Publications by authors named "Alexandra Johnson"

Article Synopsis
  • - A previously healthy 6-year-old girl developed fever and then had a generalized seizure, leading to a diagnosis of acute necrotizing encephalopathy associated with influenza A, indicated by brain MRI and lab results.
  • - Her hospital stay lasted one month and was complicated by severe neurological issues requiring intensive interventions like sedatives, paralysis, hypothermia, and immune therapies (corticosteroids, plasmapheresis).
  • - Despite a serious prognosis, she made a remarkable recovery three months later, highlighting the importance of quick recognition and treatment in achieving positive outcomes, while also underscoring the complexities in predicting pediatric neurological recovery.
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Despite many surgical advances in the treatment of early onset scoliosis (EOS) over the past two decades, this condition remains a challenge to address. While otherwise healthy children can have EOS, many of these patients have complicated comorbidities making proper treatment algorithms extraordinarily difficult. Non-operative measures can be successful when initiated early, but are many times utilized as a delay tactic until growth-friendly operative procedures can be safely performed.

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Inoculation theory research offers a promising psychological 'vaccination' against misinformation. But are people willing to take it? Expanding on the inoculation metaphor, we introduce the concept of 'inoculation hesitancy' as a framework for exploring reluctance to engage with misinformation interventions. Study 1 investigated whether individuals feel a need for misinformation inoculations.

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Background: Duchenne muscular dystrophy (DMD) is a progressive, life-limiting, neuromuscular disorder. Clinicians play an important role in informing families about therapy options, including approved gene therapies and clinical trials of unapproved therapies.

Objective: This study aimed to understand the perspectives of clinicians about gene therapy for DMD, which has not previously been studied.

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is a common cause of healthcare-acquired bloodstream infections and catheter-associated urinary tract infections (CAUTIs) in both adults and children. Treatment of infection is frequently complicated by multi-drug resistance. Based on protein homology, encodes two putative hyaluronidases, EF3023 (HylA) and EF0818 (HylB).

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is a common cause of healthcare acquired bloodstream infections and catheter associated urinary tract infections (CAUTI) in both adults and children. Treatment of infection is frequently complicated by multi-drug resistance. Based on protein homology, encodes two putative hyaluronidases, EF3023 (HylA) and EF0818 (HylB).

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Background: Many adverse events are identified as nursing-sensitive indicators (NSIs) and have evidence-based care bundles known to reduce risk of occurrence. Kamishibai cards are a tool from the manufacturing industry used for practice auditing and improvements. Use of Kamishibai cards is believed to be common in the healthcare setting, but true evidence-based guidelines do not yet exist to guide their implementation.

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Avian paramyxovirus type 1 (APMV-1) is a virus of birds that results in a range of outcomes, from asymptomatic infections to outbreaks of systemic respiratory and neurologic disease, depending on the virus strain and the avian species affected. Humans are rarely affected; those who are predominantly experience mild conjunctivitis. We report a fatal case of neurologic disease in a 2-year-old immunocompromised child in Australia.

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Article Synopsis
  • Childhood dementia is a rare group of conditions leading to neurocognitive decline in children, necessitating the identification of shared characteristics for better care and research opportunities.
  • A study involving caregivers from Sydney and Adelaide collected data on 45 children with 23 different dementia syndromes, using validated questionnaires to assess various aspects of their health and behavior.
  • Findings revealed that most children exhibited significant neurological disabilities, long diagnostic delays, and common challenges such as communication difficulties, sleep disturbances, and behavioral issues, highlighting the need for a unified approach to treatment and care.
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Background: Neuronal ceroid lipofuscinosis is a group of neurodegenerative disorders with varying visual dysfunction. CLN3 is a subtype which commonly presents with visual decline. Visual symptomatology can be indistinct making early diagnosis difficult.

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Childhood dementias are a group of over 100 rare and ultra-rare pediatric conditions that are clinically characterized by chronic global neurocognitive decline. This decline is associated with a progressive loss of skills and shortened life expectancy. With an estimated incidence of one in 2800 births and less than 5% of the conditions having disease-modifying therapies, the impact is profound for patients and their families.

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Objective: We provide succinct, evidence-based and/or consensus-based best practice guidance for the cardiac care of children living with Duchenne muscular dystrophy (DMD) as well as recommendations for screening and management of female carriers of mutations in the -gene.

Methods: Initiated by an expert working group of UK-based cardiologists, neuromuscular clinicians and DMD-patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK-cardiologists, consensus was reached on these best-practice recommendations for cardiac care in DMD.

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Implantable devices capable of targeted and reversible blocking of peripheral nerve activity may provide alternatives to opioids for treating pain. Local cooling represents an attractive means for on-demand elimination of pain signals, but traditional technologies are limited by rigid, bulky form factors; imprecise cooling; and requirements for extraction surgeries. Here, we introduce soft, bioresorbable, microfluidic devices that enable delivery of focused, minimally invasive cooling power at arbitrary depths in living tissues with real-time temperature feedback control.

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In this randomized study, use of alcohol-based hand-rub disinfection significantly reduced bacterial bioburden of stethoscopes in routine clinical use. Prior cleaning of stethoscopes on the study day did not affect baseline contamination rates, which suggests that the efficacy of alcohol disinfection is short-lived and may need to be repeated between patients.

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Objective: Rett syndrome (RTT), commonly caused by methyl-CpG-binding protein 2 (MECP2) pathogenic variants, has many comorbidities. Fifty to ninety percent of children with RTT have epilepsy, which is often drug-resistant. Cannabidivarin (CBDV), a non-hallucinogenic phytocannabinoid, has shown benefit in MECP2 animal models.

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Providing hands-on education for the next generation of plant breeders would help maximize effectiveness of future breeding efforts. Such education should include training in introgression of crop wild relative alleles, which can increase genetic diversity while providing cultivar attributes that meet industry and consumer demands in a crop such as cider apple. Incorporation of DNA information in breeding decisions has become more common and is another skill future plant breeders need.

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Article Synopsis
  • Severe wildfires are hurting the variety of plants and animals in nature all around the world.
  • Indigenous people, who have special knowledge about using fire to manage land, are being forced from their homes, which makes the problem worse.
  • Studies show that when Indigenous fire practices are used, they can actually help biodiversity and the environment, but high-severity fires caused by colonization have harmed these systems.
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Heparin-induced thrombocytopenia (HIT) is a prothrombotic disorder mediated by ultra-large immune complexes (ULICs) containing immunoglobulin G (IgG) antibodies to a multivalent antigen composed of platelet factor 4 and heparin. The limitations of current antithrombotic therapy in HIT supports the need to identify additional pathways that may be targets for therapy. Activation of FcγRIIA by HIT ULICs initiates diverse procoagulant cellular effector functions.

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Problem: Children often experience pain and anxiety during a hospital stay. Effective pain and anxiety management plays a crucial role in healing. However, recent literature has highlighted multiple barriers to managing pain and anxiety in children, such as parent and provider fears of the adverse effects of pain and anxiety medications.

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Objective: Resilience training combined with medical system changes have the potential to reduce clinician burnout. We sought to estimate effects and assess feasibility of a brief gratitude intervention for primary care clinicians.

Methods: This was a pre-test/post-test single group intervention design with longitudinal follow-up.

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