An 8-month-old girl is seen in the emergency department because her parents

are concerned about her very listless behavior and the fact that
her skin has a yellow discoloration. The child is the daughter of Vietnamese
immigrants to the United States, and has received no medical
care since her birth. On physical examination, the child is noted to be jaundiced.
The head shows prominence of the mandible, maxillary
overbite eminences, and frontal bossing. Hepatosplenomegaly is present. BIood
studies demonstrate a hemoglobin of 6.8 g/dL. A peripheral
blood smear shows predominantly a microcytic hypochromic anemia, although a
wide variety of red cell changes are noted by the examining
technician, including anisocytosis, poikilocytosis, target cells, ovalocytes,
basophilic stippling, polychromasia, macrocytes, and nucleated red
cells.
Question 1 of 5

An x-ray film of her head shows thinned cortices with widened marrow spaces of
the bones of the skulI. This would most likely be due to which
of the following processes?
/A. Bony tumor
/B. Cartilaginous tumor
/C. Genetic abnormality of bone development
/D. Hyperparathyroidism
/E. Marrow expansion
Explanation - Q: 1.1

Close

The correct answer is E. When you see thinned cortices with widened
marrow spaces, you should think of processes that can cause marrow
expansion, such as leukemias and hemolytic anemias.
Bony and cartilaginous tumors (choices A and B) would be more likely to
produce masses visible on x-ray.
Genetic abnormalities of bone development (choice C), such as
osteogenesis imperfecta, may produce bones with abnormal patterns of
calcification and evidence of multiple fractures, but do not usually produce
thinned cortices with widened medullary spaces.
Hyperparathyroidism (choice D) can cause lytic bone lesions, but does not
usually cause diffuse thinning of bone.
Question 2 of 5

This patient's jaundice is most likely due to which of the following?

/A. Gallstones
/B. Hepatitis A

/C. Hepatitis B
/D. Hepatitis C
/E. Hemolytic anemia

Explanation - Q: 1.2

Close

The correct answer is E. While many associate jaundice with liver disease,
you need to remember that the hemolytic anemias also cause jaundice (and
can cause hepatosplenomegaly secondary to extramedullary hematopoiesis).
Pigmented gallstones (choice A) related to excessive excretion of the heme
degradative product bilirubin can be seen in patients with chronic hemolytic
anemia, but would be very unusual in a young child.
None of the information in this patient's history and clinical examination
except the jaundice and hepatosplenomegaly suggests that she has hepatitis
(choices B, C, and D); more specifically, the marked anemia and marrow
expansion of the cranium would not be seen in hepatitis.
Question 3 of 5

Which of the following conditions is most likely the cause of the patient's
peripheral blood smear findings?
/A. B12 deficiency
/B. Folate deficiency
/C. Iron deficiency
/D. Sickle cell anemia
/E. Thalassemia
Explanation - Q: 1.3

Close

The correct answer is E. The severe anemia and very complex peripheral
smear pattern is most consistent with severe thalassemia. Milder cases of
thalassemia may resemble either iron deficiency (choice C) with microcytic
cells, or folate or vitamin B12 deficiencies (choices A and B) with macrocytic
cells. In these milder cases, the wide variety of red cell shapes and profound
anemia seen in severe cases are not present, and the diagnosis of
thalassemia is usually made after a failure of iron, folate, or B12 therapy to
correct the anemia.
Sickle cell anemia (choice D) would show sickled cells on peripheral smear.
Question 4 of 5

Hemoglobin electrophoresis studies are ordered, and HbF is found to be the

predominant form, with only very small amounts of other

hemoglobins present. This is most consistent with which of the following

underlying genetic defects?
/A. 1 defective alpha globin chain gene
/B. 1 defective beta globin chain gene
/C. 2 defective alpha globin chain genes
/D. 2 defective beta globin chain genes
/E. 3 defective alpha globin chain genes
Explanation - Q: 1.4

