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关键词： central venous catheter   clinical registry   paediatrics   peripherally inserted central catheter   quality care   vascular access  

摘要： Aim To describe practice evolution, complications and risk factors for multiple insertion attempts and device failure in paediatric central venous access devices (CVADs). Methods A paediatric retrospective cohort study using prospectively collected data from CVAD database 2012-2014. Data included were patient (i.e. age, condition), insertion (i.e. indication, device, technique) and removal (complications, dwell). Descriptive statistics and incidence rates were calculated per calendar year and compared. Risk factors for multiple insertion attempts and failure were explored with logistic regression and cox regression, respectively. Results A total of 1308 CVADs were observed over 273 467 catheter-days in 863 patients. Multiple insertion attempts remained static (14%) and significantly associated with non-haematological oncology (odds ratio 2.19;95% confidence interval (CI) 1.08-4.43), respiratory (3.71;1.10-12.5), gastroenterology (4.18;1.66-10.5) and other (difficult intravenous access) (2.74;1.27-5.92). CVAD failure decreased from 35% (2012) to 25% (2014), incidence rate from 1.50 (95% CI 1.25-1.80) to 1.28 (1.06-1.54) per 1000 catheter-days. Peripherally inserted CVAD failure was significantly associated with lower body weight (per kilogram decrease, hazard ratio (HR) 1.02;95% CI 1.00-1.03), cephalic vein (1.62;1.05-2.62), difficult access (1.92;1.02-3.73), sub-optimal tip placement (1.69;1.06-2.69) and gastroenterology diagnosis (2.27;1.05-4.90). Centrally placed CVAD failure was significantly associated with younger age (per year, HR 1.04;95% CI 1.00-1.07), tunnelled device (3.38;2.41-4.73) and gastroenterology diagnosis (1.70;1.06-2.73). Conclusions While advancement in CVAD practices improved overall CVAD insertion and failure outcomes, further improvements and innovation are necessary to ensure improved vessel health and preservation for children requiring CVAD.

关键词： Ovarian tumours   Paediatric age   Adnexectomy   Laparoscopy   Sparing surgery  

摘要： Introduction and objectives: Ovarian tumours are rare in childhood, and account for 1-5% of all tumours. The aim of this study is to determine the epidemiological features, histological subtypes, and therapeutic management of ovarian solid ovarian tumours of the paediatric population of the province of Cordoba, in Spain. Material and methods: A retrospective, descriptive, observational and institutional study was conducted in which a review was made of the clinical histories of patients younger than 14 years old diagnosed with ovarian tumours, excluding secondary tumours in a University Hospital between 1994 and 2017. A review was carried out on the age, clinical presentation, laterality, diagnostic methodology, treatment, histopathology, and evolution of these tumours. Results: A total of 37 ovarian tumours were reviewed in 31 patients, 6 of them being bilateral. The mean age was 10.3 (0-14) years, with 58% presenting as a palpable mass. There was no predominance of laterality. The tumour markers were negative. Conservative surgery was performed in 29.7% and adnexectomy in 70.3%. Only one case required post-operative adjuvant chemotherapy treatment (stage I immature teratoma with peritoneal gliomatosis). The histological study shows a predominance of germ cell tumours (65%) against those of epithelial lineage (22%). There were 3 stromal tumours that corresponded to fibroma (Gorlin syndrome), and bilateral gonadoblastoma associated with Frasier syndrome. The most frequent type of tumour was mature cystic teratoma (35.1%). There were no complications in the follow-up. Conclusions: Given that most childhood ovarian tumours are benign, conservative surgery is considered as the first choice, being even more important in bilateral tumours. If there is a family history, it is essential to carry out molecular genetic studies, to rule out associated syndromes. (C) 2020 Asociacion Espanola de Pediatria. Published by Elsevier Espana, S.L.U.

