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关键词： Neuroblastoma   children   proton therapy   pencil beam scanning   volumetric scanning   motion mitigation strategy  

摘要： Aims: More efforts are required to minimise late radiation side-effects for paediatric patients. Pencil beam scanning proton beam therapy (PBS-PT) allows increased sparing of normal tissues while maintaining conformality, but is prone to dose degradation from interplay effects due to respiratory motion. We report our clinical experience of motion mitigation with volumetric rescanning (vRSC) and outcomes of children with neuroblastoma. Materials and methods: Nineteen patients with high-risk (n = 16) and intermediate-risk (n = 3) neuroblastoma received PBS-PT. The median age at PBS-PT was 3.5 years (range 1.2-8.6) and the median PBS-PT dose was 21 Gy (relative biological effectiveness). Most children (89%) were treated under general anaesthesia. Seven patients (37%) underwent four-dimensional computed tomography for motion assessment and were treated with vRSC for motion mitigation. Results: The mean result of maximum organ motion was 2.7 mm (cranial-caudal), 1.2 mm (left-right), 1.0 mm (anterior-posterior). Four anaesthetised children (21%) showing <5 mm motion had four-dimensional dose calculations (4DDC) to guide the number of vRSC. The mean deterioration or improvement to the planning target volume covered by 95% of the prescribed dose compared with static three-dimensional plans were: 4DDC no vRSC, -0.6%;2 vRSC, +0.3%;4 vRSC, +0.3%;and 8 vRSC, +0.1%. With a median follow-up of 14.9 months (range 2.7-49.0) there were no local recurrences. The 2-year overall survival was 94% and distant progression-free survival was 76%. Acute grade 2-4 toxicity was 11%. During the limited follow-up time, no late toxicities were observed. Conclusions: The early outcomes of mainly high-risk patients with neuroblastoma treated with PBS-PT were excellent. With a subset of our cohort undergoing PBS-PT with vRSC we have shown that it is logistically feasible and safe. The clinical relevance of vRSC is debatable in anaesthetised children with small pre-PBS-PT motion of <5 mm. (C) 2020 Th

关键词： Pediatrics   Arterial catheter   Intensive care   Clinical audit  

摘要： Introduction: Peripheral arterial catheters (PAC) are used for haemodynamic monitoring and blood sampling in paediatric critical care. Limited data are available regarding PAC insertion and management practices, and how they relate to device function and failure. This information is necessary to inform future interventional research. Objectives: The primary objective of this study was to describe PAC insertion and management practices, and associated complications. Secondary objectives were to determine patient and clinical characteristics associated with risk of PAC successful insertion and failure. Methods: A prospective, observational study was conducted in the anaesthetic department and paediatric intensive care unit of a tertiary paediatric facility. Data were collected on PAC insertion, PAC management and PAC removal. Standard incidence and prevalence were calculated per 1,000 device days. Risk factors for multiple insertions and PAC failure were identified using Cox regression. Results: A total of 100 catheters in 89 children were examined capturing 472 device days. PACs were primarily inserted for blood sampling (78%) in the radial artery (78%) using ultrasound guidance (67%), with 31% inserted on first attempt. Heparin saline solution was used in 82% of devices. Median catheter dwell was 50.6 hours (IQR 24.0 - 158.0), with PAC failure occurring in 19 devices (20%), at a rate of 40.2 per 1000 catheter days (95% CI 25.7 - 63.1). Arm board immobilisation (HR 2.9;95% CI 1.02-8.02;p = 0.05), higher PIM3 score (HR 1.06;95% CI 1.03-1.09;p < 0.01) was associated with an increased the risk of PAC failure, and non-2% chlorhexidine antisepsis was associated with a decrease in PAC failure (HR 0.32;95% CI 0.11-0.96;p = 0.04), in univariate analysis. Conclusions: PAC insertion is challenging, and failure is common. Prospective clinical trial data is needed to identify high risk patient groups and to develop interventions which optimise practices, thereby reducing failure

关键词： Chronic Mucocutaneous Candidiasis   STAT1-GOF mutation   candida   paediatric primary immunodeficiency  

摘要： Chronic mucocutaneous candidiasis is a rare disorder that is characterised by chronic or recurrent superficial candida infection of skin, nails, and mucous membranes. We describe such a case in a young boy who failed to respond to conventional therapy. It highlights the important role of the dental team in assessment, treatment, and onwards referral. Crown Copyright (C) 2020 Published by Elsevier Ltd on behalf of The British Association of Oral and Maxillofacial Surgeons. All rights reserved.