Close

The correct answer is D. The thalassemias are a group of chronic, inherited

blood diseases that are characterized by defective hemoglobin synthesis and
resultant ineffective erythropoiesis. Severe cases (thalassemia major) tend to
present as illustrated in the case history by 12 months of age; intermediate
cases (thalassemia intermedia) present at 2-4 ages; and milder cases
(thalassemia minor and thalassemia minima) may be completely or nearly
asymptomatic throughout life. The hemoglobin molecule contains two alpha
chains (coded by 4 copies of the gene on 2 chromosomes) and two beta
chains (coded by 2 copies of the gene on 2 chromosomes). Thalassemia can
be produced by either defective alpha chain or defective beta chain
production. The fact that the child can make fetal hemoglobin, HbF, means
that he is able to make alpha chains (thereby excluding choices A, C, and
E), which occur in both adult and fetal hemoglobin. The child has severe,
rather than mild, disease (thalassemia major) and is not making normal
hemoglobin. This means that she most likely has 2 defective beta globin
chain genes rather than 1 (choice B). If you encounter a question similar to
this on the USMLE, you should analyze it as illustrated above; however, for
your own information, a patient with 2 defective beta globin genes and 1
defective alpha globin gene might have a similar electrophoresis pattern. The
thalassemias are found predominantly in the equatorial belt corresponding to
the distribution of malaria (thalassemia trait has been postulated to be
protective against falciparum malaria). Immigration from the Indian
subcontinent and Southeast Asia (including Vietnam) is increasing the
number of cases of thalassemia found in the United States; the earlier US
pool of thalassemia had been dominated by Italian immigrants and those from
other countries near the Mediterranean Sea.
Question 5 of 5

This child will require life-Iong transfusions to correct the otherwise fatal anemia,
which may cause death either due to anemia itself or due to
septicemia. The child is consequently at severe risk of developing iron overload,
which also has potentially severe to fatal complications. To
reduce the rate at which iron overload occurs, the child should be treated, after
the age of 3, with nightly subcutaneous infusions of which of the

following?
/A. Desferrioxamine
/B. Dimercaprol
/C. Edetate calcium disodium
/D. Penicillamine
/E. Succimer
Explanation - Q: 1.5

Close

The correct answer is A. Desferrioxamine is given intravenously as a

chelating agent to treat severe iron load, and subcutaneously, with slow
infusion (over 8 hours nightly via a small pump) in milder overload cases. The
use of this drug markedly extends the life span of children with thalassemia
major (who otherwise typically die before age 10), but is very problematic for
these young children and their families because the nightly prolonged
injections are painful. There is a great deal of interest in developing a safe
oral iron chelating agent, but none is yet available in this country and agents
in use in other countries have had severe toxicity problems. We have already
extended these children's life-span up to 30 or 40 years, and no one yet
knows how far we will be able to go as better agents and/or delivery systems
become available. The other choices listed are also chelating agents, but do
not work with iron.
Dimercaprol (choice B) is used to treat a variety of poisonings with antimony,
arsenic, chromium derivatives, bismuth, copper, gold, mercury, nickel,
tungsten, and zinc.
Edetate calcium disodium (choice C) is used to treat a variety of metal
poisonings with cadmium, chromium, copper, lead, manganese, nickel,
radium, selenium, tungsten, uranium, vanadium, and zinc.
Penicillamine (choice D) is used to treat a variety of poisonings with
chromium derivatives, cadmium, cobalt, copper, lead, mercury, nickel, and
zinc.
Succimer (choice E) is used to treat poisonings with lead, arsenic, and
mercury.
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A 35-year-old woman is evaluated for jaundice in an emergency department. For

several days, the patient has had mild flu-Iike symptoms of
anorexia, nausea and vomiting, fatigue, Iow-grade fever, and malaise. This
morning, she noted that her urine was brown in color, and she has
also today developed moderate, steady, pain of the right upper quadrant of her
abdomen. She has not had any similar episodes in the past.

On physical examination, the patient is noted to be jaundiced and to have an

enlarged, tender liver. BIood chemistry studies are notable for
alanine aminotransferase (ALT) of 15,000 mIU/L, aspartate aminotransferase
(AST) of 11,000 mIU/L, and alkaline phosphatase of 100 U/L.
Question 1 of 6

This patient's dark urine is due to the presence of which of the following?
/A. Bacteria
/B. Bilirubin
/C. Hemosiderin
/D. Ketone bodies
/E. Melanin
Explanation - Q: 2.1

Close

The correct answer is B. Darkly discolored urine due to the presence of

bilirubin (a degradative product of heme that is normally excreted via bile into
feces) may precede obvious jaundice in patients with acute hepatitis.
Bacteria (choice A) can cause urine to become cloudy or whitish.
Hemosiderin (choice C) can also cause brown discoloration in urine, but
would be seen in a setting in which urinary tract hemorrhage was present.
Ketone bodies (choice D), a feature of diabetic ketoacidosis, do not cause
urine discoloration.
Melanin (choice E) is a black pigment that is rarely spilled into the urine in
patients with metastatic melanoma.
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Question 2 of 6

Which of the following is the most likely diagnosis?

