Introduction: La ventilation non invasive (VNI) est un outil utilisé en soins intensifs pédiatriques (SIP) pour soutenir la détresse respiratoire aigüe. Un échec survient dans près de 25% des cas et une mauvaise synchronisation patient-ventilateur est un des facteurs impliqués. Le mode de ventilation NAVA (neurally adjusted ventilatory assist) est asservi à la demande ventilatoire du patient. L’objectif de cette étude est d’évaluer la faisabilité et la tolérance des enfants à la VNI NAVA et l’impact de son usage sur la synchronie et la demande respiratoire. Méthode: Étude prospective, physiologique, croisée incluant 13 patients nécessitant une VNI dans les SIP de l’hôpital Ste-Justine entre octobre 2011 et mai 2013. Les patients ont été ventilés successivement en VNI conventionnelle (30 minutes), en VNI NAVA (60 minutes) et en VNI conventionnelle (30 minutes). L’activité électrique du diaphragme (AEdi) et la pression des voies aériennes supérieures ont été enregistrées pour évaluer la synchronie. Résultats: La VNI NAVA est faisable et bien tolérée chez tous les enfants. Un adolescent a demandé l’arrêt précoce de l’étude en raison d’anxiété reliée au masque sans fuite. Les délais inspiratoires et expiratoires étaient significativement plus courts en VNI NAVA comparativement aux périodes de VNI conventionnelle (p< 0.05). Les efforts inefficaces étaient moindres en VNI NAVA (résultats présentés en médiane et interquartiles) : 0% (0 - 0) en VNI NAVA vs 12% (4 - 20) en VNI conventionnelle initiale et 6% (2 - 22) en VNI conventionnelle finale (p< 0.01). Globalement, le temps passé en asynchronie a été réduit à 8% (6 - 10) en VNI NAVA, versus 27% (19 - 56) et 32% (21 - 38) en périodes de VNI conventionnelle initiale et finale, respectivement (p= 0.05). Aucune différence en termes de demande respiratoire n’a été observée. Conclusion: La VNI NAVA est faisable et bien tolérée chez les enfants avec détresse respiratoire aigüe et permet une meilleure synchronisation patient-ventilateur. De plus larges études sont nécessaires pour évaluer l’impact clinique de ces résultats. / Introduction: The need for intubation after noninvasive ventilation (NIV) failure is frequent in the pediatric intensive care unit (PICU). One reason is patient-ventilator asynchrony during NIV. Neurally adjusted ventilatory assist (NAVA) is a mode of ventilation controlled by the patient’s neural respiratory drive. The aim of this study was to assess the feasibility and tolerance of NIV-NAVA in children and to evaluate its impact on synchrony and respiratory effort. Methods: This prospective, physiologic, crossover study included 13 patients requiring NIV in the PICU of Sainte-Justine’s Hospital from October 2011 to May 2013. Patients were successively ventilated in conventional NIV as prescribed by the physician in charge (30 minutes), in NIV-NAVA (60 minutes), and again in conventional NIV (30 minutes). Electrical activity of the diaphragm (EAdi) and airway pressure were simultaneously recorded to assess patient-ventilator synchrony. Results: NIV-NAVA was feasible and well tolerated in all patients. One patient asked to stop the study early because of anxiety related to the leak-free facial mask. Inspiratory trigger dys-synchrony and cycling-off dys-synchrony were significantly shorter in NIV-NAVA versus initial and final conventional NIV periods (both p< 0.05). Wasted efforts were also decreased in NIV-NAVA (all values expressed as median and interquartile values): 0 (0 - 0) in NIV-NAVA versus 12% (4 - 20) and 6% (2 - 22) in initial and final conventional NIV, respectively (p< 0.01). As a whole, total time spent in asynchrony was reduced to 8% (6 - 10) in NIV-NAVA, versus 27% (19 - 56) and 32% (21 - 38) in initial and final conventional NIV, respectively (p= 0.05). No difference in term of respiratory effort was noted. Conclusion: NIV-NAVA is feasible and well tolerated in PICU patients and allows improved patient-ventilator synchronization. Larger controlled studies are warranted to evaluate the clinical impact of these findings.

The potential of Digital twin (DT) technology outside of the industrial field has been recognized by researchers who have promoted the vision of applying DTs technology beyond manufacturing, to purposes such as enhancing human well-being and improving quality of life (QoL). The expanded definition of DTs to incorporate living and nonliving physical entities into the definition of DTs was a key motivation behind the model introduced in this thesis for building health digital twins of citizens. In contrast with DTs that have been developed in more industrial fields, this type of digital twins modeling necessitates protecting each citizen's unique identity while also representing features common to all citizens in a unified way. In nature, DNA is an example of a model that is both unified, common to all humans, and unique, distinguishing each human as an individual. DNA’s architecture is what inspired us to propose a digital twin DNA (DT-DNA) model as the basis for building health DTs for citizens. A review of the literature shows that no unified model for citizens’ health has been developed that can act as a base for building digital twins of citizens while also protecting their unique identity thus we aim to fill this gap in this research. Accordingly, in this thesis, we proposed a DT-DNA model, which is specifically designed to protect the unique identity of each citizen’s digital twin, similar to what DNA does for each human. We also proposed a DT-DNA-based framework to build standardized health digital twins of citizens on micro, meso and macro levels using two ISO standards: ISO/IEEE 11073 (X73) and ISO 37120. To achieve our goal, we started by analyzing the biological DNA model and the influencing factors shaping health in smart cities. The purpose of the first is to highlight the DNA model features which provide the building blocks for our DT-DNA model. The purpose of the latter is to determine the main bases of our DT-DNA model of health DTs. Based on the analysis results; we proposed DT-DNA to model health DTs for citizens. In keeping with our DNA analogy, we have identified four bases, A, T, G, and C, for our unified and unique DT-DNA model. The A base in the proposed model represents a citizen’s anthropometric when we build the DT-DNA on an individual level and represents the city’s regulatory authorities when we build the DT-DNA on community and city levels. The T base represents different tasks included in the provided health data that are required to model citizens’ health DT-DNA on different levels. The G base represents the geographic and temporal information of the city, where the citizen exists at the time of data collection. The C base represents the context at the time of data collection. To proof the concept, we present our initial work on building health DTs for citizens in four case studies. The first two case studies are dedicated for health DTs at the micro level, the third case study is dedicated for health DTs at the meso level and the fourth case study is dedicated for health DTs at the macro level. In addition, we developed an algorithm to compare cities in terms of their community fitness and health services status. The four case studies provide promising results in terms of applicability of the proposed DT-DNA model and framework in handling the health data of citizens, communities and cities, collected through various sources, and presenting them in a standardized, unique model.

Acid maltase deficiency (AMD) is a rare autosomal recessive genetic disorder that results in an accumulation of glycogen in the lysosomal storage vacuoles. It is classified as a glycogen storage disease (type II) and is also known as Pompe's disease. The prognosis of the patient with AMD is poor and the main cause of death is respiratory failure. We report a female patient whose respiratory insufficiency was documented to occur most severely during rapid eye movement sleep and who benefited clinically from the institution of nocturnal noninvasive bilevel positive airway pressure.