​Author(s): Doherty C.; Bowler M.; Sadadcharam M.; Perkins R.; Bateman N.; Bruce I.A.; Monks S.; English C.; Atkinson D.; McGrath B.
Source: Clinical Otolaryngology; Apr 2018; vol. 43 (no. 2); p. 674-678
Publication Date: Apr 2018
Publication Type(s): Letter
Available  at Clinical Otolaryngology -  from Wiley Online Library Medicine and Nursing Collection 2018 - NHS
Database: EMBASE

Author(s): Patel L.B.; Stuart-Booth S.; Rakshi K.
Source: Archives of Disease in Childhood; Mar 2018; vol. 103
Publication Date: Mar 2018
Publication Type(s): Conference Abstract
Available  at Archives of Disease in Childhood -  from BMJ Journals - NHS
Abstract:Aims The incidence of autism in the UK is predicted at 0.1%; 1 00 000 children and 7 00 000 adults. Diagnosis of Autism Spectrum Disorder (ASD) varies regionally though national standards are outlined; NICE Clinical guideline (CG128) and Quality standard (QS51). 
Database: EMBASE

Author(s): Patel L.B.; El Gamal M.; Murray D.; Nair P.
Source: Archives of Disease in Childhood; Mar 2018; vol. 103
Publication Date: Mar 2018
Publication Type(s): Conference Abstract
Available  at Archives of Disease in Childhood -  from BMJ Journals - NHS
Abstract:Aims 34,000 Children and Young People (CYP) in England have a diagnosis of epilepsy and receive anti-epileptic drugs. NICE have described high-priority areas for quality improvement; The Epilepsy in CYP, Quality Standard (QS27). Our local service aims to provide an excellent multidiscipli-nary service to serve its patients and their families in a holistic manner. It is delivered by consultants, a specialist nurse, rotating junior doctors and the nursing team. 
Database: EMBASE
 

​Author(s): Armer J.; De Goede C.
Source: Archives of Disease in Childhood: Education and Practice Edition; Dec 2017; vol. 102 (no. 6); p. 319-326
Publication Date: Dec 2017
Publication Type(s): Article
Available  at Archives of Disease in Childhood: Education and Practice Edition -  from BMJ Journals - NHS
Abstract:In paediatrics, one of our main aims in the diagnostic process is to identify any treatable conditions. The copper metabolism disorder Wilson's disease (WD) is one such condition that is caused by mutations in the ATP7B gene. Delay in treatment could result in irreversible disability or even death. Although liver disease is the most common presenting feature in children, some children may initially present with a subtle neurological presentation only. In patients presenting with dystonia, tremor, dysarthria or with a deterioration in school performance, there should be a high index of suspicion for WD. However, the differential of these clinical presentations is wide and exclusion of WD is difficult. No single diagnostic test can exclude WD and each of the biochemical tests has limitations. In this article, we discuss copper metabolism disorders including WD and Menke's disease. We then discuss the available diagnostic tests and how to investigate children for these rare disorders. Copyright © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved.
Database: EMBASE

Author(s): Dwan K.; Kirkham J.; Paton R.W.; Morely E.; Newton A.W.; Perry D.C.

Source: Cochrane Database of Systematic Reviews; Jul 2017; vol. 2017 (no. 7)

Publication Date: Jul 2017

Publication Type(s): Article

Abstract:This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To determine the role of splinting and the optimal treatment strategy for the non-operative management of DDH in children under six months of age. To identify if there are particular subgroups of patients for whom the optimal management strategy may differ.Copyright © 2017 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
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Database: EMBASE

Author(s): Kew K.M.; Carr R.; Crossingham I.

Source: Cochrane Database of Systematic Reviews; Apr 2017; vol. 2017 (no. 4)

