Chapter 18, 19 Slide Set 4 Flashcards by Alex Hamidi

Stain for hemochromatosis?

Prussian blue

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Adult hemochromatosis due to what mutation?

Juvenile form?

HFE or less likely TFR2

HAMP and HJV –> more severe

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

Most common tumor suppressor gene?

KRAS

P16/CDKN2A

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What is the change in acute liver failure?

Do to what?

Liver swells

Acetaminophen overdoes (50%)

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Primary biliary cirrhosis clinical course?

Primary sclerosing cholangitis clinical course?

Progressive

Unpredictable but progressive

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Mutation in a1-antitrypsin deficiency leads to what?

Unfolded protein response

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What gene mutation associated with agenesis of pancreas?

PDX1

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

Widespread what?

Confluent

Parenchymal loss

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What to look for to implicate NAFLD?

DM
High BMI
HTN
Microalbuminiurua
Insulin resistance

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

PBC (primary biliary cirrhosis)

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hepatocellular adenoma may be caused by what?

Anabolic steroids

Tamoxifen

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Route of transmission for HepA?

HepB?

Fecal-oral

Parenteral, sex, perinatal

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What is seen in autoimmune pancreatitis?

Treatment?

Increased IgG4 secreting plasma cells

Steroids

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

Dubin-Johnson syndrome

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

Chronic hepatitis

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OCP and anabolic steroids associated wth neoplasm of liver?

Adenoma

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What is a cause of Budd-Chiari syndrome?

Oral contraceptives

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

PSC

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

Dubin-johnson

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What ethnic groups are most affected by NAFLD/NASH?

Associated with what?

Hispanics > AAs > Caucasians

Metabolic syndrome

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What disease may appear Parkinsonian in nature?

Wilson’s

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

A1-antitrypsin

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What triad is associated with acute cholangitis?

Charcot’s triad
Jaundice
RUQ pain
Fever

Leads to shock

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

Steatohepatitis

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Where does biliary colic radiate?

shoulder blade

Fibrolamellar carcinoma what age? Rich in what cell? What kind of tumor?

Under 35 Mitochondria (oncocytes) Single, large, scirrhous tumor

What is the classic tetrad for hemochromatosis?

Cirrhosis w/hepatomegaly Abnormal skin pigment DM Cardiac dysfunction

What tests measure hepatocyte synthetic function?

albumin PT, PTT Ammonia

What tests measure hepatocyte integrity?

AST ALT LDH

Most common congenital anomaly of pancreas?

Pancreas divisum, failure of fusion

RFs for cholelithiasis?

``` All the Fs Female Fat Fertile 40 Family history ```

PBC serology? Duct lesion?

95% AMA (+) 50% ANA Florid duct lesion and loss of small ducts only

Lymphoid follicle in hepatocyte parenchyma?

HCV

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

A1-antitrypsin deficiency

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

HepB

Inheritance of Wilson's? Mutation in what gene? Can cause what blood problem?

AR ATP7B Hemolytic anemia

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

Steatosis

Subtype of what shows increased IgG4 levels? Also associated with what?

PSC HLA-B8

What is the most common benign neoplasm of the liver? More common in who?

Cavernous hemangioma Female

Most common liver tumor of early childhood? Activation of what?

Hepatoblastoma WNT signaling

HCC what age? What serum marker is increased? Prognosis for large tumor?

20-40 Alpha-fetal protein dismal

What allele association in caucasians with autoimmune hepatitis? Hepatocytes described as what? Treatment?

DRB1 Rosettes Immunosuppressive therapy then transplant

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

Chronic liver failure

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

Steatofibrosis/cirrhosis

What tests measure biliary excretion function?

Alkaline phosphatase | GGT

Which Hep virus never causes chronic liver disease (hepatitis)? Which causes it the most often?

HepA HepC

What hepatitis virus has detection of serum IgM antibodies?

HepA

What is the cardinal manifestation of acute pancreatitis? Lab findings?

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

What are the RFs for cholangiocarcinoma? What is the extrahepatic form called? What lesion type is the worst? Overall prognosis?

Chronic inflammation, cholestasis, HBV/HCV Klatskin tumor (perihilar/porta-hepatitis) BillN-3 Poor (15% 2 yr survival)

What is the most common HFE mutation?

C282Y

What is the most common cyst of the pancreas? Seen in what?

Pancreatic pseudocyst Alcohol use

How much blood does the portal vein supply? Hepatic artery?

70% 30

PBC serology? PSC serology?

AMA (+) P-anca (+)

Autoimmune hepatitis Type 1 most common in whom? What serum markers? Prognosis?

Middle aged women ANA and ASMA Better than 2

What extrahepatic etiology causes cholestasis in adults? Neonatal?

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

Rokitansky-Aschoff sinuses seen where?

Chronic cholecystitis

What are the 4 major consequences of portal HTN?

Ascites Portosystemic venous shunt Congestive splenomegaly Hepatic encephalopathy

What Hep virus can be transmitted via intranasal cocaine?

HepC

SMAD4 is pretty diagnostic for what?

Pancreatic adenocarcinoma

Adenocarcinomas of the pancreas occur where? What sign?

Head of the pancreas Courvoisier's sign, Trousseau (migratory thrombophlebitis)

What can cause dilated cardiomyopathy?

Hemochromatosis

Most common malignancy of the extahepatic biliary tract is what?

Adenocarcinoma Usually due to stones RF

75% of what people have gall stones?

Native americans

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

Bridging necrosis

What does Murphy's sign indicate?

Acute cholecystitis

PSC age? Gender? Associated conditions?

30 Male IBD (UC), pancreatitis

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

PSC primary sclerosing cholangitis

Age of pancreatic cancer? Ethnicity? RF? Inherited predisposition?

Older adults African americans Smoking Peutz-Jeghers HNPCC

Ground glass?

HBV

What old chemical agent can cause cholangiocarcinoma?

Thorotrast

PBC affects who? Age? Associated conditions?

Women Age 50 (30-70) Sjogren, Scleroderma, thyroid disease

What neoplasm has multiple nodules but NO fibrous septa? What associations?

Nodular regenerative hyperplasia HIV, SLE

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

Lab tests showing elevated liver enzymes (AST, ALT)

Autoimmune hepatitis type 2 most often seen in who? What serum markers?

Children and teenagers Anti-LKM-1, CYP2D6, ACL-1

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

Hemochromatosis Also causes pseudogout (disabling polyarthritis)

Primary hepatolithiasis predisposes to what? May evolve to what? High incidence where?

Biliar neoplasia Invasive cholangiocarcinoma East Asia

PSC serology? Radiology? Duct lesions?

65% p-ANCA Beading of large bile ducts Inflammatory destruction of extrahepatic and large intrahepatic ducts, fibrotic obliteration

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

Focal nodular hyperplasia Young to middle age adults

Adenoma of the liver with what mutation has very high risk for malignancy> No risk?

B-Catenin HNF1-a

What are most common etiologies of acute pancreatitis in men? Women?

Alcoholism Gallstones

Ductal adenocarcinomas elicit what response? Most frequently mutated gene in pancreatic cancer? Prognosis?

Intense desmoplastic KRAS, p16, CDK2NA, TP53, SMAD4 Poor (1-2 years)

Hemochromatosis has 40% chance of causing what?

HCC

Acute pancreatitis gene mutation?

CFTR PRSS1 --> AD SPINK1 --> AR