7,686 results match your criteria: "Children’s Hospital at Westmead[Affiliation]"

Guidelines for implementation of eye-gaze control technology by people with cerebral palsy were published in 2021. The purpose of this study was to identify barriers and facilitators clinicians perceive exist relating to use of these guidelines for eye-gaze control technology, and to explore potential resources that may be developed to support their implementation. A two-phased mixed methods explanatory sequential design was adopted.

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The development of Stereo-Electroencephalography (SEEG) in Southeast Asia and Oceania: Challenges to equity across the region.

Neurophysiol Clin

December 2024

Westmead Comprehensive Epilepsy Centre, The University of Sydney, Sydney, Australia; T.Y. Nelson Department of Neurology and Neurosurgery, The Childrens Hospital at Westmead, Sydney, Australia. Electronic address:

Background: Invasive/ intracranial EEG forms an important component of assessment for epilepsy surgery in many patients with Drug-Resistant Epilepsy (DRE). Intracranial EEG has been poorly utilized though Southeast Asia (SEA) and Oceania. This study aimed to document the development of stereo-EEG (SEEG) across the region and highlight regional barriers to utilization and access.

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Introduction: The autoimmune encephalitides (AE) are a heterogeneous group of neurological disorders with significant morbidity and healthcare costs. Despite advancements in understanding their pathophysiology, uncertainties persist regarding long-term prognosis and optimal management. This study aims to address these gaps, focusing on immunotherapeutic strategies, neoplastic associations and functional outcomes.

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HLA-compatibility remains an important triage test for deceased donor kidney allocation. Low-intermediate resolution donor HLA-typing is typically available at allocation, but its accuracy in assigning pre-transplant donor-specific anti-HLA antibody (DSA) and HLA mismatches compared to 2-field high-resolution typing is poorly characterised. Consecutive deceased donor/recipient pairs from a single centre between 2016 and 2020 were included.

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Aim: Abnormal thyroid function tests (TFTs) are found in a wide range of settings in hospitalised infants. This retrospective descriptive study reviewed management decisions and outcomes of these infants, identifying factors influencing clinicians' decision to treat with thyroxine.

Methods: Data were collected for all infants referred to the on-call endocrinology service at a tertiary Australian centre for thyroid dysfunction between 1 July 2019 and 30 June 2021.

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Compassionate access to virus-specific T cells for adoptive immunotherapy over 15 years.

Nat Commun

December 2024

Queensland Immunology Research Centre, Infection and Inflammation Program, QIMR Berghofer Medical Research Institute, Herston, Queensland, Australia.

Adoptive T-cell immunotherapy holds great promise for the treatment of viral complications in immunocompromised patients resistant to standard anti-viral strategies. We present a retrospective analysis of 78 patients from 19 hospitals across Australia and New Zealand, treated over the last 15 years with "off-the-shelf" allogeneic T cells directed to a combination of Epstein-Barr virus (EBV), cytomegalovirus (CMV), BK polyomavirus (BKV), John Cunningham virus (JCV) and/or adenovirus (AdV) under the Australian Therapeutic Goods Administration's Special Access Scheme. Most patients had severe post-transplant viral complications, including drug-resistant end-organ CMV disease, BKV-associated haemorrhagic cystitis and EBV-driven post-transplant lymphoproliferative disorder.

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Background: In Ethiopia, Maternal Death Surveillance and Response (MDSR) was integrated into the existing Integrated Disease Surveillance and Response (IDSR) system in 2014. Despite providing valuable evidence to inform policies and actions, system implementation has not been evaluated. Thus, a national-level evaluation was conducted to assess the level and status of system implementation.

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Background: Children and young people with intellectual disability have poor healthcare and are at risk of patient safety events due to lack of staff training and consensus on competencies for safe and quality care. For the adoption of reasonable adjustments in mainstream paediatric healthcare clinical competencies needed to be adapted to an existing patient safety education framework.

Methods: Thirteen experts in intellectual disability health and patient safety participated in an eDelphi survey aimed at reaching consensus on core competencies required of the paediatric healthcare workforce.

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Inborn errors of immunity reveal molecular requirements for generation and maintenance of human CD4 IL-9-expressing cells.

J Allergy Clin Immunol

November 2024

Garvan Institute of Medical Research, Darlinghurst, Australia; School of Clinical Medicine, Faculty of Medicine and Health, University of New South Wales (UNSW), Sydney, Australia. Electronic address:

Background: CD4 T cells play essential roles in adaptive immunity. Distinct CD4 T-cell subsets-T1, T2, T17, T22, T follicular helper, and regulatory T cells-have been identified, and their contributions to host defense and immune regulation are increasingly well defined. IL-9-producing T9 cells were first described in 2008 and appear to play both protective and pathogenic roles in human immunity.

