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144 Cards in this Set
- Front
- Back
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Normal Liver
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1200-1600 grams
blood flows to through porta hepatis (portal vein and hepatic artery) flow thru paranchyma to sinusoids leave through hepatic vein to IVC |
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Functional unit of liver
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zone 1 closest to portal tract - red, detox area
zone 3 closest to central vein - blue, bile, and prone to ischemia sinusoids filled with Kupffer cells space of Disse filled with Ito cells |
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Liver Functions
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glucose homeostasis
sythesize albumin, clotting factors, complement, and BP;s store glycogen, TAGs, Iron, copper, vitamins catabolize hormones and drugs excrete bile |
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Degeneration and intracellular Accumulation
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edema - balloning degeneration
bile - foamy degeneration steatosis - microvesicular (ALD, pregnancy, and Reyes Synd), macrovesicular (ALD, obesity, DM) |
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Necrosis and Apoptosis
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coagulative necrosis with ischemia
apoptosis with toxin or immuno injury centrilobular - ischemia, toxin, drugs piecemeal negrosis bridging, submassive and massive |
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Inflammation
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hepatitis
- neutrophils in alcohol hepatitis - lymphocytes in viral hepatitis granulomatous inflammation - foreign bodies, drugs, organisms |
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Hepatic Fibrosis
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generally irreversible response
starts around portal tracts - central veins then the space of Disse Ito cells - source of extracellular matrix proeins, produces collagen when inflammed |
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Liver Function test
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Albumin
Bilirubin Alkaline phosphatase GGT Transaminases (AST and ALT) |
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Albumin Test
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low levels - low production in Liver
Think chronic liver disease (not a good indicator of acute hepatic dysfunction) -could also be decreased do to malnutrition, protein loss in stool, and burn patients |
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Bilirubin Test
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indicator of hepatic uptake, metabolism, and excretory functions
- increased direct bilirbun seen in: Hepatitis or cirrhosis drug induced and pregnancy induced Dubin Johnson syndrome mechanical obstruction hepatitis and cirrhosis |
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Transaminases
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AST and ALT
best indicator of damage/necrosis ALT is more specific for liver injury AST is also found in muscle and kidney AST is increased out of proportion of ALT in alcohol induced injury |
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Ammonia Tests
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levels increase when liver cant detox - leads to portosytemic shuntings
seen in advanced liver disease hepatic encephalopathy |
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Prothrombin Time
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prolonged times in clotting factor deficiecy or inactivity
- synthesis of vitamin K dependent factors in liver supplement Vit K... if condition improves, its due to fat malabsorption, if it doesn't than its liver disease |
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Serum Globin
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mild hyperglobulinemia with chronic liver disease
increase in autoimmune chronic hepatitis |
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Urinalysis
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urobilinogen - increased in liver damaged patients, decreased in biliary obstruction
BUN - can be decreased in liver disease |
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CBC Tests
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Viral Hepatitis may cause relative or absolute lymphocytosis
increased MCV with liver disease |
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Functions of Bile
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dietary fat absorption
waste excretion - bilirubin and cholesterol |
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Bilirubin
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heme degrades into biliverdin (via heme oxygenase)
biliverdin into indirect bilirubin (via reductase on albumin) IN LIVER bilirubin-albumin conjugation by UGT IN SI bilirubin is deconjugated to urobilinogen by bacteria excreted in feces and in urine |
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Bile Acids
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Cholesterol breakdown product
promotes bile flow and secretion of cholesterol almost all is reabsorbed in ileum |
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Jaundice
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accumulation of bilirubin (direct or indirect)
--unconjungated - insoluble, cannot be excreted excess is due to excess production (hemolytic anemia), reduced uptake (drugs taking albumin binding spot), or impaired conjugation (premature infants) -- conjugated, loosely bound to albumin, excreted in urine. High levels can be due to decreased hepatic excretion, or impaired bile flow |
