Non-infectious hepatobiliary disease

Question 1

What are the three general patterns of liver disease?

Answer 1

Hepatocellular pattern
Hyperbilirubinemia
Cholestatic pattern

Question 2

What are the three components of LFTs?

Answer 2

AST/ALT
Alk phos
GGT

Question 3

What are the two tests that test the synthetic function of the liver?

Answer 3

INR

Albumin

Question 4

35 yo man with arthralgias, myalgias, and subsequent jaundice = ?

Answer 4

Viral hepatitis

Question 5

40 yo female with jaundice and severe, sudden RUQ pain and shaking chills = ?

Answer 5

Cholestatic disease

Question 6

Palmar erythema can be seen in what?

Answer 6

Advanced cirrhosis

Question 7

Ascites with jaundice usually indicates what?

Answer 7

Cirrhosis or malignancy with peritoneal spread

Question 8

Virchow’s node or sister mary joseph nodule usually suggests what?

Answer 8

Metastatic malignancy

Question 9

Liver that is TTP is seen in what?

Answer 9

EtOH liver

Hepatitis

Question 10

Grossly enlarged and nodular liver suggests what?

Answer 10

malignancy

Question 11

Hepatocellular disease is usually caused by what?

Answer 11

Alcohol

Hemachromatosis

Question 12

What is the AST:ALT ratio for EtOH liver?

Answer 12

2:1

Question 13

What is the cholestatic pattern of liver disease?

Answer 13

Elevated Alk Phos

Question 14

Which gender is more susceptible to cirrhosis?

Answer 14

Women

Question 15

What are the histological changes seen in alcoholic liver disease?

Answer 15

Macrovesicular fat accumulation

PMN infiltration

Question 16

GGT is usually elevated by what amount in alcoholic liver disease?

Answer 16

x2

Question 17

AST more than 300 indicates what?

Answer 17

Hep C on top of alcoholic cirrhosis

Question 18

What is the cause of macrocytosis in liver disease?

Answer 18

folate/B12 deficiency, or myelotoxicity of EtOH

Question 19

What is the cause of thrombocytopenia in alcoholic fatty liver?

Answer 19

Bone marrow suppression, splenic sequestration d/t portal HTN

Question 20

What is fetor hepaticus?

Answer 20

Thiols pass through the lung from a failing liver, causing horrible breath

Question 21

What are the two outcomes of alcoholic fatty liver?

Answer 21

alcoholic hepatitis or cirrhosis

Question 22

What is the treatment for etoh liver disease?

Answer 22

abstinence and liver transplant

Question 23

What is the cause of ascites in cirrhosis?

Answer 23

Portal HTN causing vasodilatation in the splanchnic system, causing an increase in the renin-angiotensin system, causing water retention

Question 24

What dietary restriction should be undertaken with ascites?

Answer 24

NaCl restriction

Question 25

What drug should be avoided in pts with cirrhotic liver and ascites? Why?

Answer 25

NSAIDs Will shut down kidneys d/t need for prostaglandin

Question 26

What is the serum ascites:albumin ratio in portal HTN?

Answer 26

>1.1

Question 27

What is the goal of treating ascitic fluid?

Answer 27

Reduce w/o intravascular volume depletion

Question 28

What are the two drugs that are used to treat alcoholic liver disease with ascites?

Answer 28

Spironolactone and furosemide

Question 29

What is a TIPS used in treating ascites?

Answer 29

Transjugular intrahepatic portosystemic shunt

Question 30

What happens to the spleen in alcoholic cirrhosis?

Answer 30

Splenomegaly

Question 31

What are the complications with alcoholic liver disease?

Answer 31

Hepatic encephalopathy

Question 32

What are the precipitating causes of hepatic encephalopathy?

Answer 32

``` Hypovolemia GIB Hypoxia Infx hypoglycemia ```

Question 33

Where does the ammonia come from in hepatic encephalopathy?

Answer 33

GI bacteria produce it , but liver cannot handle it

Question 34

What is the treatment for hepatic encephalopathy? How does this work?

Answer 34

Lactulose changes the pH of the intestines, to kill of bacteria

Question 35

What is spontaneous bacterial peritonitis (SBP) seen in alcoholic liver disease? What two bacteria usually cause this?

Answer 35

Ascitic fluid infection w/o a surgically treatable source--usually Klebsiella or E.Coli

Question 36

Ascitic fluid with PMN over what number indicate the need for immediate abx? What else should be done?

Answer 36

250 cells/mm | Paracentesis

Question 37

What protein level in ascitic fluid indicates high risk for the development of SBP?

Answer 37

Less than 1 g per dL

Question 38

What is the abx of choice for spontaneous bacterial peritonitis?

Answer 38

Cephalosporin

Question 39

What is the treatment for varices?

Answer 39

Band the varices | Beta-blockers (propanolol)

Question 40

What is the treatment for variceal rupture?

Answer 40

stabilize them Protect the airway Treat the bleed

Question 41

What is the cause of hepatorenal failure?

Answer 41

portal HTN reduces blood flow to the kidney

Question 42

What is the discriminant function calculation?

Answer 42

4.6[PTT - PT] + (serum bili)

Question 43

Over what score for the discriminant function indicates the need for treatment with prednisolone?

Answer 43

32

Question 44

What causes the BUN to increase in alcoholic pts?

Answer 44

Increased reabsorption of nitrogen

Question 45

What happens to alk phos in alcoholic cirrhosis?

Answer 45

Mildly elevated

Question 46

What is the skin pigmentation with hemochromatosis? What cancer are these pts predisposed to?

Answer 46

Gray | hepatocellular CA

Question 47

What is the pathophysiology with hemochromatosis?

Answer 47

Increased intestinal Fe absorption d/t genetic defect in hepcidin

Question 48

What are the ssx of hemochromatosis (4)

Answer 48

Hepatomegaly Gray hyperpigmentation Arthropathy Cardiac involvement

Question 49

What are the joints that are commonly affected in hemochromatosis?

Answer 49

MCPs

Question 50

What are the labs used to diagnose hemochromatosis?

Answer 50

High serum Fe, ferritin, and transferrin

Question 51

Transferrin saturation greater than what percent is seen in hemochromatosis?

Answer 51

45%

Question 52

What is the treatment for hemochromatosis?

Answer 52

Phlebotomy

Question 53

Symptomatic hemochromatosis is how many times more symptomatic in men than women?

Answer 53

10x

Question 54

What are the histological characteristics of NAFLD?

Answer 54

Similar to EtOH liver, but without h/o alcohol

Question 55

What is the LFT enzymatic ratio with NAFLD?

Answer 55

ALT >AST (opposite of alcoholic liver disease)

Question 56

What is NAFLD associated with?

Answer 56

DM Fat Metabolic syndrome HTN

Question 57

How do you diagnose NAFLD?

Answer 57

Fatty infiltration on imaging Liver biopsy Stigma of chronic liver disease

Question 58

**What is the treatment for NAFLD?**

Answer 58

Weight loss Control DM Avoid EtOH

Question 59

What is type I autoimmune hepatitis?

Answer 59

Elevated transaminases with absence of other causes, and associated with other autoimmune disease

Question 60

What is the lab that is very useful for diagnosing autoimmune hepatitis?

Answer 60

Serum protein electrophoresis (more than 80-% of pts have hypergammaglobulinemia)

Question 61

What are the Abx seen in autoimmune hepatitis? (5)

Answer 61

