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关键词： RAAS   Angiotensin   Paediatric   Cardiovascular disease   Renal disease  

摘要： The renin-angiotensin-aldosterone system (RAAS) plays a major role in the regulation of blood pressure and homeostasis. Therefore, it is a commonly used target for pharmacotherapy of cardiovascular diseases in adults. However, the efficacy of this pharmacotherapy can only be limitedly derived into children. Comprehensive knowledge of the humoral parameters acting in the paediatric RAAS (e.g. angiotensin I, angiotensin II, angiotensin 1-7, angiotensin III, and angiotensin IV) might facilitate a more effective and rational pharmacotherapy in children. Therefore, this review aims to provide an overview of the maturing RAAS. Out of 925 identified records, 35 publications were classified as relevant. Physiological and pathophysiological concentrations of angiotensin peptides were compiled and categorised according to European Medicines Agency age groups. Age has a major impact on circulating angiotensin I, angiotensin II, and angiotensin 1-7, which is reflected in an age-dependent decrease during childhood. In contrast to data obtained in adults, no gender-related differences in angiotensin levels were identified. The observed increase in peptide concentrations regarding cardiac- and renal-diseased children is influenced by surgical repair, while evidence for a pharmacological impact is conflicting. A comprehensive set of angiotensin I, angiotensin II, and angiotensin 1-7 values from neonates up to adolescents was compiled. Indicating age as a strong effector. However, evidence about potential promising targets of the RAAS like angiotensin III and angiotensin IV is still lacking in children.

关键词： Neuroblastoma   Intracranial metastasis   Paediatric  

摘要： Objective: Neuroblastoma (NB) is the most common extracranial solid malignancy in children. Metastatic involvement of brain is rare in NB. This study was established to evaluate the clinical characteristics, treatment and prognosis of NB patients with brain metastases. Patients and methods: From September 2005 to December 2016, the clinical data of 15 cases with brain metastases among 264 NB patients admitted to Beijing Tongren Hospital, Capital Medical University were collected and retrospectively analysed. The clinical features of the 15 patients were summarised, and the patients were grouped according to different treatment methods and followed up for a median time of 41 months. The survival curves were plotted, and the Log-rank test was performed to compare the effect of different treatment methods on the prognosis. Results: The proportion of brain metastases in NB patients in our hospital is 5.68% (15/264). For the prognosis of 15 NB cases, the survival time of combined radiotherapy and/or autologous peripheral blood stem cell transplantation group was longer than that of simple operation and chemotherapy group (61.79 +/- 9.59 vs. 30.00 +/- 5.99 months, P = 0.03). Among the 15 patients, 4 cases underwent intracranial tumor resection, 4 cases received craniospinal irradiation, and the rest received maintenance chemotherapy. The 2-year survival rate was 82.2%, and the 5-year survival rate was 19.9%. The survival time of combined intracranial surgery and/or radiotherapy group was significantly longer than that of the chemotherapy group (46.67 +/- 6.69 vs. 16.42 +/- 1.42 months, P = 0.003). Conclusions: The incidence of brain metastases NB in children is relatively small, but the prognosis is very poor. Active chemotherapy, radiotherapy and surgery-based comprehensive treatment can prolong the survival time.

