9,936 results match your criteria: "BC Children's Hospital & University of British Columbia[Affiliation]"

Importance: Few studies have investigated whether the associations between pregnancy-related factors and breast cancer (BC) risk differ by underlying BC susceptibility. Evidence regarding variation in BC risk is critical to understanding BC causes and for developing effective risk-based screening guidelines.

Objective: To examine the association between pregnancy-related factors and BC risk, including modification by a of BC where scores are based on age and BC family history.

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  • Genome-wide association studies (GWAS) have identified genetic links to autoimmune disorders but don't pinpoint causal variants or affected cell types; this research enhances understanding using advanced 3D genomic datasets.
  • By integrating various genomic techniques, the study maps disease-associated variants to likely regulatory effector genes across 57 human cell types, revealing the complex genetic landscape of autoimmune diseases.
  • The investigation identifies both shared and specific genetic pathways, leading to the exploration of squalene synthase as a potential drug target for controlling inflammation in conditions like multiple sclerosis and systemic lupus erythematosus.
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An INSULIN and IAPP dual reporter enables tracking of functional maturation of stem cell-derived insulin producing cells.

Mol Metab

November 2024

BC Children's Hospital Research Institute, Vancouver, BC, Canada; Department of Surgery, University of British Columbia, Vancouver, BC, Canada; School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada. Electronic address:

Objective: Human embryonic stem cell (hESC; SC)-derived pancreatic β cells can be used to study diabetes pathologies and develop cell replacement therapies. Although current differentiation protocols yield SCβ cells with varying degrees of maturation, these cells still differ from deceased donor human β cells in several respects. We sought to develop a reporter cell line that could be used to dynamically track SCβ cell functional maturation.

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  • Aberrant expression of HOX and MEIS1 family genes in certain leukemias disrupts normal blood cell differentiation and contributes to leukemia development.
  • Menin inhibitors can target the interaction between KMT2A and menin, reducing the abnormal expression of key factors and promoting differentiation in these leukemias.
  • A collaborative effort among pediatric and adult specialists aims to advance menin inhibitors in treatment, offering a comprehensive overview of clinical trials and advocating for inclusive trial designs for youth.
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Treatment differences and long-term outcomes in adults and children with Ewing sarcoma.

Cancer Epidemiol

October 2024

Department of Medical Oncology, BC Cancer, Vancouver, BC, Canada; Department of Medicine, University of British Columbia, Vancouver, BC, Canada. Electronic address:

Introduction: Ewing sarcoma is an aggressive malignancy primarily affecting children and adolescents. Limited research is available on treatment practices, clinical course, and survival in adults.

Methods: A multi-institution retrospective cohort study of all adults (>18 years) and children (≤18 years) with Ewing sarcoma treated in British Columbia, Canada between January 01, 2000 and December 31, 2018.

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Objective: To describe presenting diagnoses and rates and causes of death by age category and sex among children with acute illness brought to a district headquarter hospital in Pakistan.

Design: Prospective cohort study.

Setting: Sanghar district headquarter hospital, Sindh, Pakistan between December 2019 and April 2020 and August 2020 and December 2020.

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  • - Juvenile xanthogranuloma (JXG) typically affects the skin, but a rare form called systemic JXG (sJXG) can occur, sometimes linked with serious complications like hemophagocytic lymphohistiocytosis (HLH), particularly in younger children.
  • - A study on 17 children with sJXG found that those with HLH exhibited specific symptoms such as fever and rash, and they were treated with corticosteroids and targeted therapy, showing promising outcomes with chemotherapy.
  • - The overall survival rates for sJXG patients were similar regardless of whether they had HLH, with certain treatments like LCH-based chemotherapy and dabrafenib proving effective, especially
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Developmental and epileptic encephalopathies (DEEs) feature altered brain development, developmental delay and seizures, with seizures exacerbating developmental delay. Here we identify a cohort with biallelic variants in DENND5A, encoding a membrane trafficking protein, and develop animal models with phenotypes like the human syndrome. We demonstrate that DENND5A interacts with Pals1/MUPP1, components of the Crumbs apical polarity complex required for symmetrical division of neural progenitor cells.

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Background: Laterality and bilaterality have been reported as prognostic variables in developmental dysplasia of the hip (DDH) outcomes. However, there is little clarity across the literature on the reporting of laterality in developmental dysplasia of the hip (DDH) due to the variability in severity of the condition. It is widely accepted that the left hip is most frequently affected; however, the true incidence of unilateral left, unilateral right and bilateral cases can be hard to quantify and compare across studies.

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Building Clinical Care Capacity for Patients With Special Pathogens in Advance of the Next Outbreak.

