Block 2 Pancreas/Liver/Gall bladder Flashcards

1
Q

Describe the following for annular pancreas:

What is it?

What is a complication?

A

Patho:
A malformation of the pancreas where it grows as a ring around the duodenum

Complications:
Duodenal obstruction

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2
Q

Patho:
A malformation of the pancreas where it grows as a ring around the duodenum

Complications:
Duodenal obstruction

Describes which condition?

A

Annular pancreas

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3
Q

Describe the following for acute pancreatitis:

What is it?

What are the lab findings?

What are the causes?
- #1 vs others

What are the symptoms?

What are the complications?

A

Patho:
Inflammation with hemorrhaging of the pancreas because of prematurely activated trypsin which activates other pancreatic enzymes resulting in auto/self-digestion of the pancreatic parenchyma

Labs:
Elevated lipase & amylase
Liquefactive hemorrhagic necrosis
Fat necrosis/saponification
Hypercalcemia

Causes:
Main
1) #1 Alcohol & gallstones

Other
1) Trauma
2) Hypercalcemia
3) Hyperlipidemia
4) Scorpion sting/drugs
5) MUMPS
6) Rupture of a posterior duodenal ulcer

Signs:
1) Epigastric pain (rad to back)
2) Nausea/vomiting
3) Periumbilical & flank hemorrhaging
4) Hypercalcemia

Comps:
1) Shock
2) Pancreatic pseudocyst (rupture)
3) Pancreatic abscess
4) DIC & Acute respiratory distress syndrome (ARDS)

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4
Q

Patho:
Inflammation with hemorrhaging of the pancreas because of prematurely activated trypsin which activates other pancreatic enzymes resulting in auto/self-digestion of the pancreatic parenchyma

Labs:
Elevated lipase & amylase
Liquefactive hemorrhagic necrosis
Fat necrosis/saponification
Hypercalcemia

Causes:
Main
1) #1 Alcohol & gallstones

Other
1) Trauma
2) Hypercalcemia
3) Hyperlipidemia
4) Scorpion sting/drugs
5) MUMPS
6) Rupture of a posterior duodenal ulcer

Signs:
1) Epigastric pain (rad to back)
2) Nausea/vomiting
3) Periumbilical & flank hemorrhaging
4) Hypercalcemia

Comps:
1) Shock
2) Pancreatic pseudocyst (rupture)
3) Pancreatic abscess
4) DIC & Acute respiratory distress syndrome (ARDS)

Describes which condition?

A

Acute pancreatitis

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5
Q

What is a Pancreatic pseudocyst?

  • Patho
  • Lab findings
  • Complication
A

Patho:
An abdominal mass that’s a complication of acute pancreatitis

Labs:
Elevated serum amylase

Comps:
Rupture can release pancreatic enzymes into the abdominal cavity & cause hemorrhaging

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6
Q

Patho:
An abdominal mass that’s a complication of acute pancreatitis

Labs:
Elevated serum amylase

Comps:
Rupture can release pancreatic enzymes into the abdominal cavity & cause hemorrhaging

Describes which condition?

A

Pancreatic pseudocyst

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7
Q

What is a pancreatic abscess?

  • Patho
  • Labs
  • Symptoms
A

Patho:
A mass in the pancreas filled with E.coli

Labs:
Elevated amylase

Signs:
1) Abdominal pain
2) High fever

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8
Q

Patho:
A mass in the pancreas filled with E.coli, a complication of acute pancreatitis

Labs:
Elevated amylase

Signs:
1) Abdominal pain
2) High fever

Describes which condition?

A

Pancreatic abscess

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9
Q

Describe the following for chronic pancreatitis:

What is it?

What causes it?
- main cause in adults vs children

What are the symptoms?

What are the lab findings?

What are the complications?

A

Patho:
Fibrosis of the pancreatic parenchyma

Causes:
#1 alcohol (adults)
#1 Cystic fibrosis (children)

Signs:
1) Epigastric abdominal pain (radiating to back)
2) Pancreatic insufficiency (malabsorption, steatorrhea, & fat soluble vitamin deficiency)

Labs/histo:
1) Dystrophic calcification of pancreatic parenchyma (Chain of lakes imaging) from dilated pancreatic ducts

Comps:
1) Secondary diabetes mellitus
2) Higher risk of pancreatic carcinoma

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10
Q

Patho:
Fibrosis of the pancreatic parenchyma

Causes:
#1 alcohol (adults)
#1 Cystic fibrosis (children)

Signs:
1) Epigastric abdominal pain (radiating to back)
2) Pancreatic insufficiency (malabsorption, steatorrhea, & fat soluble vitamin deficiency)

Labs/histo:
1) Dystrophic calcification of pancreatic parenchyma (Chain of lakes imaging) from dilated pancreatic ducts

Comps:
1) Secondary diabetes mellitus
2) Higher risk of pancreatic carcinoma

Describes which condition?

A

Chronic pancreatitis

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11
Q

Describe the following for a pancreatic carcinoma:

What is it?

What are some risk factors?

What are the symptoms?

What is the treatment?
- the prognosis

A

Patho:
An adenocarcinoma arising from the pancreatic ducts usually seen in 70yr plus

Risks:
1) Smoking
2) Chronic pancreatitis

Signs:
1) Epigastric abdominal pain
2) Weight loss
3) Obstructive jaundice
4) Pale stools
5) Palpable gallbladder (ass with tumors in the head of the pancreas)
6) Migratory thrombophlebitis (Trousseaus sign)

Rx:
Surgical resection involving en bloc removal of the neck/head of the pancreas, proximal duodenum, & gallbladder (Whipple procedure)

Prog:
Very poor prognosis 1yr survival

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12
Q

Patho:
An adenocarcinoma arising from the pancreatic ducts usually seen in 70yr plus

Risks:
1) Smoking
2) Chronic pancreatitis

Signs:
1) Epigastric abdominal pain
2) Weight loss
3) Obstructive jaundice
4) Pale stools
5) Palpable gallbladder (ass with tumors in the head of the pancreas)
6) Migratory thrombophlebitis (Trousseaus sign)

Rx:
Surgical resection involving en bloc removal of the neck/head of the pancreas, proximal duodenum, & gallbladder (Whipple procedure)

Prog:
Very poor prognosis 1yr survival

A

Pancreatic carcinoma

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13
Q

Describe the following for biliary atresia:

What is it?

What are the symptoms?

What are the complications?

A

Patho:
Failure of the biliary tree to form or early destruction of the extrahepatic biliary tree

Signs:
1) Jaundice (progress to cirrhosis)

Comps:
1) Biliary obstruction in the first 2 months of life

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14
Q

Patho:
Failure of the biliary tree to form or early destruction of the extrahepatic biliary tree

Signs:
1) Jaundice (progress to cirrhosis)

Comps:
1) Biliary obstruction in the first 2 months of life

Describes which condition?

A

Biliary atresia

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15
Q

Describe the following for Cholelithiasis (Gallstones):

What is it?
- What are the subtypes?

What are the causes?

A

Patho:
Solid round stones in the gallbladder due to precipitation of either cholesterol or bilirubin in the bile

Subtypes:
1) Cholesterol stones
2) Bilirubin stones

Causes:
1) Supersaturation of cholesterol or bilirubin
2) Decreased phospholipids (lethicin) or bile acids
3) Stasis

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16
Q

Patho:
Solid round stones in the gallbladder due to precipitation of either cholesterol or bilirubin in the bile

Subtypes:
1) Cholesterol stones
2) Bilirubin stones

Causes:
1) Supersaturation of cholesterol or bilirubin
2) Decreased phospholipids (lethicin) or bile acids
3) Stasis

Describes which condition?

