Defibrotide in Children With High Risk Kawasaki Disease

• Defibrotide (Drug), also known as Defitelio®: Defibrotide 6.25 mg/kg IV q6h up to 7 days

Primary outcomes

• Number of patients with grade III/IV allergic reaction to defibrotide [30 days]: All patients will be monitored for allergic reaction probably or definitely related to defibrotide administration.
• Number of patients with grade III/IV hemorrhage attributable to defibrotide [42 days]: All patients will be monitored for hemorrhage probably or definitely related to defibrotide.

Secondary outcomes

• Number of patients with improvement in clinical progression/signs of Kawasaki disease [42 days]: Patients will have pre and post treatment assessments with imaging and blood tests to monitor clinical signs of Kawasaki disease

Sex: All, ages: 0 Years to 11 Years

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

• Mitchell S. Cairo, MD, Principal investigator, New York Medical College