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Crigler-Najjar Hyperbilirubinemia
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unconjugated type
- crigler-najjar type 1 - fatal within 18 months due to kernicterus (complete absence of bilitubin UGT) - crigler-najjar type 2 - non fatal, partial absence of bilirubin UGT |
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Gilbert Syndrome Hyperbilirubinemia
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Unconjugasted type
AD disorder relatively common, more common in men, 1/2 level of bilirubin UGT usually diagnosed after an illness or strenous exercising |
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Dubin-Johnson Syndrome
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AR - conjugated typed
impaired excretion of conjugated bilirubin from hepatocytes, across canalicular membrane DARK pigemented liver recurrent jaudice - normal life expectancy |
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Rotor Syndrome
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AR - conjugated type
rare assymptomatic liver is not pigmented |
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Cholestasis
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from a bile obstruction
pruritis - bile acids xanthomas - cholesterol increased serum alkaline phosphatase intestinal malabsorption |
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Morphology Cholestestasis
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bile pigemnts in hepatocytes (foamy degeneration)
bile plugs in canaliculi periductal inflammation tract fibrosis and biliary cirrhosis |
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Hepatic Failure
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80-90% of liver function is lost
predisposed by bleeding, infection, surgery, heart failure caused by hepatic necrosis (viral hepatitis or drugs), cirrhosis, Reyes syndrome, toxicity, fatty liver or pregnancy |
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Clinical Features of Hepatic Failure
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Jaundice
Low albumin high ammonia fetor hepaticus high estrogen (causing palmar erythemia and spider veins, and small gonads and breasts in men) |
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Consequences of Hepatic Failure
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Multisystem organ failure
increased coagulation (decrease in fibrinogen, prothrombin, II, VII, IX X) DIC Hepatic encephalopathy (from increase in ammonia) Hepato-Renal syndrome (acute kidney failure due to vasoconstriction) |
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Cirrhosis
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Progressive Fibrosis
Normally: types I and III - collagen in PT and CV Type IV - collagen in space of Disse in Cirrhosis: types I and III - collagen everywhere (increased productions from Ito cells) Stimuli for Cirrhosis: TNF alpha TGF-B Kupffer and Endo cells release cytokines ECM disruption Toxin release |
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Morphology of Cirrhosis
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fibers bridging septa
nodules of regenerating hepatocytes -- Micro or Macro nodules -- disruptions of entire liver architecture |
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Clinical Features of Cirrhosis
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might be assymptomatic
weigh loss, anorexia, fatigue death eventually from liver failure - portal hypertension - or cancer |
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Portal Hypertension
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due to obstruction
prehepatic - PV thrombosis, splenomegaly posthepatic - right sided heart failure, HV obstruction intrahepatic - cirrhosis |
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Etiology of Portal Hypertension
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- resistance to portal flow within sinusoids
- fibrosis around central vein - expansion from regenerating nodules - abnormal anastomosises |
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Clinical Features of Portal Hypertension
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ascities
porto-systemic venous shunts (at esophagus and caput medusae) congestive splenomegaly hepatic encephalopathy |
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Ascites
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excess serous fluid in peritoneal cavity
increase in PMN's increase in RBC's can cause a hydrothorax |
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Pathogenesis of Ascites
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sinusoidal hypertension - fluid moves into space of Disse (lymphatics)
lymph fluid moves into peritoneal cavity can also be due to an osmotic gradient |
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Portosystemic shunts
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3 major sites
- rectum - hemorrhoids - gastroesophageal junction - massive bleedings which can be fatal in advanced cirrhosis - caput medusae - falciform ligament of liver |
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Splenomegaly
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may turn into hyper-spleen activity (removal of cells and platelets)
dilated sinusoids and bleedings |
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Liver Inflammatory disorders
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very common
