A further case of chondrodysplasia with growth failure occurring after hematopoietic stem cell transplantation (HSCT).

There is an emerging body of evidence showing that young patients, post haematopoietic stem cell transplantation (HSCT), can develop skeletal changes that mimic an osteochondrodysplasia process. The key discriminator is that these children have had otherwise normal growth and skeletal development before the therapeutic intervention (HSCT), typically for a haematological malignancy. Herein we present that case of a boy who underwent HSCT for Haemophagocytic Lymphohistiocytosis (HLH) aged 2 years. Following Intervention with HSCT this boy's growth has severely decelerated (stature less than 1st centile matched for age) and he has developed a spondyloepiphyseal dysplasia.

Spiraling Risk: Visualizing the multilevel factors that socially pattern HIV risk among gay, bisexual & other men who have sex with men using Complex Systems Theory.

PURPOSE OF REVIEW: Global disparities in HIV infection, particularly among gay, bisexual, and other men who have sex with men (GBMSM), indicate the importance of exploring the multi-level processes that shape HIV's spread. We used Complex Systems Theory and the PRISMA guidelines to conduct a systematic review of 63 global reviews to understand how HIV is socially patterned among GBMSM. The purpose was to conduct a thematic analysis of the reviews to (1) synthesize the multi-level risk factors of HIV risk, (2) categorize risk across the socioecological model, and (3) develop a conceptual model that visualizes the interrelated factors that shape GBMSMS's HIV "risk." RECENT FINDINGS: We included 49 studies of high and moderate quality studies. Results indicated that GBMSM's HIV risk stems from the individual, interpersonal, and structural levels of the socioecological model. We identified a few themes that shape GBMSM's risk of HIV infection related to biomedical prevention methods; sexual and sex-seeking behaviors; behavioral prevention methods; individual-level characteristics and syndemic infections; lived experiences and interpersonal relationships; country-level income; country-level HIV prevalence; and structural stigma. The multi-level factors, in tandem, serve to perpetuate GBMSM's risk of HIV infection globally. The amalgamation of our thematic analyses from our systematic reviews of reviews suggests that the risk of HIV infection operates in an emergent, dynamic, and complex nature across multiple levels of the socioecological model. Applying complex systems theory indicates how multilevel factors create a dynamic and reinforcing system of HIV risk among GBMSM.

Evaluation of a national clinical programme for the management of self-harm in hospital emergency departments: impact on patient outcomes and the provision of care.

BACKGROUND: Emergency departments are important points of intervention, to reduce the risk of further self-harm and suicide. A national programme to standardise the management of people presenting to the emergency department with self-harm and suicidal ideation (NCPSHI) was introduced in Ireland in 2014. The aim of this study was to evaluate the impact of the NCPSHI on patient outcomes and provision of care. METHODS: Data on self-harm presentations were obtained from the National Self-Harm Registry Ireland from 2012 to 2017. The impacts of the NCPSHI on study outcomes (3-month self-harm repetition, biopsychosocial assessment provision, admission, post-discharge referral, and self-discharge) were examined at an individual and aggregate (hospital) level, using a before and after study design and interrupted time series analyses, respectively. The 15 hospitals that implemented the programme by January 2015 (of a total of 24 between 2015 and 2017) were included in the analyses. RESULTS: There were 31,970 self-harm presentations during the study period. In hospitals with no service for self-harm (n = 4), risk of patients not being assessed reduced from 31.8 to 24.7% following the introduction of the NCPSHI. Mental health referral in this hospital group increased from 42.2 to 59.0% and medical admission decreased from 27.5 to 24.3%. Signs of a reduction in self-harm repetition were observed for this hospital group, from 35.1 to 30.4% among individuals with a history of self-harm, but statistical evidence was weak. In hospitals with a pre-existing liaison psychiatry service (n = 7), risk of self-discharge was lower post-NCPSHI (17.8% vs. 14.8%). In hospitals with liaison nurse(s) pre-NCPSHI (n = 4), medical admission reduced (27.5% vs. 24.3%) and there was an increase in self-harm repetition (from 5.2 to 7.8%. for those without a self-harm history). CONCLUSION: The NCPSHI was associated with improvements in the provision of care across hospital groups, particularly those with no prior service for self-harm, highlighting the need to consider pre-existing context in implementation planning. Our evaluation emphasises the need for proper resourcing to support the implementation of clinical guidelines on the provision of care for people presenting to hospital with self-harm.

National Guidelines on the Provision of Outpatient Parenteral Antimicrobial Therapy (OPAT).