Health Secur

September 2024

Lauren Wiesner, MD, is Chair, Department of Emergency Medicine; Aaron Resnick, MA, is Program Director, Biocontainment Unit; Bethany Little, BSN, RN, NHDP-BC, is Nursing Operations Director and Training and Education Coordinator, Biocontainment Unit; Glenn Wortmann, MD, is Section Director, Infectious Diseases; Craig DeAtley, PA-C, is Director, Institute for Public Health Emergency Readiness; and Shane B. Kappler, MD, MS, FACEP, is Medical Director, Biocontainment Unit; all at MedStar Washington Hospital Center, Washington, DC. Lauren Wiesner is also an Associate Professor of Emergency Medicine; Glenn Wortmann is also a Professor of Clinical Medicine (Infectious Diseases); and Shane B. Kappler is also an Assistant Professor of Emergency Medicine and Microbiology and Immunology; all at Georgetown University School of Medicine, Washington, DC. Jade Flinn, MSN, RN, CCRN, CNRN, is Director of Operations, Johns Hopkins Special Pathogens Center, and Brian T. Garibaldi, MD, MEHP, FACP, FRCP(E), was Director, Johns Hopkins Special Pathogens Center, and Director, Johns Hopkins Biocontainment Unit; all at The Johns Hopkins Hospital, Baltimore, MD. Brooke Brewer, RN, MS, CIC, is Program Manager, Infection Prevention; Natalie A. Schnell, BSN, RN, R-BC, CIC, is an Infection Prevention Nurse Educator; and David A. Wohl, MD, and William A. Fischer II, MD, are Co-Directors; all at the Special Pathogen Response Center, Region 4 Emerging Special Pathogen Treatment Center, University of North Carolina Hospitals, Chapel Hill, NC. William A. Fischer II is also Director of Emerging Pathogens, Institute for Global Health and Infectious Diseases, The University of North Carolina at Chapel Hill, Chapel Hill, NC. Sharon Vanairsdale Carrasco, DNP, APRN, ACNS-BC, NP-C, CEN, FAEN, FAAN, is an Associate Professor, Nell Hodgson Woodruff School of Nursing, Emory University, and Program Director, Region 4 Ebola and Other Special Pathogens Treatment Center, Emory University Hospital/Children's Healthcare of Atlanta; both in Atlanta, GA.

In response to the growing number of outbreaks of emerging infectious diseases, the US Administration for Strategic Preparedness and Response (ASPR) has embarked on a plan to improve and expand special pathogen patient care capabilities. To achieve this, ASPR is developing a coordinated network of Regional Emerging Special Pathogen Treatment Centers (RESPTCs) to serve as state-of-the-art facilities staffed by a highly trained workforce to care for and manage special pathogen patients across the lifespan. The RESPTC network represents the operational arm of a broader US National Special Pathogen System of care to prevent and prepare for the next infectious disease outbreak.

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Flow cytometry assay modifications: Recommendations for method validation based on CLSI H62 guidelines.

Cytometry B Clin Cytom

August 2024

Division of Hematopathology, Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, Minnesota, USA.

The Clinical and Laboratory Standards Institute (CLSI) H62-Validation of Assays Performed by Flow Cytometry guideline, released in 2021, provides recommendations for platform workflow and quality system essentials, instrument setup and standardization, assay development and optimization and fit-for-purpose analytical method validation. In addition, CLSI H62 includes some recommendations for the validation strategies after a validated flow cytometric method has been modified. This manuscript builds on those recommendations and discusses the impact of different types of assay modifications on assay performance.

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Lysine from Whole Wheat Bread Consumed by Healthy Adult Males Has High Metabolic Availability When Assessed Using the Indicator Amino Acid Oxidation Method.

J Nutr

August 2024

Research Institute, Hospital for Sick Children, Toronto, ON, Canada; Department of Nutritional Sciences, University of Toronto, Toronto, ON, Canada. Electronic address:

Background: The protein quality of wheat is limited by its low content of the indispensable amino acid (AA) lysine and the metabolic availability (MA) of lysine in wheat bread for humans is unknown.

Objectives: The study objective was to determine the MA of lysine in whole wheat bread.

Methods: Five healthy young males (≤30 y, body mass index <25) were studied in a repeated-measures design using the indicator amino acid oxidation (IAAO) method, with L-[1-C] phenylalanine as the indicator.

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Background: Availability of high-level pediatric training for nurses in Malawi is limited. To address this gap, a novel pediatric critical care nurse preceptor program was developed and implemented by pediatric nurse specialists.

Aim: Evaluate the effectiveness of a pediatric critical care nurse preceptor program, via change in nurses' knowledge, skills, confidence, and precepting competence.

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Risk Factors Related to the Development of Full-thickness Pressure Injuries in Hospitalized Pediatric Patients.

Adv Skin Wound Care

September 2024

Ann Marie Nie, PhD, MSN, APRN, FNP-BC, CWOCN, is Wound, Ostomy Nurse Practitioner and Pressure Injury Preventionist, Dayton Children's Hospital, Dayton, Ohio, United States. Elizabeth Hawkins-Walsh, PhD, MSN, CPNP-PC, PMHS, FAANP, is Professor of Nursing Practice (Emerita), Conway School of Nursing, Catholic University of America, Washington, District of Columbia. Barbara Delmore, PhD, RN, CWCN, MAPWCA, FAAN, is Senior Nurse Scientist, Center for Innovations in the Advancement of Care, Departments of Nursing, NYU Langone Health, and Clinical Assistant Professor, Hansjörg Wyss, Department of Plastic Surgery, NYU Grossman School of Medicine, New York, New York.

