Liver, Gallbladder, Biliary Tract, Pancreas Flashcards Preview

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Flashcards in Liver, Gallbladder, Biliary Tract, Pancreas Deck (104):
1

Secondary biliary cirrhosis

Condition caused by obstruction of the large bile ducts outside of the liver
Most common cause is extrahepatic cholelithiasis (gallstones)
Also caused by biliary atresia, or tumors of the biliary tree or head of the pancreas

2

Sx of secondary biliary cirrhosis

Cholestasis
Bile duct proliferation with surrounding neutrophils
Periportal inflammation with progressive fibrosis

3

Primary biliary cirrhosis

Immune mediated nonsuppurative destruction of small and medium-sized intrahepatic bile ducts
Median age 50 yrs
90% in females
Insidious, presenting as pruritis, later jaundice
Elevated alkaline phosphatase levels

4

What is primary biliary cirrhosis associated with?

Sjogren syndrome
Scleroderma
Thyroid disease

5

Serology of primary biliary cirrhosis

Antimitochrondrial antibodies
Antinuclear antinuclear antibodies
Anitneutrophil cytoplasmic antibodies

6

Primary sclerosing cholangitis

Immune mediated fibrosis and destruction of extrahepatic and large intrahepatic bile ducts
Median age 30 yrs
70% males
Progressive fatigue, pruritis, jaundice

7

What is primary sclerosing cholangitis associated with?

Chronic ulcerative colitis
Pancreatitis

8

Serology of primary sclerosing cholangitis

Antimitochondrial antibodies
Antinuclear antibodies
Antineutrophil cytoplasmic antibodies

9

Hepatic vein thrombosis (Budd-Chiari syndrome)

Caused by thrombosis of two or more major hepatic veins
Hepatomegaly, weight gain, ascites, abd pain

10

Sinusoidal obstruction syndrome

Occurs primarily in the first 20-30 days after a bone marrow transplant
Believed to be caused by toxic endothelial injury secondary to chemo and radiation therapy
Damaged endothelial cells slough off and create emboli that block blood flow

11

Cavernous hemangioma

Most common benign tumor of the liver
Well-circumscribed, subcapsular, < 2 cm
Consist of multiple vascular channels lined by a single layer of endothelial cells
Frequently asymptomatic and incidentally discovered

12

Chief clinical significance of cavernous hemangioma

Do not mistake them for a metastatic tumor and perform blind percutaneous needle bx, which could cause severe intra-abdominal bleeding

13

Hepatic adenoma

Usually in women of childbearing age who have used oral contraceptives (very rare)
May regress after d/c hormone use

14

Characteristics of hepatic adenoma

Often subcapsular
Sheets and cords of hepatocytes
Portal tracts are absent
Prominent vessels throughout
Risk for rupture, esp. during pregnancy

15

Hepatocellular carcinoma (HCC)

Malignant liver tumor
Can be solitary, multifocal (75%), diffuse, and infiltrating
Strong propensity for vascular invasion
Metastasizes to lymph nodes, lungs, bones, adrenals
Well-differentiated

16

Demographics of hepatocellular carcinoma

Structural, numerical chromosome abnormalities
Incidence peaks btwn 50-60 yoa
More common in men
More than 85% of all cases of HCC occur in parts of Asia and Africa where HBV infection is high and exposure to aflatoxin is more common

17

RF for hepatocellular carcinoma

Chronic viral hepatitis
Chronic alcoholism
Aflatoxin exposure
To a lesser extent, hemochromatosis or tyrosinemia

18

Hepatocellular carcinoma in Asia and Africa

Peak incidence is 20-40 yoa
50% appear in absence of cirrhosis

19

Hepatocellular carcinoma causes

Rapid increase in liver size
Sudden worsening of ascites or appearance of bloody ascites
Fever and pain

20

Tx for hepatocellular carcinoma

Surgical resection for smaller tumors
Liver transplantation

21

What is the mcc of cholelithiasis (gallstones) in the West?

Cholesterol stones
Most commonly women in reproductive years who are obese

22

What is the mcc of cholelithiasis in the East?

