Publications by authors named "Aaron St-Laurent"

Article Synopsis
  • Spinal muscular atrophy (SMA) is a genetic disorder causing muscle weakness and breathing issues, but gene replacement therapy has improved its treatment landscape, with unknown long-term effects on sleep.
  • A study reviewed infants with SMA diagnosed through newborn screening who received the gene therapy onasemnogene abeparvovec, with polysomnography data collected over time.
  • While all eleven infants initially showed sleep disordered breathing, improvements were observed with therapy; however, some still needed respiratory support, indicating the importance of assessing both motor function and breathing symptoms for a comprehensive understanding of their health.
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Objectives: Sleep disordered breathing (SDB) is a well-documented complication of vagus nerve stimulation (VNS) in the literature. Yet, a formal consensus on its management has not been established, particularly in the pediatric population. This study aims to evaluate the current literature on VNS-associated SDB in order to further characterize its presentation, pathogenesis, diagnosis, and treatment.

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Introduction: Understanding real-world profiles from neuromuscular databases is helpful for optimizing clinical care and planning research studies. The Canadian Neuromuscular Disease Registry (CNDR) has respiratory data from a population of boys with Duchenne Muscular Dystrophy (DMD).

Objectives: To describe cross-sectional respiratory profiles from a national DMD real-world dataset.

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Objectives: To describe the current clinical practice patterns of Canadian pediatric respirologists at pediatric tertiary care institutions regarding chronic tracheostomy tube care and management of home invasive ventilation.

Methods: A pediatric respirologist/pediatrician with expertise in tracheostomy tube care and home ventilation was identified at each Canadian pediatric tertiary care center to complete a 59-item survey of multiple choice and short answer questions. Domains assessed included tracheostomy tube care, caregiver competency and home monitoring, speaking valves, medical management of tracheostomy complications, decannulation, and long-term follow-up.

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Unlabelled: With the growing prevalence of obesity in the pediatric population, reports of its severe complications are increasing. Obesity hypoventilation syndrome is an uncommon disorder in children with altered respiratory mechanics, sleep-disordered breathing, and impaired ventilatory responses leading to persistent hypercapnia. Presentation is varied, and children may remain relatively asymptomatic until challenged with a respiratory infection, when they may present with acute respiratory failure.

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Children with neuromuscular, chronic neurologic, and chest wall diseases are at increased risk of postoperative respiratory complications including atelectasis, pneumonia, and respiratory failure with the possible need for reintubation or even tracheostomy. These complications negatively impact patient outcomes, including increased healthcare resource utilization and increased surgical mortality. In these children, the existing respiratory reserve is often inadequate to withstand the stresses brought on during anesthesia and surgery.

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Objective: To provide a systematic review of the existing pediatric decannulation protocols, including the role of polysomnography, and their clinical outcomes.

Methods: Five online databases were searched from database inception to May 29, 2020. Study inclusion was limited to publications that evaluated tracheostomy decannulation in children 18 years of age and younger.

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Objective: Chiari malformation type I (CM-I) involves the herniation of the cerebellar tonsils through the foramen magnum. CM-I is associated with both obstructive sleep apnea (OSA) and central sleep apnea (CSA) in children. The primary management of symptomatic CM-I remains surgical decompression.

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Murine Ly49 receptors, which are expressed mainly on NK and NKT cells, interact with MHC class I (MHC-I) molecules with varying specificity. Differing reports of Ly49/MHC binding affinities may be affected by multiple factors, including cis versus trans competition and species origin of the MHC-I L chain (β2-microglobulin). To determine the contribution of each of these factors, Ly49G, Ly49I, Ly49O, Ly49V, and Ly49Q receptors from the 129 mouse strain were expressed individually on human 293T cells or the mouse cell lines MHC-I-deficient C1498, H-2(b)-expressing MC57G, and H-2(k)-expressing L929.

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The Nkrp1 (Klrb) family of NK cell receptors and their genetically linked Clr (Clec2) ligands are conserved between rodents and humans. Nonetheless, certain mouse and rat Nkrp1 genes exhibit significant allelic polymorphism between inbred strains. We previously demonstrated that the Nkrp1-Clr recognition system is genetically and functionally conserved between the B6 and BALB/c strains, with focused sequence divergence evident in certain genes (e.

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Ly49Q is a member of the polymorphic Ly49 family of NK cell receptors that displays both a high degree of conservation and a unique expression pattern restricted to myeloid lineage cells, including plasmacytoid dendritic cells (pDC). The function and ligand specificity of Ly49Q are unknown. Here, we use reporter cell analysis to demonstrate that a high-affinity ligand for Ly49Q is present on H-2(b), but not H-2(d), H-2(k), H-2(q), or H-2(a)-derived tumor cells and normal cells ex vivo.

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