Ummsm Gi Exam Ii: Week 2/3 Hepatobiliary

Front 1

Pathophysiology of Wilson's disease

Back 1

inadequate copper excretion

Front 2

Wilson disease gene, what is it responsible for?

Back 2

ATP7B, which is responsible for proper Cu excretion into the bile

Front 3

Presentation of Wilson's disease in children

Back 3

highly variable: hepatomegaly, fatty liver, reccurent gallstones

* can be with or without symptoms: fatigue, anorexia, abdominal pain

Front 4

Presentation of Wilson's disease in adults

Back 4

chronic liver disease: splenomegaly, ascites
OR
*** Fulminant hepatic failure:
- low alk. phos, high bili (AP/Bil < 4)
- AST/ALT > 2 (typically low levels <1500, unlike other FHF)
- AKI: hemolysis

Front 5

WIlson disease neurologic presentaiton:

Back 5

- movement disorders (juvenile parkinson's)
- rigid dystonia
- hypophonia (soft voice)
- *Normal IQ*

Front 6

Wilson disease ocular findings on slit lamp exam? What other symptoms are usually present

Back 6

Kayser-Fleischer rings (Cu deposition in Descemet's membrane of the cornea)

- usually present in CNS/psych involvement (>95%)
- absent in 40-60% w/ only hepatic involvement

Front 7

Wilson disease labs for anemia:

Back 7

Coombs' negative hemolytic anemia (b/c not immune hemolysis)

Front 8

Treatment for wilson disease

Back 8

chelator agents

Front 9

HFE-related hereditary hemochromatosis inheritance, usual age of onset, & demographics

Back 9

- AR
- caucasian male, 40-50 y/o

Front 10

Typical presentation of HFE-related hereditary hemochromatosis (list 3)

Back 10

- fatigue
- dark skin
- hepatomegaly

Front 11

Typical presentation of HJV (juvenile) hereditary hemochromatosis (list 3)

Back 11

- impotence/amonorrhea
- cardiomyopathy

Front 12

hereditary hemochromatosis pathophysiology

Back 12

Front 13

hereditary hemochromatosis significantly increases the risk for what other liver conditions?

Back 13

HCC

Front 14

bronze diabetes

Back 14

The classic triad of hemochromatosis is cirrhosis, diabetes mellitus, and skin pigmentation.

Front 15

Dx of HH

Back 15

- elevated transferrin saturation (serum Fe/TIBC) x 100%
- elevated serum ferritin
- if either abnormal → HFE mutation analysis

Front 16

Treatment of HH

Back 16

phlebotomy

Front 17

α1-antitypsin deficiency genetics

Back 17

autosomal codominant

Front 18

α1-AT deficiency w/ 0 protein →

Back 18

COPD but NOT liver problems

Front 19

Pathophysiology of liver disease in α1-AT deficiency

Back 19

abnormally folded proteins accumulate within the liver → fibrosis

Front 20

Replacement therapry w/ purified α1-AT is beneficial....

Back 20

in lung disease ONLY; no benefit in liver disease

Front 21

autoimmune hepatitis sex predominance

Back 21

4:1 female to male

Front 22

Etiology of autoimmune hepatitis

Back 22

+ molecular mimicry: multiple antigens w/ the same or similar epitopes can activate CD4 cells b/c of incomplete specificity of T-cell antigen receptors

Front 23

Clinical spectrum of autoimmune hepatitis

Back 23

Front 24

Auto antibodies seen in AIH

Back 24

usually ANA &/or SMA

Front 25

histopathology of AIH

Back 25

- inface hepatitis
- lymphocytic infiltrate

Front 26

primary treatment modality for AIH

Back 26

immune supression: steroids are first line

Front 27

Primary Biliary Cirrhosis (PBC) pathophysiology

Back 27

breach in immunologic tolerance → aberrant recognition of mitochondrial self antigens → activation of immune/inflammatory response → inflammation & fibrosis of the bile ducts

* PBC is NOT just an auto-immune process, it is a combination of genetic pre-disposition w/ environmental insults

Front 28

Which labs are usually elevated in primary biliary cirrhosis (PBC)?

Back 28

- alk phos (indicative of cholestasis)
AMA: anti-mitochondrial antibody

Front 29

epidemiology of PBC

Back 29

female > 40

Front 30

What is the pathognomonic histopathological presentation of primary biliary cirrhosis?

Back 30

distorted bile duct w/ inflammatory infiltrate

Front 31

Clinical presentation of primary biliary cirrhosis

Back 31

- majority of new cases are asymptomatic
- jaundice is a late event

Front 32

Which autoimmune disease is most commonly associated with primary biliary cirrhosis?