infections are very common - viruses most common, but also think about TB, Staph, Salmonella, and Candida |
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Viral Hepatitis
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Think about Mononucleosis (EBV) in teens
Thinks CMV, HSV, adenovirus, rubella or enterovirus in newborns or immunosuppressed classic Hep. Viruses |
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Hepatitis A
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infectious hepatitis
usually mild - self limiting 1/2 of people are sero positive by 50 1/4 of worldwide acute hepatitis ssRNA picornavirus |
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Transmission of Hepatitis A
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fecal - oral route
can be aquired from shellfish fever - malaise - anorexia (flu like illness) does not create a carrier state or cause chronic hepatitis |
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Serology of Hepatitis A
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IgM appears first with symptoms
- acute infection - decreases after months IgG appears after IgM - Indicates recovery - lifelong immunity |
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Hepatitis B
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"serum hepatitis"
can cause different clinical scernarios -assymptomatic, acute, chronic progressive and non progressive, cirrhosis, and massive necrosis and failure over a million infected in US enveloped dsDNA Hepadneavirus - in "Dane particle" -HBsAg infective glycoprotein |
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Transmission of Hepatitis B
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paranteral or close contact
blood transfusion, dialysis, needle sticks, sexual transmission, transmission to fetus present in all fluids except stool proliferative phase and integrative phase |
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Serology of Hepatitis B
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HBsAg - shows befores symptoms begin, peaks with symptoms
HBeAg, HBV-DNA, DNA polymerase - after HBsAg, but before symptoms, indicate viral replication IgM HBcAb - window period indicator, marker for acute infection HBeAb - indicates waning disease HBsAb - begining of active disease - appears after HBsAg disapears |
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Carrier and Chronic Hep. B
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5-10% are assymptomatic carriers
viral replication is halted chronic Hep B - in 5% of people HBaAg still around past 6 months of infection. Other antigens still present as well |
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Hepatitis C
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major cause worldwide
enveloped ssRNA flavivirus multiple subtypes and genotypes vaccination is difficult parenteral transmission - illicit IV drug abuse more frequently progresses to chronic hepatitis and cirrhosis, also carcinoma of the liver |
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Serology of Hepatitis C
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HCV RNA in acute infection
- increased transaminases - no immunity to virus develops HCV RNA in chronic infection - remains elevated - episodic elevations in LFT's (with flu symptoms) - intervening normal periods |
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Hepatitis D
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dependent enveloped ssRNA virus
defective replication ONLY causes infection in the presence of HBV acute infection with ACITE HBV - recover with immunity superinfection with CHRONIC HBV - very likely to develop cirrhosis paraenteral transmission, similar course to HBV |
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Serology of Hepatitis D
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with ACUTE HBV infection:
- HDV RNA, HDVAg, plus all the HBV antigens - disapears in 6 months IgM HDVAb - most reliable indicator of recent exposure IgM HDVAb AND IgM HBVAb indicator of recent co-infection in HDV superinfection, HDV Ag's persist longer IgG develops LFT's are elevated |
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Hepatitis E
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uneveloped ssRNA calcivirus
enterically transmitted, water borne infection (fecal oral route) young and middle aged adults high mortality in pregnant women no carrier state or chronic infection |
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Serology of Hepatitis E
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HEV RNA and HEVAg detected in stool before symptoms
IgM HEVAb detected at onset of symptoms IgG HEVAb replaces IgM in a couple of weeks |
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Carrier State Hepatitis
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HBV, HCV, HDV
- assymptomatic - HBV most common - leads to transmission to fetus - think of immunocomprimised patients |
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Assymptomatic Infection of Hepatitis
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mild increase in LFT's
or positive Ab serology post infection |
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Acute Viral Hepatitis
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4 phases:
- incubation period - symptomatic preicteric phase (flu - like) - symptomatic icteric phase (jaundice, dark urine and light stools, pruritis, icterus may or may not be present) - peak infectivity |
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Chronic Viral Hepatitis
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evidence of infection lasting longer than 6 months
