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•The answer is A.
Polycythemia vera (PV) is a clonal disorder that involves a multipotent hematopoietic progenitor cell. Clinically, it is characterized by a
proliferation of red blood cells (RBCs), granulocytes, and platelets. The precise etiology is unknown. Unlike chronic myelogenous leukemia, no
consistent cytogenetic abnormality has been associated with the disorder. However, a mutation in the autoinhibitory, pseudokinase domain of
the tyrosine kinase JAK2—that replaces valine with phenylalanine , causing constitutive activation of the kinase—appears to have a central role
in the pathogenesis of PV. Erythropoiesis is regulated by the hormone erythropoietin. Hypoxia is the physiologic stimulus that increases the
number of cells that produce erythropoietin. Erythropoietin may be elevated in patients with hormone-secreting tumors. Levels are usually
“normal” in patients with hypoxic erythrocytosis. In PV, however, because erythrocytosis occurs independently of erythropoietin, levels of the
hormone are usually low. Therefore, an elevated level is not consistent with the diagnosis. PV is a chronic, indolent disease with a low rate of
transformation to acute leukemia, especially in the absence of treatment with radiation or hydroxyurea. Thrombotic complications are the main
risk for PV and correlate with the erythrocytosis. Thrombocytosis, although sometimes prominent, does not correlate with the risk of thrombotic
complications. Salicylates are useful in treating erythromelalgia but are not indicated in asymptomatic patients. There is no evidence that
thrombotic risk is significantly lowered with their use in patients whose hematocrits are appropriately controlled with phlebotomy. Phlebotomy
is the mainstay of treatment. Induction of a state of iron deficiency is critical to prevent a reexpansion of the RBC mass. Chemotherapeutics and
other agents are useful in cases of symptomatic splenomegaly. Their use is limited by side effects, and there is a risk of leukemogenesis with
•The answer is B.

Cat bites are the most likely animal bites to lead to cellulitis because of deep inoculation and the frequent
presence of Pasteurella multicoda. In an immunocompetent host, only cat bites warrant empirical
antibiotics. Often the first dose is given parenterally. Ampicillin–sulbactam followed by oral amoxicillin–
clavulanate is effective empirical therapy for cat bites. However, in an asplenic patient, a dog bite can lead to
rapid overwhelming sepsis as a result of Capnocytophaga canimorsus bacteremia. These patients should be
followed closely and given third-generation cephalosporins early in the course of infection. Empirical therapy
should also be considered for dog bites in elderly adults, for deep bites, and for bites on the hand.
•The answer is D.
Although any valvular vegetation can embolize, vegetations located on the mitral valve and
vegetations larger than 10 mm are greatest risk of embolizing. Of the answer choices, C, D, and E
are large enough to increase the risk of embolization. However, only choice D demonstrates the
risks of both size and location. Hematogenously seeded infection from an embolized vegetation
may involve any organ but particularly affects those organs with the highest blood flow. They are
seen in up to 50% of patients with endocarditis. Tricuspid lesions lead to pulmonary septic
emboli, which are common in injection drug users. Mitral and aortic lesions can lead to embolic
infections in the skin, spleen, kidneys, meninges, and skeletal system. A dreaded neurologic
complication is mycotic aneurysm, focal dilations of arteries at points in the arterial wall that
have been weakened by infection in the vasa vasorum or septic emboli, leading to hemorrhage.
•The answer is B.

Bullae (Latin for bubbles) are skin lesions that are greater than 5 mm and fluid filled. They may be regular or
irregularly shaped and filled with serous or seropurulent fluid. Clostridium spp., including perfringens, may
cause bullae through myonecrosis. Staphylococcus causes scalded skin syndrome through elaboration of the
exfoliatin toxin from phage group II, particularly in neonates. Streptococcus pyogenes, the causative agent of
impetigo, may cause bullae initially that progress to crusted lesions. MRSA may also cause impetigo. The
halophilic Vibrio, including V. vulnificus, may cause an aggressive fasciitis with bullae formation. Patients with
cirrhosis exposed to Gulf of Mexico or Atlantic waters (or ingestion of raw seafood from those waters) are at
greatest risk. Infection with the dimorphic fungus, Sporothrix schenckii, presents with discrete crusted
lesions resembling ringworm. Lesions may progress to ulcerate. Patients often have a history of working with
soil or roses.
•The answer is E.
Although frequent nonbloody diarrheal illness is commonly associated with
Clostridium difficile infection, other presentations are well described, including fever in
28% of cases, abdominal pain, and leukocytosis. Adynamic ileus is often seen with C.
difficile infection, and leukocytosis in this condition should be a clue that C. difficile is
at play. Recurrent infection after therapy has been described in 15% to 30% of cases.
•The answer is A.
Common causes of urethral discomfort and discharge in men include Chlamydia
trachomatis, Neisseria gonorrhoeae, Mycoplasma genitalium, Ureaplasma
urealyticum, Trichomonas vaginalis, and herpes simplex virus. Gardnerella spp. is the
usual cause of bacterial vaginosis in women and is not a pathogen in men.
