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27 Cards in this Set
- Front
- Back
Normal phenotype for alpha1 anti-trypsin?
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PiMM
(the M allelle of the protease inhibitor = PI) |
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Most common alleles of people with a1 antitrypsin def.?
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PiZ and PiS
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Clincial findings in neonates with a1 antitrypsin def?
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cholestasis; may resemble extrahepatic bile duct atresia due to paucity of interlobular bile ducts
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Location of PAS-D positive globular inclusions in a1 anti-trypsin?
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Peri-portal
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Histological findings in neonates with alpha 1 antitrypsin def.
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parenchymal giant cells, ductular reaction, fibrosis and sometimes paucity of interlobular bile ducts
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Natural history of alpha one antitrypsin def?
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Neonates with cholestasis which resolves (90%), adults present with emphysema. Adults association with hep b and c. Homozy only have increased HCC. PiZ heteros with chronic liver disease.
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Triad of histology in acute rejection?
Chronic? |
peri-portal mixed infiltrate (eosos are a sign), endothelialitis, and bile ductulitis
Chronic is cont. of acute = ductopenia, loss of arteries, fibrosis, and scant inflammation |
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Histological findings in Autoimmune hepatitis?
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interface hep with plasma cells, and regenerative liver cell rosettes (may be vacuolated)
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Autoantibodies seen in autoimmune hepatitis?
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Type 1: ana or anti-smooth muscle or both
Type 2: anti-LKM Type 3: anti-SLA (soluble liver antigen) |
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How differentiate between von Meyenburg complex and bile duct adenoma?
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VMC have dilated ducts with mucin or bile
Adenoma: normal ducts with nothing in them and lymphocytes on edge of lesion |
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Von Meyenburg complex are associated with which diseases/abnormalities?
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normal liver, congenital hepatic fibrosis, Caroli's syndrome, and adult polycystic kidney disease
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Bile ductular cholestasis is pathoneumonic (in adults) for?
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Sepsis
Usually gram negs: E-coli |
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Primary sclerosing Cholangitis
(PSC) |
75% males, 70% UC
onion skinning of bile ducts followed by fibrosis in later disease hepatitis bm thickening (seen with PAS) of bile ducts increased risk cholangiocarcinoma |
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Budd-Chiari (hepatic vein thrombosis) / venous outflow obstruction histological findings.
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centrolobular fibrosis
lobular or portal thrombosis |
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Location of granulomas in sarcoid of the liver?
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Peri-portal
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Causes of macrovesicular steatosis?
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Ethanol, obesity, diabetes, hyperlipidemia, corticosteroids
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Causes of microvesicular steatosis?
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anti-retrovirals, acute fatty liver of pregnancy, reye's synd., tetracyline toxicity, alchohol foamy degeneration, valproic acid toxicity.
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Kupffer cell predominant iron overload.
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hemolysis, transfusions, or hemodialysis
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Hepatocyte predominant iron overload.
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HFE-related hereditary hemochromotosis
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Both kupffer cell and hepatocyte iron overload?
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Chronic systemic conditions like long-term transfusion
Long term oral iron intake |
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Oral iron intake pattern
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Peri-portal macrophages and kupffer cells.
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Mutation seen in liver cell adenoma?
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Hepatocyte nuclear factor 1 aplph (HVF-1alpha)
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Mutations in cholangiocarcinoma?
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K-ras and c-myc
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Alagille's syndrome?
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Syndromic paucity of intrahepatic bile duct associated with Jagged-1 mutations.
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Staining pattern for HCC
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positive: hep r and afp (diffuse cytoplasmic)
polyclonal CEA - apical Negative: Ck7 and Ck20 |
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paraquat toxicity histopathology?
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bile duct epethlium necrosis.
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Benign recurrent intrahepatic cholestasis.
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bouts of recurrent choestasis
liver findings: bile thrombi in canaliculi mutation in FIC-1 (ABCB11) chrom 18 (transports bile across canalicular mem) |