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QUESTION 1: What is the best therapy for children with Kawasaki disease who fail to respond to initial intravenous immunoglobulin?
  1. Tim J van Hasselt,
  2. Ian D Wacogne
  1. Birmingham Children's Hospital, Birmingham, UK
  1. Correspondence to Dr Tim J van Hasselt, Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK; t.vanhasselt{at}

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A 7-year-old girl was admitted with Kawasaki disease (KD), and treated with aspirin and intravenous immunoglobulin (Ig) therapy. After 48 hours of treatment she is still miserable and febrile, and you wonder what would be the most effective next step in treatment to reduce the risk of her developing coronary artery aneurysms.

Structured clinical question

In patients with KD who fail to respond to initial intravenous Ig therapy (population), what is the most effective next line of treatment (intervention) to reduce the risk of coronary artery aneurysm development (outcome)?


Cochrane library: 0 relevant

PubMed/Medline search: ‘((mucocutaneous lymph node syndrome) OR (kawasaki AND disease) OR (kawasaki's disease)) AND (intractable OR unresponsive OR re-treatment OR retreatment OR refractory OR resistant OR resistance OR rescue) AND ((coronary artery aneurysm) OR (coronary artery lesion))’ gave 102 results. A further search using the terms ‘((metaanalysis) OR (meta-analysis) OR (meta analysis))’ replacing the final term yielded nine results. The resulting abstracts were assessed, eight review articles were read in their entirety and further references extracted. Of the 138 total abstracts obtained, 61 were initially excluded as not relevant. Eleven articles were not in English and were excluded. Restricting to meta-analysis or randomised controlled trials (RCTs), there were five published RCTs, two prospective RCTs with results awaited1 ,2 and two relevant meta-analyses (table 1).

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Table 1

Summary of evidence


The presence of persistent or recurrent fever after initial treatment of KD is associated with treatment …

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  • Contributors TJvH: main author of the paper, including clinical question design, search and evidence review and manuscript. IDW: supervision, advice and direction including evidence review.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; internally peer reviewed.

  • Data sharing statement TJvH possesses full details of the literature search.

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