Defibrotide in Children With High Risk Kawasaki Disease

A Pilot Study to Determine the Safety of Defibrotide in Children With High Risk Kawasaki Disease

This study evaluates the safety of defibrotide with IVIG 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