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    Add as FriendKawasaki disease

    by: pankaj

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    2 : Kawasaki Disease History of Kawasaki disease Epidemiology and etiology Presentation and diagnosis Treatment Chronic cardiovascular manifestations Follow up of patients Questions in the chronic management
    3 : Kawasaki Disease(Mucocutaneous Lymph Node Syndrome) “A self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children in the United States and Japan.” Jane Burns, MD* *Burns, J. Adv. Pediatr. 48:157. 2001.
    4 : History of Kawasaki Disease Original case observed by Kawasaki January 1961 4 y.o. boy, “diagnosis unknown” CA thrombosis 1st recognized 1965 on autopsy of child prev. dx’d w/MCOS First Japanese report of 50 cases, 1967 First English language report from Dr. Kawasaki 1974, simultaneously recognized in Hawaii
    5 : What is Kawasaki Disease? Idiopathic multisystem disease characterized by vasculitis of small & medium blood vessels, including coronary arteries
    6 : Epidemiology Median age of affected children = 2.3 years 80% of cases in children < 4 yrs, 5% of cases in children > 10 yrs Males:females = 1.5-1.7:1 Recurs in 3% Positive family history in 1% but 13% risk of occurrence in twins.
    7 : Epidemiology Annual incidence of 4-15/100,000 children under 5 years of age More in Asian-Americans, African-Americans next most prevalent Seasonal variation More cases in winter and spring but occurs throughout the year
    8 : What is the Etiology of Kawasaki Disease?
    9 : Etiology Infectious agent most likely Age-restricted susceptible population Seasonal variation Well-defined epidemics Acute self-limited illness similar to known infections No causative agent identified Bacterial, retroviral, superantigenic bacterial toxin Immunologic response triggered by one of several microbial agents
    10 : New Haven Coronavirus Identified a novel human coronavirus in respiratory secretions from a 6-month-old with typical Kawasaki Disease Subsequently isolated from 8/11 (72.7%) of Kawasaki patients & 1/22 (4.5%) matched controls (p = 0.0015) Suggests association between viral infection & Kawasaki disease Esper F, et . J Inf Dis. 2005; 191:499-502
    11 : Diagnostic Criteria Fever for at least 5 days At least 4 of the following 5 features: Changes in the extremities Edema, erythema, desquamation 2. Polymorphous exanthem, usually truncal 3. Conjunctival injection 4. Erythema&/or fissuring of lips and oral cavity 5. Cervical lymphadenopathy Illness not explained by other known disease process Modified from Centers for Disease Control. Kawasaki Disease. MMWR 29:61-63, 1980
    12 : Atypical or Incomplete Kawasaki Disease Present with < 4 of 5 diagnostic criteria Compatible laboratory findings Still develop coronary artery aneurysms No other explanation for the illness More common in children < 1 year of age 2004 AHA guidelines offer new evaluation and treatment algorithm
    13 : Differential Diagnosis Infectious Measles & Group A beta-hemolytic strep can closely resemble KD Bacterial: severe staph infections w/toxin release Viral: adenovirus, enterovirus, EBV, roseola
    14 : Differential Diagnosis Infectious Spirocheteal: Lyme disease, Leptospirosis Parasitic: Toxoplasmosis Rickettsial: Rocky Mountain spotted fever, Typhus
    15 : Differential Diagnosis Immunological/Allergic JRA (systemic onset) Atypical ARF Hypersensitivity reactions Stevens-Johnson syndrome Toxins Mercury
    16 : Phases of Disease Acute (1-2 weeks from onset) Febrile, irritable, toxic appearing Oral changes, rash, edema/erythema of feet Subacute (2-8 weeks from onset) Desquamation, may have persistent arthritis or arthralgias Gradual improvement even without treatment Convalescent (Months to years later)
    17 : Trager, J. D. N Engl J Med 333(21): 1391. 1995.
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    22 : Trager, J. D. N Engl J Med 333(21): 1391. 1995.
    23 : Han, R. CMAJ 162:807. 2000.
    24 : Kawasaki Disease:symptoms and signs Respiratory Rhinorrhea, cough, pulmonary infiltrate GI Diarrhea, vomiting, abdominal pain, hydrops of the gallbladder, jaundice Neurologic Irritability, aseptic meningitis, facial palsy, hearing loss Musculoskeletal Myositis, arthralgia, arthritis
    25 : Kawasaki Disease: Lab findings Early Leukocytosis Left shift Mild anemia Thrombocytopenia/ Thrombocytosis Elevated ESR Elevated CRP Hypoalbuminemia Elevated transaminases Sterile pyuria Late Thrombocytosis Elevated CRP
    26 : Cardiovascular Manifestations of Acute Kawasaki Disease EKG changes Arrhythmias Abnormal Q waves Prolonged PR and/or QT intervals Low voltage ST-T–wave changes. CXR–cardiomegaly
    27 : Cardiovascular Manifestations of Acute Kawasaki Disease None Suggestive of myocarditis (50%) Tachycardia, murmur, gallop rhythms Disproportionate to degree of fever & anemia Suggestive of pericarditis Present in 25% although symptoms are rare Distant heart tones, pericardial friction rub, tamponade
    28 : Role of Cardiology in the Acute Setting Usually just to document baseline coronary artery status–not an emergency If myocarditis suspected–an emergency Can help diagnose “atypical” disease
    29 : Echocardiographic Findings Myocarditis with dysfunction Pericarditis with an effusion Valvar insufficiency Coronary arterial changes
    30 : Coronary Arterial Changes 15% to 25 % of untreated patients develop coronary artery changes 3-7% if treated in first 10 days of fever with IVIG Most commonly proximal, can be distal Left main > LAD > Right
    31 : Coronary Arterial Changes Vary in severity from echogenicity due to thickening and edema or asymptomatic coronary artery ectasia to giant aneurysms May lead to myocardial infarction, sudden death, or ischemic heart disease
    32 : Coronary Aneurysms Size Small = <5 mm diameter Medium = 5-8 mm Giant = = 8 mm Highest risk for sequelae Shape Saccular Fusiform
    33 : Coronary Aneurysms • Patients most likely to develop aneurysms Younger than 6 months, older than 8 years Males Fevers persist for greater than 14 days Persistently elevated ESR Thrombocytosis Pts who manifest s/s of cardiac involvement
    34 : Circulation 103(2):335-336. 2001.
