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Id = 863
Question 1 Of 39
Division : Hepatology
Item Review
A 58-year-old Russian lady, who is known to have chronic pancreatitis, comes
into your office for increasing pain, during the past month. The pain is dull,
boring, located in the mid epigastrium, and radiating to the back. She is not able
to work because of it. The pain is relieved by hydrocodone. She is also taking
pancreatic enzymes and insulin for her diabetes mellitus. She is not taking any
other drugs or alcohol, at present. On examination, she is weak and distressed.
Her vitals are normal and her laboratory report shows normal glucose, amylase,
lipase, WBC count, hemoglobin, and liver function tests. A CT scan shows
pancreatic calcifications with a stone impacted in the main pancreatic duct with
marked ductal dilatation. What would be the most appropriate next intervention in
this patient?
A. Increase her dose of hydrocodone.
B. Perform a partial pancreatectomy.
Perform an endoscopic retrograde cholangiopancreatography with
C.
removal of the stone and stent insertion.
D. Increase her dose of pancreatic enzymes.
E. Celiac plexus nerve block.
Explanation:
Chronic pancreatitis is a serious disease and often leads to severe disability.
Surgical interventions in the case of chronic pancreatitis are indicated in case of
chronic pseudocyst, debilitating pain or to treat other complications. This patient
has severe debilitating pain affecting her work and daily life. She is an ideal
candidate for ERCP and stone removal with stent insertion, which would relieve
the pain in 80% of such patients. (Choice C)
Choice A: Patient is already on hydrocodone and you have just found a treatable
cause for her pain. Increasing the dose of hydrocodone would only give
symptomatic relief and would not address the stone impaction.
Choice B: Partial or complete pancreatectomy is a last resort for severe and
advanced cases and it has high rates of pancreatic insufficiency and diabetes.
Choice C: Pancreatic enzymes have a role in relieving pain in chronic
pancreatitis; they work by inhibiting the food induced feedback loops of
pancreatic enzymes secretion. Their efficacy is variable and in the case under
discussion we should treat the underlying cause.
Choice E: Celiac plexus nerve block can be used to relieve pain in chronic
pancreatitis patients with non-dilated ducts.
Educational Objective:
Question 2 Of 39
Division : Hepatology
Item Review
A 35-year-old male is found to be positive for anti HCV during a screening 1 year
ago. Thereafter, the AST and ALT levels have been within the normal range over
1 year, on 6 occasions. The patient has no complaints and on examination, there
is no hepatomegaly, scleral icterus or cirrhotic stigmata. His PCR for HCV RNA is
still positive. What would be the most appropriate step in management of this
patient?
A. No treatment at this time, and follow up with yearly liver function tests.
B. Assure the patient that he does not have hepatitis C anymore.
C. Start antiviral treatment for hepatitis C.
D. Perform a liver biopsy.
E. Schedule the patient for liver transplantation.
Explanation:
Though this patient is still infected with HCV, he does not need any treatment
other than yearly follow-up to look for abnormal liver enzymes. Studies have
shown that patients with chronic hepatitis C who show persistently normal liver
enzymes on multiple occasions have minimal histological abnormalities and so
need not be treated (Choice A).
Choice B: In spite of repeatedly normal liver enzymes this patient is not free
from HCV as evidenced by positive PCR for HCV RNA.
Choice C: Anti viral treatment in patients with normal aminotransferases can
lead to persistently elevated ALT in about 20% of patients. Also the natural
history of chronic hepatitis C with normal aminotransferase level is relatively
benign and in this case antiviral treatment can lead to liver injury.
Choice D: Liver biopsy in chronic hepatitis C patients with persistently normal
aminotransferase shows minimal liver damage and so liver biopsy is not
indicated unless more severe liver injury is suspected.
Choice E: Liver transplantation is not indicated unless there are signs of
progressive disease and/or decompensated cirrhosis.
Educational Objective:
Studies have shown that patients with chronic hepatitis C who show persistently
normal liver enzymes on multiple occasions have minimal histological
abnormalities, therefore they do not need to be treated with interferon or antiviral
Question 3 Of 39
Division : Hepatology
Item Review
A 32-year-old pregnant female, at 30 weeks of gestation, is seen in your office
because of intense pruritus. Her laboratory tests show a total bilirubin 3.0 mg/dl;
Alkaline phosphatase 200 IU/L; AST 120 IU/L, ALT 110 IU/L; PT 12 seconds;
Platelet count 100,000/cmm; Hb 12.3 mg/dl, and markedly elevated serum bile
acids. Her viral serologies are negative. On examination, she has no edema and
her BP is 130/84 mm Hg. What is the most likely diagnosis in this patient?
A. Normal physiological changes in pregnancy.
B. Intrahepatic cholestasis of pregnancy.
C. Acute fatty liver of pregnancy.
D. Primary sclerosis cholangitis.
E. Primary biliary cirrhosis.
Explanation:
Intrahepatic cholestasis of pregnancy is a functional disorder of bile formation,
characterized by intense pruritus. Laboratory values show variable elevation in
bilirubin, alkaline phosphatase, GGT, mild elevation of amino transferase (< 200
IU/L) and markedly elevated serum bile acid levels. Elevation of serum total bile
acid concentrations may be the first or only laboratory abnormality. Serum cholic
acid increases more than chenodeoxycholic acid, resulting in a marked elevation
of the cholic/chenodeoxycholic acid ratio. Unlike most other cholestatic diseases
GGT levels are normal or modestly elevated. It resolves on its own and has no
hepatic sequelae. Hepatic failure, hemolysis, thrombocytopenia, and DIC are not
the manifestations of intra hepatic cholestasis. Treatment options include
cholestyramine with or without phenobarbital or ursodeoxycholate. Fetal
monitoring is mandatory and early delivery may be needed.
Choice A: Normal physiological changes in liver enzymes in pregnancy include
mild elevation of alkaline phosphatase due to placental secretion but normal
bilirubin, aminotransferase, and GGT.
Choice C: Acute fatty liver of pregnancy is a complication of the third trimester of
pregnancy characterized by severe nausea, malaise, headache, abdominal pain,
and is commonly seen in association of pre-eclampsia. It can also cause a
prolonged PT with moderate elevation of the transaminase levels and
histologically is characterized by microvesicular fat deposition in the hepatocytes.
Choice D: Primary sclerosing cholangitis is commonly seen in association with
ulcerative colitis and presents with jaundice, pruritus, and RUQ pain.
Choice E: Primary biliary cirrhosis is characterized by markedly elevated alkaline
phosphatase and cholesterol levels, as well as intense pruritus.
Educational Objective:
Jaundice in the third trimester of pregnancy should be evaluated for hepatic
disorders specific for pregnancy. Marked pruritus and elevation of bile acids
should make you think about the diagnosis of Intrahepatic cholestasis of
pregnancy.
47% of people answered this question correctly
Test #24 Q.Id = 1668
Question 4 Of 39
Item Review
Division : Hepatology
A. Pancreatitis
B. Liver abscess
C. Cancer
D. Bowel obstruction
E. Constipation
Explanation:
The above abdominal x-ray shows a classic finding of a porcelain gall bladder.
The shadow of the gall bladder is distinctly outlined by a line of calcium. The gall
bladder appears large and dilated. Calcium salts may be secreted into the lumen
of the gall bladder to produce calcium precipitation and diffuse hazy opacification
of bile. Occasionally the calcium salt deposits into the wall of a chronically
inflamed gall bladder and produce what is called porcelain gall bladder. Porcelain
gall bladder is frequently diagnosed on a routine abdominal x ray.
Cholecystectomy is advised in all patients with porcelain gall bladder since in a
high percentage of cases, this finding appears to be associated with the
development of gall bladder carcinoma (Option C).
(Option A): Pancreatitis is not a complication of porcelain gall bladder. Causes of
pancreatitis include alcohol, gall bladder stone, trauma, drugs like thiazides and
azathioprine, hypertriglyceridemia, etc. Symptoms may vary from mild to severe
abdominal pain. Physical exam may reveal tachycardia, hypotension, basilar
rales, and discoloration at the umbilicus or the flanks. Diagnosis is by elevation of
serum amylase or lipase.
(Option B): Liver abscess is not a complication of porcelain gall bladder. Majority
of liver abscess are amebic but may be bacterial. Causes of liver abscess
include portal vein bacteremia, systemic bacteremia, ascending cholangitis or
trauma. Ultrasound or CT is usually diagnostic. Amebic abscess require antibiotic
therapy whereas bacterial abscess require drainage plus antibiotics.
