Update on Kawasaki disease: The 25 year experience at the "A. Mayer" Children's Hospital, Florence

Kawasaki Disease (KD) is an acute febrile systemic vasculitis predominantly affecting children under 5 years of age. Coronary and peripheral artery aneurysms (CAA) develop in about 20% to 35% of untreated patients. Prompt diagnosis and early administration of high dose intravenous gammaglobulin (IVIG) and aspirin reduce the rate of coronary damage, decreasing the rate of affected children to less than 5%. KD is reported as the most common cause of acquired heart disease affecting children in developed countries, and is now considered as an additional risk for adult ischemic heart disease. Since a specific diagnostic test is still lacking, clinical criteria remain the key tools for a definitive diagnosis. Atypical and incomplete cases are a diagnostic challenge for paediatricians and a misdiagnosis, with consequent lack of timely treatment, would confer the children a high risk of developing CAA. This review will focus on the updated approach to the diagnosis and treatment of KD in a cohort of 268 children admitted to "A. Meyer" Children's Hospital in Florence, Italy, from 1982 to 2006. Our aim is to give additional clues for the diagnosis of KD, focussing mainly on atypical and incomplete cases, thus sharing with other clinicians our 25-year long experience in the management of KD patients. Our experience leads us to conclude this take-home message: a child with extreme irritability, persistently high fever and otherwise inexplicably increased indicators of inflammation should alert physicians to consider the diagnosis of KD to prevent the development of CAA by a delay in IVIG administration.