9,930 results match your criteria: "BC Children's Hospital & University of British Columbia[Affiliation]"

Article Synopsis
  • The study aims to explore hip problems in individuals with developmental central hypotonia, focusing on various rare genetic disorders while excluding Down syndrome.
  • It analyzed 89 articles, ultimately including 79 that covered 544 subjects aged from infancy to 63 years, revealing that many of these syndromes are linked to hip structural or stability issues starting from birth, which worsen over time.
  • The findings suggest that children with notable hypotonia should undergo specific ultrasound screenings and regular orthopedic evaluations to monitor and address potential hip issues that might not be detected through standard neonatal assessments.
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The host restricts serovar Typhimurium infection of the gut via inflammasome-dependent sloughing of infected epithelial cells. Here we determined that concurrent caspase 1/11-dependent release of the goblet cell-derived mucin, Muc2, into the intestinal lumen also controls burdens in infected mice. The increased release of mucins from goblet cells in the cecum and nearby proximal colon, and the subsequent thickening of the protective mucus barrier layer in the distal colon, were all dependent on the cytokines interleukin (IL)-18 and IL-22, as deficiencies in either cytokine resulted in reduced mucin secretion.

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Article Synopsis
  • Penicillin allergies often lead to unnecessary treatment delays, as many reported allergies are not true allergies; efficient tools are needed to identify low-risk patients for safe de-labeling.
  • The FIRSTLINE electronic decision support tool was implemented to help clinicians assess and stratify penicillin allergy risk among pregnant women in Vancouver, with a focus on its reliability compared to allergist evaluations and other tools like JAMA and PENFAST.
  • Results showed that 97.2% of 181 patients were not allergic, and FIRSTLINE effectively identified a high percentage of low-risk patients, emphasizing the value of clinical algorithms in improving patient care.
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Background: Adolescence is a developmental period marked by significant changes related to the body and food parenting practices. However, few studies have explored child-level psychological correlates of these practices during adolescence. Our study examined whether adolescents' weight status, weight concerns, self-esteem, and anxiety were associated with food parenting practices, and whether these associations significantly differed by adolescents' gender among 297 families in British Columbia, Canada.

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Article Synopsis
  • Homozygous familial hypercholesterolaemia (HoFH) is a rare genetic disorder that causes extremely high LDL cholesterol levels, leading to heart disease at an early age; lomitapide is a medication designed to lower these cholesterol levels in affected adults and is being tested for safety and efficacy in children.
  • The APH-19 study involved 43 pediatric patients aged 5-17 years on existing cholesterol treatments; they received varying doses of lomitapide over a 24-week period to measure its effect on LDL cholesterol levels and other lipid parameters.
  • Results indicated a significant decrease in LDL cholesterol by 53.5% after 24 weeks of treatment, suggesting lomitapide may be effective for managing cholesterol
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Fontan circulation and systemic disease - a retrospective cohort analysis over 35 years of follow-up.

Am Heart J

January 2025

McGill Adult Unit for Congenital Heart Disease Excellence, McGill University Health Centre, Montreal, QC, Canada; Division of Cardiology, McGill University, Montreal, QC, Canada. Electronic address:

Background: The Fontan operation provides lifesaving palliation for individuals with single ventricle (SV) physiology. Given recent concerns of systemic disease (SD) for patients with a Fontan circulation, we sought to (1) quantify the increase in SD incidence associated with the Fontan circulation; (2) identify the risk factor of SD; (3) assess the association between SD and mortality in patients with a Fontan circulation.

Methods: A matched retrospective cohort study design was adopted.

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Factors affecting mammogram breast cancer surveillance effectiveness in the ipsilateral and contralateral breast.

Clin Imaging

December 2024

Department of Diagnostic Radiology, Singapore General Hospital, Singapore; Department of Diagnostic Radiology, Khoo Teck Puat Hospital, Singapore.

Aim: Mammography is the mainstay of imaging surveillance after breast cancer (BC) treatment, but false negatives can occur. The objective of the study was to determine the factors that can predict poorer second breast cancer (SBC) mammogram detection of the ipsilateral and contralateral breast separately.

Methods: A multicentre retrospective review was performed on female patients with a previous history of treated BC who developed a second breast cancer (SBC) in the ipsilateral (ISBC) or contralateral breast (CSBC) within 10 years from the first BC.

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Enhancing HPV vaccine uptake in girls and boys - A qualitative analysis of Canadian school-based vaccination programs.

