Barone Flashcards
Micro vs macro cirrhosis of liver
3mm
alcohol is MCC of micro
UC
toxic megacolon -> sepsis -> death
continuous lesions
pseudopolyps (healing regions)
UC histo
superficial inflammation
crypt abscesses
UC markers
HLAB27
pANCA
anti-myeloperoxidase
Chrons
transumural inflammation -> strictures - string sign
skip lesions- cobblestone
creeping fat
Chrons histo
transmurral granulomatous inflammation
markers
NOD2
ASCA
Anti-saccharomyces cerevisiae Ab (brewers yeast - corona)
purple cyto
neutros
bacteria
Ca
pink cyto
proteins
cardiac myocytes
intercalated discs w/gap jnxs made up of kinexins
looks like little circles in a big cirle
Hemochromatosis
increased Fe
mutation in HFE
C282Y (Cys-> tyr)
what is toxic about Fe and Cu
Fenton Rx
tuns H202 -> OH radical
Tx for hemochromatosis
phlebotomy
chelation:
1st line- deferasirox (PO)
2nd line- deferoxamine (IV)
Tx for excess Cu
depencilimin
pennies are made of Cu
Prussian blue stain
Fe
congo red stain
amyloid
acid fast aka
kineyou
siehl-nelson
Fite
acid fast stain
TB
MAI
weak- nocardia
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
AML-M6
red cell form
googly elmo
erythroid leukemia
stains PAS
herpes viruses
all DNA
all double stranded (except parvo)
encapsulated by nuclear membrane like HPs invisibility cloak -> herpes is forever!
rabies infectious process
binds AChRs
Dynin retrograde transport
cyclophosphamide
Nitrogen mustard via p450 -> alkylates DNA -> kills cells
build up of acroline
acroline
hemorrhagic cystitis and transitional cell carcinoma
how do you prevent hemorrhagic cystitis
mesna should always be given w/cyclophosphamide
repetitive catalase + infections in kid
think chronic granulomatous disease
troponin
most specific marker of cell death
myoglobin
most sensitive marker of cell death
amylase
most sensitive marker of pancreatits
lypase
most specific marker of pancreatitis
how do you know if ALP is elevated d/t liver or bone?
if GGT elevated biliary
if GGT normal bone
cytotoxic T cells kill how
FasL binds FasR/CD95 on infected cell -> death domain -> caspase 8 -> caspases 3,6,7
p53
G1->S checkpoint by keeping RB hypophosphorylated
DNA strand breaks… what happens?
Sensed by ATM -> tell p53 to stop cycle and BRACA to fix it
ataxia telangetasia
mutated ATM
Lynch syndrome
mutated MLH1/MSH2 CEO colon endometrial ovarian
excision endonuclease repair
sun damage
broken in xeroderma pigmentosa -> skin CA
if mutation cannot be fined
increased p53 -> Bax punches holes in outer membrane -> cytochrome C into cyto -> APAF1 -> caspase 9 -> caspase 3
BCL2
anti-apoptotic
plugs up BAX channels
follicular NHL turns on BCL2
follicular NHL
b cell tumor (B cell markers) 14,18 IgH, BCL2 low grade, divides slowly Px 10yrs chemo or radiation do nothing
infliximab
Anti TNF
rituximab
anti CD20
check serology for HBV and HBC
kid born w/o thymic shadow
DiGeorge
SCIDs
HIV
adult w/thymic shadow
Thymoma
MG
pure red cell aplasia
viral hepatits
councilman bodies
concentric left ventricular hypertrophy
HTN
S4 gallop
PTEN mutation
chrom 10
prostate
follicular thyroid CA
endometrial CA
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
pagets carcinoma
keratin +
PAS +
Melanoma
S100 (so are pheos and schwanomas)
HMB-45
Nieman pick
chrom 11
sphingomyelin -> zebra body lysosomes -> hepatosplenomegaly
lipofusion
accumulates in aging heart, brain, liver
perioxidized undigestible lipids
normal
BRAF mutations
nevi and melanoma
RAS pathway
type of melanoma
nodular
acrolentiginous
superficial spreading
lentigo
nodular melanoma
vertical growth
worst Px
breslow thickness
acrolentiginous
flat 2nd worse Px dark skin pts (bob marley) mutations in C-kit (tyrosine kinase) Tx w/imatinib
superficial spreading
MC
eventually becomes vertical growth and is too late to cure
lentigo maligna
freckle on steroids hutchinson freckle old face best Px
psamomma bodies
meningiomas
thyroid papillomas
papillary serous adeoma in ovaries
mesothelioma (shipbuilders)
CML
9,22
BCR-ABL
TK, so Tx w/imatinib
GIST
Tx with imatinib
Dx of papillary thyroid CA
orphan annie eyes
what chemo drugs are CI in restrictive lung disease
bleomycin
busulfan
what antiarrhythmic is CI in restrictive lung dis
amioderone (Class III/K)
can also cause hypo/hyper thyroid and blue skin
lymphedema
Tx w/compression
@ risk for lymphangiosarcoma
non-pitting edema
myxedema from thyroid
lymphedema
nutmeg liver
d/t right sided heart failure
Budd-chiari d/t blocked outflow of hepatic v
zone III
what damages zone I of liver
HTN
viruses
palpable purpura
small vessel vasculitis
henlock scholein purpura
microscopic angiopathic
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
Tx of ITP
steroids
active bleeding -> IV IgG then platelets
rituximab (anti CD20)
