教学课程资源库 更多>>

关键词： Osteomyelitis   Paediatric   Polymerase chain reaction   Septic arthritis  

摘要： Background Culture yield in osteomyelitis and septic arthritis is low, emphasising the role for molecular techniques. Aims The purpose of this study was to review the laboratory investigation of childhood osteomyelitis and septic arthritis. Methods A retrospective review was undertaken in an acute tertiary referral paediatric hospital from January 2010 to December 2016. Cases were only included if they had a positive culture or bacterial PCR result from a bone/joint specimen or blood culture, or had radiographic evidence of osteomyelitis. Results Seventy-eight patients met the case definition;52 (66%) were male. The median age was 4.8 years. Blood cultures were positive in 16 of 56 cases (29%), with 11 deemed clinically significant (Staphylococcus aureus = 8, group A Streptococcus = 3). Thirty-seven of 78 (47%) bone/joint samples were positive by culture with S. aureus (n = 16), coagulase-negative Staphylococcus (n = 9) and group A Streptococcus (n = 4), being the most common organisms. Sixteen culture-negative samples were sent for bacterial PCR, and four were positive (Kingella kingae = 2, Streptococcus pneumoniae = 1, group A Streptococcus = 1). Conclusions Sequential culture and PCR testing can improve the detection rate of causative organisms in paediatric bone and joint infections, particularly for fastidious microorganisms such as K. kingae. PCR testing can be reserved for cases where culture is negative after 48 h. These results have been used to develop a standardised diagnostic test panel for bone and joint infections at our institution.

关键词： Paediatric multiple sclerosis   Juvenile multiple sclerosis   Cognition   Neuropsychology   Treatment   Disease-modifying drugs  

摘要： Background: Cognitive impairment (CI) is a critical feature for patients with childhood or juvenile multiple sclerosis (MS). Objective: To promote the understanding of CI and to address the impact of different pharmacological treatment strategies on cognitive performance in this patient group. Methods: A cohort of 19 patients with therapy-naive or beta-Interferon-treated juvenile MS completed a comprehensive neuropsychological assessment at initial presentation (baseline) and on average 2.5 years later (follow-up). The assessments were complemented with a neuropaediatric examination and conventional cerebral magnetic resonance imaging (MRI). Results: 9 patients (47%) were impaired in at least one test at baseline (z-score <-1.645 compared with age-adjusted normative data), with the highest impairment frequency in the domains processing speed and attention & executive functions. At follow-up a higher impairment frequency was prominent in those patients whose therapy had not been escalated (N = 13, 69% impaired in at least one test), while cognition was preserved or ameliorated in patients whose treatment had been escalated to highly effective drugs (N = 6, 0% impaired) during the observational period. These group differences at follow-up were not attributable to differences regarding demographics, MRI metrics or cognitive performance at baseline. Conclusion: Our findings confirm that paediatric MS is associated with considerable CI already in early disease stages. Early administration of highly effective treatment may protect from cognitive decline or alleviate CI in juvenile MS, but larger controlled trials are warranted to confirm these preliminary results. (C) 2019 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

关键词： Lumbosacral lipoma   Magnetic resonance imaging   Spinal dysraphism   Paediatric neurosurgery  

