Gi Block Two Day 2

Front 1

What diseases mainly elevate alkaline phosphatase (Cholestatic/Obstructive)

Back 1

Stones, PBC, PSC, medications

Front 2

What diseases are hepatocellular injury? (elevated ALT/AST)

Back 2

alcohol, viral hep, autoimmune hep, hemochromatosis, Wilson's dz, fatty liver, a-1 antitrypsin defic., medications

Front 3

In infiltrative diseases (tumors, amyloid, sarcoid) what does the liver panel look like?

Back 3

alkaline phosphatase elevation, occasional bilirubin elevation

Front 4

If the ALT >500 what could it be?

Back 4

HepA, B, autommune hepatitis, medications, ischemia

Front 5

What are the liver values for an alcoholic liver?

Back 5

- Alcoholic liver rarely ALT or AST > 300
- AST:ALT > 2:1 -> suggestive of alcoholic liver dz

Front 6

INR is indicative of?

Back 6

Severity of liver failure and general illness of patient

Front 7

Fulminant liver failure?

Back 7

Acute liver failure + coagulopathy + Encephalopathy

Usually d/t acetaminophen and viral hepatitis (CANT be HepC)

Front 8

Cholestasis

Back 8

When the flow of bile from the liver is inhibited or blocked

Front 9

AST (reactants and product) and where are the isoforms

Back 9

Aspartate-->oxaloacetic acid; Cytoplasm and Mitochondria (mostly mitochondria); Broad distribution in liver, cardiac muscle, sk. muscle, kidney, brain, pancreas, lung, etc

Front 10

ALT (reactants and product) and where are the isoforms

Back 10

Alanine-->pyruvic acid; Only cytoplasm isoform; More sensitive to PLP deficiency than AST

Front 11

If alk phos is high, what do you need to confirm biliary?

Back 11

GGT (gamma-glutamyl transpeptidase)

Front 12

Binge drinking?

Back 12

4/5 or more drinks on a single occasion men/women

Front 13

Heavy drinking

Back 13

1/2 drinks per day women/men

Front 14

Pathogenesis of Alcohol Liver disease?

Back 14

Increased NADH (Inhibition of TCA cycle; reduced gluconeogenesis, reduced fatty acid oxidation)

Increased Acetaldehyde (activates stellate cells to form collagen (obliterated Space of Disse), Microfilaments that maintain intracellular skeleton are sheared, Kuppfer cells produce TNFa)

Loss of fenestrations, protein cant get out, lose microvili on the hepatocyte

Front 15

Risk factors for ALD?

Back 15

Quantity of alcohol >20/30 g/day in women/men; drinking outside of meals; Binge drinking; Hepatitis C

Front 16

What is the treatment for ALD?

Back 16

Abstinence, nutritional support, Anti-inflammatory drugs-glucocorticoids, Pentoxifylline (inhibits TNFa)

Front 17

Discriminant function?

Back 17

4.6(Pt-PT control)+T Bilirubin; patients >32 have a one month mortality 30-50%

Front 18

List the stages of fibrosis

Back 18

0: no fibrosis; 1: Portal fibrosis; Stage 2 extend past portal triad; 3-stage to central vein; 4-Nodules Easy to identify

Front 19

What are the risk factors for NAFL?

Back 19

Obese, diabetes, elderly (associated with metabolic syndrome); Hypertriglycerides 3x risk with >; Hispanic, Male

Front 20

How does weight loss imporve NAFL/NASH

Back 20

Weight loss in NAFL/ NASH: 3-5% body weight to improve steatosis, 10% body weight to improve inflammation

Front 21

Treatment for biopsy proven NASH

Back 21

Vitamin E, pioglitazone=weight gain; used in non-diabetics; BOTH used in NON-diabetics; both decrease liver enzymes but NOT fibrosis

Front 22

What is the function of Alpha 1 antitrypsin?

Back 22

protease inhibitor and inhibits ELASTASE

Front 23

A1AT deficiency leads to

Back 23

Lung damage (loss of function-can replace with enzyme)
Liver damage (cant replace with enzyme; need liver t-plant b/c Gain of function)

Front 24

PiSZ & Pi Null Null

Back 24

Lung disease only

Front 25

PiZZ

Back 25

Lung and Liver disease

Front 26

Autoimmine hepatitis?

Back 26

Women with severely elevated AST/ALT (1000) with another autoimmune disease, high IgG (autoantibodies -> ANA, ASMA) and interface hepatitis (plasma cell infiltration of portal triad)

Seen with Sogrens, Psoriasis, RA, thyroid disease

Front 27

What are the two genetic mutations in hereditary hemochromatosis?

Back 27

HFE gene-->C282Y/C282Y (accounts for 82-90% of cases)
C282Y/H63D (compound heterozygote);

Only 50% of the homozygotes will become iron overloaded

Front 28

What is the pathogenesis of hereditary hemochromatosis?

Back 28

HFE Gene regulates the absorption of iron from the small intestine; a defective HFE gene fails to down-regulate transferrin and iron continues to be absorbed from small intestine-->organ damage

Front 29

What does hereditary hemochromatosis present with?

Back 29

Liver function abnormalities, weakness, skin hyperpigmentation, Diabetes, impotence, Women present at a later age d/t menstrual blood loss

Front 30

Diagnosis and tx of hereditary hemochromatosis

Back 30

transferrin >45%-->genetic testing-->biopsy-->treat with phlebotomy

Front 31

Wilsons Disease defects

Back 31

Defect in Billiary excretion of copper; and deficiency in Ceruloplasmin (95% have levels <20mg/dl)

Front 32

Wilsons etiology/presentation

Back 32

Younger pts (3-40); Acute liver failure; Neurological symptoms and psychiatric symptoms (deposits in the brain)-movement disorders, drooling, depression, migraines, etc
Kayser-Fleischer Rings

Front 33

Treatment of Wilsons Disease

Back 33

D-penicillamine-binds Cu and induces cupruria
Trientine-binds copper BETTER safety profile
Zinc-Prevents Cu being absorbed in the GI tract

Front 34

Type One Autoimmune Hepatitis

Back 34

ANA (anti-nuclear antibody) and ASMA (anti-smooth muscle antibody); ADULTS; Nucleus

Type 2-->microsome, anti-liver-kidney microsomal antibody

Front 35

Treatment for Autoimmune Hepatitis

Back 35

Prednisone or Azathioprine

Front 36

Chronic, progressive, cholestatic liver disease; Destruction of intrahepatic bile ducts; Autoimmune

Back 36

Primary Biliary Cirrhosis; Mostly women; age 52

Front 37

Lab for PBC?

Back 37

Elevated Antimitochondrial Antibodies (AMA); Elevated IgM; raised Alk Phos, elevaed cholesterol, lymphocytes and mono cells around bile ducts

Target antigen of AMA is Pyruvate Dehydrogenase Complex (PDC-E2); located on the membrane of the biliary epithelial cells; Microscopic Bile Ducts!

Front 38

Sx for PBC?

Back 38

Fatigue and PRUITUS (itch till you bleed)

Osteomalacia, prolonged PT, steatorrhea

Front 39

Tx of PBC?

Back 39

Ursodeoxycholic Acid (a bile acid that is hydroPHILIC)

Front 40

Diseases that cause Low O2 sat/SOB

Back 40

Alpha 1 antitrypsin and Hepatopulmonary syndrome