教学课程资源库 更多>>

关键词： Children   Aortic valve disease   Decellularization   Allografts  

摘要： OBJECTIVES: Options for paediatric aortic valve replacement (AVR) are limited if valve repair is not feasible. Results of paediatric Ross procedures are inferior to adult Ross results, and mechanical AVR imposes constant anticoagulation with the inherent risks. METHODS: The study design was a prospective, multicentre follow-up of all paediatric patients receiving decellularized aortic homografts (DAHs) for AVR in 8 European centres. RESULTS: A total of 106 children (77 boys) were operated (mean age 10.1 +/- 4.8 years, DAH diameter 20.5 +/- 3.8 mm). A total of 60 (57%) had undergone previous surgical interventions: 34 with 1, 15 with 2 and 11 with >3. There was one early death in a 12-year-old girl, who underwent her fourth aortic valve operation, due to intracerebral haemorrhage on extracorporeal membrane oxygenation after coronary reimplantation problems following 3-sinus reconstruction 1 year earlier. One 2-year-old patient died due to sepsis 2 months postoperatively with no evidence for endocarditis. In addition, a single pacemaker implantation was necessary and a 2.5-year-old girl underwent successful HTx due to chronic myocardial failure despite an intact DAH. After a mean follow-up of 3.30 +/- 2.45 years, primary efficacy end points mean peak gradient (18.1 +/- 20.9 mmHg) and regurgitation (mean 0.61 +/- 0.63, grade 0-3) were very good. Freedom from death/explantation/endocarditis/bleeding/stroke at 5 years was 97.8 +/- 1.6/85.0 +/- 7.4/100/100/100% respectively. Calculated expected adverse events were lower for DAH compared to cryopreserved homograft patients (mean age 8.9 years), lower than in Ross patients (9.4 years) and in the same range as mechanical AVR (12.8 years). CONCLUSIONS: Even though the overall number of paediatric DAH patients and the follow-up time span are still limited, our data suggest that DAHs may present a promising additional option for paediatric AVR.

关键词： Children   Diagnostic imaging   Treatment   Urinary stones  

摘要： Background Prompt diagnosis and treatment of paediatric urolithiasis are required to avoid long term sequelae of renal damage. Objective To systematically review the literature regarding the diagnostic imaging modalities and treatment approaches for paediatric urolithiasis. Study design PubMed, Science Direct, Scopus and Web of Science were systematically searched from January 1980-January 2019. 76 full-text articles were included. Results Ultrasound and Kidney-Ureter-Bladder radiography are the baseline diagnostic examinations. Non-contrast Computed Tomography (CT) is the second line choice with high sensitivity (97-100%) and specificity (96-100%). Magnetic Resonance Urography accounts only for 2% of pediatric stone imaging studies. Expectant management for single, asymptomatic lower pole renal stones is an acceptable initial approach, especially in patients with non-struvite, non-cystine stones<7 mm. Limited studies exist on medical expulsive therapy as off-label treatment. Extracorporeal shock wave lithotripsy (SWL) is the first-line treatment with overall stone free rates (SFRs) of 70-90%, retreatment rates 4-50% and complication rates up to 15%. Semi-rigid ureteroscopy is effective with SFRs of 81-98%, retreatment rates of 6.3-10% and complication rates of 1.9-23%. Flexible ureteroscopy has shown SFRs of 76-100%, retreatment rates of 0-19% and complication rates of 0-28%. SFRs after first and second-look percutaneous nephrolithotomy (PNL) are 70.1-97.3% and 84.6-97.5%, respectively with an overall complication rate of 20%. Open surgery is seldom used, while laparoscopy is effective for stones refractory to SWL and PNL. Limited data exist for robot-assisted management. Conclusions In the initial assessment of paediatric urolithiasis, US is recommended as first imaging modality, while non-contrast CT is the second option. SWL is recommended as first line treatment for renal stones <20 mm and for ureteral stones<10 mm. Ureteroscopy is a feasible alternative both f

关键词： adenotonsillectomy   anaesthetic drugs   emergence agitation   network meta-analysis   randomized control trial  

