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Ewing sarcoma is the second most frequent primary bone tumour of childhood and adolescence. The aim of this report is to describe the imaging, pathology, clinical findings, and treatment of a primary intradural extramedullary Ewing sarcoma with a unique intracranial metastatic component in a pediatric patient. A 14-year-old girl with a history of mood disorders presented to the emergency department with a 3-week history of neck torticollis, cervical pain, paresis, and paresthesia of the upper and lower extremities on the left side. Initially, non-organic causes such as somatization or conversion disorder were suspected. She returned 3 months later when her symptoms worsened. MRI of the head and spine was performed, and demonstrated the presence of a suprasellar, retro-chiasmatic mass lesion. There was also diffuse leptomeningeal enhancement, another well-defined intradural extramedullary lesion the sacral region and several multifocal cauda equina soft tissue nodules. The patient first underwent surgery. The patient was also treated with a combination of chemotherapy (vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide (VDC/IE)) and radiation as per the Children's Oncology Group AEWS1221 protocol. Most recent imaging conducted 22 months after the initial mass discovery revealed improvement of the suprasellar mass lesion with residual stable appearance of the prominence and enhancement of the pituitary stalk and tuber cinereum. There was interval improvement of the spinal lesions with no convincing residual. Clinically, at almost three years since initial imaging findings, and 25 months since completing treatment, she is stable from an oncology perspective.

BackgroundElectronic patient-reported outcome measures (ePROMs) are standardized digital instruments integrated into clinical care to collect subjective data regarding patients’ health-related quality of life, functional status, and symptoms. In documenting patient-reported progress, ePROMs can guide treatment decisions and encourage measurement-based care practices. Voxe is a pediatric and user-centered ePROM platform for patients with chronic health conditions. ObjectiveWe aimed to describe the user-centered design approach involving feedback from end users and usability testing of Voxe’s platform features to support implementation in a pediatric health care setting. MethodsPurposive sampling was used to recruit patients aged 8-17 years from 2 chronic illness populations in 2 pediatric hospitals in Canada. Patients’ health care team members were also purposively recruited. One-on-one iterative testing sessions were conducted digitally by research team members with participants to obtain feedback on the appearance and functionalities of the Voxe platform prototype. Patients and health care providers (HCPs) completed Voxe-related task-based activities. International Organization for Standardization key performance indicators were tracked during HCP task-based activities. HCPs also completed the System Usability Scale. To test platform usability, the think-aloud technique was used by participants during the completion of structured tasks. After completing all task-based activities, patient participants selected 5 words from the Microsoft Desirability Toolkit to describe their overall impression and experience with the Voxe platform. Qualitative data about likes, dislikes, and ease of use were collected through semistructured interviews. Feedback testing sessions were conducted with patients and HCPs until Voxe was acceptable to participating end users, with no further refinements identified. Quantitative and qualitative data analysis were completed using descriptive statistics and content analysis. ResultsA total of 49 patients and 38 HCPs were recruited. Patients were positive about Voxe’s child-centered design characteristics and notification settings. HCPs rated Voxe as user-friendly and efficient, with the time to complete tasks decreasing over time. HCPs were satisfied with the Voxe platform functionalities and identified the value of Voxe’s system notifications, summarized display of ePROM results, and its capacity to integrate with electronic medical records. Patients’ and HCPs’ high satisfaction rates with the Voxe prototype highlight the importance of being responsive to user suggestions from the inception of eHealth platform developments to ensure their efficient and effective design. ConclusionsThis paper describes the user-centered creation and usability testing of Voxe as an ePROM platform for implementation into clinical care for pediatric patients with chronic health conditions. As a patient-facing platform that can be integrated into electronic medical records, Voxe aligns with measurement-based care practices to foster quality patient-centered approaches to care. End users’ positive feedback and evaluation of the platform’s user-friendliness and efficiency suggest that Voxe represents a valuable and promising solution to systematically integrate patient-related outcome (PRO) data into complex and dynamic clinical health care settings. International Registered Report Identifier (IRRID)RR2-10.1136/bmjopen-2021-053119

Background: As of 2021, more than 6000 children and youth in Canada were living with end-stage kidney disease (ESKD), for which kidney transplantation is considered the preferred treatment by health professionals. Research shows that living donor kidney transplantation (LDKT) has superior allograft and recipient survival compared to deceased donor kidney transplantation (DDKT). However, in a pediatric setting, the choice of LDKT or DDKT is a summative consideration of factors weighed carefully by the patient’s family, health care team, and patient. Decision-making surrounding transplantation may be more complex for racial and ethnic minorities as culturally specific values and beliefs are interwoven within dominant understandings and concepts of health and accepted models of health care. For example, Chinese Canadians have an increased risk of ESKD, yet reduced access to LDKT compared to White patients, despite being the largest visible minority population in Canada. Objective: The objective of this qualitative study is to deepen our understandings of the decision-making process surrounding DDKT versus LDKT among parents of Chinese Canadian pediatric patients with chronic kidney disease (CKD). Design: Qualitative descriptive study design. Setting: The Nephrology Program at The Hospital for Sick Children in Toronto, Canada. Participants: Caregivers of Chinese Canadian patients with CKD, 18 years of age or older, and who spoke English, Cantonese, or Mandarin. Methods: One-on-one, semistructured interviews were conducted virtually, by a member of the research team and were audio-recorded and transcribed verbatim. Thematic analysis was used to explore participants’ shared experience. Results: Seven interviews were conducted with 6 mothers and 1 father of 6 Chinese Canadian pediatric patients with CKD: 4 patients had undergone a kidney transplant, and 2 were not yet listed for transplant. Analysis of data highlighted that cultural influences affected whether parents shared with others about their child’s illness and experience. The cultural understanding that it is inappropriate to burden others contributed to the creation of an isolating experience for participants. Cultural influences also impacted whether parents asked others to be a living donor as participants articulated this would place a physical burden on the living donor (e.g., potential risk to their health) and an emotional burden on the participant as they would be indebted to a willing donor. Ultimately, parents’ decision to choose DDKT or LDKT for their patient-child was a result of evaluating both options carefully and within an understanding that the ideal treatment choice reflected what was best for all family members. Limitations: Findings reflect experiences of a small sample from a single recruitment site which may limit transferability. Conclusions: Parents in this study felt that they had access to the necessary evidence-based information to make an informed decision about the choice of DDKT versus LDKT for their child. Participant narratives described feeling isolated within cultural communities of family and friends and participants’ suggestion of benefiting from increased support may guide future research directions. Practitioners can offer direct and indirect support to families, with recognition of the importance of cultural values and family-centered care on decision-making within families. Opportunities are needed for accessible, virtual social support platforms to increase parental feelings of culturally mediated peer support from parents who share similar experiences.

Neuroimaging has a key role in identifying small-vessel vasculitis from common diseases it mimics, such as multiple sclerosis. Oftentimes, a multitude of these conditions present similarly, and thus diagnosis is difficult. To date, there is no standardized method to differentiate between these diseases. This review identifies and presents existing scoring tools that could serve as a starting point for integrating artificial intelligence/machine learning (AI/ML) into the clinical decision-making process for these rare diseases. A scoping literature review of EMBASE and MEDLINE included 114 articles to evaluate what criteria exist to diagnose small-vessel vasculitis and common mimics. This paper presents the existing criteria of small-vessel vasculitis conditions and mimics them to guide the future integration of AI/ML algorithms to aid in diagnosing these conditions, which present similarly and non-specifically.