Liver II continued Flashcards

1
Q

What are the three major intrahepatic biliary tract disease?

A
  • secondary biliary cirrhosis
  • primary biliary cirrhosis
  • primary sclerosing cholangitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is secondary biliary cirrhosis due to?

A

-uncorrected obstruction of the extrahepatic biliary tree

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How can you get obstruction of the extrahepatic biliary tree resulting in secondary biliary cirrhosis?

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the clinica features of secondary biliary cirrhosis?

A
Pruritis
Jaundice
Malaise
Dark urine
Light stools
Hepatosplenomegaly
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the Laboratory findings of secondary biliary cirrhosis?

A

Conjugated hyperbilirubinemia
Increased serum alkaline phosphatase
Increased bile acids
Increased cholesterol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

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)

A

cholestasis
periportal fibrosis
hepatic scarring and nodule formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

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

A

secondary biliary cirrhosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is this:
inflammatory autoimmune disease affecting the intrahepatic bile ducts

What is the primary feature?
What are the antibodies?

A

Primary Biliary cirrhosis

nonsuppurative inflammatory destruction of medium and small sized intrahepatic bile ducts

antimitochondrial antibodies (characteristic and essential for the diagnosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the clinical features of primary biliary cirrhosis?

A
  • Disease of middle aged women
  • Insidious onset
  • fatigue
  • abdominal discomfort
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

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

A

females

-peri-portal granulomatous inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Primary sclerosing cholangitis,is a chronic (blank) disorder characterized by (blank) inflammation, (blank) and strictures of the large (blank) ducts

A

cholestatic
non-specific
sclerosing fibrosis
large intra and extra hepatic bile ducts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Primary sclerosing cholangitis may result from (blank)

A

immunologically mediated injury

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Primary sclerosing cholangitis is associated with (blank).

What antibodies are associated with this? What factor is associated with it?

A

ulcerative colitis

anti-smooth muscle antibody
anti-nuclear antibody

Rheumatoid factor
Atypical P-ANCA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the clinical features of primary sclerosing cholangitis?

A
Fatigue 
Pruritis
Jaundice
Elevation of alkaline phosphatase
Predisposition to cholangiocarcinoma
Chronic pancreatitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

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

A

Primary sclerosing cholangitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is this:

Onion-skin sclerosing pattern surrounding large intrahepatic bile ducts

A

intrahepatic biliary tract disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What are anomalies of the biliary tree?

A
  1. Von Meyenberg complexes
  2. Polycystic liver disease
  3. Congenital hepatic fibrosis/Caroli disease
  4. Alagille syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is this:
small clusters of modesly dilated bile ducts embedded in a fibrous stroma

Is it common?
What is it associated with?

A

Von Meyenberg Complexes (Bile duct hamartomas)

common and clinically signif

associated with PCKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is this:
multiple diffuse cysts in the liver

What is it associated with?

A

Polycystic liver disease

PCKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Congenital hepatic fibrosis is associated with (blank) disease. What does it arise from?
Where will you see the fibrosis?

A

Carolis Disease
persistence of embryonic form of the biliary tree
Portal tracts show fibrosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

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

A

no
Cholangiocarcinoma
PCKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

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?

A

Caroli Disease

Congenital Hepatic fibrosis

Cholangiocarcinoma

PCKD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Congenital absence/dearth of bile ducts is a rare (blank) multiorgan disorder. What are the 5 major clinical features of this disease?

A

Autosomal dominant

  • Chronic cholestasis
  • Peripheral stenosis of the pulmonary artery
  • Butterfly-like vertebral arch defects
  • Eye defects
  • Peculiar hypertelic facies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What syndrome is this:

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

A

Alagille syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What is this:
Not a true neoplasm
Common in women
Due to embryologic hepatic defect

A

Focal nodular hyperplasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

In focal nodular hyperplasia you will see a (blank) lesion in an otherwise normal liver

A

pseudo-mass

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What scar will you see with focal nodular hyperplasia?

What does focal nodular hyperplasia result from?

A

central stellate scar from which fibrous septa radiate

-congenital vascular malformation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Is focal nodular hyperplasia associated with OCs?

A

no but adenomas are!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

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

A

thick

30
Q

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

Should you biopsy it?

A

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

31
Q

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

A

Hepatic adenoma

OCs or anabolic steroid use

32
Q

Subscapular adenomas may rupture causing severe (blank

A

intra-abdominal hemorrhage

33
Q

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

A

bile gets trapped and cant escape.

dense cords of benign hepatocytes

34
Q

What is this:

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

A

Hepatoblastoma

35
Q

What are the 2 types of hepatoblastoma?

