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关键词： Paediatrics   CNS   Radiotherapy   Temporal lobe   Cognitive function  

摘要： Background and purpose: Reducing radiation exposure to the temporal lobes could be beneficial to preserve cognitive function in paediatric brain tumour patients. The distribution of doses to brain substructures associated with cognition (BSCs) both within and outside of the temporal lobe have not been reported. The aim of this study was therefore to investigate temporal lobe sparing photon vs. proton therapy for paediatric suprasellar tumours. Material and methods: Data from ten anonymized craniopharyngioma patients were used in this study. Temporal lobe sparing volumetric modulated arc therapy (VMAT) and pencil beam scanning (PBS) proton therapy plans were optimized to maintain consistent target metrics as in the delivered double scattering proton therapy (DSPT) plans. Thirty BSCs were delineated, including temporal lobe substructures (i.e. amygdala, hippocampus, entorhinal cortex). The dose/volume fractions to each BSC were analysed, and intelligence quotient (IQ) as well as memory scores were estimated to compare the different modalities. Results: The exposed volumes of the temporal lobes and their substructures were consistently reduced with PBS compared to DSPT and VMAT, e.g. the left hippocampus V10Gy from 100% (VMAT) or 41% (DSPT) to 5% with PBS (p = 0.002). Some of the ventricular substructures were better spared with VMAT compared to both proton modalities. The reduced doses to the temporal lobes achieved with PBS translated into improved predicted memory outcomes, but not for the estimated IQ. Conclusion: The irradiated volumes of temporal lobe BSCs were consistently the lowest with PBS, whereas the model-based estimates of cognitive outcomes were less consistent. (C) 2019 Elsevier B.V. All rights reserved.

关键词： Echocardiography   children   valvular disease  

摘要： We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography;however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.

摘要： Blue rubber bleb naevus syndrome (BRBNS) is an extremely rare venous malformation that often manifests as multiple haemangioma-like lesions in the skin and gastrointestinal tract. The drug sirolimus plays a key role in the signalling pathway of angiogenesis and subsequent development of BRBNS and its use has been described in several case reports. We present a case series of four patients with BRBNS who exhibited good treatment response to sirolimus. All four patients were administered oral sirolimus at doses of 1.0-1.5 mg/m(2)/day with a target drug level of 5-10 ng/mL and median treatment duration of 20 months. All patients had a reduction in the size of the lesions and a normalization of coagulopathy with tolerable drug adverse reactions at follow-up. Sirolimus may be effective and safe in paediatric patients with BRBNS. Further prospective studies are suggested to evaluate the long-term effectiveness of this drug.

关键词： Coronary fistula   Coronary bypass   Cardiac transplant   Children  

摘要： We report an asymptomatic 5-year-old boy with coronary artery fistulas to pulmonary parenchyma and to systemic thoracic arteries, like a "natural bypass", detected during a coronary angiography performed 11 months after his heart transplant. One year later, a new coronary angiography showed no fistula. Some changes to immunosuppressive therapy during this time could be related to this evolution.

关键词： child diet   child obesity   dietary questionnaire   food behaviours   parental perception   parental weight  

