McGowan Test II Flashcards
CNS defects
hemolysis (low LDH, Anemia, Haptoglobin)
<40yo
is what disease?
Wilson’s
tea colored urine and white clay colored stools
obstruction in the biliary system
grandparents w new onset of DM painless jaundice >65yo direct hyperbilirubinemia epigastric pain when laying down, relief when bending forward ca 19-9 courvoisier sign trousseas sign of malignancy
pancreatic cancer
adenocarcinoma
Crohns, DM, native american race, rapid weight loss are RF for what?
gallstones
mc in pt w cirrhosis
gram neg bacillis - e coli
gram postive - strep, enterococci, pneumococci
only single org is isolated
dx if peritoneal fluid contains 250 pmn
blood cultures bc bacteremia is common
tx: 3rd gen cephalosporin like ceftriaxone or pipercillin tazobactam
spont (primary) bacterial peritonitis
bact contaminate the peritoneum - spillage from intraabd viscus
mixed flora - gram neg bacilli and anaerobes predom
spread to peritoneal cavit - increase pain
pt lies motionless, often w knees drawn up to avoid stretching nerve fibers of peritoneal cavity
invol guarding
dx: radiographic studies to find source or immediate surg intervention, abd tap done only to exclude hemoperitoneum in trauma
tx: antibiotics aimed at inciting flora
surgery needed often
secondary bacterial peritonitis
Epigastric abd p RUQ pain, dyspepsia/ GB disease etiology? Cullen or Grey turner sign crackles, diff breathing - ARDS?
Chvostek (twitch of facial n m) and Trousseau (hand posture w bp inflatition) signs = hypocalcemia
Acute Pancreatitis
Acute Pancreatitis
pathology?
mc cause?
why is there hypocalcemia?
2-3 to dx?
MC imaging follow up? avoid this why?
tx?
Cellular injury from activation of trypsinogen to trypsin in autodigestion of pancreas and peri-pancreatic tissues
Gallstones <5mm, heavy alcohol use, but also hypertriglyceridemia, trauma, meds, ERCP, autoimmune, infections, CFTR, Idiopathic
saponification
epigastric pain
lipase (and amylase) 3 x ULN
CT changes consistent w pancreatitits
Rapid bolus IV contrast-enhanced CT, avoided if serum Cr > 1.5
fluid resuscitation - first thing IV
then tx main cause
two signs you see on xray of acute panc?
sentinel loop - seg of air filled SI (mc LUQ)
colon cutoff sign - gas filled seg of transverse colon abruptly end at area of panc inflamm
- focal linear atelectasis of lower lobe of lung w/ w/o pleural effusion
how to predict severe acute pancretitis?
Ranson criteria
GA - LAW
C & Hobbs
Bedside index for severity in acute panc (BISAP)
- bisap score = BUN >25,
- impaired mental status,
- SIRS 2 of4, age > 60,
- pleural effusion
apache II > 8 = higher mortality
- severely ill pt, predict hospital mortality
complications of acute panc
- intravascular vol dep
- –leaks fluids in panc bed = 3rd spacing
- –ileus w fluid filled loops, Pre-renal azotemia
- fluid collections (pleural effusion)
- necrosis w/ wo infection (including emphysematous pancreatitis)
- –infection needs debridement (high mortality)
- pseudocysts - high amylase
- ARDS -> cardic dysfxn
emphysematous pancreatitis caused by?
could be from c. perfringens, enterobacter aerogenes, enterococcus faecalis
epi pain, steatorrhea (chronic fatty diarrhea), unintentional wt loss fatique pain is cardinal symptom attacks may last a few hours-2 wks steatorrhea - bulky foul fatty stool occurs later in course, exocrine insuff!
Chronic Pancreatitis
Chronic Pancreatitis
mc cause?
dx?
causes?
complications?
alc, Chronic Pancreatitis
decreased fecal elastase <100
glucose/HbA1c - main -> dm after 25 yr chronic
autoimmune panc - elevated IgG4
calcifications on xray/ct
T oxic metabolic: alcoholic (mc) Idiopathic (early onset 23) Genetic: CFTR Autoimmune: celiac, hypergammaglobuminemia - IGG4 Recurrent Obstructive - stricture, stone, tumor
brittle DM, panc insufficiency, panc ca
tumefactive chronic pancreatitis is code for?
