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CLIPP 11 - Kawasaki
#ir #peds
11. 5-year-old with fever and adenopathy - Jason
January 27, 2017 2:09:56 PM EST
Knowledge
Irritability in a Child
Irritability may be the only way that a young child can express pain or discomfort from any source,
including:
Meningeal irritation
Headache from intracranial irritation
Simple exhaustion
Pediatric Vital Signs
As a child gets older, the normal ranges for vital signs change, making it important to look at ageappropriate
reference values.
The normal heart rate for a 5-year-old is 80 to 100 beats per minute.
Rashes Associated with Fever (Part 1)
Children get many different kinds of rashes-many, though not all, related to infections. Below are
descriptions and photos of rashes associated with fever.
Cho Sooyoung - sooyoung.cho@mail.utoronto.ca 1/15
Enterovirus
Usually erythematous and maculopapular, and may involve the palms and
soles. Infrequently, it can be petechial.
In hand-foot-and-mouth disease (caused by Coxsackie virus), presents as a
vesicular rash on the hands and feet and with ulcers in the mouth.
Usually seen in late summer and early fall.
While the fever associated with enteroviruses may be high, it usually lasts only
a few days.
Erythema
infectiosum
Also called fifth disease, this is caused by parvovirus B19.
There is frequently an associated low-grade fever (37.8-38.3 degrees C, or
100-101 degrees F), with a rash appearing seven to ten days later.
The characteristic rash starts as facial erythema-the "slapped cheek"
appearance. This can spread to the trunk and have an erythematous macular
appearance. Central clearing of the rash appears, giving a lacy appearance.
The rash often lasts longest on the extremities, where it has a lacy, reticular
appearance.
People with fifth disease can also develop pain and swelling in their joints
(polyarthropathy syndrome).
Individuals with underlying immune deficiencies who are exposed to this virus
are at risk for developing aplastic anemia.
Measles
After a prodrome of fever (over 38.3 C, or 101 F), cough, coryza, and
conjunctivitis, this maculopapular rash starts on the neck, behind the ears, and
along the hairline.
The rash spreads downward, reaching the feet in two or three days.
The initial rash appears on the buccal mucosa as red lesions with bluish white
spots in the center (known as Koplik spots). These have frequently
disappeared by the time the patient presents to medical attention.
Immunization is very effective in preventing this infection.
All photos in this case showing disease manifestations are courtesy of Gary Williams, MD, and the
University of Wisconsin teaching file.
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Rashes Associated with Fever (Part 2)
Meningococcemia
Caused by the bacteria Neisseria meningitidis, onset is abrupt and
accompanied by fever, chills, malaise, and prostration.
The initial rash may be urticarial, maculopapular, or petechial (marked
by small, purplish, hemorrhagic spots).
In fulminant cases, it can become purpuric, marked by large
hemorrhages into the skin.
Immunization is effective in preventing this infection.
Roseola
This macular or maculopapular rash, also called exanthem subitum,
starts on the trunk and spreads to the arms and neck.
There is usually less involvement of the face and legs.
The rash is preceded by three or four days of high fevers, which end
as the rash appears.
Usually seen in children less than two years old.
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Scarlet fever
This rash, caused by infection with group A Streptococcus, consists of
very fine papules, often described as feeling like sandpaper.
It is erythematous, but blanches.
The rash starts in the groin, axillae, and neck, but rapidly spreads.
The fever can be high, and the disease is usually self-limited, lasting
less than 10 days.
It is important to treat with antibiotics to prevent non-suppurative
complications of strep, including rheumatic fever.
Varicella
The rash, also known as chicken pox, starts on the trunk and spreads
to the extremities and head.
Each lesion progresses from an erythematous macule to papule
to vesicle to pustule, and then crusts over.
Lesions at various stages of development are seen in the same area of
the body.
There is usually a mild fever.
The disease is self-limited, lasting about a week.
Immunization is effective in preventing this infection.
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Lymphadenopathy
Diffuse adenopathy-with or without palpable splenomegaly-is more concerning than an isolated
enlarged node.
