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摘要： Introduction: In Poland, the principles for the management of rare bleeding disorders as well as financing of medication are regulated by the National Program for the Management of Haemophilia and Related Bleeding Disorders. One objective of the Program for 2012-2018 was to include the financing of ITI for all haemophilia patients with inhibitor. The qualification for ITI was performed by a physician from a Haemophilia Treatment Centre (HTC). Goal: the aim of the study is/was prospective assessment of the efficacy of ITI in real life setting. Patients and Methods: The study comprised 30 patients with severe and one with moderate HA and HT FVIII inhibitors. Factor VIII activity (FVIII:C) was measured by one-stage clotting assay and Factor VIII inhibitors by a modified Nijmegen-Bethesda assay and expressed in Bethesda Units/ml (BU/ml). Cut-off level for positive inhibitor was ≥0.5BU/ml. The following data was collected: historical peak inhibitor titre (HPT), pre-ITI titre (measured 1 month prior to ITI), peak titre recorded during the course of ITI, time between inhibitor detection and ITI start, and ITI duration. During ITI course blood samples were collected every 4 weeks. Treatment regimen with dosing and type of FVIII concentrate was at the discretion of the physician in each HTC. ITI success was defined as inhibitor titre below 0.5 BU/ml with FVIII half-life ≥6h and FVIII recovery ≥66% of normal. Partial response to ITI was defined as clinical response to FVIII therapy with no anamnestic response despite the presence of FVIII inhibitor or FVIII half-life <6h and FVIII recovery <66% of normal. Results: 31 patients with HA and inhibitor were divided into two groups: Group 1 comprised 12 adults with severe HA, median age 36 years (mean 40.1±15.1 and Group 2 included 19 boys, (<18 years) (18 with severe, one with moderate HA), median age 8 years (mean 8.1±4.3) The median (mean) duration time between inhibitor detection and ITI start was 108 months (102±91) in Group 1

摘要： Despite the accumulation of genetic mutations likely to generate antigenic neoepitopes, acute lymphoblastic leukaemia (ALL) cells are poor at eliciting anti-tumour immune responses. Similarly to dendritic cells (DCs), B cells have antigen presenting capacity. In B cell ALL (B-ALL), blast expression of co-stimulatory molecules is down regulated whilst endogenous (tumour) antigens are presented through MHC class I and II[1], thus mimicking the DC peripheral tolerance mechanism. This leads to antigen specific anergy in naïve T cells capable of recognising the malignant cells and an anti-tumour immune response is not mounted. A robust DC response, presenting tumour antigens alongside co-stimulation, should be sufficient to overcome this since no intrinsic T cell defect exists in these patients. However, several studies have reported deficiencies in DC number and function in patients with B-ALL. Maecker et al. demonstrated that paediatric patients with B-ALL have significantly reduced blood DC counts at diagnosis, a reduction not attributable to disease induced bone marrow hypoplasia since blood DC numbers do not correlate with granulocyte or monocyte numbers or haemoglobin[2]. Mami et al. identified that CD34+ peripheral blood mononuclear cells from patients with B-ALL at diagnosis are ineffective at generating functional DCs in vitro suggesting that B-ALL cells, or leukaemia derived factors, may contribute to a block in DC development[3]. In this study, we monitored DCs and plasma cytokines in paediatric patients with B-ALL at diagnosis and throughout remission induction chemotherapy, in peripheral blood (PB) and the bone marrow (BM) tumour microenvironment, to identify on treatment changes in DCs and DC regulatory cytokines. 17 paediatric patients diagnosed with precursor B cell ALL were enrolled in this study. Matched PB and BM aspirate samples were collected at time points co-ordinating with treatment protocol (diagnosis, and induction days 8 and/or 15 and 29). Mono

