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GI Week 4 > Ch 18 Robbins part II > Flashcards

Ch 18 Robbins part II Flashcards

1
Q

Which type of Crigler-Najjar syndrome is more severe?

Why?

A

Type 1 because there is no UGT1A1 activity compared to Type II which has decreased activity

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2
Q

What levels are elevated with cholestasis?

A

Elevated serum γ-glutamyl transpeptidase (GGT) and alkaline phosphatase

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3
Q

What district morphology is seen in cholestasis?

A

Feathery degeneration of periportal hepatocytes

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4
Q

How does ascending cholangitis present?

A

Charcot’s triad: fever, RUQ pain, jaundice

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5
Q

What is characterized by repeated bouts of ascending cholangitis, progressive inflammatory destruction of hepatic parenchyma, and predisposes to biliary neoplasia?

A

Primary hepatolithiasis

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6
Q

Primary hepatolithiasis causes an increased risk of?

A

Cholangiocarcinoma

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7
Q

Panlobular giant-cell transformation of hepatocytes is seen with?

A

Neonatal hepatitis

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8
Q

What is the most common cause of neonatal cholestasis and death from liver disease in early childhood?

A

Biliary atresia

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9
Q

What stool change is seen with biliary atresia?

A

Acholic stools (pale)

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10
Q

How is biliary atresia limited to common duct (type I) or right/left hepatic ducts (type II) treated?

How is obstruction above the porta hepatis (type III) treated?

A

1) Kasai procedure

2) It can’t and instead needs liver transplant

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11
Q

What population is most affected by primary biliary cirrhosis?

A

Middle-aged women

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12
Q

What Abs are seen with primary biliary cirrhosis?

A

Anti-mitochondrial antibodies (anti-PDC-E2)

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13
Q

What happens to interlobular ducts with primary biliary cirrhosis?

A

Florid duct lesion

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14
Q

What is characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts with dilation of preserved segments?

What is seen on radiographs?

A

1) Primary sclerosing cholangitis (PSC)

2) Beading

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15
Q

How does primary sclerosing cholangitis affect the smaller ducts?

A

Onion skin fibrosis around an atrophic duct lumen that leads to obliteration by a tombstone scar

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16
Q

What levels are elevated with primary sclerosing cholangitis?

A

Serum alkaline phosphatase

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17
Q

Primary sclerosing cholangitis is diagnosed with radiology of the biliary tree that shows?

A

Larger ducts with strictures and beading with pruning of the smaller ducts

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18
Q

What are congenital dilations of the common bile duct?

A

Choledochal cysts

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19
Q

What are a group of different lesions in the liver due to congenital malformations of the biliary tree?

A

Fibropolycystic disease (FPD)

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20
Q

What is characterized by small bile duct hamartomas that are normal if limited, but indicative of FPD if they are diffuse?

A

Von Meyenburg complexes

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21
Q

What pathologic finding of FPD is characterized by biliary cysts in isolation that lead to clinical symptoms?

A

Caroli disease

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22
Q

What pathologic finding of FPD is characterized by portal tracts that are enlarged by irregular broad bands of collagenous tissue that form septa to divide the liver into irregular island?

A

Congenital hepatic fibrosis

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23
Q

What is the most common cause of intrahepatic blood flow obstruction?

A

Cirrhosis

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24
Q

What hepatic issue does left sided heart failure lead to?

A

Centrilobular fibrosis

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25
Q

What is characterized by obstruction of two or more major hepatic veins that leads to hepatomegaly, pain, and ascites?

A

Budd-Chiari syndrome

26
Q

How does Budd-Chiari syndrome present?

A

1) Liver is swollen and red-purple

2) Centrilobular necrosis

27
Q

Toxic injury to the sinusoidal endothelium can lead to?

A

Sinusoidal obstruction syndrome

28
Q

How is Sinusoidal obstruction syndrome clinically diagnosed?

A

Tender hepatomegaly, ascites, weight gain, jaundice

29
Q

When is vanashing bile duct syndrome seen?

A

Chronic transplant rejection

30
Q

What is the most common cause of jaundice in pregnancy?

A

Viral hepatitis

31
Q

What levels are increased with preeclampsia and eclampsia?

A

1) Serum aminotransferases

2) Serum bilirubin

32
Q

How is acute fatty liver of pregnancy diagnosed on biopsy?

