Hepatobiliary review - the UNC Department of Medicine

Report
HEPATOBILIARY
BOARD REVIEW
Darrell Laudate
6-16-10 AM Report
Approach to Abnormal LFTs
First important to attempt to sort out hepatitis vs
cholestatic liver disease
Hepatitis

Hepatitis typically has elevations of AST/ALT
 Present
typically with fatigue, nausea, mild upper
abdominal pain, and juandice
 Recall the typical culprits of AST/ALT elevations of the
thousands
 Viral,
toxins, shock/ischemia, occasionally AIH
 However, an acute increase in biliary pressue (e.g. from
choledocolithiasis can also cause transient AST elevations as
high as 1000 but returns to normal after 48hrs
 Serum Alk Phos may not be elevated
Cholestatic Diseases




Typically heralded by elevations of Alk Phos
Refers to injury of the microscopic ducts (e.g PBC),
large bile ducts, (e.g pancreatic cancer with CBD
obstruction) or both (PSC)
Infiltrative diseases can also cause elevated Alk
Phos yet near normal serum bilirubin
Any systemic inflammatory process such as an
infection or immune disorder may result in a mixed
pattern
Serum Bilirubin



What is the fraction?
Overproduction of bilirubin (e.g. hemolysis or
hematoma resorption) is associated with ≤ 20%
conjugated bilirubin fraction
Hepatocyte dysfunction or impaired bile flow typically
causes a hyperbilirubinemia with ≥ 50% conjugated
bilirubin fraction
Direct hyperbilirubinemia more common than indirect in
those with jaundice
 Abdominal pain, fever, and/or palpable GB with direct
hyperbilirubinemia suggests a large bile duct obstruction

Bilirubin (continued)


Viral hepatitis risk factors, a total bilirubin > 15mg/dL
and persistent AST/ALT elevations suggest
hepatocellular injury
In patients with acute hepatocellular dysfunction,
improvement in serum bilirubin levels often lags behind
improvement in ALT/AST
Synthetic Liver Function (PT and Alb)

Abnormal PT and Alb levels imply severe
hepatocellular injury
 Note
that PT may also be elevated from Vit K
deficiency (via Abx administration, prolonged fasting,
samll-bowel mucosal disorders (celiac), or severe
cholestasis leading to fat-soluble deficiencies)
 Vitamin K will improve the INR within 2 days with
Vitamin K but will have no effect if due to liver disease
with poor synthetic dysfunction
MKSAP Questions
Question 24


30-year-old woman is evaluated because of an
abnormal serum total bilirubin level detected
when she had a life insurance examination.
Medical history is unremarkable. Her only
medication is an oral contraceptive agent. Physical
examination is normal.
Labs: Hgb 13; MCV 90; Total bilirubin 2.4; Direct
bilirubin 0.2; AST 23; ALT 25; Alk phos 90
Question 24 (continued)

Which of the following is the most appropriate management at this time?
 Discontinue the oral contraceptive agent

Cholestasis due to an oral contraceptive agent will typically cause conjugated
(direct) hyperbilirubinemia and an elevated serum alkaline phosphatase level

Repeat the liver chemistry tests in 3 months

Evaluate for the presence of hemolysis


Schedule abdominal ultrasonography


Patients with hemolysis significant enough to cause unconjugated hyperbilirubinemia
typically low Hgb and abnormal MCV
Ultrasound may be a helpful study for direct hyperbilirubinemia as it is usually
associated with liver disease
No additional diagnostic studies are indicated

An isolated indirect (unconjugated) hyperbilirubinemia in an asymptomatic
patient with a normal Hgb level suggestive of Gilbert's syndrome, no further
work-up indicated.
Gilbert’s Syndrome
 Common,
benign inherited disorder associated with
indirect hyperbilirubinemia (with serum total bili >
3.0mg/dL but direct is 0.3mg/dL)
 Recall
that the Bilirubin will typically rise during illness or
fasting periods
 Presumptive
diagnosis can be made in an otherwise
healthy individual with an isolated indirect
hyperbilirubinemia and a normal Hgb.
Question 32


37yo F with history of hypothyroidism (on
Levothyroxine) presents with 1-week history of
fatigue, jaundice, and slight fever. She traveled to
Mexico 5 months ago and received one dose of
hepatitis A vaccine before her trip. Physical
examination significant for mild jaundice and
hepatomegaly.
Labs: CBC normal; TSH normal; AST 310; ALT
450; Alk phos 180; total bili 2.3
Question 32 (continued)