关键词： appendicitis   complications   ethnicity   paediatric   rural   severity  

摘要： Background Appendicitis is the most common surgical emergency affecting children. Rurality has been shown to be a predictor of worse surgical outcomes in patients with acute appendicitis compared to urban residents. There are no previously published studies investigating this in Australasia. Methods A 10-year retrospective study of all patients aged <= 16 years who underwent an acute appendicectomy in Northland, New Zealand, was conducted. The cohort was identified by searching the hospital database for theatre events and admission diagnoses coded as appendicitis. Primary outcome of interest was the difference in the American Association for the Surgery of Trauma (AAST) anatomical severity grading of appendicitis and the Clavien-Dindo complication rate. The role of ethnicity was also examined. Results A total of 470 children underwent appendicectomy during this period. On multivariate analysis, increased AAST grade was twice as likely in rural patients (odds ratio 2.04). Post-operatively, rural patients had higher Clavien-Dindo complication grade (P = 0.001), longer median length of stay and increased rates of intra-abdominal collection (19% versus 4%;P = 0.018), 30-day readmission (19% versus 4%;P = 0.020) and perforation (27% versus 19%;P = 0.031). Maori children had increased perforation rates (28.9% versus 19.0%;P = 0.014) but ethnicity was not found to be independently associated with increasing AAST grade. Conclusion Accounting for ethnicity, socio-economic deprivation and age, we implicate rural patient status as being associated with increasing severity and complicated paediatric appendicitis. This work adds to the evolving description of inequities in rural health outcomes. Further prospective studies are needed to confirm these findings at a national level.

摘要： Adrenocortical neoplasms are rare and presents as a large mass in the abdomen. Immunohistochemistry markers like Melan-A, Inhibin and synaptophysin can be used for establishing the diagnosis. Weiss criteria can be used to determine the prognosis.

关键词： radiation-induced second cancer risk   BNCT   Monte Carlo simulation   equivalent dose  

摘要： This study firstly explored the risks of secondary cancer in healthy organs of Chinese paediatric patients with brain tumours after boron neutron capture therapy (BNCT). Three neutron beam irradiation geometries (i.e. right lateral, top to bottom, posterior to anterior) were adopted in treating patients with brain tumours under the clinical environment of BNCT. The concerned organs in this study were those with high cancer morbidity in China (e.g. lung, liver and stomach). The equivalent doses for these organs were calculated using Monte Carlo and anthropomorphic paediatric phantoms with Chinese physiological features. The risk of secondary cancer, characterised by the lifetime attributable risk (LAR) factor given in the BEIR VII report, was compared among the three irradiation geometries. The results showed that the LAR was lower with the PA irradiation geometry than with the two other irradiation geometries when the 2 cm diameter tumour was at a depth of 6 cm on the right side of the brain. Under the PA irradiation geometry, the LAR in the organs increased with increasing tumour volume and depth because of the long irradiation time. As the patients aged from 10-15 years old, the LAR decreased, which was related to the increased patient height and shortened life expectancy. Female patients had a relatively higher risk of secondary cancer than male patients in this study, which could be due to the thinner body thickness and the weaker protective effect on the internal organs of the female patients. In conclusion, the risks of secondary cancer in organs were related to irradiation geometries, gender, and age, indicating that the risk of secondary cancer is a personalised parameter that needs to be evaluated before administering BNCT, especially in patients with large or deep tumours.

关键词： consultation   family   centred care   family empowerment   information   paediatric rehabilitation  

摘要： Background To explore experiences of parents of children with disabilities using the WWW, roadmap, a tool to support them in exploring needs, finding information, and asking questions of professionals and to explore differences between parents who had used the WWW-roadmap to prepare for consultation with their rehabilitation physician and parents who had not. Methods In a sequential cohort study, we included 128 parents;54 used the WWW-roadmap prior to consultation and 74 received care-as-usual. Both groups completed questionnaires after consultation, assessing empowerment, self-efficacy, parent and physician satisfaction, family centredness of care, and experiences using the tool. Additionally, 13 parents were interviewed. Results Parents who used the WWW-roadmap looked up more information on the Internet. No other differences between parents and physicians were found. In the interviews, parents said that the WWW-roadmap was a useful tool for looking up information, exploring and asking questions, and maintaining a comprehensive picture. Conclusion Using the WWW-roadmap prior to consultation did not improve self-efficacy, satisfaction, or family centredness of care. Findings suggest positive experiences regarding factors determining empowerment, creating conditions for a more equal parent-physician relationship. The WWW-roadmap is useful for parents to explore their needs and find information, but more is needed to support empowerment in consultations.