(Option D): Porcelain gall bladder does not cause bowel obstruction. However,
gall stone ileus is not a rare event. Occasionally large gallstones may form a
fistula between gall blabber and the small bowel. The gallstone then obstructs
the small bowel. Diagnosis is confirmed by the presence of air in the biliary tract.
This condition is typically seen in elderly females and all require surgery.
(Option E): Porcelain gall bladder does not cause constipation. However, if the
gall bladder is inflamed, it may cause an ileus that may last for the duration of
infection.
Educational objective:
Porcelain gall bladder is an entity usually diagnosed on an abdominal x- ray. The
condition predisposes individuals to gall bladder carcinoma and requires
resection.
16% of people answered this question correctly
Test #24 Q.Id = 884
Question 5 Of 39
Division : Hepatology
Item Review
A 43-year-old African-American female comes with the acute onset of right upper
quadrant abdominal pain, fever with chills, and jaundice. On examination, she
looks very ill and distressed and is also dehydrated. Her vitals are T:
39.0C(102F), RR: 20/min, and PR: 90/min. Ultrasonography of her abdomen
shows a dilated common bile duct with stones both in the duct and in the gall
bladder. Her liver function tests show total bilirubin: 10 mg/dl, direct bilirubin: 8
mg/dl, ALT: 40 IU/L, ALT: 35 IU/L, alkaline phosphatase: 700 IU/L and PT: 11sec.
You admit her and start her on intravenous fluids and broad-spectrum antibiotics.
What would be the next best step in the management of this patient?
A. Endoscopic retrograde cholangiopancreatography (ERCP).
B. Laparotomy with removal of stone and cholecystectomy.
C. Insert a T tube for drainage.
D. Do a transhepatic cholangiopancreatography.
E. Do a laparoscopic cholecystectomy.
Explanation:
This patient has acute ascending cholangitis, which is characterized by the triad
of fever, right upper quadrant abdominal pain and severe jaundice (Charcots
triad). Cholangitis is an infection of the common bile duct and is generally
secondary to obstruction of the common bile duct by a stone as in this patient
leading to the dilatation of common bile duct. It is an acute condition and broadspectrum antibiotics and supportive care should be started immediately.
However, it is very important to decompress the biliary ducts and provide their
drainage.
Of all the available options to drain the biliary tree ERCP (Endoscopic retrograde
cholangiopancreatography) is the method of choice as it is minimally invasive,
has diagnostic use and has the least rate of complications. ERCP can be used to
do a sphincterotomy with the stone removal and drain the bile via the sphincter
or by placement of a stent. Early drainage of the biliary tree in acute cholangitis
can significantly decrease its morbidity and mortality (Choice A).
Choice B: Before the advent of ERCP, this was the preferred treatment but it has
a very high morbidity and mortality and is no longer the preferred method.
Choice C: This is not the first choice in a patient with cholangitis and is used only
if ERCP fails to drain the bile ducts or a stricture is encountered with the ERCP.
Choice D: PTC has more complications than ERCP when used to drain the
biliary tree.
Choice E: Laparoscopic cholecystectomy will not provide the drainage for the
biliary tree. Though this patient will eventually need a cholecystectomy, at
present the drainage of biliary tree gains priority over cholecystectomy.
Educational Objective:
The treatment of acute cholangitis is to give supportive care, broad-spectrum
antibiotics, and biliary drainage with an endoscopic retrograde
cholangiopancreatography.
21% of people answered this question correctly
Test #24 Q.Id = 811
Question 6 Of 39
Division : Hepatology
Item Review
A 22-year-old Westerner comes into your office with the chief complaints of right
upper abdominal pain and yellow urine for the past 2 days. He also feels
fatigued, anorexic, and has recently developed an aversion to smoking. He
returned with his family from a trip to Mexico about 3 weeks ago. His brother
Educational Objective:
The epidemiology of various viral hepatitis infections can aid in the correct
clinical diagnosis.
70% of people answered this question correctly
Test #24 Q.Id = 834
Question 7 Of 39
Division : Hepatology
Item Review
A 46-year-old American male is planning to go on a 2-week trip to Mexico within
one week. He comes to you for advice regarding prophylaxis for viral hepatitis.
He is a monogamous heterosexual male without any history of illicit drug use. He
is feeling perfectly normal at present and his physical examination is normal.
Viral serology done 2 weeks ago in a health fair is negative. What should be your
advice to this patient?
A. Advice him to take Hepatitis A vaccine.
B. Advice him to take serum immunoglobulin against Hepatitis -A.
C. Advice him to take Hepatitis B vaccine.
D. Advice him to take hepatitis B immune globulin.
E. Advice him that he need not to take any prophylaxis.
Explanation:
Hepatitis A is endemic in many parts of the world outside United States. A nonimmunized person going out of United States to an endemic region needs
prophylaxis against Hepatitis A. There are two modes of prophylaxis against
hepatitis A:
1. Hepatitis A vaccine: provides active immunity
2. Serum immune globulin: provides passive immunity.
This patient is going on a trip within one week and if the vaccine is being given
this might take at least one month for the immunity to become effective, therefore
the best way to confer immunity would be the passive one by administering
serum immune globulin.
Choice C and D: This patient is a heterosexual male with no use of illicit drugs
and so has no risk factors for hepatitis B. At the same time, hepatitis B is not
transmitted by the feco-oral route therefore an international travel to an endemic
country is not a risk factor for hepatitis B.
Choice E: This patient does not have hepatitis A antibodies and is traveling to an
endemic country, which puts him at high risk of contracting hepatitis A therefore
giving no prophylaxis cannot be the best choice.
Educational Objective:
Question 8 Of 39
Item Review
Division : Hepatology
2. Alkalosis.
3. Diuretic therapy.
4. Extensive gastro intestinal bleeding.
5. Narcotics, hypnotics, and sedatives.
6. Medication containing ammonium or amino compounds.
7. High volume paracentesis.
8. Hepatic or systemic infection.
9. Portocaval shunts.
Hepatic encephalopathy is characterized by reversal of sleep cycle, asterixis,
progressive coma, and characteristic delta waves on electroencephalogram
(EEG).
Choice B: Urea is the detoxified product of ammonia, which is produced by the
liver through the urea cycle and excreted by the kidney.
Choice C: The liver is a major site of gluconeogenesis and liver failure can lead
to hypoglycemia and coma but this patients profile and development of
symptoms after receiving a thiazide along with asterixis is suggestive of hepatic
encephalopathy.
Choice D: Salt restricted diet is beneficial in patients with cirrhosis with ascites
and has nothing to do with hepatic encephalopathy.
Choice E: Protein rich diet and not protein poor diet is a precipitating factor for
encephalopathy as protein rich diet produces more ammonia in gut.
Educational Objective:
Hepatic encephalopathy is a central nervous system complication of liver failure
secondary to accumulation of ammonia in blood because of inability of liver to
detoxify ammonia into urea.
63% of people answered this question correctly
Test #24 Q.Id = 889
Question 9 Of 39
Division : Hepatology
Item Review
A 65-year-old Caucasian male undergoes coronary artery bypass grafting.
Intraoperatively, he requires 10 units packed red blood cells to maintain his O2
carrying capacity. The surgery is successful and patient is stable postoperatively. However, on the second postoperative day he develops jaundice. He
is afebrile and his abdominal examination is unremarkable. His laboratory reports
shows total bilirubin: 9.0 mg/dl, AST: 55 IU/L, ALT: 50 IU/L, alkaline phosphatase:
350IU/L, PT: 12 sec, and serum albumin: 3.8 g/dl. What is the most likely cause
of these findings in this patient?
Question 10 Of 39
Division : Hepatology
Item Review
A 45-year-old male from Mexico is seen in your clinic for pain in the right upper
quadrant. He says he has had diarrhea for a week and abdominal pain. He
denies fevers, chills or bloody stools. He denies the use of drugs or any allergies.
Examination reveals tenderness localized to the right upper quadrant. Labs
showed a total WBC count of 11,000/cmm without bandemia or eosinophilia. The
CT scan of the abdomen is shown below. Aspiration reveals material that
resembles anchovy paste. What is the most appropriate next step in his
management?
