Goljan Chapter 18 Hepatobiliary And Pancreatic Disorders

Front 1

describe Bilirubin metabolism

Back 1

Front 2

which disorders could have the following lab findings

Increased production of UCB , absent Urine Bilirubin, increase UBG, urobilinogen,

Back 2

Extravascular hemolytic anemias: e.g., hereditary spherocytosis, Rh and ABO HDN

Front 3

which disorders could have the following lab findings

Decreased uptake or conjugation of UCB , absent Urine Bilirubin, Normal UBG, urobilinogen,

Back 3

Gilbert syndrome: common genetic defect in uptake/conjugation of UCB; jaundice occurs with fasting
Crigler-Najjar syndromes: genetic disorders with decreased to absent conjugating enzymes
Physiologic jaundice of newborn: begins on day 3 of life; caused by normal macrophage destruction of fetal RBCs

Front 4

which disorders could have the following lab findings

CB >50% increase Urine Bilirubin, absent UBG, urobilinogen,

Back 4

Decreased intrahepatic bile flow Drug-induced (e.g., OCP)
Primary biliary cirrhosis

Dubin-Johnson syndrome: genetic defect in secretion into intrahepatic bile ducts; black pigment in hepatocytes

Rotor's syndrome: similar to Dubin-Johnson syndrome but without black pigment in hepatocytes

Decreased extrahepatic bile flow Gallstone in common bile duct Carcinoma of head of pancreas

Front 5

name the component of bilirubin metabolism

Senescent red blood cells (RBCs) are phagocytosed by splenic macrophages.

end product of heme degradation.

is lipid-soluble.

Back 5

Unconjugated bilirubin (UCB)
UCB is the end product of heme degradation.

Front 6

name the component of bilrubin metabolism

combines with albumin in the blood

Back 6

Unconjugated bilirubin (UCB)

Front 7

name the compent of bilirubin metabolism

UCB is taken up by hepatocytes. and then UCB is conjugated to produce which product

Back 7

onjugated bilirubin (CB).
CB is water-soluble.

Front 8

which components of bilirubin metabolism is the following

Temporarily stored in the gallbladder

Enters the duodenum via the common bile duct

Back 8

Conguated Bilirubin is secreted into the intrahepatic bile ducts.

Front 9

name the component of bilirubin metabolism

Intestinal bacteria convert CB to which product

Back 9

Intestinal bacteria convert CB to urobilinogen (UBG).

Front 10

what causes jaundice and what are the signs

Back 10

Jaundice is due to an increase in UCB and/or CB.

Jaundice is first noticed in the sclera.

Front 11

name the component of the liver function test

Specific enzyme for liver cell necrosis
Present in the cytosol

Back 11

Serum alanine transaminase (ALT)

Front 12

name the condition with the following component of the liver function test

ALT >AST:

Back 12

viral hepatitis

Front 13

name the component of the liver function test

Present in mitochondria
Alcohol damages mitochondria

Back 13

Serum aspartate transaminase (AST)

Front 14

name the condition in with the component of the liver function test

AST >ALT

Back 14

indicates alcoholic hepatitis

Front 15

name the component of the liver function test

Intra- or extrahepatic obstruction to bile flow

Induction of cytochrome P-450 system

Back 15

Serum γ-glutamyl transferase (GGT)

Front 16

name the classic condition with component of the liver function test

increases GGT

Back 16

alcoholic liver

Front 17

describe the component of the liver function test

Normal GGT and increased ALP-Serum alkaline phosphatase (ALP)

Back 17

source of ALP other than liver (e.g., osteoblastic activity in bone)

Front 18

name the condition with component of the liver function test

Increased GGT and ALP:

Back 18

liver cholestasis

Front 19

name the condition with component of the liver function test

Urine bilirubin present in excess

Back 19

Bilirubinuria: viral hepatitis, intra- or extrahepatic obstruction of bile ducts

Front 20

name the condition with component of the liver function test

excess Urine UBG

Back 20

Increased urine UBG: extravascular hemolytic anemias, viral hepatitis

Absent urine UBG: liver cholestasis

Front 21

where is Albumin is synthesized

Back 21

liver

Front 22

where are the Majority of coagulation factors are synthesized

Back 22

in the liver

Front 23

what does increase Prothrombin time (PT) indicate

Back 23

Increased PT: severe liver disease

Front 24

where does the Urea cycle take place

Back 24

liver

Front 25

what does a decrease in Blood urea nitrogen (BUN) indicate

Back 25

cirrhosis

Front 26

where is Ammonia metabolized

Back 26

Ammonia is metabolized in the urea cycle

Front 27

what does Increased serum ammonia indicate

Back 27

cirrhosis, Reye syndrome

Front 28

Serum IgM is increased in which GI condition

Back 28

Increased in primary biliary cirrhosis

Front 29

Antimitochondrial antibody is present in which GI condition

Back 29

primary biliary cirrhosis

Front 30

An antimicrosomal antibody is an antibody directed against microsomes.In which conditions is it associated with

Back 30

Hashimoto's thyroiditis

Front 31

Anti-smooth muscle antibody is present in which GI condition

Back 31

Autoimmune hepatitis

Front 32

α-Fetoprotein (AFP) is a tumor marker for which GI condition

Back 32

Hepatocellular carcinoma

Front 33

name the condition

Fever, painful hepatomegaly

Serum transaminases increase steadily.

