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Ch 18 Liver Path

LECOM Ch 18 Liver hepatic path robbins

Causes of Pre-hepatic portal HTN (3) obstructive thrombus, narrowing of portal vein, massive splenomegaly
Most common causes of Intrahepatic portal HTN cirrhosis
Causes of post-hepatic portal HTN (3) R sided heart failure, constrictive pericarditis, hepatic vein outflow obstruction
Bile is used to excrete 3 things: bilirubin, cholesterol, xenobiotics
Neo-nate w/ normal looking liver, super high unconjugated bili, colorless bile. Dies. Disease and genetic defect AD: Crigler-Nijar Type 1. Absent UGT1A1. Kernicterous
Only monoglucoronidated bilirubin, yellow skin. Cause and name of Dsx AR: Mild UGT1A1 activity, Crigler-Najjar type 2
Mild fluctuating uconjugated hyperbilirubinemia, with stress, no hemolysis or liver disease Name and inheiritance Gilbert Syndrome AR; Decreased UGT1A1
Chronic conjugated hyperbilirubinemia, dark pigmented hepatocyte cytoplasm. Name and genetic defect AR; Dubin-Johnson syndrome, MDRP-2 loss
Chronic conjugated hyperbilirubinemia, normal liver. Dx Rotor Syndrome
Green-brown plugs in dilated bile canaliculi Cholestasis
BUZZ: “Feathery degeneration” cholestasis, bile droplets in hepatocytes
Bile lakes filled with cellular debris and pigment in addition to feathery degeneration points to chronic or prolonged cholestasis
With cholestasis what enzyme is characteristically elevated? GGT
GGT levels in PFIC-1, 2, and 3 Normal; normal; High
Infantile cholestasis, normal GGT, pruritis, high serum bile acid levels, not ductal proliferation, liver failure by adulthood. Disease and faulty gene PFIC-1, ATP8B1
Infant cholestasis, normal GGT, pruritis, growth failure, cirrhosis by age 10. Dx and mutation PFIC-2 and ABCB11
Cholestatis, HIGH GGT, toxic destruction of hepatic epithelia. Dx and mutation PFIC-3; ABCB4
necrosis with portal bridging chronic hepatitis
which type of hepatitis is associated with focal, lobular macrovesicular steatosis HCV
which type of hepatitis is associated with highest incidence of chronic hepatitis HCV
focal hepatocyte necrosis, giant cell transformation, cholestasis, portal mononuclear cell infiltrate, extramedullary hematopoiesis hepatitis
persistence of HBsAg in serum >6mo carrier state
Name this viral hepatitis: - enveloped ds DNA HBV
Name this viral hepatitis:- fecal-oral transmission HAV
Name this viral hepatitis:- water borne HEV
Name this viral hepatitis:- long incubation period HCV; HBV
Name this viral hepatitis:- no carrier state HAV
Name this viral hepatitis: - no chronic hepatitis HAV; HEV
Which phase of viral hepatitis is this? (prodrome, jaundice, recovery) - fever, painful hepatomegaly - increase serum transaminase - atypical lymphocytosis prodrome
Which phase of viral hepatitis is this? (prodrome, jaundice, recovery) - increased urine bilirubin and urobiliniogen - IgM antibodies jaundice
Which phase of viral hepatitis is this? (prodrome, jaundice, recovery) - IgG antibodies recovery
Which viral hepatitis is this? - DNA polymerase with reverse transcriptase activity HBV
Major causes of fulminant hepatitis. - drugs: acetominophen, isoniazid, MAOI, halothane, methyldopa - toxins: mushroom - HBV
What is the most important prognostic factor of chronic hepatitis? etiology
Name some organisms that cause hepatocellular carcinoma. - HBV - Aflatoxin from food spoilage mold
Hepatocytes with speres and tubules in cytoplasm, “ground glass” hepatocytes. Which virus? HBV
shrunken liver with a wrinkled capsular surface and microscopically shows massive irregular areas of necrosis acute fulminant hepatitis
Clusters of plasma cells in portal tract/hepatic lobule interface is character istic of what autoimmune hepatitis
Hepatitis w/ elevated serum IgG, gamma globulin, and ANA, SMA, AAA, or anti-SLA/LP Autoimmune hepatitis type 1