Aim Outpatient parenteral antimicrobial therapy (OPAT) is an option in patients who require parenteral antimicrobial administration and are clinically well enough for hospital discharge. This is an update of the Irish National OPAT guidelines which were last reviewed in 2011. Methods The guideline was devised through a collaborative process with the national OPAT Working Group and a review of the literature. It is intended for clinicians who prescribe any intravenous (IV) antimicrobials outside of the inpatient setting in the Republic of Ireland. Results Patient care while on OPAT should be provided by a designated OPAT service, with clear managerial and clinical governance lines of responsibility. It should be conducted using a team approach with a clinical lead on each site either as an infection specialist, or a general medical physician with infection specialist input and an OPAT nurse. An antimicrobial pharmacist is also desirable. Several factors must be considered when assessing patient's suitability for OPAT including exclusion criteria, infection-specific factors, and patient specific factors such as physical, social and logistic criteria. Conclusion This updated guideline advocates a more individualised OPAT approach, with the recognition that specific antimicrobials and/or specific delivery models may be more appropriate for certain patient groups. Full guidelines are available through www.opat.ie.

Measurements of Nonsinglet Moments of the Nucleon Structure Functions and Comparison to Predictions from Lattice QCD for Q

We present extractions of the nucleon nonsinglet moments utilizing new precision data on the deuteron F_{2} structure function at large Bjorken-x determined via the Rosenbluth separation technique at Jefferson Lab Experimental Hall C. These new data are combined with a complementary set of data on the proton previously measured in Hall C at similar kinematics and world datasets on the proton and deuteron at lower x measured at SLAC and CERN. The new Jefferson Lab data provide coverage of the upper third of the x range, crucial for precision determination of the higher moments. In contrast to previous extractions, these moments have been corrected for nuclear effects in the deuteron using a new global fit to the deuteron and proton data. The obtained experimental moments represent an order of magnitude improvement in precision over previous extractions using high x data. Moreover, recent exciting developments in lattice QCD calculations provide a first ever comparison of these new experimental results with calculations of moments carried out at the physical pion mass, as well as a new approach that first calculates the quark distributions directly before determining moments.

High Rates of Diabetic Ketoacidosis in Patients with New and Known Type 1 Diabetes over a Six-Year Period

Aims To analyse all paediatric patients who presented with diabetic ketoacidosis (DKA) from 2012 to 2017. Methods A retrospective observational study was carried out analysing all cases of diabetic ketoacidosis admitted to a regional centre from 2012-2017. Results We identified 133 cases of DKA, 81 (61%) were newly diagnosed patients and 52 (39%) were patients with known T1DM. There were 215 new diagnoses of T1DM during the study period giving a DKA rate at diagnosis of 38%. Among the 52 cases with established T1DM, 13 cases (25%) presented in severe DKA and 37 cases (71%) occurred in adolescents aged over 12 years. Precipitating factors included chronic suboptimal control and psychosocial factors (28/52), acute illness (16/52), and pump technical failure (5/52). There were two cases treated for suspected cerebral oedema and one case each of subarachnoid haemorrhage and cardiac arrhythmia. Conclusion The current proportion of new T1DM presenting in DKA is higher than international data. The high frequency of DKA in known T1DM indicates a need for particular focus on adolescents.

Development of an Insulin-Prescribing Chart for Paediatric Diabetes

Aim Our aim was to design a new insulin prescribing tool in compliance with the Irish Medicines Safety Network recommendations. Methods In 2015, we undertook a review of the existing paediatric subcutaneous insulin-prescribing sheet introduced to Cork University Hospital in 2013. This involved a retrospective analysis of 15 consecutive in-patient insulin prescribing charts and a questionnaire distributed to health professionals. Following this a new insulin prescribing chart was designed and implemented in 2016 and a re-audit was performed in 2017. Results The 2017 re-audit demonstrated that the new insulin chart was viewed as easier (95% of previous users n=18) and safer (n=16) to use. There was less confusion (2017: 28%, n=11/39 vs 2015: 50%, n=17/34 2015) and the ALERT system helped staff standardise hypo/hyperglycaemia management (71%, n=28). Conclusion The new paediatric insulin prescribing chart has improved safety and ease of prescribing insulin. The colour coded quasi graph and ALERT system has made it easier to appreciate capillary blood glucose trends and manage them safely.

A Unique Case of Concurrent Long Bone Osteomyelitis and Empyema Caused by Fusobacterium nucleatum.