panc ca
shows significant steatorrhea = malabsp of triglycerides
wt loss, gas distention and farts, large greasy foul smelling stool diarrhea
fecal elastase <100
others - trypsinogen <20, malabsorption, stim test (cholecystokinin/secretin)
exocrine detection of decreased fecal chymotrypsin
- decreased panc fecal elastase <100
Endocine -DM after 25yrs of chronic P
pancreatic insufficiency
AD, rare, Type 1
endocrine glands benign or malignant
parathyroid
- hyperca, increased PTH
pancreas
- gastrinoma - ZE (mc in duodenum, then panc), PUD
- insulinoma - hypoglycemia = blood sugar drop symp
pituitary
- acromegaly: lots growth of hands, feet, haw, internal organs
- cushing disease
MEN 1
increased bili production, impair bilirubin uptake/storage
unconjugated (indirect)
ie. hemolytic syndrome
gilbert syndrome
impaired excretion, hepatocellular dysfxn, biliary obstruction
conjugated (direct)
ie dubin johnson syndrome rotor syndrome drug rxn pregnancy viral hep crigler najjar intrahepatic cholestasis of pregnancy hepatitis, cirrhosis obstruction
prehepatic, hepatic, post hepatic
transfusions
sickle cell
thalassemia
autoimmune
hepatitis ca cirrhosis congenital disorders drugs
gallstones inflammation scar tissue tumors block flow of bile into intestines
with prehapatic or unconjugated bili, you should order what test?
order CBC (hemolysis!)
- anemia and thrombocytopenia
- haptoglobin, reticulocyte count, LDH to look for hemolysis
- leukocytosis
Peripheral smear - schistocytes, sickle cells
benign, asymp, hereditary, fasting -> increases indirect hyperbilirubinemia, abn lab results, no tx, reduced mortality from cv disease reduced UDGT
gilbert syndrome
reduced excretory fx of hepatocytes benign, asyp hereditary, gb does NOT visualize on oral cholecystography liver dark pigment on examination bx show centrilobular brown pigment
dubin johnson
reduced hepatic reuptake of bilirubin conjugates
sim to DJ but liver isnt pigmented and gb is visualized on oral cholecystography
rotor syndromes
cholestasis benign, 3rd trimester of preg, itching, gi symp, abn liver excretory fxn test cholestatsis noted on liver bx recurrence w subsequent preg or use of oral contraceptives
intrahepatic cholestasis of pregnancy
rapid liver shrinking size
rapid rising bilirubin level
marked prolongation of PT
signs of confusion, disorientation, somnolence,
ascites, edema
leads hepatic failure w encephalopathy
life threatening coagulation abnormalities INR > 1.5
hepatic encephalopathy -> deep coma (cerebral edema)
asterixis
Acute (fulminant) Liver Failure (ALF)
Acute (fulminant) Liver Failure (ALF)
mc causes?
Labs?
complications of ALF
acetaminophen
idiosyncratic drug rxn = sporatic (prescription drugs, antiTB, antiepileptics, antibiotics, herb, dietary supplements)
- rapidly rising bilirubin level + marked prolongation of PT even as AST/ALT levels fall
- in acetaminophen toxicity AST/ALT > 5000uL
- ammonia level elevated -> encephalopathy and intracranial HTN >200
- acetaminophen level ~ use Rumack-Matthew Nomogram
cerebral edema (brainstem compression) and sepsis are the lead causes of death
if acetomnophen OD what should you give?
what factor improves ALF survival?
N acetylcysteine (NAC) in acetomnophen OD
meticulous intensive care + prophylactic antibiotic coverage = one factor improves survival
viral, drugs, ischemia can cause this
RUQ pain = tenderness over liver, jaundice, hepatomegaly, skin changes, abd p
aversion to smoking?
acholic stools
what dx test should you run?
tx?
Acute Hepatitis
CBC
CMP
- liver enzymes, total bili, albumin, renal fxn
PT/INR
acetaminophen level - use rumack-matthew nomogram
N-acetylcysteine aka NAC or Mucomyst
ssRNA
acholic stools
aversion to smoking
n/v/anoexia, malaise, ruq p or epigastric, fever, hepatomegaly, jaundice, arthralgia, tires easy, UR symp, diarrhea/const
acute only
international travel, inadequately cooked shellfish
fecal-oral
international travel, inaqequately cooked shellfish
HAV
what do you see on HAV Labs?
elevated aminotransferases (AST/ALT) - hepatocellular elevated bilirubin and ALP = cholestasis
IgG & IgM Anti-HAV both are detectable in serum soon after onset
dx w IgM anti-HAV
IgG anti-HAV indicates previous exposure to HAV, non infectivity and immunity
partially dsDNA
n/v/anoexia, malaise, fever, hepaatomegaly, jaundice
Acute - 2/3 wk
Chronic - 5-10%
MSM, inject drugs, ppl at hemodialysis, healthcare workers, jailers, tx sexually transmitted diseases
- parenteral, sexual, perinatal
- infected blood or b. products - -percutaneous from needle
- perinatal - endemic in sub-saharan africa and SE asia
- HBsAg + mom may transmit to bb in delivery, risk of chronic infection in bb is high
cirrhosis (esp w HDV), hepatocellular carcinoma
HBV
what is the window period of acute hbv?
why is this usefully clinically?
bw HBsAg disappearing and HBsAb appearing, pt still considers acute HBV, only detectable w HBcAb IgM
important to screen blood donations
IgM Anti-HBc
window period
IgM Anti-HBc
HBsAg
HBeAg
HBV DNA
acute infection
Anti-HBs
IgG Anti-HBc
prior infection
HBsAg
IgG Anti-HBc
HBV DNA?
chronic infx, inactive
HBsAg IgM Anti-HBc IgG Anti-HBc HBeAg HBV DNA
chronic infx, active
Anti-HBs
immunization
what do all these mean?
