Defibrotide in Children With High Risk Kawasaki Disease

Inclusion Criteria: * Kawasaki disease presumptive diagnosis defined according AHA criteria; * Signed informed consent and patient assent (if applicable) * Diagnosis of KD and initiation of defibrotide within 96 hours from the conclusion of IVIG treatment * Age: 0 - 11 years old * High risk category defined as patient meeting ≥2 of the following criteria: male, age \<6 months or \>8yrs, IVIG-resistance, Fever lasting greater than 10 days, prior to diagnosis, Coronary artery aneurysms, and/or Laboratory tests indicating worse systemic inflammation * PT and PTT within institutional normal limits * Platelet count ≥100,000/mm3 Exclusion Criteria: * History of Grade III or IV hemorrhage or active bleeding; * Previous Grade II-IV hypersensitivity to defibrotide * Current systemic anti-coagulant therapy and/or fibrinolytic therapy, excluding aspirin (5 mg/kg/dose maximum). * Patients on an active experimental trial for Kawasaki disease