摘要： Aim To assess the weight status and diets of a sample of paediatric outpatients, explore the relationship between the two and compare child weight status with parental perception of child weight and parents' self-reported weight. Methods Parents/carers of 1-12-year-olds attending paediatric outpatients at Flinders Medical Centre, South Australia, between October 2015 and May 2016 completed a demographic and validated age-based dietary questionnaire (toddlers (1-3 years), pre-schoolers (>3 to <5 years) or children (5-12 years)). Dietary risk scores (low/moderate/high/very high) were calculated for toddlers and pre-schoolers and diet quality and food behaviours scores for children. Body mass index (BMI) z-score and weight status were determined from measured height and weight. Results Of 114 toddlers, 115 pre-schoolers and 250 children, 65% were of a healthy weight, 10% underweight and 25% overweight or obese. Most (~80%) toddlers and pre-schoolers had diets classified as 'moderate' risk, and the diets (35-90%) and behaviours (90%) of most 5-12-year-olds fell short of the guidelines. There was no significant relationship between overall diet risk or quality and BMI z-score. Healthier food behaviours scores were inversely correlated with BMI z-scores (ss -0.061, 95% confidence interval (CI) -0.089, -0.033, P < 0.005). Parents' perception of child weight status was inaccurate. Parent's self-reported weight status was significantly associated with the BMI z-scores of toddlers (ss 0.301, 95% CI 0.189-1.174, P = 0.007) and pre-schoolers (ss 0.220, 95% CI 0.032-0.859, P = 0.035). Conclusions Poor diets and high rates of overweight/obesity highlight the need for screening within the paediatric outpatient setting. Parents' own weight status, and their inaccurate perception of their child's, should be considered future intervention targets for improving child and parent health.

摘要： Linked Article: Rongioletti. Br J Dermatol 2020; 182:29–38.

关键词： acute pain   paediatrics   perioperative care   recovery   tonsillectomy  

摘要： Aim Tonsillectomy procedures are a core element of paediatrics;however, perioperative management differs. This study aimed to describe tonsillectomy management, including the burden of pain, nausea and delayed recovery. Methods A prospective cohort study was undertaken through an audit of tonsillectomy perioperative practice and recovery and survey interviews with family members 7-14 days post-surgery. The study was undertaken at an Australian tertiary referral paediatric hospital between June and September 2016. Results The audit included 255 children undergoing tonsillectomy, with 127 family members interviewed. Most participants underwent adenotonsillectomy (n = 216;85%), with a primary diagnosis of obstructive sleep apnoea (n = 205;80%) and a mean age of 7 years (standard deviation;3.9). A variety of intra-operative pain relief and antiemetics was administered. Pain was present in 29% (n = 26) of participants at ward return, increasing to 32-45% at 4-20 h and decreasing to 21% (n = 15) at discharge. A third of the children (32%;n = 41) had moderate to severe pain at post-discharge interview, and 30% (n = 38) experienced nausea at home. Most parents (82%;n = 104) were still giving regular paracetamol at 7 days post-operatively, and 31% (n = 39) had finished their oxycodone. Of the participants, 14% (n = 26) presented to the emergency department within 7 days of discharge;8% (n = 20) of the total cohort were re-admitted. Conclusions There was variety in perioperative and post-discharge care. Pain scores were infrequently documented post-tonsillectomy, and parents are generally dissatisfied with the management of post-operative pain and nausea. Further research is needed to provide a more consistent approach to perioperative management to promote recovery.

关键词： annualized bleeding rate   Haemophilia A   immunogenicity   previously untreated patients   recombinant factor VIII   turoctocog alfa  

摘要： Introduction Turoctocog alfa is a recombinant, B domain-truncated factor VIII (FVIII) approved for patients with haemophilia A. Aim To evaluate the safety and efficacy of turoctocog alfa in previously untreated patients (PUPs) with severe haemophilia A. Methods Guardian 4 was a multicentre, multinational, non-randomized, open-label phase 3 trial comprising a main and extension phase. The former concluded once >= 50 patients had received treatment for >= 50 exposure days (EDs) or developed inhibitors. Patients received turoctocog alfa intravenously for prevention and treatment of bleeds. The primary endpoint was the incidence rate of FVIII inhibitors (>= 0.6 Bethesda Units) reported during the first 50 EDs. Results Of the 58 patients who completed the main phase, 25 (43.1%) patients developed inhibitors (detected within 6-24 [mean: 14.2] EDs from treatment start). High-risk mutations were identified in 60% of patients who developed inhibitors in the main phase and were a significant predictor of inhibitor development (P = .003). Of the 21 patients who started immune tolerance induction therapy, 85.7% completed treatment with a negative inhibitor test (note that data on the last 3 patients completing ITI are based on information collated from sites prior to the final database lock). Haemostatic response (including missing values as failure) was rated as 'excellent' or 'good' for 86.1% of bleeds occurring during prophylaxis. The estimated mean annualized bleeding rate for patients on prophylaxis was 4.26 bleeds/patient/year (95% CI: 3.34 - 5.44). Conclusions Turoctocog alfa was effective at preventing and stopping bleeds and was well tolerated. Inhibitor development was within the expected range for this PUP population.