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Bivalent Omicron BA.1 vaccine booster increases memory B cell breadth and neutralising antibodies against emerging SARS-CoV-2 variants.

EBioMedicine

December 2024

Centre for Virus Research, The Westmead Institute for Medical Research, Westmead, NSW, Australia; Sydney Infectious Diseases Institute (Sydney ID), Faculty of Medicine and Health, The University of Sydney, Camperdown, NSW, Australia; School of Medical Sciences, Faculty of Medicine and Health, The University of Sydney, Camperdown, NSW, Australia. Electronic address:

Background: Current literature informs us that bivalent vaccines will generate a broader serum neutralizing antibody response to multiple SARS-CoV-2 variants, but studies on how this breadth relates to the memory B cell (MBC) and T cell responses are sparse. This study compared breadth of neutralising antibody, and memory B and T cell responses to monovalent or a bivalent ancestral/Omicron BA.1 COVID-19 booster vaccine.

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Article Synopsis
  • - The study aimed to investigate parents' experiences with continuous glucose monitoring (CGM) for their young children who have early-stage type 1 diabetes enrolled in the ENDIA study.
  • - A total of nine parents participated in semi-structured phone interviews after a CGM monitoring period, highlighting three key themes: empowerment through information, acceptance of CGM use, and the benefits of involvement in research.
  • - Results showed that parents had a positive experience with their children's use of CGM, feeling supported and less uncertain about managing their child's diabetes with the valuable data provided by the monitoring.
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Aim: To explore parents' experiences of early screening for cerebral palsy (CP) in three Australian states.

Method: This is a qualitative description study using semi-structured interviews. Participants were parents of children who had CP (n = 5), or high risk of CP (n = 10), or no CP (n = 11) at 2 years, and had completed early screening for CP.

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Fenoldopam for preventing and treating acute kidney injury.

Cochrane Database Syst Rev

November 2024

Cochrane Kidney and Transplant, Centre for Kidney Research, The Children's Hospital at Westmead, Westmead, Australia.

Article Synopsis
  • Fenoldopam is a drug that selectively activates dopamine A1 receptors, increasing kidney blood flow, and it's being evaluated for its effectiveness and safety in preventing and treating acute kidney injury (AKI) in both adults and children.
  • The review examined randomized controlled trials (RCTs) involving fenoldopam related to AKI caused by surgery, radiocontrast exposure, or sepsis, using rigorous data collection and analysis methods.
  • A total of 25 RCTs were identified, involving 3,339 participants, indicating a substantial body of research focused on fenoldopam's impact on AKI.
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Background: BK polyomavirus-associated nephropathy (BKPyVAN) occurs when BK polyomavirus (BKPyV) affects a transplanted kidney, leading to an initial injury characterised by cytopathic damage, inflammation, and fibrosis. BKPyVAN may cause permanent loss of graft function and premature graft loss. Early detection gives clinicians an opportunity to intervene by timely reduction in immunosuppression to reduce adverse graft outcomes.

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The superiority of TBI-based versus chemotherapy-conditioning for allo-HSCT in children with ALL has been established in the international, prospective phase-III FORUM study (#NCT01949129), randomizing 417 patients ≤ 18 years at diagnosis (4-21 years at HSCT) in CR, transplanted from HLA-matched sibling or unrelated donors. Due to the unavailability of TBI in some regions and to accommodate individual contraindications, this study reports the pre-specified comparison of outcomes of patients receiving busulfan-based (BU) or treosulfan-based (TREO) regimens from 2013 to 2018. 180 and 128 patients (median age 9.

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Our aim is to present two cases of infantile sinonasal tract myxoma with orbital involvement and conduct a comprehensive literature review of the topic. We aim to provide a summary of the presentation of infantile sinonasal tract myxomas to effectively aid clinicians in considering this rare entity as a potential diagnosis. We present a case series and a retrospective review of the published literature in the English language.

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Newborn Genomic Sequencing Needs Confirmation but Not Repeating.

Children (Basel)

October 2024

Sydney Genome Diagnostics, Western Sydney Genetics Program, Sydney Children's Hospitals Network, Westmead, NSW 2145, Australia.