Which will confirm the diagnosis?
 Anti-mitochondrial antibody


Antinuclear antibody and anti–smooth muscle antibody


Does have travel risk factor but this was 5 months ago, incubation period for Hep A is
typically 2-6 weeks of exposure. Furthermor she also received 1 dose of the Hep A vaccine
before travel, which typically protects people for at least 4 weeks
Serum acetaminophen


Most likely AIH given concomitant autoimmune thyroid disease and abnormal liver test
results. Antinuclear antibody and anti–smooth muscle antibody titers should therefore
be obtained (titers >1:80 for both assays support the diagnosis)
IgM antibody to hepatitis A virus (IgM anti-HAV)


Serologic marker for PBC, a cholestatic disease, thus will have a typically higher Alk Phos
and T bili, lower AST/ALT
Measurement of Acetaminophen level is appropriate for acute hepatitis of uncertain cause
but is typically associated with more significantly elevated AST/ALT values and is typically
not associated with the week-long prodrome noted prior to presentation
Endoscopic retrograde cholangiopancreatography

ERCP is indicated for evaluation of suspected biliary obstruction and could be considered
given the fever, jaundice and acutely elevated ALT/AST values but the absence of pain
makes obstruction very unlikely
Autoimmune Hepatitis





Typically develops in patients 20-40 years old
Females> Males (3.6:1)
1/3rd to 1/2 have concomittant autoimmune disease
Most pts have features of chronic disease but up to
40% will have an acute or fulminant presentation
Fatigue present in 85%; jaundice (46%), anorexia
(30%), myalgias (30%), diarrhea (28%)
Acne, hirsutism, menstrual irregularities, and fever are less
common
 Pruritis and weight loss are uncommon thus alternative
etiologies should be considered

AIH (continued)

Some patients have features of both autoimmune hepatitis and a cholestatic
liver disease







Exam often shows enlarged liver (78%) but otherwise typically normal
despite presence of advanced disease
AST/ALT typically elevated (typically 150->1000 but often < 500)
Serum gamma globulin ≥ 1.5 of upper limit of normal
Mild hyperbilirubinemia (often < 3mg/dL) present in 83%
Elevated AlkP often present but values > 2x normal suggest another
disorder
ANA, Anti-smooth muscle Ab, or antibody to liver/kidney microsome type 1
(anti-LKM1) is present in 87%


Referred to as an overlap syndrome
typically titers ≥1:80 support diagnosis
Biopsy shows Interface hepatitis with portal plasma cell infiltrate.
Treatment of AIH

Prednisone alone or Pred + Azathioprine associated
with remission in 80% at 3 years
Relapse occurs in 50-86%, typically within 6 months of
withdrawal of therapy & associated with an increase in
AST/ALT
 Relapse treatment is same as initial treatment
 Liver transplant reserved for patients who do not respond to
treatment alone



AIH can develop in the transplanted liver but is typically mild
Prognosis excellent for patients with treated AIH and is
same for that of healthy persons matched for age, sex, and
geographic location
Question 133


21yo F brought to ER by her roommate for slurred
speech, tremor, and clumsy gait. Roommate last saw
patient 2 days ago and does not know how long these
changes have been present. Roommate believes the
patient is health and does not take any prescription
medications. However, a decline in her school
performance over the last several months has been
noted. PE shows she is jaundiced, tremulous, and
delirious.
Labs: Hgb 8.2, WBC 6000/µL, plts 250,000/µL
AST 250, ALT 275, AlkP 40, t bili 8.5 (direct 1.5)
Question 133 (continued)

Which of the following studies is most likely to suggest the diagnosis?

Measurement of serum acetaminophen


Measurement of serum ceruplasmin


Helpful in diagnosis of AIH but this diagnosis would be hard to explain her cognitive
deficits and hemolysis
Serologic studies for viral hepatitis


Her chronic progressive decline along with her liver test abnormalities and evidence of
hemolytic anemia (via a disproportionate elevation of the T bili) are highly suggestive
of Wilson’s. A ceruplasmin level <20mg/dL will be supportive of this diagnosis.
Measurement of ANA and serum gamma-globulin


Should always be obtained for evaluation of her AST/ALT but does not explain all of
her features, e.g. prolonged cognitive decline, hemolytic anemia
Should always be done with AST/ALT elevations but presentation and labs studies are
lab studies are not strongly suggestive of viral hepatitis (more likely to have more
significantly elevated AST/ALT; AlkP not likely
Urine Toxicology Screen