/A. Acute hepatitis
/B. Chronic hepatitis
/C. Gallstone disease
/D. Hepatic cirrhosis
/E. Wilson disease
Explanation - Q: 2.2

Close

The correct answer is A. This person's markedly elevated AST and ALT with
modest elevation of alkaline phosphatase strongly suggests that she has
acute hepatitis. The clinical presentation with flu-like symptoms that progress
to jaundice is also typical.
Chronic hepatitis (choice B) and cirrhosis (choice D) would present more

insidiously and would not have the extremely high elevations of AST and ALT.
Gallstone disease (choice C) can cause acute abdominal pain, and
occasionally jaundice (if a small stone occludes the common bile duct), but
would not usually cause the very high elevations of AST and ALT seen in this
patient.
Wilson disease (choice E) in adults usually causes a chronic hepatitis that
may progress to cirrhosis.
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Question 3 of 6

The patient has recently returned from a 6-month tour working with infants and
young children in a daycare facility in southern Mexico. During
the past two years, she has not had any sexual encounters, has not used drugs,
and has not received blood products. Which of the following
best describes the most likely pathogen?
/A. Enveloped, defective circular RNA virus
/B. Enveloped, DNA virus in the Hepadnavirus family
/C. Enveloped, RNA virus in the FIavivirus family
/D. Naked capsid, RNA virus in the Calicivirus family
/E. Naked capsid, RNA virus in the Picornavirus family
Explanation - Q: 2.3

Close

The correct answer is E. Viral infection is an important cause of acute

hepatitis, and in this case, the individual's history of exposure to infants in an
area endemic for hepatitis A is very suggestive. Unlike many of the hepatitis
viruses, hepatitis A is spread through a fecal-oral route. The patient's history
also excludes other probable routes of spread, such as through sexual
transmission, use of intravenous drugs, or use of blood products. The
diagnosis can be confirmed by serum measurement of anti-hepatitis A
immunoglobulin M. The hepatitis A virus is an unencapsulated, singlestranded, positive sense, linear RNA enterovirus in the Picornavirus family.
The virus replicates (apparently exclusively) in hepatocytes and is excreted
via bile into the stool.
Enveloped, defective circular RNA virus (choice A) describes hepatitis D.
Hepatitis D, also called delta hepatitis, is a defective hepatitis virus that must
coinfect or superinfect with hepatitis B to cause disease. It can cause severe
acute hepatitis, but is unlikely in this patient because the usual routes of
spread are parenteral or sexual.

Enveloped, DNA virus in the Hepadnavirus family (choice B) describes

hepatitis B, while enveloped RNA virus in the Flavivirus family (choice C)
describes hepatitis C. Hepatitis B and hepatitis C are usually spread by
parenteral or sexual routes, and usually do not cause severe acute hepatitis.
Naked capsid, RNA virus in the Picornavirus family (choice D) describes
hepatitis E. Hepatitis E is spread by the fecal-oral route, but usually causes
mild disease unless the patient is pregnant.
Question 4 of 6

What percentage of adults who develop this infection have symptomatic

disease?
/A. 10%
/B. 25%
/C. 50%
/D. 75%
/E. 95%
Explanation - Q: 2.4

Close

The correct answer is D. In adults who acquire hepatitis A infection,

symptomatic cases are common (75%) and most of these symptomatic adults
develop jaundice.
Question 5 of 6

What percentage of children less than 2 years of age who develop this infection
have symptomatic disease?
/A. 10%
/B. 25%
/C. 50%
/D. 75%
/E. 95%
Explanation - Q: 2.5

Close

The correct answer is A. In marked contrast to the situation with adults, 90%
of children under the age of 2 who acquire hepatitis A infection (common in
endemic areas) are asymptomatic.

