GI Liver Pathology

Question 1

Pathogenesis of hepatocellular steatosis (alcohol, 3 things)

Answer 1

1) shunting toward lipid synthesis; making NADH 2) impaired assembly and secretion of lipoproteins 3) peripheral catabolism of fat (increase FFA in blood)

Question 2

Pathology of alcoholic hepatitis (3 things)

Answer 2

1) Hepatocyte swelling and necrosis 2) Mallory-Denk bodies 3) Neutrophilic inflammation

Question 3

Earliest pattern of hepatic fibrosis in alcoholic liver disease

Answer 3

Perivenular fibrosis

Question 4

Liver enzymes in alcoholic liver dz

Answer 4

AST>ALT; 2:1 ratio or higher

Question 5

Cause of death in alcoholic liver dz (5 things)

Answer 5

1) Coma 2) GI hemorrhage 3) infection 4) hepatorenal syndrome 5) hepatocellular carcinoma

Question 6

Associated diseases with NAFLD

Answer 6

Metabolic syndrome

Question 7

NASH

Answer 7

Subset of NAFLD with clinical features: elevated serum transaminases, hepatocyte injury

Question 8

Features of pediatric NAFLD

Answer 8

1) more diffuse steatosis 2) portal more than central fibrosis 3) portal and parenchymal mononuclear infiltration

Question 9

NASH vs. Alcoholic liver dz pathology

Answer 9

Same! (may have more portal fibrosis with NASH)

Question 10

Budd-Chiari syndrome

Answer 10

Hepatic vein thrombosis with tender hepatomegaly, abdominal pain, and ascites

Question 11

Inflammatory mediators of sepsis

Answer 11

Kupffer cells

Question 12

Diseases leading to bile plugging up the canaliculi and hepatocytes, respectively

Answer 12

Canalicular cholestasis and hepatocellular cholestasis

Question 13

Liver diseases of pregnancy (3)

Answer 13

1) HELLP syndrome 2) acute fatty liver of pregnancy 3) intrahepatic cholestasis of pregnancy (most common)

Question 14

Pathogenesis of intrahepatic cholestasis

Answer 14

Epiallopregnanolone sulfate inhibition of farnesoid X receptor mediated bile efflux

Question 15

Tx of intrahepatic cholestasis

Answer 15

Ursodeoxycholic acid

Question 16

Toxic metabolite of Tylenol

Answer 16

N-acetyl-p-benzoquinoneimine (NAPQI)

Question 17

Most susceptible part of liver in Tylenol overdose

Answer 17

Centrolobular hepatocytes (runs out of glutathione first)

Question 18

Lobular metabolic gradient

Answer 18

Periportal gets most blood, centrolobular gets least

Question 19

Zones of necrosis for acetaminophen, yellow fever/mushrooms, phosphorus, respectively

Answer 19

Central, midzone, and periportal

Question 20

Pathology of acute viral hepatitis

Answer 20

1) swollen pale hepatocytes 2) portal and lobular infiltration by lymphcytes, macrophages, and plasma cells 3) Kupffer cell hypertrophy and hyperplasia 4) cholestasis

Question 21

Viral hepatitis vs. autoimmune hepatitis

Answer 21

Autoimmune has more plasma cells

Question 22

Manifestation of alcoholic hepatitis

Answer 22

1) bleeding (increased PT) 2) confusion 3) fever

Question 23

Conditions with macrovesicular steatohepatitis

Answer 23

Alcoholic hepatitis, NASH, glucocorticoids

Question 24

Conditions with microvesicular steatohepatitis

Answer 24

Acute fatty liver of pregnancy, Reye syndrome, hornet stings, alcoholic hepatitis (mixed with macrovesicular)