Vaccine

December 2024

Vaccine Evaluation Center, BC Children's Hospital Research Institute, University of British Columbia, 950 W 28th Ave rm a5-174, Vancouver, (British Columbia) V5Z 4H4, Canada.

The purpose of this study was to better understand barriers and enabling conditions for HPV vaccination in school-based vaccination programs in Canada. Semi-structured interviews were conducted by telephone or in person with parents, nurses, and school staff (n = 50) in three Canadian provinces. Interviews explored views on HPV and HPV vaccination, strengths and weaknesses of the school-based HPV vaccination programs and proposed interventions to increase uptake.

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Introduction: Understanding the heterogeneity of brain structure in individuals with the Motoric Cognitive Risk Syndrome (MCR) may improve the current risk assessments of dementia.

Methods: We used data from 6 cohorts from the (N=1987). A weakly-supervised clustering algorithm called HYDRA was applied to volumetric MRI measures to identify distinct subgroups in the population with gait speeds lower than one standard deviation (1SD) above mean.

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Pathognomonic EEG patterns have been described in genetic conditions such as Angelman and Rett syndromes. EEG patterns along the ictal-interictal continuum have been increasingly recognized with the greater availability of continuous EEG monitoring; however, treatment decisions may be difficult with unpredictable clinical implications. Rhythmic High-Amplitude Delta Activity with Superimposed (Poly) Spikes (RHADS) has been described as a particular EEG pattern in POLG1 Alpers Syndrome.

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BIVV003 is a gene-edited autologous cell therapy in clinical development for the potential treatment of sickle cell disease (SCD). Hematopoietic stem cells (HSC) are genetically modified with mRNA encoding zinc finger nucleases (ZFN) that target and disrupt a specific regulatory GATAA motif in the BCL11A erythroid enhancer to reactivate fetal hemoglobin (HbF). We characterized ZFN-edited HSC from healthy donors and donors with SCD.

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Clear lens extraction and intraocular lens implantation to correct extreme myopia after retinopathy of prematurity.

J AAPOS

December 2024

Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, Canada, and Department of Ophthalmology, BC Children's Hospital, Vancouver, Canada. Electronic address:

We report 3 patients who developed extreme myopia after ROP laser treatment. Their subsequent optical correction was problematic, prompting bilateral clear lens extractions with intraocular lens implantation.

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Participant-reported neurological events following immunization in the Canadian National Vaccine Safety Network-COVID-19 vaccine (CANVAS-COVID) study.

Vaccine

December 2024

Vaccine Evaluation Center, BC Children's Hospital Research Institute, Vancouver, British Columbia, Canada; Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada.

Introduction: The Canadian National Vaccine Safety Network (CANVAS) conducted active participant-based surveillance for adverse events following immunization during the COVID-19 vaccine campaign. This study evaluated the association between COVID-19 vaccination and neurological adverse events.

Methods: Participants were invited to complete online surveys to report health events that prevented daily activities and/or required medical attention within 7 days after COVID-19 vaccination or 7 days prior to the survey (unvaccinated controls); follow-up surveys were sent 7 months later.

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Article Synopsis
  • Regulatory T cells (T) help maintain immune balance, but their dysfunction can cause diseases like autoimmunity and rejection of transplanted organs, spurring interest in T cell therapies.
  • The study introduces a new type of CAR-T cell that specifically targets OX40 ligand (OX40L), controlled by a synthetic promoter to enhance effectiveness and stability in treating these conditions.
  • The engineered OX40L-CAR-T cells showed improved activation, suppressing unwanted immune responses more effectively than standard T cells, and demonstrated potential for controlling both graft-versus-host disease and other autoimmune disorders.
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Background: Phenylketonuria (PKU) is caused by phenylalanine hydroxylase deficiency. Treatment is primarily a low-Phe diet combined with l-amino acid-based products (l-AA). Protein requirements in adults with PKU have not been directly determined.

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Article Synopsis
  • This study investigates microRNAs (miRNAs) and their modified versions called isomiRs, focusing on their role in lung adenocarcinoma (LUAD) and non-cancerous lung tissues.
  • The researchers identified various types of isomiRs, noting significant differences in their expression between LUAD and non-malignant samples, particularly in A-to-I editing and adenylation rates.
  • The findings suggest that isomiRs could serve as potential biomarkers for lung cancer, as machine learning models showed high accuracy in distinguishing between cancerous and non-cancerous tissues based on isomiR data.
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Although more than 140 genes have been associated with non-syndromic hereditary hearing loss (HL), at least half of the cases remain unexplained in medical genetic testing. One reason is that pathogenic variants are located in 'novel' deafness genes. A variant prioritization approach was used to identify novel (candidate) genes for HL.

