title: Abnormal matrix remodeling in adolescents and young adults with Kawasaki disease late after onset.
abstract: BACKGROUND
  Patients with a history of Kawasaki disease (KD), have been found to have pericoronary and myocardial fibrosis. Serum biomarkers of fibrosis may be sensitive indices for detection of these late cardiac complications in KD patients.
  
  
  METHODS
  We studied a cohort of 60 adolescents and young adults comprising 10 KD patients with persistent coronary artery lesions (CAL) occurring at a mean (SD) time of 14.5 (4.4) years after disease onset, 25 KD patients with no CAL after disease onset, and 25 healthy age-matched volunteers. We compared laboratory data from the patients and volunteers, including lipid profile, liver function, amino-terminal propeptide of type III procollagen (PIIINP), matrix metalloproteinase 9 (MMP-9), tissue inhibitor of metalloproteinase 1 (TIMP-1), and MMP-9:TIMP-1 ratios. Severity of CAL was determined on the basis of computed tomography determinations of the frequency of aneurysms and the extent of coronary stenosis/occlusion, thrombosis, and calcification.
  
  
  RESULTS
  Increased PIIINP and decreased MMP-9 and TIMP-1 concentrations and decreased MMP-9:TIMP-1 ratios were found not only in KD patients with persistent CAL but also in KD patients without CAL, although to a lesser extent in the latter group. In KD patients, the concentrations of PIIINP were positively associated with the severity of coronary stenosis/occlusion (r = 0.72, P = 0.011) and with the extent of coronary thrombus (r = 0.64, P = 0.014). The concentrations of high-sensitivity C-reactive protein, however, did not differ across groups.
  
  
  CONCLUSIONS
  Our results demonstrate alterations in extracellular matrix biomarkers in KD patients, suggesting enhanced collagen synthesis and ameliorated degradation in adolescents and young adults late after the onset of KD. We also observed an association between the concentrations of PIIINP and the extent of coronary stenosis/occlusion or thrombosis in KD patients, a finding that needs confirmation in further studies.
authors.affiliation: Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.
authors.forename: Ming-Tai
authors.initials: MT
authors.lastname: Lin
authors.forename: Shyh-Jye
authors.initials: SJ
authors.lastname: Chen
authors.forename: Yi-Lwun
authors.initials: YL
authors.lastname: Ho
authors.forename: Kuo-Chin
authors.initials: KC
authors.lastname: Huang
authors.forename: Chun-An
authors.initials: CA
authors.lastname: Chen
authors.forename: Shuenn-Nan
authors.initials: SN
authors.lastname: Chiu
authors.forename: Li-Chuan
authors.initials: LC
authors.lastname: Sun
authors.forename: Wen-Jeng
authors.initials: WJ
authors.lastname: Lee
authors.forename: Hui-Chi
authors.initials: HC
authors.lastname: Chen
authors.forename: Jou-Kou
authors.initials: JK
authors.lastname: Wang
authors.forename: Mei-Hwan
authors.initials: MH
authors.lastname: Wu
mesh_terms: D000328:Adult; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D015331:Cohort Studies; D005109:Extracellular Matrix / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D020780:Matrix Metalloproteinase 9 / Q000097:blood; D009080:Mucocutaneous Lymph Node Syndrome / Q000000981:diagnostic imaging / Q000473:pathology*; D010446:Peptide Fragments / Q000097:blood; D011347:Procollagen / Q000097:blood; D019715:Tissue Inhibitor of Metalloproteinase-1 / Q000097:blood; D014057:Tomography, X-Ray Computed
chemical_list: D015415:Biomarkers; D010446:Peptide Fragments; D011347:Procollagen; D019715:Tissue Inhibitor of Metalloproteinase-1; C041876:procollagen Type III-N-terminal peptide; D020780:Matrix Metalloproteinase 9