Question 11 Of 39
Division : Hepatology
Item Review
A 45-year-old male comes to the emergency room with the complaints of severe
respiratory distress. His chest x-ray is consistent with bilateral hydrothorax. A
thoracentesis is done and the pleural fluid analysis shows findings consistent
with a cirrhotic hydrothorax. A therapeutic thoracentesis is performed, and he is
started on spironolactone and furosemide. However, the hydrothorax recurs and
patients diuretic dose is increased to 120mg/day furosemide and 200mg/day
spironolactone. A repeat therapeutic thoracocentesis is done but again it recurs
after 3 days. What is the best intervention for this patient now?
A. Do a transjugular intrahepatic portosystemic shunt (TIPS).
B. Do pleurodesis.
C. Put a intercostal drainage.
D. Increase the dose of his diuretics.
E. Do repeated large volume thoracocentesis.
Explanation:
This patient has refractory hydrothorax as evident by recurrent hydrothorax even
with maximal dose of diuretics. TIPS procedure is the treatment of choice for
patients with refractory recurrent cirrhotic hydrothorax (Choice A). Studies have
shown TIPS to be more effective than medical therapy and repeated large
volume thoracocentesis (Choice E) in patients with cirrhotic hydrothorax. Some
of the common indications for TIPS are:
1. Refractory cirrhotic hydrothorax.
2. Refractory ascites (defined as diuretic resistant or diuretic refractory ascites).
3. Recurrent variceal bleed not controlled by other minimal invasive means.
4. Patients waiting for liver transplantation and needing portocaval shunts.
Choice B: Pleurodesis is an choice for refractory pleural effusions secondary to
malignancy but not for a hydrothorax secondary to cirrhosis.
Choice C: An intercostal drainage is an choice for a hemothorax or pyothorax
but not for a hydrothorax.
Choice D: The patient is already on the maximal doses of diuretic indicated for
cirrhotic ascites or hydrothorax. Also, further increasing the dose makes the
toxicity from diuretics more likely.
Educational objective:
Transjugular intrahepatic portosystemic shunt procedure is used in cirrhotic
patient for refractory ascites, refractory hydrothorax, and surgical management of
acute recurrent variceal bleeding.
29% of people answered this question correctly
Test #24 Q.Id = 869
Question 12 Of 39
Division : Hepatology
Item Review
A 52-year-old alcoholic male comes with the complaints of abdominal distention
and respiratory distress. He also complains of weight loss over the past 2
months. On examination, he has diffuse rales in the chest, 2+ pedal edema, and
shifting dullness in abdomen. His vitals are: PR: 90/min, Temperature:
37.2C(99F), RR: 18/min. What should be the next best step in the management
of this patient?
A. Ultrasonogram.
B. Paracentesis.
C. Chest-X ray.
D. CT abdomen.
E. Liver function tests.
Explanation:
This patient has massive ascites, which by definition is severe enough to cause
respiratory distress. In this situation a paracentesis would be the next best step,
as it will be both a diagnostic and therapeutic in this patient. Ascitic fluid
examination would help us focus on the cause of ascites and volume
paracentesis will drain the fluid and relieve the respiratory distress in the patient.
Later the patient should be started on diuretics (Choice B).
Choice A and D: Ultrasonogram of abdomen and CT scan will tell us about the
amount of fluid and if there is any hepatomegaly but still wont tell us about nature
of ascitic fluid and would not relieve the respiratory distress. However, either one
of them should be done on this patient later.
Choice C: Chest x-ray can tell us more about the rales and any cardiomegaly,
but it too will not address the respiratory distress. However, it should be done
later.
Choice D: LFTs will be of no use unless the ascitic fluid turns out to be a
transudate. However, it should be done later.
Educational Objective:
Paracentesis can be used for both diagnostic and therapeutic purposes in
patients with ascites.
27% of people answered this question correctly
Test #24 Q.Id = 872
Question 13 Of 39
Division : Hepatology
Item Review
A 52-year-old African-American male comes for a regular health check-up. He is
a chronic smoker and has been drinking about 2 beers/day for the past 10 years.
He also drinks about 7 cups of coffee/day. He was diagnosed with diabetes 5yrs
ago and takes metformin for it. On examination, he has a Body Mass Index (BMI)
of 29 and BP: 130/80 mm Hg. His random blood sugar is 190 mg/dl. His elder
brother died of pancreatic cancer at age of 58 and he is worried that he might
also get pancreatic cancer. Which of the following interventions would decrease
his risk of pancreatic cancer the most?
A. Stop the alcohol intake.
B. Stop smoking.
C. Stop the coffee intake.
D. Stop metformin.
E. Achieve better control of blood sugar with insulin.
Explanation:
Pancreatic cancer is a highly malignant cancer making it fifth most common
cause of cancer related mortality. It results in death in 98% of afflicted individuals
(98% fatality rate). Risk factors for pancreatic cancer are:
1. Male sex.
2. Increasing age (50 years).
3. Black race.
4. Cigarette smoking: It is the most consistent risk factor. Pancreatic cancer is 23 times more likely in heavy smokers than in nonsmokers.
5. Chronic pancreatitis.
6. Long-standing diabetes.
7. Obesity.
8. Familial pancreatitis.
9. Pancreatic cancer in a close relative.
The following are not risk factors for pancreatic cancer:
1. Alcohol consumption.
2. Gall stones
3. Coffee intake.
Choice A and C: Alcohol intake and coffee intake are not risk factors for
pancreatic cancer.
Choice D: Metformin is not associated with pancreatic cancer.
Choice E: Long standing diabetes is a risk factor for pancreatic cancer. However,
this patient has been diagnosed with diabetes 5 years ago and the benefits of
smoking cessation in prevention of pancreatic cancer outweigh the benefits of
sugar control.
Educational Objective:
Cigarette smoking is the most consistent reversible risk factor for pancreatic
cancer.
31% of people answered this question correctly
Test #24 Q.Id = 887
Question 14 Of 39
Division : Hepatology
Item Review
A 40-year-old Asian female comes to your office with the complaint of severe
shortness of breath, cough and hemoptysis. A chest radiograph shows right
upper lobe fibrosis and her sputum is positive for acid-fast bacilli (AFB). A
diagnosis of active pulmonary tuberculosis is made and she is started on
isoniazid, rifampin, ethambutol, and pyrazinamide. She comes to you after one
month for follow up and says that she is feeling better than before. Her sputum is
negative for AFB. Her routine laboratory reports shows total bilirubin: 1.0 mg/dl,
AST: 50 IU/L, ALT: 140 IU/L, and alkaline phosphatase: 40 IU/L. What would be
your next step in management of this patient?
A. Do nothing except for regular follow up.
B. Start second line anti tubercular drugs.
C. Stop antitubercular drugs.
D. Do a liver biopsy.
Question 15 Of 39
Division : Hepatology
Item Review
A 52-year-old male comes with the complaints of fever and fatigue. He has been
consuming alcohol in the form of 12-24oz beer/day, for the past 20 years. He has
never had any blood transfusion, tattoos or high-risk sexual behaviors. On
examination, he has tender hepatomegaly with spider angiomata on his upper
torso. Liver biopsy shows steatosis, polymorphonuclear cellular (PMN) infiltrates
and necrosis. Which of the following statements is true about alcoholic liver
disease?
Males are more susceptible than females to have an alcoholic liver
disease.
B. Alcoholic fatty liver is an irreversible condition.
C. In alcoholic liver disease the AST/ALT is > 2.
D. 50 percent of alcoholics develop alcohol related hepatitis.
E. Presence of Mallory bodies is highly specific for alcoholic hepatitis.
Explanation:
A.
peri-sinusoidal space of Disse. Though Mallory bodies are often found in severe
cases, this is neither specific nor required for the diagnosis of alcoholic hepatitis.
Educational Objective:
Alcoholic Hepatitis is characterized by AST/ALT > 2.
45% of people answered this question correctly
Test #24 Q.Id = 883
Question 16 Of 39
Division : Hepatology
Item Review
A 40-year-old Asian male comes to your office with the chief complaint of yellow
urine. On examination, he has scleral icterus and his urine dipstick is positive for
bilirubin. Which of the following could be the cause of these findings?
A. Hemolytic anemia.
B. Thalassemia.
C. Rotors syndrome.
D. Gilberts syndrome
E. Paroxysmal nocturnal hemoglobinuria.
Explanation:
This patient has jaundice as indicated by scleral icterus and yellow urine. Next
step is to determine the type of jaundice i.e. conjugated or unconjugated. This
can be done in two ways:
1. A very simple and convenient method is to measure the urinary excretion of
bilirubin by urine dipstick method. Normally more than 95% of blood bilirubin is
due to the unconjugated fraction. The unconjugated fraction of bilirubin is
insoluble as it is bound to the albumin and therefore cannot be filtered by the
glomerulus and is not excreted in urine. Thus normally urine has no detectable
bilirubin. However, the conjugated fraction is soluble in plasma, can be filtered by
the glomerulus and excreted in the urine. Patients with conjugated
hyperbilirubinemia have >50% of bilirubin in form of conjugated bilirubin so
enough of it remains unbound to be filtered and excreted by kidney. So the
presence of bilirubin in urine is indicative of conjugated hyperbilirubinemia.