A

Cholelithiasis (Gallstones)

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17
Q

Describe the following for cholesterol stones:

What are they?

What are the lab findings?

What are the symptoms?

What are the risk factors?

A

Patho:
A type of gallstone that are solid yellow round stones that are made up of supersaturated or precipitated cholesterol.

Labs/histo:
1) Radiolucent on imaging

Signs:
1) Biliary colic
2) Acute & chronic cholecystitis
3) Gallstone ileus
4) Gallbladder cancer

Risks:
1) Age (40+yrs)
2) Elevated estrogen (obesity, female, multiple preggos)
3) Clofibrate (lipid catabolism drug)
4) Native American descent
5) Chron’s disease
6) Cirrhosis

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18
Q

Patho:
A type of gallstone that are solid yellow round stones that are made up of supersaturated or precipitated cholesterol.

Labs/histo:
1) Radiolucent on imaging

Signs:
1) Biliary colic
2) Acute & chronic cholecystitis
3) Gallstone ileus
4) Gallbladder cancer

Risks:
1) Age (40+yrs)
2) Elevated estrogen (obesity, female, multiple preggos)
3) Clofibrate (lipid catabolism drug)
4) Native American descent
5) Chron’s disease
6) Cirrhosis

Describes which condition?

A

Cholesterol stones

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19
Q

Describe the following for Bilirubin stones:

What are they?

What are the lab findings?

What are the symptoms?

A

Patho:
A type of gallstone that are solid pigmented/dark round stones made up of supersaturated/precipitated bilirubin

Labs:
1) usually, radio opaque on imaging
2) Elevated bilirubin in bile (Extravascular hemolysis)

Signs:
1) Biliary colic
2) Biliary tract infection (E.coli, Ascaris lumbriocoides, & Clonorchis Sinesis)
3) Acute & chronic cholecystitis
4) Gallstone ileus
5) Gallbladder cancer

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20
Q

Patho:
A type of gallstone that are solid pigmented/dark round stones made up of supersaturated/precipitated bilirubin

Labs:
1) usually, radio opaque on imaging
2) Elevated bilirubin in bile (Extravascular hemolysis)

Signs:
1) Biliary colic
2) Biliary tract infection (E.coli, Ascaris lumbriocoides, & Clonorchis Sinesis)
3) Acute & chronic cholecystitis
4) Gallstone ileus
5) Gallbladder cancer

Describes which condition?

A

Bilirubin stones

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21
Q

A round worm infection that is contracted by the fecal-oral route that infects the biliary tract resulting in a high risk of which complication?

A

Gallstones due to Ascaris Lumbricoides

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22
Q

The Chinese liver fluke (common in Korea, China, & Vietnam) that infects the biliary tract causing an increased risk of developing which conditions?

A

Gallstones
Cholangitis
Cholangiocarcinoma

Due to a Clonorchis Sinensis infection

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23
Q

Describe the following for Biliary colic:

What is it?

What are the symptoms?

What are the complications?

A

Patho:
When a gallstone gets stuck in the cystic duct & the gallbladder has to contract against it

Signs:
1) Waxing & wanning RUQ pain (relieved with stone passing)

Comps:
1) Acute pancreatitis
2) Obstructive jaundice

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24
Q

Patho:
When a gallstone gets stuck in the cystic duct & the gallbladder has to contract against it

Signs:
1) Waxing & wanning RUQ pain (relieved with stone passing)

Comps:
1) Acute pancreatitis
2) Obstructive jaundice

Describes which condition?

A

Biliary colic

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25
Q

Acute cholecystitis:

What is it?

What are the symptoms?

What are the lab findings?
-Imaging

What is a complication?

A

Patho:
Acute inflammation of the gallbladder wall, because of an impacted stone in the cystic duct resulting in dilation with pressure ischemia, bacterial overgrowth (E.coli), & inflammation

Signs:
1) RUQ pain (radiating to the right scapula)
2) Fever
3) Nausea/vomiting
4) Murphy sign (sudden pausing during inspiration upon deep palpation of the RUQ due to pain)

Labs:
1) Elevated WBC count
2) Elevated serum alkaline phosphatase (duct damage)
3) Elevated CRP

Imaging:
gallbladder wall thickening and/or edema (double wall sign)

Comps:
1) Risk of rupture in not treated

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26
Q

Patho:

An impacted gallstone in the cystic duct causing gallbladder wall inflammation & thickening (double wall), pressure ischemia, & ecoli overgrowth

Signs:
1) RUQ pain (radiating to the right scapula)
2) Fever/Nausea/vomiting
5) Murphy sign

Labs:
1) Elevated WBC count
2) Elevated serum alkaline phosphatase
3) Elevated CRP

Comps:
1) Risk of rupture in not treated

Describes which condition?

A

Acute cholecystitis

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27
Q

Describe the following for Cholelithiasis:

What is it? & What causes it?

What are the lab findings?
- imaging

A

Patho/causes:
Gallstones in the gallbladder due to bile cholesterol oversaturation, stasis, or impaired bile circulation (precipitation of gallstones)

Normal labs

Imaging:
- Gallstones with a post-acoustic shadow

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28
Q

Patho/causes:
Gallstones in the gallbladder due to bile cholesterol oversaturation, stasis, or impaired bile circulation (precipitation of gallstones)

Normal labs

Imaging:
- Gallstones with a post-acoustic shadow

Describes which condition?

A

Cholelithiasis

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29
Q

Describe the following for Choledocholithiasis:

What is it?

What are the symptoms?

What are the lab findings?
-images

A

Patho:
Gallstones in the common bile duct

Signs:
1) RUQ pain
2) Jaundice

Labs:
Elevated total bilirubin
Elevated GGT (glutamyl transpeptidase)
Elevated ALP, AST, & ALT

Imaging:
Dilated common bile duct

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30
Q

Patho:
Gallstones in the common bile duct

Signs:
1) RUQ pain
2) Jaundice

Labs:
Elevated total bilirubin
Elevated GGT (glutamyl transpeptidase)
Elevated ALP, AST, & ALT

Imaging:
Dilated common bile duct

Describes which condition?

A

Choledocholithiasis

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31
Q

Describe the following for acute/ascending cholangitis:

What is it?

What are the symptoms?

What are the complications?

A

Patho:
Bacterial infection of the bile ducts usually from gram -ve enteric bacteria

Signs:
1) Sepsis (fever/chills)
2) Jaundice
4) Abdominal pain

Comps:
1) Increased risk

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32
Q

Patho:
Bacterial infection of the bile ducts usually from gram -ve enteric bacteria

Signs:
1) Sepsis (fever/chills)
2) Jaundice
4) Abdominal pain

Comps:
1) Increased risk

Describes which condition?

A

Acute/ascending cholangitis

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33
Q

Describe the following for Chronic cholecystitis:

What is it?

What causes it?

What are the lab findings?
- histology

What are the symptoms?

What are the complications?

A

Patho:
Chronic inflammation of the gastric wall

Causes:
From chemical irritation due to long-standing cholelithiasis with/without superimposed bouts of acute cholecystitis

Labs/histo:
Rokitansky-Aschoff sinus (Herniation of the gallbladder mucosa into the muscular wall)

Signs:
1) Vague RUQ pain that’s worse after eating

Comp:
Porcelain gallbladder (it becomes shrunken & hard because of chronic inflammation, fibrosis & dystrophic calcification)

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34
Q

Patho:
Chronic inflammation of the gastric wall

Causes:
From chemical irritation due to long-standing cholelithiasis with/without superimposed bouts of acute cholecystitis

Labs/histo:
Rokitansky-Aschoff sinus (Herniation of the gallbladder mucosa into the muscular wall)

Signs:
1) Vague RUQ pain that’s worse after eating

Comp:
Porcelain gallbladder (it becomes shrunken & hard because of chronic inflammation, fibrosis & dystrophic calcification)

Describes which condition?

