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摘要： Size mismatch between donor and recipient in solid organ transplantation can add significant morbidity and mortality to the recipient (1). However due to the scarcity of both deceased donors, as well as suitable living donors, discrepancies between the size and weight of the donor, compared with the recipient are often a necessity, if solid organ transplantation is to proceed in a particular individual.◆ (This article is protected by copyright. All rights reserved.)

关键词： aplastic anaemia   eltrombopag   childhood   immunosuppressive therapy  

关键词： adverse event   burnout   child   children's nursing   nurse   paediatric   professional well-being   quality of care   safety   safety culture   work environment  

摘要： Aim To explore Italian paediatric nurses' reported burnout and its relationship to their perceptions of safety and adverse events. Design A cross-sectional study using the RN4CAST@IT-Ped database with a web-based survey design. Methods The RN4CAST@IT-Ped questionnaire was used to collect data in 2017. This comprised three main components: three dimensions (22 items) of the Maslach Burnout Inventory including emotional exhaustion, depersonalization and personal accomplishment. Participants also scored an overall grade of patient safety and estimated the occurrence of adverse clinical events. Results Nurses (N = 2,243) reported high levels of burnout. Most rated clinical safety as high. The risk of adverse events ranged from 1.3-12.4%. The degree of burnout appeared to influence the perception of safety and adverse events. Conclusion The association between nurses' burnout and perceptions of higher rates of adverse events and reduced safety in clinical practice is an important finding. However, it is unclear whether this was influenced by a negative state of mind, and whether reduced safety and increased adverse events negatively influenced nurses' well-being, thus leading to burnout. Regardless, the association between nurses' burnout and these quality concepts needs further exploration to examine the effect, if any, on burnout and safety, and identify supportive mechanisms for nurses. Impact The association between reported burnout and perception of safety and risk of adverse events in Italian paediatric nurses has been reported for the first time. Nurses reporting burnout are at greater risk of intensely negative perceptions of clinical safety and adverse events. This is an important finding as perceptions can influence practice and behaviours. Quality measures in children's clinical environments need to go beyond obvious indicators to examine nurses' well-being as this also influences quality and safety.

关键词： autoimmune hepatitis   paediatric   children  

摘要： Objectives: To describe the clinical characteristics, biochemical and histological features, outcomes and predictors of prognosis of children with autoimmune hepatitis (AIH) from a paediatric centre in South Africa. Methods: Thirty-nine children diagnosed with AIH at Red Cross War Memorial Children's Hospital between 2005 and 2015 were included. Relevant patient's data were retrieved from the hospital's medical records and database. Liver biopsy slides were reviewed. Ethical approval was obtained. Data were analysed using SPSS. Results: Females were 29 (74%). Mean age at presentation was 7.2763.35 years and the mean follow-up was 4.5 +/- 2.4 years. Jaundice was present in 97% of patients at presentation. An acute presentation was observed in 26 (67%) even though cirrhosis was detected in 22 (56%). Autoantibody screening was completed in 35 patients, 20 (57%) were AIH-1, 1 (3%) was AIH-2 and 14 (40%) were seronegative AIH. Of the 25 patients who underwent magnetic resonance cholangiography 17 (68%) had associated autoimmune sclerosing cholangitis. The remission rate was 79%. However, 11 children relapsed later. One child required liver transplantation and one demised. Seronegative and seropositive patients have comparable characteristics and outcomes. While a higher alanine transaminase (ALT) level at presentation is a significant predictor of remission, a lower ALT level and cirrhosis are significant risk factors for unfavourable outcome. Overall survival rate was 97%. Conclusion: AIH responds well to therapy with excellent survival. Hence, it should be considered in any child presenting with viral screen negative hepatitis and start therapy timeously to prevent disease progression.

