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关键词： Hodgkin lymphoma   paediatric   neuropathy  

摘要： This phase II trial was designed to determine the safety and efficacy of a modified paediatric risk-stratified protocol in young adults (18-30 years) with classical Hodgkin Lymphoma. The primary end-point was neurotoxicity rate. The incidence of grade 3 neurotoxicity was 11% (80% CI, 5-19%);a true rate of neuropathy of >15% cannot be excluded. Neuropathy and associated deterioration in quality of life was largely reversible. The overall response rate was 100% with 40% complete remission (CR) rate. Twelve months disease-free survival (DFS) was 91%. We demonstrate that a risk-stratified paediatric combined modality treatment approach can be delivered to young adults without significant irreversible neuropathy.

关键词： human leukocyte antigen   alloimmunization   bone marrow transplant   transfusion   sickle cell disease  

摘要： The relevance of donor-specific human leukocyte antigen (HLA) antibodies in HLA-mismatched haematopoietic cell transplant (HCT) is known, but the importance of HLA antibodies in HLA-matched HCT is unclear. We hypothesized that HLA antibodies detected before HCT would cause platelet transfusion refractoriness during HCT and investigated this in a multi-centre study. Pre-HCT samples from 45 paediatric patients with sickle cell disease (SCD) undergoing HLA-matched HCT were tested for HLA class I antibodies. The number of platelet transfusions received before day +45 was compared between those with and without antibodies. Thirteen of 45 (29%) patients had a positive HLA class I antibody screen, and these patients received significantly more platelet transfusions than patients without antibodies (median 19 vs. 7 center dot 5, P = 0 center dot 028). This platelet transfusion association remained significant when controlling for conditioning regimen. Among alloimmunized patients, there was no association between the panel-reactive antibody and the number of platelet transfusions. Patients with HLA class I antibodies also had a higher incidence of acute graft-versus-host disease (GVHD): 6/13 (46%) vs. 3/32 (9%), P = 0 center dot 011. Pre-HCT HLA class I alloimmunization is associated with increased platelet transfusion support and acute GVHD in paediatric HLA-matched HCT for SCD. Further studies are needed to investigate the pathobiology of this association.

关键词： coagulopathy   fibrinogen   major haemorrhage   paediatric   trauma   viscoelastic haemostatic assay  

摘要： Objective Haemorrhage in paediatric trauma remains a significant cause of morbidity and mortality. Over recent years there has been increasing attention to the role of fibrinogen in traumatic haemorrhage and the association of low fibrinogen levels with poor patient outcomes. In addition, there has been a move towards using viscoelastic haemostatic assays (VHAs) to rapidly assess coagulation status and guide clinicians in the replacement of coagulation factors, including fibrinogen. In the paediatric population, there has been limited uptake of these principles and a paucity of data to support a change in practice. This paper summarises the available evidence in the published literature through a systematic review, presented in narrative format. Results There is limited high-quality prospective data on the use of VHA in the management of acute traumatic coagulopathy in the paediatric population. While the use of fibrinogen early in major haemorrhage is becoming standard practice, there are currently no randomised prospective studies comparing fibrinogen concentrate to cryoprecipitate. Conclusions The early identification of hypo-fibrinogenemia and acute traumatic coagulopathy in paediatric trauma using VHA testing and subsequent early fibrinogen replacement with a concentrated off the shelf product is an attractive treatment option. However, there is currently insufficient high-level evidence to support the use of fibrinogen concentrate over cryoprecipitate in the paediatric trauma population. Pilot studies currently under way will go some way to addressing this important knowledge gap, and facilitate the design of larger definitive multi-centre randomised trials.

关键词： adenotonsillectomy   dexmedetomidine   paediatric patients   tramadol  

摘要： What is known and objective Adenotonsillectomies are common surgical procedures performed under general anaesthesia in childhood. Post-operative pain and agitation are complications associated with this procedure. We compared the effects of dexmedetomidine and tramadol on post-operative pain, agitation, haemodynamic parameters, and extubation quality in patients undergoing an adenotonsillectomy using sevoflurane as an anaesthetic agent. Methods Sixty paediatric patients who had undergone an adenotonsillectomy were included in this study. The patients were randomized into two groups that received either dexmedetomidine (n = 30) or tramadol (n = 30). Haemodynamic variables, extubation time, post-operative pain, agitation and adverse events were recorded for all patients. Post-operative pain was assessed with the pain point system scale (PPSS), and agitation was assessed using the Riker Sedation-Agitation Scale (SAS). Results Patients in the dexmedetomidine group had significantly lower heart rates than the tramadol group. In addition, patients in the dexmedetomidine group had significantly lower post-operative PPSS and Riker SAS scores than patients in the tramadol group. Not breathing, cough-bucking and desaturation after extubation occurred less frequently in patients who received dexmedetomidine than in patients who received tramadol. However, the time to extubation was significantly longer for patients who received dexmedetomidine. What is new and conclusion Our study demonstrated that dexmedetomidine was more effective than tramadol for mitigating post-operative pain and agitation in paediatric patients following an adenotonsillectomy with sevoflurane. Although dexmedetomidine was associated with a longer time to extubation, it was also associated with fewer complications following extubation compared with tramadol.

