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关键词： phenotype   staphylococcus aureus   enterococcus   bacteremia   adult   australia   child   drug resistance   microbial   pediatrics   genetics   guidelines   mortality   pathogenic organism   antimicrobials   gram-negative bacteremia   virulence factors   antimicrobial susceptibility   surveillance   medical   extended-spectrum beta lactamases   surveillance program   bloodstream infections   blood culture positive   datasets  

摘要： Background: There is increasing knowledge of antimicrobial usage in children yet limited availability of nationally representative paediatric-specific data on antimicrobial resistance. Objectives: Paediatric data from this national surveillance programme are presented to explore differences between childhood and adult bloodstream infections and antimicrobial resistance surveillance. Methods: Using information collected from a prospective coordinated antimicrobial resistance surveillance programme, children <= 18years and adults >18years with a positive blood culture for Staphylococcus aureus, Enterococcus spp. or Gram-negative spp. presenting to one of 34 Australian hospitals during 2013-16 were evaluated. Consistent methodologies for key sepsis pathogens were employed and a comparative analysis between children and adults was conducted. Results: There are stark contrasts between children and adults in this national antimicrobial resistance (AMR) data set. Notable differences include lower rates of AMR, different clinical and molecular phenotypes and lower mortality amongst children. The burden of Gram-negative resistance is disproportionately experienced in children, with higher odds of death with an ESBL versus non-ESBL bacteraemia in comparison with adults. Conclusions: These data support that children are not just 'little adults' in the AMR era, and analyses by age group are important to detect differences in antibiotic susceptibility, clinical phenotype and genetic virulence factors. Antimicrobial surveillance incorporated into routine laboratory practice is vital to inform an array of wider applications including antimicrobial guidelines, stewardship and direction for prioritization of novel antimicrobial development.

关键词： biomarkers   dietary assessment   micronutrients   nutritional assessment   trace elements   vitamins  

摘要： Assessment of vitamin and trace element status (VTE) is important in the clinical management of the sick child. In this position paper, we present the various assessment methods available to the clinical practitioner, and critically discuss pitfalls with interpretation of their results. There are 4 main approaches to assess the VTE body status of an individual patient including clinical examination, dietary assessment, and measurement of direct and indirect biomarkers of VTE in biological samples. Clinical signs of VTE deficiencies usually present only when body stores are substantially depleted and are often difficult to detect or differentiate from other nonnutrient-related causes. In isolation, dietary assessment of micronutrients can be inaccurate and imprecise, in disease and in individual patient assessment but may be useful to complement findings from other VTE assessment methods. Use of biomarkers is the most common approach to assess VTE status in routine practice but in the presence of systemic inflammatory response and in the absence of appropriate paediatric reference intervals, interpretation of biomarker results might be challenging and potentially mislead clinical practice. The use of a multimodal approach, including clinical examination, dietary assessment, and laboratory biomarkers is proposed as the optimal way to ascertain the VTE status of individual patients. In the presence of acute inflammatory conditions, VTE measurements in plasma should be replaced by biomarkers not affected by systemic inflammatory response or delayed until inflammatory state is resolved.

关键词： pediatric hematology   venetoclax   myelodysplastic syndrome   acute myeloid leukaemia  

摘要： Venetoclax and decitabine for relapsed paediatric myelodysplastic syndrome-related acute myeloid leukaemia with complex aberrant karyotype after second stem cell transplantation Keywords: pediatric hematology; venetoclax; myelodysplastic syndrome; acute myeloid leukaemia EN pediatric hematology venetoclax myelodysplastic syndrome acute myeloid leukaemia e251 e254 4 06/18/20 20200615 NES 200615 Paediatric myelodysplastic syndrome (MDS) is a rare clonal haematologic disorder characterised by cytopenia and ineffective, dysplastic haematopoiesis. For treatment-naive elderly AML patients ineligible for intensive chemotherapy, DiNardo I et al i .[4] recently published promising data for the oral I BCL-2 i inhibitor venetoclax, combined with a hypomethylating agent (HMA), decitabine or azacitidine. [Extracted from the article]

关键词： immunological profiling   inflammatory bowel disease   unsupervised machine learning  

