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关键词： congenital heart defect   elastin   electrospinning   fibrinogen   nanofibre   vascular graft  

摘要： The development of tissue-engineered vascular grafts (TEVGs) for paediatric applications must consider unique factors associated with this patient cohort. Although the increased elastogenic potential of neonatal cells offers an opportunity to overcome the long-standing challenge of in vitro elastogenesis, neonatal patients have a lower tolerance for autologous tissue harvest and require grafts that exhibit growth potential. The purpose of this study was to apply a multipronged strategy to promote elastogenesis in conjunction with umbilical cord-derived materials in the production of a functional paediatric TEVG. An initial proof-of-concept study was performed to extract fibrinogen from human umbilical cord blood samples and, through electrospinning, to produce a nanofibrous fibrinogen scaffold. This scaffold was seeded with human umbilical artery-derived smooth muscle cells (hUASMCs), and neotissue formation within the scaffold was examined using immunofluorescence microscopy. Subsequently, a polycaprolactone-reinforced porcine blood-derived fibrinogen scaffold (isolated using the same protocol as cord blood fibrinogen) was used to develop a rolled-sheet graft that employed topographical and biochemical guidance cues to promote elastogenesis and cellular orientation. This approach resulted in a TEVG with robust mechanical properties and biomimetic arrangement of extracellular matrix (ECM) with rich expression of elastic fibre-related proteins. The results of this study hold promise for further development of paediatric TEVGs and the exploration of the effects of scaffold microstructure and nanostructure on vascular cell function and ECM production.

关键词： child   EZ-IO   intraosseous line   paediatric emergency department  

摘要： Aim Intraosseous access is an alternative to conventional intravenous access. A number of studies have demonstrated the utility and safety of EZ-IO, a recently introduced semi-automatic intraosseous device. However, few studies have investigated the use of EZ-IO in the paediatric emergency setting. The aim of this study was to describe the epidemiological characteristics EZ-IO use including complications among paediatric patients in a paediatric emergency department. Methods We conducted a retrospective descriptive study enrolling children younger than 16 years who visited our emergency department between January 2013 and August 2018. Data on age, gender, diagnosis, insertion success rate and complications were collected and analysed. Results Seventy-two patients were enrolled;of these 38 (52.8%) were male. The median age was 9 months (interquartile range: 3-34.5 months). Of the total, 22 (30.6%) received more than one IO insertion. Ninety-seven consecutive IO insertions were identified. The rate of successful insertions was achieved at 92.7% (90 insertions). There were 21 complications (21.6%), including 17 extravasations (17.5%) of fluid and four dermal abrasions (4.1%). We found no cases of complications that may have compromised patient safety. Conclusions The success rate of the EZ-IO insertion was high in paediatric patients. However, we found a higher incidence of dermal abrasions, which might be a complication specific to children.

关键词： Children with ASD   knowledge about ASD   paediatric nurses  

摘要： Hospitalization of children with autism spectrum disorders (ASDs) is a challenge for paediatric nurses. The literature reveals a lack of knowledge among the clinical and behavioural characteristics of autism. The aim of the study is to investigate the knowledge of paediatric nurses about ASDs and their experience with children with ASD. Ninety-three nurses completed Knowledge About Childhood Autism Among Healthcare Workers questionnaire, a sociodemographic questionnaire and an open-ended questionnaire to evaluate the experience of nurses. Findings showed that nurses have a basic knowledge of ASD, except for comorbidity and onset of the ASD, in which they obtained lower scores. Older nurses and those who had more experience in a paediatric ward or with children with ASD showed higher scores. Analysis of the content of open-ended questionnaire showed that nurses have difficulties in managing the relationship with children with ASD, and that they wonder about how to improve it, seeking useful strategies for this purpose. During interactions, nurses experience mixed feelings such as sadness, suffering, uneasiness, inadequacy, displeasure, embarrassment and tenderness. They declare the need for more knowledge about ASD. They would like to improve their own capacity for interactions with these children, also through training.

