MASKd: a Study on Kawasaki Disease (KD) Complicated by Macrophage Activation Syndrome (MAS)

MI-összefoglaló

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The trial is looking for children and teens with Kawasaki disease (KD) who also had macrophage activation syndrome (MAS), a severe overreaction of the immune system. It collects data from many centers in Europe. It also includes children with KD who did or did not respond to first-line treatment, as comparison groups.

Életkor

4 weeks to under 18 years at the time of KD diagnosis

Nem

All

A betegség stádiuma

KD diagnosed according to the 2024 AHA guidelines. MAS diagnosed by the treating doctor within 30 days from the start of KD.

A részvétel fő kizáró okai

• KD diagnosis not confirmed, for example a condition that looks like KD
• Primary (genetic) HLH
• No informed consent given
• MAS diagnosed more than 30 days after or more than 15 days before the start of KD

A regiszter feltételei, szó szerint

Mit akar kideríteni a vizsgálat

Kawasaki Disease (KD) is one of the most common vasculitides in childhood and represents a leading cause of acquired heart disease in developed countries. Macrophage Activation Syndrome (MAS) is a potentially life threatening hyperinflammatory condition belonging to the spectrum of hemophagocytic lymphohistiocytosis (HLH), and it can complicate various rheumatologic diseases. Awareness of MAS in the context of KD has recently increased, supporting the hypothesis that it is an underdiagnosed complication. The study aims to define the epidemiology, clinical characteristics, management, and therapeutic strategies of MAS in patients with KD, through a multicenter data collection in Europe.

A vizsgálat 3 csoportja

• MAS-KD patients.

  Patients with diagnosis of KD made according to the 2024 AHA guidelines.

• KD-resistant controls

  Patients with KD resistant to first-line therapy

• KD-responsive controls

  Patients with KD responsive to first-line therapy.