Publication Date: Apr 2017

Publication Type(s): Review
​
Abstract:Background: Adolescents with asthma are at high risk of poor adherence with treatment. This may be compounded by activities that worsen asthma, in particular smoking. Additional support above and beyond routine care has the potential to encourage good self-management. We wanted to find out whether sessions led by their peers or by lay leaders help to reduce these risks and improve asthma outcomes among adolescents. Objectives: To assess the safety and efficacy of lay-led and peer support interventions for adolescents with asthma. Search methods: We identified trials from the Cochrane Airways Trials Register, which contains reports of randomised trials obtained from multiple electronic and handsearched sources, and we searched trial registries and reference lists of primary studies. We conducted the most recent searches on 25 November 2016. Selection criteria: Eligible studies randomised adolescents with asthma to an intervention led by lay people or peers or to a control. We included parallel randomised controlled trials with individual or cluster designs. We included studies reported as full text, those published as abstract only and unpublished data. Data collection and analysis: Two review authors screened the searches, extracted numerical data and study characteristics and assessed each included study for risk of bias. Primary outcomes were asthma-related quality of life and exacerbations requiring at least a course of oral steroids. We graded the analyses and presented evidence in a 'Summary of findings' table. We analysed dichotomous data as odds ratios, and continuous data as mean differences (MD) or standardised mean differences, all with a random-effects model. We assessed clinical, methodological and statistical heterogeneity when performing meta-analyses, and we described skewed data narratively. Main results: Five studies including a total of 1146 participants met the inclusion criteria for this review. As ever with systematic reviews of complex interventions, studies varied by design (cluster and individually randomised), duration (2.5 to 9 months), setting (school, day camp, primary care) and intervention content. Most risk of bias concerns were related to blinding and incomplete reporting, which limited the meta-analyses that could be performed. Studies generally controlled well for selection and attrition biases. All participants were between 11 and 17 years of age. Asthma diagnosis and severity varied, as did smoking prevalence. Three studies used the Triple A programme; one of these studies tested the addition of a smoke-free pledge; another delivered peer support group sessions and mp3 messaging to encourage adherence; and the third compared a peer-led asthma day camp with an equivalent camp led by healthcare practitioners. We had low confidence in all findings owing to risk of bias, inconsistency and imprecision. Results from an analysis of asthma-related quality of life based on the prespecified random-effects model were imprecise and showed no differences (MD 0.40, 95% confidence interval (CI) -0.02 to 0.81); a sensitivity analysis based on a fixed-effect model and a responder analysis suggested small benefit may be derived for this outcome. Most other results were summarised narratively and did not show an important benefit of the intervention; studies provided no analysable data on asthma exacerbations or unscheduled visits (data were skewed), and one study measuring adherence reported a drop in both groups. Effects on asthma control favoured the intervention but findings were not statistically significant. Results from two studies with high levels of baseline smoking showed some promise for self-efficacy to stop smoking, but overall nicotine dependence and smoking-related knowledge were not significantly better in the intervention group. Investigators did not report adverse events. Authors' conclusions: Although weak evidence suggests that lay-led and peer support interventions could lead to a small improvement in asthma-related quality of life for adolescents, benefits for asthma control, exacerbations and medication adherence remain unproven. Current evidence is insufficient to reveal whether routine use of lay-led or peer support programmes is beneficial for adolescents receiving asthma care. Ongoing and future research may help to identify target populations for lay-led and peer support interventions, along with attributes that constitute a successful programme.Copyright © 2017 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Database: EMBASE

Author(s): Srinivas S.M.; Gowda V.K.; Owen C.M.; Moss C.; Hiremagalore R.

Source: Clinical and Experimental Dermatology; Jan 2017; vol. 42 (no. 1); p. 68-71

Publication Date: Jan 2017

Publication Type(s): Journal: Article

Available in full text at Clinical and Experimental Dermatology -  from John Wiley and Sons

Abstract:Chronic ulcerating lesions on the face are rarely seen in toddlers. Blistering disease, vasculitis, infections and self-mutilation due to neurometabolic disease can usually be excluded on clinical and histological grounds. In the absence of identifiable disease, such lesions are sometimes attributed to child abuse or fabricated illness. We describe three toddlers with chronic mid-face erosions, two from India and one from the UK. One had moderate developmental delay and one had had seizures. The lesions appeared to be self-inflicted, no underlying disease was identified and there was no suspicion of child abuse. Recognition of the same disease pattern in different continents implies a distinct pathological entity. The pattern closely resembles that seen in some patients with mutations in the pain-insensitivity genes PRDM12 and SCN11A. We suggest the term 'mid-face toddler excoriation syndrome' (MiTES) to acknowledge the existence of this condition, encourage further reports and help clarify the pathogenesis. Copyright © 2016 British Association of Dermatologists
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Database: EMBASE

Source: The Journal of pediatrics; Nov 2016; vol. 178 ; p. 219

Publication Type(s): Journal Article

Author(s): Laraway, Sarah; Mercer, Jean; Jameson, Elisabeth; Ashworth, Jane; Hensman, Pauline; Jones, Simon A

Abstract:To evaluate long-term outcomes of laronidase enzyme replacement therapy in patients with attenuated mucopolysaccharidosis type I.         Retrospective analyses of case notes, laboratory results, and data from clinical trials were used to evaluate urinary glycosaminoglycans, forced vital capacity (FVC), 6-minute walk test (6MWT), height-for-age Z score, cardiac valve function, corneal clouding, and visual acuity in 35 patients with attenuated mucopolysaccharidosis type I (Hurler-Scheie and Scheie syndromes) for up to 10 years following the initiation of laronidase therapy. Statistically significant (P < .001) reductions in mean urinary glycosaminoglycan levels relative to baseline were observed 6 months after treatment initiation and were sustained throughout follow-up. Disease remained stable after treatment initiation with no statistically significant changes in mean FVC, 6MWT, or height-for-age Z score. At last assessments, mitral and aortic valve function remained stable in 65% (22/34) of patients; corneal clouding remained stable in 78% (18/23); visual acuity remained stable in 33% (8/24) and improved in 42% (10/24) of patients. Younger patients (<10 years at treatment initiation) maintained disease measures closer to norms for age for FVC, 6MWT, and height and showed fewer deteriorations in mitral and aortic valve disease and corneal clouding compared with patients aged ≥10 years at treatment initiation.         Laronidase treatment resulted in disease stabilization in the majority of patients with a mean follow-up of 6.1 years. Data suggest that early treatment may result in better outcomes. Crown Copyright © 2016. Published by Elsevier Inc. All rights reserved.