Kawasaki Disease Immunoglobulin Resistance. Diagnosis and Therapeutical Management

Peertechz Journal of Pediatric Therapy
Research Article
Abstract 
Background: Kawasaki disease (KD) is an acuteself-limited vasculitis that primarily affects children under 5 years. It is the leading cause of acquired heart disease in children in developed countries. Intravenous immunoglobulin (IVIG) and aspirin are the main stay of treatment. 20% of patients are resistant to IVIG, presenting increased risk of coronary aneurysms. Several scores have been developed in Japan to identify patients with increased risk of resistance to IVIG. Objectives and Methods: To analyze the cases of KD diagnosed, during four years, in a tertiary hospital, and analyze the characteristics of patients who were resistant to IVIG. Retrospective study of cases that met clinical criteria for EK, in our hospital, during the years 2009-2013 was performed. Results: 48 patients were diagnosed. The boy / girl ratio was 26/22. The age range was 3.3months to 13 years. Thirteen patients had atypical age. The most common clinical manifestations were fever (100%), followed by polymorphous rash (89%), changes in oral cavity (83%), bilateral conjunctivitis (78%), changes in extremities (68%) and cervical lymphadenopathy (43%). 18% were incomplete forms and three patients developed shock. 25% had echocardiographic abnormalities in the acute phase. All were treated with IVIG and aspirin. There were 8 cases refractory to IVIG. None has cardiac sequelae. Conclusions: To a KD with severity data (refractory to IVIG, persistently elevated CRP, <1 Year old or shock) there is an increased risk of developing coronary aneurysms. 
http://www.peertechz.com/Pediatric-Therapy/pdf/PJPT-2-106.pdf 

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