15 results match your criteria: "Dalhousie University and IWK Health Center[Affiliation]"

Article Synopsis
  • The article discusses the discovery of germline biallelic null mutations in the ARPC5 gene, affecting the Arp2/3 actin nucleator complex, in two patients with severe recurrent infections, early-onset autoimmunity, and other health issues.
  • The mutations lead to compromised functions across multiple cell types, but restoring protein expression in vitro can rescue the complex's conformation and functions.
  • The study reveals that IL-6 signaling is uniquely affected, with important distinctions between classical and trans-signaling pathways, suggesting potential therapeutic targets for treatment.
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Background: Children ≤36 months with diffuse intrinsic pontine glioma (DIPG) have increased long-term survival (LTS, overall survival (OS) ≥24 months). Understanding distinguishing characteristics in this population is critical to improving outcomes.

Methods: Patients ≤36 months at diagnosis enrolled on the International DIPG Registry (IDIPGR) with central imaging confirmation were included.

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Our understanding of inflammatory bowel disease is changing as we identify genetic variants associated with immune dysregulation. Inflammatory bowel disease undetermined, even when diagnosed in older children and adolescents, in the setting of multiple inflammatory and infectious diseases should raise the suspicion of complex immune dysregulation with a monogenic basis. We report a case of inflammatory bowel disease undetermined triggered by exposure to a nonsteroidal antiinflammatory drug in a 16-year-old girl with a background history of juvenile idiopathic arthritis, cytopenias, recurrent respiratory tract and middle ear infections, and esophageal candidiasis.

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Background: Fentanyl is a commonly used off-label medication for pain control and sedation in preterm infants. Yet, the effect of fentanyl on cerebral hemodynamics in preterm neonates remains unexplored.

Objective: To evaluate the effect of a bolus dose of fentanyl on the regional cerebral oxygen saturation (RcSO2), cerebral fractional tissue oxygen extraction (cFTOE) and left ventricular output (LVO) as compared with pre-administration baseline in preterm infants.

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Presently, undernutrition still goes undetected in pediatric hospitals despite its association with poor clinical outcomes and increased annual hospital costs, thus affecting both the patient and the health care system. The reported prevalence of undernutrition in pediatric patients seeking care or hospitalized varies considerably, ranging from 2.5 to 51%.

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Objective: To assess the prevalence, causes, and consequences of malnutrition, as well as the evolution of nutritional status, in Canadian pediatric health care institutions.

Study Design: In this multicenter prospective cohort study, a total of 371 patients were recruited from pediatric hospitals in 5 Canadian provinces. Subjects were aged 1 month to 18 years; admitted to a medical, surgical, or oncology ward; and had a planned hospital stay of >48 hours.

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Objectives: To compare the respiratory syncytial virus (RSV)-related hospitalization rate, hospital length of stay (LOS), and need for assisted ventilation in children aged <2 years with Down syndrome and those without Down syndrome.

Study Design: MEDLINE, Embase, and CINAHL databases were searched from inception up to December 2017. Studies that provided data on RSV-related hospitalization in children aged <2 years with Down syndrome and those without Down syndrome were included.

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Importance: Despite increasing emphasis on conservative management of patent ductus arteriosus (PDA) in preterm infants, different pharmacotherapeutic interventions are used to treat those developing a hemodynamically significant PDA.

Objectives: To estimate the relative likelihood of hemodynamically significant PDA closure with common pharmacotherapeutic interventions and to compare adverse event rates.

Data Sources And Study Selection: The databases of MEDLINE, Embase, and the Cochrane Central Register of Controlled Trials were searched from inception until August 15, 2015, and updated on December 31, 2017, along with conference proceedings up to December 2017.

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Background And Objectives: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease of unknown etiology, with limited population-based estimates of pediatric incidence. We reported the incidence of pediatric AIH in Canada and described its clinical characteristics.

Methods: We conducted a retrospective cohort study of patients aged <18 years diagnosed with AIH between 2000-2009 at all pediatric centers in Canada.

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Unlabelled: Under the auspices of the Initiative on Methods, Measurement, and Pain Assessment in Clinical Trials (IMMPACT), 26 professionals from academia, governmental agencies, and the pharmaceutical industry participated in a 2-stage Delphi poll and a consensus meeting that identified core outcome domains and measures that should be considered in clinical trials of treatments for acute and chronic pain in children and adolescents. Consensus was refined by consultation with the international pediatric pain community through announcement of our recommendations on the Pediatric Pain List and inviting and incorporating comments from external sources. There was consensus that investigators conducting pediatric acute pain clinical trials should consider assessing outcomes in pain intensity; global judgment of satisfaction with treatment; symptoms and adverse events; physical recovery; emotional response; and economic factors.

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The objective of the study was to survey recently hired physicians to Canadian Academic Departments of Pediatric and Internal Medicine to understand the factors that underlay successful recruitment. Recruits and Chairs agreed on the 10 most important values. Chairs overvalued the 10 least important Recruit values.

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Background: Although all jurisdictions in Canada offer annual influenza immunization to people at high risk of complications, only Ontario has provided universal annual immunization of healthy adults and children. Use of chemotherapy (amantidine, neuraminidase inhibitors) to prevent influenza varies among provinces. We sought to systematically review the evidence for the prevention of influenza infection in the general population.

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