Critique: 1
Preferred Response: B
71-2003
The child described in the vignette has clinical findings suggestive of Sydenham chorea, the
neurologic sequela of some cases of acute rheumatic fever. Migratory arthritis and rheumatic
carditis are more common clinical manifestations of acute rheumatic fever. The other two
signs that are included in the five major criteria for the diagnosis of acute rheumatic fever,
erythema marginatum and subcutaneous nodules, are the least common manifestations. One
mnemonic for the five major criteria that constitute the T. Duckett Jones Criteria for the
diagnosis of rheumatic fever is “ JONES.” “J ” represents joints, which signifies migratory
polyarthritis. “O ” represents the “obvious” reason for diagnosing and treating these patients,
which is potential rheumatic carditis or cardiac involvement. Although the migratory arthritis
is painful, it can resolve without treatment and never results in joint damage (unlike juvenile
rheumatoid arthritis). In contrast, the carditis may cause permanent valvular damage, most
frequently mitral valve regurgitation. “N” is for “nodules,” which are subcutaneous, usually
pea-sized, and found over the occiput of the skull or over extensor surfaces of the elbows or
knees. “E ” represents “erythema marginatum,” a serpentine, slightly raised red or pink rash
that typically is seen on the trunk and extremities and may be confused with erythema
multiforme. True target lesions are not seen with erythema marginatum of acute rheumatic
fever. Circular, raised lesions that become larger and more irregular are more typical. “ S ”
stands for “Sydenham chorea.” The diagnosis of acute rheumatic fever is established when
two major criteria are present and there is evidence of a pre-existing group A hemolytic
streptococcal infection. The best evidence of streptococcal infection is elevation of
antistreptococcal titers, such as the antistreptolysin titer or antiDNAse B titer. If two major
criteria cannot be demonstrated, the diagnosis still can be established by the presence of one
major criterion and any two of the following minor criteria: fever, elevated acute-phase
reactants (erythrocyte sedimentation rate or C-reactive protein), and prolonged PR interval on
electrocardiography. Arthralgia may be used as a minor criterion in a patient who does not
have arthritis. The diagnosis of rheumatic chorea is more obvious when the movement
disorder occurs soon after other rheumatic manifestations, such as migratory arthritis or the
development of a new murmur of mitral valve regurgitation or a diastolic murmur of aortic
valve regurgitation. However, rheumatic chorea often occurs late enough after the
streptococcal infection for evidence of streptococcal cause to be lacking. An elevated
erythrocyte sedimentation rate or C-reactive protein level also may be absent in isolated cases
of rheumatic Sydenham chorea. Icteric sclerae may be apparent in the presence of
intravascular hemolysis, such as can be seen with sudden cardiac valvular regurgitation from
a perforated valve leaflet. This so-called “Waring blender phenomenon” of mechanically
lysed red blood cells most often occurs following dehiscence of the valve ring after prosthetic
valve surgery, but it can occur from mechanical destruction of a perforated cardiac valve
leaflet in some cases of infective endocarditis. It does not occur in rheumatic carditis. Osler
nodes and splenomegaly are other manifestations of infective endocarditis, presumably
reflecting an immunologic reaction to chronic bacteremia. They are not seen in acute
rheumatic fever. Osler nodes are small, often tender nodular lesions on the palmar or volar
surface of the hands or fingers that differ from the subcutaneous nodules of acute rheumatic
fever. Severe hypertension is not seen in acute rheumatic fever. It is a common manifestation
of poststreptococcal acute glomerulonephritis, which is a different streptococcal complication.
For reasons that are not entirely elucidated, acute rheumatic fever only occurs following a
streptococcal respiratory tract infection (streptococcal pharyngitis); poststreptococcal acute
glomerulonephritis can occur following any streptococcal infection, including impetigo,
cellulitis, or streptococcal pharyngitis.
