Liver II continued Flashcards
What are the three major intrahepatic biliary tract disease?
- secondary biliary cirrhosis
- primary biliary cirrhosis
- primary sclerosing cholangitis
What is secondary biliary cirrhosis due to?
-uncorrected obstruction of the extrahepatic biliary tree
How can you get obstruction of the extrahepatic biliary tree resulting in secondary biliary cirrhosis?
Extrahepatic cholelithiasis
Malignancy of the biliary tree or head the pancreas
Strictures from previous surgical procedures
Biliary atresia
Cystic fibrosis
Choledochal cysts
Paucity of bile duct syndromes
What are the clinica features of secondary biliary cirrhosis?
Pruritis Jaundice Malaise Dark urine Light stools Hepatosplenomegaly
What are the Laboratory findings of secondary biliary cirrhosis?
Conjugated hyperbilirubinemia
Increased serum alkaline phosphatase
Increased bile acids
Increased cholesterol
In secondary biliary cirrhosis, there is initially (blank) which is reversible with the correction of the obstruction.
Secondary inflammation initiates (blank) that leads to (blank and blank)
cholestasis
periportal fibrosis
hepatic scarring and nodule formation
If you have poorly formed proliferating bile ducts, what is the disease?
secondary biliary cirrhosis
What is this:
inflammatory autoimmune disease affecting the intrahepatic bile ducts
What is the primary feature?
What are the antibodies?
Primary Biliary cirrhosis
nonsuppurative inflammatory destruction of medium and small sized intrahepatic bile ducts
antimitochondrial antibodies (characteristic and essential for the diagnosis)
What are the clinical features of primary biliary cirrhosis?
- Disease of middle aged women
- Insidious onset
- fatigue
- abdominal discomfort
What gender is primary biliary cirrhosis common in? what is it characterized by?
females
-peri-portal granulomatous inflammation
Primary sclerosing cholangitis,is a chronic (blank) disorder characterized by (blank) inflammation, (blank) and strictures of the large (blank) ducts
cholestatic
non-specific
sclerosing fibrosis
large intra and extra hepatic bile ducts
Primary sclerosing cholangitis may result from (blank)
immunologically mediated injury
Primary sclerosing cholangitis is associated with (blank).
What antibodies are associated with this? What factor is associated with it?
ulcerative colitis
anti-smooth muscle antibody
anti-nuclear antibody
Rheumatoid factor
Atypical P-ANCA
What are the clinical features of primary sclerosing cholangitis?
Fatigue Pruritis Jaundice Elevation of alkaline phosphatase Predisposition to cholangiocarcinoma Chronic pancreatitis
If you see beading of contrast media in radiographs of the intra and extrahepatic biliary tree, what is the disorder?
Primary sclerosing cholangitis
What is this:
Onion-skin sclerosing pattern surrounding large intrahepatic bile ducts
intrahepatic biliary tract disease
What are anomalies of the biliary tree?
- Von Meyenberg complexes
- Polycystic liver disease
- Congenital hepatic fibrosis/Caroli disease
- Alagille syndrome
What is this:
small clusters of modesly dilated bile ducts embedded in a fibrous stroma
Is it common?
What is it associated with?
Von Meyenberg Complexes (Bile duct hamartomas)
common and clinically signif
associated with PCKD
What is this:
multiple diffuse cysts in the liver
What is it associated with?
Polycystic liver disease
PCKD
Congenital hepatic fibrosis is associated with (blank) disease. What does it arise from?
Where will you see the fibrosis?
Carolis Disease
persistence of embryonic form of the biliary tree
Portal tracts show fibrosis
In congenital hepatic fibrosis, will you see cirrhosis?
What is there an increased risk of?
What is it associated with?
no
Cholangiocarcinoma
PCKD
What is this:
larger ducts of the intrahepatic biliary tree are segmentally dilated
What is it associated with?
What will you be at increasd risk of?
What is it associated with?
Caroli Disease
Congenital Hepatic fibrosis
Cholangiocarcinoma
PCKD
Congenital absence/dearth of bile ducts is a rare (blank) multiorgan disorder. What are the 5 major clinical features of this disease?
Autosomal dominant
- Chronic cholestasis
- Peripheral stenosis of the pulmonary artery
- Butterfly-like vertebral arch defects
- Eye defects
- Peculiar hypertelic facies
What syndrome is this:
a patient presents with pointy chin, down sloping eyes, low ears, small mouth
Alagille syndrome
What is this:
Not a true neoplasm
Common in women
Due to embryologic hepatic defect
Focal nodular hyperplasia
In focal nodular hyperplasia you will see a (blank) lesion in an otherwise normal liver
pseudo-mass
What scar will you see with focal nodular hyperplasia?
What does focal nodular hyperplasia result from?
central stellate scar from which fibrous septa radiate
-congenital vascular malformation
Is focal nodular hyperplasia associated with OCs?
no but adenomas are!