Worse scores but similar patterns of disease activity: interpreting outcomes in women with axial spondyloarthropathy.

OBJECTIVE: The aim of this study was to evaluate the impact of sex on disease activity in axial spondylitis (axSpA). METHOD: Data were extracted from the Ankylosing Spondylitis Registry of Ireland (ASRI). In this cross-sectional study, patients were analysed on the basis of sex, with a series of comparison analyses performed. RESULTS: Overall, 886 participants were enrolled in the ASRI [232 (26.2%) women, 644 (72.6%) men]. Females recorded significantly worse Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) (4.57 vs 3.83, p < 0.01) and Ankylosing Spondylitis Quality of Life questionnaire (ASQoL) (7.51 vs 6.12, p < 0.01) scores than males. There was a stronger correlation in the Bath Ankylosing Spondylitis Functional and Metrology Indices (BASFI and BASMI) in females (rs = 0.619, p < 0.01) than in males (rs = 0.572, p < 0.01). Analysis of factors in BASDAI revealed that the higher total scores in females compared to males were due not to any single component, but to worse scores in all six components of the BASDAI combined. Ranking of components by severity between sexes revealed identical ranking in four of the six components of the BASDAI. CONCLUSIONS: Women with axSpA reported significantly worse disease activity, quality of life, and functional ability than men. However, the BASDAI capturedsimilar patterns of disease activity. Limitation of spinal mobility in women with axSpA corresponded to greater impairment in functional ability. Further evaluation of disease monitoring tools is required to ensure that disease activity is accurately captured in men and women with axSpA.

Exploring the psychometric properties of the Intellectual Disability versions of the Short Warwick-Edinburgh Mental Wellbeing Scale and Kidscreen10, self-reported by adolescents with intellectual disability.

BACKGROUND: There are no validated self-report measures that can be used to assess health and wellbeing in adolescents with intellectual disability (ID). METHOD: The aim of this study was to explore the psychometric properties of two newly adapted self-report health-related quality of life (HRQoL) and mental wellbeing measures: the Intellectual Disability versions of the Short Warwick-Edinburgh Mental Wellbeing Scale (ID-SWEMWBS) and Kidscreen10 (ID-Kidscreen10). For this, we used data from a sample of 427 adolescents (aged 11-19) with ID recruited from special school settings. RESULTS: Confirmatory factor analysis (CFA) was conducted to validate the factor structure of both measures. Internal consistency was assessed using Cronbach's alpha and test-retest reliability was analysed using intra-class correlation coefficients. The internal consistency measured using Cronbach's alpha was found to be in the range of 0.70-0.78, test-retest reliabilities were expressed using intra-class correlation coefficients that were found to be high for both measures (ID-SWEMWBS, 0.758; ID-Kidscreen10, 0.723), and the CFA supported the unidimensional structure of both measures. CONCLUSIONS: The results of this study indicate that the ID-SWEMWBS and ID-Kidscreen10 have very good psychometric properties and can be used as self-report measures to assess HRQoL and mental wellbeing in adolescents with ID.

The toll of unemployment in axial spondyloarthropathy: high prevalence and negative impact on outcomes captured in a national registry.

OBJECTIVE: Axial spondyloarthropathy (axSpA) is an inflammatory arthritis of the axial skeleton. Persistent disease activity can result in significant disability and affect the ability to maintain employment. This study aimed to determine the prevalence of unemployment in axSpA and the impact on patient outcomes. METHOD: Data from the Ankylosing Spondylitis Registry of Ireland (ASRI) were cleaned, and information on employment, demographics, and disease characteristics was extracted. Patients were analysed on the basis of employment and categorized as employed or unemployed. RESULTS: Of the 759 participants included in the analysis, 23.5% (178) were unemployed, higher than national averages of 6.2-13.1% during the study period. Unemployed participants reported significantly worse Bath Ankylosing Spondylitis Disease Activity Index (BASDAI; 5.1 vs 3.6), Metrology Index (BASMI; 4.8 vs 3.4), Functional Index (BASFI; 5.2 vs 3.0), Health Assessment Questionnaire (HAQ; 0.82 vs 0.40), and Ankylosing Spondylitis Quality of Life (ASQoL; 9.4 vs 5.4) scores compared to employed (all p < 0.01). Male gender (odds ratio, 95% confidence interval: 2.65, 1.46-4.83), worse BASMI (1.16, 1.02-1.33), and worse HAQ scores (2.18, 1.13-4.19) were significantly associated with unemployment. CONCLUSION: The prevalence of unemployment in axSpA patients is higher than in the general population, and is associated with worse quality of life, poorer levels of function, and higher levels of disease activity. Predictors of unemployment in axSpA were male gender, worse spinal mobility, and poorer level of function. Recognition of patients at risk of unemployment will improve opportunities for intervention and maintain participation in the workforce.