关键词： drug interactions   amphotericin b   systemic mycosis   hematopoietic stem cell transplantation   azoles   child   hematologic neoplasms   pediatrics   safety   infections   cytomegalovirus   mortality   engraftment   toxic effect   risk reduction   coinfection   allogeneic hematopoietic stem cell transplant   mold   antifungal prophylaxis   candida   adverse event   amphotericin b liposomal  

摘要： Background: Primary antifungal prophylaxis in paediatric allogeneic HSCT recipients is mainly based on azoles, which can have related toxicity and drug interactions. Low-dose liposomal amphotericin B (L-AmB) is an attractive intravenous alternative because of its low toxicity and lower risk of interactions. Objectives: To evaluate the effectiveness and safety of L-AmB (1 mg/kg/day) for primary antifungal prophylaxis in pre-engraftment paediatric HSCT patients. Patients and methods: Retrospective, observational study including all consecutive patients aged <= 18 years who underwent HSCT and received antifungal prophylaxis with intravenous L-AmB (1 mg/kg/day, from day #1 to 48 h before discharge) between January 2012 and December 2016. Results: In total, 125 HSCT procedures in 118 patients were included, median age 7.2 years (IQR 4.2-11.5). Haematological malignancies were the main underlying condition (63.6%), and 109 (87.2%) were considered at high risk for invasive fungal infection (IFI). Ten patients (7.7%), all high risk, developed breakthrough IFI (three Candida spp., seven invasive mould infections) and tended to have higher overall mortality. The only statistically significant risk factor for IFI was cytomegalovirus co-infection. Adverse events, all grade I, occurred in 25 (20%), requiring L-AmB withdrawal in one case. Overall survival at 30 days was 99.2%. At study completion, one patient had died of IFI. Conclusions: The incidence of breakthrough IFI was comparable to that of previous reports, with a very low rate of significant toxicity. Thus, prophylactic L-AmB may be a safe, effective option for antifungal prophylaxis in the pre-engraftment phase for children undergoing HSCT, even those at high risk.

关键词： Pedi-IKDC   Pedi-FABS   Pediatric ACL   PAMI   Italian validation  

摘要： Purpose The aim of the present study was to translate, cross-culturally adapt, and assess the psychometric properties of the Pedi-IKDC and Pedi-FABS scores in the Italian paediatric population with various knee pathologies. Methods In accordance with the Paediatric Anterior Cruciate Ligament Monitoring Initiative (PAMI) research protocol, the original English versions of the questionnaires were translated into Italian. All patients aged 8-16 and scheduled for knee surgery were considered eligible in the study. An open-source platform was implemented to collect responses to the surveys which included general patient information, the questionnaires Pedi-IKDC, and Pedi-FABS. Two surveys were sent under stable clinical conditions before surgery (Q(1) and Q(2));a third survey was sent 3-4 months after surgery (Q(3)). The following properties were calculated: reliability, internal consistency, criterion validity, responsiveness, and floor/ceiling effects. Results Eighty-nine patients completed Q(1), 81 patients completed Q(2), and 49 patients completed Q(3). Both questionnaires demonstrated acceptable properties. Pedi-IKDC: standard error of measurement (SEM) = 4.4, smallest detectable change (SDC) = 12.3, interclass correlation coefficient (ICC) = 0.96, Cronbach alpha (alpha) = 0.92, moderate-to-low correlation to Pedi-FABS, effect size (ES) = 0.79, standardized response mean (SRM) = 0.86, floor = 0%, ceiling = 22%. Pedi-FABS: SEM = 2.1, SDC = 5.8, ICC = 0.94, Cronback alpha (alpha) = 0.93, moderate-to-low correlation to Pedi-IKDK, ES = 0.60, SRM = 0.51, floor = 19%, ceiling = 0%. Conclusions The Italian version of Pedi-IKDC and Pedi-FABS is valuable tools for patient assessment, by demonstrating good psychometric properties. In clinical setting, these questionnaires can be used to properly evaluate outcomes in Italian pediatric patients with knee pathologies.