Would explain her overt psychosis and hepatitis but again would not explain her
hemolytic anemia
Wilson’s Disease




A condition of aberrant biliary copper excretion that
should be considered in a young patient with abnormal
liver chemistry studies
A low ceruplasmin (esp. < 20mg/dL) is most likely to be
strongly supportive of Wilsons
Hepatic Manifestations range from asymptomatic LFT
elevations to fulminant hepatic failure
Copper deposition in the basal ganglia manifests as
cognitive decline to overt pyschosis or delirium


1/3rd will present with Parkinsonian features
Also associated with cardiomyopathy, endocrine
dysfunction, and Fanconi’s syndrome
Wilson’s Disease (continued)



PE may show the Kayser-Fleischer rings (usually can
only be seen on slit lamp examination)
Labs may show variable AST/ALT elevations but Alk
Phos will typically be lower than normal
A ceruplasmin will typically be < 20mg/dL with
Wilson’s but this test is neither confirmatory nor
diagnostic
Wilson’s Disease (continued)

Wilson’s should be considered in a young patient
with characteristic clinical features and a serum
ceruplasmin
 An
elevated urine copper level (usually >250ug/24h is
also characteristic
 Biopsy will confirm the diagnosis via hepatic copper
concentration
Treatment of Wilson’s

Pencillamine is in the initial therapy of choice, is lifelong



Side effects include neurologic deficits, hypersensitivity
reactions, bone marrow suppression, and autoimmune
disorders
Trientine and zinc acetate are alternative agents
Transplant indicated for those with fulminant disease or
ESLD who do not respond to medical therapy

Significant improvement in neurologic function may occur s/p
transplantation thus severe neurologic dysfunction should not
be a contraindication to transplantation
Question 9

42yo F w/ history of dysmenorrhea (on estrogen/
progesterone) and hypothyroidism (on
levothyroxine) has progressive fatigue without
dyspnea, chest pain, or systemic symptoms. She
sleeps well at night with no features of sleep
apnea. Exam significant for slight but nontender
thyromegaly and xanthomas on extensor surfaces.
Labs: nml CBC & TSH; AST 25, ALT 32, t bili 1.1,
AlkP 278
Question 9 (continued)

In addition to fasting serum lipid profile, which of the following
studies would most likely be helpful in establishing the diagnosis?

Antimitochondrial antibody assay


Serum 25-OH Vitamin D


Although metabolic bone disease is associated with PBC, it vitamin D
deficiency would not explain exam/lab findings
ERCP


80% of PBC pts report fatigue but presence of xanthomas and elevated
AlkP are characteristeic of PBC; Antimitochondrial Antibody titer ≥ 1:40
present in > 90% of PBC pts
Indicated for assessing cholestatic disease that affects large ducts (such as
PSC) but would not be helpful in the diagnosis of PBC
Abdominal U/S

Helpful in detecting bile duct dilatation for those with an elevated AlkP but
would be neither sensitive or specific for diagnosis PBC as U/S may be
normal in PBC
PBC



Chronic, progressive, autoimmune cholestatic liver disease
Occurs predominantly in females (80-90%) between ages
of 40-60 years old
80% of PBC pts report fatigue but presence of xanthomas
and elevated AlkP are characteristeic of PBC





Localized or generalized pruritis frequently develops; often in the
perineal area, or the palmar/plantar surfaces and worsens at
night or in a warm environement
Jaundice or abdominal pain may also develop
However many patients may be asymptomatic on presentation
Other autoimmune diseases are frequently present
Metabolic Bone disease, hypercholesterolemia, and fatsoluble Vitamin deficiencies are common
PBC (continued)

Exam typically include:
 Skin
thickening and hyperpigmentation from repeated
excorations
 Exanthomas, xanthelasma and
 Hepatamegaly
 Advanced disease may have clinical manifestations of
portal hypertension
PBC (continued)

Diagnostic triad associated with PBC includes


Cholestatic liver profile
Positive Antimitochondrial antibody titers




Compatible histologic findings on liver biopsy
AlkP and GGT are usually elevated 10x or more above
normal
Bilirubin increases with disease progression thus is a helpful
prognostic factor


>1:40 titers is serologic hallmark occurring in 90-95% of patients
AMA titers do not correlate severity or prognosis
Biopsy characteristically shows nonsuppurative cholangitis
plus findings ranging from bile duct lesions to cirrhosis
Question 105

42yo M is evaluated after an elevated Alk Phos is
noted during a life insurance exam. Denies pruritis,
abdominal pain, or jaundice. He has loos bowel
movements for many years and occasionally has
rectal bleeding, which he attributes to hemorrhoids.
PE is unremarkable.
Labs show a Hgb of 11.9, MCV 74, AST 45, ALT
52, Alk Phos 620, t bili 2.1 (1.6 direct)
Question 105 (continued)

Which of the following diagnostic studies is most appropriate at this time?

