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关键词： aquaporin 4   demyelination   multiple sclerosis   myelin oligodendrocyte glycoprotein   neuromyelitis optica   paediatric  

摘要： Acquired demyelinating syndromes of the central nervous system are rare disorders in childhood. Immune-mediated damage affects the myelin sheath of the optic nerves, brain and spinal cord. The majority are monophasic. Relapsing demyelinating syndromes include multiple sclerosis, aquaporin-4 antibody-associated disease and myelin oligodendrocyte glycoprotein antibody-associated disease. The relapsing syndromes are associated with gradual accrual of neurological deficits and cognitive impairment. In this review, we discuss the presentation, diagnosis and management of monophasic and relapsing acquired demyelinating syndromes. We highlight the fact that CNS autoantibody mediated disorders are distinct clinical entities separate to multiple sclerosis and discuss the treatment options and prognosis for each. Finally, we detail current available therapeutic drugs for paediatric demyelinating conditions and their adverse effects. © 2019 Elsevier Ltd

关键词： Paediatric fractures   Humerus supracondylar fractures   Radiation exposure   Image intensifier   Fluoroscopy   Percutaneous fixation   Mini C-arm   Radiation safety   Quality improvement   Upper limb  

摘要： Background: Pediatric supracondylar humerus fractures have traditionally been reduced and fixed with conventional C-arm (CCA) fluoroscopy guidance. With the increase in popularity of the newer mini C-arm (MCA) system within other fields of surgery due to its ease of use and lower radiation emission, the same adoption should be explored in pediatric orthopedic surgeries. The purpose of this study is to compare the MCA and CCA primarily in reduction and fixation outcomes and secondarily in other perioperative parameters. Methods: Retrospective analysis of 193 patients who underwent surgical fixation for displaced supracondylar humerus fractures. 44 and 149 cases were performed with the MCA and CCA respectively. Baumann's angle and the intersection of anterior humeral line (AHL) were assessed on postoperative anterior posterior and lateral radiographs and adequate reduction was defined by an angle between 64 and 818, and AHL intersecting middle third of the capitellum. Surgical time, fluoroscopy duration, number of images and radiation exposure were obtained from the surgical notes. Results: Amongst the CCA cases, there were greater satisfactory coronal plane reduction (p < 0.05), while no difference in sagittal plane accuracy (p > 0.05) was seen. In MCA group, longer surgical (p < 0.05) and fluoroscopy times (p < 0.05), and greater number of shots (p < 0.05) was noted. However overall radiation exposure in the MCA group was still lower (p < 0.05). Conclusion: The MCA system is potentially less accurate in coronal plane reduction and more challenging to use. However, pitfalls can easily be avoided. Usage should be advocated as overall radiation exposure can be reduced. (C) 2019 Elsevier Ltd. All rights reserved.

关键词： Bruner   constructivism   first grade narratives   Halliday   Korean   Vygotsky  

摘要： The work of L.S. Vygotsky was popularised in the West between two great waves of educational thought: constructivism and cognitivism. Reception was therefore colored by three metaphors introduced by Jerome Bruner: 'construction', 'scaffolding' and 'narrative'. Narratives were to be characterized by features we call SELF: Subjects, Expectancy and counter-expectancy, a Linear subject-verb-object clause grammar, and a Focalizing voice. In this paper, we try to understand how narratives might be learned in Korean, where subjects are optional and often dispreferred, processes tend to predominate in expectancy over participants, linearity is subject-object-verb rather than subject-verb-object, and even the focal voice must often be shared. For help, we return to Vygotsky's work in 'pedology', the holistic science of the child, and to similarly inspired work on child language by M.A.K. Halliday. First, we explore Vygotsky's own unit for the development of consciousness, perezhivanie, an untranslatable term for the way in which the child 'over-lives' experience through language. Second, we show how Halliday's system networks can help us describe how perezhivanie might develop and we argue that Halliday's term 'construal' is a more useful, non-metaphorical, description of what Vygotsky had in mind. [ABSTRACT FROM AUTHOR]

关键词： Health-related quality of life   Acute lymphoblastic leukaemia   Systematic review   Post-treatment completion   Paediatric cancer  

摘要： Purpose Despite survival rates greater than 90%, treatment for paediatric acute lymphoblastic leukaemia (ALL) remains challenging for families. The early post-treatment phase is an especially unique time of adjustment. The primary aim of this review was to identify and synthesise research on health-related quality of life (HRQoL) for patients up to five years post-treatment. The secondary aim was to identify if theorised risk/resistance model factors could explain any variance in reported HRQoL. Methods We conducted a systematic review using the PRISMA guidelines across five databases: Embase, Medline, Psychinfo, Pubmed, and Cochrane. Only studies examining HRQoL up to five years post-treatment were included. Studies were excluded if they covered periods greater than five years post-treatment or did not differentiate between patients with ALL and other cancers. After assessing the quality of each study sample size, patient characteristics, HRQoL outcomes and HRQoL correlates were extracted and summarised. Results A total of 14 studies representing 1254 paediatric patients, aged 2-18 years, were found. HRQoL findings were mixed, dependent on time since completion and comparison group. Patient HRQoL was mostly lower compared to normative data, whilst higher compared to healthy control groups, patients on treatment, and patients with other types of cancers. Lower HRQoL was also found to be associated with demographic (age and sex), family dysfunction, and treatment-related factors. Conclusions Completing treatment signalled a significant improvement in HRQoL for patients compared to being on treatment. Overall, however, HRQoL was still significantly lower than the population during the early post-treatment period.

