Liver Pathology

Front 1

Normal phenotype for alpha1 anti-trypsin?

Back 1

PiMM

(the M allelle of the protease inhibitor = PI)

Front 2

Most common alleles of people with a1 antitrypsin def.?

Back 2

PiZ and PiS

Front 3

Clincial findings in neonates with a1 antitrypsin def?

Back 3

cholestasis; may resemble extrahepatic bile duct atresia due to paucity of interlobular bile ducts

Front 4

Location of PAS-D positive globular inclusions in a1 anti-trypsin?

Back 4

Peri-portal

Front 5

Histological findings in neonates with alpha 1 antitrypsin def.

Back 5

parenchymal giant cells, ductular reaction, fibrosis and sometimes paucity of interlobular bile ducts

Front 6

Natural history of alpha one antitrypsin def?

Back 6

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.

Front 7

Triad of histology in acute rejection?
Chronic?

Back 7

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

Front 8

Histological findings in Autoimmune hepatitis?

Back 8

interface hep with plasma cells, and regenerative liver cell rosettes (may be vacuolated)

Front 9

Autoantibodies seen in autoimmune hepatitis?

Back 9

Type 1: ana or anti-smooth muscle or both
Type 2: anti-LKM
Type 3: anti-SLA (soluble liver antigen)

Front 10

How differentiate between von Meyenburg complex and bile duct adenoma?

Back 10

VMC have dilated ducts with mucin or bile
Adenoma: normal ducts with nothing in them and lymphocytes on edge of lesion

Front 11

Von Meyenburg complex are associated with which diseases/abnormalities?

Back 11

normal liver, congenital hepatic fibrosis, Caroli's syndrome, and adult polycystic kidney disease

Front 12

Bile ductular cholestasis is pathoneumonic (in adults) for?

Back 12

Sepsis
Usually gram negs: E-coli

Front 13

Primary sclerosing Cholangitis
(PSC)

Back 13

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

Front 14

Budd-Chiari (hepatic vein thrombosis) / venous outflow obstruction histological findings.

Back 14

centrolobular fibrosis
lobular or portal thrombosis

Front 15

Location of granulomas in sarcoid of the liver?

Back 15

Peri-portal

Front 16

Causes of macrovesicular steatosis?

Back 16

Ethanol, obesity, diabetes, hyperlipidemia, corticosteroids

Front 17

Causes of microvesicular steatosis?

Back 17

anti-retrovirals, acute fatty liver of pregnancy, reye's synd., tetracyline toxicity, alchohol foamy degeneration, valproic acid toxicity.

Front 18

Kupffer cell predominant iron overload.

Back 18

hemolysis, transfusions, or hemodialysis

Front 19

Hepatocyte predominant iron overload.

Back 19

HFE-related hereditary hemochromotosis

Front 20

Both kupffer cell and hepatocyte iron overload?

Back 20

Chronic systemic conditions like long-term transfusion

Long term oral iron intake

Front 21

Oral iron intake pattern

Back 21

Peri-portal macrophages and kupffer cells.

Front 22

Mutation seen in liver cell adenoma?

Back 22

Hepatocyte nuclear factor 1 aplph (HVF-1alpha)

Front 23

Mutations in cholangiocarcinoma?

Back 23

K-ras and c-myc

Front 24

Alagille's syndrome?

Back 24

Syndromic paucity of intrahepatic bile duct associated with Jagged-1 mutations.

Front 25

Staining pattern for HCC

Back 25

positive: hep r and afp (diffuse cytoplasmic)
polyclonal CEA - apical
Negative: Ck7 and Ck20

Front 26

paraquat toxicity histopathology?

Back 26

bile duct epethlium necrosis.

Front 27

Benign recurrent intrahepatic cholestasis.

Back 27

bouts of recurrent choestasis
liver findings: bile thrombi in canaliculi
mutation in FIC-1 (ABCB11) chrom 18 (transports bile across canalicular mem)