Pigment stones-Ca bilirubinate salts

23

S/Sx of cholelithiasis

Asymptomatic in 70-80% of cases
Intense pain from gallbladder obstruction
Cholecystitis
Erosion of large stone into bowel causing obstruction

24

Cholecystitis

Almost always occurs in association with cholelithiasis

25

Acute calculous cholecystitis

Inflammation of gallbladder containing stones
Inflammation from irritation, obstruction of bile flow
Most common major complication of gallstones
Most common reason for emergency cholecystectomy

26

Choledocholithiasis

Presence of stones in the biliary tree
West- most stones in gallbladder
Asia- most stones in biliary ducts

27

Sx of choledocholithiasis

10% asymptomatic
Biliary obstruction
Pancreatitis
Cholangitis
Liver disease
Acute calculous cholecystitis

28

Cholangitis

Acute inflammation of wall of bile ducts
Almost always caused by bacterial infection
Due to biliary obstruction, usually choledocholithiasis

29

Characteristics of cholangitis

Bacterial infection from the gut
-Fever
-Chills
-Abd pain
-Jaundice
Sepsis is a concern, so prompt diagnosis and intervention are imperative

30

What is the most frequent malignant tumor of the biliary tract?

Adenocarcinoma of the gallbladder
Highest in Hispanics, Native Americans in US
Rarely detected when it can be resected
Most frequent at 60-70 yoa
Gallstones present in 60-90% of cases

31

What does the exocrine pancreas consist of?

98%
Acinar cells
Ductules
Ducts

32

What does the endocrine pancreas consist of?

2%
Islets of Langerhans

33

What does the exocrine pancreas do?

Releases digestive proenzymes into duodenum

34

What does the endocrine pancreas do?

Releases hormones into the blood

35

What are the most significant diseases of the endocrine pancreas?

DM
Neoplasms

36

Cell types of endocrine pancreas

Alpha: produce glucagon
Beta: produce insulin and amylin
Delta: produce somatostatin
PP cells: produce pancreatic polypeptide

37

What do acinar cells in the exocrine pancreas produce?

Inactive digestive enzymes (proenzymes)
Amylase and lipase in active forms
Trypsin inactivator
Bicarbonate (antacid)

38

Where are products of acinar cells of the exocrine pancreas sent?

To the duodenum when it releases secretin and cholecystokinin (CCK)

39

What are the most significant diseases of the exocrine pancreas?

Acute and chronic pancreatitis
Carcinoma of the pancreas
Cystic fibrosis

40

Acute pancreatitis

80% of cases caused by either cholelithiasis or alcoholism

41

Pathophysiology of acute pancreatitis

Autodigestion of the pancreas caused by inappropriate activation of pancreatic enzymes (activation of trypsin is a critical triggering event)
-Duct obstruction
-Acinar cell injury
-Defective intracellular transport

42

Clinical features of acute pancreatitis

Abd pain is the cardinal manifestation
Full-blown is a significant medical emergency
Systemic release of digestive enzymes and an explosive activation of the inflammatory response
-Increased vascular permeability
-Leukocytosis
-DIC
-ARDS
-Diffuse fat necrosis

43

Findings in acute pancreatitis

Elevated plasma levels of amylase and lipase
Enlarged inflamed pancreas can be visualized by CT or MRI

44

Management of acute pancreatitis

Maintain BP and alleviate pain
Total restriction of food and fluids to rest pancreas

45

Chronic pancreatitis

Longstanding inflammation, fibrosis, and destruction of the pancreas
Destruction is irreversible
Most are alcohol-related, some idiopathic

46

Sx of chronic pancreatitis

Silent, or bouts of jaundice and pain

47

Dx of chronic pancreatitis

Difficult
Acinar destruction is significant and enzymes may not be elevated

48

Complications of chronic pancreatitis

Secondary diabetes
Malabsorption
Severe chronic pain

49

Pancreatic adenocarcinoma

Arises in ductal epithelial cells of the exocrine pancreas
One of the most lethal cancers
80% of cases between 60-80 yoa
More common in blacks
Smoking doubles the risk

50

Genetic mutations in pancreatic adenocarcinoma

Telomerase
K-RAS
p16
p53
SMAD4
BRCA2

51

Clinical features of pancreatic adenocarcinoma

Silent until metastasis has already occurred
Obstructive jaundice can be associated with cancer in the head of the pancreas
Weight loss, anorexia, generalized malaise, weakness
Surgery, radiation, chemo

52

What is hepatic vein thrombosis (Budd-Chiari syndrome) associated with?