Involving adolescents with intellectual disability in the adaptation of self-reported subjective well-being measures: participatory research and methodological considerations.

BACKGROUND: The measurement of subjective well-being is challenging with samples of adolescents with intellectual disability (ID) given the cognitive and linguistic difficulties they face in comprehending standardised measures, and as such is primarily based on proxy reports. The lack of appropriate tools needs to be addressed so that adolescents with ID can self-report on their own well-being. The current study reports on the use of participatory research methods to adapt and modify two standardised self-report measures of subjective well-being suitable for completion by adolescents with ID. METHOD: Two special schools were recruited for this study. At each school, staff (n = 15) and pupils aged 11-17 years (n = 35) participated. A series of co-design workshops were conducted to adapt two standardised subjective well-being measures: Kidscreen-10 and short-form Warwick-Edinburgh Mental Well-being Scale. RESULTS: Specific aspects for measure adaption were identified: simplifying the item wording and phrasing; inclusion of pictorial communication symbols and visual prompts to represent the meaning of items; changing of tense of questions from past to present; asking questions rather than statements; reducing 5-point Likert scales to 3-point or dichotomous; presenting one item at a time during administration; and developing alternate formats of the survey to ensure inclusivity. CONCLUSIONS: This paper illustrates the value of using participatory research methods when working alongside adolescents with ID and offers methodological, as well as practical, guidance in the context of adapting subjective self-report measures for this target group, serving as a guide to fellow researchers and clinicians interested in modifying or developing self-report measures for adolescents with ID.

Assessment of structural chromosomal instability phenotypes as biomarkers of carboplatin response in triple negative breast cancer: the TNT trial.

BACKGROUND: In the TNT trial of triple negative breast cancer (NCT00532727), germline BRCA1/2 mutations were present in 28% of carboplatin responders. We assessed quantitative measures of structural chromosomal instability (CIN) to identify a wider patient subgroup within TNT with preferential benefit from carboplatin over docetaxel. PATIENTS AND METHODS: Copy number aberrations (CNAs) were established from 135 formalin-fixed paraffin-embedded primary carcinomas using Illumina OmniExpress SNP-arrays. Seven published [allelic imbalanced CNA (AiCNA); allelic balanced CNA (AbCNA); copy number neutral loss of heterozygosity (CnLOH); number of telomeric allelic imbalances (NtAI); BRCA1-like status; percentage of genome altered (PGA); homologous recombination deficiency (HRD) scores] and two novel [Shannon diversity index (SI); high-level amplifications (HLAMP)] CIN-measurements were derived. HLAMP was defined based on the presence of at least one of the top 5% amplified cytobands located on 1q, 8q and 10p. Continuous CIN-measurements were divided into tertiles. All nine CIN-measurements were used to analyse objective response rate (ORR) and progression-free survival (PFS). RESULTS: Patients with tumours without HLAMP had a numerically higher ORR and significantly longer PFS in the carboplatin (C) than in the docetaxel (D) arm [56% (C) versus 29% (D), PHLAMP,quiet = 0.085; PFS 6.1 months (C) versus 4.1 months (D), Pinteraction/HLAMP = 0.047]. In the carboplatin arm, patients with tumours showing intermediate telomeric NtAI and AiCNA had higher ORR [54% (C) versus 20% (D), PNtAI,intermediate = 0.03; 62% (C) versus 33% (D), PAiCNA,intermediate = 0.076]. Patients with high AiCNA and PGA had shorter PFS in the carboplatin arm [3.4 months (high) versus 5.7 months (low/intermediate); and 3.8 months (high) versus 5.6 months (low/intermediate), respectively; Pinteraction/AiCNA = 0.027, Padj.interaction/AiCNA = 0.125 and Pinteraction/PGA = 0.053, Padj.interaction/PGA = 0.176], whilst no difference was observed in the docetaxel arm. CONCLUSIONS: Patients with tumours lacking HLAMP and demonstrating intermediate CIN-measurements formed a subgroup benefitting from carboplatin relative to docetaxel treatment within the TNT trial. This suggests a complex and paradoxical relationship between the extent of genomic instability in primary tumours and treatment response in the metastatic setting.