histological evidence of inflammation or necrosis Dx: almost always viral DDx: Wilson's disease, A1-AT def., alcohol, drugs, AI HCV - increased propensity for chronic disease and cirrhosis See spider veins, palmar erythema, hepatosplenomegaly, elevated LFT's can lead to an immune complex disease - kidney failure |
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Histology of Hepatitis
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ground glass appearance of hepatocytes due to HBsAg
fatty change - lymph aggregates in HCV Acute - Balloning degeneration, cholestasis, necrosis, apoptosis (councilman bodies), HCV-bile duct proliferation Chronic portal tract inflammation, lobular inflammation, PT fibrosis (w/ nodules) |
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Fulminant Hepatis
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progresses to hepatic encephalopathy
could be from any of the hepatitis viruses. drug toxicity (tylenol, INH, MAOs) rarely due to ischemia, Wilsons, heat stroke, steatosis, malinancy |
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Morphology of Fulminant Hepatitis
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necrosis throughout the liver
liver SHRINKS and becomes wrinkled soft, muddy red surface when cut complete lobular destruction but preserved PT's regeneration is zonal and organized or massive and disorderly |
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Clinical Features of Fulminant Hepatitis
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Jaundice
encephalopathy fetor hepaticus DIC and bleeding CV problems Kidney failure ARDS Sepsis likely to die without transplant |
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Non Viral Liver Infections
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Staph and Salmonella
Parasites: Entamoeba, Ascaris, Plasmodium, schistosoma, Strongyloides, Cryptosporidia, Echinococcus form abscesses more common in developing countries |
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Liver Abscesses
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secondary to GI or other infection
past abscesses - from appendicitis, diverticulitis, colitis (portal) present day - artery spread or from biliary tree infections arterial or portal - multiple small abscesses direct invasion/trauma - single large abscess can lead to empyema or lung abscesses |
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Clinical Manifestation and Treatment of Liver Abscesses
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fever
RUQ pain jaundice treat by surgically draining (not for amoebic disease) and proper medication |
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Autoimmune Hepatitis
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chronic hepatitis + immuno condition
looks same as chronic viral infection histologically young/perimenopausal women have negatve hepatitis markers and increased IgG levels (autoantibodies - ANA and ASMA) increased likelihood in HLA-B8 or HLA-DRw3 associated with RA, thyroiditis, Sjogren's, UC |
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Drug and Toxin induced Liver Disease
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hepatocyte necrosis - cholestasis - or insidous onset liver dysfunction
drug induced hepatitis is histologically indistinguishable to chronic viral hepatitis - (must use serology) |
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Drugs to ask for in History for Liver Disease
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Acetaminophin
Tetracycline Amanita toxin (mushrooms) CCl4 ALCOHOL chlorpromazine, sulfonamides, halothane |
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Alcohol liver disease
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leading cause of liver disease in west
steatosis - hepatitis - cirrhosis moderate intake - microvesicular chronic intake - macrovesicular starts out Centrilobular - moves outward apears to be soft and yellow (reversible) fibrosis is irreversible |
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Morphology of Alcoholic Hepatitis
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balloon degeneration and necrosis
Mallory bodies (tangles of keratin and IF) lobular neutrophillic infiltrate cholestasis |
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Morphology of Alcoholic Cirrhosis
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evolves slowly but is irreversible
yellow/tan liver -> shrunken brown liver micronodules -> macronodules ischemic necrosis and fibrous obliteration of nodules -> scars cholestasis |
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Alcoholic Steatosis
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excess NADH -> shunting toward lipid synthesis
impaired lipoprotein synthesis and secretion increased peripheral fat catabolism |
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Pathogenesis of Alcoholic synthesis
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CYP450 induction -> toxic metabolites
free radicals -> impair surface proteins microtubes and mitochondria stop to work properly acetaldehyde -> lipid peroxidation -> disrupts cytoskeleton |
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Pathogenesis of Alcoholic Cirrhosis
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fibrosis from collagen deposits by ITO (stellate) cells and Kupffer cell activation -> neutrophil activation
leads to malnution and wasting GI dysfunction |
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Clinical manifestations of alcoholic Steatosis
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alcoholic steatosis - hepatomegaly, hyperbilirubinemia, increase in Alk. Phos.