关键词： avian influenza   fatal case   human infection   reassortment  

摘要： Avian influenza A(H5N6) keeps evolving, causing outbreaks in birds and sporadic infections in human. Here, we report a fatal paediatric infection caused by a novel reassortant H5N6 virus. The patient was an obese 9-year-old girl. She initiated with fever and cough, then developed pneumonia, acute respiratory distress syndrome (ARDS) and respiratory failure. Lower respiratory tract aspirates and anal swabs were serially taken till the patient's death. Viral isolation, genome sequencing and phylogenetic analysis were conducted. A novel reassortant H5N6 virus was isolated from the patient. Except the PA gene, all other 7 genes of the virus belonged to H5N6 genotype A (S4-like virus). The PA gene was probably obtained from Eurasian waterfowl influenza viruses. The H5N6 virus was consistently detected from the patient's respiratory samples till the 17th day after symptom onset, but not from anal swabs or urine sample by real-time reverse transcription polymerase chain reaction (RT-PCR). Significantly elevated (32-fold) serum antibodies to H5N6 virus were observed during the patient's course of disease. Aside from the identified novel reassortant H5N6 viral strain, obesity, delayed confirmation of aetiology and specific antiviral treatment, and prolonged virus shedding could have contributed to the poor clinical outcome.

关键词： Electronic medical record   Action plan   Asthma   Eczema   Allergy   Anaphylaxis   Healthcare quality  

摘要： Background: Individualised Action Plans (APs) are a key management tool for paediatric asthma, allergy, anaphylaxis and eczema. They provide salient care instructions for patients and caregivers and are thought to improve disease outcomes, albeit with minimal supporting evidence. Whilst the provision of an AP has become a widely measured healthcare quality marker, the content of the plans provided has been relatively neglected. The Royal Children's Hospital, Melbourne (RCH) implemented an AP generation tool integrated within its Electronic Medical Record (EMR) in 2017. This case study aims to exhibit the potential benefits of a hospital-wide integrated AP tool, assess its uptake and usage at our institution and demonstrate quality and user interface issues detected through audit of APs provided. Methods: A retrospective observational analysis of all APs created within the RCH EMR for patients aged 0-18 years between January 1 and December 31, 2018 was conducted. For each AP, automatically populated and clinician entered fields were extracted and analysed. Results: 2637 APs were completed during the study period. The most used AP was for asthma. Omission rates of critical information were low but could be improved. Depending on clinical condition, there was variation in both the location of completion (ED vs clinic/hospital visit) and role of clinician completing the AP (consultant vs junior medical staff). Conclusion: There was considerable uptake of an EMR-integrated AP tool across our institution. An electronic AP allowed for qualitative evaluation of usage and audit of AP content and will guide further system and user interface improvements to improve AP quality.

关键词： Genetic kidney disease   Children   Precision medicine   Kidney organoids   Pharmacogenomics   Genes  

摘要： Purpose of ReviewThis review aims to outline the current state of precision medicine in paediatric nephrology with a focus on how genomics will impact on patient care in the *** FindingsBroader availability of genomic sequencing, direct-to-consumer genetic testing, a growing list of genes implicated in childhood-onset kidney disease, patient-specific kidney organoid models of disease and pharmacogenomics will all shape the future for patients with kidney *** is a bright future for patients with kidney disease as greater understanding of the pathogenetic mechanisms of disease will result in earlier accurate diagnoses and new therapeutic options. Paediatric nephrology is poised to become a less acute specialty with a stronger focus on preventative medicine and counselling.