We describe a patient presenting with constitutional symptoms, poor oral hygiene and leg swelling who was diagnosed with Fusobacterium nucleatum osteomyelitis of femur and empyema. Long bone osteomyelitis is rarely caused by this microorganism. This unusual case was successfully managed with drainage and antimicrobial therapy.

Prevalence and characteristics of paediatric Type 2 diabetes in the Republic of Ireland.

OBJECTIVE: To establish the prevalence of paediatric Type 2 diabetes in the Republic of Ireland and describe patient demographics, initial presentation, management, outcomes, comorbidities and complications. METHODS: Using a standardized proforma we conducted a cross-sectional survey of children and adolescents aged < 16 years with a diagnosis of Type 2 diabetes between October and December 2015 in each of the 19 centres in the Republic of Ireland responsible for the care of children with diabetes. RESULTS: Twelve cases of Type 2 diabetes were identified, giving a prevalence in children aged <16 years of 1.2/100 000 (95% CI 0.6 to 2). Six of these children (50%) were white, two (33%) of whom were members of the travelling community. Four (33%) were of black ethnicity. The prevalence of Type 2 diabetes in traveller children was 16.1/100 000 (95% CI 1.9 to 58.1) and was similar to that in black children, a known high-risk group, which was 13.3/100 000 (95% CI 3.6 to 34.1). The median current HbA1c value was 51 mmol/mol (6.8%) and four (33%) of the children achieved the International Society for Pediatric and Adolescent Diabetes target HbA1c of ≤48 mmol/mol (6.5%). Seven (59%) children were managed on metformin monotherapy, three (25%) were managed on insulin and metformin in combination, and two (16%) were receiving dietary management. CONCLUSION: This was the first national study to estimate the prevalence of childhood Type 2 diabetes in Ireland. Despite their white ethnicity, traveller children appear to be a high-risk group, but this finding requires further study.

A comprehensive scoring system to evaluate patient-centred risk factors regarding lower extremity amputation.

Care of the patient with a presumed life- or limb-threatening lower extremity wound poses many challenges. The mindset regarding potential outcomes of such conditions is mostly driven by the experiences and expertise of those providing the care. This mindset generally appears as two primary actions presented to the afflicted patient: attempted resolution of the problem via medical, surgical or combination treatment, with the hope of low recurrence risk, or exacerbation and amputation-amputations at a level sufficient to, at least in the mind of the surgeon, eliminate the problem. Achieving the former outcome is dependent on a number of factors associated with both patient and caregiver. If healing is achieved, the secondary goal of prevention of recurrence may be no less arduous, with failure most likely resulting in amputation. Clearly, these considerations appear to be based more on the health professionals perception, of the patient's physical and medical status rather than on patient-centred considerations. This article will review considerations and recommendations for lower extremity amputation, and the short- and long-term implications. Based on our research, there is clear need for a set of criteria against which to weigh not just the medical issues, but also definitive patient-centred issues when considering a lower extremity amputation. We offer a set of patient-centred, easily verified and recognised criteria that we believe addresses this need. The goal of the Miller-Newgent Amputation Scale (MENACE) is to provide a decision base from which to consider and evaluate all factors in determining the need for a lower extremity amputation. This involves identification of patient-centred issues, which are likely to produce satisfactory short- and long-term physical and quality-of-life outcomes if the amputation does proceed.

Degradation of spent craft brewer's yeast by caprine rumen hyper ammonia-producing bacteria.

UNLABELLED: Spent yeast from craft beers often includes more hops (Humulus lupulus L.) secondary metabolites than traditional recipes. These compounds include α- and ß- acids, which are antimicrobial to the rumen hyper ammonia-producing bacteria (HAB) that are major contributors to amino acid degradation. The objective was to determine if the hops acids in spent craft brewer's yeast (CY; ~ 3·5 mg g(-1) hops acids) would protect it from degradation by caprine rumen bacteria and HAB when compared to a baker's yeast (BY; no hops acids). Cell suspensions were prepared by harvesting rumen fluid from fistulated goats, straining and differential centrifugation. The cells were re-suspended in media with BY or CY. After 24 h (39°C), HAB were enumerated and ammonia was measured. Fewer HAB and less ammonia was produced from CY than from BY. Pure culture experiments were conducted with Peptostreptococcus anaerobiusBG1 (caprine HAB). Ammonia production by BG1 from BY was greater than from CY. Ammonia production was greater when exogenous amino acids were included, but similar inhibition was observed in CY treatments. These results indicate that rumen micro-organisms deaminated the amino acids in CY to a lesser degree than BY. SIGNIFICANCE AND IMPACT OF THE STUDY: Spent brewer's yeast has long been included in ruminant diets as a protein supplement. However, modern craft beers often include more hops (Humulus lupulus L.) than traditional recipes. These compounds include α- and ß- acids, which are antimicrobial to the rumen hyper ammonia-producing bacteria (HAB) that are major contributors to amino acid degradation. This study demonstrated that hops acids in spent craft brewer's yeast protected protein from destruction by HABin vitro. These results suggest that the spent yeast from craft breweries, a source of beneficial hops secondary metabolites, could have value as rumen-protected protein.