关键词： Corrected count increment   Human leukocyte antigens   Platelet cross match   Platelet refractoriness   Paediatric Oncohaematology   Solid phase red cell adherent assay  

摘要： Immune platelet destruction is a significant cause for platelet refractoriness. The platelet crossmatch-a solid phase red cell adherence assay utilizes donor platelets and patient serum to assess compatibility and appears to be a feasible option in resource constrained settings. This study was done to evaluate the frequency of platelet crossmatch positivity among Paediatric Oncohaematology patients and also to assess whether a positive crossmatch is predictive of unsuccessful platelet transfusions in this group of patients. Paediatric Oncohaematology patients who received platelet transfusions between March 2013 and September 2013 were included in the study. The pre-transfusion patient sample and a segment from the transfused donor unit were used for performing the platelet crossmatch. A blood sample was collected one hour after the transfusion to assess post-transfusion platelet count. Corrected count increment (CCI) was calculated using the standard formula. CCI <= 7500/mu L/m(2)/10(11) was considered evidence of an unsuccessful transfusion. Seventy-three platelet crossmatches were performed for 69 patients, of which 30 patient samples (41%) showed crossmatch positivity. 25 (89.2%) of 28 unsuccessful transfusions showed crossmatch positivity, and 40 (88.9%) of 45 successful transfusions showed negative crossmatches (p = 0.03). Crossmatch positivity among transfusion dependent Paediatric Oncohaematology patients was as high as 42%, when ABO matched platelet units were allocated without further testing. Our results indicate that this test may be a reliable tool to select compatible platelet units and an effective intervention in the management of patients at risk of immune platelet refractoriness.

关键词： Paediatrics   Electronic early warning system   Simulation   Usability   Evidence-based care  

摘要： Background: The Bedside Paediatric Early Warning System (BedsidePEWS) is a clinical decision support tool designed to augment clinician expertise, objectively identify children at risk for clinical deterioration, and standardize and prioritize care to improve outcomes in community settings. Although the paper-based BedsidePEWS documentation record has been shown to improve clinicians' perception of their ability to detect deterioration and follow care recommendations, research is needed to asses this impact empirically. Furthermore, as hospitals progressively move toward electronic clinical systems, knowledge regarding the impact of BedsidePEWS' novel electronic interface on clinicians' performance and user experience is required. Objectives: The primary objectives of this study were (1) to compare adherence to evidence-based care recommendations using a) electronic health record software, b) paper BedsidePEWS, and c) a novel electronic BedsidePEWS interface, and (2) to describe end-users' experiences of usability and opportunities for improvement of both paper and electronic BedsidePEWS. Methods: Paediatric nurses participated in a repeated measures simulation study. Participants assessed simulated patients, documented patient data, and responded to a series of questions regarding follow-up care for each patient. Three patient types (i.e., stable, mild deterioration, severe deterioration) were assessed in each of three intervention conditions (i.e., electronic health record, paper BedsidePEWS, electronic BedsidePEWS). Following simulation scenarios, participants provided comments regarding the usability of the paper and electronic tools. Results: Participants made 12.7% and 18.0% more appropriate care decisions with paper and electronic BedsidePEWS, respectively, than with the electronic health record intervention (p < 0.001). Accurate BedsidePEWS severity of illness score calculation was related to better adherence to evidence-based care recommendations (65%), com