关键词： Forgotten Baby syndrome   Heatstroke   Hot car deaths   Hyperthermia  

摘要： An average of 37 children die of hyperthermia inside parked vehicles annually in the USA. The majority of cases are due to a caregiver forgetting them (similar to 55%), while similar to 13% are due to intentionally leaving children unattended and similar to 28% occur when children climb into unlocked vehicles. The cause of four per cent is unknown. There are no published data on incidence in Canada. Through information provided from provincial and territorial coroner's offices, Canadian government agencies and media reports, six cases of vehicular hyperthermia deaths were confirmed since 2013. Three were attributed to children left unintentionally in vehicles;one occurred after a child climbed into an unlocked vehicle and two cases are undetermined. Attention or memory lapses are hypothesized to occur due to distraction, stress, fatigue, or routine changes. Educating caregivers about the dangers of leaving children in vehicles and providing preventative strategies through anticipatory guidance may reduce these tragedies (see graphic abstract).

关键词： Aboriginal   children   tertiary hospital   cultural competence   experiences  

摘要： Objective: To explore caregiver perspectives of their children's journey through the specialist paediatric service, the Aboriginal Ambulatory Care Coordination Program (AACC), and non-AACC services at the Perth Children's Hospital. Methods: Eighteen semi-structured interviews with families of Aboriginal children were completed. Indigenous research methodology and a phenomenological approach guided data collection and analysis. Results: Four key themes were identified from interviews: hospital admissions, discharge and follow-up outpatient appointments;communication;financial burden;and cultural issues. Our findings suggest Aboriginal children and their caregivers using the AACC program had more positive and culturally secure experiences than those using non-AACC services. However, barriers relating to health providers' understanding of Aboriginal cultural issues and lived experience were commonly discussed, regardless of which service families received. Conclusions: Australian Aboriginal children have an increased use of tertiary hospital care compared to non-Indigenous children. Healthcare programs specifically designed for Aboriginal children and their families can improve their experience of care in hospital. However, improvements in cultural awareness for other hospital staff is still needed.

关键词： Sarcopenia   Children   Chronic Disease  

摘要： Background Sarcopenia, the unintentional loss of skeletal muscle mass, is associated with poor outcomes in adult patient populations. In adults, sarcopenia is often ascertained by cross-sectional imaging of the psoas muscle area (PMA). Although children with chronic medical illnesses may be at increased risk for muscle loss because of nutritional deficiencies, physical deconditioning, endocrine anomalies, and systemic inflammation, consistent quantitative definitions for sarcopenia in children are lacking. We aimed to generate paediatric reference values for PMA at two intervertebral lumbar levels, L3-4 and L4-5. Methods In this cross-sectional study, we analysed abdominal computed tomography scans of consecutive children presenting to the emergency department. Participants were children 1-16 years who required abdominal cross-sectional imaging after paediatric trauma between January 1, 2005 and December 31, 2015 in a large Canadian quaternary care centre. Children with a documented chronic medical illness or an acute spinal trauma at presentation were excluded. Total PMA (tPMA) at levels L3-4 and L4-5 were measured in square millimetres (mm(2)) as the sum of left and right PMA. Age-specific and sex-specific tPMA percentile curves were modelled using quantile regression. Results Computed tomography images from 779 children were included. Values of tPMA at L4-5 were significantly larger than at L3-4 at all ages, but their correlation was high for both girls (r = 0.95) and boys (r = 0.98). Amongst girls, tPMA 50th percentile values ranged from 365 to 2336 mm(2) at L3-4 and from 447 to 2704 mm(2) for L4-5. Amongst boys, 50th percentile values for tPMA ranged between 394 and 3050 mm(2) at L3-4 and from 498 to 3513 mm(2) at L4-5. Intraclass correlation coefficients were excellent at L3-4 (0.97, 95% CI 0.94 to 0.981) and L4-5 (0.99, 95% CI 0.986 to 0.995). Weight and tPMA were correlated, stratified by sex for boys (L3-4 r = 0.90;L4-5 r = 0.90) and for girls (L3-4 r = 0.8

