Chapter 18, 19 Slide Set 4 Flashcards

1
Q

Stain for hemochromatosis?

A

Prussian blue

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2
Q

Adult hemochromatosis due to what mutation?

Juvenile form?

A

HFE or less likely TFR2

HAMP and HJV –> more severe

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4
Q

What is the most common of oncogene mutation seen in pancreatic cancers?

Most common tumor suppressor gene?

A

KRAS

P16/CDKN2A

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5
Q

What is the change in acute liver failure?

Do to what?

A

Liver swells

Acetaminophen overdoes (50%)

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6
Q

Primary biliary cirrhosis clinical course?

Primary sclerosing cholangitis clinical course?

A

Progressive

Unpredictable but progressive

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8
Q

Mutation in a1-antitrypsin deficiency leads to what?

A

Unfolded protein response

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9
Q

What gene mutation associated with agenesis of pancreas?

A

PDX1

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11
Q

What kind of necrosis is seen in acute toxic, ischemic injury, severe viral, or autoimmune hepatitis?

Widespread what?

A

Confluent

Parenchymal loss

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12
Q

What to look for to implicate NAFLD?

A
DM
High BMI
HTN
Microalbuminiurua
Insulin resistance
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13
Q

What presents with obstructive jaundice and autoimmune granulomatous destruction of intrahepatic bile ducts?

A

PBC (primary biliary cirrhosis)

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15
Q

hepatocellular adenoma may be caused by what?

A

Anabolic steroids

Tamoxifen

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16
Q

Route of transmission for HepA?

HepB?

A

Fecal-oral

Parenteral, sex, perinatal

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17
Q

What is seen in autoimmune pancreatitis?

Treatment?

A

Increased IgG4 secreting plasma cells

Steroids

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18
Q

What is associated with pigmented cytoplasmic globules (black liver)?

A

Dubin-Johnson syndrome

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19
Q

Interface hepatitis (portal inflammatory infiltrate spilling out of the portal tract into limiting plate)?

A

Chronic hepatitis

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21
Q

OCP and anabolic steroids associated wth neoplasm of liver?

A

Adenoma

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22
Q

What is a cause of Budd-Chiari syndrome?

A

Oral contraceptives

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23
Q

What causes periductal fibrosis with an “onion-skin” appearance?

A

PSC

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25
Q

What disease has a mutation in MRP2 and defects in canalicular membrane transport?

A

Dubin-johnson

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27
Q

What ethnic groups are most affected by NAFLD/NASH?

Associated with what?

A

Hispanics > AAs > Caucasians

Metabolic syndrome

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28
Q

What disease may appear Parkinsonian in nature?

A

Wilson’s

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29
Q

What disease has round-oval cytoplasmic globular inclusions in hepatocytes and is PAS (+)?

A

A1-antitrypsin

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30
Q

What triad is associated with acute cholangitis?

A

Charcot’s triad
Jaundice
RUQ pain
Fever

Leads to shock

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33
Q

What is described by tender hepatomegaly, cholestasis, hyperbilirubinemia, 2:1 AST to ALT levels, elevated alk phos?

A

Steatohepatitis

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34
Q

Where does biliary colic radiate?

A

shoulder blade

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35
Q

Fibrolamellar carcinoma what age?

Rich in what cell?

What kind of tumor?

A

Under 35

Mitochondria (oncocytes)

Single, large, scirrhous tumor

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36
Q

What is the classic tetrad for hemochromatosis?

A

Cirrhosis w/hepatomegaly
Abnormal skin pigment
DM
Cardiac dysfunction

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37
Q

What tests measure hepatocyte synthetic function?

A

albumin
PT, PTT
Ammonia

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38
Q

What tests measure hepatocyte integrity?

A

AST
ALT
LDH

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38
Q

Most common congenital anomaly of pancreas?

A

Pancreas divisum, failure of fusion

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39
Q

RFs for cholelithiasis?

A
All the Fs
Female
Fat
Fertile
40 
Family history
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40
Q

PBC serology?

Duct lesion?

A

95% AMA (+)
50% ANA

Florid duct lesion and loss of small ducts only

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41
Q

Lymphoid follicle in hepatocyte parenchyma?

A

HCV

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42
Q

What is the most commonly diagnosed inherited hepatic disorder in infants and children?

A

A1-antitrypsin deficiency

45
Q

Which hepatitis is highly concentrated in Southeast Asia, India, and Africa?

A

HepB

46
Q

Inheritance of Wilson’s?

Mutation in what gene?

Can cause what blood problem?

A

AR

ATP7B

Hemolytic anemia

47
Q

What is described by hepatomegaly w/min sx, elevated bilirubin and alk phos?

A

Steatosis

48
Q

Subtype of what shows increased IgG4 levels?

Also associated with what?

A

PSC

HLA-B8

49
Q

What is the most common benign neoplasm of the liver?

More common in who?

A

Cavernous hemangioma

Female

50
Q

Most common liver tumor of early childhood?

Activation of what?

A

Hepatoblastoma

WNT signaling

51
Q

HCC what age?

What serum marker is increased?

Prognosis for large tumor?

A

20-40

Alpha-fetal protein

dismal

52
Q

What allele association in caucasians with autoimmune hepatitis?

Hepatocytes described as what?

Treatment?

A

DRB1

Rosettes

Immunosuppressive therapy then transplant

53
Q

Pruritis, palmar erythema, spider angiomata, hyopgonadism, gynecomastia in a pt may indicate what?

