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关键词： Congenital heart disease   Acquired heart disease   Health care services  

摘要： Background There is a paucity of contemporary information regarding the prevalence and related health care expenditure of the most common cardiac conditions in Australian children, including congenital heart disease (CHD). Methods The 10 most common cardiac conditions managed by Queensland Paediatric Cardiac Service during 2014-2015 were identified from an electronic database: ventricular septal defect (VSD), pulmonary stenosis, aortic stenosis, tetralogy of Fallot, atrioventricular septal defect, transposition, Ebstein's anomaly, long QT syndrome, dilated cardiomyopathy, and rheumatic carditis. Demographic data, clinic attendance, investigational procedures, and therapeutic interventions were extracted from the electronic health records to derive indicative population estimates and direct health care expenditure relating to CHD. Results A total of 2,519 patients diagnosed with the 10 target conditions were being actively managed, including 456 (18.1%) new-born and 787 prevalent cases (2.5/1,000 population) aged ,5 years. A total of 12,180 (4.8/ case) investigations were performed (6,169 echocardiographic and 279 cardiac catheterisation procedures) costing $2.25 million/annum. A further 5,326 clinic visits (2.1/case, 22% regional) were conducted at a cost of $550,000/annum. A combined total of 804 catheter-based interventions and surgical procedures were performed in 300 cases (11.9%) at a cost of $13.6 million/annum. VSD (38.6% of cases) was the single greatest contributor ($5.1 million/annum) to total combined direct health care costs of $13.6 million/ annum for the 2,519 patients. Conclusions These pilot data indicate a significant patient population and health care burden imposed by CHD in Queensland. Future initiatives to better quantify this burden, from an individual to health system perspective, are urgently needed.

关键词： Academic surgery   Research   Scholarship   RACS   Paediatric surgery   Academic surgeon  

摘要： Traditionally, academic surgeons have been expected to excel in research, administration, teaching and clinical work. For many, to be strong in all of these areas is aspirational rather than a reality - and it may not always be a desirable expectation. It is more likely that future academic surgeons will have exceptional ability in several of these domains, but probably not all. Clinical expertise (even if it is within a narrow field) is critical to gaining credibility with non-academic surgical colleagues;and research leadership and substantial ongoing academic output is critical to maintaining credibility among academic surgical colleagues -and facilitates funding success. The Board of Paediatric Surgery is the specialty training board of the Royal Australasian College of Surgeons (RACS) that is responsible for the training program in paediatric surgery for both Australia and New Zealand. "Scholarship and teaching" is designated as being one of the nine competencies RACS expects of all surgeons. Expertise in the domain of scholarship (and research) occurs at two levels: (1) A working knowledge of scientific method, having a critical and curious mind matched with an ability to formulate a research question and contribute to research studies, and an ability to analyse research data and to use it to inform clinical practice. This is expected of all surgeons;and (2) A career academic surgeon with a formal commitment to research which becomes a major component of their work, with the requisite expertise in scientific method to be able to design, set up and complete research studies. The RACS provides support for academia in surgery to flourish in multiple ways and at various stages in the surgeons' career, as described in this chapter. Increasingly, the academic surgeon has to forge links and to collaborate with other research groups. At least in Australia and New Zealand, departments should work to ensure that their academic surgeons are not excessively burdened with

