomes -> hepatosplenomegaly

52

lipofusion

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

53

BRAF mutations

nevi and melanoma
RAS pathway

54

type of melanoma

nodular
acrolentiginous
superficial spreading
lentigo

55

nodular melanoma

vertical growth
worst Px
breslow thickness

56

acrolentiginous

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

57

superficial spreading

MC
eventually becomes vertical growth and is too late to cure

58

lentigo maligna

freckle on steroids
hutchinson freckle
old
face
best Px

59

psamomma bodies

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

60

CML

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

61

GIST

Tx with imatinib

62

Dx of papillary thyroid CA

orphan annie eyes

63

what chemo drugs are CI in restrictive lung disease

bleomycin
busulfan

64

what antiarrhythmic is CI in restrictive lung dis

amioderone (Class III/K)
can also cause hypo/hyper thyroid and blue skin

65

lymphedema

Tx w/compression
@ risk for lymphangiosarcoma

66

non-pitting edema

myxedema from thyroid
lymphedema

67

nutmeg liver

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

68

what damages zone I of liver

HTN
viruses

69

palpable purpura

small vessel vasculitis
henlock scholein purpura
microscopic angiopathic

70

ITP

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

Tx of ITP

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

72

TTP

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

73

Abs in TPP

ADAMTS13
usually inactivates vWF

74

other signs of TTP

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

75

Tx of TTP

plasma exchange

76

vWD

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

how do you differentiate vWD from hemophilia

hemophilia normal BT
hemophilia males
low vWF Ag in vWD

78

Tx of vWD

avoid aspirin
give desmopressin

79

hemophilia 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

mixing studies

determines whether deficiency or inhibitor

81

Tx of hemophilia A

mild bleeding- desmopressin (release factor 8 and vWF)
severe- give 8

82

hemophilia B

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

83

hemophilia C

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

84

factor 7 deficiency

normal pTT
abnormal pT

85

vit k deficiency

new borns
C. diff
rat poison/warfarin overdoese

86

liver disease and bleeding

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

87

warfarin

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

heparin

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

89

what if you Tx w/heparin and nothing happens

anti thrombin III deficiency

90

which CA most likely to cause DIC

AML M3 (15,17)

91

AML M3

(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

auer rods

lysosomes -> TF -> coag

93

brutons agammaglobulinemia

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

94

digeorger

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

95

SCIDs

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

96

wiscott-aldrich

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

97

how do you tell TB and MAC appart

TB makes niacin
TB has porin factor and sulfatides

98

what advantage does foscarnet have over gancyclovir?

it does not need to be activated by viral kinase

99

periventricular calcifications

congenital CMV

100

basal ganglia calcification

congenital toxo

101

anti arrythmics

No Better Pot then in Cali
Na
BB
K
Ca

102

when do you shock an arrythmia

decreased BP
change in mental status
chest pain
severe SOB

103

myocarditis w/eos

loefflers endomyocarditis

104

right sided cardiac fibrosis

tricuspid regurg, pulmonic valve stenosis d/t carcinoid syndrome

105

what vit is deficient in carcinoid syndrome

niacin, bc tryptophan used up making serotonin
DO NOT supplement tryptophan (restrict it) and supplement niacin

106

Tx of carcinoid syndrome

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

107

tumor in heart that stains S100 and HMB-45

metastatic melanoma

108

tumor in heart that stains cytokeratin (CK) 7

metastatic lung CA

109

tumor in heart that stains CK 2D

metastatic colon CA

110

increased unconjugated/indirect bilirubin

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

111

crigler-najjar tyep 1

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

112

crigler najjar type 2

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

113

increased conjugated/direct bilirubin

dubin-johnson
rotors
obstructive

114

dubin-johnson

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

115

rotors

normal color of liver

116

which parasite causes obstructive jaundice

clonorchis sinensis

117

primary biliary cirrhosis

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

118

primary sclerosing cholangitis

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

what cells lay down fibrosis in liver?

ITO cells, aka hepatic stellate cell
usually fat and vit A

120

what signals fibrosis

TGF-beta

121

trichome stain

think fibrosis
blue-fibrosis
red- regenerative nodules

122

how do you Tx esophageal varices

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

hepatic encephalopathy

d/t ammonia build up
cannot eat protein

124

HBV

ground glass cytoplasm

125

HCV

fatty change
lymphoid aggregates

126

amebic liver abscess

brown fluid, looks like chocolate
Tx w/metronidasole

127

mallory bodies

swollen hepatocytes w/pink ropey stuff in alcoholic hepatitis

128

wilsons disease

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

Dx of wilsons

ceroplasmin low
high urine Cu
liver Bx shows Cu

130

what level to horseshoe kidneys get stuck at?

IMA at L3

131

ARPCKD

PKHD1- codes for fibrocystin
fatal near birth
radiating cysts