``` ANA Anti-dsDNA **SMA** ANCA anti-actin ```

Question 62

How do you confirm the diagnosis of autoimmune hepatitis?

Answer 62

Liver biopsy

Question 63

What is the treatment for autoimmune hepatitis? Is there a cure?

Answer 63

Prednisone and azathioprine No cure, but very good prognosis

Question 64

What is the cause of Wilson's disease?

Answer 64

Impaired excretion of Cu into bile and build up

Question 65

What is the genetic cause of Wilson's disease?

Answer 65

AR

Question 66

What is the cause of arthritis seen in Wilson's disease?

Answer 66

Chondrocalcinosis

Question 67

When are pts diagnosed with Wilson's disease, usually?

Answer 67

5-25 yo

Question 68

What are the ceruloplasmin levels in Wilson's disease?

Answer 68

Low

Question 69

How do you confirm the diagnosis of Wilson's disease?

Answer 69

Liver biopsy

Question 70

What is the treatment for Wilson's disease?

Answer 70

Chelation with penicillamine or trientine/Zn salts

Question 71

Is there a cure for Wilson's disease?

Answer 71

yes--liver transplant

Question 72

What is the pathophysiology of Alpha-1-antitrypsin deficiency?

Answer 72

Pathologic polymerization of variant AAT molecules that accumulate in hepatocytes

Question 73

How do you diagnose alpha-1-antitrypsin deficiency?

Answer 73

Direct serum measurement of APR | Genotype testing

Question 74

What is the treatment for alpha-1-antitrypsin deficiency?

Answer 74

Liver transplant

Question 75

What are the two causes of conjugated bili elevation?

Answer 75

Decreased excretion | Backward leakage

Question 76

In whom is Gilbert syndrome common?

Answer 76

Young, white males

Question 77

What are the bilirubin findings with Gilbert syndrome?

Answer 77

Unconjugated is less than 6 mg/dL, but high

Question 78

What are the 4 sources of Alk phos in the body? How do you determine if it is from the liver?

Answer 78

Liver Bone Prego (baby) Gut GGT will be elevated if it is from the liver.

Question 79

AMA ab is diagnostic for what biliary disease?

Answer 79

Primary biliary cirrhosis

Question 80

What is intrahepatic cholestasis? What diseases is this associated with?

Answer 80

not dilated bile duct, but slow bile movement Primary sclerosing cholangitis Primary biliary cirrhosis

Question 81

What is primary sclerosing cholangitis?

Answer 81

Progressive disease with inflammaiton, fibrosis, and sclerosis of intra and extra hepatic bile ducts

Question 82

What disease is primary sclerosing cholangitis associated with?

Answer 82

UC

Question 83

What cancer can primary sclerosing cholangitis are susceptible to?

Answer 83

Cholangiocarcinoma

Question 84

What ab is elevated in Primary sclerosis? Which is NOT elevated?

Answer 84

``` Elevated = pANCA NOT = AMA ```

Question 85

What mineral is element is elevated in the liver of patients with primary sclerosing cholangitis?

Answer 85

Cu

Question 86

Beaded appearance to the bile duct = what disease?

Answer 86

primary sclerosing cholangitis

Question 87

What are the things that are needed to diagnose Gilbert syndrome?

Answer 87

Unconjugated hyperbilirubinemia | + no evidence of hemolysis

Question 88

What is the characteristic finding of a liver biopsy with primary sclerosing cholangitis?

Answer 88

Onion skin appearance

Question 89

In whom is primary biliary cirrhosis common?

Answer 89

Middle aged women

Question 90

What are the diagnostic labs for primary biliary cirrhosis?

Answer 90

Elevated alk phos | AMA +

Question 91

Does the AMA antibody found in primary biliary cirrhosis correlate with the disease severity?

Answer 91

No