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关键词： Matrix algebra  

摘要： Liver plays an important role in drug metabolism, so studying the grade of maturation of this organ would help to develop more appropriate dosing regimens for paediatric populations. Nevertheless, considering the invasive nature of liver analyses there are obvious ethical boundaries, particularly in babies and children. In this work, we investigated the suitability of blood plasma as an alternative matrix to evaluate the biological age of liver. With this aim, we studied the correlation of plasma and liver metabolomic profiles obtained by HPLC-TOF-MS for piglets of different ages (newborns, neonates and infants). By means of Pearson correlation analysis we observed that 360 and 1784 pairs of metabolite features were significantly correlated in positive and negative ionization mode, respectively. Procrustes analysis was applied in order to assess the similarity of the clustering resulting from the data obtained from the two matrices and the two ionisation modes. The Procrustes distances were low for both ESI+ (0.3753) and ESI- (0.3673) and, hence, liver and plasma are expected to provide similar discriminatory information. Furthermore, we found that Multiblock Principal Component Analysis (MB-PCA) readily allowed us to combine the data obtained from both matrices and to better understand the clustering according to the three study groups. Considering all these results, we suggest that plasma can provide valuable insight into the maturation grade of liver in order to provide accurate dosing in paediatric population.

摘要： Introduction: Paediatric Inflammatory Multisystem Syndrome - temporally associated with SARS-CoV2 (PIMS-TS) is a newly described syndrome during the COVID-19 pandemic. It is characterised by a state of persistent fever, inflammation and organ dysfunction following exposure to SARS-CoV-2 virus. It shares similar clinical features to Kawasaki disease, toxic shock syndrome and macrophage activation syndrome. Like adult COVID-19 infection, profound inflammatory response significantly increases the risk of thromboembolism, which is a major cause of morbidity and mortality. In this review we share our experience, observation and prevention strategy with regard to thromboembolism in PIMS-TS. Method: Between the period of 14th March 2020 to 31st May 2020, Evelina London Children's Hospital admitted 68 patients with PIMS-TS from the South Thames Network. Patients were broadly treated with a combination of high dose aspirin and immunomodulation in the initial period. Realising its pro-thrombotic potential, from 1st May 2020, treatment strategy was changed to incorporate prophylactic subcutaneous low molecular weight heparin (dalteparin 100units/kg once daily) in combination with low dose aspirin (5mg/kg once daily, maximum 75mg) when a working diagnosis of PIMS-TS was made. We retrospectively collected data to understand the prevalence and pattern of thromboembolism in this cohort of patients. Results: Five patients (7.4%) developed thromboembolic complications during their acute illness with 6 recorded events overall. (Table 1) Three events happened without prophylactic anticoagulation cover. Four (5.9%) were cardiovascular/arterial events and 2 (2.9%) were venous thromboembolism. Those who required ventilation support and extracorporeal membrane oxygenation (ECMO) were associated with higher thrombotic rate using 2-tailed Fisher's exact test (ventilation: p=0.02; ECMO: p=0.004). One ischaemic cerebrovascular event resulted in death. Two bleeding events were recorded whilst

摘要： Congenital sideroblastic anaemias (CSA) are a rare group of disorders characterized by the presence of pathologic iron deposits within the mitochondria of erythroid precursors (ring sideroblasts) in the bone marrow due to heterogenous germline mutations leading to defects in mitochondrial heme synthesis, iron-sulfur (Fe-S) cluster biogenesis, or protein synthesis. Patients present with anaemia and relative reticulocytopenia, and systemic iron overload secondary to chronic ineffective erythropoiesis, leading to end-organ damage. The disease is heterogenous underlying the genetic variability and the variable response to treatment. Although a number of CSA patients have received a bone marrow transplant, the outcomes and toxicities are not known. This status makes it very difficult to understand the role of BMT in the management of CSA. A search in the EBMT database identified 28 patients receiving a HSCT for CSA between 1998 to 2018 by 24 participating centres. The median year of transplantation was 2014 (IQR 2004-2016). The distribution was equal between males (n=14) and females (n=14). The median age at transplantation was 7 years of age (3-10 years). Fifteen patients had a sibling HSCT (88%), one a family matched donor HSCT (6%) and one an unrelated matched (6%), the type of transplant being unknown in others (n=11). The source of stem cells was bone marrow in 20 cases (74%), peripheral blood in 4 cases (15%), cord blood in 2 (7%) and combined bone marrow and cord in one (4%). Five cases had a Bu/Cy based conditioning regimen, 4 had Bu/fludarabine based regimen and three fludarabine/treosulfan based conditioning with the rest having a variety of approaches. Eighty-six percent of cases had serotherapy with ATG or alemtuzumab. The median follow-up was 31.6 months (95% CI, 12.2-74.1%). The overall survival at 12 and 24 months was 88% (76-100) and 82% (66-99), respectively (figure 1). The median neutrophil engraftment was 18 (15-21) days and platelet engraftment >20 x

