title: Association between congenital heart defects and severe infections in children with Down syndrome.
abstract: BACKGROUND
  There is a high prevalence of congenital heart disease (CHD) in Down syndrome (DS) patients. Children with DS and CHD also present greater susceptibility to pulmonary infections than those without CHD.
  
  
  OBJECTIVE
  To investigate the prevalence and types of CHD and their association with severe infections in children with DS in southern Brazil seen in a reference outpatient clinic.
  
  
  METHODS
  Children aged between six and 48 months with a diagnosis of DS were included consecutively in the period May 2001 to May 2012, and the presence of CHD and severe infections (pneumonia and sepsis) was investigated, classified and analyzed.
  
  
  RESULTS
  A total of 127 patients were included, of whom 89 (70.1%) had some type of CHD, 33 (37.7%) of them requiring surgical correction. Severe infections (pneumonia and sepsis) were seen in 23.6% and 5.5%, respectively. Of the cases of pneumonia, 70% had associated CHD (p=0.001) and of those with sepsis, 85% presented CHD (p=0.001).
  
  
  CONCLUSIONS
  Our study showed a high prevalence of CHD and its association with severe infections in children with DS seen in southern Brazil.
authors.affiliation: Faculdade Evangélica do Paraná, Curitiba, Paraná, Brasil.
authors.forename: Paula Foresti
authors.initials: PF
authors.lastname: Faria
authors.affiliation: Faculdade Evangélica do Paraná, Curitiba, Paraná, Brasil.
authors.forename: Juliana Augusta Zeglin
authors.initials: JA
authors.lastname: Nicolau
authors.affiliation: Faculdade Evangélica do Paraná, Curitiba, Paraná, Brasil.
authors.forename: Marina Zaponi
authors.initials: MZ
authors.lastname: Melek
authors.affiliation: Ambulatório de Síndrome de Down, Hospital de Clínicas-UFPR, Curitiba, Brasil.
authors.forename: Nanci de Santa Palmieri
authors.initials: Nde S
authors.lastname: de Oliveira
authors.affiliation: Ambulatório de Síndrome de Down, Hospital de Clínicas-UFPR, Curitiba, Brasil.
authors.forename: Beatriz Elizabeth Bagatin Veleda
authors.initials: BE
authors.lastname: Bermudez
authors.affiliation: Faculdade Evangélica do Paraná, Curitiba, Paraná, Brasil; Departamento de Medicina, Universidade Positivo, Curitiba, Paraná, Brasil. Electronic address: renatonisihara@up.com.br.
authors.forename: Renato Mitsunori
authors.initials: RM
authors.lastname: Nisihara
mesh_terms: D002675:Child, Preschool; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007239:Infections / Q000209:etiology*; D008297:Male; D015995:Prevalence; D012720:Severity of Illness Index
keywords: Cardiopatias; Congenital heart disease; Down syndrome; Infections; Infecções; Síndrome de Down