Infectious Causes of Generalized Lymphadenopathy
Non-Infectious Causes of
Generalized
Lymphadenopathy
Measles (presents with a marked generalized
lymphadenopathy and splenomegaly that may last several
weeks)
Infectious mononucleosis-caused by either Epstein-Barr
virus or cytomegalovirus
Human immunodeficiency virus (HIV)
Histoplasmosis
Toxoplasmosis
Mycobacteria (can also present as localized
lymphadenopathy)
Lymphomas
Leukemia
Histiocytosis
Metastatic
neuroblastoma
Rhabdomyosarcoma
Causes of Unilateral Cervical Lymphadenopathy
In addition to Kawasaki disease and a reactive node from an oral inflammatory or infectious
process, the following can all cause unilateral cervical lymphadenopathy:
Bacterial
cervical
adenitis
Typically seen in children ages one to five years with a history of a recent
upper respiratory tract infection.
Staphylococcus aureus and Streptococcus pyogenes are the organisms
most commonly identified.
Patients may have high fevers and a toxic appearance.
Overlying cellulitis and development of fluctuance are common.
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Cat scratch
disease
Infections caused by the bacterium Bartonella henselae can be
asymptomatic or symptomatic.
The infection usually is introduced by a scratch from a cat or kitten, with
subsequent infection of the node or nodes draining that site.
The site most commonly involved is the axilla, followed by cervical,
submandibular, and inguinal areas.
Usually a self-limited disease, with regression of the lymph node in four to
six weeks.
Mycobacterial
infection
Mycobacterial infections can cause diffuse lymphadenopathy or isolated
lymphadenitis.
Lymphadenitis is the most common manifestation of nontuberculous
mycobacteria in children, with a peak age of presentation of 2 to 4 years.
Tuberculosis is the most common cause of mycobacterial lymphadenitis in
children over 12 years of age.
Children with these infections usually appear well with minimal if any
constitutional signs and symptoms.
The overlying skin may be erythematous initially, but left untreated often
becomes violaceous as the nodes enlarge.
Nodes may rupture through the skin, resulting in a draining sinus tract.
Treatment is surgical excision, as incision and drainage can also result in a
sinus tract.
Strawberry Tongue
"Strawberry tongue," an erythematous tongue with prominent papillae, is a characteristic finding of:
Streptococcal pharyngitis
Kawasaki disease
Toxic shock syndrome
Though this finding can be seen in infectious mononucleosis, it is usually in the presence of a
concomitant streptococcal pharyngitis.
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Diagnostic Criteria for Kawasaki Disease
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Diagnostic Criteria for Kawasaki Disease
In addition to high fever for at least five days, four of the following five criteria are needed for a
diagnosis of Kawasaki disease:
Changes in oral mucosa
Extremity changes (redness/swelling)
Unilateral cervical lymphadenopathy
Rash
Conjunctivitis
The one least likely to be present is cervical adenopathy.
The final criterion is that there is no other apparent cause for the presentation (i.e., a child
presenting with an obvious site of infection, even if meeting all criteria for Kawasaki, cannot be
given the diagnosis).
Though irritability is very common, it is not part of the diagnostic criteria.
Complications of Kawasaki Disease
The complications associated with Kawasaki disease-and the approximate percentages of
patients who develop these complications-are as follows:
Complication
Percentage of KD Patients Who
Experience
Central nervous system manifestations (including irritability,
lethargy, aseptic meningitis)
90%
Coronary artery aneurysm 20-25% of untreated patients
Liver dysfunction 40%
Arthritis 30%
Hydrops of the gallbladder 10%
Of these, the greatest risk is the development of coronary aneurysms. The main purpose of
treatment is to minimize this risk.
Timing
Aneurysms may be present by the end of the first week, but usually present later, almost always
within four weeks of the onset of the disease.
Monitoring
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All patients should receive an echocardiogram during the acute phase, both to look for the
presence of aneurysms and to provide a baseline for future comparison.
