Coronary Artery Disease
Factors Associated With Resource Utilization and Coronary Artery Dilation in Refractory Kawasaki Disease (from the Pediatric Health Information System Database)

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Management guidelines for refractory Kawasaki disease (KD) are vague. We sought to assess practice variation and identify factors associated with large/complex coronary artery aneurysms (LCAA) and resource utilization in refractory KD. This retrospective cohort study identified patients aged ≤18 years with KD (2004 to 2014) using the Pediatric Health Information System. Refractory KD was defined as receiving >1 dose of intravenous immunoglobulin. Demographics, medications, concomitant infections, length of stay (LOS), and charges were collected. Antithrombotic therapy was a surrogate for LCAA. LOS and hospital charges assessed resource utilization. Multivariate regression identified factors associated with LOS, charges, and LCAA. Of 14,194 patients with KD, 2,974 (21%) had refractory KD and 203 of those 2,974 (7%) had LCAA. Additional intravenous immunoglobulin was the sole medication in 77%. Other medications added were steroids (18%), infliximab (2%), and both (3%). Warfarin, low-molecular-weight heparin, tissue plasminogen activator, and clopidogrel were prescribed with equal frequency (2%). Male gender (adjusted relative risk 1.52, 95% confidence interval [CI] 1.08 to 2.16, p <0.01), admission to an intensive care unit (4.79, 95% CI 3.40 to 6.74, p <0.001), arrhythmia (3.00, 95% CI 1.94 to 4.65, p <0.001), and concomitant viral infection (2.29, 95% CI 1.49 to 3.52, p <0.001) were associated with LCAA. Severe illness, race, region, and payer were independently associated with increased charges (p <0.05 for all). In conclusion, treatment for refractory KD varies widely. Concomitant viral infection was associated with a greater risk of LCAA in refractory KD. Better understanding of optimal management may improve outcomes and decrease both variability in management and resource utilization for refractory KD.

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Methods

For this retrospective, observational cohort study, data were obtained from the Pediatric Health Information System (PHIS). The PHIS database was queried for patients aged ≤18 years discharged with KD (International Classification of Diseases, Ninth Revision, code: 446.1) and a medication charge for IVIG from January 1, 2004, to March 31, 2014. PHIS is a large, inpatient, administrative database which obtains information from 45 tertiary care, free-standing, not-for-profit pediatric hospitals

Results

Of the 14,194 patients identified as having KD, 2,974 (21%) received >1 dose of IVIG and formed the refractory KD study group (62% men). Compared to the responsive KD cohort, patients with refractory KD were older, had longer hospital LOS, and higher total hospital charges (Table 1, p <0.01 for all). Medicaid and private insurance were the most common providers for both KD groups. The proportion of patients admitted for refractory KD was highest in the south (37%) and the lowest was in the

Discussion

This study showed wide practice variation in PHIS hospitals in the use of immunomodulator therapy for patients with refractory KD. Consistent with a previous report that IVIG was the most common (65%) initial medication chosen to treat patients with refractory KD,11 it was the only medication used for a larger proportion (77%) in this PHIS database study. The previous investigators also reported greater use of solumedrol and infliximab than that in our study (27% vs 18% and 8% vs 2%,

Disclosures

The authors have no conflicts of interest to disclose.

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Funding: This investigation was supported by the University of Utah Study Design and Biostatistics Center, with funding in part through Grant 5UL1TR001067-02 (formerly 8UL1TR000105 and UL1RR025764) from the National Center for Research Resources and the National Center for Advancing Translational Sciences, National Institutes of Health.

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