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Clinical manifestations of Alcoholic Hepatitis
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alcoholic hepatitis - malaise, anorexia, weight loss, RUQ pain, increase in ALT and AST, increase in WBC's
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Clinical Manifestations of Alcoholc Cirrhosis
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malaise weakness weight loss
PORTAL HYPERTENSION jaundice increase in estrogen increase in transaminases, bilirubin, alk. phos. ANEMIA |
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End Stage Alcoholic Liver Disease
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hepatic coma
massive GI hemorrhage sepsis hepatorenal syndrome hepatocellular carcinoma |
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Non-Alcoholic Steatohepatitis
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resembles alcoholic version
different etiology obesity type II DM hypertriglyceridemia abdominal surgery rarely develop cirrhosis |
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Genetic Hemochromatosis
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AR disease - 6p gene
more common in men symptoms occur late in life - after a lifetime of iron deposits defective dietary Fe absorption - iron accumulates - sxs begin after 20 grams accumulate - iron leads to defective collagen formation and interacts with DNA - increases likelihood for Hepatocellular Carcinoma |
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Morphology of Genetic Hemochromatosis
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enlarged - red brown
hemosiderin deposits in liver, pancrease, heart, pituitary, and other edocrine organs - begins in periportal hepatocytes -> lobule ducts and Kupffer cirrhosis, pancreatic fibrosis and atrophy - NO inflammation\ heart - enlarged/pigmented skin - pigmentation/increased melanin joints - synovitis testes 0 atrophy/NO pigmentation |
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Diagnosis of Genetic Hemochromatosis
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determine iron concentration in unfixed tissue
GH will have iron levels > 10K ug/gm levels > 22K have cirrhosis |
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Clinical Manifestations of Genetic Hemachromatosis
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symptoms begin after people turn 40
abd. pain, skin pigmentation, and DM screen for increased iron and ferritin, HLA analysis, family member screenings - to prevent irreversible damage treat with phlebotomies - risk for HCC increase 200 fold |
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Wilson's Disease
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AR - chromo 13
accumulation of Copper in brain, liver, and eyes pathological changes by 5 years old fatty change in liver, hepatitis, -- see balloon degenerating and Mallory bodies diagnose by measuring copper in liver brain has cavitation of white matter, and atrophy of grey mater eyes have Kayser-Fleisher rings |
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Clinical Manifestations of Wilsons Disease
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onset of sxs after age 5 and usually before adolescence
acute and chronic liver disease psychosis Parkinson's like tremors hemolytic anemia decrease in ceuloplasmin - increase in liver copper and urinary copper treat with chelation (D penicillamine) liver transplant in cirrhosis patients NO increased risk of HCC |
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A1AT deficiency
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AR - chromo 14
PiMM - most common genotype PiMZ - intermediate levels PiZZ - extremely decreased A1AT abnormally folded A1AT accumulates in hepatocytes - hepatomegaly cells have round, PAS positive, eosinophillic inclusions PiZZ - hepatitis, cholestasis, fibrosis, cirrhosis neonates have jaundice/hepatits often - be aware to test for deficiency |
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Neonatal Cholestasis/Hepatitis
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prolonged conjugated hyperbillirubinemia
caused by bile duct obstruction or atresia infections, toxins CF, A1AT def. |
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Morphology of Newborn Cholestasis/Hepatitis
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architectural disarray
cholestasis portal inflammation panlobar giant cell transformation extramedullary hematopoiesis |
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Clinical Manifestations of Newborn Cholestasis/Hepatitis
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jaundice
dark urine light stools hepatomegaly |
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Reye's Syndrome
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hepatic failure - without inflammationg/necrosis
encephalopathy - without inflammation febrile illness in children under 4 - correlated with aspirin therapy mitochondrial abnormality in tissue microvesicular steatosis without necrosis kids vomit, irritable, lethargic, hepatomegally, sometimes with NORMAL labs treat symptoms and supportive therapy |
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Hepatocellular carcinoma
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Fibromellar - young adults, not associated with HBV or cirrhosis
- single mass with fibrous bands - nests of polygonal cells seperated by dense collagen - better prognosis |
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Cholangiocarcinoma