Does prehabilitation modify muscle mass in patients with rectal cancer undergoing neoadjuvant therapy? A subanalysis from the REx randomised controlled trial.

BACKGROUND: Patients with rectal cancer who present with sarcopenia (low muscle mass) are at significantly greater risk of postoperative complications and reduction in disease-free survival. We performed a subanalysis of a randomised controlled study [the REx trial; www.isrctn.com ; 62859294] to assess the potential of prehabilitation to modify muscle mass in patients having neoadjuvant chemoradiotherapy (NACRT). METHODS: Patients scheduled for NACRT, then potentially curative surgery (August 2014-March 2016) had baseline physical assessment and psoas muscle mass measurement (total psoas index using computed tomography-based measurements). Participants were randomised to either the intervention (13-17-week telephone-guided graduated walking programme) or control group (standard care). Follow-up testing was performed 1-2 weeks before surgery. RESULTS: The 44 patients had a mean age of 66.8 years (SD 9.6) and were male (64%); white (98%); American Society of Anesthesiologists class 2 (66%); co-morbid (58%); overweight (72%) (body mass index ≥ 25 kg/m2). At baseline, 14% were sarcopenic. At follow-up, 13 (65%) of patients in the prehabilitation group had increased muscle mass versus 7 (35%) that experienced a decrease. Conversely, 16 (67%) controls experienced a decrease in muscle mass and 8 (33%) showed an increase. An adjusted linear regression model estimated a mean treatment difference in Total Psoas Index of 40.2mm2/m2 (95% CI - 3.4 to 83.7) between groups in change from baseline (p = 0.07). CONCLUSIONS: Prehabilitation improved muscle mass in patients with rectal cancer who had NACRT. These results need to be explored in a larger trial to determine if the poorer short- and long-term patient outcomes associated with low muscle mass can be minimised by prehabilitation.

The relationship between sarcopenia and survival at 1 year in patients having elective colorectal cancer surgery.

BACKGROUND: Colorectal cancer remains a common cause of cancer death in the UK, with surgery being the mainstay of treatment. An objective measurement of the suitability of each patient for surgery, and their risk-benefit calculation, would be of great utility. We postulate that sarcopenia (low muscle mass) could fulfil this role as a prognostic indicator. The aim of this study was to determine the relationship between sarcopenia and long-term outcomes in patients undergoing elective bowel resection for colorectal cancer. METHODS: One hundred and sixty-three consecutive patients who had elective curative colorectal resection for cancer were eligible for inclusion in the study. Psoas muscle mass was assessed on preoperative computed tomography scan at the level of the L3 vertebra and standardised for patient height (total psoas index, TPI). Sarcopenia (low muscle mass) was defined as < 524 mm2/m2 in males and 385 mm2/m2 in females. In addition to clinical-pathological parameters, postoperative complications were recorded and patients were followed up for mortality for 1 year after surgery. RESULTS: Sarcopenia was present in 19.6% of the study participants and was significantly related to body mass index (p = 0.007), 30-day mortality (p = 0.042) and 1-year mortality (p = 0.046). In univariate analysis, American Society of Anesthesiologists grade (p = 0.016), tumour stage (p = 0.018) and sarcopenia (p = 0.043) were found to be significant independent predictors of 1-year mortality. CONCLUSIONS: This study has found sarcopenia to be prevalent in patients with colorectal cancer having elective surgery. Independent of age, sarcopenia was associated with poorer 30-day mortality and survival at 1 year. Measurement of muscle mass preoperatively could be used to stratify a patient's risk, allowing targeted strategies such as prehabilitation, to be implemented to modify sarcopenia and improve long-term outcomes for patients.

Ask Me! self-reported features of adolescents experiencing neglect or emotional maltreatment: a rapid systematic review.