A

Diffuse microvesicular steatosis of hepatocytes

33
Q

What levels are greatly increased with intrahepatic cholestasis of pregnancy?

A

Bile salt

34
Q

What is characterized by the entire liver transformed into nodules; looks similar to focal nodular hyperplasia, but there is no fibrosis?

What can it lead to?

A

1) Nodular regenerative hyperplasia

2) Portal HTN

35
Q

What morphologic changes are seen with nodular regenerative hyperplasia?

A

Plump hepatocytes surrounded by rims of atrophic hepatocytes

36
Q

What is the most common benign liver tumor?

A

Cavernous hemangiomas

37
Q

What benign neoplasms developing from hepatocytes may rupture leading to intraabdominal bleeding that is a surgical emergency?

What form of therapy are they associated with?

A

1) Hepatocellular adenomas

2) Oral contraceptives

38
Q

Which type of hepatocellular adenoma has no risk of malignant transformation?

Which has a very high risk of malignant transformation?

A

1) HNF1-α inactivated adenomas

2) Β-Catenin activated adenomas

39
Q

What is the most common liver tumor of early childhood?

A

Hepatoblastoma

40
Q

What do hepatoblastoma frequently activate?

Due to what mutation?

This mutation causes what to precipitate the hepatoblastoma?

A

1) WNT pathway
2) APC mutation
3) FAP

41
Q

What is the most common primary malignancy of hepatocytes?

A

Hepatocellular carcinoma

42
Q

What is a strong genetic risk factor of hepatocellular carcinoma?

A

1) B-catenin activation

2) p53 inactivation (aflatoxin)

43
Q

What is probably the most important pathway for emergence of hepatocellular carcinoma in viral hepatitis and alcoholic liver disease?

A

High-grade dysplastic nodules

44
Q

What rare variant of hepatocellular carcinoma presents as a single large hard scirrhous tumor with fibrous bands coursing through it?

A

Fibrolamellar carcinoma

45
Q

What cancer of the biliary tree is the second most common primary malignant tumor after HCC?

A

Cholangiocarcinoma

46
Q

What is a strong risk factor for Cholangiocarcinoma?

A

Liver fluke parasites (Opisthorchis, clonorchis)

47
Q

Where are the perihilar tumors (Klatskin tumors) of Cholangiocarcinoma most commonly located?

A

Junction of the hepatic ducts

48
Q

Which form of cholangiocarcinoma presents as obstruction to bile flow or symptomatic liver mass?

Which presents as biliary obstruction, cholangitis, and RUQ pain?

A

1) Intrahepatic

2) Extrahepatic

49
Q

Angiosarcomas are other primary hepatic malignant tumors that has historical associations with?

A

Vinyl chloride

50
Q

More than 95% of biliary tract disease is attributable to?

A

Cholelithiasis

51
Q

What causes cholelithiasis?

A

Stones of either cholesterol or pigment (bilirubin)

52
Q

Bacterial contamination of the biliary tract by E. coli, Ascaris lumbricoides, or liver fluke C. Sinensis leads to release of?

This causes?

A

1) Beta-glucuronidases

2) Stones of unconjugated bilirubin

53
Q

Where are black pigment stones found?

Where are brown pigment stones found?

A

1) Sterile bile ducts

2) Infected large bile ducts

54
Q

How does cholelithiasis present?

A

Pain in RUQ that radiates to right upper shoulder or back

55
Q

Which size of stones are considered more dangerous?

A

Small stones (gravel)

56
Q

Acute calculous cholecystitis is almost always caused by a stone obstructing what area?

A

Neck or cystic duct

57
Q

What is there a greater risk of in patients that have acalculous cholecystitis?

A

Gangrene and perforation

58
Q

Chronic cholecystitis can lead to what morphologic change?

What does it cause?

A

1) Porcelain gallbladder

2) Calcification of the wall that increases the risk of developing cholangiocarcinoma

59
Q

Chronic cholecystitis causes an intolerance for?

A

Fatty foods

60
Q

What is the most common malignancy of the extrahepatic biliary tract?

Which sex is more affected?

What is the biggest risk factor for it?

A

1) Adenocarcinoma of the gallbladder
2) Women
3) Gallstones

GI Week 4 (12 decks)

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