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not caused by HCC risk factors
associated with Thorotrast exposure and Opisthorchis infection (orient) not bile stained, MD with glandular and tubular structurs - lesser tendency to invade vasculature -greater tendency for metastases DEATH within 6 months |
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Metastatic Tumors
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far more common than primary tumors
usually from breast, lung, and colon - multiple necrotic nodules - patients jaundice and increase in LFTs |
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Cholelithiasis
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95% of biliary tract disease
cholesterol stones - western world pigmented stones - 3rd world native Americans, caucasian women, OC's and pregnancy, obesity and rapid wt. loss, prolonged fasting - all INCREASE risk for stones hemolytic syndromes, ileal dysfunction, bypass surgery, and infections- INCREASE risk for PIGMENTED STONES |
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Pathogenesis of Cholelithiasis
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Cholesterol stones - concentrated cholesterol supersaturates bile
- hypomotility causes increase in mucus and bile sequesteration pigment stones - increase in calcium salts form an increase in unconjugated bilirubin - can be from BT infections (E coli, Ascaris, OPisthirchis) - can be from intravascular hemolysis (sickle cell anemia) |
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Morphology of CHolelithiasis
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cholesterol stones - only in GB
- yellow/grey stones pigments stones - black - in sterile GB bile, are radiopaque - brown - from infected ducts, and radiolucent |
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Clinical Manifestations of Cholelithiasis
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mostly are assymptomatic
- can have colicky RUQ pain or referred pain can cause empyema, perforation, fistula or pancreatitis small stones obstruct large stones cause ileus - erode bowel |
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Acute Calculous Chloecystitis
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acute inflammation of GB
usually have stone obstructing neck or cystic duct can be caused by an inflammed duct that lodges stone mucus layer is disrupted and bile salts act as detergent on epithelium |
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Acute Acalculous cholecystitis
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no stones
severly ill patients - post op patients - post partum ischemic comprimise of GB mucosa secondary to sever illness - dehydration, transfusions, GB stasis, obstruction, or infection |
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Morphology of Cholecystitis
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enlarged, tense, red, violaceous, to green/black/necrotic
vascular congestion and possible abscesses |
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Clinical Manifestations of Acute Calculous Cholescystitis
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mild to moderate pain - severe colicky pain in RUQ
- may need surgical evaluation - increase in WBC's, increase in Alkaline Phos. may subside on its own |
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Clinical Manifestations of Acute Acalculous cholescystitis
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more insidous onset
dx obscured by underlying illness must have high index of suspicion |
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Chronic Cholecystitis
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usually associated with stones
similar symptoms nausea vomiting and food intolerance "porcelain" GB- dystrophic calcification - increase incidence of carcinoma can lead to infections, sepsis, perforation, rupture, and fistula with ileus |
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Ascending Cholangitis
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bacterial infection within hepatic ducts
(klebsiella, clostridium, bacteroides, enterobacter, D strep) pain, fever, chills, jaundice acute inflammation |
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Extrahepatic Biliary Atresia
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destruction or abscence of biliary tree
common cause of childhood death most people need liver transplants periductal inflammation and destruction of intrahepatic BDs - caused by the destruction or inflammation of hepatic or CBD -cholestasis and cirrhosis will follow jaundiced babies with normal birth weights acholic stools increase in serum bilirubin increase in Alk Phos |
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Subtypes of EHBA
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type 1 - CBD only
type 2 - hepatic bile dicts type 3 - MOST COMMON - obstruction of bile duct above porta hepatis - NOT surgically correctable, need transplant |
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Choledochal cysts
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common dilation of bile duct
abdominal pain and jaundice in childhood more common in females INCREASE risk of cholangiocarcinoma nad BD carcinoma |
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Gallbladder Carcinoma
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females more common - in 70's
gallstones almost always present adenocarcinomas - form glands - infiltrative type and exophytic type similar symptoms to cholelithiasis can be metatstatic very very very low survival |