Question 6 of 6

Overdose with which of the following could also be responsible for this patient's
disease?
/A. Acetaminophen
/B. Aspirin
/C. Caffeine
/D. Cocaine
/E. Codeine
Explanation - Q: 2.6

Close

The correct answer is A. Severe acute hepatitis can be caused by

processes other than viral infection, including acute drug-induced liver injury
(notably acetaminophen), drug-induced hypersensitivity reactions (notably
sulfasalazine hypersensitivity), and other viruses (CMV, EBV, and HIV).
Acetaminophen overdose can cause dose-dependent, potentially fatal hepatic
necrosis that can mimic the fulminant hepatitis of severe hepatitis A infection.
Other serious side effects of acetaminophen overdose include renal tubular
necrosis, hypoglycemic coma, and thrombocytopenia.
Aspirin (choice B) overdose can cause early CNS overstimulation followed
by coma, respiratory failure, and severe electrolyte disturbances.
Caffeine (choice C) overdose can cause CNS stimulation, difficulty breathing,
arrhythmias, and GI symptoms.
Cocaine (choice D) overdose can cause tremors, convulsions, delirium, and
arrhythmias.
Codeine (choice E) overdose can cause muscle spasticity, respiratory failure,
coma, and shock.
A 37-year-old man is evaluated by a family practitioner because of jaundice. On
physical examination, the man is noted to have an enlarged
Iiver with an irregular edge on palpation. UItrasound examination shows diffuse
echogenicity and nodularity of the liver without specific discrete
masses.
Question 1 of 7

Which of the following is the most likely diagnosis?

/A. Acute hepatitis
/B. Chronic persistent hepatitis
/C. Cirrhosis
/D. Hepatocellular carcinoma

/E.

Steatosis
Explanation - Q: 3.1

Close

The correct answer is C. The diffuse echogenicity and nodularity without

specific discrete masses are typical of a cirrhotic liver, as is the irregular liver
edge. Cirrhotic livers may be enlarged, or smaller than normal, depending
upon the degree to which regeneration is occurring.
Acute hepatitis (choice A) can cause an enlarged, tender liver, but does not
cause nodularity.
Chronic persistent hepatitis (choice B) is an inflammation of the portal areas
seen in some chronic cases of viral hepatitis that, by definition, does not
progress to cirrhosis, and consequently does not show nodularity.
Hepatocellular carcinoma (choice D) can complicate cirrhosis, but is unlikely
in this particular case, because the liver does not contain specific discrete
masses.
Steatosis (choice E), or fatty liver, does not cause nodularity of the liver.
Question 2 of 7

Serum serology studies demonstrate the following:

IgG anti-HAV: +
IgM anti-HAV:
IgM HBcAb:

IgG HBcAb:
+
HBsAg:
+
HBeAG:
+
HBV DNA:
+
anti-HCV:

This suggests that this patient is presently infected with which of the following?
/A. Both hepatitis A and hepatitis
/B. Both hepatitis A and hepatitis
/C. Hepatitis A virus only
/D. Hepatitis B virus only
/E. Hepatitis C virus only

B viruses
C viruses

Explanation - Q: 3.2

Close

The correct answer is D. This patient is presently infected with hepatitis B

virus, and was formerly exposed to hepatitis A. He has not been exposed to
hepatitis C. The hepatitis antibodies and antigens tend to be somewhat
confusing, but can be sorted through with a little care. "Ag" on the end of the
abbreviation indicates an antigen, while "Ab" indicates an antibody formed
against the antigen. Anti-HAV, or antibodies to Hepatitis A virus, can be either
in IgM form (indicating recent infection) or IgG form (indicating past infection).
Hepatitis A virus does not cause chronic infection or cirrhosis, so the patient is
not currently infected with this virus. Hepatitis B serology is complex. There
are 3 significant antigens: "c"- the core antigen, "e" - the e antigen also found
in the core of the virus, and "s"- the surface antigen. Chronic hepatitis,
including cirrhosis, due to hepatitis B, is usually characterized by persistent
circulating HBsAg, HBeAg, and HBV DNA. There are also usually HBcAb
(antibodies to core antigen), often in the IgG form. Hepatitis C exposure is
indicated by the presence of HCV antibodies, which this patient does not
have.
Question 3 of 7

What percentage of adults who acquire this patient's current viral infection(s)
develop a chronic infection?
/A. Less than 5%
/B. 10-20%
/C. 30-50%
/D. 60-70%
/E. 90% or more
Explanation - Q: 3.3

Close

The correct answer is A. Hepatitis B infection can take a variety of forms.

