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关键词： Status epilepticus   Super-refractory status epilepticus   Children   Prognosis  

摘要： Purpose: To describe the aetiology, clinical features, and short-term outcomes of children with status epilepticus (SE), in particular super-refractory SE (SRSE), by a nationwide multicentre study in China. Methods: In this retrospective study, inpatient children with SE were identified from neurology departments and paediatric intensive care units from 44 hospitals in 27 provinces of China between 2013 and 2015. Clinical data were exported from the Hospital Information System. Results: Clinical records from children with SE (n = 4255) aged 1 month to 18 years were enrolled;13.1 % were diagnosed with SRSE. The most common known SE aetiology was acute symptomatic aetiology (42.8 %) and 50.2 % of SE was caused by epilepsy of unknown aetiology. Acute central nervous system (CNS) infections (38.8 %) were associated with SRSE (P < 0.001). The overall SE in-hospital mortality rate was 3.0 %, which was significantly higher in the children with SRSE than in those with non-SRSE (15.2 % versus 1.4 %, respectively;P < 0.001). Fourteen percent of the children with SE had various levels of neurological dysfunction at discharge. SRSE was a risk factor for in-hospital mortality (hazard ratio = 4.14;95 % confidence interval [CI]: 2.34-7.32;P < 0.001) and neurological dysfunction at discharge (odds ratio = 2.85;95 % CI: 1.90-4.27;P < 0.001). Conclusion: Acute symptomatic aetiology was the most common known cause of paediatric SE. Aetiology was associated with progression to SRSE and short-term neurological dysfunction at discharge. Furthermore, SRSE was considered a risk factor for in-hospital mortality and short-term neurological dysfunction.

摘要： Background Plaque psoriasis affects children and adults, but treatment options for paediatric psoriasis are limited. Objectives To evaluate the efficacy and safety of ixekizumab (IXE), a high-affinity monoclonal antibody that selectively targets interleukin-17A, for moderate-to-severe paediatric psoriasis. Methods In a randomized, double-blind, placebo-controlled, phaseIIIstudy (IXORA-PEDS), patients aged 6 to < 18 years with moderate-to-severe plaque psoriasis were randomized 2 : 1 to weight-based dosing ofIXEevery 4 weeks (IXEQ4W,n= 115) or placebo (n= 56) through week 12, followed by open-labelIXEQ4W. Coprimary endpoints were the proportions of patients at week 12 achieving >= 75% improvement in Psoriasis Area and Severity Index (PASI75) and those achieving a static Physician's Global Assessment score of 0 or 1 (sPGA0,1). Results IXEwas superior (P< 0 center dot 001) to placebo for both coprimary endpoints ofPASI75 (IXEQ4W, 89%;placebo, 25%) andsPGA(0,1) (IXEQ4W, 81%;placebo, 11%).IXEwas also superior for all gated secondary endpoints, includingPASI75 andsPGA(0,1) at week 4, improvement in itch, and complete skin ***4W provided significant (P< 0 center dot 001) improvements vs. placebo in quality of life and clearance of scalp and genital psoriasis. Responses at week 12 were sustained or further improved through week 48. Through week 12, 45% (placebo) and 56% (IXE) of patients reported treatment-emergent adverse events. One serious adverse event was reported (IXE), one patient discontinued due to an adverse event (placebo) and no deaths were reported. Conclusions IXEwas superior to placebo in the treatment of moderate-to-severe paediatric psoriasis, and the safety profile was generally consistent with that observed in adults. What is already known about this topic? Paediatric psoriasis affects approximately 1% of children and can negatively impact health-related quality of life. Treatment options for paediatric psoriasis are typically limited to off-la

关键词： COVID-19   Pandemic   SARS-CoV-2   Training   PSS (Perceived stress scale)   Telemedicine  

摘要： Introduction The COVID-19 pandemic has led to an unprecedented need to re-organise and re-align priorities for all surgical specialties. Despite the current declining numbers globally, the direct effects of the pandemic on institutional practices and on personal stress and coping mechanisms remains unknown. The aims of this study were to assess the effect of the pandemic on daily scheduling and work balances, its effects on stress, and to determine compliance with guidelines and to assess whether quarantining has led to other areas of increased productivity. Methods A trans-Atlantic convenience sample of paediatric urologists was created in which panellists (Zoom) discussed the direct effects of the COVID-19 pandemic on individual units, as well as creating a questionnaire using a mini-Delphi method to provide current semi-quantitative data regarding practice, and adherence levels to recently published risk stratification guidelines. They also filled out a Perceived Stress Scale (PSS) questionnaire to assess contemporary pandemic stress levels. Results There was an 86% response rate from paediatric urologists. The majority of respondents reported near complete disruption to planned operations (70%), and trainee education (70%). They were also worried about the effects of altered home-lives on productivity (<= 90%), as well as a lack of personal protective equipment (57%). The baseline stress rate was measured at a very high level (PSS) during the pandemic. Adherence to recent operative guidelines for urgent cases was 100%. Conclusion This study represents a panel discussion of a number of practical implications for paediatric urologists, and is one of the few papers to assess more pragmatic effects and combines opinions from both sides of the Atlantic. The impact of the pandemic has been very significant for paediatric urologists and includes a decrease in the number of patients seen and operated on, decreased salary, increased self-reported stress levels, substanti

