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关键词： Paediatric heart surgery   Early extubation   Perioperative management  

摘要： Background Early extubation has become widely adopted in cardiac surgery practices. This study aimed to present experience of early extubation after congenital heart surgery and to explore the factors that affect successful immediate postoperative extubation and early extubation. Methods A retrospective analysis was performed of all patients who underwent congenital heart surgery with cardiopulmonary bypass (CPB) at Shenzhen Children's Hospital between 01 May 2015 and 30 September 2019. The demographic and cardiac surgery information were derived from the medical records. Multivariable logistic regression models were used to explore the influence factors for successful immediate postoperative extubation and early extubation. Results This study consisted of 2,060 patients, 65.0% of whom were extubated in the operating room and 16.1% of whom were extubated early (within 6 hours) in the Intensive Care Unit. The overall rates of reintubation and nasal continuous positive airway pressure were 2.0% and 6.4%, respectively. Preoperative weight (OR, 1.24;95% CI, 1.20-1.29), preoperative pneumonia (OR, 0.60;95% CI, 0.44-0.80), CPB type (OR, 1.23;95% CI, 1.06-1.43), CPB time (OR, 0.98;95% CI, 0.98-0.99), deep hypothermic circulatory arrest (OR, 0.42;95% CI, 0.25-0.70), and Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Congenital Heart Surgery (STAT) categories (OR, 0.54;95% CI, 0.45-0.65) were included in the immediate postoperative extubation model. In addition to the above six variables, ultrafiltration (OR, 0.63;95% CI, 0.44-0.89) was also included in the early extubation model. Similar results were found in the immediate postoperative extubation model for non-newborns. The influencing factors for early extubation in the nonnewborn population included preoperative weight, preoperative pneumonia, ultrafiltration, CPB time, and STAT categories. Conclusions Early extubation for children with congenital heart surgery was successful in this hospita

关键词： Rare disease   Quality of life   Economic evaluation   Informal care   Genomic medicine  

摘要： Purpose The complexity and severity of rare genetic conditions pose substantial burden to families. While the importance of spillovers on carers' health in resource allocation decisions is increasingly recognised, there is significant lack of empirical evidence in the context of rare diseases. The objective of this study was to estimate the health spillovers of paediatric rare genetic conditions on parents. Methods Health-related quality-of-life (HRQoL) data from children with rare genetic conditions (genetic kidney diseases, mitochondrial diseases, epileptic encephalopathies, brain malformations) and their parents were collected using the CHU9D and SF-12 measures, respectively. We used two approaches to estimate parental health spillovers. To quantify the 'absolute health spillover', we matched our parent cohort to the Australian general population. To quantify the 'relative health spillover', regression models were applied using the cohort data. Results Parents of affected children had significantly lower HRQoL compared to matched parents in the general public (- 0.06;95% CIs - 0.08, - 0.04). Multivariable regression demonstrated a positive association between parental and child health. The mean magnitude of HRQoL loss in parents was estimated to be 33% of the HRQoL loss observed in children (95% CIs 21%, 46%). Conclusion Paediatric rare genetic conditions appear to be associated with substantial parental health spillovers. This highlights the importance of including health effects on family members and caregivers into economic evaluation of genomic technologies and personalised medicine. Overlooking spillover effects may undervalue the benefits of diagnosis and management in this context. This study also expands the knowledge of family spillover to the rare disease spectrum.

关键词： Decision-making   evaluation   survey design   validation   psychometrics   paediatrics  

摘要： There is an increasing number of shared decision-making (SDM) interventions in paediatrics. However, there is little consensus as to the best instruments to assess the feasibility and impact of these interventions. This narrative review aims to answer: (1) what feasibility, knowledge and decision-making instruments have been used to assess paediatric SDM interventions and (2) what are the psychometric properties of used decision-making instruments, guided by the 'consensus-based standards for the selection of health measurement instrument' criteria. We conducted a review of the peer-reviewed literature. We identified 23 studies that evaluated a paediatric intervention to facilitate SDM for a specific health decision. Eighteen studies assessed intervention feasibility, with a wide variability in assessment between studies. Twelve studies assessed objective knowledge, and four studies assessed subjective knowledge with all but one study aggregating correct responses. We identified nine decision-making instruments that had been assessed psychometrically, although few had been thoroughly evaluated. The Decisional Conflict Scale was the most commonly-used instrument and the only instrument evaluated in paediatrics. Our study revealed a lack of consistency in the instruments used to evaluate decision-making interventions in paediatrics, making it difficult to compare interventions. We provide several recommendations for researchers to improve the assessment of SDM interventions in paediatrics.