关键词： Graft-versus-host disease   Paediatrics  

关键词： paediatric   proton therapy   brain tumour   IMPT   IMRT   target size and location   paediatric age   normal tissue sparing   patient-specific  

摘要： Background: Proton radiotherapy produces superior dose distributions compared to photon radiotherapy, reducing side effects. Differences between the two modalities are not fully quantified in paediatric patients for various intracranial tumour sites or age. Understanding these differences may help clinicians estimate the benefit and improve referral across available centres. Our aim was to compare intensity-modulated proton therapy (IMPT) and intensity-modulated photon radiotherapy (IMRT) radiation doses for select paediatric intracranial tumours. Methods: IMPT and IMRT dose distributions for gender-matched paediatric cranial CT-datasets (ages 5, 9 and 13 years) were retrospectively calculated to simulate irradiation of supratentorial (ependymoma) and infratentorial (medulloblastoma) target volumes diameters (1-3 cm) and position (central and 1-2 cm shifts). Results: Clinical dosimetric objectives were achieved for all 216 treatment plans. Whilst infratentorial IMPT plans achieved greater maximum dose sparing to optic structures (4.8-12.6 Gy optic chiasm), brainstem sparing was limited (similar to 0.5 Gy). Mean dose difference for optic chiasm was associated with medulloblastoma target position (p< 0.0197). Supratentorial IMPT plans demonstrated greater dose reduction for the youngest patients (pituitary glandp< 0.001). Conclusions: Normal tissue sparing was achieved regardless of patient age for infratentorial tumours. However, for supratentorial tumours, there was a dosimetric advantage of IMPT across 9 vs. 13-year-old patients.

关键词： adolescents   children   connective tissue disorder   Marfan syndrome   scoping review  

摘要： Aim The present study aimed to map and summarise the research on children, aged 0-18 years, with Marfan syndrome, identify research gaps and point to research agendas. Methods A scoping review was systematically performed by searching multiple databases from January 1996 to April 2019. Primary studies presenting results on at least six individuals aged 0-18 years with Marfan syndrome, diagnosed according to the Ghent nosology, were selected. Results From 2341 de-duplicated records, 92 papers were included, mapped and described. Their topics were diagnostics (12%), cardiovascular matters (50%), skeletal matters (22%), ocular matters (9%), other medical aspects (5%) and psychosocial perspectives (2%). Most studies were from Europe and North America and published between 1999 and 2019 in subject-specific or paediatric journals, while a few were published in genetics journals. All studies had quantitative designs, and very few were multicentre studies. Each study had six to 608 subjects for a total of approximately 5809. Conclusion A wide range of research topics on adolescent and paediatric Marfan syndrome was found, but qualitative studies and a focus on psychosocial matters were lacking. Future investigations addressing noncardiovascular consequences and patient experiences are needed, as well as studies reaffirming or replicating existing intervention study results.

关键词： depression   occupational burnout   paediatric dentists   United States  

摘要： Background Paediatric dentists in the United States may be at greater risk for occupational burnout and/or depression because of chronic stress associated with provision of paediatric dental care and increasing prevalence of females in the workforce. Aims To determine the prevalence of occupational burnout and/or depression among US paediatric dentists. Design A self-administered online anonymous survey was sent to members of the American Academy of Pediatric Dentistry (n = 4735). The questionnaire consisted of seven demographic items, 22 items of Maslach Burnout Inventory (Three subscales: Emotional exhaustion, Depersonalization, and Personal accomplishment), and eight items of Patient Health Questionnaire-8. Results The survey had a response rate of 11.4% (females = 53%). Twenty-three per cent of respondents had high emotional exhaustion while fewer respondents had high depersonalization (12%) or low personal accomplishment (10%). Nine per cent fulfilled the study's definition of occupational burnout (high emotional exhaustion + high depersonalization). Seven per cent of respondents had moderate-to-severe depression and showed significant correlations (P < .05) with high emotional exhaustion, high depersonalization and low personal accomplishment. Two out of five respondents with occupational burnout also had moderate-to-severe depression. There were no gender differences in prevalence of burnout or depression. Conclusions Few paediatric dentists had occupational burnout and/or depression.