Liver II continued Flashcards by Danielle Hayes

What are the three major intrahepatic biliary tract disease?

secondary biliary cirrhosis
primary biliary cirrhosis
primary sclerosing cholangitis

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What is secondary biliary cirrhosis due to?

-uncorrected obstruction of the extrahepatic biliary tree

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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

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What are the clinica features of secondary biliary cirrhosis?

Pruritis
Jaundice
Malaise
Dark urine
Light stools
Hepatosplenomegaly

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What are the Laboratory findings of secondary biliary cirrhosis?

Conjugated hyperbilirubinemia
Increased serum alkaline phosphatase
Increased bile acids
Increased cholesterol

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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

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If you have poorly formed proliferating bile ducts, what is the disease?

secondary biliary cirrhosis

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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)

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What are the clinical features of primary biliary cirrhosis?

Disease of middle aged women
Insidious onset
fatigue
abdominal discomfort

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What gender is primary biliary cirrhosis common in? what is it characterized by?

females

-peri-portal granulomatous inflammation

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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

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Primary sclerosing cholangitis may result from (blank)

immunologically mediated injury

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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

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What are the clinical features of primary sclerosing cholangitis?

Fatigue 
Pruritis
Jaundice
Elevation of alkaline phosphatase
Predisposition to cholangiocarcinoma
Chronic pancreatitis

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If you see beading of contrast media in radiographs of the intra and extrahepatic biliary tree, what is the disorder?

Primary sclerosing cholangitis

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What is this:

Onion-skin sclerosing pattern surrounding large intrahepatic bile ducts

intrahepatic biliary tract disease

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What are anomalies of the biliary tree?

Von Meyenberg complexes
Polycystic liver disease
Congenital hepatic fibrosis/Caroli disease
Alagille syndrome

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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

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What is this:
multiple diffuse cysts in the liver

What is it associated with?

Polycystic liver disease

PCKD

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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

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In congenital hepatic fibrosis, will you see cirrhosis?
What is there an increased risk of?
What is it associated with?

no
Cholangiocarcinoma
PCKD

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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

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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

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What syndrome is this:

a patient presents with pointy chin, down sloping eyes, low ears, small mouth

Alagille syndrome

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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!

focal nodular hyerplasia will have a (blank)-walled artery at the edge of the stellate scar

thick

What is the most common benign liver tumor? What is it a tumor of? Is it metastatic? Should you biopsy it?

Cavernous hemangioma Tumor of vascular channels in a bed of fibrous CT No NO!

What is this: benign tumor arising from hepatocytes What is it strongly associated with?

Hepatic adenoma OCs or anabolic steroid use

Subscapular adenomas may rupture causing severe (blank

intra-abdominal hemorrhage

Why do you get green adenomas in subscapular adenomas? What else will you see?

bile gets trapped and cant escape. | dense cords of benign hepatocytes

What is this: | What is hepatic neoplasm that is a malignant tumor of young children, usually fatal within a few years

Hepatoblastoma

What are the 2 types of hepatoblastoma?

- Epithelial type that recapitulates the developing liver | - Mixed epithelial and mesenchymal types that shows areas of primitive mesenchyme

What is hepatoblastoma associated with?

familial adenomatous polyposis syndrome of Beckwith Wiedmann syndrome

What accounts for greater than 90% of primary liver cancers? | What are the etiologic factors?

hepatocellular carcinoma Chronic viral infection Chronic alcoholism Non-alcoholic steatohepatitis Food contaminants (aflatoxins)

