Transcript Kawasaki Disease - Jill Collins MSN Portfolio

Emily Caudle
Jill Collins
Maria Cangiani
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AKA..
◦ mucocutaneous lymph node syndrome
-OR◦ infantile polyarteritis
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Characterized by an acute generalized systemic
vasculitis occurring throughout the body
Self-limiting and is the most common cause of
acquired heart disease in children in Japan and the
U.S.
Over 80% of patients with Kawasaki disease are 4
years of age or younger
◦ Male to female ratio of 4:1
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Etiology remains unknown
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Most exclusively affects young children
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Exhibits geographic and seasonal outbreaks
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Between 9.1 and 32.5 per 100,000 children contract the
disease each year in the U.S.
◦ Clinical evidence supports an infectious cause
◦ with peak incidence between 13 and 24 months
◦ Increased rate of spread among children are low
◦ Late winter and early spring
◦ Incidence is HIGHER in Asian-American children, followed by
African Americans, Hispanics and LOWEST in White children
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Disease is characterized by VASCULITIS
◦ begins in the small vessels and progresses to involve some of
the larger arteries
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Immunologic abnormalities
◦ increased activation of helper T cells
◦ increased levels of immune mediators and antibodies
 destroy endothelial cells have been detected during the acute phase
of the disease
Pathophysiology
◦ Stage 1: acute phase (0-14
days) begins with abrupt
onset of high fever that is
unresponsive to antipyretics
or antibiotics
 Significant irritability
 bilateral nonpurulent conjunctival
injection, erythema or the
oropharynx
 dryness and fissuring of the lips
 “strawberry tongue”
 cervical lymphadenopathy
 polymorphous rash
 erythema of urethral meatus
 tachycardia
 edema of extremities
 Lab findings:
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an elevated ESR
platelet count
positive CRP
leukocytosis with left shift
slight decreases in RBCs
and hemoglobin.
 Initially platelets may be
normal with gradual
increase after 7th day of
fever
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Stage 2: subacute phase (2-4 weeks after onset)
◦ Begins with resolution of fever and lasts until all other
clinical signs have disappeared
◦ Desquamation of the fingers occurs first, followed by toes
◦ Coronary artery aneurysms appear during this period in
15% -25% of untreated children and less than 5% of
treated children
◦ Death from the disease occur from cardiac sequelae 1545 days after onset of fever
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Stage 3: convalescent phase
◦ All clinical signs have resolved
◦ Lab values may not have returned to normal
◦ Phase complete when all blood values normal
 6-8 weeks from onset
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Stage 4: chronic phase
◦ 40 days to years after illness
◦ Coronary complications, if present, can persist into
adulthood
◦ Children with coronary dilation or aneurysms may have
long-term coronary endothelial changes, which place the
child at risk for early ischemic disease
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Child must exhibit FEVER for 5 days plus four of
the other five criteria, or, if fewer than four criteria,
coronary vessel involvement:
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Bilateral conjunctival injection without exudate
Polymorphous rash that may be uticarial or pruritic
Inflammatory changes in the lips and oral cavity
Changes in the extremities, such as peripheral edema,
erythema of the palms and soles, or desquamation of the
hands and feet
◦ Cervical lymphadenopathy that is often unilateral,
anterior cervical
Conjuctival erythema
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Oral mucous
membrane changes,
injected or fissured
lips,
injected pharynx
The word “injected” means
RED
Strawberry Tongue
including erythema of palms
or soles, edema of hands or feet (acute phase), and
periungual desquamation (convalescent phase)
PERIUNGUAL DESQUAMATION
(Convalescent phase)
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The diagnostic of Kawasaki disease is based on
clinical manifestations. The CDC requires that
fever and four of the six other criteria listed above
in stage I be demonstrated.
 Electrocardiogram, echocardiogram, cardiac catheterization,
and angiocarddiography may be required to diagnose
cardiac abnormalities.
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Although there are no specific laboratory tests, the following may
help support diagnosis or rule out other disease.
◦ 1.CBC
 Leukocytosis during acute stage.
◦ 2.Erythrocytes and hemoglobin
 slight decrease.