The infection usually begins as a clinical or subclinical acute infection that
may resolve completely, persist with little accumulating damage, or progress
to hepatic cirrhosis. In adults, less than 5% of individuals who become
infected develop chronic disease, with risk of eventual development of
cirrhosis. The virus is spread through contaminated blood and body fluids.
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Question 4 of 7

Approximately what percentage of children who acquire hepatitis B in the

perinatal period develop a chronic infection?
/A. 5%
/B. 20%
/C. 50%
/D. 70%

/E.

90%
Explanation - Q: 3.4

Close

The correct answer is E. While the rate at which adults with new hepatitis B
infection develop a chronic infection is less than 5%, that of neonates is 90%,
and that of children 1-5 years of age is 20-50%. The neonates usually acquire
the infection through vertical transmission from the mother.
Question 5 of 7

Which of the following is a defective virus that requires this patient's virus(es) for
propagation?
/A. Cytomegalovirus
/B. Hepatitis D virus
/C. Hepatitis E virus
/D. Herpes simplex l
/E. Herpes simplex ll
Explanation - Q: 3.5

Close

The correct answer is B. The hepatitis D virus is a defective hepatitis virus

that can coinfect or superinfect hepatitis B liver disease, causing more severe
disease with increased risk of progression to chronic disease and cirrhosis.
The other viruses listed can infect the liver, but do not require coinfection with
hepatitis B to cause disease.
Question 6 of 7

Which part of this patient's virus(es) is specifically required by the defective virus
that uses it for propagation?
/A. HBcAb
/B. HBcAg
/C. HBeAg
/D. HBsAb
/E. HBsAg
Explanation - Q: 3.6

Close

The correct answer is E. Hepatitis D can replicate independently within the

liver cell, but requires the presence of hepatitis B surface antigen (HBsAg) for
release of the virus particles in an infective form.
HBcAb (choices A) and HBsAb (choice D) are antibodies to antigens, rather

than antigens themselves, and do not participate in the hepatitis D life cycle.
The hepatitis B core and e antigens (choices B and C) are not apparently
required.
Question 7 of 7

Which of the following agents blocks viral reverse transcriptase and can be used
to treat this patient's infection?
/A. Amantadine
/B. Lamivudine
/C. Oseltamivir
/D. Prednisone
/E. Zanamivir
Explanation - Q: 3.7

Close

The correct answer is B. Formerly, hepatitis B infection was a very

frustrating disease because almost no effective therapy existed. Alpha
interferon was one of the first successful therapies for chronic hepatitis B
infection, and has been extensively used since the mid-1980's, although
unfortunately, the therapy response rate is only 30-40%. The thymidine
analog lamivudine is used in treatment of chronic hepatitis B infection, and
acts by blocking viral replication by competitive inhibition of viral reverse
transcriptase. This and other new similar antiviral agents (e.g., famciclovir,
lobucavir, adefovir dipivoxil) offer the promise of much better control of
hepatitis B infection, since these agents directly block the propagation of
hepatitis B.
The anti-viral agent amantadine (choice A) is used in treatment of hepatitis C
and acts by inhibiting uncoating of the virus.
The corticosteroid prednisone (choice D) is sometimes used in treatment of
cholestatic hepatitis A infection.
Zanamivir (choice E) and oseltamivir (choice C) are active against influenza
A and B viruses, and act by preventing clumping of virions, thereby reducing
the likelihood that the virus will penetrate infected cells.
Routine physical examination of a 19-year-old man demonstrates mild jaundice
and scleral icterus. On questioning, the patient reports he feels
welI. He says he occasionally gets a slightly yellowish tinge to his skin and eyes,
but the yellow seems to go away spontaneously after several

days. Screening chemistry studies are remarkable only for serum bilirubin of 2
mg/dL. On further evaluation, this bilirubin is found to be
predominately unconjugated. Liver enzymes are not elevated, and a complete
blood count (CBC) is within normal limits. On questioning, the
man says he does not use alcohoI, has not had unprotected sex, and does not
feel ilI. Liver biopsy is unremarkable.
Question 1 of 7

Bilirubin is a degradative product of which of the following?