关键词： Paediatric burns   parental perceptions   qualitative research   injury prevention   Malawi  

摘要： Background: Burn injuries disproportionally affect the world's poorest populations. However, there has been a lack of research that has investigated the social, cultural or contextual factors associated with this injury mechanism in these areas. As a result, there is a scarcity of information from which to develop culturally appropriate and targeted burn prevention initiatives. Methods: A community survey was used to identify households to take part in this qualitative study. Semi-structured interviews were conducted with 32 parents from four different villages in Malawi to discuss their experiences of burn injuries sustained by children in the household as well as any existing preventative strategies used. In addition, 29 of the study households took part in an observation exercise to identify and discuss burn hazards present around the home environment. Transcripts and observations were recorded and transcribed verbatim. A thematic approach was used to analyse the data. Results: The final themes could be categorised into three key areas: parental perception of hazards around the home;socio-cultural and developmental factors associated with burn injuries;and parental perception of burns prevention. Factors associated with unintentional burn injuries, as perceived by parents, included: increased exposure to hazards, children's play and development activities, increased fire use during the morning hours and the children's household roles and responsibilities. Although some parents identified possible burns prevention strategies numerous barriers to prevention were highlighted including: poverty, poor housing infrastructure and restrictions, knowledge deficit, inability to provide adequate supervision and the child's behaviour. Conclusions: Parents recognise that there are a number of burn hazards and risks present around the home however factors that prevented them acting included: a lack of knowledge about injury prevention, a lack of household safety equipment, a l

关键词： Trauma   Injury severity, 30-day mortality   Child  

摘要： Background: Adult injury severity metrics are often applied to paediatric populations despite differences in anatomy, physiological and mortality risk. Measures to assess paediatric mortality have not been conducted on a population-wide basis. Purpose: To determine the predictive ability of the International Classification of Injury Severity Score (ICISS) in assessing 30-day mortality in a paediatric population, and to examine temporal trends for serious injury for common paediatric injury mechanisms. Methods: A retrospective analysis of linked injury hospitalisation and mortality data of children aged <= 16 years during 1 July 2002 to 30 June 2012 in Australia was conducted. Both multiplicative-injury and single-worst-injury ICISS were calculated. Logistic regression examined 30-day mortality with a range of predictor variables. The models were assessed in terms of their ability to discriminate survivors and non-survivors, model fit, and ability to explain outcome variance. Results: There were 728,584 index injury admissions and 1,064 (0.15%) deaths within 30-days of hospital admission. The multiplicative-injury ICISS was identified as a better predictor of 30-day mortality than the single worst-injury ICISS;and the best model included age group, gender, all comorbidities, trauma centre type, injury mechanism, and nature of injury as covariates. Temporal trends for serious injury have remained relatively constant over the 10-year period. Examination of specific injury mechanisms showed a significant decline in road trauma and drowning hospitalisations. In comparison, hospitalisations due to fall and self-harm injuries within adolescents increased. Conclusion: ICISS was shown to be excellent indicator for predicting 30-day mortality for all paediatric hospital admissions within a national jurisdiction. The rate of paediatric hospitalisation due to all-cause serious injury has not changed over a 10-year period despite being a national public health area of high prior

关键词： inborn errors of metabolism   paediatric liver disease   urea cycle disorder  

摘要： Ornithine transcarbamylase deficiency (OTCD) is the most common of the urea cycle disorders and follows an X-linked inheritance pattern. The classical form in male infants causes vomiting and lethargy in the neonatal period;if untreated the severe hyperammonaemia can cause acute neurotoxic complications and permanent disability. OTCD may also occur in heterozygote female individuals, though the manifestations are variable. We report 2 cases of female paediatric patients with OTCD, who presented with acute liver failure. Both patients had limited oral intake at the time of presentation, causing an absence of orotic aciduria, which delayed the diagnosis. These cases demonstrate the need to consider urea cycle disorders in children presenting with acute liver failure, and that repeating the urine metabolic screen at the time of an unrestricted diet is warranted if there is a high clinical suspicion.

关键词： Paediatric cardiac surgery   Complication prediction   Machine learning