BACKGROUND: Neglect is often overlooked in adolescence, due in part to assumptions about autonomy and misinterpretation of behaviors being part of normal adolescent development. Emotional maltreatment (abuse or neglect) has a damaging effect throughout the lifespan, but is rarely recognized amongst adolescents. Our review aims to identify features that adolescents experiencing neglect and/ or emotional maltreatment report. METHOD: A rapid review methodology searched 8 databases (1990-2014), supplemented by hand searching journals, and references, identifying 2,568 abstracts. Two independent reviews were undertaken of 279 articles, by trained reviewers, using standardised critical appraisal. Eligible studies: primary studies of children aged 13-17 years, with substantiated neglect and/ or emotional maltreatment, containing self-reported features. RESULTS: 19 publications from 13 studies were included, demonstrating associations between both neglect and emotional maltreatment with internalising features (9 studies) including depression, post traumatic symptomatology and anxiety; emotional maltreatment was associated with suicidal ideation, while neglect was not (1 study); neglect was associated with alcohol related problems (3 studies), substance misuse (2 studies), delinquency for boys (1 study), teenage pregnancy (1 study), and general victimization for girls (1 study), while emotionally maltreated girls reported more externalising symptoms (1 study). Dating violence victimization was associated with neglect and emotional maltreatment (2 studies), while emotional abuse of boys, but not neglect, was associated with dating violence perpetration (1 study), and neither neglect nor emotional maltreatment had an association with low self-esteem (2 studies). Neither neglect nor emotional maltreatment had an effect on school performance (1 study), but neglected boys showed greater school engagement than neglected girls (1 study). CONCLUSIONS: If asked, neglected or emotionally maltreated adolescents describe significant difficulties with their mental health, social relationships, and alcohol or substance misuse. Practitioners working with youths who exhibit these features should recognize the detrimental impact of maltreatment at this developmental stage, and identify whether maltreatment is a contributory factor that should be addressed.

Child abuse and fabricated or induced illness in the ENT setting: a systematic review.

BACKGROUND: Child maltreatment is persistently under-recognised. Given that a third of maltreated children may return with serious or fatal injuries, it is imperative that otolaryngologists who are in frequent contact with children are able to detect maltreatment at first presentation. OBJECTIVE OF REVIEW: This review aims to identify ENT injuries, signs or symptoms that are indicative of physical abuse or fabricated or induced illness (child maltreatment). TYPE OF REVIEW: Systematic review. SEARCH STRATEGY: An all-language search, developed in Medline Ovid and consisting of 76 key words, was conducted of published and grey literature across 10 databases from inception to July 2015, for primary observational studies involving children aged <18 years. EVALUATION METHOD: Each relevant article underwent two independent reviews with full critical appraisal, applying strict quality standards. RESULTS: Of the 2448 studies identified and screened, 371 underwent full review, resulting in 38 included studies that detailed 122 maltreated children. Pharyngeal perforations (n = 20) were the most frequent abusive ENT injury, predominantly affecting neonates and infants, presenting with dysphagia, drooling, haemoptysis and surgical emphysema. At least 52% of children with abusive pharyngeal injuries had additional co-existent injuries. The majority of ear injuries were inflicted to the external ear (n = 11) and included auricular deformity, abrasions, petechiae, lacerations and burns. Fabricated or induced illness cases presented most commonly with recurrent, unexplained otorrhoea or ENT lesions that failed to heal despite appropriate therapy. CONCLUSIONS: All clinicians should be familiar with the signs of child maltreatment. Pharyngeal injuries, or injuries to the external ear, presenting in young children without an explicit history of witnessed injury should prompt a child protection referral for full evaluation. Likewise, children who present with recurrent, or apparently intractable symptoms and signs despite appropriate treatment, should raise the possibility of fabricated or induced illness, and discussion with a child protection specialist is advised. Early recognition of possible child maltreatment and instigation of appropriate safeguarding measures are essential to prevent repetition and escalation of injury. This is of paramount importance to otolaryngologists, who have the potential to identify these children in their practice.

Donegal Going against the Flow: National Differences in Long-Term Urinary Catheterisation Rates in Men (> 65 Years) With Benign Prostatic Hypertrophy.

An analysis of Primary Care Reimbursement Service (PCRS, 2013) data demonstrated high rates of urinary catheter changes in Donegal compared to other regions of Ireland. There is a catheter change rate of 10.2% in Donegal men over 65 with medical cards (GMS) compared to rates of 2.7% and 0.17% in Waterford and South Dublin, respectively1. This 60-fold difference between an area with perceived good access to services (South Dublin) and Donegal an area that does not, prompted a survey of general practitioners in each of these areas to assess whether true male catheterisation rates were similarly disproportionate in Donegal. Based on this, data was collected from a population of 23,794 GMS patients in GP training practices in Donegal (Rural), Leinster (Urban) and Waterford (Suburban). The data sampled for Donegal demonstrated 19 long-term catheters (LTCs per 8603 GMS) compared to four LTCs (per 5,800 GMS) in Leinster and 3 LTCs (per 9,391 GMS) in Waterford (Table 1). This anomaly in LTC rates may be a proxy for lack of access to basic Urology services.