摘要： Methadone continues to be an important medication for the treatment of paediatric and adult cancer-related pain. Appropriate patient selection to ensure safe and effective treatment by a team of clinicians who appreciate and are familiar with methadone and its unique pharmacology is crucial. Unlike morphine and other more common opioids, methadone is purported to have involvement with delta-opioid receptor and higher affinity as an N-methyl-d-aspartate-receptor antagonist. Clinically this gives it the advantage of being effective for both nociceptive and neuropathic pain, but also may be useful in the setting of tolerance to other opioids. Methadone also comes in multiple available formulations that can be administrated through a variety of routes beyond the oral route. Challenges with methadone in treating cancer-related pain include drug interactions specifically as it relates to new targeted cancer therapies. Recent guidelines recommend electrocardiogram monitoring with methadone and there is potential for additive cardiac toxicity in the oncology setting. Appropriate dosing of methadone for pain management given age, organ dysfunction, and patients who are on methadone maintenance therapy are also key factors. This article aims to provide clinicians with evidence and clinical practice guidelines for safe and appropriate use of methadone including indication, initiation, and monitoring given its complexity for management of pain in the dynamic oncology setting.

关键词： adjuvant   gastroenteritis   racecadotril  

摘要： Acute diarrhoea is a leading cause of morbidity and mortality in the paediatric population. Racecadotril is an antisecretory drug recommended as an adjuvant antidiarrhoeal treatment. In the small bowel, the enzyme neutral endopeptidase (NEP) inhibits the action of enkephalins, which prevent water and electrolyte hypersecretion. By inhibiting NEP, racecadotril allows enkephalins to exhibit their antisecretory effects. Consequently, racecadotril reduces the secretion of water and electrolytes in the small intestine, without having an effect on intestinal motility. No serious adverse events related to racecadotril have been reported. Racecadotril has proven its efficacy as an adjuvant antidiarrhoeal drug with a good safety profile. Its addition to oral rehydration solution (ORS) appears clinically beneficial and potentially leads to health care savings.

关键词： Low birth weight   post-transplantation hypertension   kidney transplant   children  

摘要： Hypertension (HTN) remains a common complication after kidney transplantation among paediatric patients. Although low birth weight (LBW) has been implicated as an important risk factor for cardiovascular diseases, its effect on transplantation patients has not yet been addressed. It is essential to determine whether children with LBW who undergo transplantation are more likely to develop post-transplantation HTN. For this study, the medical records of 96 kidney recipients were retrospectively examined. A total of 83 patients fulfilled the inclusion criteria. Overall, post-transplantation HTN was observed in 54% of the recipients. Multivariate logistic regression revealed that time from transplantation >14 months (odds ratio (OR) 3.6;95% confidence interval (CI) 1.31-10.06;P = 0.013), current CKD (OR 2.6;95% CI 1.01-7.20;P = 0.045), presence of LBW (OR 3.6;95% CI 1.04-12.32;P = 0.044) and current overweight/obesity (OR 3.7;95% CI 1.02-13.91;P = 0.047) were associated with post-transplantation HTN. In conclusion, our data provide evidence for the first time that LBW is a significant predictive factor in the development of post-transplantation HTN. This finding has important clinical implications as it serves to alert clinicians about this additional risk factor in paediatric patients undergoing kidney transplant.

关键词： Bleb-sparing epithelial exchange   Bleb revision   Complete success   Qualified Success   Paediatric bleb revision   Long-term outcomes  

摘要： Purpose To evaluate the long-term outcome of 'bleb-sparing epithelial exchange' surgery for dysfunctional filtering blebs in paediatric and adult eyes. Methods Patients who had undergone bleb revision >= 5 years back and were on regular follow-up were included. Age, ocular diagnosis, details of primary filtering surgery including mitomycin-C (MMC) usage, indication and year of bleb revision were recorded. After bleb revision, the mean intraocular pressure (IOP), glaucoma medications and best corrected visual acuity (BCVA) up to 5 years were noted. On last review, clinical details, bleb characteristics and swept source anterior segment tomographic (SSOCT) assessment of bleb were recorded. Comparative analysis of paediatric and adult eyes was performed. Main outcome measures Change in IOP and BCVA. 'Complete success' was defined as IOP >= 6 to <= 18mmHg without use of any topical glaucoma medications and 'Qualified success' when >= 1 topical glaucoma medication(s) was required. Results A total of 51 eyes of 51 consecutive patients were studied, among which 22 were children. The mean duration between filtering surgery and bleb revision was 4.54 +/- 1.53 years in paediatric and 6.48 +/- 3.5 years in the adult group, p = 0.04. Children underwent trabeculotomy + trabeculectomy with 0.04% MMC, while adults underwent trabeculectomy with 0.02% MMC. The mean pre-revision IOP was 6.38 +/- 2.80 and 6.51 +/- 2.78 mmHg in the paediatric and adult group respectively, p = 0.86. At 3 months post-revision, it increased to 11.81 +/- 3.48 and 12.75 +/- 3.52 mmHg respectively (p < 0.001). At final review, mean IOP of paediatric group was 10.90 +/- 2.59 and adult group was 11.86 +/- 2.66 mmHg, p = 0.20. At 5 years, complete success was 68.18% and 72.41%, and qualified success was 31.87% and 27.59% in the former and latter group respectively, p = 0.49. No failures were seen. Kaplan-Meier probability at 5 years for IOP target <= 18, <= 15 and <= 12 in children was 95.45%, 63.64% and 50% an