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Extrahepatic BDC
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uncommon
usually older men - less common with stones - increased risk for clonorchis infection, sclerosising cholangitits, UC, fibrosis and cysts small tumor in the BD wall at the confluence of the right and left hepatic ducts (Klatskin Tumors) jaundice, nausea, vomiting, acholic stools increase in TA's and Alk Phos not usually resectable |
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Primary Biliary Cirrhosis
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inflammatory duct disease with destruction of medium sized live ducts
middle aged women - most common increased risk with familial links Autoimmune disease with genetic and environmental factors Patients often have other AI disorders (Sjogren, RA) |
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Morphology of Primary Biliary Cirrhosis
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focal and variable disease through the liver
liver enlarges -> then shrinks - precrirrhotic stage - mononuclear infiltrate and noncaseating granulomas (florid duct lesions) - scarring -> bridging fibrosis - next, bile duct proliferation, inflammation and necrosis - liver becomes bile stained and cirrhotic |
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Clinical Manifestations of Primary Biliary Cirrhosis
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insidious fatigue, pruritis, abdominal discomfort, hepatomegaly
xanthelasmas, steatorrhea, bone problems, hyperpigmented skin chronic liver disease and jaundice are late signs increase in Alk Phos and cholesterol antimitochondrial antibodies increased risk for hepatocellular carcinoma people die of liver failure, hemorrhage and infection treat with ursodeoxycholic acid or liver transplant |
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Secondary Biliary Cirrhosis
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from extrahepatic biliary blockage
- biliary tree or pancreatic tumors, strictures, atresia, CF, cysts cholestasis is reversible with correction of obstruction - uncorrected, fibrosis, cirrhosis develops - liver becomes bile tinted - feathery degeneration with bile lakes |
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Primary Sclerosing Cholangitis
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inflammation and obliterative fibrosis of bile ducts - with dilated/preserved segments (beaded appearance)
immune mediated bile duct injury ANA, ASMA, RF, and p-ANCA |
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Morphology of Primary Sclerosing Cholangitis
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onion skin fibrosis
lymphocytic infiltrate and bile duct epithelial atrophy liver becomes cholestatic and cirrhotic |
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Clinical Presentation of Primary Sclerosing Cholangitis
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onset of fatigue, pruritis, and jaundice --> weight loss and portal hypertension
increased Alk Phos and conjugated bilirubin increased risk of cholangiocarcinoma no specific treatment --> must transplant |
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Von Meyenburg Complexes
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bile duct harmatomas
small clusters of moderately dilated bile ducts in fibrous stroma seen within or around portal tract associated with PKD |
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Polycystic Liver Disease
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few to hundreds of cysts - with straw colored fluid
associated with PKD |
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Congenital Hepatic Fibrosis
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irregular bands of collagen in portal tracts
abnormal bile ducts associated with PKD develop portal hypertension increased risk of Cholangiocarcinoma |
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Caroli Disease
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dilated larder intrahepatic bile ducts with bile
portal tract fibrosis associated with PKD, cholelithiasis, cholangitis, abscesses, and portal hypertension |
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Alagille Syndrome
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AD malformation - No bile ducts
mutated Jagged gene increased risk of hepatic failure and hepatocellular carcinoma |
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Hepatic Artery Comprimise
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thrombosis or compression
liver rarely infacts due to dual circulation - however, transplanted liver can infarct more easily |
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Portal Vein Obstruction and thrombosis
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portal hypertension
can cause bowel infarction caused by sepsis, DIC, trauma, tumors, pancreatitis |
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Intrahepatic Circulatory Problems
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from cirrhosis, sickle cell disease, DIC, tumors
passive congestion of centrilobular sinusoids - centrilobular necrosis "nutmeg liver" also can have blood filled cystic spaces - caused by bartonella infections in aids patients |
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Hepatic Vein Thrombosis
(Bud Chiari Syndrome) |
obstruction of 2 or more hepatic veins