Hepatitis w/ elevated serum IgG, gamma globulin, and ALKM-1, ALC-1 Autoimmune hepatitis type 2
Most common cause of fulminant hepatitis in the USA drug induced
Mallory bodies are present with :(6) alcoholic hepatitis, NAFLD, PBC, Wilson disease, chronic cholestatic syndromes, and hepatocellular tumors
Most common cause of chronic liver disease in the USA? NAFLD
Steatohepatitis is suspected. Macro/micro vesicular steatosis, multifocal parenchymal inflammation, Mallory bodies, sinusoidal fibrosis, neutrophils, AST/ALT=1 Dx NASH
AST > ALT AST is present in mitochondria: AST>ALT indicates alcohol hepatitis since alcohol damages mitochondria
ALT>AST viral hepatitis
portal fibrosis and cirrhosis liver cells filled with blue granules on Prussian blue stain Hereditary hemochromatosis
What is this liver condition? - micronodular cirrhosis - DM - slate gray skin - hepatomegaly - arrhythmias - atypical arthritis - hypogonadism hemochromatosis - destruction of pancreatic islet cell (DM) - iron deposit in myocardium, pituitary gland, adrenal gland, thyroid, parathyroid, joints, skin.
What is the treatment for Wilson's disease? Cu chelator (D-penicillamine)
What is this disease? - behavioral change - psychosis - parkinson like syndrome - Kayser Fleischer ring Wilson's disease
What is this disease? - decreased serum ceruloplasmin - increase hepatic Cu - increased urinary Cu secretion - mutated ATP7B gene (CH13) Wilson's disease - mutated transmembrane Cu transporting ATPase on canaliculi membrane - Cu spill over around age 5
liver is diffusely nodular with small, uniform nodules and microscopically shows diffuse fibrosis encircling regenerative nodules; liver cells contain PAS-pos globular cytoplasmic inclusions alpha1 anti-trypsin deficiency
What is the most sensitive test for recent alcohol intake and is used to monitor those in treatment for abuse? GGT
What is this liver condition? Morphology - fatty change: micro and macrovesicular - fibrosis - mallory body alcoholic liver disease
What is this condition? - cause neonatal hepatitis with cholestatic jaundice - PAS positive cytoplasmic globular inclusions - emphysema alpha-1-AT deficiency
What is this condition? - neonates with jaundice, dark urine, light and acholic stool - panlobular giant cell transformation (rossettes) neonatal cholestasis - 50% idiopathic - 20% biliary atresia - 15% alpha-1-AT deficiency
lg liver, yellow and greasy; preservation of architecture and marked fatty change alcoholic fatty liver
liver fluke Clonorchis sinensis predisposes to ____ carcinoma bile duct
granulomatous bile duct destruction primary biliary cirrhosis
antimitochondrial Ab primary biliary cirrhosis
obstructive jaundice, granulomatous destruction of bile ducts primary biliary cirrhosis
concentric bile duct fibrosis “onion-skin fibrosis” sclerosing cholangitis often associated with inflammatory bowel
Pathogenesis of secondary biliary cirrhosis. biliary obstruction -> secondary infection -> periportal fibrosis -> hepatic scarring, nodular formation -> bridging fibrosis -> cirrhosis
Pathogenesis of primary biliary cirrhosis. - aberrant MHCII on bile duct epithelial cells -> autoreactive T cells -> hyperagammaglobulinemia with complication activation and circulating immune complexes (IgM) - anti-mitochondrial antibodies
Etiology of secondary biliary cirrhosis. - gallstones - tumors of biliary tree and head of pancreas - stricture - in children: biliary atresia, CF, choledochal cysts, paucity of bile duct
What is this disease? - gross: fine granular appearance - micro: portal edema with neutrophils, periductal fibrosis, mononuclear infiltrate secondary biliary cirrhosis
Name a complication of secondary biliary cirrhosis. ascending cholangitis
What is the strongest indicator for prognosis in primary biliary cirrhosis? degree of fibrosis