Critique: 2
Preferred Response: C
71-2004
Acute severe myocarditis often presents with symptoms that suggest an abdominal pathology,
such as nausea, anorexia, and vomiting. Diarrhea is much less common. Many children who
have profound acute congestive heart failure due to myocarditis are admitted to the hospital
with the diagnosis of acute gastroenteritis or appendicitis. It is not known if the nausea and
vomiting of acute myocarditis are related to distention of the pericardial sac; abnormal wall
stress in the distended, poorly contracting ventricular myocardium; elevation of venous
pressures in the mesenteric veins; or mesenteric ischemia. There are nerve fibers in the
myocardial wall (C-fibers) that, when stimulated, can cause triggering of the vagus nerve,
with subsequent vomiting. This is one suggested mechanism of the nausea. Pericardial
distention also can cause abdominal distress and vomiting. Children and adolescents who
have acute pericarditis associated with large pericardial effusion and cardiac tamponade often
have abdominal pain and vomiting with no complaint of chest pain. In pericardial
tamponade, the palpable pulse may diminish during the inspiratory phase of respiration. This
is called pulsus paradoxus and is due to an accentuation of the normal decrease in left
ventricular preload during inspiration. The palpable pulse rate may be approximately half that
of the auscultated heart rate, but the palpable pulse will be felt at the same rate as the true
heart rate when the patient exhales and will not be felt at all during inspiration. For the child
described in the vignette, however, a palpable pulse rate half that of the auscultated or true
heart rate is described as regular, not variable or changing with respiration. The finding of a
palpable pulse with every other heartbeat is called pulsus alternans. Pulsus alternans occurs
with severe ventricular dysfunction. It is postulated that two cardiac cycles are needed for
adequate release of calcium from the sarcoplasmic reticulum to result in adequate excitation
contraction coupling. Hepatitis and pancreatitis obviously may present with vomiting, but
they are not associated with pulsus alternans. Rales and tachypnea, as described for the
child in the vignette, may suggest pneumonia. However, pneumonia does not cause pulsus
alternans. Pneumonia may present with abdominal symptoms severe enough to prompt
consideration of appendicitis as the diagnosis. Any child who has acute abdominal distress
serious enough to warrant abdominal radiography also requires a chest radiograph. Chest
radiography may demonstrate unsuspected pneumonia. On rare occasions, an enlarged cardiac
silhouette from myocarditis or pericarditis may be discovered.
Critique: 3
Preferred Response: E
245-2004
An elevation of acute-phase reactants is a minor criterion for the diagnosis of acute rheumatic
fever (ARF). This includes either an elevated C-reactive protein or erythrocyte sedimentation
rate. Abnormally elevated values almost always are present during the acute phase of ARF.
The sensitivity of this criterion is rather high, but its specificity is low. For this reason, it is
considered a minor criterion. Decreased serum complement and decreased serum haptoglobin
are not seen during acute inflammatory processes. The revised Jones criteria for the
diagnosis of ARF require either two major criteria or one major criterion with two minor
criteria. In addition, there must be evidence of previous group A beta-hemolytic streptococcal
infection. An elevated serum titer to group A Streptococcus , such as antistreptolysin O titer,
is neither a major nor a minor criterion. An increased (not decreased) PR interval on
electrocardiography is a minor Jones criterion. This finding is much less common in ARF
than elevated acute-phase reactants, but sometimes it is useful. The major Jones criteria are
acute polyarthritis, carditis, subcutaneous nodules, erythema marginatum, and Sydenham
chorea. The minor criteria are elevated acute-phase reactants, increased PR interval, fever,
and arthralgia. Arthralgia refers to the historical complaint of painful joints without objective
evidence of arthritis on examination. It cannot be used as a minor criterion when arthritis is
used as a major criterion. A helpful mnemonic for the major criteria is JONES. J signifies
"joints" for acute arthritis. The arthritis of ARF often is migratory and may involve several
large joints in rapid succession. The arthritis may be painful, but it produces no permanent
joint damage. The letter O signifies "obvious," meaning carditis. The association of carditis
with ARF is the "obvious" reason for diagnosing and treating the condition and for
continuous antibiotic prophylaxis to prevent recurrence. However, the carditis is not always
obvious. A subtle murmur of very mild but abnormal mitral valve regurgitation or of aortic
valve regurgitation can fulfill this criterion. Some subspecialists have suggested that
echocardiographic evidence of abnormal but clinically inaudible left heart valvular
regurgitation suffices. The carditis can produce permanent cardiac dysfunction, particularly
valvular, and especially if there are recurrent attacks of rheumatic fever because of lax
compliance with prophylaxis against recurrent streptococcal infection. The letter N signifies
"nodules." Subcutaneous nodules over the occiput or extensor surfaces of the extremities are
rare manifestations of ARF. The letter E represents "erythema marginatum". Although this
skin manifestation resembles the more common erythema multiforme, there are differences.