Peak just before jaundice occurs

Atypical lymphocytosis

Back 33

Viral Hepatitis

Front 34

what are the urine bilirubin and urine UBG findings in viral hepatitis

Back 34

Increased urine bilirubin and urine UBG

Front 35

Microscopic findings in acute viral hepatitis include councilman bodies.. what are they

Back 35

Apoptosis of hepatocytes (Councilman bodies)

Front 36

name the type of hepatitis and how it is transmitted

No carrier state

Does not lead to chronic hepatitis

Occurs in day care centers, prisons, travelers to developing countries, and male homosexuals (anal intercourse)

Back 36

Hepatitis A - fecal /oral

Front 37

name the type of hepatitis and how it is transmitted

Carrier state may occur

Chronic hepatitis in 10% of immunocompetent patients

Serum sickness prodrome (5-10%): vasculitis (PAN), polyarthritis, membranous GN

Increased incidence of hepatocellular carcinoma

Back 37

Hepatitis B Parenteral, sexual, vertical (pregnancy, breast feeding)

Front 38

name the type of hepatitis and how it is transmitted

Carrier state may occur

Mild hepatitis; jaundice uncommon

Chronic hepatitis in >70% of cases

Associated with posttransfusion hepatitis, type I MPGN, alcohol excess, PCT

Increased incidence of hepatocellular carcinoma

Back 38

Hepatitis C Parenteral, sexual

Front 39

name the type of hepatitis and how it is transmitted

Carrier state may occur

Requires HBsAg to replicate

Chronic state less likely with coinfection (HBV and HDV exposure at same time) than superinfection

Back 39

Hepatitis D Parenteral, sexual

Front 40

name the type of hepatitis and how it is transmitted

No carrier state or chronic hepatitis

Fulminant hepatitis may develop in pregnant women

Occurs in developing countries

Back 40

Hepatitis E Fecal-oral (waterborne)

Front 41

what are the finding in Earliest phase of acute HBV

Back 41

HBsAg, hepatitis B surface antigen only

Front 42

what are the serological findings in Acute infection of Hepatitis B

Back 42

1. Hepatitis B surface antigen (HBsAg)

2. Hepatitis B e antigen (HBeAg) and HBV-DNA
Infective particles
Appear after HBsAg and disappear before HBsAg

3. Anti-HBV core antibody IgM (anti-HBc-IgM)-Nonprotective antibody
Remains positive in acute infections

Front 43

what is the First marker of infection with Hepatitis B and when does it appear after infection

Back 43

Hepatitis B surface antigen (HBsAg)
Appears within 2 to 8 weeks after exposure

Front 44

Anti-HBV core antibody IgM (anti-HBc-IgM) converts to what in 6 months

Back 44

Converts to anti-HBc-IgG in 6 months

Front 45

Window phase, or serologic gap of Heptatis B contains which marker

Back 45

Anti-HBV core antibody IgM (anti-HBc-IgM) ONLY

Nonprotective antibody

Front 46

what serological marker provides that Protective antibody against Hepatitis B

Back 46

Anti-HBV surface antibody (anti-HBs)

Front 47

which marker would indicate immunization after HBV vaccination

Back 47

Anti-HBV surface antibody (anti-HBs)
ONLY

Front 48

what seroligical marker would indicate Recovered from HBV

Back 48

1. Anti-HBV surface antibody (anti-HBs)

and

2. anti-HBc-IgG

Front 49

what serological marker would indicate
"Healthy" carrier if HBsAg >6 months

Back 49

Presence of HBsAg and anti-HBc-IgG
and
Absence of DNA and e antigen

Front 50

what serological markers would indicate Infective chronic carrier of hepatitis B. what is the pt at increased risk for developing

Back 50

Presence of HBsAg, anti-HBc-IgG, and infective particles (DNA and e antigen)

Increased risk for postnecrotic cirrhosis and hepatocellular carcinoma

Front 51

Hepatitis C virus (HCV) is Screened with which modality. HCV is confirmed with which modality

Back 51

Screen with enzyme immunoassay
Presence of anti-HCV-IgG indicates infection or recovery.