关键词： Multidetector computed tomography   Paediatrics   Radiation exposure   Radiation protection  

摘要： Objectives To report on a dose survey conducted by the French societies of radiology and paediatric imaging (SFR and SFIPP) and the French public service expert in nuclear and radiological risks (IRSN), and to suggest new diagnostic reference levels (DRLs) for paediatric CT. Methods Dose reports concerning children aged 0-15 years and five CT procedures (brain, petrous bone, mediastinum, lung and abdomen-pelvis) performed in 2015-2016 were collected from 15 imaging departments on a voluntary basis. Volume CT dose index (CTDIvol) and dose-length product (DLP) were recorded for at least 10 patients per procedure, per centre and per age group. New DRLs were calculated as the 3rd quartiles of the distributions of the median values. Results Results from 3253 CT examinations were obtained. The exposure levels observed were much lower than for the previous surveys (2007-2008 and 2010-2013) and among the lowest values currently published. A good homogeneity between facilities was also observed. New DRLs are proposed for each procedure and age group. Conclusions This new survey contributes to the continuing optimisation process in French paediatric CT practice. Its results have been recently used to update the national paediatric CT DRLs.

摘要： Introduction The use of general anaesthesia in paediatric dentistry should be appropriate and justified. During the treatment of children, a clinician should have foresight into the future dental requirements of the patient and whether a future general anaesthetic will be required in quick succession. Efforts should be made when possible to avoid this. Aims This service evaluation was performed to gather information regarding repeat dental general anaesthetics within Sheffield Children's Hospital NHS Foundation Trust. Methods All children's records who had experienced a repeat dental general anaesthetic between 1 January 2015 and 31 December 2018 were analysed. Those who had experienced a repeat dental general anaesthetic within two years were investigated. Results Overall, 0.63% of children had a repeat dental general anaesthetic within a two-year period. These repeat dental general anaesthetics were mainly justified due to new dental caries. 43.9% had modifying factors, such as a complex medical history, that could have impacted the need for a second dental general anaesthetic. Conclusion The repeat rate in Sheffield Children's Hospital NHS Foundation Trust is low. We believe this is a result of specialist treatment planning and appropriate use of both 'extraction only' and 'restorative and extraction' GA lists.

关键词： AT   RT   Medulloblastoma   SMARCA4   SMARCB1   SWI   SNF  

摘要： Mutations in chromatin remodelling genes occur in approximately 25% of all human tumours (Kadoch et al. Nat Genet 45: 592-601, 2013). The spectrum of alterations is broad and comprises single nucleotide variants, insertion/deletions and more complex structural variations. The single most often affected remodelling complex is the SWI/SNF complex (SWItch/sucrose non-fermentable). In the field of paediatric neuro-oncology, the spectrum of affected genes implicated in epigenetic remodelling is narrower with SMARCB1 and SMARCA4 being the most frequent. The low mutation frequencies in many of the SWI/SNF mutant entities underline the fact that perturbed chromatin remodelling is the most salient factor in tumourigenesis and could thus be a potential therapeutic opportunity. Here, I review the genetic basis of aberrant chromatin remodelling in paediatric brain tumours and discuss their impact on the epigenome in the respective entities, mainly medulloblastomas and rhabdoid tumours.