What are some risk factors for hepatocellular carcinoma? | What is it always associated with?

``` tyrosinemia glycogen storage disease hereditary hemochromatosis non-alcoholic fatty liver disease A1-antitrypsin deficiency ``` CIRRHOSIS!!

What are the three types of hepatocellular carcinoma?

Uninodular multinodular Diffusely infiltrative

Hepatocellular carcinoma has a strong propensity for invasion of (blank) structures. So you have (blank) metastases (satellite nodules) an invasion of the (Blank)

vascular intrahepatic metastases portal vein/IVC

5% of hepatocellular carcinomas are a (Blank) variant. Who does this occur in? Serum will show elevated (blank) levels. How does it grow?

fibrolamellar variant young males and female w/ no underlying chronic liver disease or cirrhosis AFP -well circumscribed (described as hepatoid)

What is this: malignancy of the biliary tree arising from bile ducts within and outside the liver What are the risk factors for this?

Cholangiocarcinoma Primary sclerosing cholangitis Congenital fibropolycystic disease HCV infection Thorotrast

Cholangiocarcinoma is classified as (blank) or (blank). (blank) percent are perihilar (blank) percent are distal (blank) percent are intrahepatic

intrahepatic or extrahepatic 60 20-30 10

Extrahepatic perihilar tumors (near the formation of the common hepatic duct) are know as (blank) tumors.

Klatskin

What is this: | pseudogland structures, desmoplasia

Cholangiocarcinoma

What is the most common sarcoma arising in the liver? Is this malignant? What have been implicated as causative?

Angiosarcoma - Yes, very malignant tumor with widespread metastasis - thorotrast, vinyl chloride, arsenic

T or F | metastatic tumors are far more common than primary hepatic neoplasia

What are the most common primary sites for metastasis to the liver? What is a big hint that you have a metastasis?

- colon (most common) - breast - lung (esp small cell carcinoma) - pancreas multifocal

What are the three major groupings for circulatory disorders?

- impaired blood into the liver - impaired blood flow through the liver - hepatic venous outflow obstruction

What will hepatic artery compromise cause? | What is it due to?

- impaired blood inflow | - Embolism, neoplasia, polyarteritis nodosa, or sepsis

Hepatic artery compromise may result in (Blank). Is this common?

Liver infarct | no, due to dual blood supply

What will portal vein obstruction and thrombosis cause? What are the symptoms associated with this? What is extrahepatic obstruction due to? What is intrahepatic obstruction due to?

- Impaired blood flow - abominal pain, portal HTN, ascites Subclinical occlusion from neonatal umbilical sepsis or umbilical vein catherization Intrabdominal sepsis Inherited or acquired hypercoagulable disorders Trauma Pancreatitis or pancreatic cancer Invasion by HCC Cirrhosis -acute thrombosis

What are causes of impaired blood low through the liver?

``` Cirrhosis Sickle cell disease DIC Metastatic tumor Eclampsia Right and left sided heart failure Peliosis hepatis ```

What does Budd-Chiari do to the liver? | What are the clinical manifestations of Budd chiari syndrome?

hepatic venous outflow obstruction - Thombosis of 2 or more major hepatic veins - liver enlargement, pain, ascites

3/4 of patients have predisposing factors for hepatic venous outflow obstruction, what are they? (blank x 5)

``` Hypercoagulable state Polycythemia vera Factor V Leiden mutation Contraceptive use Pregnancy ```

What are three syndromes that occur in pregnancy that can cause hepatic disease?

Pre-eclampsia Eclampsia HELLP syndrome

What are the symptoms of acute fatty liver of pregnancy? What is the cause? How do you treat?

Wide range- hepatic dysfunction, hepatic failure, coma, death - mitochondrial dysfunction - termination of pregnancy

What is this: Altered hormonal state of pregnancy with biliary defects in secretion creates cholestasis Is it benign?

Intrahepatic cholestasis of pregnancy benign

Cholesthiasis is responsible for greater than (blank) % of biliary tract diseases. What are the 2 types?

95% Choelsterol stones composed of cholesterol Pigment stones composed of bilirubin calcium salts

What is cholecystitis? Is it acute or chronic?

Inflammation of the gallbladder Acute, chronic or acute superimposed on chronic

Acute cholecystitis can have 2 forms, what are they and what are each due to?

Calculous: due to chemical irritation and inflammation from obstruction of the neck or cystic duct Acalculous: due to ischemia

Chronic cholecystitis has a (Blank) evolution of the disease but can have both calcuous and acalculous forms as well.

obscure

What are the clinical features of acute cholecystitis?

``` RUQ or epigastric pain Fever Anorexia Tachycardia Nausea Vomiting ```

What are the clinical features of chronic cholecystitis?

Recurrent attacks of steady or colicky epigastric or RUQ pain Nausea Vomiting Intolerance for fatty foods

What are the complications of cholecystitis?

- Bacterial superinfection and cholangitis or sepsis - Gallbladder perforation and local abscess formation - Gallbladder rupture with diffuse peritonitis - Biliary enteric fistula - Porcelain gallbladder - Aggravation of preexisting medical illness

What is this: Complete or partial obstruction of the lumen of the extrahepatic biliary tree within the first 3 months of life What is it characterized by?

Biliary atresia Progressive inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts

What are the 2 forms of biliary atresia?

Fetal: aberrant intrauterine development of the extrahepatic biliary tree Perinatal: normally formed biliary tree is destroyed following birth

Infants with biliary atresia present with persistant (blank)

neonatal cholestasis

What is the most common type of biliary atresia?

Type 4 (72%)> Type 3 (19%)> Type 2 (6%)> Type 1 (3%)

What is this: congenital dilations of the common bile duct How do the patients present?

Choledochal cyst Jaundice or biliary colic

Patients with choledochal cyst are predisposed to (blank) formation, (blank) and stricture, (blank) and (blank) complications

stone formation stenosis and stricture pancreatitis and obstructive biliary complications