Question 25

Conditions with feathery degeneration

Answer 25

Biliary obstruction by stones, tumors or granulomas

Question 26

Councilman bodies

Answer 26

Eosinophilic apoptotic cells commonly seen with viral hepatitis

Question 27

Liver pathology in heart failure

Answer 27

Congestion, nutmeg liver, centrolobular necrosis

Question 28

Causes of direct jaundice

Answer 28

Obstruction, hepatitis, Dubin-Johnson

Question 29

Causes of indirect jaundice

Answer 29

Hemolysis, hepatitis, Gilbert

Question 30

Physiologic jaundice

Answer 30

Seen in neonates with immature liver up to 2 weeks old

Question 31

Biliary atresia

Answer 31

Inflammation and fibrosis of extrahepatic bile ducts

Question 32

Features of biliary atresia

Answer 32

Jaundice at 3-6 weeks, high GGT

Question 33

Alagille syndrome

Answer 33

Lack of intrahepatic bile ducts, jaundice, pruirits, and cholestasis plus developmental defects

Question 34

Mutation in Alagille syndrome

Answer 34

Mutation in JAGGED 1 gene

Question 35

Dubin-Johnson syndrome

Answer 35

Defective excretion of bilirubin conjugates; black pigmented lysosomes with epinephrine metabolites

Question 36

Gilbert syndrome

Answer 36

Deficiency of UGT - mild hyperbilirubinemia brought on by stress

Question 37

Primary biliary cirrhosis

Answer 37

Autoimmune cholestatic disease, FEMALES, presents with pruritis, fatigue, hepatomegaly, elevated ALP

Question 38

Pathology of primary biliary cirrhosis

Answer 38

Dense lymphocytic infiltrate in and around interlobular bile ducts, granulomas, and bile duct loss

Question 39

Ascending cholangitis

Answer 39

Infection of intrahepatic biliary ducts (acute - bacterial, chronic - parasites)

Question 40

Features of ascending cholangitis

Answer 40

Fever, chills, abdominal pain, jaundice; purulent bile distends bile ducts, liver abscesses

Question 41

Common primary sites for liver metastases

Answer 41

Colon, lung, breast, pancreas, stomach

Question 42

Two most common genetic mutations in hepatocellular carcinoma

Answer 42

Activation of beta-catenin and inactivation of p53

Question 43

Morphology of HCC

Answer 43

Unifocal, multifocal, or diffusely infiltrative (doesn't show up on imaging)

Question 44

Most common benign tumor of the liver

Answer 44

Hemangioma

Question 45

Rare malignant epithelial neoplasm of liver in children

Answer 45

Hepatoblastoma

Question 46

Malignant epithelial neoplasm with biliary differentiation, arising from cholangiocytes

Answer 46

Cholangiocarcinoma

Question 47

Pathology of cholangiocarcinoma

Answer 47

Tan-white and firm lesions

Question 48

Urine odors for tyrosenemia, phenylketonuria, trimethylaminuria, isovalaric acidemia, and maple syrup urine dz, respectively

Answer 48

Boiled cabbage, Mousy or musty, Rotting fish, Sweaty feet, Maple syrup

Question 49

Pathogenesis of tyrosinemia

Answer 49

Fumaryl-aceto-acetase deficiency that leads to backup of tyrosine that injures the liver

Question 50

Pathogenesis of Gaucher's disease

Answer 50

Beta-glucocerebrosidase deficiency, splenomegaly, Kupffer cells with expanded crinkled cytoplasm

Question 51

Glycogen storage disease type I (Von Gierke)

Answer 51

Glucose-6-phosphatase deficiency that results in glycogen accumulation in the liver, hepatomegaly, hypoglycemia

Question 52

Porphyrias

Answer 52

Defect in heme synthesis; acute or cutaenous; porphyria cutanea tarda has skin vesicles and bullae with sun exposure - more common in women; precipitated by drug, alcohol, smoking, steroids, oral contraceptives

Question 53

Acute Intermittent Porphyria

Answer 53

Urine can become dark/purple, urine test for porphobilinogen, give IV heme for Tx

Question 54

Pyloric stenosis

Answer 54

Few week old baby with immediate postprandial non-bilious projectile vomiting with olive-like mass in abdomen