关键词： Cone beam computed tomography   Radiography   dental   Paediatric dentistry   Diagnosis  

摘要： Purpose To determine in which clinical situations it is indicated or contra-indicated to prescribe cone beam computed tomography (CBCT) for paediatric patients. Methods Systematic review of in vivo paediatric research studies of diagnostic efficacy using CBCT, with supplementary searches for guideline documents on CBCT and for systematic reviews permitting inclusion of ex vivo and adult studies. Results After screening, 190 publications were included, mostly case studies. No systematic reviews were found of in vivo paediatric research. Fourteen studies of diagnostic efficacy were identified. The supplementary searches found 18 guideline documents relevant to the review and 26 systematic reviews. The diagnostic efficacy evidence on CBCT was diverse and often of limited quality. There was ex vivo evidence for diagnostic accuracy being greater using CBCT than radiographs for root fractures. The multiplanar capabilities of CBCT are advantageous when localising dental structures for surgical planning. Patient movement during scanning is more common in children which could reduce diagnostic efficacy. Conclusions No strong recommendations on CBCT are possible, except that it should not be used as a primary diagnostic tool for caries. Guidelines on use of CBCT in the paediatric age group should be developed cautiously, taking into account the greater radiation risk and the higher economic costs compared with radiography. CBCT should only be used when adequate conventional radiographic examination has not answered the question for which imaging was required. Clinical research in paediatric patients is required at the higher levels of diagnostic efficacy of CBCT.

关键词： Cardiac computed tomography scan   Congenital heart disease   Radiation dose   Acquisition protocols  

摘要： This paper aims to provide a paediatric cardiac computed tomography angiography expert panel consensus based on the opinions of experts from the Societe francaise d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV) and the Filiale de cardiologie pediatrique congenitale (FCPC). This expert panel consensus includes recommendations for indications, patient preparation, computed tomography angiography radiation dose reduction techniques and postprocessing techniques. We think that to realize its full potential and to avoid pitfalls, cardiac computed tomography angiography in children with congenital heart disease requires training and experience. Moreover, paediatric cardiac computed tomography angiography protocols should be standardized to acquire optimal images in this population with the lowest radiation dose possible, to prevent unnecessary radiation exposure. We also provide a suggested structured report and a list of acquisition protocols and technical parameters in relation to specific vendors. (C) 2020 Elsevier Masson SAS. All rights reserved.

关键词： Albumin-bound paclitaxel   Ewing sarcoma   Neuroblastoma   Paediatric   Rhabdomyosarcoma   Solid tumour  

摘要： Background: The phase I component of a phase I/II study defined the recommended phase II dose and established the tolerability of nab-paclitaxel monotherapy in paediatric patients with recurrent or refractory solid tumours. The activity and safety of nabpaclitaxel monotherapy was further investigated in this phase II study. Patients and methods: Paediatric patients with recurrent or refractory Ewing sarcoma, neuroblastoma or rhabdomyosarcoma received 240 mg/m(2) of nab-paclitaxel on days 1, 8 and 15 of each 28-day cycle. The primary end-point was the overall response rate (ORR;complete response [CR] + partial response [PR]). Secondary end-points included duration of response, disease control rate (DCR;CR + PR + stable disease [SD]), progression-free survival, 1-year overall survival, safety and pharmacokinetics. Results: Forty-two patients were enrolled, 14 each with Ewing sarcoma, neuroblastoma and rhabdomyosarcoma. The ORRs were 0%, 0% and 7.1% (1 confirmed PR), respectively. The DCRs were 30.8% (4 SD), 7.1% (1 SD) and 7.1% (1 confirmed PR and 0 SD) in the Ewing sarcoma, neuroblastoma and rhabdomyosarcoma groups, respectively. The median progression-free survival was 13.0, 7.4 and 5.1 weeks, respectively, and the 1-year overall survival rates were 48%, 25% and 15%, respectively. The most common grade III/4IVadverse events were haematologic (neutropenia [50%] and anaemia [48%]), and grade III/IV peripheral neuropathy occurred in 2 patients (14%) in the rhabdomyosarcoma group. Pharmacokinetics analyses revealed that paclitaxel tissue distribution was both rapid and extensive. Conclusions: In this phase II study, limited activity was observed;however, the safety of nabpaclitaxel in paediatric patients was confirmed. Published by Elsevier Ltd.