关键词： lamina cribrosa   optic nerve head   paediatric glaucoma   steroid-induced glaucoma  

摘要： Click to increase image sizeClick to decrease image size

关键词： HIV   adolescent   growth   stunting   cohort studies   developing countries  

摘要： Introduction Stunting is a key issue for adolescents with perinatally acquired HIV (APH) that needs to be better understood. As part of the IeDEA multiregional consortium, we described growth evolution during adolescence for APH on antiretroviral therapy (ART). Methods We included data from sub-Saharan Africa, the Asia-Pacific, and the Caribbean, Central and South America regions collected between 2003 and 2016. Adolescents on ART, reporting perinatally acquired infection or entering HIV care before 10 years of age, with at least one height measurement between 10 and 16 years of age, and followed in care until at least 14 years of age were included. Characteristics at ART initiation and at 10 years of age were compared by sex. Correlates of growth defined by height-for-age z-scores (HAZ) between ages 10 and 19 years were studied separately for males and females, using linear mixed models. Results Overall, 8737 APH were included, with 46% from Southern Africa. Median age at ART initiation was 8.1 years (interquartile range (IQR) 6.1 to 9.6), 50% were females, and 41% were stunted (HAZ<-2 SD) at ART initiation. Males and females did not differ by age and stunting at ART initiation, CD4 count over time or retention in care. At 10 years of age, 34% of males were stunted versus 39% of females (p < 0.001). Females had better subsequent growth, resulting in a higher prevalence of stunting for males compared to females by age 15 (48% vs. 25%) and 18 years (31% vs. 15%). In linear mixed models, older age at ART initiation and low CD4 count were associated with poor growth over time (p < 0.001). Those stunted at 10 years of age or at ART initiation had the greatest growth improvement during adolescence. Conclusions Prevalence of stunting is high among APH worldwide. Substantial sex-based differences in growth evolution during adolescence were observed in this global cohort, which were not explained by differences in age of access to HIV care, degree of immunosuppression or re

关键词： Cochlear implant   Complications   Posterior tympanotomy  

摘要： Cochlear implants surgery is now routinely done at this center. Complications which have been experienced in cochlear implant surgery at our center are discussed, as a detailed knowledge about the complications and their early anticipation can save an expensive device and most importantly patients morbidity. To discuss the complications of the paediatric cochlear implantation in the population of Uttarakhand. The total of 60 patients were included in this study (age group 1-5 years) who had undergone the cochlear implantation surgery between Sept. 2015 and Sept. 2016. These patients were regularly monitored and followed up for 1 year. All the surgeries were performed by same team of surgeons. Rate of minor complications is 8.33% (reversible facial nerve paresis, injury to chorda tympani nerve, local skin reaction, perilymph gussher) and major complications is 5% (implant rejection and flap necrosis). The various complications should be closely monitored by the surgeon so that these can be avoided.

关键词： Pediatric   Tympanoplasty   Cartilage   Temporalis fascia  

摘要： To compare the anatomic and functional outcomes of the cartilage and temporalis fascia graft materials in type 1 tympanoplasty in paediatric and adolescent population. A total of 55 patients aged <18 years who required type 1 tympanoplasty were selected. 30 patients underwent cartilage palisade tympanoplasty and 25 using temporalis fascia grafts. The age, the side of the operated ear, the operative technique, pre- and post operative pure tone and impedance audiological results, and the status of the graft were noted. Graft was considered taken up if there was successful closure of tympanic membrane perforation. At the end of 6 months, the graft take rate for cartilage was 90% and for temporalis fascia it was 80% (p > 0.05). ABG closure ratio in cartilage group was 58.54 +/- 23.10% and in temporalis fascia group was 56.46 +/- 27.4% (p > 0.05). Pre operatively all patients had type B tympanogram in both the groups. While post operatively either type A or C curve was seen in 80% patients of cartilage and 68% patients of temporalis fascia group. Hearing outcomes and graft success rates were high in both fascia and cartilage graft groups but not significantly different.

摘要： Therefore, effective and safe treatment methods that cause minimal pain are desirable in paediatric patients. Because painful procedures require a high degree of co-operation from paediatric patients, we decided to treat this patient with a less painful method using a pneumatic needleless injection device (INNOJECTOR; WONIK. Third, pneumatic needleless injection devices also have the advantage of minimizing the pain and fear experienced by patients, which allows it to be a well-tolerated treatment method for paediatric patients.[4] Before the treatment, applying a topical anaesthetic cream is sufficient to control the pain induced by the treatment. [Extracted from the article]

关键词： Antimicrobial resistance   Extended-spectrum beta-lactamases   AmpC beta-lactamases   Carbapenemases   Methicillin-resistant Staphylococcus aureus   Enterococcus   Pseudomonas aeruginosa  

摘要： A progressive increase in the incidence of infections caused by multidrug-resistant microorganisms is being reported. Among these resistant microorganisms, the main threats are extended-spectrum beta-lactamase-, AmpC-, and carbapenemase-producing Gram-negative bacilli, methicillin- resistant Staphylococcus aureus and vancomycin- resistant Enterococcus faecium. To address this important problem, it is essential to establish pediatric Antimicrobial Stewardship programs, perform active epidemiological surveillance and develop an adequate infection control policy. The therapeutic approach of these infections is often complex, frequently requiring antibiotics with less experience in children. In this position document made by the Spanish Association of Pediatrics and the Spanish Society of Pediatric Infectious Diseases, the epidemiology and treatment of these infections are reviewed according to the best available evidence. (C) 2019 Asociacion Espanola de Pediatria. Published by Elsevier Espana, S.L.U.