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关键词： Obsessive-compulsive disorder   Emotion regulation   Exposure and response prevention  

摘要： This study explored whether variability in emotion regulation was associated with several clinical correlates of OCD and an attenuated response to treatment. Participants in this study were 137 youth (and their parents) aged 7-17 years with a primary diagnosis of OCD. Parents completed study questionnaires and children received intensive CBT with exposure and response prevention treatment. A median split of responses to the baseline Emotional Control (EC) index of the BRIEF, resulted in two groups of children - those with relatively greater and poorer EC. The results indicated that children in the relatively poorer EC group had significantly greater OCD severity, more family accommodation, internalising and externalising symptoms. They were also more likely to have a comorbid diagnosis of oppositional defiant disorder or social phobia. Additionally, children with relatively greater EC were more likely to have attained response or remission of their symptoms immediately following treatment. Similarly, at three months following treatment there were fewer responders to treatment among the lower EC group relative to the higher EC group;however, there was no significant difference for treatment remission between groups. Therefore, EC may be an indicator for a more severe presentation of OCD and a poorer response to treatment.

关键词： Paediatric NMDAR-Ab encephalitis   Autoimmune encephalitis   ADEM   MOG antibodies   Neurodevelopmental outcomes   Cognition/behaviour  

摘要： Recognition of paediatric autoimmune/immune-mediated encephalitis and epileptic encephalopathy (e.g. NMDAR-Ab encephalitis) has rapidly increased over the last ten years. While we are succeeding in the diagnosis and identification and even early treatment of these encephalitidies, with studies describing >80% are making a "good" recovery, we are now recognising that a "good" medical outcome does not cover the cognitive, social and behavioural sequelae that can occur, particularly in paediatric patients. Basic measures of medical outcome, for example the modified Rankin Scale (MRS) or the Paediatric Cerebral Performance Category (PCPC), offer the advantage of being quick to use, but do not reveal the more complex difficulties that can impact the future of affected children. This article reviews the current literature on neurodevelopmental outcomes in children affected with autoimmune and immune-mediated encephalitis/epileptic encephalopathy and provides guidance on post-onset surveillance aimed at identifying those most likely to experience ongoing long-term difficulties. (C) 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

摘要： Dear Editor, Lichen sclerosus (LS) represents a chronic autoinflammatory disease of unknown aetiology, and is frequently encountered in paediatric dermatology centres. The 4516 identified cases of childhood LS consisted of 2804 male and 1707 female patients, and five patients without gender identification. Of 1707 female patients with LS, 94-6% (1615/1707) presented with genital lesions only, 2-8% (47/1707) had extragenital disease only and 2-9% (49/1707) had both genital and extragenital lesions. [Extracted from the article]

摘要： The authors emphasized the inefficiency of whole spine (WS) magnetic resonance imaging (MRI) in evaluation of lumbosacral lipomatous malformation (LSL) comparing with the lumbosacral spine (LS) MRI. 1 Extended lumbosacral spine MRI findings in patients with a an upwardly extended syrinx (up to T5), b an upper separate syrinx (C3-T5), and c an extensive cervicothoracic syrinx at the time of re-tethering (C2-T11). [Extracted from the article]

关键词： Cardiac surgery   CHD   surgery complications   risk analysis  

摘要： Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom. We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians. Our tool enables automatic creation of graphical summaries, displayed as a Microsoft Power Point presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window. Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.

关键词： 中医儿科学   中医学院   教材  

ISBN: (纸本)9787547846704

摘要： 本教材分总论、各论两部分，共10章。总论有2章，内容包括儿科学基础、儿科保健。各论有8章，介绍了儿科病症的诊治，病种涉及肺系疾病、脾系疾病、心肝疾病、肾系疾病、造血系统疾病、感染病、寄生虫病、新生儿疾病和其他疾病，共50个。本教材在历版教材的基础上，根据时代的发展，注重中医学传统理论，并在现代进展方面有所创新。编写中继承上一版教材的特色，发扬传统医学在诊治儿童疾病中的优势，力求突出继承、实用与优选、创新并重，致力于编写出有特色的精品教材。根据时代及学科发展适当增减病种，选择临床有代表性的典型病种。如肺系疾病中增加“鼻鼽”，脾系疾病中增加“便秘”，心肝系疾病中“小儿乙型脑炎”更换为“病毒性脑炎”；新生儿疾病中剔除了现在临床不常见的“硬肿病”和“脐部疾病”。同时，根据课程目标，编制了以扫描二维码作为本课程学习的辅助模式，包含知识链接、教学视频、学习课件、课后习题和沟通交流平台等板块，这是出版融等……