splenectomy (almost everyone eventually)
TTP
young female presents w/stroke
usually triggered by virus
pentad: CNS, fever, low platelets (stuck in brain), anemia, increased BUN/Cr
Abs in TPP
ADAMTS13
usually inactivates vWF
other signs of TTP
microangiopathic hemolytic anemia (schytocytes/helmet cells)
elevated BT
pT/pTT normal (unless in DIC)
Tx of TTP
plasma exchange
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)
how do you differentiate vWD from hemophilia
hemophilia normal BT
hemophilia males
low vWF Ag in vWD
Tx of vWD
avoid aspirin
give desmopressin
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
mixing studies
determines whether deficiency or inhibitor
Tx of hemophilia A
mild bleeding- desmopressin (release factor 8 and vWF)
severe- give 8
hemophilia B
aka christmas disease/kings disease deficiency in factor 9 prolonged pTT normal count, pT, BT mixing corrects usually severe XR
hemophilia C
factor 11 deficiency very mild bleeding AR increased pTT Tx with factor 11 or FFP
factor 7 deficiency
normal pTT
abnormal pT
vit k deficiency
new borns
C. diff
rat poison/warfarin overdoese
liver disease and bleeding
stops making factors
7 effected first -> pT
looks like vit K deficiency
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
heparin
activates anti thrombin III (factor 2)
also knocks out 12, 10, 9, 10
monitor w/pTT
what if you Tx w/heparin and nothing happens
anti thrombin III deficiency
which CA most likely to cause DIC
AML M3 (15,17)
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
auer rods
lysosomes -> TF -> coag
brutons agammaglobulinemia
x-linked BTK (burtons tyrosine kinase) mutations normal until 6mo histo- no germinal centers no gamma bumb IVIG every month
digeorger
3rd and 4th pharyngeal pouch
chacostek and trousous sign
no paraCx in nodes
TBX1 gene
SCIDs
IL-7 matures T cells, IL2 activates SCIDS have ILR mutation on T cells X linked never give live vaccine BM transplant
wiscott-aldrich
X-linked TIE: Thrombocytopenia infections eczema death- bleeding, infections, lymphoma IgM low IgA and E high eventually become mentally challenged
how do you tell TB and MAC appart
TB makes niacin
TB has porin factor and sulfatides
what advantage does foscarnet have over gancyclovir?
it does not need to be activated by viral kinase
periventricular calcifications
congenital CMV
basal ganglia calcification
congenital toxo
anti arrythmics
No Better Pot then in Cali Na BB K Ca
when do you shock an arrythmia
decreased BP
change in mental status
chest pain
severe SOB
myocarditis w/eos
loefflers endomyocarditis
right sided cardiac fibrosis
tricuspid regurg, pulmonic valve stenosis d/t carcinoid syndrome
what vit is deficient in carcinoid syndrome
niacin, bc tryptophan used up making serotonin
DO NOT supplement tryptophan (restrict it) and supplement niacin
Tx of carcinoid syndrome
surgery
somatostatin analog octreotide (tumor will take it up to ID tumor)
tumor in heart that stains S100 and HMB-45
metastatic melanoma
tumor in heart that stains cytokeratin (CK) 7
metastatic lung CA
tumor in heart that stains CK 2D
metastatic colon CA
increased unconjugated/indirect bilirubin
hemolytic anemai
physiologic jaundice of newborn
gilberts (very common)
crigler-najjar
crigler-najjar tyep 1
fatal, w/o liver transplant
no UGT
phenobarbital -> nothing
confirm w/genetic testing UGT promoter
crigler najjar type 2
too little UGT,
phenobarbital -> corrects
uncommon
UGT coding region
increased conjugated/direct bilirubin
dubin-johnson
rotors
obstructive
dubin-johnson
black liver
not bilirubin pigment from catecholamine build up
cannot transport conjugated bili out of liver
rotors
normal color of liver
which parasite causes obstructive jaundice
clonorchis sinensis
primary biliary cirrhosis
granulomas cause obstruction only destroys bile ducts inside liver xanthomas fatigue chirrosis common in SLE, RA AMA abs (mito)
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
what cells lay down fibrosis in liver?
ITO cells, aka hepatic stellate cell
usually fat and vit A
what signals fibrosis
TGF-beta
trichome stain
think fibrosis
blue-fibrosis
red- regenerative nodules
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
hepatic encephalopathy
d/t ammonia build up
cannot eat protein
HBV
ground glass cytoplasm
HCV
fatty change
lymphoid aggregates
amebic liver abscess
brown fluid, looks like chocolate
Tx w/metronidasole
mallory bodies
swollen hepatocytes w/pink ropey stuff in alcoholic hepatitis
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
Dx of wilsons
ceroplasmin low
high urine Cu
liver Bx shows Cu
what level to horseshoe kidneys get stuck at?
IMA at L3
ARPCKD
PKHD1- codes for fibrocystin
fatal near birth
radiating cysts
