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GI II - EXAM 2 > Liver Path 5 - SRS > Flashcards

Liver Path 5 - SRS Flashcards

1
Q

Hepatocellular disease primarily attacks/destroys hepatocytes. What lab values would be increased by this?

A

AST/ALT

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2
Q

What happens to bile production and flow in cholestatic diseases?

What is elevated in the lab findings?

A

Alk phosphatase

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3
Q

By what mechanism do sepsis and estrogens lead to intrahepatic cholestasis, decreased bile formation or obstruction?

A

Decreased bile formation

His slides say this, but pathoma and the literature disagree, also he contradicts this somewhat later in the presentation when discussing sepsis derived cholestasis in more detail.

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4
Q

By what mechanism do primary biliary cirrhosis and infiltration of the liver with tumor/granulomas lead to intrahepatic cholestasis, decreased bile formation or obstruction?

A

Obstruction

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5
Q

What enzyme is deficient in the Crigler-Najjar?

A

UDPGT (absent in Type I, reduced in type II)

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6
Q

What causes Gilbert’s syndrome, and what accumulates?

A

See unconjugated hyperbilirubinemia is caused by the reduced activity of the enzyme glucuronyl-transferase which conjugates bilirubin.

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7
Q

When does jaundice of the newborn typically appear/resolve?

Conjugated or unconjugated?

A

Unconjugated

2-5 days

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8
Q

What type of hyperbilirubinemia would you see in Dubin-Johnson Syndrome?

Rotor syndrome?

A

Conjugated hyperbilirubinemia for both

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9
Q

What is mutated in Dubin-Johnson syndrome?

A

ATP dependent canalicular organic anion transporter

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10
Q

Sepsis associated cholestasis is linked to infections with what types of bacteria?

A

Gram neg

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11
Q

In sepsis associated cholestasis, what are the lab values like for serum bilirubin and alkaline phosphatase?

A

Serum bilirubin is elevated out of proportion to elevation of alkaline phosphatase

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12
Q

The outcome of sepsis-associated jaundice is linked to effective treatment of the sepsis. What is the prognosis for this scenario?

A

Poor, 60-90% mortality

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13
Q

What is this?

What is the most common cause?

A

Ductular Cholestasis

Sepsis

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14
Q

What are the causes of large duct obstruction in adults?

A

–Obstruction by gallstones (Most Common)

–Malignant neoplasms of biliary tree/head of pancreas

–Primary sclerosing cholangitis

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15
Q

What are the causes of large duct obstruction in kids?

A

–Biliary atresia

–Choledochal cysts

–Cystic fibrosis

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16
Q

Initial morphologic features of cholestasis d/t large bile duct obstruction are entirely reversible with correction of the obstruction.

What can prolonged obstruction lead to?

How about intermittent obstruction?

A
  • Prolonged obstruction can lead to secondary biliary cirrhosis
  • Intermittent obstruction may promote ascending cholangitis
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17
Q

What is the most severe form of ascending cholangitis?

A

Suppurative cholangitis - purulent bile fills and distends bile ducts

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18
Q

Hepatolithiasis, is a Disorder of intrahepatic gallstone formation with high prevalence in east asia and rare elsewhere. What is this associated with?

What does this cause and have a consequent risk of developing?

A
  • recurrent ascending cholangitis
  • Progressive inflammatory destruction of parenchyma –>Risk of cholangiocarcinoma
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19
Q

What is the combination of Fever, jaundice and RUQ pain?

A

Charcot’s Triad - Indicative of Ascending cholangitis

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20
Q

If your patient with Charcot’s triad develops two additional things they have Reynold’s pentad. What are the two additional things?

A

Altered sensorium + Hypotension

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21
Q

Identify the circled structures.

What is the pathology at work?

A

Green and orange - Bile ducts

Blue - Arteries

Orange Bile duct is inflammed and has neutrophilic infiltration of the lumen and epithelium - Indicates Acute Ascending Cholangitis

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22
Q

This patient has suppurative cholangitis. Based on what you see in picture, why is treatment of this patient more difficult?

A

Star indicates abcess formation - this makes it difficult for antibiotic therapy to reach the organisms.

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23
Q

Although there are multiple causes of neonatal cholestasis, biliary atresia must be differentiated from other causes of cholestasis promptly because early surgical intervention (ie, before 2 months of age) results in a better patient outcome.

What does the evidence point to for the etiology of biliary atresia? 3

A

Viruses

Toxins

Gene sequence variations

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24
Q

What are the major causes of neonatal cholestasis?

