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An Old Autopsy Report Sheds Light on a “New” Disease: Infantile Polyarteritis Nodosa and Kawasaki Disease

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Abstract

Although Kawasaki disease (KD) was first discovered and identified in Japan by Kawasaki in the 1960s, fatal KD cases resulting from coronary artery aneurysms had been identified retrospectively in the West as early as 1871. Kawasaki initially postulated that this disease was a new, as yet unidentified, self-limiting illness with no fatal coronary sequelae. The connection between fatal cases, then diagnosed as infantile polyarteritis nodosa, was not made until the late 1970s. Kawasaki’s thoughts were reinforced by an apparent absence of nonfatal cases in the West before 1967. Close examination of a 1948 autopsy report suggests that nonfatal cases of KD did indeed exist, at least in the United States, before its emergence in Japan in the early 1950s. These nonfatal cases of KD were misdiagnosed as Stevens-Johnson syndrome. The autopsy report reviewed in this article reinforces the likelihood that KD did occur in the United States before it was identified as Kawasaki disease in Japan.

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Notes

  1. The Japanese report was not connected to Kawasaki’s publication that same year.

  2. It is worth noting that 10 percent of Kawasaki's original 50 cases do not fit any of the current case definitions of KD. See Burns, Commentary on the translation of Kawasaki, 1967.

  3. The other diagnoses that met the retrospective criteria were allergic toxic syndrome, Izumi fever (caused by Yersinia pseudotuberculosis), scarlet fever, or bacterial cervical lymphadenitis.

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Acknowledgments

We thank Jane Burns and Christena Turner of University of California, San Diego, and John Bastian of Rady Children’s Hospital, San Diego, for comments and suggestions, and Carol Kushner for her editorial support. This research was supported by a grant from Public Education and Communication on Improving Health Care Delivery of Kawasaki Disease, the National Institutes of Health, and the National Library of Medicine (G13LM007855).

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Correspondence to Howard I. Kushner.

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Kushner, H.I., Abramowsky, C.R. An Old Autopsy Report Sheds Light on a “New” Disease: Infantile Polyarteritis Nodosa and Kawasaki Disease. Pediatr Cardiol 31, 490–496 (2010). https://doi.org/10.1007/s00246-009-9625-9

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  • DOI: https://doi.org/10.1007/s00246-009-9625-9

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