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Background: To describe long-term outcomes in JDM using patient questionnaires and link to longitudinal, prospectively collected data for each patient within the Juvenile Dermatomyositis Cohort and Biomarker Study, UK and Ireland (JDCBS) to determine outcome predictors. Methods: JDCBS participants aged ≥ 16y completed the SF36, HAQ and a questionnaire regarding current disease features, medications, education and employment. Data collected from the JDCBS included disease subtype, demographics, clinical and laboratory features. Intensity indices were calculated for physician VAS, modified skin DAS, CMAS and MMT8 by dividing area under the curve (AUC) from longitudinal score trajectories by duration of study follow-up (y). Relationships between questionnaire and JDCBS clinical / laboratory data were investigated fitting statistical models appropriate for cross sectional and longitudinal data. Results: Of 190 questionnaires sent, 84 (44%) were returned. Average age of respondents was 20.6 years (SD 3.9), time since diagnosis was 12.4 years (SD 5.0), age at onset was 9.2 years (SD 4.3), female to male ratio 4.25:1. Forty-nine (59%) self-reported persistently active disease, 54 (65%) were still taking immunosuppressive medication. 14/32 at school/higher education reported myositis adversely affecting academic results. 18–24 year-olds were twice as likely to be unemployed compared the UK population (OR = 0.456, 95% CI 0.24, 0.84, p = 0.001). Participants ≥ 18 years were three times as likely to be living with a parent/guardian (OR = 3.39, p < 0.001). SF36 MCS and MMT8 intensity index scores were significantly correlated (ρ = 0.328, p = 0.007). Conclusions: After 12.4 years, questionnaire responders reported self-perceived high rates of persistently active disease and medication use, reduced rates of employment and were more likely to live with a parent/guardian. Perceived persistently active muscle disease appeared to affect quality of life in these patients and was the most significant contributor to long-term outcomes. Our findings highlight the importance of including the patient perspective in the assessment of long term outcomes, so that that we can start to target initial management strategies more effectively based on a combination of clinical and patient-reported data. [ABSTRACT FROM AUTHOR]

Objectives The primary objective was to define the incidence of JSLE in children <16 years of age in the UK and Republic of Ireland (ROI). The secondary objective was to describe presenting features, classification criteria, initial management and disease damage in newly presenting JSLE patients. Methods A prospective JSLE epidemiological study was undertaken between September 2017 and September 2019 with support of the British Paediatric Surveillance Unit and other professional groups involved in diagnosis and management of JSLE patients. Treating consultants reported all cases of JSLE seen. A follow-up study at 1 year examined management and progression of disease and treatment. Results There were 124 incident cases included in the final analysis. Incidence was estimated using ACR-1997 classification criteria (0.36/100 000), SLICC-2012 classification criteria (0.41/100 000) and clinician expert opinion (0.46/100 000). A high disease burden was seen, with 71.0% of patients requiring ongoing systemic CS treatment at 1 year; 98.2% receiving immunomodulatory treatment; and 20.4% accruing damage in the year following diagnosis (predominantly neuropsychiatric-related), with substantial involvement from multiple speciality teams. Conclusions The minimum UK and ROI incidence of JSLE is between 0.36 and 0.46/100 000, depending on the case definition used. Challenges in classification of patients with JSLE are highlighted, but overall this study supports the use of SLICC-2012 classification criteria. The high levels of disease damage and ongoing CS use 1 year after diagnosis is concerning, highlighting the need for further interventions to improve outcomes in JSLE. [ABSTRACT FROM AUTHOR]

Introduction: Juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune/inflammatory disease with significant morbidity and mortality. Neuropsychiatric (NP) involvement is a severe complication, encompassing a heterogeneous range of neurological and psychiatric manifestations. Methods: Demographic, clinical, and laboratory features of NP-SLE were assessed in participants of the UK JSLE Cohort Study, and compared to patients in the same cohort without NP manifestations. Results: A total of 428 JSLE patients were included in this study, 25% of which exhibited NP features, half of them at first visit. Most common neurological symptoms among NP-JSLE patients included headaches (78.5%), mood disorders (48.6%), cognitive impairment (42%), anxiety (23.3%), seizures (19.6%), movement disorders (17.7%), and cerebrovascular disease (14.9%). Peripheral nervous system involvement was recorded in 7% of NP-SLE patients. NP-JSLE patients more frequently exhibited thrombocytopenia (<100 × 109/L) (p = 0.04), higher C-reactive protein levels (p = 0.01), higher global pBILAG score at first visit (p < 0.001), and higher SLICC damage index score at first (p = 0.02) and last (p < 0.001) visit when compared to JSLE patients without NP involvement. Conclusions: A significant proportion of JSLE patients experience NP involvement (25%). Juvenile-onset NP-SLE most commonly affects the CNS and is associated with increased overall disease activity and damage. [ABSTRACT FROM AUTHOR]

