Kawasaki Disease Emily Caudle - PowerPoint Presentation

Kawasaki Disease Emily Caudle Jill Collins Maria Cangiani

AKA.. mucocutaneous lymph node syndrome -OR-infantile polyarteritisCharacterized by an acute generalized systemic vasculitis occurring throughout the bodySelf-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 Kawasaki Disease

Etiology remains unknownClinical evidence supports an infectious causeMost exclusively affects young children with peak incidence between 13 and 24 monthsIncreased rate of spread among children are low Exhibits geographic and seasonal outbreaks Late winter and early spring Between 9.1 and 32.5 per 100,000 children contract the disease each year in the U.S. Incidence is HIGHER in Asian-American children, followed by African Americans, Hispanics and LOWEST in White children Epidemiology

Pathophysiology Disease is characterized by VASCULITIS begins in the small vessels and progresses to involve some of the larger arteriesImmunologic 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 antibioticsSignificant irritabilitybilateral 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: 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 Clinical Manifestations: 4 stages

Stage 2: subacute phase (2-4 weeks after onset)Begins with resolution of fever and lasts until all other clinical signs have disappearedDesquamation of the fingers occurs first, followed by toesCoronary 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 15-45 days after onset of fever Clinical Manifestations

Stage 3: convalescent phase All clinical signs have resolvedLab values may not have returned to normalPhase complete when all blood values normal6-8 weeks from onsetStage 4: chronic phase40 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 Clinical Manifestations

Child must exhibit FEVER for 5 days plus four of the other five criteria, or, if fewer than four criteria, coronary vessel involvement:Bilateral conjunctival injection without exudatePolymorphous rash that may be uticarial or pruritic Inflammatory changes in the lips and oral cavityChanges 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 Diagnostic Criteria

Diagnostic Criteria (images) Conjuctival erythema Bilateral conjunctival injection

Oral mucous membrane changes, injected or fissured lips, injected pharynx Diagnostic continued… The word “injected” means RED Strawberry Tongue

Diagnostic continued….. PERIUNGUAL DESQUAMATION (Convalescent phase) Peripheral extremity changes , including erythema of palms or soles, edema of hands or feet (acute phase), and periungual desquamation (convalescent phase) ERYTHEMA of palms/soles (Acute phase)

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. Diagnostic evaluation

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 low high density lipoprotein and high triglyceride level.

Differential Diagnoses WHAT ELSE COULD IT BE ??

Kawasaki disease has replaced acute rheumatic fever as the most common cause of acquired heart disease in children .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. Kawasaki VS. Acute Rheumatic Fever

Abdominal pain FeverHeart (cardiac) problemswhich may not have symptoms, or may result in shortness of breath and chest pain Joint pain, arthritis mainly in the knees, elbows, ankles, and wristsJoint swelling; redness or warmthNosebleeds ( epistaxis)Skin nodules Skin rash ( erythema marginatum ) Skin eruption on the trunk and upper part of the arms or legs Eruptions that look ring-shaped or snake-like Sydenham chorea emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands Symptoms of RF

Contrasting KD and RF Kawasaki Disease Acute Rheumatic Fever

Once diagnosed… immediate treatment should be started Manangement plan for KD

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) 1 st Line of treatment

CARDIOVASCULAR 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. 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. 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%. Complications of Kawasaki Disease

CARDIOVASCULAR Other possible cardiac complications include:MyocarditisPericarditis CHF Pericardial effusion Mitral insufficiency Aortic insufficiency Arrythmias Complications

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 . Complications

GASTROINTESTINAL DiarrheaVomitingAbdominal pain Hydrops of the gallbladder Elevated liver enzymesHepatomegaly Acute surgical abdomen Complications

NEUROLOGICAL IrritabilityAseptic meningitisTransient hemiplegia Cerebral infarction Ataxia Seizures Focal encephalopathy LethargyFacial palsy Complications

RENAL ProteinuriaHematuriaSterile pyuria Echogenic kidneysRenal failure (rare) Complications

HEMATOLOGICAL Haemophagocytic syndrome (AKA…Hemophagocytic lymphohistiocytosis (HLH))-a rare condition caused by excess activation and proliferation of macrophages. Complications

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. Some believe it is caused by a virus or bacteria but this theory has never been proven. 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. Prevention Measures

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. Patient Education

Just a couple tidbits of information found on Kawasaki Disease that may be of interest to some: John Travolta’s son, Jett was diagnosed with Kawasaki Disease at the age of two. 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. FYI

http:// www.youtube.com/watch?v=thdcueIequ0&feature=player_detailpageENJOY and THANK YOU! Video of Kawasaki Disease

Bulbar conjunctiva (2011). Retrieved from http://www.britannica.com/EBchecked/topic/84026/bulbar-conjunctivaBurns, C., Dunn, A., Brady, M., Starr, N., & Blosser, C. (2009). Cardiovascular disorders. In S. Clark (Ed.), Pediatric Primary Care (pp. 758-764). St. Louis, MO: Saunders Elsevier. Celebrity sentry. (n.d.). Retrieved June 30, 2011, from http://www.celebritysentry.com/post/kawasaki-disease/ Chin, T. K., & Jung, L. K. (2010, February 25). Pediatric Rheumatic Fever. Medscape. Retrieved from http://emedicine.medscape.com/article/1007946-overview#a0101 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 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 KD Foundation. (2010, September 8). Kawasaki Disease [Video file]. Retrieved from http://www.youtube.com/watch?v=thdcueIequ0&feature=player_detailpage     References

Kawasaki disease. (1999, June). American Academy of Family Physicians. Retrieved from http://www.aafp.org/afp/990600ap/990600c.htmlKawasaki 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 Ogershok , P. R. (2009, August 6). Kawasaki Disease. Medscape Reference. Retrieved from http://emedicine.medscape.com/article/330081-overview Porth , C. M., & Matfin , G. (2008). Pathophysiology: Concepts of altered health states (8th ed.). Philadelphia, PA: Lippincott Williams & Wilkins. 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-kawasaki-disease?source=search_result&selectedTitle=3%7E150 Tizard , E. J. (2005). Complications of Kawasaki disease. Current Pediatrics , 62-68. References