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Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study.

Publication ,  Journal Article
Orenstein, JM; Shulman, ST; Fox, LM; Baker, SC; Takahashi, M; Bhatti, TR; Russo, PA; Mierau, GW; de Chadarévian, JP; Perlman, EJ; Trevenen, C ...
Published in: PloS one
January 2012

Kawasaki disease is recognized as the most common cause of acquired heart disease in children in the developed world. Clinical, epidemiologic, and pathologic evidence supports an infectious agent, likely entering through the lung. Pathologic studies proposing an acute coronary arteritis followed by healing fail to account for the complex vasculopathy and clinical course.Specimens from 32 autopsies, 8 cardiac transplants, and an excised coronary aneurysm were studied by light (n=41) and transmission electron microscopy (n=7). Three characteristic vasculopathic processes were identified in coronary (CA) and non-coronary arteries: acute self-limited necrotizing arteritis (NA), subacute/chronic (SA/C) vasculitis, and luminal myofibroblastic proliferation (LMP). NA is a synchronous neutrophilic process of the endothelium, beginning and ending within the first two weeks of fever onset, and progressively destroying the wall into the adventitia causing saccular aneurysms, which can thrombose or rupture. SA/C vasculitis is an asynchronous process that can commence within the first two weeks onward, starting in the adventitia/perivascular tissue and variably inflaming/damaging the wall during progression to the lumen. Besides fusiform and saccular aneurysms that can thrombose, SA/C vasculitis likely causes the transition of medial and adventitial smooth muscle cells (SMC) into classic myofibroblasts, which combined with their matrix products and inflammation create progressive stenosing luminal lesions (SA/C-LMP). Remote LMP apparently results from circulating factors. Veins, pulmonary arteries, and aorta can develop subclinical SA/C vasculitis and SA/C-LMP, but not NA. The earliest death (day 10) had both CA SA/C vasculitis and SA/C-LMP, and an "eosinophilic-type" myocarditis.NA is the only self-limiting process of the three, is responsible for the earliest morbidity/mortality, and is consistent with acute viral infection. SA/C vasculitis can begin as early as NA, but can occur/persist for months to years; LMP causes progressive arterial stenosis and thrombosis and is composed of unique SMC-derived pathologic myofibroblasts.

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Published In

PloS one

DOI

EISSN

1932-6203

ISSN

1932-6203

Publication Date

January 2012

Volume

7

Issue

6

Start / End Page

e38998

Related Subject Headings

  • Thrombosis
  • Neutrophils
  • Myofibroblasts
  • Myocytes, Smooth Muscle
  • Myocarditis
  • Mucocutaneous Lymph Node Syndrome
  • Male
  • Lymphocytes
  • Infant
  • Humans
 

Citation

APA
Chicago
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MLA
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Orenstein, J. M., Shulman, S. T., Fox, L. M., Baker, S. C., Takahashi, M., Bhatti, T. R., … Rowley, A. H. (2012). Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. PloS One, 7(6), e38998. https://doi.org/10.1371/journal.pone.0038998
Orenstein, Jan Marc, Stanford T. Shulman, Linda M. Fox, Susan C. Baker, Masato Takahashi, Tricia R. Bhatti, Pierre A. Russo, et al. “Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study.PloS One 7, no. 6 (January 2012): e38998. https://doi.org/10.1371/journal.pone.0038998.
Orenstein JM, Shulman ST, Fox LM, Baker SC, Takahashi M, Bhatti TR, et al. Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. PloS one. 2012 Jan;7(6):e38998.
Orenstein, Jan Marc, et al. “Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study.PloS One, vol. 7, no. 6, Jan. 2012, p. e38998. Epmc, doi:10.1371/journal.pone.0038998.
Orenstein JM, Shulman ST, Fox LM, Baker SC, Takahashi M, Bhatti TR, Russo PA, Mierau GW, de Chadarévian JP, Perlman EJ, Trevenen C, Rotta AT, Kalelkar MB, Rowley AH. Three linked vasculopathic processes characterize Kawasaki disease: a light and transmission electron microscopic study. PloS one. 2012 Jan;7(6):e38998.

Published In

PloS one

DOI

EISSN

1932-6203

ISSN

1932-6203

Publication Date

January 2012

Volume

7

Issue

6

Start / End Page

e38998

Related Subject Headings

  • Thrombosis
  • Neutrophils
  • Myofibroblasts
  • Myocytes, Smooth Muscle
  • Myocarditis
  • Mucocutaneous Lymph Node Syndrome
  • Male
  • Lymphocytes
  • Infant
  • Humans