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摘要： Background Lichen sclerosus (LS) is a chronic inflammatory dermatosis with a predilection for the anogenital region, which mainly affects prepubertal girls and postmenopausal women. The cause is unknown, but a number of potential aetiological factors have been identified. Aim To examine a cohort of patients with prepubertal-onset vulval LS (VLS) and assess baseline characteristics, clinical presentation, potential precipitating and predisposing factors, and response to treatment. Methods Data were collected from case notes on patients aged VLS attending a specialist vulval dermatology service. Data included clinical presentation, comorbidities, family history, therapy and response to treatment. Results In total, 26 paediatric patients were identified. The median age at onset of symptoms was 5 years (range 2-8.5 years). Many previously identified potential aetiological factors for the development of VLS were identified, including family history, trauma, autoimmune disease and hormonal factors. A significant proportion of patients had a history of urinary tract symptoms, including incontinence and urinary tract infection. Most patients responded well to a standard course of induction topical therapy followed by maintenance therapy, but some, including three patients with ongoing urinary incontinence and three postpubertal patients, continued to have active disease. Conclusion A detailed assessment is essential in patients with VLS so that potential predisposing factors and comorbidities can be identified and managed. Urinary incontinence may be implicated in the development of paediatric VLS and may prevent adequate disease control. Paediatric VLS can persist through puberty, thus long-term follow-up is advised.

关键词： Intrathoracic lesion   Children   Bronchoscopy   Paediatrics   Endobronchial ultrasound  

摘要： Introduction: In adults, linear endobronchial ultrasound (EBUS) is the preferred modality to sample intrathoracic adenopathy and radial EBUS is a useful tool to biopsy peripheral pulmonary nodules. Utility in children is less well known. Objectives: The objective of this study was to review the EBUS experience of two specialist centres to better define the current role of EBUS in paediatric practice. Methods: A retrospective record review of EBUS procedures undertaken in patients aged 0-17 years at the Royal Children's Hospital (Melbourne, Australia) and Thoraxklinik (Heidelberg, Germany) was performed. Data extracted included patient demographics, clinical presentation, bronchoscope (size and model), EBUS technique used, pathologic results, need for further invasive investigation, and complications. Results: Between 2008 and 2017, ten EBUS procedures were performed (6 linear EBUS and 4 radial EBUS). No complications were reported. Linear EBUS was performed on subjects who were between 4 and 15 years old, with a 100% diagnostic yield. Radial EBUS was non-diagnostic in three cases of non-malignant disease. In one case, it was used successfully for imaging alone. Conclusion: Both linear and radial EBUS are safe and feasible in children. Diagnostic yield of linear EBUS was 100%. Radial EBUS did not demonstrate utility, likely reflecting the pathologies of underlying parenchymal masses in paediatric populations.

关键词： arteriovenous fistula   arteriovenous graft   central venous line   children   haemodialysis  

摘要： Background. There are three principle forms of vascular access available for the treatment of children with end stage kidney disease (ESKD) by haemodialysis: tunnelled catheters placed in a central vein (central venous lines, CVLs), arteriovenous fistulas (AVF), and arteriovenous grafts (AVG) using prosthetic or biological material. Compared with the adult literature, there are few studies in children to provide evidence based guidelines for optimal vascular access type or its management and outcomes in children with ESKD. Methods. The European Society for Paediatric Nephrology Dialysis Working Group (ESPN Dialysis WG) have developed recommendations for the choice of access type, pre-operative evaluation, monitoring, and prevention and management of complications of different access types in children with ESKD. Results. For adults with ESKD on haemodialysis, the principle of "Fistula First" has been key to changing the attitude to vascular access for haemodialysis. However, data from multiple observational studies and the International Paediatric Haemodialysis Network registry suggest that CVLs are associated with a significantly higher rate of infections and access dysfunction, and need for access replacement. Despite this, AVFs are used in only similar to 25% of children on haemodialysis. It is important to provide the right access for the right patient at the right time in their life-course of renal replacement therapy, with an emphasis on venous preservation at all times. While AVFs may not be suitable in the very young or those with an anticipated short dialysis course before transplantation, many paediatric studies have shown that AVFs are superior to CVLs. Conclusions. Here we present clinical practice recommendations for AVFs and CVLs in children with ESKD. The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system has been used to develop and GRADE the recommendations. In the absence of high quality evidence, the opinion of expert