associated myeloproliferative disorders, hypercoagulability, pregnancy and OC use enlarges red/purple liver 0 centilobular congestion and necrosis leads to hepatomegaly, ascites, abdominal pain untreated leads to death |
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Sinusoidal Obstruction Syndrome
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allergic bone marrow transplants
also seen with certain chemotherapies -- toxic endothelial cell damage obliteration of hepatic vein --> collagen deposits --> congestion and necrosis --> fibrosis hepatomegaly, ascites, weight gain, jaundice |
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Graft Verses Host Disease
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Acute - chronic portal and lubular inflammation
necrosis of hepatocytes -- lymphocytes attack host cells -- Chronic - portal inflammation with bile duct duct destruction and fibrosis - endothelitis |
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Preeclampsia
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maternal hypertension, proteinuria, edema, cogaulation, DIC
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Eclempsia
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preeclampsia + hyperreflexia and convulsions
liver has small hemorrhages periportal fibrin deposition hemorrhage in space of Disse --> periportal coagulative necrosis increased tranaminases increased bilirubin coagulopathy may lead to preterm forced delivery |
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Acute Fatty Liver of Pregnancy
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microvesicular fatty change
may present like viral hepatitis bleeding, nausea, vomitting, jaundice and coma dx by liver biopsy tx by baby delivery |
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Intrahepatic cholestasis of Pregnancy
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benign condition
from hormonal imbalances and defective bile excretion pruritis, dark urine, acholic stools and jaundice mildly elevated conjugated bilirubin and alk phos |
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Focal Nodular Hyperplasia
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well demarcated area - grey white stellate scar with radiating fibers
septa have lymphocytic infiltrate and BD proliferation |
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Nodular regenerative Hyperplasia
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affeccts ENTIRE liver
without dense fibrosis nodules of plump hepatocytes with a rim of atrophic hepatocytes associated with organ and BM transplant causes portal hypertension |
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Cavernous Hemangiomas
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small - directly below capsule
don't perform blind biopsy because growths have dense vasculature |
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Hepatic Adenoma
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women taking OC
may be mistaken for hepatocellular carcinoma rarely transform into carcinomas well demarcated yellow-tan lesion absent portal tracts |
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Hepatoblastoma
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most common tumor of young childhood
enlarging abdomen, vomitting and failure to thrive increasing AFP |
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Angiosarcoma
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associated with vinyl chloride, arsenic or thorotrast exposure
hemorrhagic nodules spindle shaped neoplasm hepatomegally, jaundice, and ascites |
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Hepatocellular Carcinoma
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HBV, HCV
chronic alcoholism non-alcoholic steatohepatitis food toxins can have 1 mass, or many, or diffuse tumor is paler than surrounding parenchyma vascular invasion and extensive spread dysplasia + mutation repair damage + viral DNA inclusion |
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HBV and HCC
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high incidence in SE Asia and Africa
younger onset of tumors, and cirrhosis is much less common low incidence in western world late onset and cirrhosis is more common |
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Clinical Manifestations of HCC
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ill defined RUQ pain, malaise, fatigue, weight loss, possible masses
elevated AFP death from cachexia, bleeding, liver failure |
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FIbrolamellar HCC
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young adults
no assoiation with chronic liver disease single firm, large lesion, with fibrous cords, eosinophillic cytoplasm |
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Cholangiocarcinoma
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Risck factors include cholangitits, hepatic fibrosis, Caroli disease, thorotrast exposure and Opisthorchis sinesis infection
IL6 over expression - activation of AKT and MCL1 KRAS expression and p53 decreased |
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Morphology of Cholangiocarcinoma
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extrahepatic forms - gritty nodules, Klatskin tumors
intrahepatic forms - gritty cut surfaces more likely to show hematogenous spread to lungs, bones, adrenals, and brain |
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Clinical Presentation of Cholangiocarcinoma
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intrahepatic forms - late detection
extrahepatic forms - pain biliary obstruction, and cholangitis |