摘要： Autoimmune hemolytic anemia (AIHA) is an uncommon disease of childhood caused by the premature destruction of erythrocytes by autoantibodies. In this rare disease both diagnostic criteria and therapeutic approaches are not well standardized. The Red Cell Working Group of the Pediatric Italian Hematogy and Oncology Association (AIEOP) developed specific recommendations to help Physicians for AIHA management. The document is available on the AIEOP website since November 1st 2013. The Italian Pediatric AIHA Group began an observational, retrospective and prospective study in order to monitor the management of children with AIHA diagnosed from 2010 to 2018, and to assess whether the availability of AIEOP recommendations had an impact on the clinical management of such patients in AIEOP Centers. We collected a national cohort of 159 children with AIHA from 21 AIEOP Centers; 48 patients were diagnosed before November 2013 and 111 patients after that date. Gender was 56% males and 44% females; median age at diagnosis was 47 months, with 11.9% under 12 months of age; 8.2% of children were born prematurely and 3.9% showed congenital malformations. 23.2 % of patients had a familiar history of immunological, hematological or oncological diseases. The median hemoglobin level at diagnosis was 6.1 gr/dL. Table 1 reports the distribution of our cases, according to the different type of autoantibodies. The comparison between the retrospective and prospective study did not reveal significative differences in clinical and biological presentation. The cold IgM forms were mainly post infective (38.4%) or primary forms (53.8%), only one patient had a secondary form due to a primitive immunodeficiency. These patients did not develop other diseases during follow up (median follow up: 28,6 months). The preliminary results of treatment and follow up of the 146 patients with warm antibody AIHA revealed the following: The treatment with conventional dose of steroids (median dose 2 mg/Kg, rang

摘要： Paediatric medical emergencies can be distressing for the child, parent and clinician alike. This paper aims to remind those in primary dental care how to manage these emergencies safely and effectively. Topics covered include paediatric airway management, basic life support, defibrillation, choking, asthma, paediatric emergency drug doses and epilepsy.

摘要： Children's oral health, and the number of children attending for dental treatment under general anaesthesia continue to be in the spotlight of the dental press. But are all children across the UK given the same opportunities to access comprehensive dental care under general anaesthesia? This article gives details of a recent evaluation of dental general anaesthetic services currently provided in the UK. It outlines the current picture of services, discusses the challenges and the implications of the results, and makes suggestions to help improve the regional inequalities shown by the evaluation.

关键词： Paediatric cancers   Prevalence   Mortality   Incidence   Morbidity   Risk factors   Trends   HIV  

摘要： Background Paediatric cancers account for a minor fraction of deaths and hence receive little attention from policymakers. In low-income countries, the absence of comprehensive national paediatric strategies results in a lack of access for a majority of children with cancer. In sub-Saharan Africa (SSA), the burden of childhood cancers is underestimated due to a lack of paediatric cancer registries, poor health care systems and competing healthcare needs. The objective of this study is to map evidence on the distribution of paediatric cancers in the SSA region. Method A scoping review will be conducted to map literature on the distribution of paediatric cancers in SSA. An electronic literature search will be conducted from the following databases: PubMed, Google Scholar, EBSCOhost (CINAHL and Health Source) and World Health Organization (WHO)/International Agency for Research in Cancer (IARC) (GLOBOCAN databases). We will also search the reference lists of included studies to source relevant literature. A pilot search was conducted to determine the feasibility of the study. Study selection will be guided by the inclusion and exclusion criteria. After charting the data, a descriptive overview of the studies will be presented in a narrative format. An account of the study characteristics will be described in this narrative. The analysis will be mainly based on mapping the country-specific outcomes emerging from the studies, and a numerical summary of these outcomes will be conducted. Tables, maps and charts will be produced and presented in the result section. Discussion This review study will identify existing research gaps for future research to influence policy implementation and to improve the availability of diagnosis and treatment of paediatric cancers in SSA.