Back 32

Sjogen's/Sicca Syndrome: dry eyes & dry mouth

Front 33

Drug tx for PBC

Back 33

UDCA

Front 34

Primary sclerosing cholangitis (PSC) pathophysiology

Back 34

inflammation, obliteration & fibrosis of intra- & exra-hepatic bile ducts

etiology: unknown

Front 35

Cholangiocraphic findings in primary sclerosing cholangitis (PSC)

Back 35

multifocal bile duct strictures & dilations

Front 36

What is the characteristic (but not pathognomonic) histologic presentation of primary sclerosing cholangitis (PSC)?

Back 36

"onion-skinning" of the bile duct

Front 37

70-80% of the cases of primary sclerosing cholangitis are associated with __________, therefore at the time of diagnosis of PSC all patients should undergo _____________ (diagnostic test)

Back 37

IDB; colonscopy

Front 38

Patiens with PSC have an ↑ risk of developing (2 cancers)

Back 38

cholangiocarcinoma & colon CA

Front 39

Medical treatment for PSC

Back 39

no proven medical treatment; only symptomatic management & surveillance for CRC, GB Ca & CCA

Front 40

Gender predominance PBC vs PSC

Back 40

Front 41

Bile ducts effected in PBC vs PSC

Back 41

PBC: intrahepatic (small)

PSC: inra- & extrahepatic

Front 42

ERCP findings in PBC vs PSC

Back 42

PBC: normal (b/c can't see the the small intrahepatic bile ducts)
PSC: abnormal

Front 43

Increased risk of cholangiocarcinoma: PBC vs PSC

Back 43

PBC: no
PSC: ↑ risk

Front 44

age range of Dx for PBC vs PSC

Back 44

PBC: 30-65

PSC: 4-65 (* effects peds)

Front 45

Which type of hepatitis is transmitted via fecal-oral?

Back 45

Hep A & Hep E

Front 46

Which type of hepatitis leads to a chronic infection?

Back 46

Hep B, C, & D

Front 47

What kind of virus is Hep A? What kind of infection does it cause (chronic/acute?)

Back 47

RNA virus

causes an acute infection (much more serious in adults than in children)

Front 48

Of the patients with Hep A, which age group is most likely to get jaundice?

Back 48

older (> 14 y/o)

Front 49

In an acute infection of Hep A, what would be expected to be elevated?

Back 49

ALT & IgM anti-HAV

Front 50

What does an elevated total anti-HAV with no IgM anti-HAV indicate?

Back 50

likely person that was previously infected; this indicated elevated IgG which is a sign of previous infection

Front 51

How is Hep A transmitted?

Back 51

fecal-oral: very easily transmissible

- close personal contact
- contaminated food & H2O

Front 52

Incubation period for Hep A vs Hep B

Back 52

Hep A: ~ 30 days
Hep B: ~ 60-90 days

Front 53

Hep B modes of transmission

Back 53

- sexual
- parenteral (IVDA)
- perinatal (most common worldwide)

Front 54

Surface antigen vs IgM core antibody in Hep B

Back 54

Surface antigen: present in chronic or acute

IgM core antibody: recent acute hep B infection

Front 55

anti-HBE (E antigen) levels in chronic hep B

Back 55

usually present, although can be absent

Front 56

What antibody elevations would you expect to see in a a person with chronic hep B?

Back 56

- anti-HBs (antibody to surface antigen)
- anti-HBe (antibody to E)
- IgG anti-HBc (antibody to core)

Front 57

What would be the difference in labs in a patients with chronic hep B versus someone who was vaccinated?

Back 57

pt w/ chronic infection:
- anti-HBs (antibody to surface antigen)
- anti-HBe (antibody to E)
- IgG anti-HBc (antibody to core)

vaccinated: anti-HBs ONLY

Front 58

Why can you never completely get rid of Hep B? What is the significance of that?

Back 58

it has cccDNA (covalently closed circular DNA), therefore must use caution with immunosupression as it can cause reactivation of the virus

Front 59

Patients w/ chronic hep b have ↑ risk for...

Back 59

HCC

Front 60

Hep D

Back 60

acute infection that occurs as a co-infection with Hep B (must have Hep B in order to get infected w/ Hep D)

Front 61

What labs are elevated in acute HCV infection?

Back 61

HCV RNA

Front 62

Patients with resolved Hep C will have ↑ _________ .

Back 62

anti-HCV

Front 63

What 2 labs define chronic Hep C infection?

