教学课程资源库 更多>>

关键词： 科普讲座   慢性肾脏疾病   安徽省医学会   儿科学分会   义诊活动   儿童医院   高死亡率   志愿精神  

摘要： 3月11日是第十六个世界肾脏日。为弘扬奉献、友爱、互助、进步的志愿精神,提高人们对慢性肾脏疾病高发病率和高死亡率的认识,安徽省医学会儿科学分会联合安徽省儿童医院组织开展了"积极面对肾病,共享精彩人生"大型义诊活动。儿科学分会部分专家在主任委员邓芳教授带领下,通过现场坐诊、线上咨询及科普讲座的方式为患儿普及肾病知识,线上线下共义诊患儿近200人次。

关键词： Eye diseases   Risk factors  

摘要： Objective To analyse the epidemiological characteristics and clinical features of children under the age of 16 years with ocular trauma at the Eye and Ear, Nose and Throat Hospital of Fudan University (Shanghai, China) and to investigate the preventive measurements taken to avoid vision-threatening eye trauma. Methods The inpatient medical records of children <16 years old treated for ocular trauma between January 2007 and December 2015 were collected. The age, sex, type of trauma, cause of injury, complications and visual acuity on admission and discharge were analysed statistically. Results A total of 2211 patients (2231 eyes) were enroled. Of these, 73.7% were male, and 61.2% were children aged 0-6 years. Mechanical ocular trauma was present in 75.3% of eyes, and penetrating injuries in 59.8%. The top three offending objects were scissors (16.3%), firecrackers (8%) and pencils (4.9%). Iris prolapse (odds ratio [OR] = 2.27), retinal detachment (OR = 2.91), endophthalmitis (OR = 2.25) or an intraocular foreign body (OR = 2.80) was associated with the same or worse visual prognosis among all the subjects. Traumatic cataract (OR = 0.37) was associated with final visual improvement. Conclusions An efficient strategy for preventing eye injury should focus on male children during preschool years. Our root cause analysis showed specific environmental patterns of vision-devastating objects. Specific preventive measures are proposed to reduce the incidence of paediatric eye injuries.

关键词： Child   infant   adolescent   paediatric   advance care planning   palliative care   terminal care   decision making  

摘要： Background: Advance care planning for children with palliative care needs is an emotionally, legally and complex aspect of care, advocated as beneficial to children, families and health professionals. Evidence suggests healthcare professionals often avoid or delay initiation. An overview of evidence on the factors that influence and impact on the health care professional's initiation of paediatric advance care planning process is lacking. Aim: To review and synthesise evidence on the factors associated with health care professional's decision to initiate paediatric advance care planning. Design: Systematic integrative review using constant comparison method. Data Sources: Electronic databases (CINAHL, PubMed, PsycINFO, Ovid MEDLINE, EMBASE, Web of Science and Cochrane) using MeSH terms and word searches in Oct 2019. No limit set on year of publication or country. Grey literature searches were also completed. Results: The search yielded 4153 citations from which 90 full text articles were reviewed. Twenty-one met inclusion criteria consisting of quantitative (n = 8), qualitative (n = 6) and theoretical (n = 7) studies. Findings revealed overarching and interrelated themes 'The timing of initiation', 'What makes an initiator, 'Professionals' perceptions' and 'Prerequisites to initiation'. Conclusions: This review provides insights into the complexities and factors surrounding the initiation of advance care planning in paediatric practice. Uncertainty regarding prognosis, responsibility and unpredictable parental reactions result in inconsistent practice. Future research is required to inform intervention to assist health care professionals when initiating paediatric advance care planning conversations.

关键词： Radiotherapy   Rhabdomyosarcoma   Soft-tissue sarcoma   Ewing sarcoma   Neuroblastoma   Renal tumours   Stage IV   SIOPE   Paediatrics   Metastases  

摘要： Purpose/objective: About 20% of children with solid tumours (ST) present with distant metastases (DM). Evidence regarding the use of radical radiotherapy of these DM is sparse and open for personal interpretation. The aim of this survey was to review European protocols and to map current practice regarding the irradiation of DM across SIOPE-affiliated countries. Materials/methods: Radiotherapy guidelines for metastatic sites (bone, brain, distant lymph nodes, lung and liver) in eight European protocols for rhabdomyosarcoma, non-rhabdomyosarcoma soft-tissue sarcoma, Ewing sarcoma, neuroblastoma and renal tumours were reviewed. SIOPE centres irradiating >= 50 children annually were invited to participate in an online survey. Results: Radiotherapy to at least one metastatic site was recommended in all protocols, except for high-risk neuroblastoma. Per protocol, dose prescription varied per site, and information on delineation and treatment planning/delivery was generally missing. Between July and September 2019, 20/27 centres completed the survey. Around 14% of patients were deemed to have DM from ST at diagnosis, of which half were treated with curative intent. A clear cut-off for a maximum number of DM was not used in half of the centres. Regardless of the tumour type and site, conventional radiotherapy regimens were most commonly used to treat DM. When stereotactic radiotherapy was used, a wide range of fractionation regimens were applied. Conclusion: Current radiotherapy guidelines for DM do not allow a consistent approach in a multicentre setting. Prospective (randomised) trials are needed to define the role of radical irradiation of DM from paediatric ST. (C) 2020 The Author(s). Published by Elsevier Ltd.