Abdominal U/S


CT scan of the abdomen


PSC most likely diagnosis but this is confirmed with ERCP or MRCP showing a string of
beads” pattern of the biliary tree. His chronic loose stools and rectal bleeding is likely
to due to ulcerative colitis that often accompanies PSC in most patients.
HIDA scan


May show bile duct dilatation but this is a non-specific finding
ERCP


May show bile duct dilatation but this is a non-specific finding
May be helpful for diagnosing acute cholecystitis, which is unlikely given lack of pain
CEA determination

Metastatic colorectal cancer should be considered in a patient with rectal bleeding and
LFT elevations. However, the chronic nature of his altered bowel habits makes cancer
unlikely. Furthermore, CEA is neither specific nor diagnostic for colorectal cancer
PSC



Chronic cholestatic liver disease characterized by
progressive bile duct destruction and may lead to
secondary biliary cirrhosis
3M : 1F, typically occurs between 20-30y
80% of PSC patients have IBD, typically UC
 Conversely

only 5% of UC develop PSC
Also associated with bacterial cholangitis,
pigmented bile stones, steatorrhea, malabsoption
and metabolic bone disease
PSC (continued)

Most commonly presents as pruritis, jaundice, abd pain, and
fatigue




Almost 50% are asymptomatic at initial diagnosis
More advanced disease may have cirrhosis and its associated
complications
Labs fit a cholestatic liver profile with Alk Phos 3-5x normal
and mild hyperbilirubinemia
ERCP or MRCP confirms diagnosis with findings of multifocal
strictures and dilatation of the intra- and extrahepatic bile
ducts

Aka “beads on a string
PSC (continued)

Liver biopsy is usually done for staging rather than
dianosis and may show findings ranging from portal
hepatitis to biliary cirrhosis
 Classic
histologic lesion termed periductal (“onionskin”)
fibrosis is only in 10% of biopsy specimens
PSC (continued)

PSC pts are at risk for developing cholangiocarcinoma with
a lifetime prevalence of 10-30%



Detection at an early stage is difficult despite availability of
CA12-9, CEA, cytologic sampling and advanced imaging
techniques
Also have risk for HCC if cirrhosis is present
Pts with both PSC and UC have a higher risk of colorectal
neoplasia compared to UC alone pts

Should have aggressive surveillance immediately after diagnosis
for both diseases
Treatment of PSC

Management includes
 Assessment
of dominant strictures
 Treatment of superimposed bacterial cholangitis
 Symptomatic therapy

Median survival after diagnosis is ~12 years
 Only
life
transplant improves overall survival and quality of
Question 54

23yo F w/ no PMH presents with an 8-month
history of dyspnea and dry cough. Only
medication is an OCP. PE significant for bilateral
crackles on lung ausculation and mild
hepatomegaly.
Labs: CBC normal; AST 45; ALT 55; Alk phos 430
CXR shows shows mild diffuse pulmonary infiltrates
but normal heart size. PPD is negative. Abdominal
U/S shows mild hepatomegaly without bile duct
dilatation.
Question 54 (continued)

What is the most likely diagnosis?

Amyloid


Causes HM and cholestasis but is usually accompanied by evidence of other
organ involvement suchas nephrotic syndrome or neuropathy. Also is rare in
young pts.
Sarcoid

High serum Alk Phos is commonly associated with infiltrative liver disease and
with presence of pulmonary infiltrates and hepatomegaly. Liver biopsy
showing noncaseating granulomas will confirm the diagnosis of sarcoidosis

Tuberculosis
 Usually presents with a fever and + PPD

Primary biliary cirrhosis
 Disease of middle aged women and generally does not cause pulmonary
findings

OCP induced cholestasis
 Can rarely cause cholestasis but again would not be associated with
pulmonary findings
Question 96