Article Synopsis
  • - Newborn screening (NBS) has significantly advanced public health, particularly with innovations like tandem mass spectrometry, and is expected to further evolve with genomic testing.
  • - While NBS typically involves multiple low-cost confirmation tests, there's concern about the feasibility of repeating expensive genetic tests like whole-genome sequencing for follow-up.
  • - The study explores how to transition NBS data into diagnostic-grade data using single-nucleotide variants (SNVs), aiming to enhance rapid diagnosis and reduce family stress when genetic issues are suspected in newborns.
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MOGAD: A comprehensive review of clinicoradiological features, therapy and outcomes in 4699 patients globally.

Autoimmun Rev

January 2025

Translational Neuroimmunology Group, Faculty of Medicine and Health, University of Sydney, Kids Neuroscience Centre, Children's Hospital at Westmead, Sydney, Australia; Sydney Medical School and Brain and Mind Centre, Faculty of Medicine and Health, University of Sydney, Sydney, Australia; Department of Neurology, Concord Hospital, Sydney, Australia. Electronic address:

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is one of the most common antibody-mediated CNS disorders. Optimal diagnostic and prognostic biomarkers remain unclear. Our aim was to clarify these biomarkers and therapeutic outcomes internationally.

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Inhibition of Mitochondrial Bioenergetics and Hypoxia to Radiosensitize Diffuse Intrinsic Pontine Glioma.

Neuro Oncol

November 2024

Translational Radiation Biology and Oncology Laboratory, Centre for Cancer Research, Westmead Institute for Medical Research, NSW, Australia.

Background: Diffuse Intrinsic Pontine Gliomas (DIPG) and other H3K27M-mutated diffuse midline gliomas (DMGs) are brain tumors that primarily affect children. Radiotherapy is the standard of care but only provides temporary symptomatic relief due to radioresistance. While hypoxia is a major driver of radioresistance in other tumors, there is no definitive evidence that DIPGs are hypoxic.

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Nationwide, Couple-Based Genetic Carrier Screening.