/A. Cholesterol
/B. GIycosaminoglycans
/C. Hemoglobin
/D. Melanin
/E. Steroid hormones
Explanation - Q: 4.1

Close

The correct answer is C. Bilirubin is derived from the degradation of the

heme moiety of hemoglobin. The heme ring is opened to produce biliverdin,
which is then converted to bilirubin. The other answers are distracters.
Question 2 of 7

Which of the following is the most likely diagnosis?

/A. Acute viral hepatitis
/B. Chronic viral hepatitis
/C. Gilbert syndrome
/D. Hemochromatosis
/E. Wilson disease
Explanation - Q: 4.2

Close

The correct answer is C. Gilbert syndrome is the most common inherited

(although the pattern of inheritance may be hard to define) cause of
unconjugated hyperbilirubinemia. Episodes of clinical jaundice in Gilbert
syndrome tend to develop when there is a physiologic stressor such as
dehydration, fasting, menstrual periods, stress, illness, or vigorous exercise.
Some patients have vague, mild symptoms of abdominal cramps, fatigue, and
malaise. Between episodes, the patients may have variable (but low) degrees
of hyperbilirubinemia, sometimes with occasional normal values for serum
bilirubin. The diagnosis of Gilbert disease is established by excluding other,
more serious, causes of hyperbilirubinemia. All of the other liver diseases
listed in the choices would have abnormal liver biopsies and elevated serum
liver enzymes.

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Question 3 of 7

What is the incidence of this patient's disease in the United States?

/A. Less than 0.001% of the population
/B. 0.02-0.05% of the population
/C. 0.4-0.5% of the population
/D. 3-7% of the population
/E. More than 15% of the population
Explanation - Q: 4.3

Close

The correct answer is D. Gilbert syndrome is very common, with an

incidence of 3-7% of people in the United States.
Question 4 of 7

The function of the defective enzyme in this patient's disease is which of the
following?
/A. Adding glucuronyl residues to bilirubin
/B. Converting bilirubin to urobilinogen
/C. Converting biliverdin to bilirubin
/D. Oxidation of a methane bridge in the porphyrin ring
/E. Removing the globin chains from verdoglobin
Explanation - Q: 4.4

Close

The correct answer is A. The biochemical defect in Gilbert syndrome is a

decreased activity of the bilirubin conjugating system that converts bilirubin to
a form more soluble in bile. The affected enzyme is called bilirubin-uridine
diphosphate glucuronyl transferase (bilirubin-UGT). This enzyme conjugates
bilirubin, producing the more soluble forms: bilirubin monoglucuronides and
bilirubin diglucuronides. This enzyme is one of a family that also conjugates
(and thus render more easily excreted) a variety of carcinogens, drugs,
hormones, and neurotransmitters. The other choices are other steps in the
degradation of hemoglobin.
Hemoglobin degradation begins with oxidation of a methane bridge in the
porphyrin ring (choice D) to form verdoglobin.
Next, the globin chains are removed from verdoglobin (choice E), producing
biliverdin, which is subsequently converted to bilirubin (choice C).
The bilirubin is then conjugated and excreted into the bile, and bacteria act on
it to produce urobilinogen (choice B).

Question 5 of 7

The long-term prognosis of people with this patient's disease is which of the
following?
/A. Life span shortened on average by 5 years
/B. Life span shortened on average by 10 years
/C. Life span shortened on average by 20 years
/D. Life span shortened on average by 30 years
/E. Normal life span
Explanation - Q: 4.5

Close

The correct answer is E. Patients with Gilbert syndrome have a normal lifespan because there is no associated morbidity or mortality. Because of the
lack of associated morbidity or mortality, it is now recommended that no
medications be used in treatment of this disease.
Question 6 of 7

If the patient had instead presented with a hereditary conjugated

hyperbilirubinemia and was found to have a black liver on laparotomy, which
of the following would be the most likely diagnosis?
/A. Crigler-Najjar syndrome type l
/B. Crigler-Najjar syndrome type ll
/C. Dubin-Johnson syndrome
/D. Gilbert syndrome
/E. Rotor syndrome
Explanation - Q: 4.6

Close

The correct answer is C. Dubin-Johnson syndrome is a rare benign form of

hereditary hyperbilirubinemia with asymptomatic jaundice that has the
unusual feature of causing the liver to be darkly pigmented as the result of
deposition of an intracellular melanin-like substance. This has occasionally
been disconcerting in the surgical suite, when a patient undergoing operation
for some other purpose is incidentally found to have a "black liver". The
hyperbilirubinemia of Dubin-Johnson syndrome is conjugated, rather than
unconjugated, like Gilbert and Crigler-Najjar syndromes (choices A, B, and
D).
Rotor syndrome (choice E) is another, rare, form of benign hereditary
conjugated hyperbilirubinemia that clinically resembles Dubin-Johnson
syndrome, but without the black liver.