摘要： Aims To investigate the accuracy of phenotypic early-onset ataxia (EOA) recognition among developmental conditions, including developmental coordination disorder (DCD) and hypotonia of central nervous system origin, and the effect of scientifically validated EOA features on changing phenotypic consensus. Method We included 32 children (4-17y) diagnosed with EOA (n=11), DCD (n=10), and central hypotonia (n=11). Three paediatric neurologists independently assessed videotaped motor behaviour phenotypically and quantitatively (using the Scale for Assessment and Rating of Ataxia [SARA]). We determined: (1) phenotypic interobserver agreement and phenotypic homogeneity (percentage of phenotypes with full consensus by all three observers according to the underlying diagnosis);(2) SARA (sub)score profiles;and (3) the effect of three scientifically validated EOA features on phenotypic consensus. Results Phenotypic homogeneity occurred in 8 out of 11, 2 out of 10, and 1 out of 11 patients with EOA, DCD, and central hypotonia respectively. Homogeneous phenotypic discrimination of EOA from DCD and central hypotonia occurred in 16 out of 21 and 22 out of 22 patients respectively. Inhomogeneously discriminated EOA and DCD phenotypes (5 out of 21) revealed overlapping SARA scores with different SARA subscore profiles. After phenotypic reassessment with scientifically validated EOA features, phenotypic homogeneity changed from 16 to 18 patients. Interpretation In contrast to complete distinction between EOA and central hypotonia, the paediatric motor phenotype did not reliably distinguish between EOA and DCD. Reassessment with scientifically validated EOA features could contribute to a higher phenotypic consensus. Early-onset ataxia (EOA) and central hypotonia motor phenotypes were reliably distinguished. EOA and developmental coordination disorder (DCD) motor phenotypes were not reliably distinguished. The EOA and DCD phenotypes have different profiles of the Scale for Assessment a

关键词： Genetics   Epilepsy   Neurodevelopmental disorders   Translational genomic research  

摘要： By accumulating ever greater amounts of genomic data, scientists have identified >100 genes associated with Mendelian forms of epilepsy and neurodevelopmental disorders with seizures. For most of the identified genes a wide range of genetic variants have been identified and affected patients are clinically heterogeneous. It is not clear to which degree the clinical heterogeneity can be attributed to the disease causing variant alone. We need to improve our current understanding of biophysical effects of variants on protein function and the role of polygenic background in modifying the clinical representation. In addition, longitudinal clinical data need to be recorded using standardized methods and shared across research centers to build large virtual cohorts for each single gene disorder. Without large, comprehensive, longitudinal datasets, studying the interplay of environmental factors and genetic factors will be challenging. As a community, we must work together to set the foundation for biorepositories and the collection and sharing of 'big data' in order to allow genetic-phenotypic characterization of the epilepsies and to fully utilize the potential for drug discovery, and patient-specific tailored management. (C) 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

摘要： Background Primary cutaneous mucinoses are a heterogeneous group of diseases characterized by the abnormal deposition of glycosaminoglycans (mucin) in the skin. This is the main histopathological clue resulting in distinctive clinical presentations. They are usually diseases of adult age and are uncommon in children, in whom diagnosis and management are difficult tasks. In particular, the classification of primary cutaneous mucinoses occurring in childhood is a difficult and confusing challenge because of the very limited number of described cases and overlaps in their clinicopathological features in the absence of reports of homogeneous case series. Objectives The aim of this review is to help dermatologists and paediatricians to identify patients with cutaneous mucinoses at a paediatric age and to enable institution of appropriate management. A classification of primary paediatric cutaneous mucinoses is also proposed. Results New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses. Conclusions The current update tries to offer a more meaningful guide to the diagnosis and treatment of these rare diseases in paediatric patients.

关键词： caregivers' experiences   early intervention   family centred   paediatric physical therapy   qualitative study  

摘要： Background Caregivers' experiences during early intervention of their infant with special needs have consequences for their participation in the intervention. Hence, it is vital to understand caregivers' view. This study explored caregivers' experiences with the family-centred early intervention programme "COPing with and CAring for infants with special needs" (COPCA). Methods The data of this qualitative study were collected after 6 months of COPCA intervention in 15 families with an infant with special needs. COPCA was delivered by paediatric physiotherapists in training to become COPCA coaches. Caregivers filled out a study-specific questionnaire with three open-ended questions addressing (a) their overall experience with COPCA, (b) what aspects of COPCA they had experienced as important, and (c) what they had learned during the intervention process. The answers were analysed using a content analyses approach. Results Respondents were mothers. Interrater reliability of the content analyses of the three questions was twice excellent (kappa = .95 and kappa = .92) and once good (kappa = .77). The content analysis of the first question resulted in four categories and three subcategories, for example, evaluation of COPCA as a form of intervention and benefit from COPCA. The content analyses of the second question resulted in eight categories, for example, home-based intervention, support from the therapist, and the attainment of competences. The content analyses of the third question generated seven categories and four subcategories, for example, to support the infant autonomously at home and to recognize the competences of the infant, caregiver confidence, and relationship with the infant. Conclusions The participating mothers appreciated the COPCA early intervention programme. They especially valued its home-based setting, the support from the coach, and the experience being able to participate as active partners in the intervention make their own decisions. This me