Australian general practitioners' views regarding providing nutrition care: results of a national survey.

BACKGROUND: The prevalence of chronic disease is considerable, and dietary behaviours influence the progression of many chronic diseases. Practice guidelines recommend that general practitioners (GPs) promote healthy dietary behaviours in relevant consultations with patients in order to improve health outcomes at a population level. OBJECTIVE: To describe GPs' perceived interest, confidence and barriers to support patients to have a healthy diet. METHOD: A 24-item online and written survey was distributed in a national weekly newsletter to GPs in Australia. Results were descriptively analysed and investigated for associations with GPs' demographic characteristics. RESULTS: A total of 322 GPs responded to the survey. Nearly all (n = 295, 91.6%) were interested in supporting patients to eat well, and most (n = 231, 71.7%) reported moderately high confidence for providing nutrition care with clear public health messages for conditions, such as cardiovascular disease. Many GPs (n = 170, 52.8%) cited lack of time as the biggest barrier to providing nutrition care, and the overwhelming majority (n = 289, 89.8%) were interested in receiving additional education and training to enhance their nutrition knowledge and skills. DISCUSSION: Many GPs are interested in nutrition and would benefit from educational programmes that improve their competence to provide nutrition care. Professional development opportunities should focus on the identification of nutritional risk and the promotion of healthy dietary behaviours within the time constraints of a standard consultation.

A National Survey on the Diagnosis and Treatment of Paediatric Growth Hormone Deficiency.

Many countries have established regulations regarding growth hormone (GH) treatment in children, to standardise care and reduce cost. In this study, we describe current practice in Ireland surrounding child measurement and the approach to diagnosis of GH deficiency. A questionnaire was sent to 139 paediatricians in Ireland and 35 (9 paediatric endocrinologists) responded. Only 13 (37.1%) use the recommended 2-person technique for measuring children under 2. Amongst GH prescribers, there were a variety of GH Stimulation Tests used, sex steroid priming was used by 8 (80%) and the general cut off for a passed test was consistent (7ng/ml). Brand rotation (n=5, 50%) and cost (n=3, 30%) were the most common criteria for deciding the formulation of GH prescribed. We recommend that departments review their child measurement technique and equipment. We also advise the establishment of national guidelines for the use of GH, and a prospective registry for GH treated children.

Providing NHS staff with height-adjustable workstations and behaviour change strategies to reduce workplace sitting time: protocol for the Stand More AT (SMArT) Work cluster randomised controlled trial.

BACKGROUND: High levels of sedentary behaviour (i.e., sitting) are a risk factor for poor health. With high levels of sitting widespread in desk-based office workers, office workplaces are an appropriate setting for interventions aimed at reducing sedentary behaviour. This paper describes the development processes and proposed intervention procedures of Stand More AT (SMArT) Work, a multi-component randomised control (RCT) trial which aims to reduce occupational sitting time in desk-based office workers within the National Health Service (NHS). METHODS/DESIGN: SMArT Work consists of 2 phases: 1) intervention development: The development of the SMArT Work intervention takes a community-based participatory research approach using the Behaviour Change Wheel. Focus groups will collect detailed information to gain a better understanding of the most appropriate strategies, to sit alongside the provision of height-adjustable workstations, at the environmental, organisational and individual level that support less occupational sitting. 2) intervention delivery and evaluation: The 12 month cluster RCT aims to reduce workplace sitting in the University Hospitals of Leicester NHS Trust. Desk-based office workers (n = 238) will be randomised to control or intervention clusters, with the intervention group receiving height-adjustable workstations and supporting techniques based on the feedback received from the development phase. Data will be collected at four time points; baseline, 3, 6 and 12 months. The primary outcome is a reduction in sitting time, measured by the activPAL(TM) micro at 12 months. Secondary outcomes include objectively measured physical activity and a variety of work-related health and psycho-social measures. A process evaluation will also take place. DISCUSSION: This study will be the first long-term, evidence-based, multi-component cluster RCT aimed at reducing occupational sitting within the NHS. This study will help form a better understanding and knowledge base of facilitators and barriers to creating a healthier work environment and contribute to health and wellbeing policy. TRIAL REGISTRATION: ISRCTN10967042 . Registered 2 February 2015.

