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Kawasaki Disease

Kawasaki Disease. Morning Report July 16 th , 2013. Epidemiology. Previously known as mucocutaneous lymph node syndrome Incidence is greatest in children in East Asia or are of Asian ancestry Boys more commonly affected than girls 80-90% of cases in children < 5 yo

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Kawasaki Disease

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  1. Kawasaki Disease Morning Report July 16th, 2013

  2. Epidemiology • Previously known as mucocutaneous lymph node syndrome • Incidence is greatest in children in East Asia or are of Asian ancestry • Boys more commonly affected than girls • 80-90% of cases in children < 5 yo • Most countries noted increase in cases since early 2000s • Risk factors for poor coronary artery outcomes: • Young age, especially < 6 months • Asian and Pacific Islander race • Hispanic ethnicity

  3. Pathogenesis • Unknown! • Infectious? • Seasonal peaks occurred in US and Japan w/ increased incidence in localized areaspossibletransmissable vector • Toxin-mediated? Rash can resemble erythrodermasimilar to TSS or SSSS and IVIG could work by binding toxin • Infection-triggered Inflammation • Many patients w/ KD have documented concomitant infections • Infectious agents could trigger an inflammatory cascade

  4. Clinical Manifestations • Diagnosis requires fever > 5 days and at least 4 of the following features: Conjunctivitis Rash Adenopathy Strawberry Tongue Hands/Feet and Burn

  5. Clinical Manifestations • Fever • Must be present ≥ 5 days • Most consistent manifestation • Minimally responsive to anti-pyretics • Remains > 38.5 during most of illness

  6. Clinical Manifestations • Conjunctivitis • Bilateral non-exudative • Present in >90% of patients • Mainly bulbar injection which spares the limbus • Can be associated w/ photophobia • Typically begins within days of the onset of fever • Anterior uveitis may also be seen on slit lamp exam

  7. Clinical Manifesations • Rash • Usually appears within 5 days of fever onset • Often starts as desquamation in perineal area and evolves into a diffuse, erythematous maculopapular rash • Morbilliform and targetoid rashes can also occur • Vesicular/bullous lesions are rare

  8. Clinical Manifestations • Adenopathy • Unilateral • Located in anterior cervical chain • Non-fluctuant and non-tender • Diameter should be > 1.5 cm • Cervical lymph node enlargement is least consistent feature

  9. Clinical Manifestations • Strawberry Tongue (Mucositis) • Cracked, red lips • Strawberry tongue (sloughing of filiform papillae and denuding of inflamed tissue) • Discrete oral lesions are NOT seen

  10. Clinical Manifestations • Hand/Feet Changes • Indurated edema of the dorsum of their hands and feet • Diffuse erythema of palms and soles • Characteristic periungual peeling from fingers and toes begins 2-3 weeks after the onset of fever • Generally the last manifestation to appear

  11. Other Manifestations • Arthritis • Up to 25% of patients • Large joints primarily involved • GI (61%) • Diarrhea • Vomiting • Abdominal Pain • Hydrops of the gallbladder • Irritability (50%) • Likely due to meningeal inflammation • Cough or Rhinorrhea (35%)

  12. Lab Findings • Elevated Neutrophil count w/ bandemia • Elevated ESR and CRP • Anemia (normocytic, normochromic) • Hypoalbuminemia • Thrombocytosis (after the 1st week of illness) • Sterile pyuria • Elevated serum transaminases (40%) • Abnormal plasma lipids • Pleocytosis of CSF • Echo: evaluate for coronary aneurysms, decreased LV functioning, pericardial effusions

  13. Management • Echo • Obtained at diagnosis, 1-2 weeks later, and 6 weeks post-discharge • IVIG • 2g/kg • Ideally, given within first 7 days of illness, and by day 10 (from 1st day of fever) • 15% will have persistent fever after 1stdoseresistant to IVIG • Controversial, but most are given another dose after day 10 if persistent fever • Aspirin • High dose (80-100 mg/kg/d): Anti-inflammatory, given until afebrile x 48h • Low dose (3-5mg/kg/d): Anti-thrombotic, given until echo results are normal after 6 weeks

  14. Prognosis • Relates entirely to extent of cardiac involvement • If IVIG given in time, incidence of coronary artery lesions is 5% • Aneurysms can regress to normal lumen diameter, but endothelial function is still impaired in these segments • Stenoses can developincreased risk of myocardial ischemia • Most children do well after single dose of IVIG • Mortality is < 0.5% • Highest risk is in the first year after onset of illness because of acute MI in patients with giant aneurysms

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