Liver Pathology II Flashcards
Review the slide on bilirubin metabolism
What causes hyperbilirubinemia (2 classes)?
Either excess production of bilirubin or reduced clearance of it
(see below)
Hereditary hyperbilirubinemias can be grouped into unconjugated and conjugated hyperbilirubinemia
Which types of bilirubinemia occur at each step of bilirubin metabolism below?
Gilbert syndrome: unconj bili; decreased UDP-GT conjugation + impaired uptake
Crigler – Naijar type 1: deficient UDP-GT; increased unconj bili
Rx: plasmapheresis and phototherapy (increased water solubility + polarity >> more excretion
Type 2 not as bad, responds to phenobarbital >> increased liver enzyme synthesis
Dubin-Johnson: conj. Bili; defective excretion; dark/black liver
Rotor syndrome: basically milder Dubin Johnson, sans dark liver
**all autosomal recessive**
___ results from the UDP glucoronyl transferase not yet working properly >> transient + unconjugated hyperbilirubinemia
Physiologic jaundice of the newborn (i.e. physiologic neonatal jaundice) results from the UDP glucoronyl transferase not yet working properly >> transient + unconjugated hyperbilirubinemia
Occurs w/in 1st 24 hours of life, resolves w/in 2 weeks
Rx: phototherapy >> makes bilirubin water soluble
Conjugated hyperbilirubinemia can result from biliary tract disease or bilirubin excretion issues. Which 2 disorders result from biliary tract disease?
Primary Biliary cholangitis
Primary Sclerosing cholangitis
**both autoimmune?**
___ is autoimmune destruction of small + medium sized intrahepatic bile ducts and is characterized by **elevated AMA**, alk phos, and GGT
Primary biliary cholengitis is autoimmune destruction of small + medium sized intrahepatic bile ducts and is characterized by **elevated AMA**, alk phos, and GGT
Classically affects middle-aged women
+ve AMA, ass’d w/ other autoimmune diseases
Rx: ursodiol
__ is ass’d w/ elevated anti-mitochondrial antibody and only affects intrahepatic bile ducts
Primary biliary cholengitis
A pt comes in on suspicion of biliary tract disease. She has scleral icterus and has been excreting tan stool. She also has a family history of autoimmune thryroid disease, and elevated bilirubin, Alk Phos and cholesterol levels. You perfom a biopsy and discover a florid duct lesion.
Which disorder is on your differential?
What stage is this disease in?
If the same pt has total loss of bile ducts + fibrosis, what stage is she in?
A pt comes in on suspicion of biliary tract disease. She has scleral icterus and has been excreting tan stool. She also has a family history of autoimmune thryroid disease, and elevated bilirubin, Alk Phos and cholesterol levels. You perfom a biopsy and discover a florid duct lesion.
On differential: primary biliary cholangitis
Early stage: florid duct lesion; Late stage: loss of bile ducts, cirrhosis, ductular rxn
What are the pathologies below?
Primary biliary cholengitis:
left: florid duct lesion - inflammation targeted at the bile duct + portal tract, + granuloma
right: late state PBC >> disappearing bile duct
While PBC affects the intrahepatic bile ducts, ___ obliterates both intra and extrahepatic bile ducts
While PBC affects the intrahepatic bile ducts, primary sclerosing cholangitis obliterates both intra and extrahepatic bile ducts
____ is characterized by “onion skin” bile duct fibrosis (alternating strictures + dilation w/ beading of intra + extrahepatic bile ducts on ERCP, MRCP
Primary sclerosing cholangitis is characterized by “onion skin” bile duct fibrosis (alternating strictures + dilation w/ beading of intra + extrahepatic bile ducts on ERCP, MRCP
**90% of pts also have ulcerative colitis; less inflammation, no granulomas; increased risk of cholangiocarcinoma**
A pt w/ ulcerative cholitis, elevated alk phos and biliubin, scleral icterus + tan stools is evaluated. You notice the pathology below on histological analysis.
What is the diagnosis?
Primary sclerosing cholangitis
A patient underwent liver biopsy and her pathology report mentions the presence of a florid duct lesion. Which of the following is associated with her diagnosis?
A.Presence of anti-mitochondrial antibodies
B.Presence of ANCA
C.History of ulcerative colitis
D.History of liver fluke infestation
A. PBC = ama. Know this like your life depends on it because it *almost* does
Fill in the chart below
see table below
Proliferating bile ductules are ass’d w/ what biliary tract disease?
Proliferating bile ductules are ass’d w/ : bile duct obstruction
**anything from stones to tumors can cause obstruction. increased risk of ascending cholangitis. can lead to fibrosis >> cirrhosis**
In drug induced liver injury, the toxic intermediate ___ causes damage to hepatocytes, mainly those in Zone ___
In drug induced liver injury, the toxic intermediate NAPQI causes damage to hepatocytes, mainly those in Zone 3
***The CYP450 system is near the central vein, in Zone 3. so if you have acetaminophen overdose, the hepatocytes in Zone 3 will be damaged the most***
What is the pathology below?
Acetaminophen induced liver injury
**central vein necrosis**
What is the pathology below?
Central vein necrosis from drug induced liver injury
What is the pathology below caused by?
Drug induced liver injury >> central vein necrosis
___ is hepatic vein thrombosis resutling from obstruction of 2+ hepatic veins, and results in hepatomegaly, ascites, varices, abdominal pain.
Budd Chiari syndrome is hepatic vein thrombosis resutling from obstruction of 2+ hepatic veins, and results in hepatomegaly, ascites, varices, abdominal pain
**no jugular venous distention, ass’d w/ hypercoagulable states, might cause nutmeg liver**
What is the pathology below?
Budd Chiari syndrome
Right sided heart failure can lead to __ of the liver
Early on, this is characterized by congestion of ___, later followed by cirrhosis (aka ___)
Right sided heart failure can lead to passive congestion of the liver
Early on, this is characterized by congestion of centrilobular sinusoids later followed by cirrhosis (aka cardiac sclerosis)
**Cardiac sclerosis: liver cirrhosis resulting from heart failure-induced liver congestion
Characteristic: nutmeg liver**
(review slide below)
What is the pathology below?
Passive liver congesion >> nutmeg liver
**Zone 3 necrosis**
Fill in the chart below
Focal nodular hyperplasia
Carvenous hemangioma
Hepatic adenoma
Bile duct hamartoma