4

A
  • Extra-hepatic biliary atresia
  • Infectious hepatitis
  • Alpha-1 antitrypsin deficiency
  • Idiopathic neonatal hepatitis

–Diagnosis of exclusion

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25
Q

Hereditiary Fibropolycystic Liver Disease, describes a heterogeneous group of genetic disorders with?

A

segmental dilatations of the intrahepatic bile ducts and associated fibrosis.

[Cysts and/or fibrosis]

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26
Q

What mutations cause fibropolycystic liver disease?

A

Primary cilia protein gene mutations

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27
Q

What does the most common form of polycystic liver disease coexist with?

A

renal cystic disease (ADPKD) - linked to the mutation in polycystic kidney disease gene PKD.

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28
Q

What liver dysfunctions do you typically see with polycystic liver?

A

Typically none. May have liver tenderness/pain though

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29
Q

What is shown in each othese images?

What does the Image on the right have a high likliehood of developing into?

A

A: Epithelial variation within cysts ranges from flattened cells (left vertical surface) to cuboidal (top horizontal surface) to columnar (lower right side).

B: Biliary microhamartomas (von Meyenburg complexes) are often seen in association with ADPLD. High likeliehood of turning into a cyst.

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30
Q

What are von meyenberg complexes?

A

Peripheral bile duct malformations (Bile duct hamartoma)

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31
Q

What would multiple instances of this be called?

A

•Multiple von Meyenburg complexes are called polycystic liver disease

32
Q

What disease is congenital hepatic fibrosis associated with?

A

Autosomal recessive polycystic kidney disease

33
Q

What complications are associated with congenital hepatic fibrosis?

A

Complications of portal hypertension including splenomegal and esophageal varices.

34
Q

You have a 2 year old patient who presents with portal HTN like complications. Liver biopsy reveals this. What is going on?

Also, what stain was used here?

A

Congenital hepatic Fibrosis

Masson Trichrome

35
Q

Caroli disease is defined by what?

A

Presence of congenitally dilated intrahepatic bile ducts, often involving the entire liver.

36
Q

If you have Caroli disease plus what = caroli syndrome?

A

Congenital hepatic fibrosis

37
Q

What are two things a patient with Caroli disease is at increased risk for?

A
  • Recurrent bacterial cholangitis
  • Risk of cholangiocarcinoma
38
Q

This Section of liver shows cystically dilated bile ducts. The cystic cavities are traversed by fibrous cords, known to contain the portal vessels.

Histological evaluation reveals Dilated bile ducts have thickened walls due to marked chronic inflammation.

What condition does this person have?

A

Caroli Disease

39
Q

Describe what Choledochal cysts are.

A

Congenital cystic dilatation of the extrahepatic and intrahepatic bile ducts

40
Q

Complete inflammatory obstruction of the terminal portion of the bile duct is common in infants with choledochal cyst. What does this condition predispose patients to?

A

Stone formation

stenosis

pancreatitis

Cholangiocarcinoma

41
Q

What is the most common presentation of choledochal cysts in both kids and adults?

A

Kids: Jaundice

Adults: ascending cholangitis

42
Q

What is shown here?

A

Choledochal cyst

43
Q

What are 4 liver diseases associated with pregnancy?

A
  1. Intrahepatic cholestasis of pregnancy
  2. Acute fatty liver of pregnancy
  3. Preeclampsia and liver disease
  4. Hepatitis E virus
44
Q

What happens to 20% of pregnant women who get Hep E?

A

Death

45
Q

Intrahepatic Cholestasis of Pregnancy is a Mild cholestatic disease that occurs in <2% of pregnancies.

What else can it occur with?

What can it cause?

Is there a genetic component?

A
  • Also occurs with oral contraceptives
  • May cause intrauterine fetal death
  • Strong genetic component, 10-15% of first degree female relatives affected
46
Q

What is the most common cause of jaundice in pregnancy?

A

Acute viral hepatitis

47
Q

How does estrogen affect bile formation?

A

By affecting transporter proteins

48
Q

Intrahepatic cholestasis of pregnancy usually appears in the third trimester. What 2 symptoms occur in these patients?

Which occurs virtually always?

A
  • Pruritus occurs in virtually all patients
  • Jaundice occurs in ~25%
49
Q

Acute fatty liver of pregnancy usually appears in the third trimester before delivery (~1 in 13,000 pregnancies), and may occur in subsequent pregnancies. How do severe cases present?

What are the mortality rates for the mother and fetus?

A

Severe cases present as Acute/fulminant failure with modest increase in AST/ALT

  • Maternal mortality is 5-26%
  • Intrauterine fetal death rate is 9-32%
50
Q

What are the symptoms seen in acute fatty liver of pregnancy?