63yo F w/ 3 month history gradually increasing abdominal
distention and fatigue. She has no other symptoms and
medical history is noncontributory. PE shows jaundice, mild
muscle wasting, Xanthelasma spider angiomata,
hepatosplenomegaly and moderate abdominal distension
consistent with ascites.
Labs shows Hgb 12.3, plts 102, AST 53, ALT 47, AlkP123, T
bili 3.2, Alb 2.9, INR 1.3
U/S shows hepatomegaly, coarse echotexture of liver,
patent hepatic/portal vessels, mild splenomegaly, moderate
ascites, and no bile duct dilatation
Paracentesis signficant for 80 PMNs, protein of 1.4g/dL,
Albumin of 0.7g/dL
Question 96 (continued)

Which of the following is the most likely diagnosis?
Key here is the SAAG (2.9-0.7 = 2.2)
 Peritoneal carcinomatosis
 Has a low-gradient-high protein ascitic fluid
 Cirrhosis
 SAAG > 1.1g/DL and fluid protein of < 2.5g/DL is consistent with
siusoidal hypertension from chronic liver disease such as cirrhosis
 Budd-Chiari syndrome
 High-gradient, high protein ascitic fluid
 Dilated Cardiomyopathy
 High-gradient, high protein ascitic fluid
Serum-to-ascites albumin gradient
(aka SAAG)

Accurately identifies the presence of portal hypertension
Use of SAAG & Ascitic Fluid Protein to Determine
cause of Ascites
Ascitic Protein SAAG >1.1
SAAG < 1.1

< 2.5g/dL
Cirrhosis
> 2.5g/dL
RHF, Budd
Chiari
Nephrotic
Syndrome
Malignancy, TB
Patients with heart failure and ascites can narrow their gradient during
diuresis, whereas the SAAG in the setting of cirrhosis remains stable unless
blood pressure or portal pressure decreases significantly.
Question 7

38yo F w/ HTN presents with a 3 month history of
intermittent, moderately severe epigastric pain that is
sometimes associated with nausea and vomitting. The pain
typically begins abruptly, lasts for 30-120 minutes before
spontaneously abating, and sometimes awakens her at night.
May be precipitated by eating. Current Meds include
HCTZ. PE shows mild subjective epigastric tenderness only.
Labs show Hgb 12.1, QBC 10.1, AST 312, ALT 468,
Alk Phos 190, T bili 0.7, Amylase 182
Abd U/S shows several small gallstones but no GB wall
thickening or pericholecystic fluid; negative U/S Murphy’s
sign. CBD is 7mm (normal <6mm)
Question 7 (continued)

Which of the following is the most likely cause of her abdominal pain?

Acute Pancreatitis


Acute Cholecystitis


U/S is not supportive of this diagnosis.
Choledocholithiasis


Unlikely given episodic (vs constant) nature of her pain. Additionally will typically
see an Amylase rise of 2-3 times the upper limit of normal
Typical presentation is with epigastric rather than RUQ pain that is intermittent,
moderate-severe, not associated with N/V and can be nocturnal. LFTs particularly
AST/ALT are almost always abnormal. CBD may be normal to slightly increased.
Concomittant gallstones are present in 90%.
Peptic Ulcer Disease

Typically present with epigatric pain that is relieved with eating
Gallbladder Disease Overview
Chole this, Chole that,
Holy Moley
Cholelithiasis


Affects 20 million Americans but vast majority are asymptomatic
When symptoms do present, it is typically that of biliary pain or
colic
 Once symptoms are present, ~50% will have recurrent symptoms


Therefore Cholecystectomy is indicated for most symptomatic patients
with laprascopic preferred over the open procedure
Patients with symptomatic gallstones have a 1-2% annual risk of
developing complications
 Including acute cholecystitis, choledocholithiasis, Mirizzi’s
syndrome, and cholecystoenteric fistula
Acute Cholecystitis



Most common complication of cholelithiasis
Due to impaction of a stone within the cystic duct
that subsequently becomes distended and GB may
become inflamed
Secondary bacterial infection of bile/GB occurs in
50% of acute cholecystitis patients
 These
patients will have fever and biliary pain that
persists for more than 6 hours

Murphy’s sign is relatively specific on PE
Acute Cholecystitis (continued)


Abd U/S may show the stone but also frequently
shows a thickened gallbladder wall and
pericholecystic fluid
If findings are uncertain, a HIDA scan may fail to
visualize the GB and confirms the suspicion of cystic
duct obstruction
Acute Cholecystitis (continued)