In erythema marginatum, there is no central red macule creating a true target lesion. Instead,
there are enlarging rings, often broken with slightly raised serpiginous margins. S signifies
"Sydenham chorea", a movement disorder that may be confused with tics but usually has a
more acute onset. Gait disturbance, jerky movements of the arms, and poor coordination are
seen. Children who present with Sydenham chorea may not have fever, elevation of acutephase reactants, or elevation of antistreptococcal serum titers. The choreiform movement
disorder can present much later after the triggering group A streptococcal infection than the
other manifestations of ARF.
Critique: 4
Preferred Response: B
248-2005
Rheumatic fever follows infections caused by certain strains of group A streptococci. This
inflammatory disease affects the heart, central nervous system, joints, subcutaneous tissue,
and skin. Rheumatic fever is a significant cause of cardiovascular morbidity in developing
countries and continues to be seen in the United States despite decades of declining incidence.
The disease can be viewed as a nonsuppurative complication of group A streptococcal
infections of the upper respiratory tract that occurs in children typically between the ages of 4
and 15 years. Patients who have had rheumatic fever in the past have an increased incidence
of recurrent disease. The pathognomonic cardiac lesion in rheumatic fever is the Aschoff
body, which is a perivascular infiltrate of large cells that have polymorphous nuclei and
basophilic cytoplasm and is arranged in a rosette pattern. In 1992, the American Heart
Association presented guidelines for the diagnosis of rheumatic fever that included an update
of the original Jones criteria. The clinical manifestations of rheumatic disease can be divided
into major criteria and minor criteria. The major manifestations of the disease are: carditis,
polyarthritis, subcutaneous nodules, erythema marginatum , and chorea. Minor
manifestations are: arthralgia (not considered a criterion if polyarthritis is present), fever,
increased acute-phase reactants (erythrocyte sedimentation rate and C-reactive protein), and
prolonged P-R interval. The diagnosis requires two of the major manifestations or one of the
major manifestations with two of the minor manifestations. The carditis of rheumatic fever
may involve any of the three layers of the heart: the endocardium, myocardium, or
pericardium. If all three layers are involved, pancarditis exists. Patients who have carditis may
not have symptoms, but they are likely to have signs on the cardiac examination. When signs
or symptoms are present, they most often are the result of endocarditis, which is caused by
hyaline degeneration of the affected valve. The mitral valve is the valve affected most
commonly, followed by the aortic valve. It is rare for the tricuspid or pulmonary valve to be
affected. Therefore, the clinician should auscultate for the apical, holosystolic murmur that
radiates to the left axilla and represents mitral regurgitation. If the mitral regurgitation is
severe, there may be a low-frequency mid-to-late diastolic murmur of relative mitral stenosis,
which is referred to as the Carey Coombs murmur. The clinician also should listen for the
early diastolic decrescendo murmur of aortic regurgitation, which may be exaggerated when
the patient is in the sitting position, leaning forward. Signs and symptoms of congestive heart
failure occur in about 5% of patients who have rheumatic carditis. Echocardiography has
become an essential component of the evaluation of a patient who has rheumatic fever. It is a
noninvasive, ultrasonographic examination that can be performed at the bedside. Routine
echocardiography provides excellent visualization of the cardiac valves, myocardial
performance, pericardium, and potential pericardial effusion. Valvular dysfunction can be
assessed using Doppler and color flow mapping techniques. The patient described in the
vignette has had an infection with a group A Streptococcus and now has two of the major
manifestations of rheumatic fever: erythema marginatum and polyarthritis. She should
undergo detailed echocardiography to evaluate for the presence of carditis. Chest
radiography may provide a general assessment of the patient’s cardiac size, but it is a much
less specific test to evaluate cardiac chamber dimensions, valvular function, and myocardial
and pericardial involvement than is echocardiography. Radiography of the painful, swollen
joints in patients who have rheumatic fever is not diagnostic. Rheumatoid factor does not
have a diagnostic implication in patients who have rheumatic fever, and a skin biopsy is not
indicated in this clinical picture.