关键词： HLA typing   endomysial antibody   Coeliac disease  

摘要： Coeliac disease (CD) diagnosis is based on clinical assessment, detection of specific autoantibodies and histological examination of small intestinal biopsies. The European Society of Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) guidelines have recently been updated and recommend CD may be diagnosed without a biopsy or HLA typing in symptomatic patients with high titre IgA tissue transglutaminase antibodies (aTTG) and positive endomysial antibodies (EMA). However, the need for EMA in patients with high level aTTG has been questioned. We aimed to determine the diagnostic benefit of HLA typing, EMA and IgG antibodies to deamidated gliadin (DGP) in children with high level aTTG. We prospectively evaluated children presenting for assessment of possible CD. All patients underwent small bowel biopsy, serological testing and HLA typing. Results were analysed and correlated with histopathological diagnosis. A total of 209 children were assessed;61.5% were found to have CD and 29% could have avoided biopsy as per 2020 ESPGHAN guidelines. Titres of aTTG >= 60 U/mL or DGP >= 28 U/mL gave 100% specificity and 100% positive predictive value (PPV) for CD. HLA typing and EMA did not improve the PPV of patients with aTTG >= 60 U/mL, but addition of DGP >= 28 U/mL improved diagnostic sensitivity whilst retaining 100% specificity. Addition of HLA and EMA testing in patients with high titre aTTG antibodies does not improve diagnostic performance and may possibly be omitted from the serological workup in these patients. Our data support combining aTTG and DGP testing and optimising cut-offs to maximise specificity as an alternative biopsy-free diagnostic approach.

关键词： COVID-19   paediatric services   survey  

摘要： Aims COVID-19 is now a global pandemic. At the time of survey, fewer than 150 children in Australia and New Zealand had documented infection. The aim of this study was to assess attitudes, readiness and confidence in the early stages of the COVID-19 pandemic through an online survey of paediatric physicians and sub-specialists across Australia and New Zealand. Methods Multiple email list groups were used to contact paediatric physicians to undertake an online Likert scale survey between 17 and 24 March. Respondents' specialty, experience and work setting were recorded. Ordinal logistic regression was used to determine respondent factors. Results There were 542 respondents from across Australia and New Zealand: an estimated 11% of the paediatric physician workforce. A minority (36.6%) agreed that their national response had been well coordinated;the majority (92.7%) agreed that senior-level hospital administrators were taking the situation seriously. Most reported a good understanding of the natural history of COVID-19 in children, and knowledge of where to find local information. A large proportion of physicians (86.1%) were worried about becoming infected through their work;few (5.8%) reported that they would not come to work to avoid infection. Closure of school and childcares would reduce the ability to continue work at current capacity for 23.6% of respondents. Conclusion Despite limited experience in pandemics, most paediatric physicians felt informed. Concern about exposure at work is common;most were willing to work regardless. The closure of schools and daycares may have an impact on staffing. Coordination and leadership will be critical.

关键词： brachial plexus block   fasting   paediatric anaesthesia   regional anaesthesia   sedation   ultrasound-guided regional anaesthesia  

摘要： Morphoea, also known as localized scleroderma, is a debilitating fibrosing disorder of uncertain aetiology, affecting the skin and subcutaneous tissues. Paediatric-onset disease is not uncommon and is associated with frequent relapses. The disease has complex pathogenetic mechanisms and multiple clinical subtypes, and affects children of all ages. Recent research has focused on elucidating the disease pathophysiology and identifying measures of disease activity. We performed a literature search on PubMed, MEDLINE and Google Scholar, using keywords such as 'pediatric morphea', 'juvenile localised scleroderma' and 'juvenile systemic sclerosis'. Relevant studies, including randomized trials, reviews of standard current guidelines and original research articles, were selected, and results were analysed before being summarized. In the first of this two-part review, we provide a bird's-eye view of the current literature concerning the epidemiology, aetiopathogenesis and clinical classification of paediatric morphoea;in Part 2, we review the diagnosis, markers of disease activity, management and natural history.