ISBN: (纸本)9787572506079

关键词： Nonepileptic seizures   Breath-holding spells   Seizure   Video-EEG monitoring   Children  

摘要： Purpose: The misdiagnosis of non-epileptic seizures (NES) as epilepsy is one of the most common pitfalls in neuropsychiatric practice. This study aimed to describe the percentage and types of NES among children who were referred for a diagnosis of epilepsy in Upper Egypt. Methods: We recruited a total of 876 patients who were referred to Sohag University Hospital, a tertiary referral centre in Upper Egypt, for the evaluation of suspected epilepsy. Relevant methods for the diagnosis of epilepsy, including medical history and examination, EEG, video-EEG, laboratory investigations, and brain imaging, were performed for all study participants. Results: Among the 876 patients who were referred for the diagnosis of suspected epilepsy during the period from June 2017 to October 2018, 171 patients (19.5 %) were diagnosed as having NES. In general, we found that NES in the paediatric age groups did not differ from that reported in various studies across several different populations. The most prevalent NES in our study was breath-holding spells (32.2 %), followed by syncope (17.5 %), psychogenic nonepileptic seizures (12.3 %), motor tics (9.9 %), and benign sleep myoclonus (7.6 %). Other less frequent NES included infantile masturbation (7 %), spasmus nutans (5.3 %), migraine (2.9 %), benign paroxysmal torticollis (2.9 %), night terrors (1.8 %), and shuddering attacks (0.6 %). Conclusion: Ideally, neurologists should not misdiagnose NES as epilepsy, and whenever the diagnosis of NES is uncertain, an accurate diagnosis should be made using long-term video-EEG monitoring, especially in younger paediatric patients.

关键词： pathology   pediatric   rhabdomyosarcoma  

摘要： The diagnosis and classification of rhabdomyosarcoma (RMS) has undergone several shifts over the last 30 years. While the main diagnostic categories remained the same, changes in the histologic criteria necessary for diagnosis, as well as varied reliance on immunohistochemical and molecular data over time, have created confusion, particularly regarding how these shifts impacted risk stratification and enrollment onto clinical trials. The goal of this report is to review the evolution and current status of RMS diagnosis, focusing on diagnostic criteria in the Children's Oncology Group (COG), the European Paediatric Soft Tissue Sarcoma Group (EpSSG), and the Cooperative Weichteilsarkom Studiengruppe (CWS). In addition, we emphasize research tools used to classify RMS and address biological questions within current clinical trials run by each group. The INternational Soft Tissue SaRcoma ConsorTium (INSTRuCT) initiative will maximize potential to optimize risk stratification by recognizing and accounting for differences in historical data and current practices.

关键词： affordance   built environment   case study   design research   vulnerability  

摘要： Contemporary understandings of vulnerability highlight its critical, relational and enabling aspects. Through leveraging these understandings, this article contributes to conceptualizing the notion of everyday design by interweaving it with that of vulnerability. A case study brings vulnerability into view by zooming out from and in on everyday practices around an aquarium in a paediatric oncology ward. Subsequently, we unravel the notion of vulnerability as used in design research and in anthropology and philosophy: while vulnerability grounds everyday design philosophically and ethically as a form of care, everyday design challenges the tendency to situate vulnerability in (relations between) human beings. Our article thus exemplifies design anthropology understood as bringing in dialogue theory and methods from both anthropology and design. ? 2021 Elsevier Ltd. All rights reserved.

关键词： Crohn's disease   exome sequencing   genetics   primary immunodeficiency   ulcerative colitis   very early-onset inflammatory bowel disease  

摘要： Background: It is important to identify patients with monogenic IBD as management may differ from classical IBD. In this position statement we formulate recommendations for the use of genomics in evaluating potential monogenic causes of IBD across age groups. Methods: The consensus included paediatric IBD specialists from the Paediatric IBD Porto group of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and specialists from several monogenic IBD research consortia. We defined key topics and performed a systematic literature review to cover indications, technologies (targeted panel, exome and genome sequencing), gene panel setup, cost-effectiveness of genetic screening, and requirements for the clinical care setting. We developed recommendations that were voted upon by all authors and Porto group members (32 voting specialists). Results: We recommend next-generation DNA-sequencing technologies to diagnose monogenic causes of IBD in routine clinical practice embedded in a setting of multidisciplinary patient care. Routine genetic screening is not recommended for all IBD patients. Genetic testing should be considered depending on age of IBD-onset (infantile IBD, very early-onset IBD, paediatric or young adult IBD), and further criteria, such as family history, relevant comorbidities, and extraintestinal manifestations. Genetic testing is also recommended in advance of hematopoietic stem cell transplantation. We developed a diagnostic algorithm that includes a gene panel of 75 monogenic IBD genes. Considerations are provided also for low resource countries. Conclusions: Genomic technologies should be considered an integral part of patient care to investigate patients at risk for monogenic forms of IBD.