A

Chronic Cholecystitis

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35
Q

Describe the following for Gallstones:

What are they & what causes them?

A

Patho/cause:

Gallstones enter the small bowel via a fistula that connects the gallbladder & ileum. It can result in small bowel obstruction

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36
Q

Patho/cause:

Gallstones enter the small bowel via a fistula that connects the gallbladder & ileum. It can result in small bowel obstruction

Describes which condition?

A

Gallstones

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37
Q

Describe the following for Gall bladder carcinoma:

What is it?

What are the risk factors?

What is the classic presentation & prognosis?

A

Patho:
An adenocarcinoma arising from the glandular epithelium lining the gallbladder wall

Risks:
1) Gallstones (especially with the complication of porcelain gallbladder)

Classical prez:
The sins of cholecystitis typically in elderly women

Prognosis is POOR

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38
Q

Patho:
An adenocarcinoma arising from the glandular epithelium lining the gallbladder wall

Risks:
1) Gallstones (especially with the complication of porcelain gallbladder)

Classical prez:
The sins of cholecystitis typically in elderly women

Prognosis is POOR

Describes which condition?

A

Gall bladder cancer

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39
Q

Describe the following for Metastasis to the liver:

What is it & What are the cancers that most common metastasize to the liver?

A

More common than primary liver tumors. The most common types of cancer that metastasized to the liver include
- Colon
- Breast
- Lung
- Pancreas

They result in multiple nodules on the liver & it can be clinically detected as hepatosplenomegaly with a nodular free edge of the liver

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40
Q

More common than primary liver tumors. The most common types of cancer that metastasized to the liver include
- Colon
- Breast
- Lung
- Pancreas

They result in multiple nodules on the liver & it can be clinically detected as hepatosplenomegaly with a nodular free edge of the liver

Describes which condition?

A

Metastasis to the liver

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41
Q

Describe the following for Reye syndrome:

What is it?

What are the lab findings?

What are the symptoms?

What are the complications?

A

Patho:
When kids with viral infections are given aspirin get fulminant of liver failure & encephalopathy

(mitochondrial damage causes deficient B-oxidation & micro vesicular changes)

Labs:
Elevated AST/ALT
Increased PT time
Damaged mitochondria of hepatocytes
Hypoglycemia
Hyperammonia
Microvesicular hepatic steatosis

Signs:
1) Hepatomegaly
2) Nausea/vomiting
3) Encephalopathy (increased ICP)

Comps:
Coma & eventual death

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42
Q

Patho:
When kids with viral infections are given aspirin get fulminant of liver failure & encephalopathy

(mitochondrial damage causes deficient B-oxidation & micro vesicular changes)

Labs:
Elevated AST/ALT
Increased PT time
Damaged mitochondria of hepatocytes
Hypoglycemia
Hyperammonia
Microvesicular hepatic steatosis

Signs:
1) Hepatomegaly
2) Nausea/vomiting
3) Encephalopathy (increased ICP)

Comps:
Coma & eventual death

Describes which condition?

A

Reye syndrome

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43
Q

Describe the following for Hepatic adenoma:

What is it?
- associated with what?

What are the complications?

A

Patho/ass:
A benign liver tumor that is associated with oral contraceptive use (regresses with cessation)

Comps:
Risk of rupture which can cause intraperitoneal bleeding (especially in preggos because the subcapsular tumor grows when exposed to estrogen)

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44
Q

Patho/ass:
A benign liver tumor that is associated with oral contraceptive use (regresses with cessation)

Comps:
Risk of rupture which can cause intraperitoneal bleeding (especially in preggos because the subcapsular tumor grows when exposed to estrogen)

Describes which condition?

A

Hepatic adenoma

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45
Q

Describe the following for hepatocellular carcinoma:

What is it?

What are the symptoms?

What are the risk factors?

What are the lab findings?

What are the complications?

A

Patho:
A malignant liver tumor

Signs:
1) Painful hepatosplenomegaly
2) Ascites
3) Weight loss/anorexia
4) Right upper quadrant tenderness
5) Jaundice

Risks:
1) Chronic hepatitis infection (HCV or HBV)
2) Cirrhosis
- Alcoholic/non-alcoholic fatty liver disease
-Hemochromatosis
- Wilsons disease
-A1AT deficiency
3) Aflatoxins (derived from aspergillus resulting in P52 mutations)

Labs:
Elevated a fetoprotein (tumor marker)

Comps:
Risk of progressing to Budd-Chiari syndrome
(Cirrhosis masks symptoms resulting in late diagnosis & very poor prognosis)

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46
Q

Explain what Budd-Chiari syndrome is?

-Patho
- Symptoms
- complication of which condition?

A

Liver infarction due to hepatic vein obstruction (i.e thrombosis or compression) resulting in hepatic venous congestion & increased sinusoidal pressure.

Labs/histo:
1) Centrilobular necrosis
2) Nutmeg liver

Signs:
1) Abdominal pain
2) Painful hepatomegaly
3) Ascites
4) Jaundice
5) increased perfusion of portocaval anastomoses (e.g., esophageal varices, or caput medusae)

Complication of:
Hepatocellular carcinoma

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47
Q

Liver infarction due to hepatic vein obstruction (i.e thrombosis or compression) resulting in hepatic venous congestion & increased sinusoidal pressure.

Labs/histo:
1) Centrilobular necrosis
2) Nutmeg liver

Signs:
1) Abdominal pain
2) Painful hepatomegaly
3) Ascites
4) Jaundice
5) increased perfusion of portocaval anastomoses (e.g., esophageal varices, or caput medusae)

Complication of:
Hepatocellular carcinoma

Describes which condition?

A

Budd-Chiari syndrome

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48
Q

Patho:
A malignant liver tumor

Signs:
1) Painful hepatosplenomegaly
2) Ascites
3) Weight loss/anorexia
4) Right upper quadrant tenderness
5) Jaundice

Risks:
1) Chronic hepatitis infection (HCV or HBV)
2) Cirrhosis
- Alcoholic/non-alcoholic fatty liver disease
-Hemochromatosis
- Wilsons disease
-A1AT deficiency
3) Aflatoxins (derived from aspergillus resulting in P52 mutations)

Labs:
Elevated a fetoprotein (tumor marker)

Comps:
Risk of progressing to Budd-Chiari syndrome
(Cirrhosis masks symptoms resulting in late diagnosis & very poor prognosis)

Describes which condition?

A

Hepatocellular carcinoma

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49
Q

Describe the following for Wilsons disease:

What is it/What causes it?

What are the symptoms?

What are the lab findings?

What are the complications?

What is the treatment?

A

Patho/Cause:
An AUTO recessive defect (ATP7B) that presents in childhood. Reducing ATP-mediated hepatocyte copper transport into the bile & less corporation into the ceruloplasmin. The copper builds up in the liver & leaks into serum to deposit in tissues forming free radicals causing tissue damage.

