title: Children with Down syndrome: Clinical course and mortality-associated factors in a French medical paediatric intensive care unit.
abstract: OBJECTIVE
  To investigate clinical course and mortality-associated factors in children with Down syndrome (DS) managed in a medical paediatric intensive care unit.
  
  
  METHODS
  A single-centre, retrospective study conducted between 2001 and 2010 in DS children aged 1 month to 16 years.
  
  
  RESULTS
  Sixty-six patients with a median age of 24 months (1-192) and a male/female ratio of 1.5 were analysed. Patients presented with history of congenital heart disease (n = 52, 78.8%), mechanical ventilation (n = 40, 60.6%) and chronic upper airway obstruction (n = 10, 15.1%). The primary reason for admission was respiratory failure (n = 56, 84.8%). Pulmonary arterial hypertension (PAH) (n = 19, 28.8%), acute respiratory distress syndrome (ARDS) (n = 18, 27.2%) and sepsis (n = 14, 21.2%) were observed during their clinical course. Twenty-six patients died (39.4%). Mortality-associated factors included the following: (i) baseline characteristics: history of mechanical ventilation, chronic upper airway obstruction and congenital heart disease; (ii) clinical course during paediatric intensive care unit stay: sepsis, catecholamine support, ARDS, PAH and nosocomial infection. In multivariate logistic analysis, history of mechanical ventilation, ARDS and PAH remained independently associated with death.
  
  
  CONCLUSIONS
  The mortality rate in critically ill DS children admitted for medical reasons is high and is predominantly associated with respiratory conditions.
authors.affiliation: Paediatrics, Antoine Béclère Hospital, Assistance Publique - Hôpitaux de Paris, Clamart, France.
authors.forename: Christelle
authors.initials: C
authors.lastname: Joffre
authors.affiliation: Paediatric Intensive Care Unit, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris Descartes University, Paris, France.
authors.forename: Fabrice
authors.initials: F
authors.lastname: Lesage
authors.affiliation: Surgical Paediatric Intensive Care Unit, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris Descartes University, Paris, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Bustarret
authors.affiliation: Paediatric Intensive Care Unit, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris Descartes University, Paris, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Hubert
authors.affiliation: Paediatric Intensive Care Unit, Necker Hospital, Assistance Publique - Hôpitaux de Paris, Paris Descartes University, Paris, France.
authors.forename: Mehdi
authors.initials: M
authors.lastname: Oualha
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016638:Critical Illness / Q000401:mortality*; D004314:Down Syndrome / Q000150:complications* / Q000401:mortality*; D005260:Female; D017052:Hospital Mortality; D006760:Hospitalization; D006801:Humans; D007223:Infant; D015278:Intensive Care Units, Pediatric; D008297:Male; D012189:Retrospective Studies
keywords: Down syndrome; critically ill children; mortality