Clinical Skills
Dermatologic Nomenclature
The following are terms that are commonly used to describe rashes with accuracy:
Macule Flat, discolored spot
Papule Small, well-defined solid bump
Vesicle Small, well-defined, fluid-containing bump
Pustule Small, well-defined bump containing purulent material
Plaque Small, raised, differentiated patch or area on a body surface
Desquamation Shedding of the outer layer of skin surface
Clinical Reasoning
Differential Diagnosis for a Child with Fever and a Rash
Disease Description Typical features from H&P
Systemic-onset
juvenile idiopathic
arthritis (JIA)
One of a group of
common rheumatic
diseases subdivided into:
Oligoarthritis (or
pauciarticular
disease),
Polyarthritis, or
Systemic-onset
disease (also called
Still's disease)
Prolonged fever could suggest Still's
disease
Rash
Arthritis (although active arthritis in Still's
disease often presents months to years
after onset)
Visceral involvement
(e.g., hepatosplenomegaly,
lymphadenitis, serositis)
8/15
Kawasaki disease Vasculitis
Fever > 5 days
Nonpurulent conjunctivitis (may have
cleared prior to presentation)
Rash
Swelling and erythema of extremities
Osteomyelitis/septic
joint
Bacterial infection (80%
of cases occur in the
lower extremities)
Low-grade fever
Painful walking (if in lower extremity)
Rocky Mountain
spotted fever
(RMSF)
Tick-borne bacterial
infection (Rickettsia
rickettsii)
Fever
Headache
Rash (typically starts on ankles and
wrists and progresses centrally and to
palms and soles*; may be maculopapular
at first, quickly becoming petechial; in 5%
of cases, there may be no rash)
Myalgias
*Other causes of rash involving palms and
soles: Kawasaki disease, enteroviruses,
syphilis.
Scarlet fever Group A Streptococcus
infection
Fever (generally < 5 days)
A diffuse, erythematous, finely papular
rash (described as having a "sandpaper"
texture) is pathognomonic
Rash often begins at neck, axillae, and
groin and then spreads over trunk and
extremities, typically resolving within four
or five days
Stevens-Johnson
syndrome
A mucocutaneous
disorder caused by a
hypersensitivity reaction
to medications,
infections, or other
illnesses
Severe, pruritic rash (erythema
multiforme)
Fever (not expected to last 7 days)
Mucosal changes (e.g., stomatitis)
Conjunctivitis
9/15
Viral syndrome
(enterovirus)
Covers wide variety of
viral infections
Fever lasting 3-5 days (but not a
prolonged high fever)
Non-descript rash (enteroviruses can
cause rash on palms and soles)
Rash is not painful and should not cause
refusal to walk
Differential for Erythema, Pain, and Swelling of an Extremity
Consider arthritis if the pain and swelling involves a joint.
Cellulitis should also be considered, unless multiple extremities are involved.
Studies
Evaluation of Kawasaki Disease
CBC with differential
A CBC with differential will help evaluate for possible infection or inflammatory response. It is also
useful to evaluate for possible anemia, thrombocytopenia or thrombocytosis, any of which could be
present in this patient. Typical CBC findings in a patient with Kawasaki disease:
WBC: The white blood count is usually elevated, with a predominance of neutrophils.
Hbg/Hct: A normochromic, normocytic anemia is common.
MCV: The MCV is usually normal.
Platelets: The markedly elevated platelet count associated with Kawasaki disease is usually
not seen until the second week of the illness.
Blood culture
Although the clinical course is long for a bacteremic illnesses, the height of the fever and the
clinical appearance of the patient justify obtaining a culture.
Liver enzymes
Liver enzymes can be elevated in a number of conditions on the differential, including both
Kawasaki disease and Stevens-Johnson syndrome. Although this is an important test to obtain, it
is of little benefit in narrowing your diagnosis in this case. Serum albumin level is frequently low in
Kawasaki disease.