Signs:
1) Cirrhosis
2) Neurological changes
(behavior changes, dementia, chorea, & parkinsonian symptoms)
3) Kayser Fleisher rings in the cornea

4) Hepatosplenomegaly
5) Jaundice
6) Ascites
7) Hepatic encephalopathy
8) Cirrhosis
9) Portal hypertension

Labs:
Elevated copper in the liver & urine
Reduced serum ceruloplasmin

Comps:
1) Risk of hepatocellular carcinoma

Rx:
D-penicillamine

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50
Q

Patho/Cause:
An AUTO recessive defect (ATP7B) that presents in childhood. Reducing ATP-mediated hepatocyte copper transport into the bile & less corporation into the ceruloplasmin. The copper builds up in the liver & leaks into serum to deposit in tissues forming free radicals causing tissue damage.

Signs:
1) Cirrhosis
2) Neurological changes
(behavior changes, dementia, chorea, & parkinsonian symptoms)
3) Kayser Fleisher rings in the cornea

4) Hepatosplenomegaly
5) Jaundice
6) Ascites
7) Hepatic encephalopathy
8) Cirrhosis
9) Portal hypertension

Labs:
Elevated copper in the liver & urine
Reduced serum ceruloplasmin

Comps:
1) Risk of hepatocellular carcinoma

Rx:
D-penicillamine

Describes which condition?

A

Wilsons disease

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51
Q

Describe the following for Primary Biliary cirrhosis:

What is it/What causes it?
-Associations?

What are the symptoms?

What are the lab findings?

What are the complications?

A

Patho/causes:
Chronic Autoimmune granulomatous destruction of small-medium sized intrahepatic bile ducts (progressive ductopenia) eventually leading to chronic cholestasis & symptoms of cirrhosis & portal HTN.

Ass:
females around 40yrs old & often associated with autoimmune conditions.

Signs:
1) Obstructive jaundice
2) Pale stool
3) dark urine
4) Hepatomegaly
5) RUQ discomfort
6) Splenomegaly
7) Hyperpigmentation
8) Xanthomas and xanthelasma
9) Pruritis

Labs:
Anti-mitochondrial antibodies
↑ ALP, ↑ GGT, ↑ direct bilirubin & indirect
↑ IgM
hypercholesterolemia

Comps:
Cirrhosis & portal HTN
Osteoporosis

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52
Q
A
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53
Q

What are the stages of primary biliary cirrhosis

1
2
3
4

A

Stage I: lymphocytic infiltration of portal areas and periductal granulomas

Stage II: bile duct ductopenia, progressive fibrosis

Stage III: bridging fibrosis

Stage IV: liver cirrhosis

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54
Q

Stage I: lymphocytic infiltration of portal areas and periductal granulomas

Stage II: bile duct ductopenia, progressive fibrosis

Stage III: bridging fibrosis

Stage IV: liver cirrhosis

Are the stages of which condition?

A

Primary biliary cirrhosis

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55
Q

Patho/causes:
Autoimmune granulomatous destruction of small-medium sized intrahepatic bile ducts (progressive ductopenia) eventually leading to chronic cholestasis & symptoms of cirrhosis & portal HTN.

Ass:
females around 40yrs old & often associated with autoimmune conditions.

Signs:
1) Obstructive jaundice
2) Pale stool
3) dark urine
4) Hepatomegaly
5) RUQ discomfort
6) Splenomegaly
7) Hyperpigmentation
8) Xanthomas and xanthelasma

Labs:
Anti-mitochondrial antibodies
↑ ALP, ↑ GGT, ↑ direct bilirubin
↑ IgM
hypercholesterolemia

Comps:
Cirrhosis & portal HTN
Osteoporosis

Describes which condition?

A

Primary biliary cirrhosis

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56
Q

What are the differentials used to differentiate Primary biliary cirrhosis & Primary sclerosing cholangitis?

  • Antibodies
  • Key symptoms
  • Complications
A

PBC:
Labs:
AMA-ME (anti-mitochondrial antibodies)
Signs:
1) Xanthomas/dyslipidemia
2) Pruritis
Comp:
Osteoporosis, cirrhosis, & portal HTN

PSC:
Labs:
P-ANCA +ve
Ass: Ulcerative colitis
Signs: Acute cholangitis

Comp:
Risk of cholangiocarcinoma

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57
Q

Describe the following for Primary Sclerosing cholangitis:

What is it?
- association?

What are the symptoms?

What are the lab findings?

What are the complications?

A

Patho:
Inflammation & fibrosis of the intrahepatic/extrahepatic bile ducts that is associated with ulcerative colitis

Signs:
1) Obstructive jaundice
Signs of cholestasis
1) Jaundice/scleral icterus
2) Pale stool
3) Dark urine
4) Fatigue
5) Hepatosplenomegaly
6) Portal hypertension

Labs/histo:
P-ANCA +ve
Periductal fibrosis (onion-skin app)
Dilation of involved regions (beaded app)

Comps:
Cirrhosis & Risk of cholangiocarcinoma

58
Q

Patho:
Inflammation & fibrosis of the intrahepatic/extrahepatic bile ducts that is associated with ulcerative colitis

Signs:
1) Obstructive jaundice
Signs of cholestasis
1) Jaundice/scleral icterus
2) Pale stool
3) Dark urine
4) Fatigue
5) Hepatosplenomegaly
6) Portal hypertension

Labs/histo:
P-ANCA +ve
Periductal fibrosis (onion-skin app)
Dilation of involved regions (beaded app)

Comps:
Cirrhosis & Risk of cholangiocarcinoma

Describes which condition?

A

Primary sclerosing Cholangitis

59
Q

Describe the following for Hemochromatosis:

What is it?

What are the causes?
-primary vs secondary

What are the symptoms?

What are the lab findings?

What are the complications?

What is the treatment?

A

Patho/causes:
An accumulation of iron in the body resulting in tissue deposits (hemosiderosis) & organ damage (hemochromatosis) due to free radicals production from the iron

Causes:
1) AUTO recessive defect in iron absorption via mutations in HFE gene (282Y) (primary)
2) A complication of chronic transfusions (secondary)

Signs:
1) Triad (cirrhosis, secondary diabetes mellitus, & bronze skin)
2) Dilated cardiomyopathy
3) Arrythmias
4) Gonadal dysfunction (test atrophy)

Labs/histo:
Prussian blue stain (iron)
Lipofuscin in liver (brown wear/tear)
Elevated ferritin
Elevated serum iron
Elevated saturation %
Reduced TIBC

Comps:
Risk of hepatocellular carcinoma

Rx:
Phlebotomy

60
Q

Patho/causes:
An accumulation of iron in the body resulting in tissue deposits (hemosiderosis) & organ damage (hemochromatosis) due to free radicals production from the iron

Causes:
1) AUTO recessive defect in iron absorption via mutations in HFE gene (282Y) (primary)
2) A complication of chronic transfusions (secondary)

Signs:
1) Triad (cirrhosis, secondary diabetes mellitus, & bronze skin)
2) Dilated cardiomyopathy
3) Arrythmias
4) Gonadal dysfunction (test atrophy)

Labs/histo:
Prussian blue stain (iron)
Lipofuscin in liver (brown wear/tear)
Elevated ferritin
Elevated serum iron
Elevated saturation %
Reduced TIBC

Comps:
Risk of hepatocellular carcinoma

Rx:
Phlebotomy

Describes which condition?

A

Hemochromatosis

61
Q

How does primary hemochromatosis result in iron deposition in tissues?

A

It’s because of an AUTO recessive defect resulting in an HFE mutation causing tyrosine to replace cysteine in the 282Y amino acid inhibiting iron absorption

62
Q

Describe the following for non-alcoholic fatty liver disease:

What is it?