2. A more precise way is to do the Vandenbergh test.
This patient has bilirubin in his urine indicative of predominantly conjugated
hyperbilirubinemia. Of the given options, only Rotors syndrome, which is a
congenital defect in the excretion of bilirubin by hepatocytes, can lead to
conjugated hyperbilirubinemia (Choice C).
Choice A, B, D and E: They are all characterized by predominantly
unconjugated hyper bilirubinemia and thus the urine would be negative for
bilirubin in each of this case.
Educational Objective:
Checking for urinary excretion of bilirubin is an easy and effective way of
determining whether the cause of jaundice is conjugated or unconjugated
bilirubin.
23% of people answered this question correctly
Test #24 Q.Id = 856
Question 17 Of 39
Division : Hepatology
Item Review
A 45-year-old Asian female comes with the chief complaints of mild pruritus for
the past six months, and yellow urine for the past 2 weeks. She also complains
of severe fatigue. On examination, she has xanthelasmas and yellow sclerae.
Abdominal examination is normal and there are no cirrhotic stigmata. Laboratory
reports show total bilirubin: 5.6 mg/dl, AST: 80 IU/L, ALT: 70 IU/L, alkaline
phosphatase: 250 IU/L, positive anti mitochondrial antibodies, PT: 10 seconds,
serum albumin: 3.5 g/dl, cholesterol: 250 mg/dl, and increased IgM levels. Liver
biopsy shows chronic, nonsuppurative, destructive cholangitis of the small
interlobular bile ducts but no fibrosis. What is the most appropriate treatment for
this patient?
A. Cholestyramine.
B. Liver transplantation.
C. Ursodeoxycholic acid.
D. Corticosteroids.
E. Methotrexate.
Explanation:
This patient is having primary biliary cirrhosis, which is a chronic progressive
cholestatic liver disease commonly presenting with fatigue, pruritus, and
jaundice; it is characterized by positive anti mitochondrial antibodies in 90% of
patients.
Although there is no specific treatment for primary biliary cirrhosis,
ursodeoxycholic acid (Choice C) is the major medication used in this condition
for two reasons:
1. It relieves symptoms.
2. It also helps to slow the progression of disease and thus improve liver
transplant free survival.
Choice A: Cholestyramine can be used to control the severe pruritus and
hypercholesterolemia in patients with PBC, but it does not improve the survival
rates. It is therefore a second choice drug, mainly used to control severe
symptoms.
Choice B: Liver transplantation is the only ultimate cure of progressive PBC, but
it is indicated in patients with progressive primary biliary cirrhosis with severe
liver damage or cirrhosis. However, this patient has no symptoms or signs of
cirrhosis so a trial of ursodeoxycholic acid is appropriate.
Choice D and E: Steroids, methotrexate, and other immunosuppressant drugs,
have been used in cases of severe primary biliary cirrhosis with very severe
symptoms not controlled by other drugs and when liver transplantation is not an
option.
Educational Objective:
Ursodeoxycholic acid is the most commonly used drug for primary biliary
cirrhosis as it relieves symptoms and improves the transplant free survival time.
21% of people answered this question correctly
Test #24 Q.Id = 828
Question 18 Of 39
Division : Hepatology
Item Review
A 55-year-old white male is brought to the ER with massive hematemesis. He
has a history of peptic ulcer disease and esophageal varices from alcoholic
cirrhosis; he is on propranolol, ranitidine, and spironolactone. His vitals are: BP:
100/60 mm of Hg; PR: 110/min; RR: 23/min; Temperature: 37.6C(99.6F). Which
of the following should be the first step in the management of this patient?
A. Administer octreotide.
B. Perform an endoscopic band ligation of varices.
C. Perform an endoscopic sclerotherapy.
D. Insert two large bore intravenous needles.
E. Place a S.B (Sengstaken-Blakemore) tube.
Explanation:
Variceal bleeding is a life-threatening emergency; therefore prompt and vigorous
resuscitation with fluids should be the first step in the management of patients
with variceal bleeding. All patients with acute variceal bleeding should have
adequate vascular access available, usually with two large bore intravenous
needles followed by fluid resuscitation until transfusion of blood products can be
arranged if needed. (Choice D)
The second step in the management of variceal bleeding should be to control the
bleeding itself. In about 50% of cases the bleeding ceases on its own without any
further intervention. The medical control of bleeding consists of administration of
vasoconstrictors like octreotide and somatostatin, which are favored over
vasopressin. Control of bleeding occurs in 80% of patients however, re bleeding
is very common. (Choice A)
The use of endoscopic techniques should be the next step whenever these are
available. Endoscopic sclerotherapy has been used for the past 2 decades and
bleeding can be controlled in 90% of the cases. However, recent band ligation of
varices has gained popularity over sclerotherapy due to fewer complications.
Prophylactic endoscopic intervention in patients who have never bled is not yet
recommended. (Choice B, C)
Use of balloon tamponade with S-B tube (Sengstaken-Blakemore) or Minnesota
tube can be employed for acute control of bleeding when endoscopy is not
available or, when drugs are not working fast enough and to buy some time until
the patient is transferred to a tertiary care hospital. (Choice E)
Surgical treatment of acute variceal bleeding: If the bleeding is not controlled by
all the above medical interventions, then surgical shunt therapy can be
performed to control the bleeding.
Educational Objective:
The first step in the treatment of acute variceal bleeding is to get vascular access
with two large bore intravenous needles.
53% of people answered this question correctly
Test #24 Q.Id = 827
Question 19 Of 39
Division : Hepatology
Item Review
A 52-year-old alcoholic presents with abdominal distention and weight loss. He
has been drinking half a bottle of whisky almost everyday for the past 20 years.
He had a previous attack of acute pancreatitis 3 years ago. On examination he
has gynecomastia, firm enlarged liver, and free fluid in the abdomen.
Esophagoscopy shows large esophageal varices. However, he never had
hematemesis. Which of the following is the best intervention for prevention of
hematemesis in this patient at this time?
A. Use of octreotide.
B. Band ligation of varices.
C. Endoscopic sclerotherapy.
D. Use of beta-blockers.
E. No intervention is needed except education about quitting alcohol.
Explanation:
This patient has signs of decompensated cirrhosis secondary to alcoholism. He
has ascites and esophageal varices secondary to portal hypertension.
Prophylactic treatment of patients with large varices who have never bled, with
non-selective beta-blockers like propranolol has been shown to significantly
decrease the risk of future bleeding. Propranolol should be administered in doses
sufficient to decrease the resting pulse by 25%. Beta-blockers have also been
shown to be useful in the secondary prevention of recurrent bleeds; however
they are not useful in the acute control of hematemesis due to concomitant
hypotension caused by hypovolemia.
Choice A: Octreotide is used in the medical management of acute variceal
bleeding. It has no role in primary or secondary prevention of variceal bleeding.
Choice B and C: Whenever available, endoscopic interventions should be used
as the first line to control the acute variceal bleed. They also have some value in
the secondary prevention of recurrent variceal bleed but are not commonly used
for primary prevention.
Choice E: This patient has large varices and is in decompensated alcoholic
cirrhosis, which puts him at high risk of having a variceal bleed; therefore, doing
nothing would not be an adequate option.
Educational Objective:
Non-selective B blockers are used in primary and secondary prevention of
variceal bleeding in cirrhotic patients who have portal hypertension with varices.
18% of people answered this question correctly
Test #24 Q.Id = 807
Question 20 Of 39
Item Review
Division : Hepatology
Question 21 Of 39
Division : Hepatology
Item Review
A 45-year-old Mexican male comes with fever, anorexia, and right upper
quadrant pain, for the past 12 days. The pain radiates to the right shoulder tip
and is aggravated by deep inspiration. The patient gives the history of bloody
diarrhea six months ago, after he returned from a trip to Mexico. He has no other
major illnesses and never had any surgery. He denies any close contacts with
animals, is a non-alcoholic, non-smoker, and with a single monogamous sexual
partner. On examination, he has right upper quadrant abdominal and intercostal
tenderness, but no jaundice. An ultra sonogram of abdomen shows a single thin
wall uniform cyst on the superior surface of the right lobe of his liver. What is the
most likely diagnosis in this patient?