N Engl J Med

November 2024

From the Centre for Clinical Genetics, Sydney Children's Hospital (E.P.K., K.B., S.R., S.K.), NSW Health Pathology Randwick Genomics Laboratory (E.P.K., B.R., C.C.C., F.Z., J.F., M.B., N.Q., S.R., S.K., T.R., Y.Z.), the School of Clinical Medicine (E.P.K., M.B.), the School of Women's and Children's Health (L. Freeman, S.R., S.K.), and the Randwick Clinical Campus, Neuroscience Research Australia (Y.Z.), University of New South Wales, Randwick, Victorian Clinical Genetics Services (M.B.D., A.D.A., A.K.-P., C.H., C.L., I.D., J.E.M., K.S., L.G., L.T., M.C.O., M. Wall, M.T.M.C., M.M.F., N.L., S. Lunke, S. Eggers), the Bruce Lefroy Centre, Murdoch Children's Research Institute (M.B.D., E.A.K.), the Department of Paediatrics (M.B.D., A.D.A., E.T., J.L.H., S. Lewis, B.J.M., J. Massie, E.A.K., Z.F.), the Department of General Practice and Centre for Cancer Research (J.D.E.), and the Department of Pathology (Sebastian Lunke), University of Melbourne, Murdoch Children's Research Institute (A.D.A., E.T., J.C., J.L.H., S. Lewis, B.J.M., J. Massie, A.R., E.A.K., E.O.M., L.G., M.H., S.J., S. Lunke, S. Eggers, T.F.B.), and Australian Genomics (J.C., A.J.N., S.B., Jeffrey Braithwaite, E.O.M., K.B., S.J., Z.F., T.F.B.), Parkville, VIC, the Faculty of Medicine and Health, Sydney School of Public Health, Sydney Health Ethics, University of Sydney, Camperdown, NSW (A.J.N., L.D.), the Graduate School of Health, University of Technology Sydney (L.D., L. Freeman), Macquarie University, Australian Institute of Health Innovation (J.C.L., J. Braithwaite, T.T.), and the Faculty of Medicine and Health, University of New South Wales (K.B.-S.), Sydney (R.C.), the Peter MacCallum Cancer Centre (S.B.), the Victorian Comprehensive Cancer Centre (S.B.), the Sir Peter MacCallum Department of Oncology (S.B.) and the Department of Obstetrics and Gynaecology (S.P.W.), University of Melbourne, the Department of Respiratory Medicine and Children's Bioethics Centre, the Royal Children's Hospital (J. Massie), Genomic Diagnostics (A.K.), and Virtus Health, Virtus Genetics (S.S.-M.), Melbourne, VIC, Menzies Health Institute Queensland, Griffith University, and Griffith University School of Medicine and Dentistry, Gold Coast (M.J.D., P.A.S.), the Northern Clinical School, Faculty of Medicine and Health (K.B.S., L.B.), and Royal North Shore Hospital, Kolling Institute, Cancer Genetics Laboratory (Y.Z.), University of Sydney, St. Leonards, NSW, SA Pathology (A.K., T.H.), South Australian Clinical Genetics Service (J.L.) and the Pediatric and Reproductive Genetics Unit (L. Fitzgerald), Women's and Children's Hospital, and Repromed (J.L.), Adelaide, the Children's Hospital at Westmead, Sydney Genome Diagnostics (B.H.B., G.H., K.F.), the Specialty of Genomic Medicine, Faculty of Medicine and Health, the Children's Hospital at Westmead Clinical School, University of Sydney (B.H.B., G.H., K.F.), and the Department of Clinical Genetics, the Children's Hospital at Westmead (K.B.), Westmead, NSW, Genetic Health Queensland, Royal Brisbane and Women's Hospital (C.E., J. McGaughran, T. Clinch), and the School of Medicine, University of Queensland (Julie McGaughran), Brisbane, the Department of Diagnostic Genomics, PathWest Laboratory Medicine (D.A., M.R.D., P.K.P., R.J.N.A., R.O., T. Catchpool, N.G.L.), the School of Biological Sciences, Centre for Genetic Origins of Health and Disease (J. Beilby), the Centre for Medical Research (M.R.D., R.O., N.G.L.), and the Faculty of Health and Medical Sciences (N.P.), University of Western Australia, and Harry Perkins Institute of Medical Research (R.O., Samantha Edwards, N.G.L.), Nedlands, the Department of Pathology and Laboratory Medicine, Medical School, University of Western Australia (D.A.), and Genetic Services of Western Australia, King Edward Memorial Hospital (J.K., N.P.), Perth, the Tasmanian Clinical Genetics Service (K.H., M. Wallis) and the School of Medicine and Menzies Institute for Medical Research (M. Wallis), University of Tasmania, Hobart, the Garvan Institute of Medical Research and the School of Clinical Medicine, St. Vincent's Clinical Campus, University of New South Wales, Darlinghurst (L.B.), King Edward Memorial Hospital, Subiaco, WA (N.P.), the School of Biomedical Sciences, University of Western Australia, Crawley (R.J.N.A.), Sonic Healthcare, Douglass Hanly Moir Pathology, Macquarie Park, NSW (S.S.), Mercy Hospital for Women, Mercy Perinatal, Heidelberg, VIC (S.P.W.), and Monash IVF Group, Richmond, VIC (T.H.) - all in Australia; and the International Society for Quality in Health Care, Dublin (J. Braithwaite).

Background: Genomic sequencing technology allows for identification of reproductive couples with an increased chance, as compared with that in the general population, of having a child with an autosomal recessive or X-linked genetic condition.

Methods: We investigated the feasibility, acceptability, and outcomes of a nationwide, couple-based genetic carrier screening program in Australia as part of the Mackenzie's Mission project. Health care providers offered screening to persons before pregnancy or early in pregnancy.

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Background: New paradigms of diagnosis and treatment have changed the neurodegenerative trajectory for individuals with spinal muscular atrophy (SMA). Registries are a critical tool to provide real-world data on treatment patterns, their effects and health care provision within this evolving paradigm of care. This study aimed to evaluate the phenotypic and genotypic landscape, treatment patterns and health impact of SMA in Australia through the national registry.

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Article Synopsis
  • A study reviewed medical records from eight hospitals in Australia and New Zealand examining how intravenous aciclovir is prescribed for suspected herpes simplex virus (HSV) disease in neonates and older children between January and December 2019.
  • Out of 1,426 patients, most received aciclovir for suspected cases without definitive HSV testing; notably, only a small fraction had confirmed diagnoses among older children, indicating potentially excessive and unjustified prescribing.
  • Among the patients, adverse effects included instances of nephrotoxicity and extravasation injuries, raising concerns about the safety and necessity of such treatments in the pediatric population.
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Background: Prompt antibiotic administration for febrile neutropenia (FN) is standard of care, and targets of time to antibiotics (TTA) <60 min are common. We sought to determine the effect of TTA ≥60 versus <60 min on adverse outcomes (intensive care unit (ICU) admission or death) in children with cancer and FN. Effect modification by a decision rule that predicts infection (AUS-rule) and bacteraemia were also investigated.

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