Disordered eating is not associated with musculoskeletal injury in university athletes.

TAKE HOME MESSAGE: Musculoskeletal injuries and disordered eating are prevalent in varsity-level athletes but are not associated in our participants.

Mutations in PROP1 cause familial combined pituitary hormone deficiency.

Combined pituitary hormone deficiency (CPHD) in man denotes impaired production of growth hormone (GH) and one or more of the other five anterior pituitary hormones. Mutations of the pituitary transcription factor gene POU1F1 (the human homologue of mouse Pit1) are responsible for deficiencies of GH, prolactin and thyroid stimulating hormone (TSH) in Snell and Jackson dwarf mice and in man, while the production of adrenocorticotrophic hormone (ACTH), luteinizing hormone (LH) and follicle stimulating hormone (FSH) is preserved. The Ames dwarf (df) mouse displays a similar phenotype, and appears to be epistatic to Snell and Jackson dwarfism. We have recently positionally cloned the putative Ames dwarf gene Prop1, which encodes a paired-like homeodomain protein that is expressed specifically in embryonic pituitary and is necessary for Pit1 expression. In this report, we have identified four CPHD families with homozygosity or compound heterozygosity for inactivating mutations of PROP1. These mutations in the human PROP1 gene result in a gene product with reduced DNA-binding and transcriptional activation ability in comparison to the product of the murine df mutation. In contrast to individuals with POU1F1 mutations, those with PROP1 mutations cannot produce LH and FSH at a sufficient level and do not enter puberty spontaneously. Our results identify a major cause of CPHD in humans and suggest a direct or indirect role for PROP1 in the ontogenesis of pituitary gonadotropes, as well as somatotropes, lactotropes and caudomedial thyrotropes.

Effects of a Multi-component, Resistance-Based Exercise Program Combined with Additional Lean Red Meat on Health-Related Quality of Life in Older Adults: Secondary Analysis of a 6-Month Randomized Controlled Trial.

OBJECTIVES: To assess whether consumption of lean red meat on three exercise training days per week can promote greater improvements than exercise alone in health-related quality of life (HR-QoL) in community-dwelling older adults. DESIGN: This study is a secondary analysis from a 6 month, two-arm, parallel randomized controlled trial conducted in 2014 and 2015. SETTING: Community-dwelling older adults living in metropolitan Melbourne, Australia. PARTICIPANTS: One hundred and fifty-four men and women aged ≥65 years. INTERVENTION: All participants were enrolled in a multi-component, resistance-based exercise program (3 d/week) and randomly allocated to either a group asked to consume lean red meat (2x80g cooked servings/day) on each of the three training days (Ex+Meat, n=77) or a control group asked to consume one serving of carbohydrates (1/2 cup rice/pasta or 1 medium potato; Ex+C, n=77). MEASUREMENTS: HR-QoL was assessed using the Short-Form (SF)-36 health survey. RESULTS: Overall 62% of the participants were female, the mean age was 70.7 years (range 65 to 84 years), approximately 67% of participants were classified as either overweight or obese, and the average number of chronic conditions was two. A total of 145 participants (94%) completed the study. Mean baseline HR-QoL scores were comparable to the mean for the Australian population [Global HR-QoL (mean ± SD): Ex+Meat, 49.99 ± 6.57; Ex+C, 50.49 ± 5.27]. General Linear Mixed Models examining within and between group changes over time revealed that after 6 months, there were no within-group changes in either Ex+Meat or Ex+C nor any between-group differences for any measure of HR-QoL, with the exception that the mental health subscale improved in Ex+C versus Ex+Meat [net difference for change, -2.32 (95% CI), -4.73, 0.09, P=0.048] after adjusting for relevant covariates and the physical function subscale improved in Ex+Meat relative to baseline [mean change (95% CI), 1.88 (0.37, 3.39), P=0.011]. CONCLUSION: A multi-component resistance-based training program performed with and without the provision of lean red meat in line with current Australian dietary guidelines on each of the three training days, did not improve HR-QoL in healthy community-dwelling older adults.

New measurements of high-momentum nucleons and short-range structures in nuclei.

We present new measurements of electron scattering from high-momentum nucleons in nuclei. These data allow an improved determination of the strength of two-nucleon correlations for several nuclei, including light nuclei where clustering effects can, for the first time, be examined. The data also include the kinematic region where three-nucleon correlations are expected to dominate.