摘要： Purpose Lumbosacral lipoma (LSL) is a severe occult spinal dysraphism, frequently associated with neurological, urological and orthopaedic complications. Whole spine imaging is typically performed to identify concomitant, but spatially separate, congenital anomalies. Our hypothesis: the incidence of additional, clinically significant abnormalities of the neuraxis is low;thus, imaging should be optimised at the lumbosacral region. We aim to assess the prevalence and relevance of LSL-associated lesions. Method A single-centre, retrospective, radiological review using a prospectively maintained operative database. Inclusion criteria: children (< 16 years) with confirmed diagnosis of LSL and received whole spine MRI. Fatty filum, syndromic cases and cutaneous stigmata above lesion level were excluded. Data was extracted from radiological imaging, reports and clinical correspondence. Results One hundred twelve patients (40:72, M:F) aged 0.5 years (0.2-2.7) (median +/- IQR) with LSL had whole spine MRI between 2001 and 2017. Classification of LSL: transitional 48 (43%);dorsal 30 (27%);caudal 28 (25%) and chaotic 6 (5%). Additional anomalies included syringohydromyelia 44 (39%), subcutaneous tract 19 (17%), abnormal vertebral segmentation 18 (16%), dermoid cyst 1 and 1 Chiari I deformity. There were no Chiari II malformations. No child required surgery for an associated lesion. Binary logistic regression revealed no factors associated with predicting secondary lesions. Conclusions In congenital LSL, additional anomalies of the neuraxis are typically loco-regional rather than pan-CNS and additional lesions are rarely clinically significant. The loco-regional distribution of anomalies suggests that only lumbosacral spinal imaging is required in the initial evaluation of LSL. Such a policy would lessen the anaesthetic/sedation time for children and reduce imaging cost per patient. MRI protocols could be refined to optimise imaging quality at the region of interest.

关键词： Cow's milk allergy   Diet   Children   Pregnancy  

摘要： Background: The key role of dietary factors in immunotolerance promotion and allergic diseases prevention has been emphasised. The aim of the study was the analysis of the impact of immunomodulatory dietary components, consumed by pregnant women, on the development of cow's milk allergy (CMA) in their offspring. Materials and methods: Fifty-one pairs of mothers and their CMA-offspring were included in the study group. The analysis of a daily intake of selected dietary components was conducted retrospectively with the application of a seven-day diet of a mother in the third trimester of gestation and the authors' own questionnaire. The Diet 5.D programme was used. Results: An average daily retinol intake by study-group mothers was significantly lower than by control-group mothers and valued 375.6 mu g/d vs. 543.7 mu g/d (p = 0.040), respectively. Folates intake in the study group was 598.8 mu g/d vs. 361.1 mu g/d in the control group (p = 0.001). Vitamin Din the study group was statistically lower - 3.6 mu g/d, comparing to the control group - 6.9 mu g/d (p = 0.038). Average LC-PUFA intake by mothers with allergic children was 0.09 g/d, while in the control group 0.18 g/d (p = 0.016). An analysis of the diet revealed that significantly more mothers of children from the control group (n = 12;48%) consumed fish 2-3 times per month in comparison to the study group (n = 9;17.6%) (p = 0.007). Conclusions: Vitamin D, A, LC-PUFA, retinal, riboflavin and fish consumption by pregnant mothers of CMA-children was significantly lower, whereas beta-carotene and folates consumption was significantly higher than that of mothers with non-allergic children. (C) 2019 Published by Elsevier Espana, S.L.U. on behalf of SEICAP.

关键词： acute myeloid leukaemia   CD19  

摘要： We performed a retrospective analysis of leukaemic surface antigen expression and genomic data from a total of 100 RUNX1-RUNX1T1-positive paediatric acute myeloid leukaemia (AML) patients enrolled in the Japanese Paediatric Leukaemia/Lymphoma Study Group (JPLSG) AML-05 protocol to determine risk factors for relapse. In univariate analysis, the KIT exon 17 mutation (n = 21) and CD19 negativity (n = 59) were significant risk factors for relapse (P = 0 center dot 01). In multivariate analysis, CD19 negativity was the sole significant risk factor for relapse (hazard ratio, 3 center dot 09;95% confidence interval, 1 center dot 26-7 center dot 59;P < 0 center dot 01), suggesting that biological differences between CD19-positive and CD19-negative RUNX1-RUNX1T1 AML patients should be investigated.

