Background: Over a 36-month study period, 10 nonconsecutive neuromuscular pediatric patients (6 infants, mean age 10.16 months, and 4 children, mean age 9.3 years) presenting with acute respiratory failure (ARF) were treated by noninvasive positive pressure ventilation (NPPV). All patients required immediate respiratory support and fulfilled our intubation criteria. Objective: The aim of the study was to verify if early NPPV was able to avoid endotracheal intubation and to improve both oxygenation and ventilation within 24 h from admission in this clinical setting. Patients and Methods: A prospective pilot study was carried out on neuromuscular patients admitted to the pediatric intensive care unit (PICU) of the Catholic University of Rome because of ARF and managed exclusively with NPPV for at least 24 h following admission. All patients were treated using a flow-triggered mechanical ventilator through a face mask or using the new helmet interface. Results: Eight patients were successfully ventilated during the observation period and 2 early failures occurred. Among children undergoing face mask NPPV, the PaO2/FiO2 ratio increased from a median value of 75 (range 48–149) to 240 (range 133–385; p < 0.001) and 328 (range 180–371; p < 0.001) at selected time points (3 and 12 h after NPPV introduction, respectively); the alveolar-to-arterial oxygenation difference showed a similar trend, i.e. decreasing from a median value of 589 (range 213–659) to 128 (range 62–527; p < 0.01) and 69 (range 45–207; p < 0.001), respectively. Hypercarbic ARF resolved within 6 h from admission even in the most severe cases. Conclusions: NPPV was a safe and effective first-line therapeutic approach in hypoxemic ARF children/infants with neuromuscular disease. It seems of importance to identify children with neuromuscular disorders who may be able to achieve residual ventilator-free breathing and to perform an NPPV trial avoiding tracheal intubation. Life-threatening respiratory distress and very young age should not preclude NPPV application in the PICU setting. The new helmet interface represents a promising tool for noninvasive ventilation in older children.

Keywords:
Emergency presentation, Helmet, Hypoxemic respiratory failure, Intensive care, Neuromuscular disorders, Noninvasive ventilation, Pediatric intensive care unit
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2006
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