Confirmatory tests
Recombinant immunoblot assay (RIBA)
HCV RNA using polymerase chain reaction

Front 52

t/f anti-HCV-IgG a protective antibody.

Back 52

FALSE- anti-HCV-IgG indicates infection or recovery.
It is not a protective antibody.

Front 53

t/f Presence of anti-HDV-IgM or IgG indicates a protective antibody.

Back 53

false- Hepatitis D virus (HDV)
Presence of anti-HDV-IgM or IgG indicates active infection.
IgG is not a protective antibody.

Front 54

t/f Presence of anti-HEV-IgM indicates recovery and protective antibody

Back 54

true- Hepatitis E virus (HEV)
Presence of anti-HEV-IgM indicates active infection.
Anti-HEV-IgG indicates recovery (protective antibody).

Front 55

how does conjugated bilirubin gain access to blood to increase the content

Back 55

CB gains access to blood via damaged bile ductules.

Front 56

Increased urine UBG and urine bilirubin
is present in Viral hepatitis: defect in uptake, conjugation of UCB and secretion of CB. How does urine UBG and uring bilirubin gain access to urine

Back 56

CB is water-soluble and is filtered in the kidneys.
UBG recycled back to inflamed liver is redirected to the kidneys.

Front 57

name the pathogen that causes the following Infectious Diseases of the Liver

Usually right lobe abscess

Back 57

Entamoeba histolytica

Front 58

name the pathogen of Infectious Diseases of the Liver

Inflammation of bile ducts (cholangitis) from concurrent biliary infection and duct obstruction (e.g., stone)

Triad of fever, jaundice, right upper quadrant pain

Most common cause of multiple liver abscesses

Back 58

e. coli

Front 59

name the pathogen that produces the following Infectious Diseases of the Liver

Contracted by ingesting encysted larvae in fish; larvae enter common bile duct and become adults

May produce cholangiocarcinoma

Back 59

Clonorchis sinensis (Chinese liver fluke)

Front 60

name the pathogen that produces the following infectious disease of the liver

Single or multiple cysts containing larval forms

Dog is definitive host; human is intermediate host

Rupture of cysts can produce anaphylaxis

Back 60

Echinococcus granulosus (sheepherder's disease)

Front 61

name the pathogen that produces the following liver disease

Eggs incite a fibrotic response in the portal vein ("pipestem cirrhosis")

Complications of cirrhosis: portal hypertension, ascites, and esophageal varices

Back 61

Schistosoma mansoni

Front 62

name the condition

Clinical findings
Fever, jaundice, hepatosplenomegaly

Laboratory findings
Positive serum antinuclear antibody (ANA) test

Anti-smooth muscle antibodies

Back 62

Autoimmune hepatitis

Front 63

name the condition

Usually develops in children younger than 4 years of age

Often follows a chickenpox or influenza infection

Back 63

Reye syndrome

Front 64

name the condition

Microvesicular type of fatty liver- Small cytoplasmic globules without nuclear displacement

Clinical findings
Encephalopathy
Cerebral edema, coma, convulsions
Hepatomegaly

Back 64

Reye syndrome

Front 65

name the condition

Hypertension, proteinuria, dependent pitting edema in third trimester

Liver cell necrosis around portal triads
Increased serum transaminases

Back 65

Preeclampsia

Front 66

describe the HELLP syndrome

Back 66

Hemolytic anemia with schistocytes
Elevated serum transaminases
Low platelets-Due to disseminated intravascular coagulation

Front 67

name the condition

Acute liver failure with encephalopathy within 8 weeks of hepatic dysfunction

Back 67

Fulminant hepatic failure

Front 68

what are the Gross , microscopic and clinical findings in Fulminant hepatic failure

Back 68

Wrinkled capsular surface due to loss of hepatic parenchyma

Dull red to yellow necrotic parenchyma with blotches of green (bile)

Clinical findings
Hepatic encephalopathy and jaundice

Front 69

what are the Laboratory findings in Fulminant hepatic failure

Back 69

Decrease in transaminases-Liver parenchyma is destroyed.

Increase in PT and ammonia

Front 70

why is Liver infarction is uncommon

Back 70

because of a dual blood supply:
Hepatic artery and portal vein tributaries normally empty blood into the sinusoids

Front 71

which condition can lead to this presentation in the liver

Centrilobular hemorrhagic necrosis

Back 71

("nutmeg" liver). The liver has a mottled cut surface. Dark areas represent congested central veins and sinusoids.

Most often due to left-sided heart failure (LHF) and right-sided heart failure (RHF)

LHF decreases cardiac output causing hypoperfusion of the liver.
Causes ischemic necrosis of hepatocytes located around central vein

RHF causes a back-up of systemic venous blood into the central veins and sinusoids.