IgG Anti-HBc
HBeAg + HBV DNA
IgM Anti-HBc
Anti-HBs
HBsAg
not infectious
active viral relication
acute HBV
exposed to virus
have the virus
defective RNA virus that requires HBV
co infects w HBV or superinfects a chronic HBV carrier
non percutaneous or percutaneously in HBsAg (active) IV drug users or hemophiliac transfusion
HDV RNA - pcr
vacc against HBV
HDV
ssRNA
marked fluctuating elevations of serum aminotransferase levels
mc chronic - 8wk
HCV Ab in serum,
- most sensitive indicator is HCV RNA
- anti-HCV in serum w/o RNA -> recovery from prior infection
non to HCV, chronic liver dz should be vass against HAV and HBV
birth cohort screening of person born bw 45-65 (bb boomer) for HCV infection, all over 18 should have 1 lifetime screening
curable w proper tx
HCV
HCV RF?
complications
-coinfection w HIV, developing world unsafe med practices
-pt has tranfusion related hepatitis, HCV >90% cases
I
-IV drug use, intranasal
-bloody fights, incarceration in prison
cirrhosis, HCC, HIV coinfection
mixed cryoglobulinemia, decrease serum cholesterol
ssRNA herpevirus
immunocompromised host
endemic/epidemic form in Asia, ME, N africa, central america, india
HEV Ab, PCR for HEV RNA
clinical trials
high rate in pregnant women
acute = no carrier state
transplant pt, tx w tacrolimus instances of chronic w progression to cirrhoisis
HEV
dose-dep (direct hepatotoxins) - w/in 48hrs
- e.g. acetaminophen, tetracyclines, mushrooms
variable dose and time of onset
fever rash arthralgias eosinophilia
e.g. isoniazid, sulfonamides, methyldopa
supportive tx
- include gastric lavage and oral admin of charcoal or cholestyramine
w/in 1 hour post ingestion esp w acetaminophen
liver transplantation
acetaminophen overdose
- NAC after the 1hr as above
Toxic and Drug Induced Hepatitis (DILI drug induced liver injury)
tylenol/acetaminophen overdose - tx
*use rumack-matthew nomogram
tx w N acetylcysteine aka NAC if in toxic area
important to get 4hr acetaminop
therapy win 8 hr of ingestion
- critical ingestion tx interval for max protection against severe hepatic injury is bw 0-8hrs
SAAG > 1.1
occlusion of flow to hepatic v or IVC
caval webs, R side herat fail -> nutmeg liver
fx that predispose pt to hepatic v obstruction
- such as hereditary or acquired hypercoag states (polycythemia vera)
occlusion of flow in hepatic v or ivc
Hepatic vein obstruction - Budd Chiari Syndrome
Budd Chiari Syndrome tx and imaging?
imaging
- prominent caudate liver lobe
- screen test of choice is contrast enhanced US
- color or pulsed doppler ultrasonography
- direct venography
—delinate caval webs and occluded v = spider web pattern
- liver bx
—centrilobular congestion - nutmeg liver
hepatocellular carcinoma
symptomatic tx, anticoag, thromboylytic, liver transplant
mc cause jaundice in preg
viral hepatitis
maternal HTN, proteinuria, peripheral edema, coag abn
HELLP syndrome
severe elvation of serum aminotransferases, mild elevation of bili
pre-eclampsia
hemolysis, elevated liver enzymes, low platelets
eclampsia is?
HELLP + hyperreflexia + convulsions = eclampsia = life threatening
tx: termination of preg
pruritus 3rd trimester (dobby boy says 2-3 trimesters), darkening urine, light stools, jaundice
resolves after delivery by itself
bilirubin <5, might have ele ALP
bx: canalicular cholestatis
recurrent w preg
cholestatsis of preg
spectrum of dz from subclin or modest hepatic dysfxn (increased aminotransferase levels) -> hep failure, coma, death
3rd trimester
bleeding, n.v, jaundice, coma
tx: termination of preg
acute fatty liver of preg
inflam rxn in liver for >6mo
jaundice,
fatique/malaise/anoerxia/low grade fever
complications of cirrhosis
chronic HBV/HCV
HBV - polyarteritis nodosa
HCV - mixed cryoglobulinemia (abn proteins in blood, thicken in cold temp)
CBC, Chem panel, Coag studies
Bx - histo classification
grade - necrosis and inflam activity
stage - level of dz
progression based on degree of fibrosis
Chronic Hepatitis
Fibrosis check?