10/15
Acute phase reactants
There is great debate in the role of acute phase reactants (erythrocyte sedimentation rate [ESR] or
C-reactive protein [CRP]). Their lack of specificity limits their utility for diagnostic purposes, though
they can lend support for and against some diagnoses. They frequently play a more useful role in
following a disease process.
In this case, a negative ESR would argue strongly against Kawasaki disease. The persistence of
an elevated ESR after the fever has subsided can help to distinguish Kawasaki disease from other
infectious rash/fever illnesses.
Urinalysis
A sterile pyuria, secondary to a sterile urethritis, is associated with Kawasaki disease. A cleancatch
urine is likely to show white cells, whereas a catheterized urine may not (because the white
cells come from the urethra).
Management
Diagnosis and Treatment of Group A Streptococcus Pharyngitis
Pharyngitis secondary to group A beta-hemolytic Streptococcus (Streptococcus pyogenes) (GAS)
is a common pediatric infection. Though the acute illness is usually self-limited, diagnosis and
treatment are important to prevent potential serious sequelae, especially rheumatic fever.
Diagnosis
Patients in whom the diagnosis is suspected should have diagnostic testing performed.
A "rapid strep" test detects a streptococcal antigen from a throat swab obtained by swabbing
the tonsils and posterior pharynx. This test is highly specific, but there is variable sensitivity.
A positive rapid strep test generally does not require any further laboratory confirmation;
however, a negative result should be confirmed by standard throat culture.
Initiating Antibiotics
Ideally, there should be documentation of infection with GAS prior to initiating antibiotics. However,
some physicians will treat patients with antibiotics based on a clinical diagnosis of GAS pending
results of the culture. Others will wait for culture results in the setting of a negative rapid strep test.
Appropriate antibiotics started within nine days from the start of the acute illness will prevent acute
rheumatic fever.
11/15
Antibiotic Selection
Although penicillin is the drug of choice for GAS pharyngitis, the suspension form does not have a
pleasant taste. Therefore, many physicians opt for oral amoxicillin, which is more palatable.
In a child who refuses oral medications or when adherence to a 10-day regimen will be difficult for
the family, a single intramuscular injection of penicillin may be the best option.
Treatment of Kawasaki Disease
Aspirin and intravenous immune globulin (IVIG) have been the standard therapy for Kawasaki
disease for many years.
Aspirin
High-dose aspirin (80-100 mg/kg/day, divided into four doses) is administered for its antiinflammatory
properties.
Aspirin shortens the febrile course of the illness but has no effect on the development of
aneurysm.
Following defervescence, low-dose aspirin (3-5 mg/kg/day given in a single dose) is
administered for its anti-platelet effects.
Aspirin is discontinued altogether after a total of six to eight weeks if no coronary artery
changes are seen in follow-up echocardiograms. If there are coronary artery abnormalities,
low dose aspirin is continued indefinitely as an anti-platelet agent.
Aspirin and Reye syndrome: Children taking aspirin are at risk for experiencing Reye syndrome (a
potentially fatal illness that causes multi-organ damage) if infected with certain viruses, including
influenza virus A or B; therefore, physicians should be particularly vigilant about recommending
influenza vaccination for children receiving long-term aspirin therapy.
IVIG
The use of IVIG in Kawasaki disease has decreased the incidence of coronary artery
aneurysms from 20-25% to 2-4%.
A single dose of IVIG at a dose of 2 g/kg administered over ten to twelve hours has been
shown to be more effective in reducing the risk of coronary artery aneurysms than multiple
lower doses
Other antipyretics are usually not effective for fever control. Furthermore, use of ibuprofen has been
shown to antagonize the irreversible platelet inhibition induced by aspirin and therefore should be
avoided in children with coronary aneurysms taking aspirin for its antiplatelet effects.
Antibiotics have not been shown to have any effect on the clinical course.
The role of steroids is unclear. For years, steroids were felt to have no role, or even to be
12/15
detrimental. Newer studies suggest that they may have a role in the acute management, and
additional information is needed
Follow-up Care for Kawasaki Disease
Early cardiology follow-up with a repeat echocardiogram is needed. In Kawasaki disease, if
coronary artery aneurysms develop, they usually do so within four weeks of the acute illness, so a
follow-up visit at one to two weeks is recommended.