关键词： Rare disease   Paediatric   Mortality   Health care burden   Epidemiology  

摘要： Aims: To ascertain the number of paediatric deaths (0-14 years) with an underlying rare disease in the Republic of Ireland between the years 2006-2016, and to analyse bed usage by a paediatric cohort of rare disease inpatients prior to in-hospital death. Background: Rare diseases are often chronically debilitating and sometimes life-threatening diseases, with the majority (69.9%) of rare diseases being of paediatric onset. The Orphanet database contains information on 6172 unique rare diseases. Under-representation of rare diseases in hospital healthcare coding systems leads to a paucity of rare disease epidemiological data required for healthcare planning. Studies have cited variable incidence rates for rare disease, however the burden of rare diseases to healthcare services still remains unclear. This study represents a thorough effort to identify the percentage of child mortality and paediatric bed usage attributable to rare diseases in the Republic of Ireland, thus addressing a major gap in the rare disease field. Methods: Retrospective analysis of paediatric death registration details for the Republic of Ireland in the 11-year period 2006-2016 from the National Paediatric Mortality Register. Data was subcategorised as Neonatal (0-28 days), Post Neonatal (29 days < 1 year) and older (1-14 years). Bed usage data (ICD-10 code, narrative and usage) of paediatric inpatients who died during hospitalisation from January 2015 to December 2016 was extracted from the National Quality Assurance Improvement System of in-patient data. Orphacodes were assigned to rare disease cases from ICD-10 codes or diagnostic narrative of both datasets. Results: There were 4044 deaths registered from 2006-2016, aged < 15 years, of these 2368 (58.6%) had an underlying rare disease. Stratifying by age group;55.6% (1140/2050) of neonatal deaths had a rare disease, 57.8% (450/778) post-neonatal, and 64% (778/1216) of children aged 1-14 years. Mortality coding using ICD-10 codes identified 42

关键词： Social   History   Paediatrics   Documentation  

摘要： Background A child's home and family environment plays a vital role in neuro-cognitive and emotional development. Assessment of a child's home environment and social circumstances is an crucial part of holistic Paediatric assessment. Aims Our aim is to achieve full compliance with comprehensive documentation of biopsychosocial history, for all children medically admitted to the children's inpatient unit in University Hospital Limerick. Methods We performed a retrospective chart review to audit documentation within our department. This was followed by teaching interventions and a survey on knowledge, attitudes and behaviour of paediatric non-consultant hospital doctors (NCHDs) towards the social history. We performed two subsequent re-audits to assess response to our interventions, and provided educational sessions to seek improvement in quality of care. Results Results showed a significant improvement in quality of documentation following interventions, demonstrated by a net increase of 53% in levels of documentation of some social history on first re-audit. Though this was not maintained at an optimum level throughout the course of the year with compliance reduced from 95% to 82.5% on second re-audit, there was nonetheless a sustained improvement from our baseline. Our qualitative survey suggested further initiatives and educational tools that may be helpful in supporting the ongoing optimisation of the quality of documentation of social history in our paediatric department. Conclusion We hope this quality improvement initiative will ultimately lead to sustained improvements in the quality of patient-centred care, and early identification and intervention for children at risk in our community.

关键词： Neurorehabilitation   Paediatric   Cerebral malaria   Qualitative   Malawi  

摘要： Background We sought to identify perceptions of neurorehabilitation challenges for paediatric cerebral malaria (CM) survivors post-hospital discharge at Queen Elizabeth Central Hospital (QECH) in Blantyre, Malawi. Methods An exploratory approach was used to qualitatively investigate the perceived neurorehabilitation challenges for paediatric CM survivors. Data were collected through semi-structured in-depth interviews (IDIs) and focus group discussions (FGDs). Eighteen data-gathering sessions were conducted with 38 total participants, including 3 FGDs with 23 primary caregivers, 11 IDIs with healthcare workers at QECH, and 4 IDIs with community-based rehabilitation workers (CRWs). Results FGDs revealed that caregivers lack important knowledge about CM and fear recurrence of CM in their children. Post-CM children and families experience substantial stigma and sociocultural barriers to integrating into their community and accessing neurorehabilitative care. At a community-level, rehabilitation infrastructure, including trained staff, equipment, and programmes, is extremely limited. Rehabilitation services are inequitably accessible, and community-based rehabilitation remains largely unavailable. Conclusions There is an urgent need to establish further training of rehabilitation personnel at all levels and to build accessible rehabilitation infrastructure in Malawi for post-CM patients. Additional work is required to expand this study across multiple regions for a holistic understanding of neurorehabilitation needs.

关键词： 中西医结合医院   重点专科建设   浙江省中医   医学重点学科   中医儿科学   重大疾病   中医药防治   儿科  

摘要： 浙江省中西医结合医院儿科创建于1950年,是杭州市乃至全省具有影响力、中西医结合特色明显的小儿专科。其中中医儿科学已列入“十三五”浙江省中医药(中西医结合)重点学科建设计划和“十三五”浙江省中医药(中西医结合)重点专科建设计划。目前是杭州市医学重点学科。

关键词： Paediatric primary optic nerve sheath meningioma   treatment   diagnosis  

摘要： A 16-year-old male presented with a three year history of right proptosis. All other ocular findings were normal. Imaging demonstrated a large, calcified, contrast-enhancing mass in the apical orbit. The tumour had high gallium-68-DOTATATE uptake and low 18F-2-fluoro-2-deoxy-D-glucose uptake. An incisional biopsy revealed a diagnosis of psammomatous optic nerve sheath meningioma (ONSM). One year later stereotactic radiotherapy was performed due to tumour growth. Tumour size and visual acuity remained stable in the six months after treatment. This case differs from previously reported paediatric ONSMs by its histo-clinical characteristics (exophytic-calcified mass, visual preservation, psammomatous histology).

关键词： 走进大自然   探究活动   科学探究   资源优势   物质保障   幼儿   近距离接触  

摘要： 我园紧邻美丽的农田、苗圃、公园,利用这一独特的资源优势,我们进行了课程建设及创新。这些宝贵的植物资源,为幼儿进行科学探究提供了坚实的物质保障。幼儿在探究中走进大自然,与植物近距离接触,掌握了相关的科学探究方法,同时开展了探究活动。