Polycythemia vera or other myeloproliferative disorders
Pregnancy
Postpartum state
OCP use
Intra-abdominal cancers
Centrilobular congestion and necrosis

53

Tx of hepatic vein thrombosis (Budd-Chiari syndrome)

Surgery: portosystemic venous shunt

54

Degeneration and intracellular accumulation- hepatic injury

Damage from toxic and immunologic insult
Accumulation of substances, e.g., steatosis

55

Necrosis and apoptosis- hepatic injury

Centrilobar, submassive, massive necrosis

56

Regeneration-hepatic injury

Replication of hepatocytes, proliferation of oval cells to make new hepatocytes

57

Inflammation-hepatic injury

Hepatitis – may be limited to portal tracts or diffuse throughout parenchyma

58

Fibrosis in hepatic injury

Formed in response to inflammation or toxins

59

Cirrhosis- hepatic injury

Nodules of regeneration heaptocytes surrounded by bands of scar tissue- destroy liver architecture
End-stage liver disease
Increases risk of malignancy

60

Ductular reaction- hepatic injury

Proliferation of biliary ductules associated with fibrosis and inflammation

61

What is jaundice caused by?

Excessive production of bilirubin
Reduced hepatic uptake
Impaired conjugation
Decreased hepatocellular excretion
Impaired bile flow

62

Unconjugated hyperbilirubinemia

Excessive production of bilirubin
-Hemolytic anemias
Reduced hepatic uptake
-Neonatal jaundice

63

Conjugated hyperbilirubinemia

Decreased hepatocellular excretion
-Hepatitis
Impaired bile flow
-Inflammatory destruction of bile ducts

64

Cholestasis

Systemic retention of not only bilirubin, but also other solutes eliminated in bile, particularly bile salts and cholesterol
Impaired bile flow due to hepatocellular dysfunction or biliary obstruction
Elevated serum alkaline phosphotase

65

Hepatic failure

80-90% of hepatic functional capacity is lost
Massive hepatic necrosis
Chronic liver disease ending in cirrhosis

66

Causes of hepatic dysfunction without overt necrosis

Acute fatty liver of pregnancy
Tetracycline toxicity
Reye syndrome

67

Causes of massive hepatic necrosis

Fulminant viral hepatitis
Drugs and chemicals

68

Clinical signs of severe hepatic dysfunction

Jaundice
Hypoalbuminemia
Hyperammonemia
Hypoglycemia
Palmar erythema
Spider angiomas
Hypogonadism
Gynecomastia
Weight loss
Muscle wasting

69

Complications of hepatic failure

Multiple organ failure
Coagulopathy
Hepatic encephalopathy
Hepatorenal syndrome

70

Three defining characteristics of cirrhosis

Bridging fibrous septa- formation of scars around multiple adjacent lobules
Parenchymal nodules- proliferating hepatocytes that are encircled by fibrotic bands
Disruption of the architecture of the entire liver

71

Cirrhosis

Scarring and fibrosis of the liver, with altered liver architecture and function, caused by chronic liver disease

72

Most common causes of cirrhosis

Chronic alcoholism
Chronic hepatitis B and C
Biliary disease and hemochromatosis

73

Clinical features of cirrhosis

May be clinically silent
Anorexia, weight loss, weakness
Progression or improvement depends on the extent of the disease that caused it
Death results from: liver failure, portal htn, or hepatocellular cancer

74

Causes of portal hypertension

Pre-hepatic: occlusive thrombosis, narrowing of the portal vein
Intra-hepatic: cirrhosis, schistosomiasis, massive fatty change, diffuse granulomatous diseases
Post-hepatic: right-sided heart failure, constrictive pericarditis, hepatic vein outflow obstruction

75

Clinical features of portal htn

Ascites
Portosystemic venous shunts
Splenomegaly
Hepatic encephalopathy

76

Viral hepatitis

Infection of the liver by a small group of viruses that have a particular affinity for the liver
Most common types are hep A, hep B, and hep C

77

Hep A

Caused by infection with the hep A virus
Incubation period of about 28 days (range of 15-50)
Replicates in the liver and is shed in high concentrations in feces from 2 weeks before to 1 wk after the onset of clinical illness
Fecal-oral route transmission
Self-limited

78

Risk factors for hep A

Men who have sex with men
Illegal drug users
International travelers

79

Hep B

Incubation period of 1-6 mos
Found in highest concentrations in blood and in lower concentrations in other body fluids
Transmitted by percutaneous or mucous membrane exposure to infectious blood or body fluids that contain blood
Self-limited or chronic