Pyogenic liver
abscess
Amebic liver
B.
abscess
C. Hydatid cyst
D. Liver carcinoma
E. Hepatic adenoma
Explanation:
A.
This patient has an amebic liver abscess. The history of travel to an endemic
area followed by dysentery and right upper quadrant pain with a single cyst in
right lobe of liver is indicative of amebic liver abscess. Amebiasis is a protozoal
disease caused by Entamoeba histolytica. The primary infection is in the colon
leading to bloody diarrhea; however the ameba may be transported to the liver
by portal circulation thus leading to an amebic liver abscess (Choice B).
Amebic liver abscesses are generally single and are located usually in the right
lobe. An abscess on the superior surface of liver can cause a pleuritic-like type of
pain and the radiation to the shoulder. The diagnosis can be made by stool
examination for trophozoites, serology, and liver imaging. The aspirate from the
amebic liver abscess is usually sterile. Treatment is with metronidazole.
Choice A: Pyogenic liver abscess should be considered in the differential
diagnosis of amebic liver abscess, but this patient's history is more suggestive of
amebic liver abscess. Pyogenic liver abscess is generally secondary to surgery,
a gastrointestinal infection, or acute appendicitis. The condition of the patient
would be more severe than in amebic liver abscess.
Choice C: A hydatic cyst is usually caused by an infection with Echinococcus
granulosus, which is acquired by intimate contacts with dogs.
Choice D: Unilocular cystic lesion as a presentation of hepatic adenocarcinoma
is not common. Cystic carcinoma generally has thick irregular and hyper vascular
walls with numerous septa. The other inflammatory features are unlikely to be
Question 22 Of 39
Division : Hepatology
Item Review
A 42-year-old Asian male presents to the hospital with the acute onset of fever
and right upper quadrant abdominal pain. He has had recurrent colicky right
upper quadrant abdominal pain for the past 10 months, especially after meals.
His past medical history is significant for adult polycystic kidney disease (APKD),
diagnosed 2 years ago. On examination he has marked hepatomegaly, but no
icterus. His laboratory report shows WBC: 14,000/cmm, AST: 80 IU/L, ALT: 70
IU/L, alkaline phosphatase: 90 IU/L and negative autoimmune and viral hepatitis
screen. An ultrasonogram of abdomen shows marked irregular dilatation of
intrahepatic bile ducts. What is the most likely diagnosis in this patient?
A. Primary biliary cirrhosis
Primary sclerosing
B.
cholangitis.
C. Carolis syndrome.
D. Common bile duct stone.
E. Choledochal cyst.
Explanation:
Carolis syndrome is a rare congenital disorder characterized by intrahepatic
dilatation of biliary tree. It is often associated with autosomal recessive adult
polycystic kidney disease (APKD) but less commonly with autosomal dominant
APKD.
Patients with Carolis syndrome can present in following ways:
1. Intermittent abdominal pain due to bile stasis or intermittent passage of stone.
2. Cholangitis leading to fever and right upper quadrant abdominal pain.
3. Portal hypertension leading to hematemesis and melena.
4. Renal complaints due to APKD.
Cholangiocarcinoma is 100 times more common in patients with Carolis
syndrome than in normal population. Laboratory reports generally show normal
bilirubin and mildly elevated liver enzymes. Ultrasonogram is the diagnostic
Question 23 Of 39
Division : Hepatology
Item Review
A 5-year-old child is brought to your office with chicken pox. You prescribe aspirin
and send him home. 4 days later he is brought to the emergency room in a
comatose state. On examination, he has hepatomegaly, but no icterus. His
laboratory reports show marked increase in transaminases and ammonia levels.
His PT is prolonged. Which of the following is the most likely liver morphology in
this patient?
A. Balloon degeneration with polymorphic cellular infiltrates.
B. Panlobular mononuclear infiltration with hepatic cell necrosis.
C. Extensive fatty vacuolization of liver.
Portal necrosis including piecemeal necrosis and/or bridging
D.
fibrosis.
E. Inflammatory destruction of small intrahepatic biliary ducts.
Explanation:
This child is suffering from Reyes syndrome, which is also called fatty liver with
encephalopathy. It is seen exclusively in children younger than 15 years of age. It
generally follows an upper respiratory tract infection with influenza or chicken pox
and treatment with aspirin increases the likelihood of developing this syndrome.
Pathophysiologically, it is characterized by hepatic mitochondrial injury leading to
vomiting, hypoglycemia, signs of liver failure, and progressive CNS damage.
Question 24 Of 39
Division : Hepatology
Item Review
A 62-year-old male chronic smoker presents with anorexia, fatigue, and
9kg(20lb) weight loss within the past six months. He is not an alcoholic and has
no significant past medical illnesses. On examination, his abdomen is soft, with
deep tenderness in the mid-epigastric region, and has icteric sclerae. His
laboratory reports show bilirubin: 12 mg/dl, AST: 110 IU/l, ALT: 100 IU/L, PT: 11
seconds, albumin: 4.0 g/dl, alkaline phosphatase: 600 IU/L, negative viral
serology and negative autoimmune screen. An ultrasonogram of the abdomen
shows absence of gallstones and mild dilation of the common bile duct. What is
the next best investigation in this patient?
A. CT abdomen.
Endoscopic retrograde
B.
cholangiopancreatogram.
Percutaneous transhepatic cholangiogram
C.
(PTC).
D. Serum amylase and lipase.
E. Serum CA 19-9.
Explanation:
Anorexia, weight loss, and painless jaundice in a smoker with increased alkaline
phosphatase and absence of gallstones is suggestive of pancreatic carcinoma.
Question 25 Of 39
Division : Hepatology
Item Review
A 42-year-old Hispanic female comes to you with a complaint of amenorrhea for
the past 3 months. You order an abdominal and pelvic ultrasound, which shows a
submucosal fibroid in the uterus, along with multiple gallstones. On examination,
she has normal vital signs, no icterus or abdominal tenderness. She has no
intolerance to fatty food and is not taking any medication. What is the next best
step in the management of her gallstones?
Schedule the patient for laparoscopic
cholecystectomy.
B. Prescribe her ursodeoxycholic acid.
C. Do a laparotomy and remove the gallstones.
D. Do nothing at this time.
E. Lithotripsy to fragment the gallstones.
Explanation:
A.
uterine fibroids. Asymptomatic gallstones should not be treated because only 12% of these patients develop symptoms or complications later in life. (Choice D)
Choice A and C: The following are circumstances in which cholecystectomy
should be done on a patient with gallstones:
1. Frequent and severe symptoms.
2. History of a complication due to gallstones like acute cholecystitis or
pancreatitis.
3. Patients at a high risk of complications due to gallstones, such as patents with
PHT, cirrhosis, diabetes, and sickle cell anemia with minor symptoms.
Choice B: Ursodeoxycholate is a medical treatment intended to dissolve the
gallstone persons who are poor surgical candidates.
Choice E: This is a choice for the treatment of symptomatic gallstones in
patients with small gallstones that are poor surgical candidates.
Educational Objective:
Asymptomatic gallstones should not be treated. Know when cholecystectomy is
indicated in patients with gallstones.
44% of people answered this question correctly
Test #24 Q.Id = 815
Question 26 Of 39
Division : Hepatology
Item Review
A 40-year-old male comes for a check-up for his health insurance. His laboratory
report shows serum AST 50 IU/L; serum ALT 60 IU/L; S. Bilirubin 1.0 mg/dl and
increased serum iron and ferritin. He has no icterus or yellow urine. Liver is not
palpable. He does not drink alcohol, does not take any medication and denies
any high-risk behavior for acquisition of viral hepatitis. He says that his father
was not drinking alcohol either and died of cirrhosis at the age of 50. What is the
most likely diagnosis in this patient?
A. Hemosiderosis
B. Hemochromatosis
C. Wilsons disease
Metastatic melanoma to
D.
liver
E. Normal health check up
Explanation:
This patient is not alcoholic and has an asymptomatic elevation of serum
transaminases, increased serum iron and ferritin levels, and has a family history
of non-alcoholic liver cirrhosis. These features highly suggest the diagnosis of
Question 27 Of 39
Division : Hepatology
Item Review
A 42-year-old alcoholic male is admitted to the hospital for acute pancreatitis.