Treatment includes IVF and antibiotics followed by
lap chole
Patients with repeated episodes of acute
cholecystitis characterized by a shrunken
gallbladder containing stones or sludge
 Therefore
paitents with more than one episode of acute
cholecystitis require cholecystectomy (lap vs open)
Choledocholithiasis

~5-19% of patients with cholelithiasis have concomittant
CBD stones



These are stones that have migrated from the GB or formed de
novo within the CBD
Also often asymptomatic but may cause epigastric/RUQ pain
(that radiates to the back), biliary pancreatitis, or life-threatening
cholangitis
Should be suspected in a patient with cholelithiasis,
development of abnormal LFTs (typically AST/ALT that can
mimic that of hepatitis) and dilatation of the CBD on
imaging

Rarely the stone may be identified as well
Choledocholithiasis (continued)





U/S while very sensitive in detecting cholelithiasis is only 3050% sensitive in detecting CBD stones but can be suggested
by CBD dilatation
Helical CT scan is more sensitive (80%)
EUS very senstive (90%)’
MRCP is less sensitive in detecting small CBD stones
ERCP is sensitive and allow for stone extraction at the same
time. It is therefore the preferred test when cholethiasis is
suspected

Also indicated for patients with acute cholangitis and prior to lap
cholecystectomy when choledocholithiasis is highly suspected on
imaging and liver chemistry studies.
Management of Choledocholithiasis

Dependent on comorbid conditions and availability of
experts (laproscopic, endoscopic, and intervential radiology)



ERCP indication for those with cholangitis or for those with
pancreatitis complicated by cholangitis
 Contraindicated in patients with preveious enteric reconstruction
(e.g Billroth II, Roux-en-Y), complex stones > 1cm in diameter, or a
biliary stricture
Laproscopic transcystic bile duct exploration also effective in
detection and removal of CBD in 90% if expertise is available
Laproscopic choledochotomy with stone extraction +/- T-tube
placement should be consider if transcystic bile duct exploration
is unsuccessful
Choledocholithiasis & Pancreatitis



Most common cause of pancreatitis worldwide
Most patients with mild pancreatitis associated with
Choledocholithiasis will pass the stone spontaneously
These patients should usually undergo cholecystectomy
prior to hospital discharge to prevent further episodes of
pancreatitis.

ERCP with sphincterotomy but without cholecystectomy may be
suitable for the elderly or high risk patients and can significantly
reduce the risk of recurrent pancreatitis
Cholangitis

Associated with biliary obstruction with subsequent
suppurative infection within the biliary tree


Charcot’s triad (pain, fever, and jaundice) occurs 50-100%
of patients with cholangitis


Obstruction most often due to gallstones with ~6-9% of patients
with gallstone disease developing acute cholangitis
Hypotension and AMS occur in ~14%
 All 5 are called Reynold’s pentad
Mortality approaches 100% unless emergent bile duct
decompression is performed
Cholangitis (continued)


Serum T bili is usually > 2mg/dL but may be normal in early
cholangitis
Bacteremia occurs in 21-83%




Bile cultures grow bacteria in > 80%
Aerobic and anaerobic GN bacilli and enterococci are most
commonly found
CT scan or U/S can help differentiate cholecystitis from
cholangitis as well as detect a hepatic abscess or biliary
obstruction
EUS may also be used to exclude bile duct stones if
diagnosis of cholangitis is uncertain
Treatment of Cholangitis

Immediate IV Abx with empiric coverage for
enteroccoci
 FQ
preferred given their ability to enter an obstructed
biliary system

ERCP +/- sphincterotomy or stent placement is
essential to remove the stone or bypass the
obstruction
Question 3

51y F w/ history of well controlled DM2 presents
with acute onset of moderately severe, constant
upper abdominal pain associated with nausea and
vomiting. Current medications include an oral
hypoglycemic agent, a statin, and low dose ASA.
PE shows she is obese but afebrile and there is
moderate upper abdominal pain without rebound
Labs show a T bili 0.8, AST 180, ALT 285, Alk Phos
152, Amylase 1010, Lipase 950
Question 3 (continued)
Symptomatic treatment for pancreatitis is begun
with IVF and pain management. 12 hours later, she
has minimal symptoms.
Repeat Labs show a T bili 0.9, AST 82, ALT 100, Alk
Phos 130, Amylase 580, Lipase 410
Question 3 (continued)

Which of the following is the most appropriate next step in her management?

