McGowan Test II Flashcards

(128 cards)

1
Q

CNS defects
hemolysis (low LDH, Anemia, Haptoglobin)
<40yo

is what disease?

A

Wilson’s

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2
Q

tea colored urine and white clay colored stools

A

obstruction in the biliary system

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3
Q
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
A

pancreatic cancer

adenocarcinoma

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4
Q

Crohns, DM, native american race, rapid weight loss are RF for what?

A

gallstones

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5
Q

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

A

spont (primary) bacterial peritonitis

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6
Q

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

A

secondary bacterial peritonitis

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7
Q
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

A

Acute Pancreatitis

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8
Q

Acute Pancreatitis

pathology?

mc cause?

why is there hypocalcemia?

2-3 to dx?

MC imaging follow up? avoid this why?

tx?

A

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

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9
Q

two signs you see on xray of acute panc?

A

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

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10
Q

how to predict severe acute pancretitis?

A

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

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11
Q

complications of acute panc

A
  • 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
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12
Q

emphysematous pancreatitis caused by?

A

could be from c. perfringens, enterobacter aerogenes, enterococcus faecalis

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13
Q
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!
A

Chronic Pancreatitis

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14
Q

Chronic Pancreatitis

mc cause?

dx?

causes?

complications?

A

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

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15
Q

tumefactive chronic pancreatitis is code for?

A

panc ca

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16
Q

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

A

pancreatic insufficiency

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17
Q

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
A

MEN 1

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18
Q

increased bili production, impair bilirubin uptake/storage

A

unconjugated (indirect)

ie. hemolytic syndrome
gilbert syndrome

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19
Q

impaired excretion, hepatocellular dysfxn, biliary obstruction

A

conjugated (direct)

ie dubin johnson syndrome
rotor syndrome
drug rxn
pregnancy
viral hep
crigler najjar
intrahepatic cholestasis of pregnancy
hepatitis, cirrhosis obstruction
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20
Q

prehepatic, hepatic, post hepatic

A

transfusions
sickle cell
thalassemia
autoimmune

hepatitis
ca
cirrhosis 
congenital disorders
drugs
gallstones
inflammation
scar tissue
tumors
block flow of bile into intestines
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21
Q

with prehapatic or unconjugated bili, you should order what test?

A

order CBC (hemolysis!)

  • anemia and thrombocytopenia
  • haptoglobin, reticulocyte count, LDH to look for hemolysis
  • leukocytosis

Peripheral smear - schistocytes, sickle cells

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22
Q
benign, 
asymp, 
hereditary, 
fasting -> increases indirect hyperbilirubinemia, 
abn lab results, no tx, 
reduced mortality from cv disease
reduced UDGT
A

gilbert syndrome

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23
Q
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
A

dubin johnson

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24
Q

reduced hepatic reuptake of bilirubin conjugates

sim to DJ but liver isnt pigmented and gb is visualized on oral cholecystography

A

rotor syndromes

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25
``` 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
26
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)
27
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
28
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
29
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
30
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
31
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
32
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
33
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
34
IgM Anti-HBc
window period
35
IgM Anti-HBc HBsAg HBeAg HBV DNA
acute infection
36
Anti-HBs | IgG Anti-HBc
prior infection
37
HBsAg IgG Anti-HBc HBV DNA?
chronic infx, inactive
38
``` HBsAg IgM Anti-HBc IgG Anti-HBc HBeAg HBV DNA ```
chronic infx, active
39
Anti-HBs
immunization
40
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
41
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
42
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
43
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
44
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
45
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)
46
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
47
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
48
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
49
mc cause jaundice in preg
viral hepatitis
50
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
51
eclampsia is?
HELLP + hyperreflexia + convulsions = eclampsia = life threatening tx: termination of preg
52
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
53
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
54
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
55
Fibrosis check?
serum fibrosure or US elastography
56
``` 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
57
``` 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
58
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
59
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
60
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
61
>32 poor prognosis benefit from steroids involve PT and serum bilirubin
maddreys discriminate function (df)
62
predicts mortality based on multivariable mdoel age, bili, bun, ptt, wbc > 9 then give glucocorticoids for higher survival
glasgow alcoholic hepatitis score
63
> 21 inc significant mortality in alc hepatitis | >14 for liver transplant
model for end stage liver diesase MELD
64
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
65
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
66
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
67
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
68
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
69
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
70
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
71
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
72
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
73
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
74
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
75
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
76
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: 1. mild confusion 2. drowsiness 3. stupor 4. coma
hepatic encephalopathy
77
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
78
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
79
``` 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
80
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)
81
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
82
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
83
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
84
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) ```
85
markers for liver damage
ASL/ALT: increased necrosis and liver cell injury ALP: sensitive to cholestatic biliary obstruction Bilirubin LDH/GGT
86
liver fxn test
PT/INR - w INR tst w VIt K -> corrects, then absorption isuenot disease albumin cholesterol ammonia
87
primary injury to hepatocytes | increased AST/ALT
hepatocellular
88
primary injury to bile ducts | increase ALP/bilirubin
cholestatic | jaundice and pruitius
89
what is most specific test for the liver?
ALT
90
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)
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+ when pt takes deep breath - then stop bc of pain or cough during palp of RUQ
murphy's sign
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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
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RF for cholelithiasis
``` american indians weight loss dm, obese crohns preg oc ```
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protective for cholelithiasis
decreased carbs, increased PA, fitness of CV, coffee, Mg and healthy fats, fiver, statins, and nsaids
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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
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``` 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
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``` 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
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charcot's triad | reynolds pentad
RUQ p + fever/chills + jaundice if reynolds, then add AMS + hypotension
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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
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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
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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
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biliary colic
severe steady ache RUQ or epigastric area | radiates to R scapula
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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
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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
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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
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what is the mc gallbladder congenital anomaly?
folded funus = phrygian cap
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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
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large gallstone erode directly into adjacent loop of small bowel generating intestinal obstruction
gallstone ileus or bouveret syndrome
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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
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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
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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
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MULTIPLE nodules but no fibrous septa looks like cirrhosis portal htn where nodules impede blood flow associated w hiv, rheumatoglogic dz
nodular regenerative hyperplasia
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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
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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
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one of mc adenomas females min risk of -> HCC fatty w no atypia
HNF1-a inactivated adenoma
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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
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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
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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
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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
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rare, nonepithelial tumors caused by vinyl cl, arsenic, thorotrast
angiosarcoma
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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
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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
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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
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hepatic ca tx
surgical resection liver transplantation image guided tumor ablation
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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
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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
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what has the highest inherited pedisposition of panc carcinioma?
peutz jegher syndrome, STK11
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what is the mc herediatry panc genes?
PRSS1, SPINK1 deal with trypsinogen