Front 72

name the condition

Sinusoidal dilation due to blood
Causes
Anabolic steroids

Bartonella henselae causing bacillary angiomatosis - Occurs in AIDS

Potential for intraperitoneal hemorrhage

Back 72

Peliosis hepatis

Front 73

name the condition and give 2 examples

Obstruction of blood flow out of the liver

Back 73

Posthepatic obstruction to blood flow

Hepatic vein thrombosis

Veno-occlusive disease

Front 74

list 3 causes of Hepatic vein thrombosis leading to Posthepatic obstruction to blood flow causing:

Enlarged, painful liver
Portal hypertension, ascites, splenomegaly
High mortality rate

Back 74

Polycythemia vera

Oral contraceptive pills

Hepatocellular carcinoma- Invades hepatic vein

Front 75

name a condition that would lead to Veno-occlusive disease producing Posthepatic obstruction to blood flow

Back 75

Complication of bone marrow transplantation

Collagen develops around the central veins.

Front 76

why is Fatty liver change is the most common type of disease seen in Alcohol-related disorders

Back 76

Substrates of alcohol metabolism are used to synthesize liver triglyceride.

Front 77

describe the pathogenesis of Alcoholic hepatitis

Back 77

Due to acetaldehyde damage to hepatocytes
Stimulation of collagen synthesis around the central vein
Perivenular fibrosis

Front 78

what are the 3 Microscopic findings in Alcoholic hepatitis

Back 78

1. Fatty change with neutrophil infiltration

2. Mallory bodies- Damaged cytokeratin intermediate filaments in hepatocytes

3. Perivenular fibrosis

Front 79

name the tumor or disease associated with Chemical- or drug-induced liver disease

Vinyl chloride, arsenic, thorium dioxide (radioactive contrast material)

Back 79

Angiosarcoma

Front 80

name the tumor or disease associated with Chemical- or drug-induced liver disease

Thorium dioxide-used as a stabilizer in tungsten electrodes in TIG welding, electron tubes, and aircraft engines

Back 80

Cholangiocarcinoma

Front 81

name the tumor or disease associated with Chemical- or drug-induced liver disease

Aflatoxin (due to Aspergillus mold)

Back 81

Hepatocellular carcinoma

Front 82

name the tumor or disease associated with Chemical- or drug-induced liver disease

Oral contraceptive pills

Back 82

Liver cell adenoma or Cholestasis

Front 83

name the tumor or disease associated with Chemical- or drug-induced liver disease

Isoniazid (caused by toxic metabolite), halothane, acetaminophen, methyldopa

Back 83

Acute hepatitis

Front 84

name the tumor or disease associated with Chemical- or drug-induced liver disease

Oral contraceptive pills (estrogen interferes with intrahepatic bile secretion), anabolic steroids

Back 84

Cholestasis

Front 85

name the tumor or disease associated with Chemical- or drug-induced liver disease

Amiodarone (resembles alcoholic hepatitis; Mallory bodies and progression to cirrhosis), methotrexate

Back 85

Fatty change or fibrosis in the liver

Front 86

what is the difference between Intrahepatic cholestasis and Extrahepatic cholestasis

Back 86

Intrahepatic cholestasis
Blockage of the intrahepatic bile ducts

Extrahepatic cholestasis
Blockage of common bile duct (CBD)

Front 87

Drugs (e.g., oral contraceptive pills, anabolic steroids, Neonatal hepatitis
Pregnancy-induced cholestasis (estrogen) produce which Type of cholestatic liver disease

Back 87

Intrahepatic cholestasis

Front 88

Stone usually originating from the gallbladder, Primary sclerosing pericholangitis, Extrahepatic biliary atresia and Carcinoma head of pancreas most likely produce which Type of cholestatic liver disease

Back 88

Extrahepatic cholestasis

Front 89

the Clinical findings of obstructive liver disease include jaundice. what causes the jaundice

Back 89

Jaundice with pruritus
Pruritus due to bile salts deposited in skin

Front 90

the Clinical findings of obstructive liver disease include Malabsorption. descibe why

Back 90

Malabsorption
Bile salts do not enter the small intestine.

Front 91

the Clinical findings of obstructive liver disease include Light-colored stools. why

Back 91

Due to a lack of urobilin

Front 92

what are the Laboratory findings in obstructive liver disease

Back 92

CB >50%
Bilirubinuria
Absent urine UBG
Increase in serum ALP and GGT

Front 93

name the condition

Obliterative fibrosis of intrahepatic and extrahepatic bile ducts
Male dominant
Associated with ulcerative colitis

Clinical findings
Jaundice
Cirrhosis
Increased incidence of cholangiocarcinoma

Back 93

Primary sclerosing pericholangitis

Front 94

name the condition

Irreversible diffuse fibrosis of the liver with formation of regenerative nodules

Back 94

Cirrhosis

Front 95

describe the gross appearance of this cirrhotic liver

Back 95

Hepatocyte reaction to injury -diffuse micronodular surface of the liver.