Abdominal U/S


Cholescintigraphy (HIDA scan)


May show cystic duct obstruction (indicative of chronic cholecystistis but will not show
gallstones
ERCP


Has classic presentation for acute gallstone pancreatitis with markedly elevated LFTs
and pancreatic enzymes that rapidly return to normal. Abd U/S is required to
exclude cholelithiasis as CT scan may not detect gallstones or sludge.
Indicated if LFTs become significantly abnormal, particularly if jaundice develops
and U/S shows ductal dilatation
Laproscopic Cholecystectomy

Although relapse rate for gallstone pancreatitis is high, lap chole should be
performed before hospital d/c but should not be performed until diagnostic studies
are done
Question 17

78yo M w/ history of dementia, HTN, DM2 presents to the
ED by family members for concern of increasing somnolence
and “not acting normal” for several hours. Current meds
include HCTZ and pioglitazone. PE shows he is older than
his stated age, temperature is 38.3C, pulse 100, BP
110/82. Mild jaundice is present; he is oriented to person
and place but not year.
Labs shows Hgb 12.8, WBC 18.6, Cr 1.2, AST 186, ALT
230, Alk Phos 260, T bili 4.1, Alb 3.4
Alb U/S shows normal liver architecture, CBD of 9mm
(normal <6 mm), multiple gallstones, and no evidence of
cholecystitis. A CXR shows emphysema.
Question 17 (continued)

In addition to broad spectrum antibiotics, which of the following is most appropriate at
this time?

CT scan of the abdomen


Biliary scintigraphy (HIDA scan)


May confirm the presence of chornic cholecystitis or rarely show ductal obstruction
(via lack of contrast in duodenum) but will not show CBD stones
MRCP


CT scan may identify alternative abnormalities with similary features and may be
more sensitive than U/S for detecting CBD stones but the high likelihood of
choledocholithiasis already present means that performing this test will delay what
he really needs.
May identify the stones but has no therapeutic value
ERCP

Severe cholangitis is evident by his fever, AMS, and juandice. LFTs show bile duct
obstruction and U/S shows gallstones and a minimally dilated CBD. Even with IV
Abx, mortality is high unless ductal decompression is performed.
Question 44

68yo F presents with 2 day history of RUQ pain, lowgrade fever, and nausea. PE shows RUQ tenderness
and possible fullness.
Labs show WBC 12.9, AST 35, ALT 50, Alk Phos 148, T
bili 0.7, Alb 3.9
Abd U/S shows a slightly dilated GB, markedly
thickened GB wall w/ a small amount of pericholecystic
fluid and multiple gallstones. Positive U/S Murphy’s
sign is present but no CBD or pancreatic abnormalities
are present.
Question 44 (continued)

In addition to requesting a surgical consultation, which of the following is most
appropriate at this time?

Pain meds and broad spectrum antibiotics


Biliary scintigraphy (HIDA scan)


Both sensitive and specific for diagnosis cholecystitis but would not add any more
information already obtained with U/S
ERCP


Acute cholecystitis is evident along with U/S findings that are highly specific for this
diagnosis. Initial management should be IVF, IV ABx, and surgery c/s for elective
cholecystectomy
Despite the mild LFT abnormalities, ERCP is not indicated given the lack of bile duct
dilatation that would suggest choledocholithiasis
Percutaneous Cholecystostomy

Appropriate for draining a markedly dilated GB in a patient who cannot undergo
cholecystectomy
Question 75

26yo M w/ AIDS has 2 month history of increasingly severe
epigastrium/RUQ abdominal pain that is variably precipitated by
eating and is not associated with N/V. However, the pain is severe
enough such that he cannot work. ROS significant for low-grade
fever, loose stools, and weight loss of 4.5kg. Prior trial of HAART
failed due to nonadherence; last CD4 count was 22. PE shows
obvious signs of weight loss, thrush, a scaphoid but soft abdomen
with mild RUQ pain but no HSM.
Labs show WBC of 3.1, repeat CD4 19, AST 62, ALT 90, Alk Phos
410, T bili 0.8, Alb 2.8
Abd U/S shows slight intra- and extrahepatic bile duct dilatation
with mural wall thickening; GB is slightly distended and has a
thickened wall but no evidence of cholecystitis.
Question 75 (continued)

In addition to counseling about the need for HAART, which of the following is most
appropriate at this time?