serum fibrosure or US elastography
endemic in asia and sub-saharan africa males risk of HCC, cirrhosis asymp healthy carrier state younger the dz, high prob of chronicity
chronic HBV
cirrhosis progression increased in: males, drink 50g ETOH qd, infect after 40yo, immunocomp, tobacco/cannibis smoking/fatty liver coffee slows normal ast/alt risk of cirrhosis and HCC, genotype 1 Ab + RNA = chronic most is curable
chronic HCV
female, 30-50
progressive jaundice, epistaxis, amenorrhea
exam shows healthy young woman w stigmata of cirrhosis
- mult spider telangiectasis,
- cut striae,
- acne,
- hirsutism,
- hepatomegaly
DX:
- aminotransferases may be>1000
- total bilirubin is increased
- T1: adults, hypergammaglobulinemia (IgG), SMA, ANA (anti smooth m(SMA) or antinuclear antibodies)
- T2: kids, anti LKM antibody (anti liver/kidney microsomal antibodies )
“focus of lobular hepatitis w/ prominent plasma cells”, CD4 and CD8
tx glucocorticoids -prednisone (w-w/o azathroprine)
cirrhosis -> HCC if untreated
liver transplant
Autoimmune Hepatitis
asymp -> severe liver failure w jaundice, ascites, gi bleed, encephalopathy
typically anorexia, n, v, fever, jaundice, tender heptomegaly, RUQ P
DF > 32 MELD > 21 GAD > 9
tx: steroids, pentoxifylline
alc hep
excessive alc, exceeds 80g/day males, 30-40g/day females for 10+ yrs
steatosis (Fatty liver) -> alc hepatitis -> cirrhosis
fatty liver = asymp hepatomegaly and mild elevations in biochem liver test - bili and alp
SAAG > 1.1
tx: abstinence from alc
daily multivitamin, thiamine 100mg, folic acid 1mg, zinc
-glucose admin increase the thiamine requirment bc w/o -> wernicke-korsakoff syndorme
Alc Liver Dx
> 32 poor prognosis
benefit from steroids
involve PT and serum bilirubin
maddreys discriminate function (df)
predicts mortality based on multivariable mdoel
age, bili, bun, ptt, wbc
> 9 then give glucocorticoids for higher survival
glasgow alcoholic hepatitis score
> 21 inc significant mortality in alc hepatitis
>14 for liver transplant
model for end stage liver diesase MELD
alc liver dx labs?
high
- AST 2x > ALT
- WBC - leukocytosis w left shift if severe
- bilirubin >10mg/dL
- ALP and GGT 3x
low
-albumin, wbc (leukopenia), Hgb (anemia, macrocytic), folate, platelets (thrombocytopenia)
marked prolongatin of PTT
US, CT w intravenous contrast or MRI, US elastography
Liver bx: mallory bodies - alc hyaline
alc liver complications?
mc precursor to cirrhosis in US. -> HCC
-wernicke encephalopathy - thiamine tx
confusino, ataxia, abn eye movements
- korsakoff syndrome -severe mem issues/ confab stories
- adverse prog fx:
predict poor prog - ascites, variceal hemorrhage, encephalopathy, heptorenal syndrome
—critically ill pt w alc hepatitis have 30 day mortality rates
—severe alc hepatitis characterized by:
—–total bili > 8-10mg, PTT >6sec
—–susceptible to infections including invasive aspergillosis
—–hypoalbuminemia, azotemia
alch related or NAFLD
mc chronic liver disease
causes: metabolic syndrome - obese, dm, hypertriglyceridemia
risk for cv dz, ckd, colorectal ca
protective: activity, coffee
uncommon in AA (NASH - nonalc steatohepatitis)
asymp w RUQ discomfort, hepatomegaly
labs mild elevation ast/alt, alp, maybe normal
image: US elastography - liver sitffness est hepatic fibrosis
liver bx: diagnostic , focal infiltration by polymorhponuclear neutrophils and mallory hyaline, a pic indistinguishable from alc hepatitis
lifestyle mod,
statin not contraindicated
advanced cirrhosis. -> liver transplant
fatty liver - hepatic steaotsis
AR, low levels of a1-antitrypsin
pulmonary emphysema (protease activity crazy)
panacinar emphysema at young age - lower lobes
hep accumulation of misfolded protein -> liver dz
MC diagnosed inherited hepatic dz in infants and children
mc genetic cause requiring liver transplant in children
micronodular cirrhosis -> hcc
alpha 1 antitrypsin def
dx by low alpha 1 antitrypsin level
- check phenotype of this
smoking abstinence/cessation
liver transplant
progressive non-suppurative destructive intrahepatic cholangitis
infections w novosphingobium aromaticivorans or chlamydophila pna triggers