关键词： anaphylaxis   ATR   children   FNHTR   occurrence pattern  

摘要： Background and objectives Like adults, children can have allergic transfusion reactions (ATRs) and febrile non-haemolytic transfusion reactions (FNHTRs). But published information about the incidence of paediatric ATR and FNHTR is scarce. Materials and methods This retrospective study was conducted from April 2002 to June 2018 on children who had ATRs and/or FNHTRs to platelet (PLT), red blood cell (RBC) or washed PLT/RBC concentrate transfusions. We analysed ATR/FNHTR clinical presentations, such as severity, time of occurrence and other features when they occurred. Results During the study, 2742 children received 23 444 bags of PLT and RBC concentrate (including washed products). ATRs occurred in 100 cases (3 center dot 6% of total patients), caused by 201 products (0 center dot 9% of total products). In contrast, 28 patients (1 center dot 0% of total patients) had 42 FNHTRs caused by 42 products (0 center dot 2% of total products). Upon analysis of cases with detailed clinical information, the median onset time for ATRs and FNHTRs was 2 center dot 0 h after the start of transfusion. Of the 40% of ATRs that necessitated the discontinuation of blood transfusions, 10% escalated to anaphylaxis. Compared with minor ATRs, anaphylaxis tended to develop quickly. Moreover, 96% of patients with FNHTRs had a fever less than 39 degrees C. There were no associations between blood product types and numbers or occurrence patterns of these reactions. Conclusion The occurrence of ATRs and FNHTRs in children was variable, although there are common features such as severity and time of occurrence.

关键词： Crohn disease   epidemiology   inflammatory bowel disease   paediatric gastroenterology  

摘要： Objectives:Stricturing duodenal Crohn disease (CD) is a rare but serious presentation of CD causing significant morbidity. We aim to provide the first robust incidence data and case studies on this severe presentation in ***:A regional cohort of prospectively acquired incident cases of paediatric CD diagnosed <16 years of age in South-East Scotland was captured over a 19-year period (1999-2018). A retrospective review was conducted on the medical records of all patients together with a review of the available literature and consensus guidelines. Incidence rates for all CD and for duodenal stricturing CD were ***:A total of 247 new cases of paediatric CD were diagnosed within the study period. Median age at diagnosis was 12.5 years with 62% male predominance. Overall paediatric CD incidence rate was 5.70/100,000/year with a specific duodenal B2 phenotype disease incidence rate of 0.05/100,000/year;representing 0.8% of incident cases at diagnosis. Two incident cases of stricturing duodenal CD presented with systemic symptoms of weight loss, abdominal pain, anorexia, and lethargy, together with persistent vomiting suggestive of obstruction. Both cases partially responded to intensive medical therapy but eventually required laparoscopic gastroduodenostomy. A detailed literature search confirmed there are no paediatric incidence data, guidelines, or case reports relating to duodenal stricture as either a presentation or complication of ***:Duodenal structuring disease is a rare but serious presentation of CD causing significant morbidity and not currently covered in the paediatric literature or consensus guidelines. Best practice medical and surgical management remain uncertain and require further research.

关键词： computerized provider order entry system   evaluation research   hospital oncology services   medication errors   paediatrics   patient safety  

摘要： Objective To explore medication safety issues related to use of an electronic medication management system (EMM) in paediatric oncology practice, through the analysis of patient safety incident reports. Methods We analysed 827 voluntarily reported incidents relating to oncology patients that occurred over an 18-month period immediately following implementation of an EMM in a paediatric hospital in Australia. We identified medication-related and EMM-related incidents and carried out a content analysis to identify patterns. Results We found 79% (n = 651) of incidents were medication-related and, of these, 45% (n = 294) were EMM-related. Medication-related incidents included issues with: prescribing;dispensing;administration;patient transfers;missing chemotherapy protocols and information on current stage of patient treatment;coordination of chemotherapy administration;handling or storing medications;children or families handling medications. EMM-related incidents were classified into four groups: technical issues, issues with the user experience, unanticipated problems in EMM workflow, and missing safety features. Conclusions Incidents reflected difficulties with managing therapies rich in interdependencies. EMM, and especially its 'automaticity', contributed to these incidents. As EMM impacts on safety in such high-risk settings, it is essential that users are aware of and attend to EMM automatic behaviours and are equipped to troubleshoot them.