Young people are responsible for a significant number of the sexual offenses that are committed every year. These young people are generally referred to specialist services for treatment. This article explores the health characteristics and service experiences of 117 young people with sexual behavior problems, and the issues that services face when working with them. The study is based on analysis of 117 case files, identified from nine specialist services in the UK. The case files were thematically analyzed. Case files provided information on the following topics: the reasons why the young people were referred to harmful sexual behavior services; the young people's personal characteristics; their medical and mental health problems; the young people's interests and aspirations; their attitudes toward services and interventions; continued problematic sexual incidents in services; progress in services; and post-service experiences. Overall, the findings of the study indicate that these young people have a number of strengths, but often have problems across a range of personal and health domains. A number of them continue to remain sexually and generally violent in services, particularly in residential settings, which has risk management implications for staff. [ABSTRACT FROM AUTHOR]

Objectives. Toll-like receptors (TLRs) essential in the functioning of the immune system have been implicated in the development of autoimmunity. TLR3, 7, 8 and 9 are capable of recognizing nucleic autoantigens typical of SLE. Their expression correlates positively with disease activity in adult-onset SLE. This study aimed to determine the role of TLRs in JSLE and whether apoptotic neutrophils are a source of nuclear autoantigen being detected through TLR3, 7, 8 and 9, leading to an inflammatory response.Methods. TLR3, 7, 8 and 9 mRNA and protein expression were measured in peripheral blood mononuclear cells (PBMCs) in JSLE patients compared with JIA and non-inflammatory controls. Activation of the TLRs by JSLE serum-induced apoptotic neutrophils was detected by measuring IFN-α mRNA and protein expression, and confirmed using myeloid differentiation factor 88 (MyD88) and TIR domain-containing adapter-inducing IFN-β (TRIF) inhibitors.Results. JSLE patients have increased TLR3, 8 and 9 mRNA and protein expression compared with controls (P < 0.05). Incubation of PBMCs with apoptotic neutrophils demonstrated a dose–response relationship for IFN-α mRNA expression. Inhibition of TLR signalling by blocking MyD88 and TRIF signalling decreased IFN-α mRNA expression in PBMCs incubated with apoptotic neutrophils (P < 0.05).Conclusions. This study demonstrated significantly increased TLR expression in JSLE compared with controls. Our data indicate that apoptotic neutrophils trigger TLR activation through their presentation of autoantigens. The role of TLRs in this inflammatory response was demonstrated by a dose–response relationship to apoptotic neutrophil concentration and confirmed by a decrease in IFN-α production after inhibition of TLR signalling. [ABSTRACT FROM PUBLISHER]

The ‘appropriate adult’ has received relatively little attention from academics and even less from policymakers. That said, when the United Kingdom's Labour government was displaced in 2010, it had just completed, in March 2010, a three-year review of the Police and Criminal Evidence Act 1984 (PACE), which included proposals for extending, professionalising and further reviewing the appropriate adult scheme. Under the new coalition government, the review of the provision of appropriate adults is continuing. It is, therefore, timely to assess the merits of the existing proposals and consider which further issues should be taken into account by the ongoing review. This article makes this assessment with reference to the existing literature, including the previously unpublished results of a survey of professional appropriate adults and their coordinators. The article argues that a number of appropriate adults support the extension and professionalisation of the role and, in practice, have already extended their role, in a manner akin to Marx's (1988) concept of ‘creep’. However, the main priority for the new government should be to clarify the definition of the role. [ABSTRACT FROM AUTHOR]

Little information is available on the occurrence of neoplasms in dogs up to the age of 12 months. This is a retrospective review of histopathological diagnoses of neoplasia in dogs up to the age of 12 months based on biopsy specimens submitted to a commercial veterinary diagnostic laboratory in the United Kingdom between 1993 and 2008. In 20 280 histological submissions, 9522 neoplasms were identified. Canine cutaneous histiocytoma ( n = 8465; 89%) was the most common histological type. Neoplasms other than histiocytoma ( n = 1057; 11%) were grouped as benign epithelial ( n = 375; 4%), haematopoietic ( n = 229; 2%), benign mesenchymal ( n = 145; 2%), miscellaneous ( n = 118; 1%), non-hematopoietic malignant mesenchymal ( n = 118; 1%) or malignant epithelial tumours ( n = 72; <1%). Excluding canine cutaneous histiocytoma, 52% of tumours ( n = 547) were benign, and 66% were from the skin or soft tissues. These data provide valuable epidemiological information on neoplasms occurring in juvenile dogs in the United Kingdom. [ABSTRACT FROM AUTHOR]