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Article Synopsis
  • This study investigates how the expression of prohormone convertase 1/3 (PC1/3), an enzyme crucial for processing peptide hormones, is affected in islet cells during the progression of type 1 and type 2 diabetes.
  • Researchers analyzed pancreatic samples from a diverse group of 54 donors, using immunostaining for detailed examination of PC1/3 in various islet cells at different diabetes stages.
  • The results revealed significant changes in islet cell morphology and reduced co-localization of PC1/3 with insulin in type 1 diabetes, while an increase in glucagon and somatostatin in these islets was also noted.
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Article Synopsis
  • Solid organ transplantation is critical for saving lives in children, but the rate of pediatric organ donations is still low in Canada.
  • A survey of Canadian organ donation organizations and pediatric transplant programs revealed considerable discrepancies in policies regarding organ allocation and acceptance criteria.
  • There is a need for improved standardization in training, policies, and guidelines for pediatric organ donation and transplantation to enhance the process across the country.
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This study used meta-analysis to examine the role of baseline absolute lymphocyte count (ALC) in the prognosis of advanced breast cancer (ABC) or metastatic breast cancer (MBC). A comprehensive search encompassing PubMed, The Cochrane Library, Embase, and Web of Science databases was undertaken to identify and screen literature based on predefined inclusion and exclusion criteria. Progression-free survival (PFS), time to treatment failure (TTF), post-progression survival (PPS), and overall survival (OS) were selected as outcome measures.

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Background: Trust is a foundation of the therapeutic relationship and is associated with important patient outcomes. Building trust between parents of children with medical complexity (CMC) and physicians during inpatient care is complicated by lack of relational continuity, cumulative (sometimes negative) parent experiences and the need to adjust roles and expectations to accommodate parental expertise. This study's objective was to describe how parents of CMC conceptualize trust with physicians within the pediatric inpatient setting and to provide recommendations for building trust in these relationships.

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Exploring the effects of COVID-19 outbreak control policies on services offered to people experiencing homelessness.

BMC Public Health

October 2024

Canadian Center for Vaccinology, Dalhousie University, IWK Health, Nova Scotia Health, Goldbloom RCC Pavilion, 4th floor, 5850/5980 University Avenue, Halifax, NS, B3K 6R8, Canada.

Article Synopsis
  • The COVID-19 pandemic worsened existing inequalities, especially for people experiencing homelessness (PEH), highlighting the need for effective housing policies as a health defense.
  • Service providers in Nova Scotia faced challenges in implementing outbreak control measures but developed innovative solutions like pop-up shelters and dedicated phone lines to assist PEH.
  • The study emphasizes the necessity for adaptable and specific policies to better support service providers and PEH during health crises, particularly in rural areas with unique challenges.
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Article Synopsis
  • Type 1 diabetes (T1D) management can be tough for young adults transitioning from pediatric to adult healthcare, as they face both health care challenges and typical life changes.
  • * The DiaBetter Together study aims to assess a 12-month intervention where Peer Mentors guide young adults (ages 17-25) through this transition, focusing on healthcare navigation and self-management skills.
  • * The trial measures primary outcomes like HbA1c levels and secondary outcomes such as the time taken to switch to adult care and overall psychosocial well-being.
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We analyzed invasive group A streptococcal puerperal sepsis cases in a large health zone in Alberta, Canada between 2013 and 2022. Of the 21 cases, 85.7% were adjudicated as hospital/delivery-acquired, with 2 clusters having identical isolates found through whole genome sequencing.

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From Fetus to Eight: the CHILD Cohort Study.

Am J Epidemiol

October 2024

Department of Medicine, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada.

Article Synopsis
  • - The CHILD Cohort Study is a long-term Canadian research project tracking the health and development of infants from pregnancy until adulthood, focusing on how early life environments affect disease outcomes like asthma and developmental issues.
  • - The study involves detailed measurements and data collection including biological samples, environmental assessments, and psychosocial factors, with a strong participant retention rate over the years.
  • - A sub-cohort was created during the COVID-19 pandemic to examine its impact on families, and the next major assessment phase will occur from 2022 to 2025, contributing valuable insights into chronic diseases and health complexities.
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