You are treating him supportively, with intravenous fluids and NPO; the patient is
improving, with decrease in his WBC count and serum amylase. However, 4
weeks later, he develops a palpable mass in the epigastrium with pain radiating
to his back. On examination, his vitals are stable and his labs show a marked
increase in serum amylase. What is the most likely diagnosis in this patient?
A. Hemorrhagic pancreatitis.
Infected pancreatic
B.
necrosis.
C. Pancreatic ascites.
D. Pancreatic abscess.
E. Pseudopancreatic cyst.
Explanation:
The development of a palpable mass in epigastrium 4 weeks after the onset of
acute pancreatitis is highly suggestive of Pseudocyst formation (Choice E).
Although pseudocysts are relatively common in chronic pancreatitis, they can
also complicate acute pancreatitis. About 15 % of patients with acute pancreatitis
develop pseudocysts. 85 % are located in the body or the tail and 15% in the
head of pancreas. Pseudocysts are not true cysts as they lack an epithelial
lining. They represent a collection of tissue, fluid, and necrotic debris around the
pancreas. Amylase rich fluid, which leaks into circulation, leads to a marked
elevation of serum amylase. USG is the investigation of choice. Pseudocysts
should be drained only if they persist for more than 6 weeks or are more than 5
cm in diameter.
Choice A: Hemorrhagic pancreatitis is characterized by retroperitoneal
hemorrhage and pancreatic necrosis. It is considered to be a progression of
edematous pancreatitis and the patients are usually very sick. However, this
patient is having normal vitals with an abdominal mass.
Choice B: Infected pancreatic necrosis is the diffuse infection of necrotic
pancreatitis, usually 1-2 weeks after acute pancreatitis and characterized by
gravely ill patient with spiking fever and leucocytosis. Surgical debridement is the
treatment of choice.
Choice C: Pancreatic ascites occurs from a leaking pseudocyst with ductal
destruction. It leads to abdominal distention and shifting dullness.
Choice D: Pancreatic abscess is a localized ill-defined collection of pus from
necrotic pancreatic tissue and is characterized by persistent fever, leukocytosis,
and elevated serum amylase.
Educational Objective:
Pancreatic pseudocyst is a complication of both acute and chronic pancreatitis
and is diagnosed by ultrasound.
67% of people answered this question correctly
Test #24 Q.Id = 826
Question 28 Of 39
Division : Hepatology
Item Review
A 42-year-old male comes with the complaints of yellow skin and urine for the
past 3 days. He has been having intermittent right upper abdominal pain for the
last 2-3 years. He was diagnosed with ulcerative colitis 3 years ago and takes
steroid enemas during the flare-ups. On examination, he has a normal
temperature and yellow sclerae. His laboratory reports show total bilirubin 4.5
mg/dl, alkaline phosphatase 800 IU/L, AST 200 IU/L, ALT 280 IU/L and positive
P-ANCA. ERCP shows a beaded appearance of the intrahepatic and
extrahepatic biliary tree. What is the most likely diagnosis in this patient?
A. Primary biliary cirrhosis.
Primary sclerosing
B.
cholangitis.
C. Polyarteritis nodosa.
D. Acute cholecystitis.
E. Acute cholangitis.
F. Microscopic polyangiitis
Explanation:
Primary sclerosing cholangitis is caused by inflammatory destruction of both the
intrahepatic and extrahepatic biliary ducts. It is equally prevalent in males and
females and is associated with inflammatory bowel disease especially ulcerative
colitis in 70% of cases. Patients with primary sclerosing cholangitis have variable
presentations, with jaundice, pruritus, RUQ abdominal pain, or acute cholangitis
suggestive of chronic or intermittent biliary obstruction. It may progress later to
complete biliary obstruction, secondary biliary cirrhosis, hepatic failure, or portal
hypertension. ERCP is the investigation of choice as involvement of intrahepatic
duct might make percutaneous cholangiography difficult. Cholangiography shows
characteristic beading due to strictures and dilation of intra and extra-hepatic
ducts. Liver function tests show a very high alkaline phosphatase with mild
elevation of transaminases. The serum aminotransferases are typically less than
300 IU/L. Additional serologic findings in patients with PSC include.
1. Hypergammaglobulinemia 30%
2. Increased serum IgM levels 40 to 50%
3. Atypical perinuclear antineutrophil cytoplasmic antibodies (P-ANCA) 30 to
80%
4. A number of other autoantibodies (ANA, Anti SMA, anticardiolipin,
thyroperoxidase, and rheumatoid factor) may also be present, but they have
uncertain clinical significance.
Treatment is directed toward the relief of obstruction and treatment of the
infectious complications. Liver transplantation is indicated in deteriorating PSC.
Choice A: Primary biliary cirrhosis is more common in females (9:1), and is not
characterized by jaundice and pruritus until later in disease, and not associated
with IBD. They have positive AMA.
Choice C and F: P-ANCA is also positive in PAN and microscopic polyangiitis
but they do not cause the biliary tree pathology.
Choice D: Acute cholecystitis will be characterized by triad of fever, RUQ pain,
and leucocytosis and will not have this kind of cholangiographic abnormality.
Choice E: Patient with PSC can have acute cholangitis but this patient is not
having it right now, as acute cholangitis is characterized by severe jaundice,
fever and RUQ pain with leucocytosis.
Educational Objective:
Primary sclerosing cholangitis is caused by inflammatory destruction of both the
intrahepatic and extrahepatic biliary channels. It is often associated with
ulcerative colitis.
57% of people answered this question correctly
Test #24 Q.Id = 806
Question 29 Of 39
Division : Hepatology
Item Review
A 32-year-old prostitute comes to your office with the chief complaint of chronic
fatigue. A number of tests are performed, showing that she is positive for HBsAg.
She is started on supportive treatment. Her follow-up labs show a progressive
increase in her PT and a decrease in her transaminase level. These laboratory
findings are indicative of which of the following conditions?
A. Progression to fulminant hepatitis.
B. Superinfection with hepatitis D.
C. Recovery phase of hepatitis B.
D. Coexisting hepatitis C.
Presence of hepatocellular
E.
carcinoma.
Explanation:
The laboratory tests used in the evaluation of liver diseases can be classified into
two broad categories.
Tests to assess the functions of liver:
1. PT.
2. S. Albumin.
3. S. Cholesterol.
4. S. Bilirubin.
Tests to assess the structural integrity and cellular damage.
1. Transaminases
2. Gamma glutaryl transferase
3. Alkaline phosphatase
PT is considered the single most important test to assess the function of liver as
almost all of the clotting factors except factor VIII are synthesized in the liver.
Elevated serum levels of transaminases are indicative of liver cell damage as this
intracellular enzyme leaks from the injured hepatocytes. A marked increase in the
transaminase is indicative of active ongoing tissue destruction. A progressive
decrease in transaminase levels can represent either recovery from liver injury or
that there is very little tissue left from destruction. Thus, the interpretation of the
transaminase levels should be made in the light of other liver function tests.
In this patient's case, a rise in PT with a decrease in the transaminase levels is
therefore indicative of very little functional tissue remaining in the liver, which can
happen so rapidly with fulminant hepatic failure.
Choice B: This would lead to an increase in the transaminase levels due to
additional hepatic tissue injury by HDV.
Choice C: A sign of recovery would rather be a decrease in the transaminase
levels with an improvement in PT.
Choice D: Coexisting Hepatitis C can only be confirmed by serological tests for
HCV and not by aminotransferase levels and PT.
Choice E: The diagnosis of hepatocellular carcinoma requires the performance
of a liver biopsy and would be suggested by an increase in the alpha-fetoprotein
levels.
Educational Objective:
Liver disease should be evaluated on the basis of tests to assess both the
function and the structural integrity of the liver.
39% of people answered this question correctly
Test #24 Q.Id = 814
Question 30 Of 39
Division : Hepatology
Item Review
A 52-year-old alcoholic male comes with fatigue and abdominal distention. On
examination he has spider nevi on his upper torso and has abdominal shifting
dullness. His liver is 4cm below the left costal margin and his spleen is palpable.
Which of the following drugs is the best initial treatment for ascites in this
patient?