A

Chronic liver failure

55
Q

What is described by hepatic dysfunction if enough liver tissue left, hypoproteinemia, coag abnormalities?

A

Steatofibrosis/cirrhosis

56
Q

What tests measure biliary excretion function?

A

Alkaline phosphatase

GGT

57
Q

Which Hep virus never causes chronic liver disease (hepatitis)?

Which causes it the most often?

A

HepA

HepC

58
Q

What hepatitis virus has detection of serum IgM antibodies?

A

HepA

59
Q

What is the cardinal manifestation of acute pancreatitis?

Lab findings?

A

Pain constant and intense, referred to upper back on left shoulder

Elevated amylase and lipase

60
Q

What are the RFs for cholangiocarcinoma?

What is the extrahepatic form called?

What lesion type is the worst?

Overall prognosis?

A

Chronic inflammation, cholestasis, HBV/HCV

Klatskin tumor (perihilar/porta-hepatitis)

BillN-3

Poor (15% 2 yr survival)

61
Q

What is the most common HFE mutation?

A

C282Y

63
Q

What is the most common cyst of the pancreas?

Seen in what?

A

Pancreatic pseudocyst

Alcohol use

64
Q

How much blood does the portal vein supply?

Hepatic artery?

A

70%

30

64
Q

PBC serology?

PSC serology?

A

AMA (+)

P-anca (+)

66
Q

Autoimmune hepatitis Type 1 most common in whom?

What serum markers?

Prognosis?

A

Middle aged women

ANA and ASMA

Better than 2

67
Q

What extrahepatic etiology causes cholestasis in adults?

Neonatal?

A

Large duct obstruction

Biliary atresia (#1 cause of death from liver disease in kids)

68
Q

Rokitansky-Aschoff sinuses seen where?

A

Chronic cholecystitis

69
Q

What are the 4 major consequences of portal HTN?

A

Ascites
Portosystemic venous shunt
Congestive splenomegaly
Hepatic encephalopathy

69
Q

What Hep virus can be transmitted via intranasal cocaine?

A

HepC

69
Q

SMAD4 is pretty diagnostic for what?

A

Pancreatic adenocarcinoma

69
Q

Adenocarcinomas of the pancreas occur where?

What sign?

A

Head of the pancreas

Courvoisier’s sign, Trousseau (migratory thrombophlebitis)

70
Q

What can cause dilated cardiomyopathy?

A

Hemochromatosis

71
Q

Most common malignancy of the extahepatic biliary tract is what?

A

Adenocarcinoma

Usually due to stones RF

73
Q

75% of what people have gall stones?

A

Native americans

74
Q

What kind of necrosis is seen when a zone may link central veins to portal tracts or bridge adjacent portal tracts?

A

Bridging necrosis

75
Q

What does Murphy’s sign indicate?

A

Acute cholecystitis

76
Q

PSC age?

Gender?

Associated conditions?

A

30

Male

IBD (UC), pancreatitis

76
Q

What causes inflammation and fibrosis of intrahepatic and extrahepatic bile ducts?

A

PSC primary sclerosing cholangitis

77
Q

Age of pancreatic cancer?

Ethnicity?

RF?

Inherited predisposition?

A

Older adults

African americans

Smoking

Peutz-Jeghers
HNPCC

78
Q

Ground glass?

A

HBV

78
Q

What old chemical agent can cause cholangiocarcinoma?

A

Thorotrast

78
Q

PBC affects who?

Age?

Associated conditions?

A

Women

Age 50 (30-70)

Sjogren, Scleroderma, thyroid disease

78
Q

What neoplasm has multiple nodules but NO fibrous septa?

What associations?

A

Nodular regenerative hyperplasia

HIV, SLE

79
Q

What is the most common way to figure out has pt has HCV?

A

Lab tests showing elevated liver enzymes (AST, ALT)

79
Q

Autoimmune hepatitis type 2 most often seen in who?

What serum markers?

A

Children and teenagers

Anti-LKM-1, CYP2D6, ACL-1

79
Q

What can cause hypogonadism and infertility? May also cause acute synovitis?

A

Hemochromatosis

Also causes pseudogout (disabling polyarthritis)

79
Q

Primary hepatolithiasis predisposes to what?

May evolve to what?

High incidence where?

A

Biliar neoplasia

Invasive cholangiocarcinoma

East Asia

79
Q

PSC serology?

Radiology?

Duct lesions?

A

65% p-ANCA

Beading of large bile ducts

Inflammatory destruction of extrahepatic and large intrahepatic ducts, fibrotic obliteration

79
Q

What neoplasm is a single well demarcated lesion with a central scar?

Common in who?

A

Focal nodular hyperplasia

Young to middle age adults

79
Q

Adenoma of the liver with what mutation has very high risk for malignancy>

No risk?

A

B-Catenin

HNF1-a

79
Q

What are most common etiologies of acute pancreatitis in men?

Women?

A

Alcoholism

Gallstones

79
Q

Ductal adenocarcinomas elicit what response?

Most frequently mutated gene in pancreatic cancer?

Prognosis?

A

Intense desmoplastic

KRAS, p16, CDK2NA, TP53, SMAD4

Poor (1-2 years)

82
Q

Hemochromatosis has 40% chance of causing what?

A

HCC

83
Q

Acute pancreatitis gene mutation?

A

CFTR
PRSS1 –> AD
SPINK1 –> AR