Children with Down syndrome: Clinical course and mortality-associated factors in a French medical paediatric intensive care unit

Aim 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.