autoimmune T lymphocyte destruction of small intrahep bile ducts and cholestatis, florid duct lesions (lymph or granulomatous bile duct desctruction)
female, 50, US and N Europe
rf: UTI, smoke, hormone replacement, hair dye
associated autoimmune dz
acute: asymp isolated elevation in ALP
advanced dz: pruitius, progressive jaundice, xanthelasma, skin hyperpigmentation, steatorrhea/vit d malabsorption, osteomalacia/porosis, hypercholesterolemia
antimito antibodies against E2. of PDC-E2 (ANA, ANCA)
increased IgM, GGT/ALP (increased for 6mo to dx), bili cholesterol
tx: ursodeoxycholic acid
liver failure prognosis - age, bili in serum, alb, ptt, edema
–>HCC
primary biliary cholangitis - 1 biliary cirrhosis
associated w sjogrens syndrome, scleroderma, thyroid dz
HFE gene mut on ch 6
increase in:
- iron abs from duodenum
male
- iron sat and increase serum ferritin or a fam hx
- accum of iron as hemosiderin in livr, panc, heart, adrenals, testes, pituitary, kidneys
50 >
classic triad: cirrhosis w hepatomegaly, abn skin pigmentation, dm, cardiac dysfxn (HF)
early - nonspecific, later - skin pigmentation to bronze color, dm, erectile dysfxn
HFE gene mut, mild abn liver enzymes, elevated plasma iron w greater than 45% transferrin saturation
elevated serum ferritin
imaging:
- MRI - show liver iron stores and fibrosis
- CT
- liver bx
- – homozygous for C282Y, determines if cirrhosis is present
screening for HFE in all first degree fam
phlebotomy therapy, deplete iron stores
avoid food rich in iron and monitor
admin ppi
liver transplantation
rf for fibrosis - male, excess alc, dm
if hep or panc insuff , hf,
hypogonadism -> increases mortality
hemochromatosis
ch 13, ATP7B
person under 40
impaired cu excretion into bile and failure to incorporate copper into ceruloplasmin
accum in liver, brain, eye (also kidney, bones, joints, parathyroid glands)
copper is also toxic to RBC -> hemolytic anemia
liver dz in adults, neuropsychiatric dz in ya
child or ya w: hepatitis, hemolytic anemia, neurologic, psychiatric abnorm, splenomegaly w hypersplenism, coombs-neg, portal hypertension
neurologic manifestations - parkinsonism, tremor, ataxia, dysarthria, dysphagia, incoordination, spasticity
psychiatric - behavioral and personality changes and emotional lability
pathognomonic sign of condition is is brownish or gray -green Kayser-Fleischer ring
assoc: renal calculi, hemolytic anemia, subcut lipomas
kayser fleischer rings
elevated liver enzymes
increased urinary copper excetion, hep copper conc
low serum ceruloplasmin
MRI of brain should cooper there in the basal gang, brainstem
mol analysis of ATP7B mut
tx: oral penicillamin
liver transplant
cirrhosis
wilson’s dz
SAAG > 1.1
passive congestion of liver - nutmeg in RHF
ischemic hep -> acute fall in cardiac output
statin thx prior to admin - protect against ischemic hepatitis
hypotension may be absent
hepatojugular reflux present, tricuspid regurg the liver may be pulsatile
jaundice associated w worse outcomes
ast/alt mild ele in abs of superimposed ischemia
markedly elevated serum N-terminal-proBNP or BNP
shock liver: rapid and striking elevation of serum aminotrans levers
greater than 5000
early rapid rise in LDH
elevations of serum alkaline phosp and bili are mild
mortality rate due to underlying dz is high
liver in HF
liver cell injur -> inflammation/necrosis/stellate cell activation -> fibrosis -> destroys vascu and lobar architecture -> diminishing blood low, forming regenerative nodules
hx:
- viral - tattoos, iv/intralnasal drug use, maternal hx, blood transfusion
- alc consumption
- hx of viral hep or jaundice
rf:
- alc
- iv drug abuse (intranasal) -> viral
- obesity
- viral
reduces risk: coffee, tea
mc = hep c, alc liver dz, nafld
Cirrhosis
path accum of fluid in peritoneal cavity
mc cause is portal htn, second to chronic liver disease
increased abd girth and or pain
asterixis - secondary to encephalopathy
shifting dullness - percussion that changes w position, >1500ml for reliability
abd US see fluid +doppler (detect vascular in Budd Chiari)
abd paracentesis
ascites
ascites labs?