摘要： What is known and objective The comparative efficacy of ancillary drugs on sevoflurane-related emergence agitation (EA) in paediatric anaesthesia for adenotonsillectomy remains unclear. The purpose of this Bayesian network meta-analysis was to investigate the efficacy of ancillary drugs on sevoflurane-related EA in paediatric anaesthesia for adenotonsillectomy. Methods MEDLINE, Embase, the Cochrane Library and Web of Science databases were electronically searched to identify randomized controlled trials (RCTs) of different ancillary drugs used in adenotonsillectomy from inception to April 2019. Two reviewers independently screened the literature, extracted data and assessed the risk of bias in included studies. Subsequently, a network meta-analysis was performed using the R software and RevMan 5.3 software. Results and discussion We included 25 RCTs, involving 2151 participants. The proportion of patients with sevoflurane-related EA was significantly lower in the dexmedetomidine, ketamine, propofol, fentanyl, midazolam, sufentanil, remifentanil and clonidine groups than in the placebo group (P < .05). Fentanyl was superior to sufentanil (P < .05), whereas dexmedetomidine was superior to fentanyl (P < .05). Among ancillary drugs, dexmedetomidine (90.04%) showed the highest possibility of reducing the risk of EA, followed by fentanyl (87.45%), remifentanil (63.85%), ketamine (52.07%), midazolam (51.27%), clonidine (49.94%), propofol (29.89%), sufentanil (21.38%) and placebo (4.09%). What is new and conclusion Evidence suggests that the effects of dexmedetomidine in reducing the risk of sevoflurane-related EA in paediatric anaesthesia for adenotonsillectomy were better than the effects of other drugs. However, large, high-quality RCTs are required to further confirm this.

关键词： massive transfusion protocols   paediatrics   trauma outcomes   systematic review  

摘要： Background Our main objective was to review the literature to answer the following questions regarding paediatric massive transfusion (PMT) protocols: (a) How is PMT defined?;(b) Which blood product ratios have been investigated, and what is their effect on outcomes?;and (c) What evidence exists regarding PMT outcomes? Methods The PRISMA guidelines were used. We searched PubMed, Google Scholar, Cochrane Library, EMBASE, Wiley Online Library and Ovid. Articles were screened for inclusion based on relevance to PMT. Articles were assessed for study design, presence of established/tested PMT, PMT definition, PMT activation criteria and Transfusion Ratios for the final determination of article inclusion. Results Our search produced 3213 articles, with 33 included for final review. Existing definitions of PMT are based on volume administered/kg but vary in timeframe criteria (over 4 hours vs 24 hours). Some studies have investigated "high" balanced transfusion ratios as seen in adults (1:1 FFP:pRBC), with a few showing statistically significant improvement in paediatric mortality vs lower ratios. PMT protocol implementation has not been shown to consistently reduce paediatric trauma mortality across multiple centres. However, other operational aspects, such as reduced time to first transfusion, are apparent benefits. Conclusions There is poor consensus over the definition of PMT. Definitions that involve early recognition have the most promise for practice and future studies. Evidence supporting an optimal blood product ratio in PMT is also lacking but trends towards supporting balanced approaches. Implementation of PMT protocols has been limited in showing significant improvement of overall paediatric trauma mortality but may reduce associated morbidity.

关键词： metanephric stromal tumour   partial nephrectomy   rare tumours   renal tumours   robotic  

摘要： Background Perivascular epithelioid cell tumours (PEComas) represent a rare group of mesenchymal tumours with an unpredictable outcome. They are usually observed in middle-aged women whilst they are very rare in children, with limited cases described. Due to the rare anatomical location in kidneys, a correct diagnosis and treatment is really challenging. Methods We observed and treated a case of kidney's PEComa in a 14-year-old boy. Results An individualized approach based on anatomical (3D CT-reconstructions) and histopathological (US-guided preliminary biopsy) features lead us to perform a successful robotic-sparing surgery enabling the preservation of two-thirds of the kidney involved. Conclusions A meticulous preoperative planning in selected patients can lead to a minimally invasive approach even in some paediatric kidney's neoplasms.

