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.