Back 63

presence of anti-HCV ALT elevation

Front 64

Common extrahepatic manifestations of HCV

Back 64

- mixed cryoglobuinemia
- vasculitis

Front 65

Hep E greatest fatality rate is in (which group of people)

Back 65

pregnant women

Front 66

AST: ALT in alcholoic hepatitis

Back 66

AST: ALT >2

Front 67

Major histological feature of alcoholic hepatitis

Back 67

mallory bodies

Front 68

The prevalence of Hep __ is much higher among alcoholics.

Back 68

C

Front 69

Most drug hepatotoxicity affects which population?

Back 69

women > 40

Front 70

antidote for acetaminophen hepatotoxicity

Back 70

n-acetyl cysteine (it promotes glutathione)

Front 71

What is the most common hepatic manifestations of statin drugs?

Back 71

asymptomatic elevations in aminotransferase levels

Front 72

In a patient with unexplained jaundice you should think of.....

Back 72

drug hepatotoxicity

Front 73

NAFL vs NASH

Back 73

NASH (nonalcoholic steatohepatitis) is a subgroup of NAFL (nonalcholic fatty liver) in which steatosis co-exists w/ liver cell injury & inflamation

Front 74

NAFL is most common in which ethnicity?

Back 74

hispanics

Front 75

How does metabolic syndrome contribute to NAFL?

Back 75

there is an imbalance between pro-inflammatory & anti-inflammatory mediators as a result of increased visceral fat

Front 76

What is more severe: microvesicular or macrovesicular steatosis?

Back 76

microvesicular; poor prognosis (usually death)

Front 77

Six F's: Risk Factors for Cholesterol Gallstones

Back 77

Front 78

What is a common cause of black pigmented gall stones? What gives it the black color?

Back 78

hemolytic disorders; ↑ levels of unconjugated bilirubin

Front 79

Brown gallstones have an increased composition of _______. They are usually caused by...

Back 79

fatty acids; bacterial infection causes by bile stasis

Front 80

Murphy's sign

Back 80

P/E finding in acute cholecystitis: right subcostal tenderness w/ pain & respiratory arrest during palpation

Front 81

Diagnostic criteria for acute acalculous cholecystitis

Back 81

thickened gallbladder wall in the absence of ascites & hypoalbuminemia

Front 82

Classic presentation (3 things) of acute ascending cholangitis

Back 82

fever, jaundice & abdominal pain

Front 83

etiology of acute ascending cholangitis

Back 83

bacterial infection

Front 84

What is characteristically seen on imaging of ascending cholangitis?

Back 84

bile duct dilation

Front 85

Presentation of primary sclerosis cholangitis (list 4 things)

Back 85

jaundice, fever, pruritus, & abdominal pain

Front 86

Ultrasound of gallbladder polyps vs stones

Back 86

Polyps do not move or case a shadow like stones

Front 87

What is the most common location of cholangiocarcinoma?

Back 87

bifurcation of bile ducts

Front 88

2 causes of prehepatic portal HTN

Back 88

- portal vein thrombosis
- splenic vein thrombosis

Front 89

Causes of posthepatic portal hypertension

Back 89

- cardiomyopathy, valvular disease, constrictive pericarditis
- budd-chiari syndrome, IVC blockage

Front 90

Variceal bleeding prevention

Back 90

non-selective beta-blockers (propanolol, nadolol, or timolol)

Front 91

Pharmacological treatment for acutely bleeding varices (3 drugs)

Back 91

- somatostatin
- vasopressin
- non-selective beta-blockers

Front 92

most common site of variceal bleeding

Back 92

distal esophagus

Front 93

How does the TIPS procedure work to reduce the portal HTN?

Back 93

a direct connection is formed between the hepatic veins & the portal venous system, thus bypassing the intrahepatic restrictions

Front 94

Serum-ascities albumin gradient (SAAG) in portal HTN vs everything else

Back 94

portal HTN: SAAG > 1.1 (indicates that it is high in protein)
everything else SAAG < 1.1

Front 95

Testing of ascites fluid

Back 95

- cell count (WBC/RBC)
- serumm albumin - ascites gradient (SAAG)
- cultures

Front 96

Medical management of ascites

Back 96

- salt restricted diet
- diuretics

Front 97

primary diagnostic test in spontaneous bacterial peritonitis (SBP)

Back 97

PMN cell count > 250

Front 98

empiric medical therapy for hepatic encephalopathy

Back 98

- lactulose
- Abx
- dietary protein restriction

Front 99

pathophysiology of hepatorenal syndrome

Back 99

- peripheral arterial vasodilation
- markedly increased renal vascular resistance

