小児科と治療学
オープンアクセス

概要

Kawasaki disease

Deepu Abraham

Kawasaki disease (KD) is one of the most common vasculitides of childhood. It is in fact the most common cause of acquired heart disease in childhood in the developed world. The underlying pathology is infiltration of inflammatory cells into vascular tissue resulting in predominantly medium-sized artery vasculitis with a predilection for the coronaries.
There are many diagnostic challenges for KD. These include absence of confirmatory laboratory or imaging investigations, dependence on clinical criteria to diagnose and the fact that presentation is often “incomplete”, especially in infant age group. The primary goal in KD treatment is to reduce inflammatory response early in the disease course there by preventing coronary complications.
The mainstay of treatment for KD is IVIG (Intra Venous Immunoglobulin) and aspirin therapy. However, there is an increasing incidence of patients who do not respond to IVIG therapy. The incidence of coronary complications is significantly higher in the refractory KD group of children. This presentation is looking into the diagnostic challenges and management options in refractory KD cases.