关键词： Acute lymphoblastic leukaemia   Endovascular thrombectomy   Endovascular clot retrieval   Hyperviscosity  

摘要： Optimal treatment for ischaemic stroke in paediatric acute lymphoblastic leukaemia (ALL) is unclear. We describe an ischaemic stroke in an adolescent with ALL who underwent successful endovascular thrombectomy following leukaemic arterial occlusion. Endovascular thrombectomy should be considered in paediatric ALL patients with acute ischaemic stroke and large vessel occlusion. (C) 2019 Elsevier Ltd. All rights reserved.

关键词： cerebral palsy   emergency department use   feeding tube  

摘要： Aim To describe the characteristics of emergency department (ED) presentations due to complications from gastrostomy or gastrojejunal feeding tubes among children with cerebral palsy (CP), the complexity of complications and the management approaches taken. Methods The Victorian CP Register was linked to the ED databases of Victoria's two tertiary paediatric hospitals, and data on presentations due to feeding tube complications were identified based on discharge diagnosis codes. Additional data on presentations were extracted from medical records. Results Over 5 years, there were 234 ED presentations due to feeding tube-related complaints among a CP cohort (n = 2183). ED notes were located for 183 of the 234 presentations. The majority of presentations (90%) involved children with severe gross motor impairment. A total of 46% of presentations (n = 84) was triaged as lower urgency, and 68% (n = 124) took place between 08:00 am and 06:00 pm. The most common presenting complaint was tube dislodgement (n = 105;70%). No investigations were recorded in the majority of cases, and in almost 90% of cases, the feeding tube was successfully replaced in the ED, usually by an ED physician (n = 74) and less frequently by a surgeon (n = 9), gastroenterologist (n = 2) or nurse (n = 8);9% (n = 17) resulted in a hospital admission. Conclusions Most ED presentations due to feeding tube complaints in children with CP are in children with severe gross motor impairment but are able to be managed in the ED. As such, it is likely that care givers and other health professionals could manage some of the complications experienced in primary health-care settings closer to home.

关键词： catheterisation   child   evidence-based medicine   hospitals   paediatric   peripheral venous device  

摘要： Aim To improve paediatric peripheral intravenous catheter (PIVC) care through the implementation of care bundles. Methods A pre-post study using mixed methods (clinical audit, staff survey, parent interviews) in 2016 at a tertiary paediatric hospital in Brisbane was conducted to evaluate the effectiveness of a PIVC insertion and maintenance bundle to improve PIVC insertion, promote function and support practice. Participants included children with PIVC, parents and staff inserting and managing PIVCs. A mnemonic care bundle, SUCCESS PIVCS (At insertion: Skills, Understand and prepare, Consent, Clean site, Escalate, Secure, Sign and document. During management: Prompt removal, Inspect hourly, Vein patency, Clean hands and Scrub the hub), was developed and implemented via visual aids, workshops and change champions. During audit, PIVC first-attempt insertion success, PIVC failure, PIVC dwell, escalation to senior clinicians and insertion and management procedures were measured. Results Pre-implementation audit (n = 102) and survey (n = 117) data described high rates of PIVC failure (n = 50;49%), difficulty obtaining equipment (n = 64;55%) and pressure to insert (n = 50;43%). Parent interviews (n = 15) identified lack of communication, fear, appreciation of skilled technicians and technology and care giver roles as key to improving the experience. After implementation first-attempt insertion success (45 vs. 62%;risk ratio 1.37, 95% confidence interval 1.05-1.78), first-attempt escalation to senior clinicians (junior doctor 72 vs. 41%;P = <0.001) and median PIVC dwell (40 vs. 52 h;P = 0.021) improved. Conclusion This multi-level care bundle demonstrated improvements in the insertion and management of PIVCs;however, PIVC failure remained high.

