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关键词： diabetes   organizational change   patient involvement   user involvement   utilization  

摘要： It is recommended that children and adolescents with diabetes are seen at paediatric diabetes clinics at least four times a year. This study examined whether an increased involvement of parents in planning the contacts would help them take more responsibility and thus lead to better metabolic control. Methods Patients with type 1 diabetes at the paediatric outpatient clinic at Lillebaelt Hospital were included in a before-after study. During the intervention, the parents were asked to contact the clinic whenever they needed help or wanted to book a consultation. HbA1c levels 1 year before, at the start of, and 1 year after the intervention were compared, as were the number of consultations and telephone contacts during the year prior to and during the intervention. Results 139 patients were included in the analyses. The mean HbA1c levels were 1 year before the start of the intervention (HbA1c-1) 61.8 mmol/mol (7.8%), and at the start of the intervention (HbA1c-2) 59.9 mmol/mol (7.6%) (P = .01), and after the end of the intervention (HbA1c-3) 63.1 mmol/mol (7.9%) (HbA1c-2 vs HbA1c-3: P = .0002). During the year prior to the intervention, the mean number of consultations was 5.8, and during the intervention period, the mean was 4.4 (P = .0001);the mean number of telephone calls was 1.8 and 3.8 (P < .0001), respectively. Conclusion Children were seen less often when parents were responsible for booking appointments, leading to higher HbA1c levels indicating that frequent consultations are essential for good metabolic control and cannot be replaced by frequent telephone contacts.

关键词： Atrial septal defect   ASD   optimal utilisation   standardisation  

摘要： Background: There is variation in care of secundum atrial septal defects. Defects <3 mm and patent foramen ovale are not clinically significant. Defects >3 mm are often followed clinically and may require closure. Variation in how these lesions are monitored may result in overutilisation of routine studies and higher than necessary patient charges. Purpose: To determine utilisation patterns for patients with secundum atrial septal defects diagnosed within the first year of life and compare to locally developed optimal utilisation standard to assess charge savings. Methods: This was a retrospective chart review of patients with secundum atrial septal defects diagnosed within the first year of life. Patients with co-existing cardiac lesions were excluded. Total number of clinic visits, electrocardiograms, and echocardiograms were recorded. Total charge was calculated based on our standard institutional charges. Patients were stratified based on lesion and provider type and then compared to "optimal utilisation" using analysis of variance statistical analysis. Results: Ninety-seven patients were included, 40 had patent foramen ovale (or atrial septal defect <3 mm), 43 had atrial septal defects not requiring intervention and 14 had atrial septal defects requiring intervention. There was a statistically significant difference in mean charge above optimal for these lesions of $1033, $2885, and $5722 (p < 0.02), respectively. There was statistically significant variation of charge among types of provider as well. Average charge savings per patient would be $2530 with total charge savings of $242,472 if the optimal utilisation pathway was followed. Conclusion: Using optimal utilisation and decreasing variation could save the patient significant unnecessary charges.

关键词： CNS cancer  

摘要： High-grade glioma (HGG) is highly resistant to therapy, prompting us to investigate the contribution of insulin-like growth factor receptor (IGF-1R), linked with radioresistance in other cancers. IGF-1R immunohistochemistry in 305 adult HGG (aHGG) and 103 paediatric/young adult HGG (pHGG) cases revealed significant association with adverse survival in pHGG, with median survival of 13.5 vs 29 months for pHGGs with moderate/strong vs negative/weak IGF-1R (p = 0.011). Secondly, we tested IGF-1R inhibitor BMS-754807 in HGG cells, finding minimal radiosensitisation of 2/3 aHGG cell lines (dose enhancement ratios DERs < 1.60 at 2-8 Gy), and greater radiosensitisation of 2/2 pHGG cell lines (DERs <= 4.16). BMS-754807 did not influence radiation-induced apoptosis but perturbed the DNA damage response with altered induction/resolution of gamma H2AX, 53BP1 and RAD51 foci. These data indicate that IGF-1R promotes radioresistance in pHGG, potentially contributing to the association of IGF-1R with adverse outcome and suggesting IGF-1R as a candidate treatment target in pHGG.