Question 7 of 7

If the patient had instead died of a hereditary cause of hyperbilirubinemia in

infancy, which of the following would be the most likely diagnosis?
/A. Crigler-Najjar syndrome type l
/B. Crigler-Najjar syndrome type ll
/C. Dubin-Johnson syndrome
/D. Gilbert syndrome
/E. Rotor syndrome

Explanation - Q: 4.7

Close

The correct answer is A. All of the diseases listed are hereditary

hyperbilirubinemias, but only Crigler-Najjar syndrome type I causes death in
infancy. The affected babies have severe hyperbilirubinemia, and typically die
before 1 year of age of kernicterus (bilirubin damage to developing brain).
Fortunately, this is a rare disease.
Crigler-Najjar syndrome type II (choice B) is also rare, and features a
hyperbilirubinemia that is less severe than in Type I, but is still much more
marked than in Gilbert syndrome. These patients usually live into adulthood
without neurologic damage, and may at least partially respond to therapy with
barbiturates.
Dubin-Johnson syndrome, Gilbert syndrome, and Rotor syndrome (choices
C, D, and E) are all benign forms of hyperbilirubinemia.
A 27-year-old man presents to a dermatologist because his skin has become
chronically itchy. He has also been experiencing chronic,
progressive fatigue. On physical examination, no specific skin lesions are seen,
but the patient is noted to be mildly jaundiced. He is referred,
for further evaluation, to an internist specializing in liver disease. Serum
chemistry studies demonstrate elevated serum alkaline phosphatase
and bilirubin with minimally increased transaminases. The mitochondrial antibody
test is negative. Endoscopic retrograde cholangiography
demonstrates multiple short strictures and saccular dilatations involving the
intrahepatic and extrahepatic bile ducts.
Question 1 of 6

Which of the following is the most likely diagnosis?

/A. Ascending cholangitis
/B. Bile duct tumor
/C. Primary biliary sclerosis
/D. Primary sclerosing cholangitis

/E. Viral hepatitis

/F. Wilson disease

Explanation - Q: 5.1

Close

The correct answer is D. This presentation is typical for primary sclerosing

cholangitis, which is an inflammatory disease that affects the bile duct
system, most often in young men. Most of the findings are fairly nonspecific,
but the characteristic cholangiographic picture illustrated is the clue that gives
the diagnosis, and will probably be mentioned in any case history about the
disease that you encounter.
Ascending cholangitis (choice A) is a bacterial infection of the bile duct
system, and does not produce the characteristic sacculations of primary
sclerosing cholangitis.
A bile duct tumor (choice B) can produce a diffuse dilation of the biliary tree
above it, secondary to back-pressure effects, but would not produce the
alternating strictures and sacculations seen in this case.
Primary biliary sclerosis (choice C) affects the intrahepatic, but not the
extrahepatic bile ducts.
Viral hepatitis (choice E) does not usually affect the larger intrahepatic bile
ducts and the extrahepatic duct system.
Wilson disease (choice F) typically produces fatty change, hepatitis, or
cirrhosis, rather than cholangitis. Also, it is often accompanied by neurologic
dysfunction

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Question 2 of 6

A positive mitochondrial antibody test would have suggested which of the

following diagnoses?
/A. Hepatitis A infection
/B. Hepatitis B infection
/C. Hepatitis C infection
/D. Primary biliary sclerosis
/E. Wilson disease
Explanation - Q: 5.2

Close

The correct answer is D. Antibodies directed against mitochondria are fairly

specific for primary biliary sclerosis (an important differential diagnosis for this
patient's disease), and are not seen in the other liver diseases listed in the
choices.
Question 3 of 6

The presence of which of the following antibodies would have more specifically
suggested the disease this patient has?
/A. Anti-centromere antibodies
/B. Anti-double-stranded DNA antibodies
/C. Anti-Golgi antibodies
/D. Anti-ribonucleoprotein antibodies
/E. Perinuclear antineutrophil cytoplasmic antibodies
Explanation - Q: 5.3