Lack normal liver architecture

Lack of portal triads and sinusoids

Surrounded by bands of fibrosis
Compress sinusoids and central veins

Intrasinusoidal hypertension
Reduction in the number of functional sinusoids
Increase in hydrostatic pressure in portal vein

Front 96

list 4 causes of this type of liver

Back 96

1. Alcoholic liver disease (most common)

2. Postnecrotic cirrhosis (HBV, HCV)

3. Autoimmune disease (primary biliary cirrhosis)

4. Metabolic disease:
Hemochromatosis, Wilson's disease
α1-Antitrypsin deficiency, galactosemia

Front 97

desribe the common condition that produce this symptom

Back 97

spider angioma (telangiectasia) on the cheek of a patient with cirrhosis.

Multiple spider angiomas are common in cirrhosis and pregnancy.

Front 98

why does hepatic failure lead to Multiple coagulation defects

Back 98

Due to inability to synthesize coagulation factors
Produces a hemorrhagic diathesis

Front 99

why does hepatic failure produce dependent pitting edema and ascites

Back 99

Hypoalbuminemia from decreased synthesis of albumin

Front 100

what cause the Hepatic encephalopathy
in hepatic failure

Back 100

1. Increase in aromatic amino acids (e.g., phenylalanine, tyrosine, tryptophan)- Converted into false neurotransmitters (e.g., gamma aminobutyric acid)

2. Increase in serum ammonia
Due to a defective urea cycle that cannot metabolize ammonia

Front 101

what are the Clinical findings in hepatic encephalopathy due to hepatic failure

Back 101

Alterations in the mental status
Somnolence and disordered sleep rhythms

Asterixis (i.e., inability to sustain posture, flapping tremor)

Coma and death in late stages

Front 102

describe the Pathogenesis of portal hypertension

Back 102

Resistance to intrahepatic blood flow due to intrasinusoidal hypertension

Anastomoses between portal vein tributaries and the arterial system

Front 103

describe 5 Complications of portal hypertension

Back 103

1. Ascites

2. Congestive splenomegaly- Increased hydrostatic pressure in splenic vein. Hypersplenism with various cytopenias may occur

3. Esophageal varices

4. Hemorrhoids,

5. periumbilical venous collaterals (caput medusae)

Front 104

what cause the Hyperestrinism in males
with liver cell failure

Back 104

Liver cannot degrade estrogen and 17-ketosteroids (e.g., androstenedione).
Androstenedione is aromatized into estrogen in the adipose cell.

Front 105

name the condition

Autoimmune disorder

Granulomatous destruction of bile ducts in portal triads

Occurs more often in women between 40 and 50 years of age

Progresses from a chronic inflammatory reaction to cirrhosis

Back 105

Primary biliary cirrhosis (PBC)

Front 106

name the condition

Pruritus- Deposition of bile salts in skin. EARLY finding well before jaundice appears

Hepatomegaly

Jaundice- LATE finding after most of the bile ducts have been destroyed
Cirrhosis with portal hypertension
Increased risk for hepatocellular carcinoma

Back 106

Primary biliary cirrhosis (PBC)

Front 107

name the condition

Laboratory findings
Antimitochondrial antibodies (>90% of cases)

Increase in IgM

Back 107

Primary biliary cirrhosis (PBC)

Front 108

name the condition

Complication of chronic extrahepatic bile duct obstruction
Example-cystic fibrosis, where bile is dehydrated
No increase in antimitochondrial antibodies or IgM

Back 108

Secondary biliary cirrhosis

Front 109

describe the The normal function of the HFE gene produc

Back 109

to facilitate the binding of plasma transferrin (binding protein of iron) with its mucosal cell transferrin receptor so that transferrin can be endocytosed by intestinal cells. The amount of endocytosed transferrin iron determines how much mucosal cell iron is released into the plasma.

this pic-Liver biopsy stained with Prussian blue in a patient with hereditary hemochromatosis. The hepatocytes are filled with blue iron granules. This is an early stage before parenchymal damage and fibrosis develop.

