title: Fibrosis and Fibrotic Gene Expression in Pediatric and Adult Patients With Idiopathic Dilated Cardiomyopathy.
abstract: BACKGROUND
  Although fibrosis seems to be prognostic for adverse outcomes in adults with idiopathic dilated cardiomyopathy (IDC), little is known about the prevalence and development of fibrosis in pediatric IDC hearts. We hypothesized that there is less activation of fibrosis at a molecular level in pediatric IDC hearts than in failing adult hearts.
  
  
  RESULTS
  Pediatric hearts were analyzed histologically to determine the prevalence of fibrosis. Left ventricular tissue from adult and pediatric IDC hearts and adult and pediatric nonfailing (NF) hearts were subjected to quantitative reverse-transcription polymerase chain reaction to study the expression of important mRNAs that affect fibrosis. We found age-specific differences between IDC and NF hearts in the regulation of noncoding galectin-3, Corin, matrix metalloproteinase (MMP) 2, MMP-9, tissue inhibitor of metalloproteinase (TIMP) 2, and TIMP-3. We also found markers that were similarly altered in both adult and pediatric IDC hearts (interleukin-1 receptor-like 1 receptor, TIMP-1, and TIMP-4). Finally, microRNAs 29a-c were significantly decreased in the pediatric IDC patients.
  
  
  CONCLUSIONS
  Pediatric IDC patients demonstrate age-specific differences in the molecular pathways implicated in fibrosis in the adult heart. At the ultrastructural level the unique gene expression pattern appears to limit fibrosis in the failing pediatric heart.
authors.affiliation: Department of Cardiology, University of Colorado, Aurora, Colorado.
authors.forename: Kathleen C
authors.initials: KC
authors.lastname: Woulfe
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
authors.forename: Austine K
authors.initials: AK
authors.lastname: Siomos
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
authors.forename: Hieu
authors.initials: H
authors.lastname: Nguyen
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
authors.forename: Megan
authors.initials: M
authors.lastname: SooHoo
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
authors.forename: Csaba
authors.initials: C
authors.lastname: Galambos
authors.affiliation: Department of Cardiology, University of Colorado, Aurora, Colorado; Division of Cardiology, Denver Health and Hospital Authority, Denver, Colorado.
authors.forename: Brian L
authors.initials: BL
authors.lastname: Stauffer
authors.affiliation: Department of Cardiology, University of Colorado, Aurora, Colorado.
authors.forename: Carmen
authors.initials: C
authors.lastname: Sucharov
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado, University of Colorado, Aurora, Colorado. Electronic address: shelley.miyamoto@childrenscolorado.org.
authors.forename: Shelley
authors.initials: S
authors.lastname: Miyamoto
mesh_terms: D000367:Age Factors; D002311:Cardiomyopathy, Dilated / Q000150:complications*; D002648:Child; D005260:Female; D005355:Fibrosis; D037502:Galectin 3 / Q000032:analysis; D020869:Gene Expression Profiling; D006333:Heart Failure / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D006352:Heart Ventricles / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007375:Interleukin-1 / Q000032:analysis; D008297:Male; D020778:Matrix Metalloproteinase 2 / Q000032:analysis; D035683:MicroRNAs / Q000235:genetics*; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D015398:Signal Transduction; D013223:Statistics as Topic; D019714:Tissue Inhibitor of Metalloproteinases / Q000032:analysis
chemical_list: D037502:Galectin 3; D007375:Interleukin-1; C532045:MIRN29a microRNA, human; D035683:MicroRNAs; D019714:Tissue Inhibitor of Metalloproteinases; D020778:Matrix Metalloproteinase 2
keywords: Pediatric idiopathic cardiomyopathy; fibrosis; gene expression