Anticipatory Guidance for Kawasaki Disease
Aspirin
Children are typically prescribed a low dose of aspirin to be taken for six to eight weeks.
Since aspirin can cause gastrointestinal bleeding and other problems, parents should watch
for warning signs, such as a stomachache or blood in the stool.
Aspirin can cause children to bruise easily, so certain activities should be avoided.
Parents should also make sure the child is not exposed to anyone with the flu or chicken pox
to avoid the risk of Reye's syndrome, which has been linked to aspirin use in these illnesses.
Influenza vaccine
Children should receive the flu vaccine.
Anti-inflammatories
The arthritis from Kawasaki's disease is always temporary, but can be uncomfortable.
If a child is having difficulty walking or is in pain, the physician may prescribe an antiinflammatory
medication.
Physical therapy may also be helpful, or parents may receive a list of exercises to do at
home.
Monitor for fever
If the child has symptoms of fever, parents should take their child's temperature and alert their
physician.
References
Diagnostic Standards and Classification of Tuberculosis in Adults and Children. Official Statement
13/15
of the American Thoracic Society and the Centers for Disease Control and Prevention was
Adopted by the ATS Board of Directors, July 1999. Statement was Endorsed by the Council of the
Infectious Disease Society of America. Am J Resp Crit Care Med. September 2000;161:1376-
1395, 2000.
Dunlap NE, Bass J, Fujiwara P, Hopewell P, Horsburgh CR, Salfinger M, and Simone PM.
Diagnostic standards and classification of tuberculosis in adults and children. Am. J. Respir. Crit.
Care Med 2000. 161:1376-1395
Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management
of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic
Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young,
American Heart Association. (http://pediatrics.aappublications.org/content/114/6/1708.full) Pediatrics 2004;
114:1708-1733
Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management
of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic
Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young,
American Heart Association. (http://pediatrics.aappublications.org/content/114/6/1708.full) Pediatrics 2004;
114:1708-1733
Scott JS, Ettedgui JA, Neches WH. Cost-effective use of echocardiography in children with
Kawasaki disease. Pediatrics. Nov. 1999;104(5):e57.
Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dillon
MJ. Kawasaki disease: an evidence-based approach to diagnosis, treatment, and proposals for
future research. Archives of Disease in Childhood. 2002;86(4):286-90.
Catella-Lawson F, Reilly MP, Kapoor SC, et al. Cyclooxygenase inhibitors and the antiplatelet
effects of aspirin. N Engl J Med. 2001;345 :1809 -1817
CDC Flu Guidelines: http://www.cdc.gov/flu/professionals/acip/persons.htm
Durongpisitkul K, Gururaj VJ, Park JM, Martin CF. The prevention of coronary artery aneurysm in
Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin
treatment. Pediatrics. 1995;96:1057-61.
Newburger JW, Takahashi M, Beiser AS, Burns JC, Bastian J, Chung KJ, Colan SD, Duffy CE,
Fulton DR, Glode MP, et al. A single intravenous infusion of gamma globulin as compared with
four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med. June 1991;
324(23):1633-9.
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Newburger JW, Takahashi M, Gerber MA, Gewitz MH, et al. Diagnosis, treatment, and long-term
management of Kawasaki disease: a statement for health professionals from the Committee on
Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the
Young, American Heart Association. Pediatrics 2004; 114:1708-1733
Wooditch AC, Aronoff SC. Effect of initial corticosteroid therapy on coronary artery aneurysm
formation in Kawasaki disease: a meta-analysis of 862 children. Pediatrics. 2005;116:989-995.
Brogan PA, Bose A, Burgner D, Shingadia D, Tulloh R, Michie C, Klein N, Booy R, Levin M, Dillon
MJ. Kawasaki disease: an evidence based approach to diagnosis, treatment and proposals for
future research. Archives of Disease in Childhood. 2002;86(4):286-90.
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