关键词： bronchiolitis   emergency medicine   general paediatrics  

摘要： Aim Bronchiolitis is the commonest cause of hospitalisation for infants. Evidence-based Australasian bronchiolitis guideline was developed and introduced in 2017. This audit was to determine if the knowledge translation process of the updated local tertiary hospital bronchiolitis guideline (based on the Australasian guideline) reduced unnecessary interventions. Methods A retrospective chart review of infants with bronchiolitis diagnosis during the pre-guideline (1 July to 31 August 2015) and post-guideline (1 July to 31 August 2017) period, with the primary outcome of the number/proportion of unnecessary interventions. Results Presentations between 1 July to 31 August 2015 (n= 465) were compared with 2017 (n= 343). There was no difference in undertaking chest X-ray (24 (5.2%) vs. 17 (5.0%), odds ratio (OR) 0.98 (95% confidence interval (CI) 0.71-1.35),P= 0.911), salbutamol (23 (4.9%) vs. 10 (2.9%), OR 0.86 (95% CI 0.65-1.13),P= 0.279), glucocorticoids (2 (0.4%) vs. 5 (1.5%), OR 1.89 (95% CI 0.83-4.31),p= 0.129), antibiotics (11 (2.4%) vs. 5 (1.5%), OR 0.86 (95% CI 0.65-1.15),P= 0.307) or nasopharyngeal aspirate (172 (37%) vs. 124 (36.2%), OR 1.00 (95% CI 0.87-1.67),P= 0.937) in hospital. Adrenaline was not administered in both years. There was reduced hospital admissions (303 (65.2%) vs. 192 (56.0%), OR 0.82 (95% CI 0.71-0.95),P= 0.008) with no difference in paediatric intensive care unit admissions (10 (2.2%) vs. 8 (2.3%), OR 1.04 (95% CI 0.65-1.67),P= 0.863). Conclusion The dissemination process of the updated local hospital bronchiolitis guideline did not show any statistically significant reduction of unnecessary interventions in the hospital. Further studies are required to determine the effective process to instigate changes in health services.

关键词： comprehensive care   health care costs   resource use   sickle cell disease  

摘要： Background While multiple studies have examined the cost of health care for one aspect of sickle cell disease care, few have focussed on the overall cost of comprehensive care for sickle cell disease. Methods We conducted a retrospective cohort study of children with sickle cell disease treated in a comprehensive care centre from 1 January 2015 to 31 December 2016. Health care utilisation of included patients was based upon data from two main sources. The clinical practice guideline was used to determine the expected resource use of routine comprehensive care (planned elective care), and the financial claims database was used to estimate real-world resource use associated with acute and inpatient care (additional care). Results A total of 125 children with sickle cell disease were analysed. Expenditures for these patients averaged euro5049 [standard deviation (SD) euro1634] per child per year. Total yearly costs per patient varied considerably, ranging from euro669 to euro84 010, and less than 15% of patients were responsible for 50% of the health care costs. The majority (37%) of costs was associated with inpatient hospital care, which increased by age group, 27% with diagnostics, 19% with treatment, 11% with outpatients' visits and 6% with emergency care. Conclusion We have described real-world resource use and expenditures for children with sickle cell disease in a European comprehensive care centre. It seems that costs of a comprehensive approach with effective management in the outpatient setting is favourable when compared to episodic health care.

关键词： anti-fibrinolytics   blood loss   blood transfusion   craniosynostosis surgery   paediatric anaesthesia   pharmacokinetics   tranexamic acid  

摘要： Background: Tranexamic acid (TXA) reduces blood loss and transfusion in paediatric craniosynostosis surgery. The hypothesis is that low-dose TXA, determined by pharmacokinetic modelling, is non-inferior to high-dose TXA in decreasing blood loss and transfusion in children. Methods: Children undergoing craniosynostosis surgery were enrolled in a two-centre, prospective, double-blind, randomised, non-inferiority controlled trial to receive high TXA (50 mg kg(-1) followed by 5mg kg(-1) h(-1)) or low TXA (10 mg kg(-1) followed by 5 mg kg(-1) h(-1)). Primary outcome was blood loss. Low dose was determined to be non-inferior to high dose if the 95% confidence interval (CI) for the mean difference in blood loss was above the non-inferiority margin of -20 ml kg(-1). Secondary outcomes were transfusion, TXA plasma concentrations, and biological markers of fibrinolysis and inflammation. Results: Sixty-eight children were included. Values were non-inferior regarding blood loss (39.4 [4.4] vs 40.3 [6.2] ml kg(-1) [difference=0.9;95% CI: -14.2, 15.9]) and blood transfusion (21.3 [1.6] vs 23.6 [1.5] ml kg(-1) [difference=2.3;95% CI: -2.1, 6.7]) between high-dose (n=32) and low-dose (n=34) groups, respectively. The TXA plasma concentrations during surgery averaged 50.2 (8.0) and 29.6 (7.6) mg ml(-1). There was no difference in fibrinolytic and inflammatory biological marker concentrations. No adverse events were observed. Conclusions: Tranexamic acid 10 mg kg(-1) followed by 5 mg kg(-1) h(-1) is not less effective than a higher dose of 50 mg kg(-1) and 5 mg kg(-1) h(-1) in reducing blood loss and transfusion in paediatric craniosynostosis surgery.