关键词： Klebsiella pneumoniae   ESBLs   Antimicrobial susceptibility   Molecular characterization  

摘要： Background: Emergence and spread of beta-lactamase resistant Klebsiella pneumoniae have posed a serious threat, especially in paediatric patients globally. The present study focuses on explore drug resistance profile and molecular characterization of carbapenemase and extended-spectrum beta-lactamase producing K. pneumoniae isolated from paediatric patients in Shenzhen, China. Methods: Present study, a total of 31 isolates of multi-drug resistant K. pneumoniae were collected from Shenzhen Children's Hospital, China during Jan 2014 to December 2015. ESBLs production was confirmed by using the combination disc diffusion method followed by antimicrobial susceptibility. In addition, beta-lactamase encoding genes were determined by PCR assay and sequencing. The genotypic diversity and phylogenetic relationship were determined by multi-locus sequence typing (MLST) method and pulsed-field gel electrophoresis (PFGE). Results: We examined 31, unique K. pneumoniae isolates collected from 2014 and 2015 in Shenzhen Children's Hospital, China. All the 31 isolates 100% were resistant to ceftazidime, ertapenem, ampicillin, cefazolin and ampicillin-sulbactam followed by ceftriaxone 94% (n = 29), aztreonam 89% (n = 26), cefepime 84% (n = 26), nitrofurantoin 75% (n = 24), piperacillin 52% (n = 16), and levofloxacin 49% (n = 15). Of the 31 beta-lactamase gene coding isolates, bla(CTX-M) was mainly detected in about 100% (n = 31), followed by bla(KPC) 71% (n = 22), bla(SHV) 61% (n = 19), bla(NDM) 25% (n = 8), bla(CYM) 13% (n = 4), bla(OXA-48) 9% (n = 3), bla(GES) 9% (n = 3) and bla(TEM) 6% (n = 2). Seventeen distinct sequences type were observed with ST20 being mostly identified 16% (n = 5). Pulsed-field gel electrophoresis (PFGE) typing showed that identical profile for the isolates recovered from the Department of Intensive Care Unit and Department of Neurology of our hospital. Plasmid replicon typing result indicates the presence of IncFIS type as highest in all isolates as 61% (n =

关键词： Integrated care   paediatric   implementation   co-location   utilization   primary care  

摘要： Several studies have demonstrated clinical benefits of integrated care for a range of child and adolescent mental health outcomes. However, there is a significant gap between the evidence for efficacy of integrated care interventions vs their implementation in practice. While several studies have examined large-scale implementation of co-located integrated care for adults, much less is known for children. The goal of this scoping review was to understand how co-located mental health interventions targeting children and adolescents have been implemented and sustained. The literature was systematically searched for interventions targeting child and adolescent mental health that involved a mental health specialist co-located in a primary care setting. Studies reporting on the following implementation outcomes were included: acceptability, adoption, appropriateness, feasibility, fidelity, implementation cost, penetration, and sustainability. This search identified 34 unique studies, including randomized controlled trials, observational studies, and survey/mixed method approaches. Components facilitating implementation of on-site integrated behavioural healthcare included interprofessional communication and collaboration at all stages of implementation;clear protocols to facilitate intervention delivery;and co-employment of integrated care providers by specialty clinics. Some studies found differences in service use by demographic factors, and others reported funding challenges affecting sustainability, warranting further study.

关键词： 精品科技期刊   中华医学会系列杂志   核心期刊   来源期刊   新乡医学院   儿科学   核心学术期刊   儿科临床  

摘要： 《中华实用儿科临床杂志》(原《实用儿科临床杂志》)是由中国科学技术协会主管、中华医学会主办、新乡医学院承办的中华医学会系列杂志,是以儿科临床与基础研究为主要报道内容的儿科学类核心期刊。本刊为中国科学引文数据库(CSCD)来源期刊,儿科学类核心期刊,中国科技论文统计源期刊(中国科技核心期刊),RCCSE中国核心学术期刊,中国精品科技期刊,中国科学技术协会精品科技期刊.