What is a risk factor?

What are the symptoms?

What are the lab findings?

A

Patho:
Fatty change, hepatitis, &/or cirrhosis that develops without exposure to alcohol

Risk:
Obesity

Signs:
1) Hepatomegaly
2) Signs of cirrhosis
(jaundice, scleral icterus, spider angioma, gynecomastia, palmar erythema, ascites, asterixis, and encephalopathy)

Labs:
Elevated liver enzymes ALT> AST

63
Q

Patho:
Fatty change, hepatitis, &/or cirrhosis that develops without exposure to alcohol

Risk:
Obesity

Signs:
1) Hepatomegaly
2) Signs of cirrhosis
(jaundice, scleral icterus, spider angioma, gynecomastia, palmar erythema, ascites, asterixis, and encephalopathy)

Labs:
Elevated liver enzymes ALT> AST

Describes which condition?

A

Non-alcoholic fatty liver disease

64
Q

Describe the following for Alcohol-induced fatty liver:

What is it & how is it treated?

What are the symptoms?

What are the lab findings?
-Histology

A

Patho/Rx:
Copious consumption of alcohol results in excessive fat storage in the liver (only spot to metabolize alcohol). Reversible with alcohol cessation.

Symptoms:
1) Hepatomegaly

Labs/histo:
Heavy/greasy liver

65
Q

Patho/Rx:
Copious consumption of alcohol results in excessive fat storage in the liver (only spot to metabolize alcohol). Reversible with alcohol cessation.

Symptoms:
1) Hepatomegaly

Labs/histo:
Heavy/greasy liver

Describes which condition?

A

Alcohol-induced fatty liver

66
Q

Describe the following for alcohol-induced hepatitis:

What is it?

What are the symptoms?

What are the lab findings?
- histology

What are the complications?

A

Patho:
Metabolism of alcohol produces a byproduct acetaldehyde which mediates damage to the liver tissue.

Ethanol metabolism leads to excess NADH, promoting triglyceride synthesis, liver inflammation, and cirrhosis.

Signs:
1) Cirrhosis symptoms
(jaundice, scleral icterus, spider angioma, gynecomastia, palmar erythema, ascites, asterixis, and encephalopathy)
2) Swollen hepatocytes
3) Mallory bodies
4) Necrosis
5) Painful hepatosplenomegaly

Labs/histo:
Elevated AST/ALT
Elevated GGT
Elevated CDT
Macrocytic anemia

67
Q

Patho:
Metabolism of alcohol produces a byproduct acetaldehyde which mediates damage to the liver tissue.

Ethanol metabolism leads to excess NADH, promoting triglyceride synthesis, liver inflammation, and cirrhosis.

Signs:
1) Cirrhosis symptoms
(jaundice, scleral icterus, spider angioma, gynecomastia, palmar erythema, ascites, asterixis, and encephalopathy)
2) Swollen hepatocytes
3) Mallory bodies
4) Necrosis
5) Painful hepatosplenomegaly

Labs/histo:
Elevated AST/ALT
Elevated GGT
Elevated CDT
Macrocytic anemia

Describes which condition

A

Alcohol-induced hepatitis

68
Q

Describe the following for Chronic hepatitis:

What is it?

What are the symptoms?

What are the complications?

A

Patho:
A hepatitis infection due to the Hep virus, EBV, or CMV that lasts longer than 6 months

Signs:
1) Jaundice
2) Dark urine
3) Malaise/fever/nausea
4) Upper abdominal pain
5) Weight loss

Labs:
IgM +ve
Inflammation in the portal tract mostly
↑ GGT
↑ Alkaline phosphatase
↑ Bilirubin
↑ PT/INR
↓ Albumin
↓ Total protein

Comps:
Risk of progressing to cirrhosis

69
Q

Patho:
A hepatitis infection due to the Hep virus, EBV, or CMV that lasts longer than 6 months.

Signs:
1) Jaundice
2) Dark urine
3) Malaise/fever/nausea
4) Upper abdominal pain
5) Weight loss

Labs:
IgG +ve
Inflammation in the portal tract mostly
↑ GGT
↑ Alkaline phosphatase
↑ Bilirubin
↑ PT/INR
↓ Albumin
↓ Total protein

Comps:
Risk of progressing to cirrhosis

Describes which condition?

A

Chronic hepatitis

70
Q

Describe the following for Cirrhosis:

What is it?

What are the lab findings?

What are the symptoms?

A

Patho:
End-stage liver damage due to fibrosis of the liver which is mediated by TGF-B in stellate cells (underneath the endothelium lining the sinusoids)

Labs/histo:
Bands of fibrosis
Regenerative nodules of hepatocytes

Signs:
1) Ascites
2) Congestive splenomegaly & hypersplenism
3) Portal systemic shunts
(esophageal varices, hemorrhoids, caput medusae)
4) Hepatorenal syndrome (rapid renal failure secondary to cirrhosis)
5) Reduced detoxification
(mental changes, asterixis, coma, gynecomastia, spider angiomata, & palmar erythema)
6) Jaundice
7) Reduced protein synthesis (Hypoalbuminemia/edema & coagulopathy/less clotting factors)

71
Q

Patho:
End-stage liver damage due to fibrosis of the liver which is mediated by TGF-B in stellate cells (underneath the endothelium lining the sinusoids)

Labs/histo:
Bands of fibrosis
Regenerative nodules of hepatocytes

Signs:
1) Ascites
2) Congestive splenomegaly & hypersplenism
3) Portal systemic shunts
(esophageal varices, hemorrhoids, caput medusae)
4) Hepatorenal syndrome (rapid renal failure secondary to cirrhosis)
5) Reduced detoxification
(mental changes, asterixis, coma, gynecomastia, spider angiomata, & palmar erythema)
6) Jaundice
7) Reduced protein synthesis (Hypoalbuminemia/edema & coagulopathy/less clotting factors)

Describes which condition?

A

Cirrhosis

72
Q

Describe the following for acute hepatitis:

What is it?/ what causes it?

What are the symptoms?

What are the lab findings?
- histology

A

Patho/causes:
Inflammation of the liver due to hep viruses, EBV, or CMV infection lasting less than 6 months

Signs:
1) Jaundice
2) Dark urine
3) Malaise/fever/nausea

Labs/histo:
IgM +ve
Elevated ALT> AST
Elevated CB
Elevated UCB
Inflamed liver lobules with portal cell hepatocyte apoptosis

73
Q

Patho/causes:
Inflammation of the liver due to hep viruses, EBV, or CMV infection lasting less than 6 months

Signs:
1) Jaundice
2) Dark urine
3) Malaise/fever/nausea

Labs/histo:
IgM +ve
Elevated ALT> AST
Elevated CB
Elevated UCB
Inflamed liver lobules with portal cell hepatocyte apoptosis

Describes which condition?

A

Acute hepatitis

74
Q

Describe the following for Hepatitis B infection:

How is it transmitted?

What is the pathogenesis?

What are the symptoms?

A

Trans:
Parenternal (IV drugs, Birth, Sex)

Patho:
It causes acute hepatitis that can progress to chronic hepatitis in ~20% of cases

Signs:
1) Fever, skin rash, arthralgias, myalgias, fatigue
2) Nausea, anorexia
3) Jaundice
4) Right upper quadrant pain

75
Q

Trans:
Parenternal (IV drugs, Birth, Sex)

Patho:
It causes acute hepatitis that can progress to chronic hepatitis in ~20% of cases

Signs:
1) Fever, skin rash, arthralgias, myalgias, fatigue
2) Nausea, anorexia
3) Jaundice
4) Right upper quadrant pain

Describes which condition?