Biliary scintigraphy (HIDA scan)


CT scan of the abdomen


Will confirm biliary tract obstruction and perhaps identify other intra-abdominal
abnormalities but his presentation and studies are suggestive of AIDS
cholangiopathy for which CT scan has no therapeutic value
Colonoscopy


May confirm nonfunctioning GB but will not detect any findings already noted on
U/S
Evaluation of loose stools is appropriate but routine non-invasive stool studies should be done
before colonoscopy is done
ERCP

AIDS cholangiopathy is evident by his upper abdominal pain and obstructive liver
injury pattern. ERCP will confirm the diagnosis but given the likely extrahepatic duct
obstruction, ERCP with sphincterotomy may improve drainage and relieve pain
caused by ampullary stenosis
AIDS cholangiopathy






Most often affects paitents who have HIV infection with a CD4 count <
200.
Sclerosing cholangiopathy, papillary stenosis, cholecystitis, extrahepatic
biliary strictures and bile duct dilatation may occur together or
independently in these patients
Associated with infections due to Cryptosporidium, Microsporidium,
MAC, CMV
May present with cholangitis but will most commonly present with RUQ
pain
Almost all have abnormal LFTs with a predominantly elevated Alk Phos
Most patients with AIDS cholangiopathy with papillary stenosis have
symptomatic improvement after ERCP with sphincterotomy
Question 95

78yo F w/ history of CHF has 2 week history of
jaundice, loose stools, dark urine, and marked pruritis.
Current medications are atorvastatin, HCTZ, and ACEI.
PE discloses jaundice, mild temporal wasting, and
fullness in the RUQ.
Labs show WBC of 8.6, plts 180, AST 99, ALT 140, Alk
Phos 520, T bili 16.2, Alb 3.1
U/S shows marked intrahepatic bile duct dilatation,
dilated gall bladder, and normal distal CBD.
CT scan confirms these findings and shows a normal
pancreas.
Question 93

Which of the following should be done next?

EUS


MRCP


May help localize the level of obstruction but this has already been done by
U/S; MRCP cannot provide therapy either
Percutaneous transhepatic cholangiography


May identify a bile duct tumor, but this patient will require palliative therapy,
which this procedure cannot provide
Is an alternative to ERCP if ERCP is not technically possible or if local
expertise for PTC is lacking
ERCP

New onset of obstructive jaundice in an elderly person is most often due to
pancreatic or biliary tract carcinoma. U/S shows the obstruction in the CBD
and pancreas is well-visualized and unremarkable (thus pancreatic carcinoma
is unlikely). Since she is symptomatic, ERCP is preferable because it can
confirm the diagnosis and provide therapy.
Cholangiocarcinoma



Rare tumor (incidence of 1/100,000 in US)
Typically arises between the ages of 50-70 years
60-80% arise near porta hepatis (aka Katskin’s
tumor
 20%
are located in the distal bile duct
 5% are intrahepatic
Cholangiocarcinoma (continued)

~90% will have obstructive jaundice


CA19-9 and CEA may be elevated but specificity is poor




Advanced disease may also have HM or distended palpable GB
(Courvoisier’s sign) if a distal obstruction is present
CEA cut-off of > 100U/ML increases the diagnostic value
CT scans or MRCP may suggest the diagnosis and define the
level of obstruction
ERCP with brushings or biopsy may be diagnotic
EUS with biopsy may be helpful in diagnosising a distal
tumor
Treatment of Cholangiocarcinoma







Hepatic resection may be effective for an intrahepatic lesions
35% of patients with perihilar and ductal tumors can be treated with
resection and subsequent Roux-en-Y hepaticojejunostomy
Median survial for perihilar tumors is 12-24 months
Radiation therapy provides some improvement in survival rates when
combined with other therapeutic modalities
Endoscopic or percutaneous biliary stent placement can be palliative
Photodynamic therapy may offer porlonged palliation and survival
compared with stent alone
Pre- or postoperative chemotherapy does not improve survival or
quality of life
Summary of High yield Hep topics
NAFLD
 EtOH disease
 Viral Hepatitis


Including the Hepatitis B
window
Autoimmune Hepatitis
 PBC
 PSC
 Variceal Management
 Ascites and SAAG
evaluation

Hepatic Encephalopathy
 HRS
 HPS and other exceptions
to transplant
 HCC
 TIPS
 Pregnancy & Liver disease



Cholestasis of Liver disease
Genetic Liver Disease

Wilson’s, Hemochromatosis
References



MKSAP
Uptodate
Sid Barrett’s Hepatology
Board Review 6/9/2010
And when in doubt for your
boards, ask yourself:
“What would Clint Eastwood do?”

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