A neuron-glia interaction involving GABA transaminase contributes to sleep loss in sleepless mutants.

Sleep is an essential process and yet mechanisms underlying it are not well understood. Loss of the Drosophila quiver/sleepless (qvr/sss) gene increases neuronal excitability and diminishes daily sleep, providing an excellent model for exploring the underpinnings of sleep regulation. Here, we used a proteomic approach to identify proteins altered in sss brains. We report that loss of sleepless post-transcriptionally elevates the CG7433 protein, a mitochondrial γ-aminobutyric acid transaminase (GABAT), and reduces GABA in fly brains. Loss of GABAT increases daily sleep and improves sleep consolidation, indicating that GABAT promotes wakefulness. Importantly, disruption of the GABAT gene completely suppresses the sleep phenotype of sss mutants, demonstrating that GABAT is required for loss of sleep in sss mutants. While SSS acts in distinct populations of neurons, GABAT acts in glia to reduce sleep in sss flies. Our results identify a novel mechanism of interaction between neurons and glia that is important for the regulation of sleep.

Robust anti-obesity and metabolic effects of a dual GLP-1/glucagon receptor peptide agonist in rodents and non-human primates.

AIMS: To characterize the pharmacology of MEDI0382, a peptide dual agonist of glucagon-like peptide-1 (GLP-1) and glucagon receptors. MATERIALS AND METHODS: MEDI0382 was evaluated in vitro for its ability to stimulate cAMP accumulation in cell lines expressing transfected recombinant or endogenous GLP-1 or glucagon receptors, to potentiate glucose-stimulated insulin secretion (GSIS) in pancreatic ß-cell lines and stimulate hepatic glucose output (HGO) by primary hepatocytes. The ability of MEDI0382 to reduce body weight and improve energy balance (i.e. food intake and energy expenditure), as well as control blood glucose, was evaluated in mouse models of obesity and healthy cynomolgus monkeys following single and repeated daily subcutaneous administration for up to 2 months. RESULTS: MEDI0382 potently activated rodent, cynomolgus and human GLP-1 and glucagon receptors and exhibited a fivefold bias for activation of GLP-1 receptor versus the glucagon receptor. MEDI0382 produced superior weight loss and comparable glucose lowering to the GLP-1 peptide analogue liraglutide when administered daily at comparable doses in DIO mice. The additional fat mass reduction elicited by MEDI0382 probably results from a glucagon receptor-mediated increase in energy expenditure, whereas food intake suppression results from activation of the GLP-1 receptor. Notably, the significant weight loss elicited by MEDI0382 in DIO mice was recapitulated in cynomolgus monkeys. CONCLUSIONS: Repeated administration of MEDI0382 elicits profound weight loss in DIO mice and non-human primates, produces robust glucose control and reduces hepatic fat content and fasting insulin and glucose levels. The balance of activities at the GLP-1 and glucagon receptors is considered to be optimal for achieving weight and glucose control in overweight or obese Type 2 diabetic patients.

Isolated anterior mediastinal tuberculosis in an immunocompetent patient.

BACKGROUND: The differential diagnosis of a mediastinal mass is a common challenge in clinical practice, with a wide range of differential diagnosis to be considered. One of the rarer causes is tuberculosis. Atypical presentations of tuberculosis are well documented in immunocompromised patients, but should also be considered in the immunocompetent. CASE PRESENTATION: This case outlines a previously healthy 22 year-old immunocompetent male presenting with worsening chest pain, positional dyspnea, dry cough and dysphagia. Chest x-ray showed evidence of an isolated anterior mediastinal mass, which was confirmed on computed tomography. A mediastinoscopy was diagnostic as histology revealed necrotizing granulomatous inflammation and the presence of acid-fast bacilli, indicating mediastinal tuberculosis. CONCLUSION: Typically the underlying presentation of mediastinal tuberculosis is mediastinal lymphadenitis. This case was unusual in that we detected an isolated large anterior mediastinal mass accompanied by a relatively small burden of mediastinal lymphadenitis. Cases similar to this have been documented in immunosuppressed patients however in our case no evidence of immunosuppression was found. This case report emphasizes the importance that a detailed and logical pathway of investigation is pursued when encountering a mediastinal mass.