Article Synopsis
  • Current pediatric pressure injury prevention measures are largely based on adult risk factors, but children's unique developmental stages require different considerations.
  • This study analyzed 799 hospitalized children to identify specific risk factors for full-thickness pressure injuries (PIs) across different age groups, using a detailed statistical model.
  • Findings revealed that risk factors for PIs vary significantly by age, with conditions like tissue perfusion, fragile skin, and malnutrition playing key roles in younger patients, highlighting the need for tailored prevention strategies for children.
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Objective: Evaluate the cognitive, behavioural and affective processes involved in therapeutic change for young people with epilepsy and mental health difficulties receiving an integrated mental health intervention.

Methods: As part of a mixed methods convergent design, qualitative data were gathered in parallel to quantitative data at two timepoints in a randomised controlled trial testing the Mental Health Intervention for Children with Epilepsy in addition to usual care. Twenty-five young people and/or their families were interviewed before and after the intervention about the young person's mental and physical health, and their experience of therapy.

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Penicillin allergy is reported in 10% of the population; however, over 90% of patients are deemed non-allergic upon allergist assessment. The goal of this quality improvement project is to validate a patient-driven assessment tool to safely identify patients at low risk of penicillin allergy and de-label them. Pediatric patients and pregnant women referred to the institution's allergy clinics for penicillin allergy assessment were invited to use the patient tool to complete a self-assessment, resulting in the assignment of a risk category.

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  • The study investigates how residential air pollution, specifically particulate matter (PM), black carbon (BC), and sulfate, affects the progression of kidney disease in patients with primary glomerulopathies over a duration of at least two years.
  • Participants from two cohorts were analyzed to determine the link between air pollution exposure and decline in kidney function, measured by estimated glomerular filtration rate (eGFR).
  • Results indicate that higher exposure to air pollutants correlates with faster disease progression and increased systemic inflammation markers, suggesting air quality may significantly impact kidney health.
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Antisense Oligonucleotide Therapy for Calmodulinopathy.

Circulation

October 2024

Department of Cardiology (R.H.B., F.N., C.P., M.A.T., M.P., M.E.S., P.W., J.C., F.L., C.L., P.B., E.M.K., D.Y., N.P., T.S., Q.M., D.J.A., V.J.B., W.T.P.).

Background: Calmodulinopathies are rare inherited arrhythmia syndromes caused by dominant heterozygous variants in , , or , which each encode the identical CaM (calmodulin) protein. We hypothesized that antisense oligonucleotide (ASO)-mediated depletion of an affected calmodulin gene would ameliorate disease manifestations, whereas the other 2 calmodulin genes would preserve CaM level and function.

Methods: We tested this hypothesis using human induced pluripotent stem cell-derived cardiomyocyte and mouse models of pathogenic variants.

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Introduction/objectives: Exposure to adverse social determinants of health (SDoH) in childhood is associated with poorer long-term health outcomes. Within structurally marginalized populations, there are disproportionately high rates of developmentally vulnerable children. The RICHER (Responsive, Intersectoral, Child and Community Health, Education and Research) social pediatric model was designed to increase access to care in marginalized neighborhoods.

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  • The study aims to investigate the rising cesarean section rates in Greece and implement interventions to promote vaginal delivery through a trial named ENGAGE, focusing on evidence-based practices.
  • Twenty-two maternity units across Greece will participate in a multicenter trial involving 20,000 to 25,000 births, employing a stepped-wedge design whereby units will gradually implement interventions over 8-18 months.
  • Key interventions include applying updated clinical guidelines, training on cardiotocography, and providing ongoing support to healthcare professionals, with data on cesarean rates and outcomes collected for analysis throughout the study.
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Background: Clinicians and researchers should consider the expected benefits and potential harms of an intervention. Parenting programmes are a widely used evidence-based intervention for child behaviour problems. However, few data are available on potential negative effects.

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Disturbed glucose homeostasis and its increased allostatic load in response to individual, joint and fluctuating air pollutants exposure: Evidence from a longitudinal study in prediabetes.

Sci Total Environ

November 2024

Department of Epidemiology and Biostatistics, Institute of Basic Medical Sciences Chinese Academy of Medical Sciences, School of Basic Medicine Peking Union Medical College, Beijing 100005, China; Center of Environmental and Health Sciences, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100005, China. Electronic address:

We investigated the effect of individual, joint and fluctuating exposure to air pollution (PM, BC, NO, NH, OM, SO, PM, NO, SO, O) on glucose metabolisms among prediabetes, and simultaneously explored the modifying effect of lifestyle. We conducted a longitudinal study among prediabetes during 2018-2022. Exposure windows within 60-days moving averages and their variabilities were calculated.

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