关键词： adolescents   mental health   paediatrics  

摘要： Objective To explore trends in presentation and key performance indicators with respect to children seeking mental health services at The Prince Charles Hospital Children's Emergency Department (TPCH-CED), Brisbane. Methods A retrospective audit was performed utilising data obtained from the local Emergency Department Information System for all mental health presentations between 1 January 2013 and 1 January 2018 to TPCH-CED. Results A total of 1078 children presented to TPCH-CED requiring mental health review between 2013 and 2018. Mental health presentations almost doubled over the 5 years (185 attendances in 2013;342 in 2017);however, as a proportion of overall presentations, there was no significant increase. The percentage of mental health presentations meeting the National Emergency Access Target criteria dropped from 63% in 2013 to 39% in 2017 (P < 0.0001). Those requiring a mental health inpatient stay were more likely to have very extended length of stay within the CED. Suicidal ideation was the most common presenting complaint (72% of mental health presentations). Violence and aggression were noted in a consistent proportion of mental health presentations (4.4-12%). Conclusions Despite failing to demonstrate an interval increase in the proportion of children presenting to TPCH-CED requiring mental health services, it is clear that children with mental health needs, like their adult counterparts, disproportionately experience delays to appropriate care when compared with children presenting with other emergency conditions. Ongoing research is warranted to assess standards of care and the impact of mental health presentations on CEDs.

摘要： This article presents an agenda to improve the care and wellbeing of children with paediatric feeding disorder who require tube feeding (PFD-T). PFD-T requires urgent attention in practice and research. Priorities include: routine collection of PFD-T data in health-care records;addressing the tube-feeding lifecycle;and reducing the severity and duration of disruption caused by PFD-T where possible. This work should be underpinned by principles of involving, respecting and connecting families.

摘要： The field of acquired CNS neuroimmune demyelination in children is transforming. Progress in assay development, refinement of diagnostic criteria, increased biological insights provided by advanced neuroimaging techniques, and high-level evidence for the therapeutic efficacy of biological agents are redefining diagnosis and care. Three distinct neuroimmune conditions-multiple sclerosis, myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and aquaporin-4 antibody-associated neuromyelitis optica spectrum disorder (AQP4-NMOSD)-can now be distinguished, with evidence from humans and animal models supporting distinct pathobiological disease mechanisms. The development of highly effective therapies for adult-onset multiple sclerosis and AQP4-NMOSD that suppress relapse rate by more than 90% has motivated advocacy for trials in children. However, doing clinical trials is challenging because of the rarity of these conditions in the paediatric age group, necessitating new approaches to trial design, including age-based trajectory modelling based on phase 3 studies in adults. Despite these limitations, the future for children and adolescents living with multiple sclerosis, MOGAD, or AQP4-NMOSD is far brighter than in years past, and will be brighter still if successful therapies to promote remyelination, enhance neuroprotection, and remediate cognitive deficits can be further accelerated.

关键词： Paediatric IBD   Renal disease in IBD   Inflammatory bowel disease   Kidneys and IBD   Extraintestinalmanifestation IBD  

摘要： Inflammatory bowel disease (IBD), which includes Crohn's disease, ulcerative colitis and inflammatory bowel disease unclassified, is a chronic inflammatory disorder that predominantly affects the gastrointestinal (GI) tract and has a rising incidence in both children and adults. Symptoms are caused by inappropriate inflammatory response triggered by interaction between the environment, gut microbiome and host immune system in a genetically susceptible individual. Extranintestinal manifestations of IBD are common and can affect any body system outside the gut;they can precede or run parallel to GI inflammation. Renal involvement in IBD is uncommon and can be part of extraintestinal manifestation or metabolic complications of IBD. Many medications used to treat IBD can cause renal damage. Renal manifestation in children with IBD can range from asymptomatic biochemical abnormalities to variable stages of renal impairment with significant morbidity and even mortality burden.

关键词： children   coronavirus   COVID-19   SARS-CoV-2  

摘要： Aim This study is a comprehensive review with the purpose of collecting the most relevant data in several sections including current treatment guidelines in the paediatric population. Methods Literature was systematically searched in different databases. Results were limited to 2019+ and English, French and Spanish language. Results Children can exhibit mild and less severe COVID-19 disease than adults and also have asymptomatic carriage of SARS-CoV-2, while severe disease is more frequently noted during infancy (<1 year). SARS-CoV-2 binds the angiotensin-converting enzyme 2 (ACE-2) receptor;age-, racial-, and gender-specific differences in ACE-2 expression need to be elucidated in order to explain the differential clinical profiles between children and adults. Multisystem inflammatory syndrome in children (MIS-C) is an important condition to recognise in children. The decision to use antiviral or immunomodulatory therapy in a child or adolescent should be individualised based on the clinical scenario. Remdesivir is the only FDA-approved therapy available for children older than 12 years old who require hospitalisation for COVID-19. Conclusion Further studies are urgently required to address prevention and treatment in at-risk and infected children, especially with underlying comorbidities. The chapter on the overall impact of COVID-19 in children has not yet been written. Nevertheless, SARS-CoV-2 has now joined a long list of human pandemics, which may forever change the world's history.