A
  • Epithelial type that recapitulates the developing liver

- Mixed epithelial and mesenchymal types that shows areas of primitive mesenchyme

36
Q

What is hepatoblastoma associated with?

A

familial adenomatous polyposis syndrome of Beckwith Wiedmann syndrome

37
Q

What accounts for greater than 90% of primary liver cancers?

What are the etiologic factors?

A

hepatocellular carcinoma

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

38
Q

What are some risk factors for hepatocellular carcinoma?

What is it always associated with?

A
tyrosinemia
glycogen storage disease
hereditary hemochromatosis
non-alcoholic fatty liver disease
A1-antitrypsin deficiency

CIRRHOSIS!!

39
Q

What are the three types of hepatocellular carcinoma?

A

Uninodular
multinodular
Diffusely infiltrative

40
Q

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

A

vascular
intrahepatic metastases
portal vein/IVC

41
Q

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

A

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

42
Q

What is this:
malignancy of the biliary tree arising from bile ducts within and outside the liver

What are the risk factors for this?

A

Cholangiocarcinoma

Primary sclerosing cholangitis
Congenital fibropolycystic disease
HCV infection
Thorotrast

43
Q

Cholangiocarcinoma is classified as (blank) or (blank).

(blank) percent are perihilar
(blank) percent are distal
(blank) percent are intrahepatic

A

intrahepatic or extrahepatic
60
20-30
10

44
Q

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

A

Klatskin

45
Q

What is this:

pseudogland structures, desmoplasia

A

Cholangiocarcinoma

46
Q

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

A

Angiosarcoma

  • Yes, very malignant tumor with widespread metastasis
  • thorotrast, vinyl chloride, arsenic
47
Q

T or F

metastatic tumors are far more common than primary hepatic neoplasia

A

T

48
Q

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

A
  • colon (most common)
  • breast
  • lung (esp small cell carcinoma)
  • pancreas

multifocal

49
Q

What are the three major groupings for circulatory disorders?

A
  • impaired blood into the liver
  • impaired blood flow through the liver
  • hepatic venous outflow obstruction
50
Q

What will hepatic artery compromise cause?

What is it due to?

A
  • impaired blood inflow

- Embolism, neoplasia, polyarteritis nodosa, or sepsis

51
Q

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

A

Liver infarct

no, due to dual blood supply

52
Q

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?

A
  • 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

53
Q

What are causes of impaired blood low through the liver?

A
Cirrhosis
Sickle cell disease
DIC
Metastatic tumor
Eclampsia
Right and left sided heart failure
Peliosis hepatis
54
Q

What does Budd-Chiari do to the liver?

What are the clinical manifestations of Budd chiari syndrome?

A

hepatic venous outflow obstruction

  • Thombosis of 2 or more major hepatic veins
  • liver enlargement, pain, ascites
55
Q

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

A
Hypercoagulable state 
Polycythemia vera
Factor V Leiden mutation
Contraceptive use
Pregnancy
56
Q

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

A

Pre-eclampsia
Eclampsia
HELLP syndrome

57
Q

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

A

Wide range- hepatic dysfunction, hepatic failure, coma, death

  • mitochondrial dysfunction
  • termination of pregnancy
58
Q

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

Is it benign?

A

Intrahepatic cholestasis of pregnancy

benign

59
Q

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

A

95%

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

60
Q

What is cholecystitis? Is it acute or chronic?

A

Inflammation of the gallbladder

Acute, chronic or acute superimposed on chronic

61
Q

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

A

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

62
Q

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

A

obscure

63
Q

What are the clinical features of acute cholecystitis?

A
RUQ or epigastric pain
Fever
Anorexia
Tachycardia
Nausea 
Vomiting
64
Q

What are the clinical features of chronic cholecystitis?

A

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

65
Q

What are the complications of cholecystitis?

A
  • 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
66
Q

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?

A

Biliary atresia

Progressive inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts

67
Q

What are the 2 forms of biliary atresia?

A

Fetal: aberrant intrauterine development of the extrahepatic biliary tree

Perinatal: normally formed biliary tree is destroyed following birth

68
Q

Infants with biliary atresia present with persistant (blank)

A

neonatal cholestasis

69
Q

What is the most common type of biliary atresia?

A

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

70
Q

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

A

Choledochal cyst

Jaundice or biliary colic

71
Q

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

A

stone formation
stenosis and stricture
pancreatitis and obstructive biliary complications