•The answer is E.
Probably because of its ubiquity and ability to stick to foreign surfaces, Staphylococcus epidermidis is the
most common cause of infections of central nervous system shunts as well as an important cause of
infections on artificial heart valves and orthopedic prostheses. Corynebacterium spp. (diphtheroids), similar
to S. epidermidis, colonize the skin. When these organisms are isolated from cultures of shunts, it is often
difficult to be sure if they are the cause of disease or simply contaminants. Leukocytosis in cerebrospinal
fluid, consistent isolation of the same organism, and the character of a patient’s symptoms are all helpful in
deciding whether treatment for infection is indicated.
•The answer is D.

Resistance to ampicillin and vancomycin is far more common in strains of Enterococcus faecium than E.
faecalis. Linezolid and quinupristin–dalfopristin are approved by the U.S. Food and Drug Administration for
the treatment of some vancomycin-resistant enterococci (VRE) infections. Linezolid is not bactericidal, and
its use in severe endovascular infections has produced mixed results; therefore, it is recommended only as
an alternative to other agents. Quinupristin–dalfopristin is not active against most E. faecalis isolates.
Resistance to VRE strains of E. faecium is also emerging with increasing usage. Cephalosporins are generally
inactive against enterococcal infections.
•The answer is A.
Neisseria meningitidis is an effective colonizer of the human nasopharynx, with asymptomatic infection rates of greater
than 25% described in some series of adolescents and young adults and among residents of crowded communities. Despite
the high rates of carriage among adolescents and young adults, only 10% of adults carry meningococci, and colonization is
very rare in early childhood. Colonization should be considered the normal state of meningococcal infection. Meningeal
pharyngitis rarely occurs. Meningococcal disease occurs when a virulent form of the organism invades a susceptible host.
The most important bacterial virulence factor relates to the presence of the capsule. Unencapsulated forms of N.
meningitides rarely cause disease. A nonblanching petechial or purpuric rash occurs in more than 80% of cases of
meningococcal disease. Of patients with meningococcal disease, 30% to 50% present with meningitis, approximately 40%
with meningitis plus septicemia, and 20% with septicemia alone. Patients with complement deficiency, who are at highest
risk of developing meningococcal disease, may develop chronic meningitis.
•The answer is B.
The major reservoirs in the human body for anaerobic bacteria are the mouth, lower
gastrointestinal tract, skin, and female genital tract. Generally, anaerobic infections
occur proximal to these sites after the normal barrier (i.e., skin or mucous membrane)
is disrupted. Thus, common infections resulting from these organisms are abdominal
or lung abscess, periodontal infection, gynecologic infections such as bacterial
vaginosis, and deep tissue infection. Properly obtained cultures in these circumstances
generally grow a mixed population of anaerobes typical of the microenvironment of
the original reservoir.
•The answer is E.
Sinoatrial dysfunction is often divided into intrinsic disease and extrinsic disease of the node.
This is a critical distinction, as extrinsic causes are often reversible and pacemaker placement is
not required. Drug toxicity is a common cause of extrinsic, reversible sinoatrial dysfunction, with
common culprits including beta blockers, calcium channel blockers, lithium toxicity, narcotics,
pentamidine, and clonidine. Hypothyroidism, sleep apnea, hypoxia, hypothermia, and increased
intracranial pressure are all reversible forms of extrinsic dysfunction. Radiation therapy can
result in permanent dysfunction of the node and therefore is an irreversible, or intrinsic, cause
of sinoatrial node dysfunction. In symptomatic patients, pacemaker insertion may be indicated.
•The answer is E.
Patients at the highest risk for stroke associated with atrial fibrillation include those with a prior
history of stroke, TIA, or embolism, and patients with hypertension, diabetes mellitus,
congestive heart failure, rheumatic heart disease, LV dysfunction, and marked left atrial dilation
of greater than 5.0 cm or age greater than 65 years. Anticoagulation should be strongly
considered in these patients. Increased left atrial size is a risk factor for chronic atrial fibrillation.
•The answer is E.
Atrial septal defect (ASD) is a not uncommon simple congenital heart disease lesion that is often
diagnosed in adults. Because of chronic left-to-right shunting of intracardiac blood, pulmonary
arterial hypertension is a well-recognized common complication. With the development of
pulmonary arterial hypertension, the potential for paradoxical embolization of either air or
thrombotic material from the right atrium to the systemic circulation is increased. Similarly, with
exertion in the context of pulmonary arterial hypertension and ASD, blood may shunt right to left,
leading to systemic arterial oxygen desaturation. Atrial fibrillation or other supraventricular arrhyth-
mias may occur, also as a result of atrial stretching with the lesion. While atherosclerosis and
unstable angina may certainly occur in adults, is not a reported complication
•The answer is A.