Close

The correct answer is E. The perinuclear antineutrophil cytoplasmic

antibodies, also called p-ANCA, are most characteristic of primary sclerosing
cholangitis and ulcerative colitis.
Anti-centromere antibodies (choice A) suggest the CREST variant of
scleroderma.
Anti-double-stranded DNA antibodies (choice B) suggest systemic lupus
erythematosus.
Anti-Golgi antibodies (choice C) are seen most often in systemic lupus
erythematosus and Sjgren syndrome.
Anti-ribonucleoprotein antibodies (choice D) are seen most often in systemic
lupus erythematosus and mixed connective tissue disease.
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Question 3 of 6

The presence of which of the following antibodies would have more specifically
suggested the disease this patient has?
/A. Anti-centromere antibodies
/B. Anti-double-stranded DNA antibodies
/C. Anti-Golgi antibodies
/D. Anti-ribonucleoprotein antibodies
/E. Perinuclear antineutrophil cytoplasmic antibodies
Explanation - Q: 5.3

Close

The correct answer is E. The perinuclear antineutrophil cytoplasmic

antibodies, also called p-ANCA, are most characteristic of primary sclerosing
cholangitis and ulcerative colitis.
Anti-centromere antibodies (choice A) suggest the CREST variant of
scleroderma.
Anti-double-stranded DNA antibodies (choice B) suggest systemic lupus
erythematosus.
Anti-Golgi antibodies (choice C) are seen most often in systemic lupus
erythematosus and Sjgren syndrome.
Anti-ribonucleoprotein antibodies (choice D) are seen most often in systemic
lupus erythematosus and mixed connective tissue disease.
Question 4 of 6

This patient's liver disease is most strongly associated with which of the
following?
/A. Amoebic colitis
/B. Celiac disease
/C. Tropical sprue
/D. UIcerative colitis
/E. Whipple disease
Explanation - Q: 5.4

Close

The correct answer is D. There is a specific association between ulcerative

colitis and primary sclerosing cholangitis. Half to three-quarters of patients
with primary sclerosing cholangitis also have inflammatory bowel disease
(ulcerative colitis more commonly than Crohn disease), and approximately
5% of patients with inflammatory bowel disease also have primary sclerosing
cholangitis. The pathophysiologic basis for this association remains unclear.
The other intestinal diseases listed are not specifically associated with
primary sclerosing cholangitis.
Question 5 of 6

Which of the following HLA types is seen with increased frequency in patients
with this patient's disease?
/A. HLA-A3
/B. HLA-B8
/C. HLA-B27
/D. HLA-B35

/E.

HLA-Cw6
Explanation - Q: 5.5

Close

The correct answer is B. Primary sclerosing cholangitis is associated with

increased frequencies of class I antigen HLA-B8 and of class II antigen HLADR3. HLA-B8 is also associated with other autoimmune disorders, and may
partially account for the fact that approximately one-quarter of patients with
primary sclerosing cholangitis also have other autoimmune diseases outside
the liver and colon. Therapy for primary sclerosing cholangitis remains
problematic, with many patients progressing to cirrhosis within 10-15 years.
Existing therapies often attempt to modulate the immune system with steroids
or antimetabolites.
HLA-A3 (choice A) is seen with increased frequency in patients with
idiopathic hemochromatosis.
HLA-B27 (choice C) is seen with increased frequency in patients with acute
anterior uveitis, ankylosing spondylitis, and Reiter syndrome.
HLA-B35 (choice D) is seen with increased frequency in patients with
duodenal ulcer and subacute thyroiditis
HLA-Cw6 (choice E) is seen with increased frequency in patients with
psoriasis vulgaris.
Question 6 of 6

Which of the following histologic findings on liver biopsy is considered to be most

specific for this patient's disease?
/A. Concentric obliterative fibrosis of interlobular bile ducts
/B. Ductular proliferation
/C. Individual hepatocyte necrosis
/D. Periductal concentration of mononuclear cells
/E. Regenerating nodules of hepatic parenchyma
Explanation - Q: 5.6

Close

The correct answer is A. All of the findings illustrated can be seen in primary
sclerosing cholangitis at different stages, but most are non-specific markers
of liver injury. The only finding considered to be specific for primary sclerosing
cholangitis is concentric obliterative fibrosis of interlobular ducts.