关键词： Biological Agents   CNTO 1275   Weight Gain   Psoriasis   Survival Rate   Adalimumab   Biological Therapy   Etanercept   Child  

摘要： Background Three biotherapies - etanercept, adalimumab and ustekinumab - are licensed in childhood psoriasis. The few data available on their efficacy and tolerance are mainly derived from industry trials. However, biological drug survival impacts long-term performance in real-life settings. Objective The objective of this study was to evaluate the survival rates of biological therapies in children with psoriasis in real-life conditions. Secondary objectives were to evaluate the factors associated with the choice of the biological therapy and to report severe adverse events. Materials and methods This study was an observational retrospective study. Data were extracted from the clinical records of 134 children. Kaplan-Meier estimates were used to analyse drug survival overall and in subgroups of plaque psoriasis, bio-naive and non-naive patients. Results We analysed 184 treatment courses: 70 with etanercept, 68 with adalimumab and 46 with ustekinumab. Factors associated with the choice of first-line biological agent were age at initiation (younger for adalimumab, P < 0.0001), age at onset of psoriasis (younger for adalimumab and etanercept, P = 0.03) and baseline Psoriasis Assessment Severity Index and Physician global assessment (both higher for adalimumab, P < 0.001). Drug survival rates were higher for ustekinumab than for adalimumab and etanercept (P < 0.0001) for all treatment and all psoriasis types, plaque-type psoriasis (P = 0.0003), patients naive for biological agents (P = 0.0007) and non-naive patients (P = 0.007). We reported eight serious adverse events (SAEs): severe infections (n = 3), significant weight gain (n = 2), psoriasis flare (n = 1) and malaise (n = 1). Biological therapy was discontinued in three children (one with psoriasis flare and two with weight gain). Only the two cases of weight gain resulted in an unfavourable outcome. Conclusions Our real-life comparative study found that ustekinumab had the best drug survival outcome. The profile of

关键词： Alveolar rhabdomyosarcoma   Children   Computed tomography   Embryonal rhabdomyosarcoma   Magnetic resonance imaging   Rhabdomyosarcoma  

摘要： Rhabdomyosarcoma is the most common soft-tissue sarcoma in children. The most common sites are head and neck, genitourinary tract and extremities. In this review we outline the clinical and radiologic features of paediatric rhabdomyosarcoma, as well as imaging considerations and imaging of relapse.

关键词： Pyoderma   Scabies   Skin infections   Fungal diseases   Staphylococcus aureus   Medical risk factors   Seasons   Group A streptococcal infection  

摘要： Background Scabies is a WHO neglected tropical disease common in children in low- and middle-income countries. Excoriation of scabies lesions can lead to secondary pyoderma infection, most commonly by Staphyloccocus aureus and Streptococcus pyogenes (group A streptococcus, GAS), with the latter linked to acute post-streptococcal glomerulonephritis (APSGN) and potentially rheumatic heart disease (RHD). There is a paucity of data on the prevalence of these skin infections and their bacterial aetiology from Africa. Methodology/Principal findings A cross-sectional study, conducted over a four-month period that included the dry and rainy season, was conducted to determine the prevalence of common skin infections in Sukuta, a peri-urban settlement in western Gambia, in children <5 years. Swabs from pyoderma lesions were cultured for S. aureus and GAS. Of 1441 children examined, 15.9% had scabies (95% CI 12.2-20.4), 17.4% had pyoderma (95% CI 10.4-27.7) and 9.7% had fungal infections (95% CI 6.6-14.0). Scabies was significantly associated with pyoderma (aOR 2.74, 95% CI 1.61-4.67). Of 250 pyoderma swabs, 80.8% were culture-positive for S. aureus, and 50.8% for GAS. Participants examined after the first rains were significantly more likely to have pyoderma than those examined before (aRR 2.42, 95% CI 1.38-4.23), whereas no difference in scabies prevalence was seen (aRR 1.08, 95% CI 0.70-1.67). Swab positivity was not affected by the season. Conclusions/Significance High prevalence of scabies and pyoderma were observed. Pyoderma increased significantly during the rainy season. Given the high prevalence of GAS pyoderma among children, further research on the association with RHD in West Africa is warranted.