A
  • Nausea or vomiting (75%)
  • Epigastric abdominal pain (50%)
  • Signs of preeclampsia (50%)
  • Jaundice (90%)
  • Anorexia
51
Q

Acute Fatty Liver of Pregnancy Laboratory Tests will reveal elevated aminotransferases (under 500 u/L) and increased serum bilirubin.

Evidence of hepatic insufficiency may be present. What are three ways this may manifest?

A

–Hypoglycemia

–Encephalopathy

–Abnormalities in coagulation studies

52
Q

Acute fatty liver of pregnancy must be differentiated from HELLP syndrome, which is characterised by what? 3

A
  1. Hemolysis
  2. Elevated liver enzymes
  3. low platelet count
53
Q

The histological examination of the fatty liver of pregnancy is similar to what is seen in Reye’s syndrome. In what way?

A

Microsteatosis

54
Q

What are the four symptoms that characterize preeclampsia?

A
  1. •Hypertension
  2. •Proteinuria
  3. •Peripheral edema
  4. •Coagulation abnormalities
55
Q

Preeclampsia affects 5-10% of pregnancies, and the liver is affected in 10-20% of women with preeclampsia. What is the most common form of preeclampsia related liver disease?

A

HELLP syndrome

56
Q

What are the components of HELLP?

A

–Hemolysis

–Elevated liver tests (AST/ALT)

–Low Platelets

57
Q

HELLP syndrome typically manifests in the third trimester, but may start immediately postpartum. What is injured and what results from this injury histologically?

A

Systemic endothelial cell injury with fibrin deposition and ischemic infarcts.

58
Q

Preeclampsia leads to hemorrhage into the space of Disse, whith leads to what?

A

Periportal hepatocellular coagulative necrosis

59
Q

This pregnant patient presented with AST/ALT in the 100-300 U/L range but has a chance at elevation over 1000 U/L. Based on the lab values and the attached findings, what does this patient have?

What do each of the arrows indicate?

Is this life threatening?

A

Preeclampsia related liver injury

Top left arrow = viable hepatocytes

Bottom right arrow = periportal necrosis

Not lifethreatening (Unless HELPP syndrome arises)

60
Q

What are the three broad categories of hepatic circulatory disorders?

A
  1. Hepatic vein outflow obstruction
  2. Impaired intrahepatic blood f low (cirrhosis, sinusoid occlusion)him
  3. Impaired blood inflow
61
Q

What would we call hepatic vein thrombosis in 2 or more veins?

A

Budd Chiari

62
Q

What are three specific scenarios that predispose a patient to Budd Chiari syndrome?

A

–Polycythemia vera

–Pregnancy

–Postpartum state

63
Q

What are two causes of hepatic venous outflow obstruction?

A
  • Hepatic vein thrombosis
  • Sinusoidal Obstruction Syndrome
64
Q

What happens in sinusoidal obstruction syndrome?

A

Obliteration of terminal hepatic venules

65
Q

What are two things that cause obliteration of terminal hepatic venules and sinusoidal obstruction syndrome?

A

Chemotherapeutic agents

Immunosuppressive agents

66
Q

What does the liver look like in Budd-Chiari syndrome?

A

•swollen and red-purple and has a tense capsule.

67
Q

What did this patient have?

A

Budd-Chiari Syndrome

68
Q

What is shown here?

A

Massive centrilobular and mid-lobule congestion with obliteration of a terminal hepatic vein (indicated by the arrow).

Indicative of acute sinusoidal obstruction syndrome.

69
Q

What are three causes of impaired intrahepatic flow?

A
  1. Cirrhosis
  2. sinusoid occlusion (not Sinusoidal obstruction syndrome though, that’s a vaso-occlusive disease)
  3. Systemic circulatory disease
70
Q

What are three causes of impaired blood inflow to the liver?

A

Hepatic artery compromise

portal vein obstruction

Intra- or extra- hepatic thrombosis

71
Q

What is the most common cause of impaired blood flow through the liver?

A

Cirrhosis

72
Q

What are three things that cause sinusoid occlusion?

A

–Sickle cell disease

–Right sided heart failure

–Eclampsia

73
Q

Right-sided heart failure results in transmission of increased central venous pressure from the heart directly to the?

A

Hepatic sinusoids

74
Q

What is shown here?

A

Sickle cell disease causing plugged sinusoids and intrahepatic blood flow disruption.

75
Q

What are two causes of hepatic artery compromise?

A

–Arteriosclerosis

–Thrombosis or emboli

76
Q

What are some causes of Portal vein obstruction and thrombosis?

A

–Intra-abdominal sepsis

–Hypercoagulable disorders with thrombosis

–Cirrhosis (5% to 25% have portal vein thrombosis)

–Pancreatitis

77
Q
A

GI II - EXAM 2 (11 decks)

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