关键词： Klebsiella pneumoniae   Carbapenemase   Virulence   Epidemiology   Paediatric  

摘要： Objective: We investigated the epidemiological characteristics of drug resistance and virulence factors of carbapenem-resistant Klebsiella pneumoniae (CRKP) isolates from paediatric patients in Shanghai. Methods: CRKP strains were consecutively collected between January and December in 2018. Antimicrobial susceptibility was determined by VITEK 2 compact. Polymerase chain reaction (PCR) was used to analyse drug resistance determinants, virulence genes and plasmid types. wzi sequencing and multilocus sequence typing was used to determine clonal relatedness. Results: Among 172 CRKP strains, bla(KPC-2) and bla(NDM-5) were the predominant carbapenemase genes. Compared with NDM-5, KPC-2 producers showed higher resistance rates to fluoroquinolones and aminoglycosides. The majority of KPC-2 producers belonged to KL64-ST11 background, while NDM-5 producers were mainly identified as KL62-ST48. Plasmid typing shown that IncF and IncFIB were the most prevalent plasmids in KPC-2 producers and IncX3 was widely spread in NDM-5-KP. Thirty-seven isolates carried various hypervirulence genes and the profiles of these genes showed high diversity. Conclusions: The predominant carbapenemase of CRKP strains from paediatric patients in Shanghai were KPC-2 and NDM-5. KL47-ST11 KPC-2-KP and KL62-ST48 NDM-5-KP were representative clonal lineages. Although not prevalent, hypervirulence associated genes have begun to spread. Active long-term surveillance should be performed in both drug resistance characteristics and virulence factors. (C) 2020 The Authors. Published by Elsevier Ltd on behalf of International Society for Infectious Diseases.

关键词： Bronchoalveolar lavage   Fibre Optic Bronchoscopy   Immunocompromised   Children  

摘要： Purpose: The objective of this study was to describe the diagnostic yield and safety of bronchoalveolar lavage (BAL) in the evaluation of pulmonary lesions in immunocompromised children. Methods: We conducted a systematic review of literature published during the past 20 years, searching Medline, Medline EPub, EMBASE, and Scopus. Studies included involved paediatric patients (<18 years) on treatment for an oncological diagnosis or other immune compromise who underwent BAL for evaluation of pulmonary lesions. Only English language publications were included. Results: In all, 272 studies were screened and 19 included. All were observational studies with moderate (11/19) or serious (8/19) risk of bias. BAL yielded a potential pathogen in 43% of cases (496/1156). Two papers reported improved diagnostic yield with early BAL (less than 3 days of presentation). A change in patient management after BAL was reported in 53% of cases (275/519). Adverse events were reported in 19% of cases following BAL (193/993) but were generally mild with no procedure-related mortality reported. Conclusion: BAL appears to be useful for evaluation of pulmonary lesions in immunocompromised children with generally acceptable safety, though included studies had at least moderate risk of bias. Future prospective studies may provide more definitive estimates of benefit, timing and risk of BAL in this population. (C) 2020 Published by Elsevier Ltd.

关键词： Cardiomyopathy   paediatric cardiology   epidemiology   heart failure  

摘要： Background: Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these disorders could have on Latin American health systems, a comprehensive epidemiologic study regarding the clinical profile and sociodemographic characteristics of these patients will influence the way we approach paediatric cardiomyopathies. Methods: An observational retrospective study was conducted at a tertiary referral centre for Colombian and Latin American paediatric cardiology. We analysed all cases of primary cardiomyopathies in children younger than 18 years of age who presented at our institution between 2010 and 2016. Cases of cardiomyopathies were classified according to World Health Organization guidelines. Results: From a total of 29,533 children who attended our institution during the study period, 89 new cases of primary cardiomyopathies were identified. The median age at diagnosis was 11 years (interquartile range 4-9). Dilated cardiomyopathy accounted for 57.3% (n = 51) of cases;hypertrophic cardiomyopathy, 12.3% (n = 11);restrictive cardiomyopathy, 8.9% (n = 8);non-compacted cardiomyopathy, 7.8% (n = 7);arrhythmogenic ventricular cardiomyopathy, 6.7% (n = 6);and unspecified cardiomyopathy, 6.7% (n = 6). Heart failure was observed in 53.93% of the patients. The overall mortality was 12.36% (n = 11), which included two of eight patients who underwent cardiac transplantation.