What is this disease? - hyperagammaglobulinemia - increased circualting immune complex (IgM) Morphology - early: portal mononuclear inflammation with granulomatous response - late: fibrosis primary biliary cirrhosis
What is this disease? Morphology - bile duct with onion skin periductal fibrosis and mononuclear infiltrate Cholangiography: - beaded bile ducts with strictures Primary sclerosing cholangitis - chronic disease with inflammation, obliterative fibrosis, segmental constriction of intra- and extra- hepatic bile ducts
MCC ascending cholangitis E.coli; MC risk factor is obstruction from lithiasis
centrilobular necrosis hepatic congestion with R-sided CHF
MCC Budd-Chiari syndrome Polycythemia vera
Gross appearance of a liver of an individual with cor pulmonale nutmeg caused by chronic passive congestion from right-sided heart failure
liver is diffusely nodular, green w/ small, uniform nodules; fibrous bridging between portal areas biliary cirrhosis
30yo pregnant female previously on oral contraceptives presents with severe abdominal pain. US shows well-circumscribed subcapsular hepatic mass hepatic adenoma
Pathogenesis of hepatocellular carcinoma. repeated cycle of liver cell death and regeneration -> more opportunity for gene mutation
Which of the following class of hepatocellular carcinoma has the best prognosis? - well differentiated - moderately differentiated - poorly differentiated - fibrolamellar variant - fibrolamellar variant well differentiated cell separated by dense collagen
What is this malignancy? - moderately differentiated adenocarcinoma - distorted ducts with prominent sclerotic stroma - primary mass with numerous intrahepatic satellite tumors bile duct carcinoma
Complication of liver adenoma. rupture of subcapsular tumor (especially pregnancy) -> intraperitoneal hemorrhage
What is this neoplasm? - highly vascular - well circumscribed - can't see bile ducts, central vein - thickened plates (1-2 cell layers) liver adenoma
What is this neoplasm? gross: - yellow nodule with a central scarr micro:- central fibrous scar focal nodular hyperplasia- 85% female
What is this neoplasm? - gross: spongy, red-purple mass with hemorrhage and fibrosis - micro: dialted vessels lined by single layer endothelial cells. Hemangioma
What is this neoplasm? - diffuse spherical nodules of regenerating hepatocytes, arising in non-cirrhotic liver - nodules not separated by fibrous septae nodular regenerative hyperplasia
Which condition exhibit both macro- and micro-vesicular steatosis? alcoholic fatty liver
Which condition would you think of if you see periportal necrosis? eclampsia - periportal sinusoids contain fibrin deposits with hemorrhage into space of Disse
How to treat eclampsia? Termination of pregnancy
What intrahepatic biliary architectual anomaly is associated with AD PKD? polycystic liver disease
What intrahepatic biliary architectual anomaly is associated with AR PKD? congenital hepatic fibrosis - abdominal pain on stooping
What is this intrahepatic biliary architectual anomaly? - clusters of dilated bile ducts in/near portal tract- no clinical significance von meyenburg complexes
What is this intrahepatic biliary architectual anomaly? - multiple diffuse cysts detached drom biliary tree - abdominal pain on stooping - associated with AD PKD polycystic liver disease
What is this intrahepatic biliary architectual anomaly? - extensive fibrosis with abnormally shaped bile ducts - associated with AR PKD - complication: portal HTN, bleeding varices congenital hepatic fibrosis
What is this intrahepatic biliary architectual anomaly? - larger bile ducts segmentally dilated with inspissated bile - often seen with congenital hepatic fibrosis - complication: gallstones, cholangitis, hepatic abscess, portal HTN Caroli disease
Which circulatory disorder of the liver is this? - portal HTN with esophageal varices - splenomegaly - ascites - infarction of intestines portal vein thrombosis
Created by: csheck