关键词： Non-invasive ventilation   Respiratory failure   Bronchiolitis   Paediatric intensive care units  

摘要： Introduction: Despite there being limited evidence, non-invasive ventilation (NIV) has become a common treatment for acute respiratory failure (ARF). The aim of this study was to identify the predictive factors of NIV failure, in order to enable early detection of patients failing the treatment. Patients and methods: Prospective cohort study was conducted that included all ARF patients that received NIV as the initial treatment between 2005 and 2009 in a fourteen-bed Paediatric Intensive Care Unit (PICU) of a tertiary university hospital. Information was collected about the NIV, as well as clinical data prior to NIV, at 2, 8, 12, and 24 hrs. The haemoglobin saturation (SpO2)/fraction of inspired oxygen (FiO2) ratio (S/F) was retrospectively calculated. NIV failure was defined as the need for intubation or requiring rescue with bi-level pressure (BLPAP). Univariate and multivariate statistical analyses were performed. Results: A total of 282 patients received non-invasive support, with 71 receiving Continuous Pressure (CPAP), and 211 with BLPAP treatment. The overall success rate was 71%. Patients receiving BLPAP vs. CPAP, patients with higher S/F ratios at 2 hours (odds ratio [OR] 0.991, 95% CI 0.986-0.996, P= .001], and patients older than 6 months (Hazard ratio [HZ] 0.375, 95% CI 0.171-0.820, P= .014], were also more likely to fail. Patients with higher heart rates (HR) at 2 hours (OR 1.021, 95% CI [1.008-1.034], P= .001) and higher inspiratory positive airway pressure (IPAP) at 2 hours were more prone to failure (HZ 1.214, 95% CI [1.046-1.408], P= .011). Conclusions: Age below 6 months, S/F ratio, HR, and IPAP at 2 hours are independent predictive factors for initial NIV failure in paediatric patients with ARF admitted to the PICU. (C) 2019 Asociacion Espanola de Pediatria. Published by Elsevier Espana, S.L.U.

关键词： hyponatraemia   length of stay   outcomes   paediatric anaesthesia   perioperative mortality  

摘要： Background: Preoperative hyponatraemia is an independent risk factor for postoperative mortality in adults. To our knowledge, this has not been investigated in children. Methods: Using data from the 2014 and 2015 data sets of the American College of Surgeons National Surgical Quality Improvement Program-Pediatric (NSQIP-P), we conducted a retrospective study of children undergoing surgery. The primary outcome was 30-day all-cause mortality. The secondary outcomes of interest were postoperative seizure within 30 days and prolonged length of stay. To identify any independent association between preoperative hyponatraemia, defined as mild (serum sodium of 131-135 mEq L-1) or severe (<= 130 mEq L-1), and death, postoperative seizures, or prolonged length of stay, multivariable logistic regression models were generated. Results: A total of 152 894 patients were identified, and of these 35 291 were included in the final analysis. Preoperative hyponatraemia was present in 5422 patients or 15.4% of the final cohort. There were 432 (0.80%) deaths at 30 days. Compared with patients with a normal preoperative sodium concentration, those with mild (P=0.003;odds ratio [OR]: 1.59;95% confidence interval [CI]: 1.17-2.18) and severe (P=0.002;OR: 2.16;95% CI: 1.32-3.54) hyponatraemia had increased rates of death, after adjusting for co-morbidity and procedural complexity. Both mild (P<0.001;OR: 1.53;95% CI: 1.42-1.65) and severe (P<0.001;OR: 1.51;95% CI: 1.19-1.93) hyponatraemia were independently associated with prolonged length of stay, after adjusting for relevant co-variates. Conclusions: This retrospective analysis identified an association between preoperative hyponatraemia and perioperative mortality and length of stay in paediatric patients.