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关键词： access to transplantation   cardiovascular complication   chronic kidney disease in children   early versus late dialysis   timing of dialysis initiation  

摘要： Background There is no consensus regarding the timing of dialysis therapy initiation for end-stage kidney disease (ESKD) in children. As studies investigating the association between timing of dialysis initiation and clinical outcomes are lacking, we aimed to study this relationship in a cohort of European children who started maintenance dialysis treatment. Methods We used data on 2963 children from 21 different countries included in the European Society of Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association Registry who started renal replacement therapy before 18years of age between 2000 and 2014. We compared two groups according to the estimated glomerular filtration rate (eGFR) at start: eGFR >= 8mL/min/1.73 m(2) (early starters) and eGFR >= 8mL/min/1.73 m(2) (late starters). The primary outcomes were patient survival and access to transplantation. Secondary outcomes were growth and cardiovascular risk factors. Sensitivity analyses were performed to account for selection- and lead time-bias. Results The median eGFR at the start of dialysis was 6.1 for late versus 10.5mL/min/1.73 m(2) for early starters. Early starters were older [median: 11.0, interquartile range (IQR): 5.7-14.5 versus 9.4, IQR: 2.6-14.1years]. There were no differences observed between the two groups in mortality and access to transplantation at 1, 2 and 5 years of follow-up. One-year evolution of height standard deviation scores was similar among the groups, whereas hypertension was more prevalent among late initiators. Sensitivity analyses resulted in similar findings. Conclusions We found no evidence for a clinically relevant benefit of early start of dialysis in children with ESKD. Presence of cardiovascular risk factors, such as high blood pressure, should be taken into account when deciding to initiate or postpone dialysis in children with ESKD, as this affects the survival.

关键词： B-cell precursor acute lymphoblastic leukaemia   EWSR1   ZNF384   integrated genomic analysis  

摘要： Novel three-way fusions among ZNF384, EWSR1 and EHMT1 genes in paediatric B cell precursor acute lymphoblastic leukaemia with translocations resembling Philadelphia chromosomes Treatment at various timepoints is shown along the top of the timeline, the percentage of blast cells by flow cytometry is indicated by the red line, clinical response is indicated along the bottom, EWSR1-ZNF384 expression monitored by quantitative polymerase chain reaction (PCR) is also shown along the bottom. Although I EWSR1-ZNF384 i is extremely rare, I ZNF384 i rearrangements are reported in 1-6% of childhood BCP-ALL and 5-15% of adult BCP-ALL and constitute a new molecular subtype (Lilljebjorn & Fioretos, [5]). Schematic comparison of fusion points in reported cases with EWSR1-ZNF384 and TCF3-ZNF384, and in the present case with *** SI. 1300 leukemia-related genes. [Extracted from the article]

关键词： acute respiratory infection   antibiotic resistance   antibiotic stewardship   child pneumonia  

摘要： Aim To characterise paediatricians' antibiotic-prescribing behaviour when managing community acquired pneumonia. Methods We conducted a knowledge and attitudes survey of paediatric doctors practicing at a regional provincial hospital in central Vietnam over a 2-week period (from 12 December 2017 to 29 December 2017). Results Of 79 eligible paediatric doctors, 69 (87.3%) completed the questionnaire, of whom 65 (94.2%) thought that antibiotics were overused in Vietnam. Thirty-eight doctors (55.1%) indicated that they routinely hospitalised children with pneumonia to provide intravenous antibiotics. Most doctors reported discharging children with non-severe pneumonia after 5 days (76.9%) and those with severe pneumonia after 7-10 days (88.4%);older doctors generally continued intravenous antibiotics for longer. The two most important factors driving discharge decisions were clinical assessment (95.6%) and completion of the full course of intravenous antibiotics (80.0%). Antibiotic prescription was influenced by local guidelines (62.3%), drugs used before admission (50.0%) and the opinion of senior clinicians (37.7%). Most doctors believed antibiotic stewardship was necessary (98.6%) and that over-the-counter use of antibiotics should be restricted (97.1%). Conclusions Paediatricians recognised an urgent need for more effective regulation and antibiotic stewardship in Vietnam. Routinely completing a full course of intravenous antibiotics leads to unnecessary and prolonged hospitalisation.

关键词： Paediatric patient   Moyamoya disease   Tourette's syndrome   Cerebral revascularisation  