关键词： children   emergency care   health promotion  

关键词： ATP5L-KMT2A   acute lymphoblastic leukemia   t(11)(q23.3)   reciprocal KMT2A fusion   MLL  

关键词： adolescents   dermatofibrosarcoma protuberans   paediatric  

摘要： Background As dermatofibrosarcoma protuberans (DFSP) are rare with no prospective series within paediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of DFSP enrolled in a multinational study of non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). Patients and methods Forty-six patients with confirmed DFSP were enrolled into the EpSSG NRSTS 2005 study. All had surgical resection and none had any further therapy at diagnosis. Results The median age at diagnosis was 6.9 years (range 0.4-17.5). All patients had localised disease, and the majority had small <5 cm tumours (93%), and 76% had Intergroup Rhabdomyosarcoma Study (IRS) I tumours. All patients had up front surgery, 32 requiring two operations. There were 11 patients with IRS II tumours, of which only two went on to have a local recurrence. After a median follow up of 49.0 months (range 4.2-130.9), all patients were alive at the time of this report, with 5-year event-free survival of 92.6% (CI 78.8-97.6) with a 100% overall survival. Conclusion This report demonstrates the ability to run prospective paediatric studies in NRSTS in multiple European countries, with reasonable numbers of DFSP patients, with few events and no deaths, and hence excellent outcomes.

关键词： burn debridement   enzymatic debridement   escharotomy   NexoBrid   paediatric burns  

摘要： NexoBrid (NXB) has been proven to be an effective selective enzymatic debridement agent in adults. This manuscript presents the combined clinical trial experience with NXB in children. Hundred and ten children aged 0.5 to 18 years suffering from deep thermal burns of up to 67% total body surface area were treated with NXB in three clinical trials. Seventy-seven children were treated with NXB in a phase I/II study, where 92.7% of the areas treated achieved complete eschar removal within 0.9 days from admission. Thirty-three children (17 NXB, 16 standard of care [SOC]) participated in a phase III randomized controlled trial. All wounds treated with NXB achieved complete eschar removal. Time to complete eschar removal (from informed consent) was 0.9 days for NXB vs 6.5 days for SOC (P < .001). The incidence of surgical excision was 7.9% for NXB vs 73.3% for SOC (P < .001). Seventeen of these children participated in a phase III-b follow-up study (9 NXB and 8 SOC). The average long-term modified Vancouver Scar Scale scores were 3.4 for NXB-treated wounds vs 4.4 for SOC-treated wounds (NS). There were no significant treatment-related adverse events. Additional studies are needed to strengthen these results.

关键词： Tablet(s)   Analysis   Bioavailability   Biopharmaceutics classification system (BCS)   Buccal delivery   Caco-2 cells   Computational ADME  

摘要： Tuberculosis (TB) is believed to affect around 10 million people worldwide. Treatment for TB includes isoniazid and rifampicin, with fixed-dose combination (FDC) recommended for improved patient compliance. Similarly, orally disintegrating tablets (ODTs) are an increasingly popular dosage form that aid compliance since they do not require swallowing. In this study ODTs of isoniazid and rifampicin, either as discrete or FDC doses, were formulated and bioequivalence between single and combination doses compared using in vitro and in silico approaches. Dissolution profiles were compared using FDA advised difference (f(1)) and similarity (f(2)) testing in biorelevant media. Rifampicin release from FDCs decreased by approximately 15% in fed-state media (failed f(1) and f(2) ), which was attributed to enhanced rifampicin degradation in the presence of isoniazid at lower pH. Apparent permeability (P-app) values derived from Caco-2 transport studies were included alongside dissolution results into a physiologically based pharmacokinetic (PBPK) model, to simulate in vivo bioavailability in healthy subjects. Models showed no difference in bioavailability between formulations or dosing (fasted or fed) state, despite the failures in dissolution-based bioequivalence testing, highlighting shortcomings in f(1) and f(2) assessment and the strength of PBPK models. (C) 2020 American Pharmacists Association (R). Published by Elsevier Inc. All rights reserved.