A

HBV infection

76
Q

What are the serological markers of an acute HBV infection?

A

HBsAG+ve

HBeAG & HBV DNA +ve

HBcAB IgM+ve

77
Q

HBsAG+ve

HBeAG & HBV DNA +ve

HBcAB IgM+ve

These serological markers describe which phase of an HBV infection?

A

Acute HBV infection

78
Q

What are the serological markers of a window HBV infection?

A

HBaAB IgM+ve

79
Q

HBaAB IgM+ve

serological markers describe which phase of an HBV infection?

A

Window HBV infection

80
Q

What are the serological markers of a resolved HBV infection?

A

HBcAB IgG+ve

HBaAB IgG+ve

81
Q

HBcAB IgG+ve

HBaAB IgG+ve

These serological markers describe which phase of an HBV infection?

A

Resolved HBV infection

82
Q

What are the serological markers of a chronic HBV infection?

A

HBaAG +ve for over 6 months

HBeAG & HBV DNA +ve or -ve

HBcAB IgG +ve

83
Q

HBaAG +ve for over 6 months

HBeAG & HBV DNA +ve or -ve

HBcAB IgG +ve

These serological markers describe which phase of an HBV infection?

A

A chronic HBV infection

84
Q

What are the serological markers of an HBV immunization?

A

HBsAB IgG+ve

85
Q

HBsAB IgG+ve is the serological marker for which type of HBV serotype?

A

Immunized to HBV

86
Q

Describe the following for Hepatitis C infection:

How is it transmitted?

What is the pathogenesis?

What are the symptoms?

What is the diagnostic test used?
- labs

A

Trans:
Parenternal (IV drugs & Sex)

Patho:
It causes acute hepatitis that will progress to chronic hepatitis

Signs:
1) Malaise, fever, myalgias, arthralgias
2) RUQ pain
3) Painful hepatomegaly
4) Nausea, vomiting, diarrhea
6) Jaundice

Tests:
HCV-RNA test to confirm infection

Labs:
Low levels of RNA indicate recovery
↑ GGT
↑ Alkaline phosphatase
↑ Bilirubin
↑ PT/INR
↓ Albumin
↓ Total protein

87
Q

Trans:
Parenternal (IV drugs & Sex)

Patho:
It causes acute hepatitis that will progress to chronic hepatitis

Signs:
1) Malaise, fever, myalgias, arthralgias
2) RUQ pain
3) Painful hepatomegaly
4) Nausea, vomiting, diarrhea
6) Jaundice

Tests:
HCV-RNA test to confirm infection

Labs:
Low levels of RNA indicate recovery
↑ GGT
↑ Alkaline phosphatase
↑ Bilirubin
↑ PT/INR
↓ Albumin
↓ Total protein

Describes which condition?

A

HCV infection

88
Q

Describe the following for Hepatitis D:

What is the pathogenesis?
- what are the effects of each infection route

A

Patho:
Hep D needs Hep B to cause infection, it can happen in two ways:

1) Person with prior HBV gets infected with HDV (B before D) results in a super infection
- risk of liver cirrhosis and Hepatocellular carcinoma

2) HBV & HDV infection at the same time resulting in a co infection (less severe)
- severe acute hepatitis.

89
Q

Describe the following for Hep A & Hep E infections:

How are they transmitted?

What effect do they both cause?

What are the serological markers?
- active infection vs protection

What is a major complication of Hep E infections in preggos?

A

Trans:
Both are transmitted via the fecal-oral route (via food/water) usually in poorer areas

Hep A is for trAvelers
Hep E is for sEEfood

Effect:
Both cause acute hepatitis only

Serological markers:

Anti-virus IgM +ve = active infection
Anti-virus IgG +ve = protective
(either post infection or immunization)

Comp of Hep E in preggos:
Progresses to fulminant hepatitis (Liver failure with massive liver necrosis)

90
Q

Trans:
Both are transmitted via the fecal-oral route (via food/water) usually in poorer areas associated with travelers & shellfish

Effect:
Both cause acute hepatitis only

Serological markers:

Anti-virus IgM +ve = active infection
Anti-virus IgG +ve = protective
(either post infection or immunization)

Comp in preggos:
Progresses to fulminant hepatitis (Liver failure with massive liver necrosis)

Describes which two conditions?

A

Hep A (trAvelers) & Hep E (sEEfood & prEggo complications)

91
Q

Describe the following for viral hepatitis:

What is it?
-causes

What are the symptoms?

What are the lab findings?

A

Patho/cause”
Inflammation that disrupts the hepatocytes & small bile ductulus due to infection from either a hepatitis virus strain, EBV, or CMV

Signs:
1) Jaundice
2) Dark urine
3) Pale stools & pruritis

Labs:
Elevated Conjugated bilirubin
Elevated Alkaline phosphatase
Reduced Urobilinogen (urine)

92
Q

Patho/cause”
Inflammation that disrupts the hepatocytes & small bile ductulus due to infection from either a hepatitis virus strain, EBV, or CMV

Signs:
1) Jaundice
2) Dark urine
3) Pale stools & pruritis
4) Hypercholesterolemia (xanthomas, steatorrhea, & malabsorption of fat soluble vitamins)

Labs:
Elevated Conjugated bilirubin
Elevated Alkaline phosphatase
Reduced Urobilinogen (urine)

Describes which condition?

A

Viral hepatitis

93
Q

Describe the following for gallstone ileus:

What is it?/what causes it?

What are the symptoms?

A

Patho:
Gallstones enter & block the small bowel (ileus) it’s caused by cholecystitis & the formation of a fistula connecting the gallbladder to the small bowel.

Signs:
1) RUQ pain
(More severe and prolonged (may last > 6 hours) than in cholelithiasis)

2) Nausea, vomiting

3) extrahepatic cholestasis
(e.g., jaundice, pale stool, dark urine, pruritus)

94
Q

Patho:
Gallstones enter & block the small bowel it’s caused by cholecystitis & the formation of a fistula connecting the gallbladder to the small bowel.

Signs:
1) RUQ pain
(More severe and prolonged (may last > 6 hours) than in cholelithiasis)

2) Nausea, vomiting

3) extrahepatic cholestasis
(e.g., jaundice, pale stool, dark urine, pruritus)

Describes which condition?

A

Gallstone ileus

95
Q

Describe the following for Jaundice:

What is it?

What causes it?

What are the key symptoms?

A

Patho:
Increased concentration of bilirubin (>2.5)

Causes:
Disturbances in bilirubin metabolism

Signs:
1) Scleral icterus (earliest)
2) Yellow skin

96
Q

Describe the following for extra or intrahepatic vascular hemolysis:

What is it?

What are the symptoms?

What are the lab findings?

What is a complication?

A

Patho:
Increased destruction of RBCs resulting in very high unconjugated bilirubin levels that overwhelms the liver

Signs:
1) Dark urine (high urobilinogen)
2) Jaundice
3) Scleral icterus

Labs:
Elevated unconjugated bilirubin
Elevated urobilinogen
↓ Hb, Hct, and RBC count
↑ MCV

Comp:
Risk of pigmented bilirubin gallstones

97
Q

Patho:
Increased destruction of RBCs resulting in very high unconjugated bilirubin levels that overwhelms the liver

Signs:
1) Dark urine (high urobilinogen)
2) Jaundice
3) Scleral icterus

Labs:
Elevated unconjugated bilirubin
Elevated urobilinogen
↓ Hb, Hct, and RBC count
↑ MCV

Comp:
Risk of pigmented bilirubin gallstones

Describes jaundice due to which conditions?