摘要： Objectives: The current classification of inflammatory bowel disease (IBD) is based on clinical phenotypes, which is blind to the molecular basis of the disease. The aim of this study was to stratify a treatment-naive paediatric IBD cohort through specific innate immunity pathway profiling and application of unsupervised machine learning (UML). Methods: In order to test the molecular integrity of biological pathways implicated in IBD, innate immune responses were assessed at diagnosis in 22 paediatric patients and 10 age-matched controls. Peripheral blood mononuclear cells (PBMCs) were selectively stimulated for assessing the functionality of upstream activation receptors including NOD2, toll-like receptor (TLR) 1-2 and TLR4, and the downstream cytokine responses (IL-10, IL-1 beta, IL-6, and TNF-alpha) using multiplex assays. Cytokine data generated were subjected to hierarchical clustering to assess for patient stratification. Results: Combined immune responses in patients across 12 effector responses were significantly reduced compared with controls (P = 0.003) and driven primarily by "hypofunctional" TLR responses (Pvalues 0.045, 0.010, and 0.018 for TLR4-mediated IL-10, IL-1 beta, and TNF-alpha, respectively;0.018 and 0.015 for TLR1-2 -mediated IL-10 and IL-1 beta). Hierarchical clustering generated 3 distinct clusters of patients and a fourth group of "unclustered" individuals. No relationship was observed between the observed immune clusters and the clinical disease phenotype. Conclusions: Although a clinically useful outcome was not observed through hierarchical clustering, our study provides a rationale for using an UML approach to stratify patients. The study also highlights the predominance of hypo-inflammatory innate immune responses as a key mechanism in the pathogenesis of IBD.

关键词： Diastolic wall strain   Stiffness index   Myocardial stiffness   Paediatric end-stage kidney disease   Chronic kidney disease   Children  

摘要： Background We tested the hypothesis that myocardial stiffness is altered in paediatric patients with end-stage kidney disease (ESKD) and explored its association with clinical parameters of chronic kidney disease (CKD). Methods Thirty-five patients with ESKD (16 males) aged 17.5 +/- 3 years old, 18/35 of whom were receiving dialysis and 17 post kidney transplant, were studied. Left ventricular (LV) myocardial stiffness was determined by measurement of diastolic wall strain (DWS) and stiffness index (SI), while LV diastolic function was interrogated by pulsed-wave and tissue Doppler echocardiography. Results Compared with available literature data, both dialysis and transplanted patients had significantly lower DWS and greater SI, reduced transmitral early (E) to late diastolic velocity ratio and septal and lateral mitral annular early (e ') diastolic velocities, and greater septal and lateral E/e ' ratios (all p < 0.05). Multivariate analysis revealed that z score of diastolic blood pressure (beta = 0.43, p = 0.004) and the duration of renal replacement therapy (beta = 0.55, p < 0.001) were significant determinants of LV SI. Subgroup analysis in post-transplant patients showed z score of diastolic blood pressure (beta = 0.54, p = 0.025) remained as a significant determinant of LV SI. Conclusion Increased LV myocardial stiffness is evident in paediatric dialysis and transplanted patients with ESKD, and is associated with blood pressure and duration of renal replacement therapy.

摘要： We describe a complex change process for the paediatric cardiac service in Queensland that involved transitioning the service out of an essentially adult hospital into one of two children's hospitals in Brisbane. This initial step was complex as the governance was changed from Queensland Health to Mater Health, an independent faith-based organisation who became the new employer. Six years later, the service was again transitioned;this time to the newly constructed Queensland Children's Hospital, with a Hospital and Health Services Board as the employer under the aegis of Queensland Health. This was a complex journey. As with all change processes there was resistance to change on the part of some individuals. Five years on from the second major change, the service is settled, has an excellent workplace culture, has excellent clinical outcomes and has become research intensive.

摘要： Here we report on a paediatric case from Germany where TB focused abdominal ultrasound during the diagnostic TB work-up was the determining diagnostic tool to establish a timely diagnosis of active TB. In our case, the additional diagnostic value of TB focused abdominal ultrasound was decisive in establishing the diagnosis of active TB at the time of presentation and hence several weeks before cultures confirmed the diagnosis. Irrespective of TB endemicity children have a higher risk for extra-thoracic TB even in the absence of HIV-infection and may therefore benefit from abdominal ultrasound during TB work-up. In summary, available evidence and our case report suggest that the diagnostic utility of (TB focused) ultrasound as a standard diagnostic examination during paediatric and adult TB work-up should also be investigated in settings with an overall low TB burden. [Extracted from the article]

关键词： metanephric   adenoma   adenofibroma   stromal tumour   renal   paediatric  

摘要： To characterize metanephric tumours in children, we performed a literature review investigating paediatric metanephric adenomas (MA), metanephric stromal tumours (MST) and metanephric adenofibromas (MAF). Including two patients from our own institution (MA, MAF), 110 individual cases (41 MA, 20 MAF, 49 MST) were identified. Additionally, fifteen composite tumours were identified, with areas of MA/MAF and Wilms tumour (WT) or papillary carcinoma. No distinct clinical or radiological features could be defined. In pure metanephric tumours, histologically proven distant metastases were reported once (MA), relapse was reported once (MST) and one tumour-related death occurred (MST). Somatic BRAF-V600E mutations were tested in 15 cases, and identified in 3/6 MA, 3/3 MAF, and 6/6 MST. In our institution the MA harboured a somatic KRAS-G12R mutation. Overall, paediatric metanephric tumours are difficult to discriminate from other renal tumours at presentation, behave relatively benign, and the occurrence of composite tumours warrants analysis of underlying (genetic) pathways.