A. Furosemide
B. Thiazide
Spironolacton
C.
e
D. Triamterene
E. Mannitol
Explanation:
Question 31 Of 39
Division : Hepatology
Item Review
A 52-year-old female presents with the chief complaints of marked itching and
fatigue. On physical examination she has hepatomegaly, with bilateral
xanthelasma and skin excoriations. She has no scleral icterus. Her laboratory
profile shows alkaline phosphatase of 400 IU/L; bilirubin 1.2 mg/dl; AST 120 IU/L;
ALT 130 IU/L; Serum cholesterol 400 mg/dl and elevated IgM fraction. The liver
biopsy shows destruction of the bile ducts in the portal triad. Which of the
following is most likely to be present in this patient's serum?
Anti mitochondrial
antibodies
B. Anti nuclear antibodies
Anti smooth muscle
C.
antibodies
D. Anti smith antibodies
E. P-ANCA.
Explanation:
A.
This patient has primary biliary cirrhosis. Primary biliary cirrhosis usually
presents in middle age and 90 percent of patients are females. Pruritus is
generally the first symptom and is very severe, especially at night. It is
characterized by markedly elevated serum cholesterol levels, alkaline
phosphatase, IgM, and minimally elevated aminotransferases. Jaundice and
osteopenia occur in later stages of disease.
Primary biliary cirrhosis may be associated with Sjgrens disease, RA, and
CREST syndrome. Liver biopsy is pathognomonic, with granulomatous
destruction of bile ducts in portal triad. The presence of anti mitochondrial
antibodies (Choice A) is characteristic, and is found in 90% of patients.
Ursodeoxycholic acid decreases the pruritus and slows the rate of progression.
The definitive treatment is liver transplantation, which has a 5yr survival rate of
more than 70%.
Choice B: These are seen in patients with lupus erythematosus and Type 1
autoimmune hepatitis.
Choice C: These are seen in patients with Type 1 autoimmune hepatitis.
Choice D: These are highly specific ANA for the diagnosis of Lupus
Erythematosus.
Choice E: These are generally seen in polyarteritis nodosa (PAN) and
microscopic polyangiitis.
Educational Objective:
Anti mitochondrial antibodies are present in 90% of patients with primary biliary
cirrhosis.
68% of people answered this question correctly
Test #24 Q.Id = 855
Question 32 Of 39
Division : Hepatology
Item Review
A 65-year-old alcoholic male was diagnosed with cirrhosis. He has had 2
episodes of hematemesis and had sclerotherapy done. He has minimal ascites
at present and is on spironolactone, as well as a salt restricted diet. He has 2+
peripheral edema, but no asterixis or confusion. He now develops severe
epistaxis, bleeding from the intravenous sites, and bloody diarrhea. Which of the
following would be the treatment of choice for his bleeding disorder?
A. Give him vitamin K.
B. Give him fresh frozen plasma.
Give him whole blood
C.
transfusion.
Question 33 Of 39
Division : Hepatology
Item Review
A 52-year-old male patient presents to you with chronic fatigue. He is not taking
any medication and is not drinking alcohol. He complains of occasional blood in
his stools. His past medical history is insignificant, except for 4 units of blood
transfusion in 1982 after bleeding heavily in a road traffic accident. A diagnosis of
anemia secondary to diverticular disease is made in this patient. Apart from the
treatment of diverticulosis and anemia which of the following screening test
would you like to perform?
A. Screening for HIV infection.
Screening for hepatitis B and
B.
C.
C. Screening for hepatitis C.
D. Screening for hepatitis B.
Question 34 Of 39
Division : Hepatology
Item Review
A 42-year-old Asian male presents with fever, yellow urine, and peripheral
edema. He is known to have ulcerative colitis for 4 years and has had
intermittent flare-ups with bloody diarrhea. He is a non-alcoholic but smoked
cigarettes 1pack/day since he was 11 year old. On examination, he has icteric
sclerae and 2 + peripheral edema. His liver margin is 4cm below the costal
margin in the right mid-clavicular line and the tip of his spleen is palpable.
His laboratory reports show Hb: 12.5 mg/dl, WBC: 14,000/cmm, Platelets:
250,000/cmm, total bilirubin: 4.5 mg/dl, alkaline phosphatase: 1300 IU/L, ALT:
150 IU/L, AST: 160 IU/L, PT: 13 seconds, serum albumin: 2.0 g/dl, negative viral
serology, and negative autoimmune screen. An ultrasonogram of the abdomen
shows minimal ascites and focal dilatations in the biliary tree. An ERCP is done
which shows multiple dilatations of the biliary tree with a prominent stricture at
the junction of cystic duct and hepatic duct. What is the next best step in the
management of this patient?
A. Give prednisone and sulfasalazine.
Refer the patient for liver
B.
transplantation.
Perform a biopsy of the dominant
C.
stricture.
D. Give ursodeoxycholic acid.
E. CT scan of abdomen.
Explanation:
This patient has ulcerative colitis and his labs are consistent with cholestatic liver
disease. Peripheral edema, hepatosplenomegaly, minimal ascites,
hypoproteinemia are suggestive of early cirrhosis of liver. Cholestatic cirrhosis in
the context of ulcerative colitis is highly suggestive of primary sclerosing
cholangitis as in this patient's case. The ultrasonogram (USG) and ERCP
findings of multiple dilatations of biliary tree support the diagnosis of primary
sclerosing cholangitis (PSC).
Cholangiocarcinoma is a complication of PSC especially in smokers with
concurrent ulcerative colitis. One of the early manifestations of
cholangiocarcinoma is a severe stricture in the biliary tree leading to cholangitis
characterized by fever, jaundice, and leucocytosis. Cholangiocarcinoma can
occur in up to 20% of patients with long term PSC, so any severe stricture of
biliary tree in patients with PSC should be biopsied to rule out
cholangiocarcinoma. (Choice C)
Choice A: Prednisone and sulfasalazine are the treatment of choice for a flare
up of ulcerative colitis; however this patient is not having a acute flare up of
ulcerative colitis at present.
Choice B: Primary sclerosing cholangitis is a chronic progressive disease and
may eventually need a liver transplantation; however cholangiocarcinoma is a
relative contraindication for liver transplantation. Thus it should be ruled out
before referring the patient for transplantation.
Choice D: Ursodeoxycholic acid can be used in patients of primary sclerosing
cholangitis to lower the liver enzymes. However, its role in prevention of cirrhosis,
cholangiocarcinoma or in delaying the need for transplantation is not known.
Choice E: A CT scan of abdomen can be done to look for mass lesions in the
liver or pancreas, however both the ultrasonogram and the ERCP in this patient
are indicative of PSC in which case the CT won't be helpful.
Educational Objective:
Cholangiocarcinoma can complicate primary sclerosing cholangitis especially in
presence of ulcerative colitis and smoking.
14% of people answered this question correctly
Test #24 Q.Id = 842
Question 35 Of 39
Division : Hepatology
Item Review
A 44-year-old alcoholic male comes with the complaints of anorexia and fatigue.
On examination, he has yellow sclera, some spider angiomas on his chest, caput
medusae, and hepatosplenomegaly. The ultrasonogram (USG) of the abdomen
shows nodularity of the liver, hepatosplenomegaly, and minimal ascitic fluid. His
laboratory reports shows total bilirubin: 5.8 mg/dl, AST: 400 IU/L, ALT: 200 IU/L,
GGT: 250 IU/L, and PT: 13 seconds. Which of the following investigations would
you like to order next, in this patient?
Esophageal
endoscopy.
B. Proctoscopy.
Serum ammonia
C.
levels.
D. Portal vein pressure.
E. CT scan of abdomen.
Explanation:
A.
This patient has signs and symptoms suggestive of alcoholic cirrhosis. The
jaundice, spider angiomas, caput medusae, hepatosplenomegaly,
ultrasonographic nodularity of liver, minimal ascites, and elevated transaminases
and GGT are all consistent with alcoholic cirrhosis. Portal hypertension in this
patient is suggested by caput medusae and splenomegaly. Patients with cirrhosis
develop esophageal varices secondary to PHT and oftentimes, severe
hematemesis is the presenting symptom of esophageal varices, therefore all
patients with cirrhosis of the liver should be screened for esophageal varices with
an endoscopy (Choice A). Primary prophylactic treatment with beta-blockers, (in
the case of esophageal varices, which have never bled) can significantly
decrease the risk of bleeding.
Choice B: Proctoscopy in cirrhotic patients is performed to diagnose piles. Piles
can occur in patients with cirrhosis secondary to portal HTN but they are much
less common than esophageal varices.
Choice C: Serum ammonia level can be useful to diagnose hepatic
encephalopathy; however this patient has no signs or symptoms suggestive of
hepatic encephalopathy.