studies on ascitic fluid 1. cell count wbc = abd is inflam, SBP? = pmn 250
2. albumin and total protein
serum ascites albumin gradient
3. culture and gram stain for aerobic and anaerobic cultures
acute gastro hemorrhage w melena, hematochezia, hematemesis - hypovolemia manifested by vital signs and shock
dilated submucosal v in esophagus, secondary portal htn from cirrhosis
risk of bleeds eso varices?
esophageal varices
size larger than 5mm, red wale markings (long dilated venules on varix), severity of liver disease, active alc abuse
AMS in presence of liver fail
ammonia levels increased in enceph
proceeds: gi bleed, constipation, sepsis
clincial dx
ammonia levels high
lactulose = acidifys colon, diarrhea,
maintstay of tx
goal 2-3 soft stools a day
poor abs antibiotics in pt don’t tol
tx: lactulose - ie rifaximin
hx: asterixis - flapping tremor, neuro symp
grades:
- mild confusion
- drowsiness
- stupor
- coma
hepatic encephalopathy
male, 50-60,
cirrhosis leads to this,
asia, africa
cachexia, abd p, fever, jaundice, itch, tremors, disorientation, hepatomegaly, ascites, peri edema
AFP and US every 6 mo
pt w liver disase see abn on US or AFP
abd liver function test and liver enzymes
tx:
surgical resection or liver transplantation
radiofreq ablation
transcathter arterial embolization
HCC
hemochromatosis + anemia or thalassemia cant tolerate phlebotomy so use what?
affected pt are at increased risk of infection w/ what?
deferoxamine
vibrio vulnificus, listeria monocytogenese, yersinia enterocolitica
normal until end stg dz jaundice, scleral icterus, spider angiomata (telangiectasias - upper body), gynecomastia, ascites, esophageal varices, palmar erythema, asterixis, caput medusae duputrans contracture, umbilical hernia, hypoalbuminemia - muehrckes lines and terrys nails
cirrhosis
best imaging for cirrhosis?
ultrasongoraphy
- RUQ liver
- w/ doppler of veins
paracentesis - dx/tx
-SAAG =Serum albumin – ascites albumin
>1.1 portal HTN related
>250 PMN, think SBP
esophagoduodenoscopy -dx/tx
noninvasive predictive indices for hep fibrosis
US elastography
serum dx specific for fibrosis - fibrosure (estatography score)
cirrhosis complications?
tx?
Spon bacterial peritonitis
blood culture, paracentesis ( albumin, wbc w diff, culture and gram stain)
hepatocellular carcinoma, decompensated cirrhosis
hiv co infection
tx:
- underlying cuase - viral help, pbc, wilsons, autoimmune, hemochromatosis
-portal htn supportative
varices
ascites
encephalopathy
pruritus
prophylactic antibiotics
sbp/gi bleed/invasive
procedures -cipro
-TIPS (trangsjugular intrahepatic portosystemic shunt)
SBP ceftrizxone or cefotaxime - iv albumin for renal perf pressure
HCC: ablation, resection
Liver transplant
management for cirrhosis?
abstain from alc, drugs, smoking, liver toxic meds, nsaids
mod acetaminophen - 2g/24hrs
egd for varices
moniter labs - liver enzymes, platelets, pt, inr
alpha fetoprotein and US every 6 mo to look for HCC
immunizations - pneumococcal, hep a and b, flu
NAFLD wt reduction, exercise, control dm
liver transplant
refer = 1st onset of major complications - ascites, variceal bleed, enceph, jaundice, liver lesion, hep dysfxn
worsening meld or child pugh scores
abstinent for 6mo, no alc
severity rating for cirrhosis
MELD/ + Na - bili, creatinine, na (CMP) MELD >10 worse, >14 transplant child turcotte pugh scoring bili, albumin -cmp pt, inr, PE for enceph and ascites A-C (c is worst)
markers for liver damage
ASL/ALT: increased necrosis and liver cell injury
ALP: sensitive to cholestatic biliary obstruction
Bilirubin
LDH/GGT
liver fxn test
PT/INR - w INR tst w VIt K -> corrects, then absorption isuenot disease
albumin
cholesterol
ammonia
primary injury to hepatocytes
increased AST/ALT
hepatocellular
primary injury to bile ducts
increase ALP/bilirubin
cholestatic
jaundice and pruitius
what is most specific test for the liver?
ALT
how are bilirubin, liver enzymes, blood counts, etc affected with:
liver dz
hemolysis
gilbert’s
liver dz: increased bili (Both) and liver enzymes
hemolysis: normal direct, increased indirect, anemia, increased reticulocyte count. normal liver enzymes
gilbert’s: normal direct and liver enzymes, no anemia, increased indirect (esp in fasting)
+ when pt takes deep breath - then stop bc of pain or cough during palp of RUQ
murphy’s sign
types: cholesterol, pigment
asmp until they obstruct or trigger inflamm
- then get biliary colic (this is?)