We investigated the survival of 57 rehabilitated juvenile Tawny Owls (Strix aluco) that were 'hard released' (without the provision of a release aviary or support food) by means of radio tracking. The birds were released in the month of August in three consecutive years: 2005, 2006 and 2007, in the counties of Somerset and Hampshire, United Kingdom. Tracking of the owls was successfully carried out for between three and 160 days. Mortality was recorded for 16 birds (28%). The transmitter was shed by 24 (42%) owls, the signal was lost for 12 (21%) and tracking was ceased for five (9%) owls. Survival of the owls was compared with results from previous studies on wild Tawny Owls and also rehabilitated 'soft released' Tawny Owls (released with provision of food and shelter after release) and found to be similar. This study suggests that employing costly and time-consuming soft release techniques may be unnecessary for juvenile Tawny Owls as their survival is not significantly reduced using hard-release methods. Measuring post-release success of rehabilitated birds of prey is discussed in relation to benchmarks used in previous studies. [ABSTRACT FROM AUTHOR]

Relatively few studies have compared female and male juveniles who sexually abuse. These studies have reported that while female juveniles with sexually abusive behaviour are more likely to have experienced childhood sexual abuse, they display similar patterns of abusive behaviour. However, to date these findings have not been replicated in a UK sample. The current study compared 22 female and 254 male juveniles, referred to a UK specialist community forensic service, in relation to family environment, maltreatment experience, psychiatric diagnoses and perpetrated abusive behaviour. Consistent with previous studies perpetrated behaviour was similar across genders, but females were significantly more likely to have been sexually victimized as children, at a younger age and by a greater number of abusers. In addition, they were more likely to have been exposed to inadequate sexual boundaries at home. These findings suggest that females and males may follow different pathways to sexually abusive behaviour. [ABSTRACT FROM AUTHOR]

Amongst the British Government's plans for the future regulation of gambling is the proposal to continue to allow children to play low stake/low prize gaming machines. No other country permits juvenile gaming machine playing, and the 2001 Gambling Review Report bowed to industry pressure to allow it to continue. Evidence from the British Gambling Prevalence Survey and elsewhere suggests that machine gambling is one of the more addictive forms of gambling, and that children and adolescents are particularly vulnerable to problem machine gambling. Theories of child development and of habit formation would predict that playing gaming machines would be dangerous for young people. Given the level of funding that will be available for gambling research, the Government's recommendation that research sould be carried out within five years on the harm or lack of it, caused by juvenile gaming machine playing is unrealistic. By proposing to perpetuate the anomaly of juvenile machine gambling, the British government is acting irresponsibly by failing to protect young people and their families from a practice which all existing evidence leads us to believe is likely to be hazardous. [ABSTRACT FROM AUTHOR]

Background Childhood cases of cytotoxic T-cell lymphoma have not been well described. Objectives We have undertaken an immunohistochemical and genotypic analysis of patients presenting with juvenile onset mycosis fungoides (MF). Patients/methods Of 10 patients presenting over a 3-year period, six exhibited a CD8-positive phenotype. These six patients were also CD2, CD3 and TIA1 positive, but CD56 negative. Apart from the cytotoxic phenotype, these patients had clinicopathological features that were indistinguishable from ordinary cases of MF, with slowly evolving patches and plaques. Three patients were staged as 1A and three as 1B, with no evidence of nodal or systemic disease. Results Patients responded well to conventional therapy, with no evidence of disease progression after 3 years follow-up. Epidermotropism was a prominent feature in four of the six cytotoxic cases. In two patients with an equivocal histology the diagnosis was confirmed by the finding of a clonal population, using polymerase chain reaction/single strand conformational polymorphism analysis of the T-cell receptor gamma gene in lesional skin. The same technique revealed that all blood samples analysed were polyclonal. Conclusions These data show that cytotoxic T-cell lymphoma can pursue an indolent course and that cases of CD8-positive MF may be over-represented in childhood. [ABSTRACT FROM AUTHOR]

