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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
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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
/C. Hepatitis B
/D. Hepatitis C
/E. Hemolytic anemia
Explanation - Q: 1.2
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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
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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
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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
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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
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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
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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
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/E.
Steatosis
Explanation - Q: 3.1
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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
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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
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/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
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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
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
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
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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
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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
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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
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Question 7 of 7
Explanation - Q: 4.7
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Explanation - Q: 5.1
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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 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
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
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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
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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.