Front 110

describe the pathogenesis of hereditary hemochromatosis

Back 110

1. Unrestricted reabsorption of iron in the small intestine

2. Mutations involving hereditary hemochromatosis gene (HFE)

3. Iron stimulates the production of hydroxyl free radicals-Free radicals damage tissue and cause fibrosis.

Front 111

in which organs does iron from
hereditary hemochromatosis deposit other than The hepatocytes are filled with blue iron granules

Back 111

Liver, pancreas, heart, joints, skin, pituitary

Front 112

describe the clinical findings in pt with hereditary hemochromatosis (6)

Back 112

1. Cirrhosis- Iron deposits primarily in hepatocytes . Increased risk of hepatocellular carcinoma

2. "Bronze diabetes"- Type I diabetes mellitus. Destruction of β-islet cells

3. Hyperpigmentation- Iron deposits in skin and increases melanin production

4. Malabsorption- Destruction of exocrine pancreas

5. Restrictive cardiomyopathy, degenerative joint disease

6. Increased serum iron, percent saturation, and ferritin

Front 113

why is there a Decreased in total iron-binding capacity in pt with hereditary hemochomatosis

Back 113

Transferrin synthesis is decreased when iron stores are increased

Front 114

describe the pathogenesis of this disorder

Back 114

1. Gene mutation- AR- Defective hepatocyte transport of copper into bile for excretion. Decreased synthesis of ceruloplasmin (binding protein for copper in blood)

2. Unbound copper eventually accumulates in blood

Loosely attached to albumin
Copper deposits in other tissues causing a toxic effect.

Front 115

what causes the Kayser-Fleischer ring
in Wilson's disease

Back 115

Due to free copper deposits in Descemet's membrane in the cornea

The gene defect in Wilson's disease affects a copper transport system that produces a dual defect-decreased synthesis of ceruloplasmin in the liver and decreased excretion of copper into bile.

Front 116

what causes the Central nervous system symptoms in Wilson's

Back 116

Copper deposits in the putamen
Produces a movement disorder resembling parkinsonism

Copper deposits in the subthalamic nucleus- Produces hemiballismus

Copper is toxic to neurons in the cerebral cortex- Produces dementia

Front 117

what is Useful in diagnosing Wilson's disease in its early stages

Back 117

Decreased serum ceruloplasmin

Front 118

what is Useful in diagnosing Wilson's disease in the later stages

Back 118

Increased serum and urine free copper

Front 119

name the condition and describe the pathogensis

Neonatal hepatitis with intrahepatic cholestasis

Most common cause of cirrhosis in children

Increased risk for hepatocellular carcinoma

Young adults with panacinar emphysema

Back 119

α1-Antitrypsin (AAT) deficiency

Alleles are inherited codominantly (each allele expresses itself).
Normal genotype is PiMM.
Most common abnormal allele is Z.
PiZZ variant has decreased AAT levels in serum.

Production of a mutant protein that cannot be secreted into blood
Accumulation of AAT in hepatocytes causes liver damage.

Front 120

what causes the Decreased serum blood urea nitrogen (BUN) and increased serum ammonia in cirrhosis

Back 120

Due to disruption of the urea cycle in the liver

Front 121

what causes the Fasting hypoglycemia
in cirrhosis

Back 121

Defective gluconeogenesis and decreased glycogen stores

Front 122

name the condition and describe the pathogensis

Neonatal hepatitis with intrahepatic cholestasis

Most common cause of cirrhosis in children

Increased risk for hepatocellular carcinoma

Young adults with panacinar emphysema

Back 122

α1-Antitrypsin (AAT) deficiency

Alleles are inherited codominantly (each allele expresses itself).
Normal genotype is PiMM.
Most common abnormal allele is Z.
PiZZ variant has decreased AAT levels in serum.

Production of a mutant protein that cannot be secreted into blood
Accumulation of AAT in hepatocytes causes liver damage.

Front 123

what cause the Chronic respiratory alkalosis in cirrhosis

Back 123

Toxic products from hepatic dysfunction overstimulate respiratory center

Front 124

what causes the Decreased serum blood urea nitrogen (BUN) and increased serum ammonia in cirrhosis

Back 124

Due to disruption of the urea cycle in the liver

Front 125

what causes the Lactic acidosis in cirrhosis

Back 125

Liver dysfunction in converting lactic acid to pyruvate

Front 126

what causes the Fasting hypoglycemia
in cirrhosis

Back 126

Defective gluconeogenesis and decreased glycogen stores

Front 127

what cause the Chronic respiratory alkalosis in cirrhosis

Back 127

Toxic products from hepatic dysfunction overstimulate respiratory center

Front 128

what causes the Lactic acidosis in cirrhosis

Back 128

Liver dysfunction in converting lactic acid to pyruvate

Front 129

in what pt population is Liver (hepatic) cell adenoma most commonly seen and why

Back 129

Benign tumor of hepatocytes
Usually occur in women of childbearing age

Associated with the use of oral contraceptive pills

Front 130

in what pt population is Liver (hepatic) cell adenoma most commonly seen and why

Back 130

Benign tumor of hepatocytes
Usually occur in women of childbearing age

Associated with the use of oral contraceptive pills

Front 131

Hepatocellular carcinoma. Multiple large, hemorrhagic tumor masses are present in the liver. There is also diffuse infiltration of tumor blending in with the remaining liver.

list 3 causes

Back 131

1. chronic HBV and HCV

2. Aflatoxins (from Aspergillus mold in grains and peanuts)

3. Hereditary hemochromatosis, alcoholic cirrhosis, PBC, AAT deficiency

Front 132

what is the characteristic microscopic finding in Hepatocellular carcinoma.