◦ 3.Platelet count
 increased during second to fourth week of illness.
◦ 4.IgM, IgA, IgG, and IgF
 transiently elevated.
◦ 5.Urine
 protein and leukocytes present.
◦ 6.Acute phase reactants
 (ESR, C-reactive protein, alpha I antitrypsin) are elevated during the acute
phase.
◦ 7.Myocardial enzyme levels (serum CK-MB)
 suggest MI if elevated.
◦ 8.Liver enzymes (AST, ALT)
 moderately elevated.
◦ 9.Lipid profile
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low high density lipoprotein and high triglyceride level.
Measles
Erythema
Multiforme
Leptospirosis
Adenovirus
Kawasaki
Disease
StevensJohnson
Syndrome
Kawaskaki
Disease
Scarlet
Fever
Drug
reaction
Inflammatory
Bowel
Disease
Toxic shock
Rickettsial
Infection
SLE
Sarcoidosis
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Kawasaki disease has replaced acute rheumatic fever
as the most common cause of acquired heart disease in
children.
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Rheumatic fever (RF) is an inflammatory disease that
may develop after an infection with Streptococcus
bacteria (such as strep throat or scarlet fever). The
disease can affect the heart, joints, skin, and brain.
◦ It arises as a complication of untreated or inadequately
treated strep throat infection. Rheumatic fever can
seriously damage the valves of the heart.
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Abdominal pain
Fever
Heart (cardiac) problems
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Joint pain, arthritis
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Joint swelling; redness or warmth
Nosebleeds (epistaxis)
Skin nodules
Skin rash (erythema marginatum)
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Sydenham chorea
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◦ which may not have symptoms, or may result in shortness of
breath and chest pain
◦ mainly in the knees, elbows, ankles, and wrists
◦ Skin eruption on the trunk and upper part of the arms or legs
◦ Eruptions that look ring-shaped or snake-like
◦ emotional instability, muscle weakness and quick, uncoordinated
jerky movements that mainly affect the face, feet, and hands
Acute Rheumatic Fever
Kawasaki Disease
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Once diagnosed…
◦ immediate treatment
should be started
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The recommended initial therapy includes:
◦ IVIG
 (2 gm/kg) administered as a single infusion over 8 to 12
hours
◦ Aspirin
 (initial dose of 80 to 100 mg/kg daily divided into four
doses).
◦ The AHA and the AAP recommend these two
medications for the treatment of acute KD.
(Additional agents are used only for children who fail to respond to standard therapy)
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CARDIOVASCULAR
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The most common and potentially life-threatening complication of
KD is coronary artery aneurysm.
The aneurysm is a result of the chronic inflammation of the blood
vessels (vasculitis) which causes a weakening in the vessel wall.
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◦ The aneurysm can eventually burst leading to internal bleeding or
more often, blood clots form in the area leading to occlusion of the
coronary artery and myocardial infarction.
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If untreated, up to 25% of patients with KD develop coronary
artery aneurysms. Most aneurysms develop within 6-8 weeks
from the onset of illness. If treatment is started within 10 days of
the diagnosis, the incidence of coronary artery
disease/complications drops to approximately 2%.
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CARDIOVASCULAR
Other possible cardiac complications include:
 Myocarditis
 Pericarditis
 CHF
 Pericardial effusion
 Mitral insufficiency
 Aortic insufficiency
 Arrythmias
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LONG TERM CARDIAC SEQUELAE
A multi-centre follow-up study was done in Japan
obtaining cardiac status on 1594 patients who presented
with KD in 1996. Of the 1338 in whom follow up data
was available, 10.3% had cardiac sequelae at 1 month
and 4.2% at 1 year. The prevalance was greater in
males. About 50% of aneurysms regressed within 5
years. The main cause of death in KD is myocardial
infarction secondary to thrombosis of an aneurysm or
stenosis.