A

Intra/extravascular hemolysis

98
Q

Describe the following for physiological jaundice of newborns:

What is it?

What are the symptoms?

What are the lab findings?

What is a complication?

What is the treatment?

A

Patho:
Newborn livers have low uridine glucuronyl transferase (UGT) levels resulting in low levels of conjugation > 24 hours after birth

NOTE it’s always unconjugated hyperbilirubinemia

Signs:
1) Kernicterus (UCB deposits in the basal ganglia causing neuro defs)
2) Jaundice
3) Scleral icterus
4) Hypotonia
5) Poor feeding

Labs:
Elevated Unconjugated bilirubin

Comp:
Kernicterus (chronic bilirubin encephalopathy) resulting in:
1) Cerebral paresis, hearing impairment, vertical gaze palsy
2) Movement disorder (choreoathetosis)

Rx:
Phototherapy (to make UCB water soluble)

99
Q

Patho:
Newborn livers have low uridine glucuronyl transferase (UGT) levels resulting in low levels of conjugation > 24 hours after birth

NOTE it’s always unconjugated hyperbilirubinemia

Signs:
1) Kernicterus (UCB deposits in the basal ganglia causing neuro defs)
2) Jaundice
3) Scleral icterus
4) Hypotonia
5) Poor feeding

Labs:
Elevated Unconjugated bilirubin

Comp:
Kernicterus (chronic bilirubin encephalopathy) resulting in:
1) Cerebral paresis, hearing impairment, vertical gaze palsy
2) Movement disorder (choreoathetosis)

Rx:
Phototherapy (to make UCB water soluble)

Describes which condition?

A

Physiological jaundice of newborns

100
Q

Describe the following for Gilbert-Syndrome:

What is it/What causes it?

What are the symptoms?

What are the lab findings?

A

Patho/cause:
An AUTO recessive disorder causing Mutation in the promoter region of UGT1A1 gene resulting in mildly low UGT levels in the liver (reduced conjugation capacity)

Signs:
1) Jaundice during stress
(i.e. severe infection, trauma, exhaustion, Fasting periods, Alcohol)

Labs:
Slightly ↑ unconjugated bilirubin but < 3 mg/dL (indirect)

101
Q

Patho/cause:
An AUTO recessive disorder causing Mutation in the promoter region of UGT1A1 gene resulting in mildly low UGT levels in the liver (reduced conjugation capacity)

Signs:
1) Jaundice during stress (i.e severe infection)

Labs:
Slightly ↑ unconjugated bilirubin but < 3 mg/dL (indirect)

Describes which condition?

A

Gilbert syndrome

102
Q

Another name for unconjugated bilirubin is …

A

indirect bilirubin

103
Q

Describe the following for Crigler-Najjar syndrome:

What is it?
- Type 1 vs 2

What causes it?

What are the symptoms?

What are the lab findings?

What is a complication?

What are the treatment options?

A

Patho:
Absent UGT enzyme in the liver resulting in reduced conjugation capacity

Type 1 = fatal AR
Type 2 = not fata AD

Causes:
autosomal recessive mutation resulting in nearly absent UGT

Signs:
1) persistent neonatal jaundice
2) Kernicterus (dep of UCB in basal ganglia)

Labs:
Elevated unconjugated bilirubin (↑ Indirect bilirubin (20–50 mg/dL))

Comps:
Kernicterus that is usually fatal

Rx:
Phototherapy
Plasmapheresis
Liver transplant

104
Q

Patho:
Absent UGT enzyme in the liver resulting in reduced conjugation capacity

Type 1 = fatal AR
Type 2 = not fata AD

Causes:
autosomal recessive mutation resulting in nearly absent UGT

Signs:
1) persistent neonatal jaundice
2) Kernicterus (dep of UCB in basal ganglia)

Labs:
Elevated unconjugated bilirubin (↑ Indirect bilirubin (20–50 mg/dL))

Comps:
Kernicterus that is usually fatal

Rx:
Phototherapy
Plasmapheresis
Liver transplant

Describes which condition?

A

Crigler-Najjar syndrome

105
Q

Describe the following for Dubin-Johnson syndrome:

What is it?/What causes it?

What are the symptoms?

What are the lab findings?

A

Patho/causes:
An AUTO recessive disorder that causes a deficiency in the bilirubin canalicular transport protein (aka multidrug resistance-associated protein 2 (MRP2)) resulting in impaired excretion of conjugated bilirubin from the hepatocytes into the bile canaliculi

Signs:
1) Jaundice/icterus
2) Dark urine
3) Liver biopsy: dark, granular pigmentation (due to accumulation of epinephrine metabolites)

Labs:
Elevated conjugated bilirubin (Direct hyperbilirubinemia)

106
Q

Patho/causes:
An AUTO recessive disorder that causes a deficiency in the bilirubin canalicular transport protein (aka multidrug resistance-associated protein 2 (MRP2)) resulting in impaired excretion of conjugated bilirubin from the hepatocytes into the bile canaliculi

Signs:
1) Jaundice/icterus
2) Dark urine
3) Liver biopsy: dark, granular pigmentation (due to accumulation of epinephrine metabolites)

Labs:
Elevated conjugated bilirubin (Direct hyperbilirubinemia)

Describes which condition?

A

Dubin-Johnson syndrome

107
Q

Describe the following for Rotor syndrome:

What is it?

A

Patho:
Defect in bilirubin canalicular transport protein (aka multidrug resistance-associated protein 2 (MRP2)) resulting in more mildly impaired excretion of conjugated bilirubin from the hepatocytes into the bile canaliculi

Signs:
1) Jaundice/icterus
2) Dark urine

Labs:
Elevated conjugated bilirubin (Direct hyperbilirubinemia)

108
Q

Patho:
Defect in bilirubin canalicular transport protein (aka multidrug resistance-associated protein 2 (MRP2)) resulting in more mildly impaired excretion of conjugated bilirubin from the hepatocytes into the bile canaliculi

Signs:
1) Jaundice/icterus
2) Dark urine

Labs:
Elevated conjugated bilirubin (Direct hyperbilirubinemia)

Describes which condition?

A

Roter syndrome

109
Q

Describe the following for Obstructive jaundice (aka biliary tract obstruction):

What is it?

What are the symptoms?

What are the lab findings?

A

Patho:
A complication that is associated with conditions like gallstones, pancreatic carcinoma, cholangiocarcinoma, Chinese liver fluke (c. SINENSIS)

Signs:
1) Jaundice/icterus
2) Pale stools & pruritis
3) Hypercholesterolemia
(Xanthomas, steatorrhea, & Malabsorption (fat soluble vitamin deficiency)

Labs:
Elevated conjugated bilirubin
Elevated alkaline phosphate
Reduced urobilinogen (urine)

110
Q

Patho:
A complication that is associated with conditions like gallstones, pancreatic carcinoma, cholangiocarcinoma, Chinese liver fluke (c. SINENSIS)

Signs:
1) Jaundice/icterus
2) Pale stools & pruritis
3) Hypercholesterolemia
(Xanthomas, steatorrhea, & Malabsorption (fat soluble vitamin deficiency)

Labs:
Elevated conjugated bilirubin
Elevated alkaline phosphate
Reduced urobilinogen (urine)

Describes which condition?