Back 132

characteristic finding is the presence of bile in neoplastic cells.

Front 133

list 3 important Laboratory markers for heptocellluar carcinoma

Back 133

1. Increased α-fetoprotein (AFP)

2. Production of ectopic hormones
Erythropoietin (secondary polycythemia)

Insulin-like factor (hypoglycemia)

Front 134

what is the most common site for metastasis of a hepatocellular carcinoma

Back 134

Lung most common metastatic site

Front 135

Exposure to vinyl chloride (most common cause), arsenic, or thorium dioxide increases the risk for which type of cancer

Back 135

Angiosarcoma

Front 136

name the cystic disease

most common cyst in biliary tract in children younger than 10 years old

Clinical findings
Abdominal pain with persistent or intermittent jaundice

Back 136

Choledochal cyst

Front 137

name the cystic condition


AR
Segmental dilatation of intrahepatic bile ducts

Clinical findings
Association with polycystic kidney disease
Increased incidence of cholangiocarcinoma

Back 137

Caroli disease

Front 138

name the condition

Most common malignancy of bile ducts

Clinical findings
Obstructive jaundice

Palpable gallbladder (Courvoisier's sign)

Back 138

Cholangiocarcinoma

Front 139

name 4 cause of Causes of cholangiocarcinoma

Back 139

1. Primary sclerosing pericholangitis
Most common cause in United States

2. Clonorchis sinensis (Chinese liver fluke)

3. Thorotrast (thorium dioxide)

4. Choledochal cyst and Caroli disease

Front 140

(80% of cases) Gallstones (cholelithiasis)

They are radiolucent

Back 140

Yellow cholesterol stones with centers containing entrapped bile pigments. The wall of the gallbladder is scarred

Front 141

name the type of gallstone

Black and brown pigment stones
Some are radiopaque.

Back 141

Pigment stones

Front 142

name the type of gallstone

Black and brown pigment stones
Some are radiopaque.

Back 142

Pigment stones

Front 143

describe the pathogenesisi

Back 143

Supersaturation of bile with cholesterol

Decreased bile salts and lecithin
Both normally solubilize cholesterol in bile

Front 144

describe the pathogenesis

Back 144

black pigment stones -Sign of chronic extravascular hemolytic anemia (e.g., sickle cell anemia)
Excess bilirubin in bile produces calcium bilirubinate

Brown pigment stones
Sign of infection in the Collecting Bile Dut

Front 145

what are the risk factors

Back 145

4 F's

Female over 40 years old

Obesity

Cholesterol is increased in bile.

Use of oral contraceptive pills
Estrogen increases cholesterol in bile.

Rapid weight loss, use of lipid-lowering drugs

Native Americans (e.g., Pima and Navajo Indians)

Front 146

name the condition

Fever with nausea and vomiting

Usually 15 to 30 minutes after eating

Initial midepigastric colicky pain
Pain eventually shifts to the right upper quadrant.

Pain is constant and dull

Pain may radiate to right scapula

Back 146

Acute cholecystitis

Front 147

what is the GOLD standard for testing for gall stones

Back 147

Ultrasound is the gold standard

Radionuclide scan identifie

Front 148

name the condition

Severe, persistent pain 1 to 2 hours postprandially

Recurrent epigastric distress, belching, and bloating

Back 148

Chronic cholecystitis

Pathogenesis
Cholelithiasis with repeated attacks of minor inflammation

Front 149

name the condition

Gallbladder with dystrophic calcification

Dominant in elderly women

Poor prognosis

Back 149

Gallbladder adenocarcinoma

Front 150

name the condition

Dorsal and ventral buds form a ring around the duodenum.