Critique: 5
232-2006
Preferred Response: B
Although it is relatively uncommon, endocarditis continues to be a life-threatening disease
associated with significant mortality and morbidity. Because of this, primary prevention of
the infection is paramount. Several important points need to be kept in mind when
considering endocarditis prophylaxis for patients who have congenital heart disease. First,
there are no randomized, carefully controlled human trials in patients who have underlying
structural heart disease to establish definitively that antibiotic prophylaxis provides protection
against the development of endocarditis during bacteremia-inducing procedures. Second,
most cases of endocarditis are not attributable to an invasive procedure. Third, the incidence
of endocarditis following most procedures in patients who have underlying structural heart
disease is low. Therefore, a reasonable approach for endocarditis prophylaxis should consider
the following: the degree of risk for endocarditis of the patient’s underlying heart disease, the
risk of bacteremia for the given procedure, the potential adverse reactions of the antibiotic
prophylaxis, and the cost-benefit aspects of the recommended antibiotic prophylaxis.
Certain cardiac conditions are associated with endocarditis more often than are others. Item
C232A separates cardiac conditions associated with endocarditis based on the severity (highrisk, moderate-risk, and negligible-risk) of endocarditis if it develops. In general, those
conditions associated with highly turbulent blood flow are at greater risk for the development
of infection. Conversely, those conditions associated with nonturbulent blood flow, such as
atrial septal defect, do not appear to be at increased risk for endocarditis and, as a result, do
not require antibiotic prophylaxis. Intracardiac devices, such as an atrial septal defect closure
device described for the girl in the vignette, are sufficiently endothelialized within 6 months
of implantation and do not require antibiotic prophylaxis when the patient is at risk for
bacteremia. It is important for pediatric clinicians to recognize that poor dental hygiene
may produce bacteremia even in the absence of dental procedures. Individuals who are at risk
for developing bacterial endocarditis should establish and maintain the best possible oral
health to reduce potential sources of bacterial seeding.
Critique:6
248-2006
Preferred Response: B
The carditis of rheumatic fever may involve any of the three layers of the heart: the
endocardium, myocardium, or pericardium. If all three layers are involved, pancarditis exists.
Patients who have carditis may not have symptoms, but they are likely to have signs on
cardiac examination. When signs or symptoms are present, they most often are the result of
endocarditis, which is caused by hyaline degeneration of the affected valve. The mitral valve
is the most commonly affected valve, followed by the aortic valve. It is rare for the tricuspid
or pulmonary valve to be affected. Therefore, the clinician should auscultate for the apical,
holosystolic murmur (Item C248A) that radiates to the left axilla and represents mitral
regurgitation. If the mitral regurgitation is severe, there may be a low-frequency mid-to-late
diastolic murmur of relative mitral stenosis, which is referred to as the Carey Coombs
murmur. The clinician also should listen for the early diastolic decrescendo murmur of aortic
regurgitation (Item C248B), which may be exaggerated when the patient is in the sitting
position and leaning forward. Signs and symptoms of congestive heart failure occur in about
5% of patients who have rheumatic carditis. The patient described in the vignette has three
of the major manifestations of rheumatic fever: erythema marginatum, polyarthritis, and
carditis. The latter is evidenced by the presence of new cardiac murmurs. The holosystolic
murmur results from mitral insufficiency, and the diastolic murmur results from aortic
insufficiency. Coronary aneurysms are not seen in rheumatic heart disease, but can be a
complication of Kawasaki disease. The murmur of a patent ductus arteriosus is continuous
(Item C248C) because there is a constant gradient (during systole and diastole) between the
high-pressure aorta and low-pressure pulmonary artery. Incidentally, there is no association
between Kawasaki disease and a patent ductus arteriosus. Aortic stenosis is not seen during
acute rheumatic fever and typically is characterized by a systolic ejection murmur (Item
C248D) heard at the right upper sternal border. Mitral stenosis (Item C248E), an important
late complication of rheumatic heart disease, is characterized by a diastolic murmur. The
murmur of a ventricular septal defect (Item C248F) is holosystolic and typically heard along
the left sternal border. Ventricular septal defects are not associated with rheumatic fever.