- n.v
dx: ultrasonography (RUQ US/hepatobiliary UD) which you see stones and acoustic shadow
tx: decreased fat diet
cholelithiasis
RF for cholelithiasis
american indians weight loss dm, obese crohns preg oc
protective for cholelithiasis
decreased carbs, increased PA, fitness of CV, coffee, Mg and healthy fats, fiver, statins, and nsaids
mc acute cholecystitis?
calculous - gallstones in cystic duct, smaller = more dangerous
other little is acalculous w no stones - from infection, illness, ca, could be from ischemia, more dangerous bc can have no symptoms referable to gb, increased incidence of gangrene and perf
- s. typhi and staph can cause
- along w poly nodosa, atherosclerotic ischemic dz, aids - crypotsporidium, ascending infection
tea color urine, acholic stools muscle guarding and tender get pain when you eat a fatty meal jaundice - fever, anorexia, tachy, sweat, n/v
obstruction of the neck of the gb, or cystic duct
leukocytosis, increased ALP/GGT, AST
tx: antibiotics and surgery (cholecystectomy)
Image: RUQ abd ultrasonography
- thicken gb, pericholecystic fluid, sonographyc murphy sign
complications: gangrene -> perf -> abscess -> peritionitis
emphysematous cholecystitis (from gas form org)
assciated w increased risk of gb carcinoma
acute cholecystitis
stones in common bile duct recurrent attacks of RUQ pain for hours chills + fever hx jaundice w pain Increased AST/ALT (LOTS), bilirubin, leukocytosis, w slow rise of alp, ggt
this can lead to what?
gold std for dx and theraputic?
choledocholithiasis
acute ascending cholangitis
ERCP - check inr first, + sphincterotomy for stone extraction and stent placement
could do lap cholecystectomy
charcot’s triad
reynolds pentad
RUQ p + fever/chills + jaundice
if reynolds, then add AMS + hypotension
charcot triad/reynolds pentad
recurrent attacks of RUQ pain for hours
chills + fever
hx jaundice w pain
labs: + blood cultures (for what?)
image: ERCP + sphinceterotomy
tx: ERCP or lap cholecystectomy
ascending cholangitis
e.coli, klebsiella, entercoccus
what labs will be elevated if the stone was at:
cystic duct?
common bile duct
sphincter of vadar
increased wbc (inflamm and infection)
increased AST/ALT/ALK, direct bili
increased of all - AST/ALT/ALK, both bili, wbc, lipases amylase
RUQ p that limits activites of daily living, nausea
pain sim to biliary colic
normal US
normal enzymes across the board
dx: Rome III dx criteria
HIDA scan + CCK (tc 99)
abn: gb not see (with just hida?)
abn ejection fraction -> choleycystectomy < 35-38
biliary dyskinesia
biliary colic
severe steady ache RUQ or epigastric area
radiates to R scapula
chronic inflamed bladder + gallstones
repeat acute/subacute cholecystitis
can have rochan. sinuses - dilated epi space, invag into fibrotic tissue
inflammation cells in LP (lymphocytes, macrophages, plasma cells)
granulomatous and multi nuc giant cells from cholesterol crystals
complications: porcelain gb on xray -> incidental calcified lesion
risk of gb ca
need surgery
chronic cholecystitis
liver bx shows periductal fibrosis (aka what?)
gold standard dx: on ercp, mrcp it look like beads on a string from strictures/inflammatory destruction and beading of large bile ducts
progressive, associated w IBD, panc, fibrosing dz
pANCA +, sometimes see increased ALP in esp UC pt
male 20-50
assoc w UC
coffee and smoking decreases risk
pruritus, jaundice, fatigue
increased risk of cholangiocarcinoma and colon ca
not satisfiying tx, tx like cholangitis
primary sclerosing cholangitis
onion skinning
can be associated w ascending cholangitis (charcot triad), along w chronic pancreatitis, chronic cholescystitis
–> hepatic fibrosis and cirrhosis -> cholangiocarcinoma
ischemic changes in liver parenchyma causes destruction of bile ducts resulting in this
from inflammation and endothelial damage from antibodies binding to vascular endothelium, activating complement in acute rejection
can also occur in chronic rejetion - TGFB stim fibroblast from stellate cells and vascular sm cell stimulation (proliferation) -> interstitial fibrosis, inflammation and endothelial damage
vanishing bile duct syndrome
what is the mc gallbladder congenital anomaly?
folded funus = phrygian cap
mc biliary tract disease is attributable to?
mc type?