Associated with small bowel obstruction

Back 150

Annular pancreas

Front 151

describe how the Major pancreatic duct and CBD are confluent in their terminal part

Back 151

Both empty their contents into the duodenum via the ampulla of Vater

Front 152

describe the Important in the pathogenesis of acute pancreatitis

Back 152

Stone(s) obstruct terminal part of the CBD →
Increased back-pressure refluxes bile into the major pancreatic duct →
Bile activates pancreatic proenzymes causing acute pancreatitis

Front 153

t/f an increase in amylase is specific for pancreatitis

Back 153

false- increase in amylase is not specific for pancreatitis

Front 154

what are the 2 major causes of acute pancreatitis

Back 154

Alcohol abuse and gallstones are the major causes

Front 155

what are the 2 major causes of acute pancreatitis

Back 155

Alcohol abuse and gallstones are the major causes

Front 156

describe the pathogenesis of acute pancreatitis

Back 156

Must be activation of pancreatic proenzymes (inactive enzymes)-Activation leads to autodigestion of the pancreas

Mechanisms of activation of proenzymes
a. Obstruction of the main pancreatic duct or terminal CBD

b. Gallstones

c. Alcohol thickens ductal secretions
Also increases duct permeability to enzymes

d.Chemical injury of acinar cells
Examples-thiazides, alcohol, triglyceride (>1000 mg/dL)

e. Infectious injury of acinar cells
Examples-CMV, mumps, coxsackievirus

f. Mechanical injury of acinar cells
Examples-seat belt trauma, posterior penetration of duodenal ulcer

Metabolic activation of proenzymes (e.g., hypercalcemia, ischemia, shock)

Trypsin is important in the activation of proenzymes.

Front 157

name the condition

fever, nausea and vomiting

Severe, boring midepigastric pain with radiation into the back
Radiation into back is due to its retroperitoneal location.

Back 157

acute pancreatitis

Front 158

what causes the shock in acute pancreatitis

Back 158

Due to hemorrhage and loss of enzyme-rich fluid around the pancreas (called "third spacing")

Front 159

what causes the Hypoxemia in acute pancreatitis

Back 159

Circulating pancreatic phospholipase destroys surfactant.
Loss of surfactant produces atelectasis and intrapulmonary shunting.

Acute respiratory distress syndrome (ARDS) may occur.

Front 160

what causes the Hypoxemia in acute pancreatitis

Back 160

Circulating pancreatic phospholipase destroys surfactant.
Loss of surfactant produces atelectasis and intrapulmonary shunting.

Acute respiratory distress syndrome (ARDS) may occur.

Front 161

what are Grey-Turner's signand Cullen's sign in acute pancreatitis

Back 161

Grey-Turner's sign (flank hemorrhage), Cullen's sign (periumbilical hemorrhage)

Front 162

what causes the Tetany in acute pancreatitis

Back 162

Hypocalcemia is caused by enzymatic fat necrosis.
Calcium binds to fatty acids leading to a decrease in ionized calcium.

Front 163

what lab findings are specific for acute pancreatitis

Back 163

Increased serum lipase

More specific for pancreatitis

Serum levels return to normal in 3 to 5 days.

Is not excreted in urine

Front 164

what is the gold standard for pancreatic imaging.

Back 164

Computed tomographic (CT) scan is the gold standard for pancreatic imaging.

Plain abdominal radiograph
Sentinel loop in subjacent duodenum or transverse colon (cut-off sign)
Localized ileus, where the bowel does not demonstrate peristalsis
Left-sided pleural effusion containing amylase (10% of cases)

Front 165

Complications of acute pancreatitis include Pancreatic pseudocyst. describe thise pseudocysts

Back 165

Collection of digested pancreatic tissue around pancreas
Abdominal mass with persistence of serum amylase longer than 10 days
Amount of amylase in the fluid surpasses renal clearance of amylase.

Front 166

list the common causes of Chronic pancreatitis in adults, children, and developing countries

Back 166

Alcohol abuse is the most common known cause.

Cystic fibrosis is the most common cause in children.

Malnutrition is the most common cause in developing countries.

Front 167

name the condition

Radiographic dyes show a "chain of lakes" appearance in the pancreatic major duct.

Pancreatic calcifications (CT scan best study)

Clinical findings
Severe pain radiating into the back
Malabsorption
Type 1 diabetes mellitus
Pancreatic pseudocyst

Back 167

Chronic pancreatitis

Front 168

what causes Exocrine pancreatic cancer

Back 168

Smoking (most common cause)

Chronic pancreatitis

Hereditary pancreatitis

Front 169

describe the Pathogenesis of exocrine pancreatic cancer

Back 169

Association with K-RAS gene mutation
Mutation of suppressor genes (TP16 and TP53)

Front 170

what is the location of most exocrine pancreatic cancers

Back 170

Most occur in the pancreatic head (65% of cases)
Often blocks CBD causing jaundice
Remainder occur in the body and tail

Front 171

what are the clinical and lab findings of exocrine pancreatic cancer

Back 171

Epigastric pain with weight loss

Signs of CBD obstruction (carcinoma of head of pancreas)

Jaundice (CB > 50%)

Light-colored stools (absent UBG)

Palpable gallbladder (Courvoisier's sign)

Superficial migratory thrombophlebitis (see Chapter 8)

Increased CA19-9
Gold standard tumor marker