Critique: 7
87-2007
Preferred Response: B
Rheumatic fever follows infections caused by certain strains of group A streptococci. This
inflammatory disease affects the heart, central nervous system, joints, subcutaneous tissue,
and skin. Rheumatic fever is a significant cause of cardiovascular morbidity in developing
countries and continues to be seen in the United States, despite decades of declining
incidence. The disease can be viewed as a nonsuppurative complication of group A
streptococcal infections of the upper respiratory tract that typically occurs in children between
the ages of 4 and 15 years. Patients who have had rheumatic fever in the past have an
increased incidence of recurrent disease. The pathognomonic cardiac lesion in rheumatic
fever is the Aschoff body, which is a perivascular infiltrate of large cells that have
polymorphous nuclei and basophilic cytoplasm and are arranged in a rosette pattern. In
1992, the American Heart Association presented guidelines for the diagnosis of rheumatic
fever that included an update of the original Jones criteria. The clinical manifestations of
rheumatic fever can be divided into major criteria and minor criteria. The major
manifestations of the disease are :
•
Carditis
•Polyarthritis
•Subcutaneous nodules
•Erythema marginatum
•Chorea
Minor manifestations are :
•
Arthralgia (not considered a criterion if polyarthritis is present)
•Fever
•Increased acute-phase reactants (erythrocyte sedimentation rate, C-reactive protein)
•Prolonged P-R interval
The diagnosis requires two of the major manifestations or one of the major manifestations
plus two of the minor manifestations. The carditis of rheumatic fever may involve any of
the three layers of the heart: the endocardium, myocardium, or pericardium. If all three layers
are involved, pancarditis exists. Patients who have carditis may not have symptoms, but they
likely have signs on cardiac examination. When signs or symptoms are present, they most
often are the result of endocarditis, which is caused by hyaline degeneration of the affected
valve. The mitral valve is the most commonly affected valve, followed by the aortic valve. It
is rare for the tricuspid or pulmonary valve to be affected. Therefore, the clinician should
auscultate for the apical, holosystolic murmur that radiates to the left axilla and represents
mitral regurgitation (Item C87A). If the mitral regurgitation is severe, there may be a lowfrequency mid- to late diastolic murmur of relative mitral stenosis, which is referred to as the
Carey Coombs murmur. The clinician also should listen for the early diastolic decrescendo
murmur of aortic regurgitation (C87B), which may be exaggerated when the patient is in the
sitting position, leaning forward. Signs and symptoms of congestive heart failure occur in
about 5% of patients who have rheumatic carditis. The patient described in the vignette has
fever and two major Jones criteria: migratory arthritis and carditis represented by mitral
regurgitation. Therefore, she meets the diagnostic criteria for rheumatic fever. The acute
management of rheumatic fever encompasses three primary strategies: treat the infection
causing the disease, control and alleviate the symptoms, and provide supportive care as
indicated. Because rheumatic fever is caused by group A beta-hemolytic streptococci,
antibiotic therapy should be directed at eradication of this pathogen. Typically, a 10-day
course of oral penicillin V is adequate. Alternatively, intramuscular or intravenous penicillin
preparations may be used. For those who have a penicillin allergy, an alternate antibiotic such
as erythromycin may be used. Doxycycline, a treatment for Lyme arthritis, is not the best
choice for management of arthritis resulting from acute rheumatic fever. Antiinflammatory agents are important for symptomatic relief and to combat the inflammatory
process. Salicylates are particularly effective for the migratory arthritis, with improvement
often occurring in the first 12 to 24 hours of therapy. In addition, these medications frequently
relieve the accompanying fever. Aspirin typically is administered in high doses (80 to 100
mg/kg per day to a serum level of 25 mg/dL) for several weeks before a gradual tapering is
begun. For patients who cannot tolerate aspirin, the use of other nonsteroidal antiinflammatory drugs may be considered. Steroids generally are reserved for patients who
demonstrate moderate-to-severe carditis with congestive heart failure. Supportive care has
historically included the use of bed rest, especially during the acute phase of the illness.