cholelithiasis
cholesterol in US, western europe, native americans (radiolucent)
pigment is the other type, mc in non western pop
- in setting of bacterial infections, parasitic infestations, chronic red cell hemolysis dz
- –liver flukes ( clinorchis sinensis, ascaris lumbricoides, e coli)
- black stones (radiopaque) - in gb, brown - large bile ducts (radiolucent)
- asians, rural
large gallstone erode directly into adjacent loop of small bowel generating intestinal obstruction
gallstone ileus or bouveret syndrome
green pus, increased wall thickening, serosis that is hemorrhagic bc there is only one blood vessel -> necrosis is what?
acute cholesthisis
cystic artery is the only blood supply -> emphysematous cholestitis - air around gb
mc malignancy of extrahepatic biliary tract
carcinoma of gb, women bc of gallstones
- adenocarcinomas mc in fundus
- travel into liver, cystic duct, bile ducts, portal hepatic lymph nodes,
- peritoneum, git, lungs common seeding sites
SINGLE well demarcated lesion w central scare
incidental finding, normal liver
ya to middle adults
maplike pattern of strong cytoplasmic gs staining
confused with malignancy
focal nodular hyperplasia
MULTIPLE nodules but no fibrous septa
looks like cirrhosis
portal htn where nodules impede blood flow
associated w hiv, rheumatoglogic dz
nodular regenerative hyperplasia
mc benign neoplasm of the liver?
cavernous hemanigioma
females, asymp, incidental discovery
unless intraabd emergency from hemorrhage due to subcap location (rupture easy) - life threatening
often mistaken for malignancy
benign neoplasm in young women
associated w oral contraceptives and anabolic steroids
increased risk w obesity and metabolic syndrome
incidental finding on imaging
pain from rapid growth or hemorrhage or rupture
hepatocellular adenoma
one of mc adenomas
females
min risk of -> HCC
fatty w no atypia
HNF1-a inactivated adenoma
one of mc adenomas
females
assoc w obese, metabolic syndrome
B catenin mutations - high risk of malig
overexpress of crp and amyloid A - mimcs FNH
inflammatory adenomas
high risk for malignant transformation to HCC
oral contraceptive and anabolic steroid use association
men
B catenin activated adenomas
see w b catenin staining and dysplasia
mc peds hepatocellular tumor?
<3yo
abd swelling in asymp child
could have jaundice or pruritis
associated with what?
metastasized to?
hepatoblastoma
FAP, beckwith-wiedemann syndome
lung metastasis
mc epithelial tumors:
mc primary malig of hepatocytes?
hcc
- mc in asia and subsaharan africa w HBV, male, 20-40yo
- hcv in western countries, metabolic syndrome (NAFLD)
can also be from a1-at, hemochromatosis, wilsons,
-mc caused from CLD, cirrhosis
or adenomas aflatoxins (noncirrhotic livers)
cholangiocarcinoma
rare, nonepithelial tumors
caused by vinyl cl, arsenic, thorotrast
angiosarcoma
rare
mc primary hepatic lymphoma
middle aged men
assoc w HBV, HCV, HIV, PBC
second mc lymphoma?
- mc in ya males
- predilection for growth w/in sinusoids of liver, spleen and bone marrow
diffuse large b cell lymphoma is mc, followed by malt
hepatosplenic t cell lymphoma
what are the types of hepatoblastoma?
epithelial type - polygonal fetal cells and smaller embryonal cells forming acini, tubules and papillary structures
mixed epi and mesenchymal type: foci of mesenchymal differentiation consist of primitive mesenchyme, osteoid, cartilage, striated m
distinctive HCC variant
under 35yo
single large, hard scirrhous tumor w fibrous bands
under microscope - large polygonal cells w granular cytoplasm w abundant mitochondria
vesicular nuclei w prominent nucleolus, parallel lamellae w dense collagen bundles
fibrolamella carcinoma
hepatic ca tx
surgical resection
liver transplantation
image guided tumor ablation
adenocarcinomas from intrahepatic biliary tree?
- mc malig tumor after HCC
- common in SE asain contries - thailand, laos, cambodia (liver flukes)
- found indicentally or have a cholestatic picture
adenocarcinomas from extrahepatic bile ducts?
- biliary obstruction symp
intrahepatic cholangiocarcinoma
biliary adenocarcinoma
- rf: chronic inflammation, psc, liver flukes, hepatolithiasis, HBV, HCV, NAFLD, fibropolycystic liver dz
- glandular structures lined by mali epi cells in abundant fibrous stroma
- lymphovascular and perinueral invasion common
what is more common: primary hepatic neoplasia or metastatic malignancy involving the liver?
metastatic malignancy involving the liver
mc colon, breast. lung, panc spread
anorexia, fever, jaundice, n/ RUQ pain, sweat, wt loss
what has the highest inherited pedisposition of panc carcinioma?
peutz jegher syndrome, STK11
what is the mc herediatry panc genes?
PRSS1, SPINK1
deal with trypsinogen
