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Objective: To evaluate variations in treatment practice and compliance with national guidelines for the diagnostic evaluation of children with Kawasaki disease (KD)., Study Design: We used the Pediatric Hospital Information System database to analyze demographic, laboratory and treatment data from patients admitted with KD between January 1, 2006, and December 31, 2015., Results: During the study period, 12,089 children with KD were diagnosed. Nearly all patients had a complete blood cell count, erythrocyte sedimentation rate, and C-reactive protein ordered. Fewer patients had alanine aminotransferase (48.6%) or a urinalysis (75.3%). A small percentage of children had abdominal imaging (11.5%), neck imaging (5.9%), and lumbar punctures (4.5%), and 36.0% of patients received antibiotic therapy. Obtaining echocardiograms pretreatment and the use of steroids and infliximab significantly increased over the study period (P < 0.001). For patients who failed initial intravenous immunoglobulin (IVIG) monotherapy, 82.0% received a second dose of IVIG, 7.7% received steroids, 6.5% received infliximab, and 3.9% received combination therapy. Patients receiving infliximab or steroids as second therapy had a higher response rate than those who received only a second IVIG dose (87.9% versus 83.0% versus 73.3%, P < 0.001)., Conclusions: KD remains a challenging diagnosis. Opportunities exist for earlier use of echocardiograms in the evaluation of children with potential KD. Significant variations in practice exist surrounding second-line therapy. Our data suggest superiority of second-line therapy use of infliximab or steroids over IVIG in terms of reducing need for additional therapies. Prospective, controlled studies are needed to confirm this finding.

Background and Aims: abnormalities of liver panel (liver function test [LFT]) are frequently documented in patients with Kawasaki disease (KD). We sought to define the spectrum of abnormalities in liver panel tests in children with KD. We studied the characteristics of KD patients who presented with an abnormal liver panel and their response to treatment., Methods: we retrospectively reviewed the medical records of all KD patients admitted between 2004 and 2009 with one or more LFTs done at presentation and compared patients with and without at least 1 abnormal liver panel test including alanine aminotransferase, aspartate aminotransferase, gamma glutamyl transferase, and bilirubin. These patients were divided into 2 groups: those with normal LFTs (normal LFT group) and those with at least one abnormal LFT at presentation (abnormal LFT group)., Results: Of 259 patients, 240 (92.7%) patients with KD reviewed had one or more LFTs performed. One hundred nine (45.4%) had at least 1 abnormal liver panel test. Patients in the abnormal LFT group presented earlier (P = 0.01) and were more likely to have intravenous immunoglobulin (IVIG) resistant disease (P = 0.01). There was no significant difference between groups in development of coronary artery abnormalities or aneurysms. Multivariate analysis identified C-reactive protein and total bilirubin at admission as significant predictors for IVIG resistant disease., Conclusion: we report the largest US single center study of the spectrum of liver panel abnormalities in children with acute KD. Abnormalities of LFTs are frequently found in patients with acute KD and children with abnormal LFTs were at higher risk for IVIG resistance.