Joint aspiration, immunotherapy, and elevation and immobilization of the right knee do not
address the underlying problem for the patient in the vignette.
Critique: 8
199-2007
Preferred Response: C
Kawasaki disease (KD) is believed to be a multisystem illness characterized by vasculitis of
small- and medium-size blood vessels, including the coronary arteries. Although the precise
cause remains unclear, recent investigations suggest that at least some of the cases are related
to an infectious organism (New Haven coronavirus). The median age of patients affected by
KD is 2.3 years, and nearly 80% of cases occur in children younger than 4 years of age. More
cases are reported in the winter and spring than during the summer and fall, and males are
affected approximately 1.5 times more frequently than females. KD can be seen in patients of
all ethnicities, races, and cultures, but it is most prevalent in those of Asian descent. KD is
diagnosed using a set of diagnostic criteria that include the presence of fever for at least 5
days and at least four of the five following clinical findings :
•
Changes in the extremities (edema, erythema, desquamation [Item C199A])
• Polymorphous exanthem (C199B) that usually is truncal
• Conjunctival injection (C199C)
• Erythema or fissuring of the lips (C199D), oral cavity, and tongue
•Cervical lymphadenopathy
In addition, the diagnosis of KD requires that the constellation of findings with which the
patient presents cannot be explained by any other known disease process. Some children may
have an incomplete form of the disease but still are at risk for its complications. KD has an
acute phase that lasts 1 to 2 weeks after the onset of fever, which is when the diagnostic
criteria typically are present. The subacute phase follows from 2 to 8 weeks after onset.
Patients may demonstrate desquamation of the fingers and toes. It is during this phase when
coronary aneurysms may develop, particularly in those who have not been treated with
intravenous gamma globulin during the acute phase. The convalescent phase lasts for months
following the illness. Patients who have KD may have involvement of the cardiovascular
system that can include myocarditis, valvulitis, or arteritis. The latter is diagnosed by the
presence of edematous coronary walls on echocardiography, usually between days 5 and 8 of
the illness. Coronary artery aneurysms can develop after the acute phase and typically in the
subacute phase. The incidence of coronary aneurysms may be as high as 22% to 25% in
untreated patients compared with an incidence of 3% to 5% in those who receive intravenous
immune globulin therapy in the acute phase. Exercise stress testing is not part of the initial
evaluation and cannot be performed reliably in a child as young as the one in the vignette; it
usually is reserved for older, more cooperative children toward the age of 7 to 8 years. Highdose aspirin therapy (80 to 100 mg/kg per day) is administered until the patient is afebrile for
48 hours, at which time the dose is decreased to 3 to 5 mg/kg per day for 6 to 8 weeks or until
platelet concentrations normalize. Echocardiography usually is performed at the time of
diagnosis to assess for the presence of a subclinical myocarditis, to evaluate for coronary
arteritis, and to serve as a baseline for future studies. Aneurysms of the coronary arteries are
not seen in the acute phase and, therefore, their absence on echocardiography should reassure
neither the parents nor the pediatrician.