Patients with severe aortic regurgitation will have a “water-hammer” pulse that collapses suddenly as
arterial pressure rapidly falls during late systole and diastole, a so-called Corrigan’s pulse. Capillary pulsations
seen in the nail bed in severe aortic regurgitation are named Quincke’s pulse. Traube’s sign, or a pistol shot
sound, may be heard over the femoral arteries and Duroziez’s sign, with a to-and-fro murmur over the
femoral artery, have also been described. Pulsus parvus et tardus is found in severe aortic stenosis. Pulsus
bigeminus occurs when there is a shorter interval after a normal beat with a following low volume pulse,
often with a premature ventricular beat. Pulsus paradoxus has been described with pericardial tamponade
or severe obstructive lung disease. Pulsus alternans is alternating large and small volume pulses seen in
severe heart failure.
•The answer is E.
Tricuspid regurgitation is most commonly caused by dilation of the tricuspid annulus due to right-ventricular
enlargement of any cause. Any cause of left-ventricular failure that results in right-ventricular failure may
lead to tricuspid regurgitation. Congenital heart diseases or pulmonary arterial hypertension leading to right-
ventricular failure will dilate the tricuspid annulus. Inferior wall infarction may involve the right ventricle.
Rheumatic heart disease may involve the tricuspid valve, although less commonly than the mitral valve.
Infective endocarditis, particularly in IV drug users, will infect the tricuspid valve, causing vegetations and
regurgitation. Other causes of tricuspid regurgitation include carcinoid heart disease, endomyocardial
fibrosis, congenital defects of the atrioventricular canal, and right-ventricular pacemakers.
•The answer is A.
Bioprosthetic valves are made from human, porcine, or bovine tissue. The major advantage of a bioprosthetic valve is the
low incidence of thromboembolic phenomena, particularly 3 months after implantation. Although in the immediate
postoperative period some anticoagulation may occur, after 3 months there is no further need for anticoagulation or
monitoring. The downside is the natural history and longevity of the bioprosthetic valve. Bioprosthetic valves tend to
degenerate mechanically. Approximately 50% will need replacement at 15 years. Therefore, these valves are useful in
patients with contraindications to anticoagulation, such as elderly patients with comorbidities and younger patients who
desire to become pregnant. Elderly people may also be spared the need for repeat surgery, as their life span may be shorter
than the natural history of the bioprosthesis. Mechanical valves offer superior durability. Hemodynamic parameters are
improved with double-disk valves compared with single-disk or ball-and-chain valves. However, thrombogenicity is high and
chronic anticoagulation is mandatory. Younger patients with no contraindications to anticoagulation may be better served
by mechanical valve replacement.
•The answer is E.
Many infectious etiologies have been associated with the development of
inflammatory myocarditis including viral agents (coxsackie, adenovirus, HIV, hepatitis
C) and parasitic agents, with Chagas disease or T. cruzi being most prominent, but also
toxoplasmosis. Additionally, bacterial etiologies like diphtheria, spirochetal disease like
Borrelia burgdorferi, rickettsial disease, and fungal infections have been associated.
•The answer is B.
Pulsus paradoxus is an exaggeration of the normal phenomenon in which systolic blood pressure
declines 10 mmHg or less with inspiration. Pulsus paradoxus is typically seen in patients with
pericardial tamponade and in patients with severe obstructive lung disease (COPD, asthma). In
pulsus paradoxus due to pericardial tamponade, the inspiratory systolic blood pressure decline
is greater due to the tight incompressible pericardial sac. The right ventricle distends with
inspiration, compressing the left ventricle and resulting in decreased systolic pulse pressure in
the systemic circulation. In severe obstructive lung disease, the inspiratory decline of systolic
blood pressure may be due to the markedly negative pleural pressure either causing left
ventricular compression (due to increased RV venous return) or increased LV impedance to
ejection (increased afterload).
•The answer is C.
Beck’s triad can be used to alert clinicians to the potential presence of cardiac tamponade. The
principal features are hypotension, muffled or absent heart sounds, and elevated neck veins,
often with prominent x-descent and absent y-descent. These are due to the failure of ventricular
filling and limited cardiac output. Kussmaul’s sign is seen in restrictive cardiomyopathy and
pericardial constriction, not tamponade. Friction rub may be seen in any condition associated
with pericardial inflammation.
•The answer is B.
The functional residual capacity of the lung refers to the volume of air that remains in the lung following a
normal tidal respiration. This volume of air represents the point at which the outward recoil of the chest wall
is in equilibrium with the inward elastic recoil of the lungs. The lungs would remain at this volume if not for
the actions of the respiratory muscles. The functional residual capacity is comprised of two lung volumes:
the expiratory reserve volume and the residual volume. The expiratory reserve volume represents the
additional volume of air that can be exhaled from the lungs when acted upon by the respiratory muscles of
exhalation. The residual volume is the volume of air that remains in the lung following a complete exhalation
and is determined by the closing pressure of the small airways.