Background: In the UK, juvenile idiopathic arthritis is the most common inflammatory disorder in childhood, affecting 10 : 100,000 children and young people aged < 16 years each year, with a population prevalence of around 1 : 1000. Corticosteroids are commonly used to treat juvenile idiopathic arthritis; however, there is currently a lack of consensus as to which corticosteroid induction regimen should be used with various disease subtypes and severities of juvenile idiopathic arthritis., Objective: The main study objective was to determine the feasibility of conducting a randomised controlled trial to compare the different corticosteroid induction regimens in children and young people with juvenile idiopathic arthritis., Design: This was a mixed-methods study. Work packages included a literature review; qualitative interviews with children and young people with juvenile idiopathic arthritis and their families; a questionnaire survey and screening log to establish current UK practice; a consensus meeting with health-care professionals, children and young people with juvenile idiopathic arthritis, and their families to establish the primary outcome; a feasibility study to pilot data capture and to collect data for future sample size calculations; and a final consensus meeting to establish the final protocol., Setting: The setting was rheumatology clinics across the UK., Participants: Children, young people and their families who attended clinics and health-care professionals took part in this mixed-methods study., Interventions: This study observed methods of prescribing corticosteroids across the UK., Main Outcome Measures: The main study outcomes were the acceptability of a future trial for children, young people, their families and health-care professionals, and the feasibility of delivering such a trial., Results: Qualitative interviews identified differences in the views of children, young people and their families on a randomised controlled trial and potential barriers to recruitment. A total of 297 participants were screened from 13 centres in just less than 6 months. In practice, all routes of corticosteroid administration were used, and in all subtypes of juvenile idiopathic arthritis. Intra-articular corticosteroid injection was the most common treatment. The questionnaire surveys showed the varying clinical practice across the UK, but established intra-articular corticosteroids as the treatment control for a future trial. The primary outcome of choice for children, young people, their families and health-care professionals was the Juvenile Arthritis Disease Activity Score, 71-joint count. However, results from the feasibility study showed that, owing to missing blood test data, the clinical Juvenile Arthritis Disease Activity Score should be used. The Juvenile Arthritis Disease Activity Score, 71-joint count, and the clinical Juvenile Arthritis Disease Activity Score are composite disease activity scoring systems for juvenile arthritis. Two final trial protocols were established for a future randomised controlled trial., Limitations: Fewer clinics were included in this feasibility study than originally planned, limiting the ability to draw strong conclusions about these units to take part in future research., Conclusions: A definitive randomised controlled trial is likely to be feasible based on the findings from this study; however, important recommendations should be taken into account when planning such a trial., Future Work: This mixed-methods study has laid down the foundations to develop the evidence base in this area and conducting a randomised control trial to compare different corticosteroid induction regimens in children and young people with juvenile idiopathic arthritis is likely to be feasible., Study Registration: Current Controlled Trials ISRCTN16649996., Funding: This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment ; Vol. 24, No. 36. See the NIHR Journals Library website for further project information., Competing Interests: Athimalaipet V Ramanan has received speaker fees/honoraria/consulting fees from Abbvie Inc. (North Chicago, IL, USA), Union Chimique Belge (Brussels, Belgium), Eli Lilly and Company (Indianapolis, IN, USA), Novartis (Basel, Switzerland), Roche Holding AG (Basel, Switzerland) and Bristol-Myers Squibb (New York, NY, USA). Paula R Williamson reports grants from the National Institute for Health Research (NIHR) Health Technology Assessment programme outside the submitted work and involvement with a clinical trials unit funded by NIHR.

Background and Objectives: The multisystem involvement and variable course of juvenile-onset systemic lupus erythematosus (JSLE) make it difficult to assess disease activity over time. International consensus definitions of inactive disease and clinical remission have been proposed. The aim of this study was to determine the proportion of patients meeting these criteria in a large national cohort of JSLE patients and the association between achieving inactive disease and clinical remission with disease activity at presentation and time to diagnosis., Methods: Patients diagnosed with JSLE aged ≤17 years with a minimum of 12 months follow-up participating in the UK JSLE Cohort Study were assessed against these criteria at baseline, 1 year and final clinic visit., Results: A total of 218 patients with mean follow-up duration of 4.7 years were included and analyzed at baseline visit, of which 93 and 209 were available for analysis at the 1-year and the last follow-up visits, respectively. Eighty-five percent at 1 year and 62% at final follow-up still had active disease while only 6% and 9%, respectively, achieved inactive disease according to the proposed criteria. The majority of patients continued to require immunosuppressive treatment despite their prolonged follow-up with only two patients achieving clinical remission on medication and none off medication. A large number of patients did not meet the criteria for inactive disease due to isolated laboratory abnormalities such as reduced lymphocyte count. Isolated low lymphocyte count was the reason for not fulfilling the inactive disease criteria in 20/79 (25%) patients at 1 year and 14/130 (11%) patients at final follow-up visit. No statistically significant differences in relation to time to diagnosis and disease activity at presentation were found between patients achieving inactive disease compared to those who did not, at 1 year and final follow-up., Conclusion: The majority of patients failed to achieve the proposed criteria for inactive disease and continued to require immunosuppressive treatment. This reflects the high burden of disease in JSLE despite immunosuppressive therapy. A significant proportion of patients had isolated laboratory abnormalities of potentially limited clinical significance, suggesting that some modifications of the proposed criteria may be required.