Front 100

most common benign liver tumor

Back 100

hemangioma

Front 101

epidemiology of hemangiomas (benign liver tumor)

Back 101

women ages 30-50

Front 102

clinical features of hemangiomas

Back 102

mostly small & asymptomatic

Front 103

Hemangiomas on US & contrast CT

Back 103

US: hyperechoic
CT: early arterial peripheral nodular enhancement

Front 104

Fibronodular hyperplasia (benign liver tumor) epidemiology

Back 104

same as hemangiomas: women ages 30-50

Front 105

clinical features of Fibronodular hyperplasia (FNH)

Back 105

- usually asymptomatic
- normal P/E
- imagining: CT may reveal central scar

Front 106

pathognomonic presentation of fibronodular hyperplasia (FNH) on contrast CT

Back 106

central stellate scar

Front 107

Hepatic Adenoma symptoms (anything serious?)

Back 107

Abd pain
*** can have life threatening bleeding ***

Front 108

What is seen on CT of a hepatic adenoma?

Back 108

hyper-intense capsule

Front 109

treatment of hepatic adenomas

Back 109

- stop OCP
- surgical resection if solitary & large

Front 110

Oral contraceptive use must be stopped in which benign hepatic lesion?

Back 110

hepatic adenoma

Front 111

most common liver tumor in childhood

Back 111

hepatoblastoma

Front 112

In an infant > 1 y/o, what lab test is suspicious for hepatoblastoma?

Back 112

↑ AFP (alpha fetal protein)

Front 113

Presentation of gallbladder carcinoma

Back 113

- incidental finding @ cholecystectomy
- RUQ pain found in 80% of pts

Front 114

______________ is the most common site for metastastasis.

Back 114

Liver

Front 115

Major causes of HCC

Back 115

Front 116

2 components for the diagnosis of HCC

Back 116

AFP > 500 + solid liver nodule on imaging

Front 117

Staging system for HCC

Back 117

BCLC staging, NOT TNM

Front 118

definition of acute liver failure

Back 118

coagulation abornomality (INR > 1.5)

AND

any degree of mental alteration in a pt w/o pre-existing cirrhosis & w/ an illness of less than 26 weeks duration

Front 119

3 variables to predict outcome in acute liver failure

Back 119

- degree of encephalopathy
- prothrombin time/INR
- etiology

Front 120

What is a life-threatening neurological complication of acute liver failure aside from encephalopathy?

Back 120

cerebral edema/increased ICP

Front 121

Two common complications of acute liver failure

Back 121

- infection (50-80% of pt w/ develop bacteremia w/in 1st few days after onset of ALF)
- coagulopathy (FFP only given in setting of active hemorrhage)

Front 122

What is the most common indication for liver transplantation in the United States?

Back 122

HCV

Front 123

MELD score uses which 3 lab tests?

Back 123

- INR
- Total Bilirubin
- Creatinine

Front 124

What are the poorest prognostic indicators of a patient requiring liver transplant?

Back 124

↑ INR & ↑ creatinine

Front 125

MELD > ___ = transplant list

Back 125

15

Front 126

What do changes in ALT & AST indicate?

Back 126

hepatocyte damage, they DO NOT measure function

Front 127

What is the primary lab test for cholestasis?

Back 127

alklaline phospatase

Front 128

Causes of ↑ unconjugated bilirubin (list 4)

Back 128

- hemolysis
- resporption hematoma
- gilbert syndrome
- drugs ie. rifampin

Front 129

Causes of ↑ conjugated bilirubin (list 3)

Back 129

- liver disease
- biliary obstruction (most common cause)
- inheritied (Dubin-Johnson, Rotor)

Front 130

What causes aminotransferase elevations > 1000?

Back 130

- acute viral hepatitis
- toxins
- ischemia

Front 131

Common symptoms of primary biliary sclerosic (PBC)

Back 131

- pruritus
- fatigue
- abdominal pain

Front 132

UDCA treatment in PBC vs PSC

Back 132

PBC: reduction in morbidity/mortality

PSC: only improves #s, no improvement in morbidity/mortality

Front 133

Progression of Hep B when acquired in childhood vs adulthood

Back 133

Children --> chronic Hep B

Adults --> resolve

Front 134

What is the only liver disease which can end in HCC without first developing cirrhosis?

Back 134

Hep B: 5-10% of people can go from chronic Hep B --> HCC

Front 135

Gallstones in the biliary ducts can lead to... (name 3 conditions)

Back 135

- obstructive jaundice
- ascending cholangitis
- acute pancreatitis

Front 136

Which type of HCC effects young people without underlying liver disease?

Back 136

fibrolamellar