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GASTROINTESTINAL
Diarrhea
Vomiting
Abdominal pain
Hydrops of the gallbladder
Elevated liver enzymes
Hepatomegaly
Acute surgical abdomen
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NEUROLOGICAL
Irritability
Aseptic meningitis
Transient hemiplegia
Cerebral infarction
Ataxia
Seizures
Focal encephalopathy
Lethargy
Facial palsy
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RENAL
Proteinuria
Hematuria
Sterile pyuria
Echogenic kidneys
Renal failure (rare)
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HEMATOLOGICAL
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Haemophagocytic syndrome (AKA…Hemophagocytic
lymphohistiocytosis (HLH))-a rare condition caused by excess
activation and proliferation of macrophages.
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At this point in time, the exact cause of Kawasaki
Disease remains unknown and so it is unknown how
to prevent the onset of the disease.
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Some believe it is caused by a virus or bacteria but
this theory has never been proven.
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It is important, however, to note that when the disease
is diagnosed and treatment is initiated in the very early
stages, all most all of the complications that were
discussed previously can be prevented. Full recovery
can be expected for most patients diagnosed with the
disease.
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Patient education about this disease revolves
around early recognition of the symptoms as well
as seeking treatment as soon as possible. There
is a great educational handout on Kawasaki
Disease that can be obtained by visiting the
American Family Physician website at
http://www.aafp.org/afp/990600ap/990600c.html.
The handout is copyrighted but permission is
given to print and photocopy for nonprofit
educational uses.
Just a couple tidbits of information found on Kawasaki
Disease that may be of interest to some:
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John Travolta’s son, Jett was diagnosed with Kawasaki Disease
at the age of two.
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There has been some speculation that the vaccine used to help
prevent rotavirus infection (RotaTeq) has been linked to
development of Kawasaki Disease.
◦ To this date there has not been enough data to support this claim and the
CDC continues to support the safety and effectiveness of the RotaTeq
vaccine in preventing rotavirus infection. For more information on this
visit http://www.cdc.gov/vaccinesafety/vaccines/rotavirus.html.
http://www.youtube.com/watch?v=thdcueIequ0&feat
ure=player_detailpage
ENJOY and THANK YOU!
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Celebrity sentry. (n.d.). Retrieved June 30, 2011, from http://www.celebritysentry.com/post/kawasakidisease/
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Chin, T. K., & Jung, L. K. (2010, February 25). Pediatric Rheumatic Fever. Medscape. Retrieved from
http://emedicine.medscape.com/article/1007946-overview#a0101
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Fisman, D. N. (2000, Nov-Dec). Hemophagocytic syndromes and infection. Emerging Infectious Diseases,
6(6). Retrieved from http://www.cdc.gov/ncidod/eid/vol6no6/fisman.htm
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Jatla, K. K. (2011). Medscape reference. In H. Roy, Sr (Ed.), Ophthalmologic Manifestions of Kawasaki
Disease. Retrieved from http://emedicine.medscape.com/article/1197545-overview#aw2aab6b3
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KD Foundation. (2010, September 8). Kawasaki Disease [Video file]. Retrieved from
http://www.youtube.com/watch?v=thdcueIequ0&feature=player_detailpage
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Kawasaki disease. (1999, June). American Academy of Family Physicians. Retrieved from
http://www.aafp.org/afp/990600ap/990600c.html
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Kawasaki syndrome and RotaTeq vaccine. (2011, February 8). Centers for Disease Control and
Prevention: Vaccine Safety. Retrieved from http://www.cdc.gov/vaccinesafety/vaccines/rotavirus.html
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Ogershok, P. R. (2009, August 6). Kawasaki Disease. Medscape Reference. Retrieved from
http://emedicine.medscape.com/article/330081-overview
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Porth, C. M., & Matfin, G. (2008). Pathophysiology: Concepts of altered health states (8th ed.).
Philadelphia, PA: Lippincott Williams & Wilkins.
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Sundel, R. (2011, January). Epidemiology and etiology of Kawasaki disease. UpToDate. Retrieved from
http://0-www.uptodate.com.topekalibraries.info/contents/epidemiology-and-etiology-of-kawasakidisease?source=search_result&selectedTitle=3%7E150
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Tizard, E. J. (2005). Complications of Kawasaki disease. Current Pediatrics, 62-68.