关键词： Paediatric   Children   Fracture   Frequency   Incidence   Epidemiology  

摘要： Introduction Recent studies investigating the frequency of paediatric fractures in Germany are not available. The primary aim of this study was to report frequencies of the most common paediatric upper extremity fractures treated in German hospitals in 2002 and 2017 and to detect changes over time. Methods This study used inpatient data from the German National Hospital Discharge Registry. Absolute frequencies and incidences of the following fracture localisations were analysed: clavicle, proximal humerus, humerus shaft, distal humerus, ulna shaft, radius shaft, forearm shaft, distal radius, and distal forearm. Four age groups were formed: 0-4, 5-9, 10-14, and 15-19 years. The boy-girl ratio (BGR) was calculated for all fracture localisations for both years, respectively. Incidence rate ratios (IRRs) were calculated to compare fracture incidences between 2002 and 2017. Results The absolute number of the nine fracture localisations together decreased from 38,480 in 2002 to 35,128 in 2017. The overall BGR was 2.0 in both years. The BGR increased with increasing patient age. The incidence of clavicle fractures increased from 2002 to 2017 (IRR >= 1.72), while that of humerus fractures (proximal, shaft, and distal) remained the same or decreased (IRR <= 1.00) within all age groups. The incidence of isolated ulna or radius shaft fractures increased slightly or remained the same in the two lower age groups (IRR >= 1.00), while it decreased in the two higher age groups (IRR <= 0.80). Furthermore, complete forearm fractures were more frequent in the 0-4, 5-9, and 10-14 year age groups (IRR >= 1.44) in 2017 compared to 2002. The incidence of distal radius und forearm fractures changed only slightly. Conclusions The absolute number of paediatric upper extremity fractures decreased from 2002 to 2017, while the incidence of in-hospital treatment of clavicle and forearm fractures increased significantly, indicating a trend towards operative treatment.

关键词： Hypopituitarism   Sellar   Children   Relapse   Progression  

摘要： Purpose Craniopharyngioma is one of the most frequent benign tumours of the central nervous system in the paediatric population. Although it is a benign tumour according to the WHO classification, it significantly deteriorates the patient's quality of life. The aim of this study is to assess if proliferation index Ki67 can be a useful marker of the risk of craniopharyngioma's recurrence. Methods Expression of Ki67 was examined in 85 specimens of primary craniopharyngioma and in 11 specimens of the recurring tumour. In all the cases, adamantinomatous type of craniopharyngioma was diagnosed. Values of Ki67 expression were compared between patients with and without recurrence, between patients with progression and relapse and between primary and recurrent tumours. Results No statistically significant differences were found between proliferation index Ki67 values in tumours with recurrence and without (median values 2.5% and 3%, respectively, p = 0.69). The median value of proliferation index Ki67 in progression group was 1% and in the relapse group 4%;no statistical significance between those groups was found (p = 0.067). The median value of proliferation index Ki67 in primary tumours was 3% (0-20%) and in recurrent tumours it was 5% (0-14%). Despite the lack of statistical significance (p = 0.61), a tendency towards higher values of Ki67 in recurring tumours in comparison with primary tumours was shown. Conclusions Proliferation index Ki67 is not a reliable prognostic factor of craniopharyngioma's recurrence.