关键词： communication   decision-making   otolaryngology   paediatrics   patient-centred care   physician-patient relations   shared   tonsillectomy  

摘要： Objectives Physician and patient/parent communication is of utmost importance in consultations to improve the shared decision-making (SDM) processes. This study investigated SDM-related outcomes through turn analysis and an assessment of patient-centred dialogue. Design Multi-centre prospective cohort study analysing audio- and video-recorded patient/parent-physician interactions. Setting Two tertiary paediatric hospitals in Halifax, Nova Scotia and Salt Lake City, Utah. Participants Paediatric otolaryngologists, patients and parents during consultation for adenotonsillectomy. Main outcome measures Medical dialogue measures (turn analysis, patient-centredness scores via the Roter Interaction Analysis System) and SDM questionnaires (SDM-Q-9). Results Turn density was significantly higher for physicians than patients/parents (P < .001), as were total statements (P < .001), and total time talking (P < .001). The opening statement was completed by the physician in 91.5% of interactions and was significantly longer than family opening statements (P = .003). The mean number of informed consent elements addressed per interaction was 4.5 out of 6. The mean patient-centredness score was 0.2 (range 0-0.56). Significant negative correlations between patient-centredness score and physician turn density (r = -.390, P = .002), physician mean turn time (r = -.406, P = .001), total physician statements (r = -.426, P = .001) and total physician speaking time (r = -.313, P = .016) were noted. There were no correlations in SDM questionnaire scores with turn analysis variables, informed consent elements or patient-centredness scores. Conclusions Surgeons dominated the consultation in terms of talking, mostly in a unidirectional manner. Neither patient-centredness nor turn analysis correlated with perceptions of SDM from the parents' perspective.

关键词： Cervical   fracture   paediatric   spinal   spine   trauma  

摘要： Significant spinal trauma in the paediatric population is uncommon. Cervical spine injuries account for approximately 1.5% of injuries. In paediatric patients a far higher proportion of injuries that affect the spinal column occur in the cervical spine in comparison with adults. The assessment of paediatric spinal injuries and radiographs can be challenging even for experts. This is due to a combination of the evolving osseous anatomy and the increase in ligamentous injury. This article looks at the initial assessment and management of injuries. It also presents some of the common radiographic measurements which can be used to assess spinal injuries, paediatric specific injuries of the spine and common variants confused with fractures. © 2020

关键词： cerebral palsy   botulinum toxin   healthcare transition  

摘要： Young people (YP) with neurological disabilities such as cerebral palsy are increasingly living into adulthood and require healthcare transition for services including for botulinum toxin A (BoNT-A). We analysed medical records in the three children's hospitals in New South Wales (NSW) and identified 253 YP who are expected to transition from paediatric to adult BoNT-A services in NSW and Australian Capital Territory during 2018-2023. A substantial proportion of these YP have additional needs that will require paediatric and adult health services to work together to improve their life-long health outcomes.

摘要： Aim To assess the efficacy, safety, and tolerability of oromucosal nabiximols cannabinoid medicine as adjunct therapy for children with spasticity due to cerebral palsy/traumatic central nervous system injury with inadequate response to existing treatment. Method Overall, 72 patients (mean [SD] age 12y 4mo [3y 1mo], range 8-18y) were randomized at a ratio of 2:1 to receive nabiximols (n=47;29 males, 18 females) or placebo (n=25;15 males, 10 females) for 12 weeks (12 sprays/day max. based on clinical response/tolerability). The primary outcome was change from baseline in level of spasticity on a 0 to 10 Numerical Rating Scale (NRS), assessed by the primary caregiver at 12 weeks. Secondary outcomes included additional measures for spasticity, sleep quality, pain, health-related quality of life, comfort, depression, and safety. Results There was no significant difference in the spasticity 0 to 10 NRS between nabiximols versus placebo groups after 12 weeks. No statistically significant differences were observed for any secondary endpoint. Adverse events were predominantly mild or moderate in severity;however, three cases of hallucinations were reported. Interpretation Nabiximols was generally well tolerated;however, neuropsychiatric adverse events were observed. No significant reduction in spasticity with nabiximols treatment versus placebo was observed. What this paper adds Oromucosal nabiximols is generally well tolerated by paediatric patients. However, three cases of hallucinations were observed, one of which involved auditory hallucinations and a suicide attempt. Oromucosal nabiximols versus placebo did not reduce cerebral palsy/central nervous system injury-related spasticity.