摘要： Background: Moyamoya disease, an uncommon chronic intra-cerebral arteriopathy asymmetrically affecting the proximal vasculature, is rarely associated with clinical features pertaining to movement disorders. Case Description: A 5-year-and-9-month-old boy developed repetitive episodic involuntary winking of the right eye along with ipsilateral shoulder shrugging movements in an absolutely conscious state, associated with paroxysmal shouts and loud laughs and punctuated with abusive verbal expressions (coprolalia). These episodic features, over the course of the next 1.5 years, got progressively accentuated by situations which evoked stress. In addition, there was progressive regression of verbal and cognitive milestones, emotional lability and aspects of attention deficit hyperkinetic disorder. The child was evaluated by a neurologist with magnetic resonance imaging of the brain, which showed characteristic ischaemic areas involving the basal ganglia and fronto-parietal cortical areas along the middle cerebral artery territory, predominantly on the left side. Subsequent cerebral angiography revealed extensive stenosis of bilateral (predominantly left-sided) internal cerebral arteries and middle cerebral arteries with evidence of diffuse leptomeningeal collaterals. The electroencephalography was reported to be normal. He was eventually diagnosed to be suffering from Moyamoya disease with associated Tourette's syndrome. Subsequently, the child underwent left-sided superficial temporal artery to middle cerebral artery anastomosis along with encephalo-duro-arterio-myo-synangiosis. Significant clinico-radiological improvement was noted after 3 months. The clinical deficiencies had dramatically resolved. There was evidence of excellent development of both direct and indirect surgical collaterals along the left middle cerebral artery territory. He could go back to school. Conclusion: Ours is probably the first case reporting an association of paediatric Moyamoya disease with

关键词： Analytical chemistry   Anatomy   Antimicrobial resistance   Antimicrobials   Bacteria   Bacterial pathogens   Biochemistry   Biology and life sciences   Chemistry   Computer and information sciences   Data management   Digestive system   DNA   Drugs   Enterobacteriaceae   Evolutionary biology   Evolutionary systematics   Forms of DNA   Gastrointestinal tract   Genetic elements   Genetics   Genomics   Klebsiella   Klebsiella pneumoniae   Mass spectrometry   Matrix-assisted laser desorption ionization time-of-flight mass spectrometry   Medical microbiology   Medicine and health sciences   Microbial control   Microbial pathogens   Microbiology   Mobile genetic elements   Nucleic acids   Organisms   Pathogens   Pathology and laboratory medicine   Pharmacology   Phylogenetic analysis   Phylogenetics   Physical sciences   Plasmids   Rectum   Research and analysis methods   Research Article   Spectrum analysis techniques   Taxonomy  

摘要： In Argentina, NDM metallo-β-lactamase was first reported in 2013. By now, it has disseminated throughout the country in diverse Gram negative bacteria. Here, we report the case of a paediatric patient that underwent a 1-year hospitalisation due to erythrodermic psoriasis in 2014 and received multiple antimicrobial treatments. During his stay, five isolates were obtained from rectal swabs (rs) or blood culture (bc) suspicious of carbapenemase production: a K. quasipneumoniae subsp. quasipneumoniae (rs), Citrobacter freundii (rs), Escherichia coli (bc), Enterobacter cloacae (rs), and a Serratia marcescens (bc). The isolates were studied with broth microdilution, biparental conjugation and plasmid and whole genome sequencing (Illumina). All isolates harboured an 138,998-bp type 1 IncC plasmid that carried bla_NDM-1, ble_MBL, bla_CMY-6, rmtC, aac(6’)-Ib, and sul1 resistance genes. Additionally, the bla_NDM-plasmids contained ISKpn8 an insertion sequence previously described as associated only to bla_KPC. One isolate, a colistin-resistant E. coli, also carried a mcr-1-containing an IncI2 plasmid, which did not harbour additional resistance. The whole genome of K. quasipneumoniae subsp. quasipneumoniae isolate was fully sequenced. This isolate harboured, additionally to bla_NDM, three plasmid-mediated quinolone resistance genes: qnrB4, qnrB52 and aac(6’)-Ib-cr1. The E. cloacae isolate also harboured qnrA1. These findings alert to the underestimated horizontal dissemination of multidrug-resistant plasmids limiting treatment options with last resort antimicrobials.

关键词： Interprofessional training ward   Patients' evauation   Parental evaluation   Medical education   Interprofessional learning   Interprofessional collaboration  

摘要： Background Interprofessional training wards (ITWs) have been established in different fields of adult medicine to promote interprofessional learning and interprofessional collaboration of health care profession students. High patient satisfaction rates have been reported for ITWs. No data of parents' and especially patients' evaluation of care on a paediatric ITW have been reported so far. This study aims to evaluate parents' and patients' perceptions of medical and nursing care on a paediatric ITW. Methods In 2017 we established and started an interprofessional training ward in the setting of a general paediatric ward (IPAPAED). Medical students and nurse trainees care for 4-6 patients under supervision of registered nurses and certified physicians. All parents and all patients older than 8 years were invited to evaluate different aspect of their care on the IPAPAED. Results Since November 2017 until February 2019 parents (n = 109) rated the overall care of their children on the IPAPAED ward with m = 1.21 (SD +/- .43) (1 = "excellent", 4 = "poor"). Patients (n = 56) rated their overall care with m = 1.29 (SD +/- 0.5). Other aspects of care and interprofessional collaboration were rated equally well. Analysis of the (limited) free-text commentaries revealed that perceived quality of care, friendliness and communication were especially valued by patients and parents. Discussion & conclusion On a paediatric ITW, in the view of parents and patients in our sample, a high level of care is delivered and satisfaction rates are excellent. An ITW seems, from a patient and parent point of view, feasible, even in paediatrics.