关键词： HIV   sickle cell disease   Uganda   Africa   CD4   paediatric  

摘要： Objectives HIV and sickle cell disease (SCD) are significant causes of morbidity and mortality in sub-Saharan Africa. Given their separate roles in immune dysregulation, our objective was to characterise the impact that SCD has on the presentation and progression of paediatric HIV. Methods The study was a retrospective cohort study (study period 2004-2018). Cases of HIV + and SCD-afflicted patients (HIV+/SCD+) were obtained via electronic chart review from a paediatric HIV clinic in Kampala, Uganda and matched 1:3 with HIV + controls without SCD (HIV+/SCD-). Results Thirty-five HIV+/SCD + subjects and 95 HIV+/SCD- controls were analysed (39% female (51/130), age 3.6 years (SD3.9)). At baseline, WHO clinical stage (64% total cohort Stage III/IV) and nutritional status (9.4% severe acute malnutrition) were similar for both groups, whereas HIV+/SCD + had higher though non-significant baseline CD4 count (1036 (SD713) vs 849 (SD638) cells/microlitre, P = 0.20, two-tailed t-test). There were 19 deaths, 6 (17%) HIV+/SCD + and 13 (14%) HIV+/SCD-, with unadjusted/adjusted models showing no significant difference. Nutritional progression and clinical stage progression showed no significant differences between groups. Kaplan-Meier analysis showed a slower rate of treatment failures in the HIV+/SCD + cohort (P = 0.11, log-rank survival test). Trajectory analysis showed that in the time period analysed, the HIV+/SCD + cohort showed a more rapid rise and higher total CD4 count (P = 0.012, regression analysis). Conclusion The study suggests that SCD does not adversely affect the progression of HIV in patients on ART. Further, HIV+/SCD + achieved higher CD4 counts and fewer HIV treatment failures, suggesting physiological effects due to SCD might mitigate HIV progression.

关键词： bronchiolitis   critical care   intensive care unit   length of stay   paediatric   patient discharge  

摘要： Aim To examine the patient and hospital admission characteristics associated with direct discharge home from paediatric intensive care. Methods This was a single-centre retrospective analysis of all admissions to a tertiary metropolitan general paediatric intensive care unit (ICU) surviving to discharge over a 10-year period between 1 January 2007 and 31 December 2016, divided into two epochs defined by changes in health service structure. Patient and admission characteristics were compared between groups discharged direct to home and discharged to ward across these two epochs. Results There was a marked increase in the annual rate of direct discharge to home from ICU between the two epochs (3.7-9.5%, P < 0.0001). There was an inverse relationship between monthly ICU activity and rates of direct discharge to home. Patients discharged directly home were significantly more likely to experience delay to discharge (46.4 vs. 30.7%, P < 0.0001), for that delay to exceed 24 h and comprise a greater proportion of total ICU length of stay. Bronchiolitis accounted for an increasing proportion of admissions between epochs (7.0-15.1%) and was over-represented among patients discharged direct to home (up to 18.2%). Conclusions The high observed rate of direct discharge home is likely to have resulted from increased delays to discharge and changes to patient admission characteristics, attributable to organisational restructuring and possibly changing approaches to clinical management of bronchiolitis. It is imperative to now consider how we ensure that our systems support the proper use of intensive care resources.

关键词： COVID-19   Coronavirus   Paediatric   Radiotherapy   Treatment   Resources  

摘要： Objective: To derive evidence-based recommendations for the optimal utilisation of resources during unexpected shortage of radiotherapy capacity. Methods and materials: We have undertaken a rapid review of published literature on the role of radiotherapy in the multimodality treatment of paediatric cancers governing the European practise of paediatric radiotherapy. The derived data has been discussed with expert paediatric radiation oncologists to derive a hierarchy of recommendations. Results: The general recommendations to mitigate the potential detriment of an unexpected shortage of radiotherapy facilities include: (1) maintain current standards of care as long as possible (2) refer to another specialist paediatric radiotherapy department with similar level of expertise (3) prioritise use of existing radiotherapy resources to treat patients with tumours where radiotherapy has the most effect on clinical outcome (4) use chemotherapy to defer the start of radiotherapy where timing of radiotherapy is not expected to be detrimental (5) active surveillance for low-grade tumours if appropriate and (6) consider iso-effective hypofractionated radiotherapy regimens only for selected patients with predicted poor prognosis. The effectiveness of radiotherapy and recommendations for prioritisation of its use for common and challenging paediatric tumours are discussed. Conclusion: This review provides evidence-based treatment recommendations during unexpected shortage of paediatric radiotherapy facilities. It has wider applications for the optimal utilisation of facilities, to improve clinical outcome in low- and middle-income countries, where limited resources continue to be a challenge. (C)20 Elsevier B.V. All rights reserved.