Marzo 2017 - Volume XXXVI - numero 3
Aggiornamento
1Università di Trieste; 2Università “La Sapienza”, Roma; 3Policlinico Universitario “G. Martino”, Università di Messina; 4Clinica Pediatrica, Dipartimento di Medicina Clinica e Sperimentale dell’Università di Pisa, Azienda Ospedaliera-Universitaria Pisana; 5Servizio di Reumatologia Pediatrica, Ospedale “Anna Meyer”, Firenze; 6Scuola di Specializzazione in Pediatria, Università di Cagliari
Indirizzo per corrispondenza: elisa.benelli@gmail.com
Key words: Kawasaki disease, Retropharyngeal abscess, Febrile cholestatic jaundice, Hemophagocytic syndrome, Corticosteroids, Acetylsalicylic acid
Kawasaki disease (KD) is a systemic vasculitis that typically affects children of 2-5 years of age and whose diagnosis and treatment are usually easy, as described in the first case report. Anyway, in spite of adequate therapy, 10-15% of patients develop coronary artery aneurysms. The high-risk patients are those under 1 year of age or with incomplete or atypical presentations. For this reason, to recognize them promptly is fundamental to start adequate therapy. This article describes some cases of the atypical presentation of KD: a retropharyngeal abscess, a febrile cholestatic jaundice and a haemophagocytic syndrome. Moreover, it reports some severe cases of KD such as a severe cardiac shock, the case of a patient that received anti-TNF therapy because of inefficacy of standard therapy and finally the case of an adult that underwent coronary artery bypass surgery because of the consequences of a giant coronary aneurism developed at 2 years of age after a KD. At the end of the article, the messages inferred from the described case reports are summarized in 10 points.
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