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Ch 18 Liver Path
LECOM Ch 18 Liver hepatic path robbins
Question | Answer |
---|---|
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 |