关键词： hippo signaling pathway   YAP   osteosarcoma   Ewing sarcoma  

摘要： Osteosarcoma and Ewing sarcoma are the most prevalent bone pediatric tumors. Despite intensive basic and medical research studies to discover new therapeutics and to improve current treatments, almost 40% of osteosarcoma and Ewing sarcoma patients succumb to the disease. Patients with poor prognosis are related to either the presence of metastases at diagnosis or resistance to chemotherapy. Over the past ten years, considerable interest for the Hippo/YAP signaling pathway has taken place within the cancer research community. This signaling pathway operates at different steps of tumor progression: Primary tumor growth, angiogenesis, epithelial to mesenchymal transition, and metastatic dissemination. This review discusses the current knowledge about the involvement of the Hippo signaling pathway in cancer and specifically in paediatric bone sarcoma progression.

关键词： Parents' questions   Paediatric healthcare   Hearing impairment   Conversation Analysis   Clinician-patient interaction  

摘要： Objective: To examine parents' questions to clinicians and how clinicians respond in paediatric hearing healthcare appointments with children with hearing loss. Methods: The data consisted of 48 video-recorded hearing habilitation appointments. Participants included 22 clinicians, 41 children, and 48 parents/carers. Data were analysed using conversation analysis. Results: A total of 89 questions from parents were directed to the clinicians in the appointments. Parents' questions were mostly designed as polar (yes/no) questions. The questions covered several action and topical agendas, and in most instances functioned as something other than merely soliciting information. The most prominent action agenda of parents' questions involved questions that displayed parental concern. These questions sought reassurance as well as information from the clinician. Conclusion: While parents in this study did not ask a large number of questions during the appointments their questions performed important functions. Parents' questions cannot be assumed to be only about seeking information. Often parents' questions are in pursuit of reassurance from the clinician about their child's progress. Practice Implications: Clinicians may need to provide encouragement to parents to ask questions during appointments. As part of delivering family-centred care, clinicians need to be aware that their responses may need to go beyond information provision. (C) 2019 Elsevier B.V. All rights reserved.

关键词： Mild traumatic brain injury   concussion   knowledge   symptoms   terminology  

摘要： Background: Traumatic brain injury (TBI) is common among children. However, their caregivers' knowledge and understanding of symptoms may influence how the injury is managed. Primary objective: To investigate the knowledge of New Zealand (NZ) parents about TBI and concussion. Method and procedures: Parents (n = 205) of children aged 5-13 years completed a pen-and-paper or online survey containing questions examining their knowledge of TBI terminology, TBI symptoms and knowledge about concussion management. Main outcomes and results: A high proportion (61%) of parents did not think that a concussion was the same as a brain injury. Loss of consciousness (LOC) was the most endorsed symptom of TBI. However, 69% of participants were aware that TBI could occur without LOC. On average, parents correctly identified 19.5 (67.3%) of the 29 symptoms of TBI, but also identified 2.0 (11.9%) of the 17 distractor symptoms as being TBI related. Demographic factors and experience of TBI/concussion were associated with TBI symptom identification accuracy and concussion knowledge. Conclusions: Further education of parents is needed to ensure they recognise the signs and symptoms of concussion/mild TBI so that they can make informed decisions on how best to manage their child's injury.

关键词： fluid maintenance   fluid overload   mechanical ventilation   oedema   renal replacement therapy  

摘要： Aim We evaluated the influence of early fluid overload on critically ill children admitted to a paediatric intensive care unit by examining mechanical ventilation (MV), mortality, length of stay and renal replacement therapy. Methods This retrospective cohort study covered January 2015 to December 2016 and focused on all episodes of MV support that exceeded 24 hours. The fluid overload percentage (FO%) was calculated daily for the first 72 hours and we estimated its effect on outcomes. Results We included 186 MV episodes in 154 patients. The median age was 13.8 months, with an interquartile range (IQR) of 3.8-34.0 months, and the mortality rate was 12.4%. The median FO% in the first 72 hours was 8.0% (IQR 3.6%-11.2%). An FO% of >= 10% was associated with higher ventilatory parameters, namely peak inspiratory pressure (P = .023) and positive end expiratory pressure (P = .003), and renal replacement therapy (P = .02) and higher mortality (8.8% vs 19.7%). In a multivariate Cox regression model, FO >= 10% at 72 hours was independently associated with longer MV support, but not mortality (P = .001). Conclusion In a heterogeneous paediatric population given MV, an early cumulative FO of >= 10% was associated with more aggressive ventilatory parameters and prolonged length of MV, but not mortality.