Choice D: Portal vein pressure can be measured to diagnose portal
hypertension, however in this patient screening for varices is a priority over
measuring portal vein pressure.
Choice E: CT scan of the abdomen would be an choice if you were expecting to
find hepatocellular carcinoma.
Educational Objective:
Patients with cirrhosis should have an endoscopy to screen for varices.
18% of people answered this question correctly
Test #24 Q.Id = 851
Question 36 Of 39
Division : Hepatology
Item Review
A 12-year-old American boy comes to you with the complaints of abdominal pain
and yellow urine. On examination he has normal vitals, icteric sclerae, and
abdominal tenderness with a mass palpable in right upper quadrant. His
laboratory report shows bilirubin: 6.5 mg/dl, negative viral serologies and mild
elevations of amylase and lipase. Ultrasonography shows a cystic extra hepatic
mass and a gall bladder separated from the mass. What is the most likely
diagnosis in this patient?
A. Carolis syndrome.
B. Choledochal cyst.
C. Biliary abscess.
D. Biliary atresia.
Pseudo pancreatic
E.
cyst.
Explanation:
A choledochal cyst is a congenital abnormality of the biliary ducts characterized
by the dilatation of intra or extra-hepatic biliary ducts or both. It has a
multifactorial origin, but most of the cases are related to an anomalous
pancreaticobiliary junction, which leads to weakness and dilatation of the biliary
wall due to the reflux of alkaline pancreatic secretions into the biliary tree
(Choice B). Various types of choledochal cysts are:
Type 1: Most common type, features the dilatation of the entire common hepatic
and common bile ducts or segments of each.
Type 2: Relative isolated protrusions or diverticulae from the common bile duct
wall.
Type 3: Cyst found in the intraduodenal part of common bile duct.
Type 4: Multiple dilatations in the intra and extra hepatic biliary tree.
Type 5: Isolated dilatation of intrahepatic bile ducts.
The clinical presentation varies with the age. In infants it may present with
jaundice and the passage of acholic stools. In children it causes abdominal pain,
jaundice, and attacks of recurrent pancreatitis, which may be evident by
increases in the amylase and lipase levels. Adults with choledochal cysts
commonly present with vague epigastric or right upper quadrant abdominal pain
or cholangitis. Choledochal cysts can degenerate into cholangiocarcinoma. The
initial investigation of choice is an ultrasonogram followed by CT scan or
magnetic resonance imaging (MRI) as needed.
Choice A: Carolis syndrome is a congenital disorder characterized by
intrahepatic dilatation of bile ducts.
Choice C: Biliary abscess will generally present with clinical signs of fever,
leucocytosis, and the patients are usually severely ill.
Choice D: Biliary atresia usually presents early in the infancy with marked
obstructive jaundice and acholic stools.
Choice E: Pseudo pancreatic cyst is a complication of acute or chronic
pancreatitis characterized by retroperitoneal/epigastric mass and high amylase.
However, it will rarely cause a right upper quadrant mass and very mild jaundice
by compressing the common bile duct. It is easily diagnosed by ultrasonogram.
Educational Objective:
Choledochal cysts are congenital abnormalities of the biliary tree characterized
by dilatation of the intra and/or extra hepatic biliary ducts.
49% of people answered this question correctly
Test #24 Q.Id = 879
Question 37 Of 39
Division : Hepatology
Item Review
A 45-year-old female undergoes an elective cholecystectomy for gallstones
causing recurrent severe right upper quadrant abdominal pain. However, even
after the surgery her pain does not resolve and she experiences similar kind of
pain. During an acute pain episode, her laboratory reports show ALT: 80 IU/L,
AST: 70 IU/L, alkaline phosphatase: 450 IU/L, and total bilirubin: 1.6 mg/dl.
Ultrasonogram of abdomen and ERCP are done and show normal bile ducts and
Question 38 Of 39
Division : Hepatology
Item Review
A 52-year-old patient comes to ER with severe vomiting, fever and abdominal
pain. On examination he has epigastric tenderness and xanthomas. His
laboratory report shows WBC: 18,000/cmm, normal amylase and elevated lipase,
serum triglycerides: 4,000 mg/dl, AST: 120 IU/L, ALT: 50 IU/L, and alkaline
phosphatase: 120 IU/L. The patient is not taking any medication, except for
naproxen (NSAID) for arthritis for the past 3 years. He occasionally drinks a can
of beer on weekends. His past medical history is insignificant except for an
elective cholecystectomy 8 years ago for recurrent abdominal pain. He says that
his father had a lipid disorder and died of a heart attack. What is the most likely
cause of pancreatitis in this patient?
A. Gall stone pancreatitis.
Drug induced
B.
pancreatitis.
C. Hypertriglyceridemia.
D. Alcohol induced.
E. Idiopathic.
Explanation:
Acute pancreatitis is most commonly secondary to gallstones or alcohol abuse.
However, it may be also secondary to drug toxicity, idiopathic etiology,
hypertriglyceridemia, infection, severe trauma or burns, hypercalcemia,
pancreatic abnormality, tumors, toxins or due to post operative or post ERCP
etiology.
This patient is not a big time alcoholic and had his gallbladder removed long time
ago so thus the two most common causes are unlikely. However, he has
markedly elevated triglyceride levels, along with a positive family history of early
cardiovascular disease and xanthomas making the hypertriglyceridemia the most
likely cause of his pancreatitis. Triglyceride levels > 1000 mg/dl is a risk factor for
acute pancreatitis and during the attack the triglyceride levels can be as high as
3000-5000 mg/dl. Though acute pancreatitis itself can cause an increase in
triglycerides it is usually not so high. Hyperlipidemia types 1 and 5 are associated
with acute pancreatitis (Choice C). Acute pancreatitis secondary to
hypertriglyceridemia often has normal levels of amylase.
Choice A: The patient had his gallbladder removed 8 years ago. Also, in patients
with pancreatitis due to gallstone the alkaline phosphatase is more than 150
IU/L.
Choice B: NSAIDs in general do not cause acute pancreatitis. Some important
drugs causing acute pancreatitis are azathioprine, sulfonamides, sulindac,
tetracycline, valproic acid, didanosine, methyldopa, estrogens, furosemide, 6mercaptopurine, pentamidine, 5-aminosalicylic acid, corticosteroids, and
octreotide.
Choice D: This patient drinks alcohol only occasionally and it usually takes more
than that to cause acute pancreatitis.
Choice E: Idiopathic pancreatitis is seen in about 10% of cases, however the
diagnosis is made only after other causes have been ruled out.
Educational Objective:
Question 39 Of 39
Division : Hepatology
Item Review
A 33-year-old female presents with anorexia, nausea, and yellow urine for one
week. On examination, she has yellow sclera, spider nevi over upper torso,
choreiform movements, and greenish brown deposits around her cornea. Liver is
enlarged and the edge is irregular. Her laboratory reports show: Total bilirubin 4.5
mg/dl, AST 350 IU/L, ALT 400 IU/L, PT 13 sec. What is the most likely diagnosis
in this patient?
A. Hemochromatosis.
B. Wilsons disease.
C. Acute viral hepatitis.
D. Alcoholic hepatitis.
Hepatic
E.
encephalopathy.
Explanation:
Patient is having insidious onset of complains of yellow urine with nausea along
with mild increase in aminotransferases, spider nevi, irregular hepatomegaly, and
normal PT, suggestive of compensated cirrhosis.
The presence of cirrhosis in a young patient with choreiform movements and the
finding of a Kayser Fleischer ring (yellow brown pigmentation of the cornea) are
highly suggestive of Wilsons disease. (Choice B)
Wilsons disease is a rare autosomal recessive disease characterized by low
ceruloplasmin levels, leading to abnormal copper deposition in liver, basal
ganglia (hepatolenticular degeneration), and cornea. Liver involvement in
Wilsons disease can be in the form of fulminant hepatitis, chronic hepatitis, or
macro nodular cirrhosis. Wilsons disease also causes hemolytic anemia and
neuropathy. The diagnosis is made by the finding of low serum ceruloplasmin
levels, increased urinary copper and hepatic copper deposition on liver biopsy.
Treatment consists of copper chelators like d-penicillamine. Fulminant hepatitis
requires liver transplantation.
Choice A: Hemochromatosis is an autosomal recessive disease characterized
by abnormal iron metabolism and can result in increased skin pigmentation,
decreased libido, diabetes, and liver involvement.
Choice C: Acute viral hepatitis would not lead to choreiform movement and the
serum transaminases will be much higher.