A

Obstructive jaundice

111
Q

Describe the normal metabolic pathway for bilirubin metabolism steps 1-5

A
  1. RBCs eaten by macrophages
  2. Protoporphyrin (from heme) is converted to UCB
  3. Albumin carried UCB to liver where UGT conjugates UCB
  4. CB is stored in gallbladder & released into the small bowel to aid digestion
  5. Intestinal flora convert CB to urobilinogen which is oxidized into sterobilin (brown) & urobili (yellow)
112
Q

Describe the following for MEN 1 mutations:

What is it?

What causes it?

What the effects?
-Main vs other

What are the common lab findings?

A

Patho:
Mutation of the MEN1 gene (located on chromosome 11) → altered expression of menin protein

Cause:
AUTO dominant mutation

Effects:
1) Pancreatic tumors
2) Parathyroid hyperplasia
3) Pituitary adenoma
4) Gastrinoma
5) Insulinoma

Labs:
Elevated insulin
Elevated C peptide
Reduced Serum glucose (<50)

113
Q

Patho:
Mutation of the MEN1 gene (located on chromosome 11) → altered expression of menin protein

Cause:
AUTO dominant mutation

Effects:
Main:
1) Primary hyperparathyroidism (parathyroid adenoma)

Other:
1) Pancreatic tumors
2) Parathyroid hyperplasia
3) Pituitary adenoma
4) Gastrinoma
5) Insulinoma

Labs:
Elevated insulin
Elevated C peptide
Reduced Serum glucose (<50)

Describes which condition?

A

MEN 1 mutations

114
Q

Describe the following for MEN 2 mutations:

What is it?

What the effects?
- most common vs other

What are the common lab findings?

What is the main treatment?

A

Patho:
Altered expression of the RET proto-oncogene → elevated tyrosine kinase activity

Effects:
Main:
1) Medullary thyroid carcinoma (A2)

Other:
1) Pheochromocytoma (A2)
2) Parathyroid adenoma (A2)
3) Ganglioneuromas of oral mucosa (B2)

Labs:
Low calcium

Rx. Prophylactic thyroidectomy

115
Q

MEN 1: 3 “P”s

A

= Parathyroid, Pancreas, Pituitary gland

116
Q

MEN 2A: 1 “M”, 2 “P”s =

A

Medullary thyroid carcinoma, Pheochromocytoma, Parathyroid

117
Q

MEN 2B: 2 “M”s, 1 “P” =

A

Medullary thyroid carcinoma, Marfanoid habitus/Multiple neuromas, Pheochromocytoma

118
Q

Patho:
Altered expression of the RET proto-oncogene → elevated tyrosine kinase activity

Effects:
Main:
1) Medullary thyroid carcinoma

Other:
1) Pheochromocytoma
2) Parathyroid adenoma
3) Ganglioneuromas of oral mucosa

Labs:
Low calcium

Rx. Prophylactic thyroidectomy

Describes which condition

A

MEN 2 mutations

119
Q

What do each color stones in cholelithiasis mean:

Black stones

Brown stones

Yellow stones

A

Black: Hemolysis (bilirubin)

Brown: Infection

Yellow: Cholesterol (fat)

120
Q

Dystrophic calcification of the gallbladder (porcelain) is associated with what condition?

A

Adenocarcinoma of the gall bladder

121
Q

Patient presents with Hypertension & Hypercalcemia, what are the 2 likely conditions & what would be the differentials?

A

Ret protp oncogene mutations causing either MEN 2A or 2B conditions

Differentials:

MEN 2A
1) Medullary thyroid carcinoma
2) Parathyroid tumor
3) Pheochromocytoma

MEN 2B
1) Mucosal neuromas *
2) Marfanoid Habitus *
3) Pheochromocytoma
4) Medullary thyroid carcinoma

122
Q

What is Migratory thrombophlebitis (trousseaus sign) indicative of?

A

Pancreatic adenocarcinoma
(arise from ductal cells that cause a hypercoagulable state & thrombi)

123
Q

Palpable, enlarged & NON-tender gallbladder with a +ve Courvoisier (obstructed common bile duct) is indicative of which condition?

A

Carcinoma of the head of the pancreas

124
Q

Grey turners sign vs Cullen sign

A

Gray turners sign: ecchymosis (bruising) of the flank

Cullen sign: ecchymosis (bruising) of the umbilicus

125
Q

Patient has anemia, mild hyperglycemia, & necrotic migratory erythema what is this indicative of?

A

Glucagonoma (alpha cell pancreatic tumor)

126
Q

Hepatic angiosarcoma is associated with exposure to which substance?

A

PVC (factory workers)

127
Q

What is a tumor marker that is indicative of Hepatocellular carcinoma?

A

Elevated alpha fetoprotein

128
Q

Hemochromatosis can progress to which condition?

A

Hepatocellular carcinoma (Elevated alpha fetoprotein)

129
Q

What is the most common primary tumor of the liver?

A

hemangioma (benign tumor that can rupture causing intraperitoneal bleeding)

130
Q

OCDs are associated with which type of liver cancer?

A

Hepatocellular adenoma which is a benign subcapsular tumor that can rupture & cause intraperitoneal hemorrhage

Rx resolves with ODP cessation

131
Q

Segmental dilation (beaded string) of larger bile ducts in the intrahepatic biliary tree causing cholelithiasis, abscesses, & cholangiocarcinoma is indicative of which condition?

A

Caroli disease

132
Q

Describe what Caroli disease is?

A

Segmental dilation (beaded string) of larger bile ducts in the intrahepatic biliary tree causing cholelithiasis, abscesses, & cholangiocarcinoma is indicative of which condition?

133
Q

What conditions are associated with Caroli disease?

A

Cholangiocarcinoma & polycystic kidney disease

134
Q

What conditions are associated with non-alcoholic fatty liver disease? & what is the histological changes?

A

Obesity
Dyslipidemia
Diabetes mellitus (2)

Histo:
Macrovascular steatosis

135
Q

Histology describes Bile duct hamartomas made up of small clusters of dilated ducts/cysts within fibrous stroma

These are indicative of what type of complexes?

A

Von Meyenburg complexes (embryonic bile duct remnants)

136
Q

What is the most common cause of cirrhosis in children (also emphysema)?

A

A1AT deficiency the PiZZ varient (worst one) (PiH normal & PiS mild)

Increased risk of Hepatocellular carcinoma

137
Q

What is hepatic hemangioma?

What are the symptoms?

What are the lab findings?

A

The most common BENIGN liver tumor influenced by estrogen levels (usually middle aged adults)

Signs:
1) RUQ pain
2) Early satiety
3) N/V

Labs:
The tumor is well demarcated, uniform & homogenous
Cavernous vascular spaces lined with flat endothelial cells

138
Q

What is hepatocellular adenoma?

What are the symptoms?

What are the lab findings?

What are the complications?

A

Highest female ratio very associated with oral contraceptives & anabolic steroids

Signs:
1) RUQ pain
2) Early satiety
3) N/V

Labs:
Enlarged hepatocytes with abnormal lobules

Complications: rupture & hemorrhage & risk of transformation into HCC

139
Q

Highest female ratio very associated with oral contraceptives & anabolic steroids

Signs:
1) RUQ pain
2) Early satiety
3) N/V

Labs:
Enlarged hepatocytes with abnormal lobules

Complications: rupture & hemorrhage & risk of transformation into HCC

A

Hepatocellular adenoma

140
Q

The most common BENIGN liver tumor influenced by estrogen levels (usually middle aged adults)

Signs:
1) RUQ pain
2) Early satiety
3) N/V

Labs:
The tumor is well demarcated, uniform & homogenous
Cavernous vascular spaces lined with flat endothelial cells

A

hepatic hemangioma

141
Q
A