摘要： The improvements in the diagnosis and treatment of childhood cancers over the past four decades6 were built on a strong foundation of cross-border, multidisciplinary, international research, more recently supported by EU Framework funding programmes.7–9 Although there are distinct areas of research that uniquely address the needs of childhood cancer, active engagement with the adult cancer and rare disease communities is in place because of the important synergies, exemplified most recently in collaborations on the European Reference Network framework and the Joint Action on Rare Cancers. Fostering biology-driven therapeutic innovation, addressing the needs of children and teenagers with cancers typically diagnosed in adulthood, and long-term surveillance and outcome research in survivors are just some examples of areas common to both adult and paediatric cancer communities. In 2015, the European Society for Paediatric Oncology (SIOPE), in partnership with the patient advocate groups Childhood Cancer International-Europe and Unite2Cure, published a well defined long-term strategic plan to increase survival and the quality of life for children and adolescents with cancer in Europe by 2025.7 This strategic plan is now evolving to keep pace with emerging innovations.

关键词： acute kidney injury   continuous renal replacement therapy (CRRT)   fluid overload   fluid therapy   multiple organ failure   paediatric  

摘要： Background Patients with multiple organ failure (MOF) often receive large amounts of resuscitation fluid, making them at high risk of fluid overload (FO). Our main objective was to investigate if the ability to achieve a negative fluid balance during the first 3 continuous renal replacement therapy (CRRT) days was associated with mortality in children with MOF. Methods Retrospective cohort study in a tertiary multidisciplinary academic paediatric hospital. The study included 63 patients (age 0-18 years) with 3 or more failing organs receiving CRRT due to acute kidney injury and/or fluid overload. Results The median age was 4 months, and PICU mortality was 29%. Survivors had significantly lower degree of FO at CRRT initiation, (median 15% (Interquartile range 9-22)) than non-survivors (24% (17%-37%), P = 0.002). On PICU admission, PIM-3 score was significantly higher in non-survivors (P = 0.01), but at CRRT initiation there was no difference in PELOD-2 score (P = 0.98). Mortality in patients achieving a cumulative net negative fluid balance during the first 3 days after CRRT initiation was 12%, compared to 86% in those not achieving this (P < 0.0001). In multivariate analysis, the inability to achieve a net negative fluid balance during 3 days after CRRT initiation (P < 0.0001) and FO >20% at CRRT initiation (P = 0.0019) remained associated with mortality. Conclusion Our results suggest that early fluid removal is associated with improved patient outcome in critically ill children receiving CRRT, and that prompt measures should be taken to prevent fluid overload in critical illness. These results need to be verified in further, prospective studies.

关键词： donation after circulatory determined death   heart transplantation   normothermic regional perfusion   paediatric  

摘要： This is a report of a unique DCD paediatric heart transplant whereby normothermic regional perfusion was used to assess DCD heart function after death followed by ex situ heart perfusion of the graft during transportation from donor to recipient hospitals. The DCD donor was a 9-year-old boy weighing 84 kg. The recipient was 7-year-old boy with failing Fontan circulation and weighed 23 kg. It was an ABO-compatible heart transplantation. The DCD heart was reperfused and assessed using normothermic regional perfusion followed by portable ex situ heart perfusion during transportation. The orthotopic heart transplantation was successful with good graft function and no evidence of rejection on endomyocardial biopsy at 30 days post-transplant. At 1-year follow-up, excellent graft function is maintained, and he is attending school with a good quality of life. DCD heart transplantation in children is a promising solution to reducing paediatric waiting times. The case demonstrates the feasibility of using normothermic regional perfusion in the donor and ex situ heart perfusion during graft transportation. This combination allowed a functional assessment whilst minimizing warm ischaemia resulting in a successful outcome. More research and long-term follow-up are needed in order to benefit from the huge potential that paediatric DCD heart transplantation has to offer.