    35 : Coronary Aneurysm Approximately 50% of aneurysms resolve Smaller size Fusiform morphology Female gender Age less than 1 year Giant aneurysms (>8mm) worst prognosis
    36 : Cardiovascular Sequelae 0.3-2% mortality rate due to cardiac disease 10% from early myocarditis Aneurysms may thrombose, cause MI/death MI is principal cause of death in KD 32% mortality Most often in the first year Majority while at rest/sleeping About 1/3 asymptomatic
    37 : Acute Kawasaki Disease: Treatment IVIG: 2g/kg as one-time dose Mechanism of action is unclear Significant reduction in CAA in pts treated with IVIG plus aspirin vs. aspirin alone (15-25%?3-5%) Efficacy unclear after day 10 of illness
    38 : Acute Kawasaki Disease: Treatment IVIG 70-90% defervesce & show symptom resolution within 2-3 days of treatment Retreat those with failure of response to 1st dose or recurrent symptoms ? Up to 2/3 respond to a second course
    39 : Acute Kawasaki Disease: Treatment Aspirin High dose (80-100 mg/kg/day) until afebrile x 48 hrs &/or decrease in acute phase reactants Need high doses in acute phase due to malabsorption of ASA Dosage of ASA in acute phase does not seem to affect subsequent incidence of CAA
    40 : Acute Kawasaki Disease: Treatment Aspirin Decrease to low dose (3-5 mg/kg/day) for 6-8 weeks or until platelet levels normalize No evidence f/effect on CAA when used alone Due to potential risk of Reye syndrome instruct parents about symptoms of influenza or varicella
    41 : Acute Kawasaki Disease: Treatment Aggressive support with diuretics & inotropes for some patients with myocarditis Antibiotics while excluding bacterial infection
    42 : Acute Kawasaki Disease: Treatment Conflicting data about steroids Reports of higher incidence of aneurysms & more ischemic heart dz in pts w/aneurysms Case report of KD refractory to IVIG but responsive to high-dose steroids & cyclosporine.
    43 : Patient Follow-Up Categories Five categories based on coronary arteries findings No coronary changes at any stage of illness Transient CA ectasia, resolved within 6-8 wks Small/medium solitary coronary aneurysm One or more large or giant aneurysms or multiple smaller/complex aneurysms in same CA, without obstruction Coronary artery obstruction
    44 : Management Categories Pharmacologic therapy Physical activity Follow-up and diagnostic testing Invasive testing
    45 : I. No coronary changes at any stage of illness Pharmacologic Therapy None beyond 6-8 weeks Physical Activity No restrictions beyond 6-8 weeks Follow-up and diagnostic testing CV risk assessment, counseling @ 5 yr intervals Invasive testing None recommended
    46 : II. Transient CA ectasia, resolved within 6-8 wks Pharmacologic Therapy None beyond 6-8 weeks Physical Activity No restrictions beyond 6-8 weeks Follow-up and diagnostic testing CV risk assessment, counseling @ 5 yr intervals Invasive testing None recommended
    47 : III. Single Small or Medium Size Aneurysm Pharmacologic Therapy Low dose ASA until regression documented Physical Activity None beyond 1st 6-8 weeks in patients <11 y.o. 11-20 y.o.: Restrictions based on biennial stress test/myocardial perfusion scan Contact/high-impact discouraged if taking anti-plt drugs Follow-up and diagnostic testing Annual exam, echo, EKG CV risk assessment, counseling Invasive testing Angiography if suggestion of ischemia
    48 : IV. Aneurysms without Stenosis Pharmacologic Therapy Long-term antiplatelet tx & warfarin or LMWH Physical Activity Restrictions based on stress test/myocardial perfusion scan Contact/high-impact avoided due to risk of bleeding Follow-up and diagnostic testing Biannual exam, echo, EKG Annual stress test/myocardial perfusion scan Invasive testing Angiography @ 6-12 mos, sooner/repeated if clinically indicated Elective repeat in certain circumstances
    49 : V. Obstruction Pharmacologic Therapy Long-term low-dose ASA, ± warfarin or LMWH if giant aneurysm persists Consider ß-blockade to reduce myocardial O2 consumption Physical Activity No contact or high impact sports Other activity guided by stress testing or perfusion scan Follow-up and diagnostic testing Biannual exam, echo and EKG Annual stress test/myocardial perfusion scan Invasive testing Angiography indicated to assess lesions and guide therapy. Repeat angiography with change in symptoms.
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