Traumatic central cord syndrome: neurological and functional outcome at 3 years.

STUDY DESIGN: Retrospective cohort analysis with prospective follow-up. OBJECTIVES: To evaluate neurological and functional recovery following central cord syndrome. SETTING: Northern Ireland, population 1.8 million. METHODS: Twenty-seven cords were identified in 1 year. Five managed conservatively and 22 with surgery. American Spinal Injury Association (ASIA) motor scores (AMS) were calculated to assess neurological recovery. Rotterdam scores assessed functional independence at 3 years. RESULTS: Average age was 62 years. Mechanism of injury was a fall with neck hyperextension in 81% patients. Average AMS in surgical patients improved from injury, preoperatively, postoperatively, 6 months and 3 years from 51, 81, 83, 90 to 96, respectively. Conservative patients improved from time of injury to day 10 from 57 to 86 and then fell to 84 at 6 months. By 3 years, this had recovered to 91. There was no statistical significant difference in AMS (P=0.15)/change in AMS (ΔAMS) (P=0.92) or percentage of motor deficit resolution (P=0.23) between groups at 3 years. Two patients underwent surgery within 48 h and achieved full motor recovery by 3 years, but this was not significant (P=0.2). ASIA score improvement had a positive correlation with age at injury. Patients treated with surgery had better Rotterdam scores at 3 years than those managed conservatively (P=0.05). CONCLUSIONS: This study confirms the natural history of central cord syndrome. Although it demonstrates equivocal neurological recovery for both groups, patients treated with surgery regained a greater degree of functional independence.

Assessment of clinical occupational dose reduction effect of a new interventional cardiology shield for radial access combined with a scatter reducing drape.

OBJECTIVES: To assess the occupational dose reduction effect of a new interventional cardiology shield for radial access combined with a scatter reducing drape. BACKGROUND: Transradial access for catheterization has been shown to increase occupational radiation dose. Current shielding techniques are primarily based on the femoral access. This article looks at the clinical occupational combined dose reduction effect of a commercially available shield and drape which is specific to access type. METHODS: The evaluation took place in a busy interventional cardiology laboratory, with a single plane 30×40 cm flat panel detector (Siemens Artis Zee, Germany). Radiation exposure to staff was measured using electronic personal dosimeters (Unfors RaysafeAB, Sweden) placed at the collar. Patient radiation exposure was assessed using screening time and dose area product per case. Both staff and patient radiation exposure were monitored for a number of case types and operators before, during, and after deployment of the new shield and drapes. RESULTS: The cardiologists' overall median collar badge reading per case reduced from 15.4 µSv per case without the shield/drape combination to 7.3 µSv per case with the shield drape combination in situ (P<0.001). The radiographers badge reading was reduced from 4.2 µSv per case without to 2.5 µSv per case with the shield drape combination in situ (P<0.001). There was no statistical difference in the cardiac technician's badge reading. Patient's dose area product was not significantly affected by the placement of the shield and drape combination. CONCLUSIONS: The shield/drape combination can significantly reduce operator exposure in a cardiac catheterization laboratory.

Patient journey to a specialist amyotrophic lateral sclerosis multidisciplinary clinic: an exploratory study.

BACKGROUND: The multidisciplinary approach in the management of Amyotrophic Lateral Sclerosis (ALS) has been shown to provide superior care to devolved care, with better survival, improved quality of care, and quality of life. Access to expert multidisciplinary management should be a standard for patients with ALS. This analysis explores the patient journey from symptom onset and first engagement with health services, to the initial visit to a specialist ALS Multidisciplinary Clinic (MDC) in Dublin, Ireland. METHODS: A retrospective exploratory multi-method study details the patient journey to the MDC. Data from medical interviews and systematic chart review identifies interactions with the health services and key timelines for thirty five new patients presenting with a diagnosis of ALS during a 6 month period in 2013. RESULTS: The time from first symptom to diagnosis was a mean of 16 months (median 13 months), with a mean interval of 19 months (median 14.6) from first symptoms to arrival at the MDC. The majority of patients were seen by a general practitioner, and subsequently by neurology services. There was an average of four contacts with health services and 4.8 investigations/tests, prior to their first Clinic visit. On the first visit to the MDC patients are linked into an integrated 'system' that can provide specialist care and link with voluntary, palliative and community services as required. CONCLUSIONS: Engagement with a multidisciplinary team has implications for service utilization and quality of life of patients and their families. We have demonstrated that barriers exist that delay referral to specialist services. Comprehensive data recording and collection, using multiple data sources can reconstruct the timelines of the patient journey, which can in turn be used to identify pathways that can expedite early referral to specialist services.