80

Risk factors for hep B

Unprotected sex with infected partner
Men who have sex with men
Birth to an infected mother
History of other STDs
Illegal IV drug use

81

Hep C

Hep C and chronic alcoholism are the main cause of chronic liver disease in the US
Incubation period of 6-12 wks
Major route of transmission is through blood inoculation, with illegal IV drug use accounting for 40% of cases

82

Clinical features and outcomes of viral hepatitis

Asymptomatic
Acute viral hepatitis
Chronic viral hepatitis
Fulminant viral hepatitis

83

Autoimmune hepatitis

Mild to severe chronic hepatitis
Female predominance
Elevated serum igG (>2.5 g/dL)
Absence of serological markers of viral infection
Response to immunosuppressive therapy is good but remission is unusual

84

Alcoholic liver disease

Effects on liver: steatosis, hepatitis, cirrhosis

85

Causes of death in end-stage alcoholism

Liver failure
Massive GI hemorrhage
Infection
Hepatorenal syndrome
Hepatocellular carcinoma

86

Signs of drug-induced liver disease

Cholestasis
Hepatitis
Steatosis
Fibrosis
Necrosis
Liver failure

87

Nonalcoholic fatty liver disease

Common, asymptomatic in most people
Most often associated with insulin resistance, type 2 diabetes, obesity, and dyslipidemia

88

Nonalcoholic steatohepatitis

Fatigue, URQ pain, weight loss
Inflammation and scarring in the liver
Severe cases can progress to liver failure

89

Hereditary hemochromatosis

Excessive accumulation of iron in tissues and organs, esp the liver and pancreas
Type I is most common
Caused by autosomal recessive mutations in the HFE gene
80% of pts have a mutation in nucleotide 845
Men more common than women

90

Early sx of hereditary hemochromatosis

Fatigue
Joint pain
Abd pain
Loss of sex drive

91

Later s/sx of hereditary hemochromatosis

Cirrhosis
DM
Skin pigmentation

92

Tx for hereditary hemochromatosis

Phlebotomy
Iron chelators

93

Wilson disease

Excessive amts of copper accumulate in the body, esp the liver, brain, and eye
Autosomal recessive mutations in the ATP7B gene
This protein transports copper to other parts of the body via the blood

94

Defective ATP7B protein

Failure to add copper to ceruloplasmin, which accumulates in the liver
Failure to excrete copper into bile
Excessive copper accumulation causes toxic liver injury

95

S/sx of Wilson disease

Jaundice of skin or white of eyes
Fatigue
Loss of appetite
Abd swelling
Kayser-Fleisher ring around the cornea

96

Neuropsychiatric manifestations of Wilson disease

Clumsiness
Trembling
Difficulty walking
Speech problems
Deteriorating school work
Depression
Anxiety
Mood swings

97

Alpha1-antitrypsin deficiency

SERPINA1 gene encodes alpha-1 antitrypsin
Autosomal codominant mutations
Produced in liver then transported to lungs in the blood
Protects the liver and lungs from neutrophil elastase, a protein released from WBCs to fight infection

98

Alleles involved with alpha-1 antitrypsin

M allele= nl levels of alpha-1 antitrypsin
S allele= reduced levels of alpha-1 antitrypsin
Z allele= very low levels of alpha-1 antitrypsin

99

Signs of cirrhosis in alpha-1 antitrypsin deficiency

Swollen abdomen
Swollen feet or legs
Jaundice

100

Neonatal cholestasis

Failure of bilirubin secretion (and other components in the bile), resulting in conjugated hyperbilirubinemia and jaundice
Typically presents in first 2 wks of life

101

S/sx of neonatal cholestasis

Jaundice
Dark urine (conjugated bilirubin)
Acholic stools
Hepatomegaly

102

Most common causes of neonatal cholestasis

Idiopathic hepatitis
Biliary atresia

103

Reye syndrome

Rare; characterized by fatty change in the liver and acute onset encephalopathy
Typically develops in children less than 4 yrs old
Often following infection with influenza A or B, or varicella

104

S/sx of Reye syndrome

Persistent vomiting
Listlessness
Irritability or combativeness
Disorientation or confusion
Delirium
Convulsions
Loss of consciousness