关键词： Paediatric meningioma  

摘要： Paediatric meningiomas are extremely rare. These tumours constitute only 2 to 3% of all childhood brain tumours. Despite similarities in histological features between PMs and their adult counterparts, there are important distinctions between them. In this case series, the authors describe their experience in paediatric meningiomas in Singapore's 2 children's hospitals from 1998 to 2018. The primary aim of this retrospective study is to evaluate the clinical, radiological and pathological characteristics, and associated outcomes of paediatric patients diagnosed with meningioma managed in our local institutions. Following that, the study's findings are secondary aims are corroborated with published literature. A total of 10 patients (4 males and 6 females) were identified for this study within the period of 01 January 1998 to 31 December 2018. Their ages ranged from 1 year old to 18 years old (median age 10.5 years old). Two of the patients had NF1 and NF2 respectively. There were 9 intracranial and 1 intraspinal paediatric meningiomas. Seven patients achieved gross total resection and 3 patients had subtotal resection. Eight patients did not have tumour recurrence or increase in size of tumour remnant during the course of their follow-up. In congruency with the literature, up to 40% of our patients had higher grade meningiomas and 55.6% had large tumour volumes more than 30 cm(3). Owing to the paucity of knowledge for this unusual tumour, the authors emphasize the need for closer surveillance and in-depth genomic studies to identify novel therapies for this challenging condition. (C) 2020 Elsevier Ltd. All rights reserved.

关键词： Duplicate   Vas deferens   Herniotomy   Spermatic cord  

摘要： Background Congenital abnormalities of the vas deferens are very uncommon. Duplicate vas deferens is a rare abnormality. It is the presence of two separate vasa deferentia within one spermatic cord. It has been encountered during inguinal hernia repair, orchidopexy, varicocoelectomy, vasectomy and radical prostatectomy. Identification of the vas deferens is mandatory during surgeries involving manipulation of the spermatic cord because if duplication exists and is not detected, there is increased chance of iatrogenic injury. Case presentation We present a 3-year-old boy with duplicate vas deferens identified during herniotomy for a right hydrocele. A postoperative abdominopelvic ultrasound scan revealed no abnormalities. Conclusion The risk of iatrogenic injury to the vas is increased in the presence of a duplication. A deliberate identification of the vas during procedures involving manipulation of the spermatic cord reduces this risk.

关键词： Paediatric glaucoma   Paediatric ophthalmology   Glaucoma surgery   Service planning   Scotland  

摘要： Background The primary aim was to estimate the incidence of primary and secondary childhood glaucoma in Scotland over a 2-year period. The secondary aim was to gauge the confidence and experience of ophthalmologists in Scotland in managing these patients. Methods A 7 question electronic survey was distributed to all consultant members of the Scottish Paediatric Club and Scottish Glaucoma Club. Respondents were asked to report the number of cases and types of childhood glaucoma they had managed in the last 2 years. Respondents were also asked about experience and confidence in a range of glaucoma procedures, number of patients requiring referral to specialist centres and interest in the development of a centre of excellence in Scotland. Results The survey returned a 56% response rate, reporting 85 new cases of paediatric glaucoma in Scotland over the preceding 2 years. 11 (12.9%) had primary glaucoma and 74 (87.1%) had secondary glaucoma. The most common subtype of secondary glaucoma was uveitic glaucoma (n = 29). None of the respondents declared confidence or experience in trabeculotomy or goniotomy procedures. Eleven children required referral to a specialist unit outside Scotland. 85.7% of respondents felt Scotland would benefit from a specialist unit for paediatric glaucoma. Conclusions This survey reflects an appetite for a specialist service for paediatric glaucoma in Scotland. However, further consideration is needed to determine if there is sufficient patient load to maintain such a service.