关键词： biomarkers   gastroenterology   children   pharmacogenomics   pharmacokinetic-pharmacodynamic  

摘要： Aims Identifying DNA variants associated with trough serum anti-tumour necrosis factor (TNF) levels could predict response to treatment in inflammatory bowel disease (IBD). To date, no specific studies have been performed in children. The aim of this study was to identify genetic variants associated with trough serum anti-TNF levels and whether these variants are differential markers for infliximab and adalimumab. Methods We included 154 children (age < 18 years) from 17 hospitals who had been diagnosed with IBD and actively treated with infliximab or adalimumab. Twenty-one polymorphisms were genotyped using real-time PCR. Trough serum anti-TNF levels were measured using enzyme-linked immunosorbent assay (ELISA). The association between DNA polymorphisms and the therapeutic range or the absolute values of anti-TNF drugs was analysed by Fisher exact test, student'st-test and logistic regression. Results The variants rs5030728 (TLR4) and rs11465996 (LY96) were associated with subtherapeutic infliximab levels. rs1816702 (TLR2) was associated with supratherapeutic levels and rs3397 (TNFRSF1B) with subtherapeutic levels of adalimumab (P< .05). In addition, rs1816702 (TLR2) and rs2569190 (CD14) were associated with absolute values of trough serum adalimumab, and rs2569190 (CD14) was associated with absolute values of trough serum adalimumab and infliximab (P< .05). Conclusion Genotyping of these DNA variants before starting treatment may help to select the best anti-TNF drug in paediatric patients. The SNP rs1816702 is the most promising marker for tailoring the anti-TNF regimen in children with IBD. For the first time, DNA variants are associated with trough serum anti-TNF levels.

关键词： Lactate   pyruvate   euthyroid sick syndrome   congenital heart disease   cardiopulmonary bypass   low cardiac output syndrome   thyroid hormone replacement  

摘要： Objective: To determine if triiodothyronine alters lactate, glucose, and pyruvate metabolism, and if serum pyruvate concentration could serve as a predictor of low cardiac output syndrome in children after cardiopulmonary bypass procedures. Methods: This study was ancillary to the Oral Triiodothyronine for Infants and Children undergoing Cardiopulmonary bypass (OTICC) trial. Serum pyruvate was measured in the first 48 patients and lactate and glucose were measured in all 208 patients enrolled in the OTICC study on the induction of anaesthesia, 1 and 24 hours post-aortic cross-clamp removal. Patients were also defined as having low cardiac output syndrome according to the OTICC trial protocol. Result: Amongst the designated patient population for pyruvate analysis, 22 received placebo, and 26 received triiodothyronine (T3). Lactate concentrations were nearly 20 times greater than pyruvate. Lactate and pyruvate levels were not significantly different between T3 and placebo group. Glucose levels were significantly higher in the placebo group mainly at 24-hour post-cross-clamp removal. Additionally, lactate and glucose levels peaked at 1-hour post-cross-clamp removal in low cardiac output syndrome and non-low cardiac output syndrome patients, but subsequently decreased at a slower rate in low cardiac output syndrome. Lactate and pyruvate concentrations correlated with glucose only prior to surgery. Conclusion: Thyroid supplementation does not alter systemic lactate/pyruvate metabolism after cardiopulmonary bypass and reperfusion. Pyruvate levels are not useful for predicting low cardiac output syndrome. Increased blood glucose may be regarded as a response to hypermetabolic stress, seen mostly in patients with low cardiac output syndrome.