A systematic review of the emotional, behavioural and cognitive features exhibited by school-aged children experiencing neglect or emotional abuse.

BACKGROUND: Interventions to minimize the long-term consequences of neglect or emotional abuse rely on prompt identification of these children. This systematic review of world literature (1947-2012) identifies features that children aged 5-14 years experiencing neglect or emotional abuse, as opposed to physical or sexual abuse, may exhibit. METHODS: Searching 18 databases, utilizing over 100 keywords, supplemented by hand searching, 13,210 articles were identified and 111 underwent full critical appraisal by two independent trained reviewers. RESULTS: The 30 included studies highlighted behavioural features (15 studies), externalizing features being the most prominent (8/9 studies) and internalizing features noted in 4/6 studies. Four studies identified attention deficit hyperactivity disorder (ADHD) associated features: impulsivity, inattention or hyperactivity. Child difficulties in initiating or developing friendships were noted in seven studies. Of 13 studies addressing emotional well-being, three highlighted low self-esteem, with a perception of external control (1), or depression (6) including suicidality (1). A negative internal working model of the mother increased the likelihood of depression (1). In assessing cognition or academic performance, lower general intelligence (3/4) and reduced literacy and numeracy (2) were reported, but no observable effect on memory (3). CONCLUSIONS: School-aged children presenting with poor academic performance, ADHD symptomatology or abnormal behaviours warrant assessment of neglect or emotional abuse as a potential underlying cause.

Treating severe and enduring anorexia nervosa: a randomized controlled trial.

BACKGROUND: There are no evidence-based treatments for severe and enduring anorexia nervosa (SE-AN). This study evaluated the relative efficacy of cognitive behavioral therapy (CBT-AN) and specialist supportive clinical management (SSCM) for adults with SE-AN. METHOD: Sixty-three participants with a diagnosis of AN, who had at least a 7-year illness history, were treated in a multi-site randomized controlled trial (RCT). During 30 out-patient visits spread over 8 months, they received either CBT-AN or SSCM, both modified for SE-AN. Participants were assessed at baseline, end of treatment (EOT), and at 6- and 12-month post-treatment follow-ups. The main outcome measures were quality of life, mood disorder symptoms and social adjustment. Weight, eating disorder (ED) psychopathology, motivation for change and health-care burden were secondary outcomes. RESULTS: Thirty-one participants were randomized to CBT-AN and 32 to SSCM with a retention rate of 85% achieved at the end of the study. At EOT and follow-up, both groups showed significant improvement. There were no differences between treatment groups at EOT. At the 6-month follow-up, CBT-AN participants had higher scores on the Weissman Social Adjustment Scale (WSAS; p = 0.038) and at 12 months they had lower Eating Disorder Examination (EDE) global scores (p = 0.004) and higher readiness for recovery (p = 0.013) compared to SSCM. CONCLUSIONS: Patients with SE-AN can make meaningful improvements with both therapies. Both treatments were acceptable and high retention rates at follow-up were achieved. Between-group differences at follow-up were consistent with the nature of the treatments given.

Public perceptions of people with eating disorders: Commentary on results from the 2022 Australian national survey of mental health-related stigma and discrimination.

Mental illness is highly prevalent in the community. As such, significant attention has been paid in recent years to raising awareness of the mental health disorders (including eating disorders). This includes efforts to normalise help-seeking, campaigns to reduce stigma and discrimination, targeted research funding and advocacy for improved and accessible mental health service provision. But have these initiatives changed public attitude? The 2022 National Survey of Mental Health-Related Stigma and Discrimination is the first of four national surveys canvassing the general public's perceptions of people with mental health disorders (including stigmatising and discriminatory beliefs) conducted since 1995 to include eating disorders. It finds significant prejudice against those with mental health disorders still exists within the community, particularly among younger Australians. For eating disorders, this is primarily related to attributions of blame and personal weakness. Findings from the survey are discussed in this commentary.