Medullary thyroid cancer in a 9-week-old infant with familial MEN 2B: Implications for timing of prophylactic thyroidectomy

BACKGROUND: Patients with Multiple Endocrine Neoplasia type 2 (MEN 2) are at high risk of developing aggressive medullary thyroid carcinoma (MTC) in childhood, with the highest risk in those with MEN type 2B (of whom >95% have an M918T RET proto-oncogene mutation). Metastatic MTC has been reported as young as 3 months of age. Current guidelines recommend prophylactic thyroidectomy within the first year of life for MEN 2B. PATIENT FINDINGS: We report a 9-week-old infant with MTC due to familial MEN 2B. A full-term male infant, born to a mother with known MEN 2B and metastatic MTC, had an M918T RET proto-oncogene mutation confirmed at 4 weeks of age. He underwent prophylactic total thyroidectomy at 9 weeks of age. Pathology showed a focal calcitonin-positive nodule (2.5 mm), consistent with microscopic MTC. SUMMARY: This case highlights the importance of early prophylactic thyroidectomy in MEN 2B. Although current guidelines recommend surgery up to a year of life, MTC may occur in the first few weeks of life, raising the question of how early we should intervene. In this report, we discuss the risks, benefits and barriers to performing earlier thyroidectomy, soon after the first month of life, and make suggestions to facilitate timely intervention. Prenatal anticipatory surgical scheduling could be considered in familial MEN 2B. Multidisciplinary collaboration between adult and pediatric specialists is key to the optimal management of the infant at risk

What is the risk of death or severe harm due to bone cement implantation syndrome among patients undergoing hip hemiarthroplasty for fractured neck of femur? A patient safety surveillance study.

OBJECTIVE: To estimate the risk of death or severe harm due to bone cement implantation syndrome (BCIS) among patients undergoing hip hemiarthroplasty for fractured neck of femur. SETTING: Hospitals providing secondary and tertiary care throughout the National Health Service (NHS) in England and Wales. PARTICIPANTS: Cases reported to the National Reporting and Learning System (NRLS) in which the reporter clearly describes severe acute patient deterioration associated with cement use in hip hemiarthroplasty for fractured neck of femur (assessed independently by two reviewers). OUTCOME MEASURES: Primary-number of reported deaths, cardiac arrests and periarrests per year. Secondary-timing of deterioration and outcome in relation to cement insertion. RESULTS: Between 2005 and 2012, the NRLS received 62 reports that clearly describe death or severe harm associated with the use of cement in hip hemiarthroplasty for fractured neck of femur. There was one such incident for every 2900 hemiarthroplasties for fractured neck of femur during the period. Of the 62 reports, 41 patients died, 14 were resuscitated from cardiac arrest and 7 from periarrest. Most reports (55/62, 89%) describe acute deterioration occurring during or within a few minutes of cement insertion. The vast majority of deaths (33/41, 80%) occurred on the operating table. CONCLUSIONS: These reports provide narrative evidence from England and Wales that cement use in hip hemiarthroplasty for fractured neck of femur is associated with instances of perioperative death or severe harm consistent with BCIS. In 2009, the National Patient Safety Agency publicised this issue and encouraged the use of mitigation measures. Three-quarters of the deaths in this study have occurred since that alert, suggesting incomplete implementation or effectiveness of those mitigation measures. There is a need for stronger evidence that weighs the risks and benefits of cement in hip hemiarthroplasty for fractured neck of femur

Pharmacists in Pharmacovigilance: Can Increased Diagnostic Opportunity in Community Settings Translate to Better Vigilance?

The pharmacy profession has undergone substantial change over the last two to three decades. Whilst medicine supply still remains a central function, pharmacist’s roles and responsibilities have become more clinic and patient focused. In the community (primary care), pharmacists have become important providers of healthcare as Western healthcare policy advocates patient self-care. This has resulted in pharmacists taking on greater responsibility in managing minor illness and the delivery of public health interventions. These roles require pharmacists to more fully use their clinical skills, and often involve diagnosis and therapeutic management. Community pharmacists are now, more than ever before, in a position to identify, record and report medication safety incidents. However, current research suggests that diagnostic ability of community pharmacists is questionable and they infrequently report to local or national schemes. The aim of this paper is to highlight current practice and suggest ways in which community pharmacy can more fully contribute to patient safety

The contribution of social work and social care to the reduction of health inequalities: four case studies

•Inequalities in UK health outcomes are widening. Although the Marmot Review makes few references to social work and social care, it concedes that social care recipients are likely to be among the most disadvantaged of populations. •There is no systematic body of research evidence that focuses on whether social work and social care has a direct effect on the distribution of health outcomes at the individual or population level. •However, the health of most users of social care services is already damaged and for many this is a central factor in their involvement with social care services. Social work and care interventions targeted at social care clients, and at the general population, can reduce health disadvantage across the life course. •To illustrate this, we chose interventions from across the life course. Studies referred to all measured impact on health. They are Sure Start/Children’s Centres; the placement of looked-after children with kinship carers; support for parents with intellectual disabilities, and extra care housing for older people at risk of entering care. Research shows that social interventions impact on health disadvantage in individuals and populations, but to impact on health inequality, they must be adequately and sustainably resourced. •The key message of this briefing is that social work and social care practitioners work with some of the most disadvantaged groups in our society, and this offers many opportunities to improve the lifetime health of those most at risk. The achievement of health benefits and healthcare cost savings requires financial and research investment in the social care sector

Variability in cecal intubation rate by calculation method:a call for standardization of key performance indicators in endoscopy

Background and Aims The cecal intubation rate (CIR) is a widely accepted key performance indicator (KPI) in colonoscopy but lacks a universal calculation method. We aimed to assess whether differences in CIR calculation methods could impact on perceived trainee outcomes. Methods A systematic review of CIR calculation methods was conducted on major societal guidelines (United Kingdom, European Society of Gastrointestinal Endoscopy [ESGE] and American Society for Gastrointestinal Endoscopy [ASGE]) and trainee-inclusive studies. Trainees awarded colonoscopy certification between June 2011 and 2016 were identified from the United Kingdom e-portfolio and selected as a validation cohort. For each trainee, both the crude and unassisted CIR were calculated over 50 post-certification procedures using definitions from the 3 international guidelines. The resulting CIRs, and the proportions of endoscopists failing to meet the minimum standard of CIR ≥90%, were then compared across these definitions. Results Across the 3 guidelines and 37 eligible studies identified, differences in CIR calculation methodology were demonstrated. These related to adjustment criteria (18 studies), and whether unassisted CIR was stipulated (18 studies). In the validation cohort of 733 trainees (36,650 procedures), the median crude CIR ranged from 96% (ESGE) to 98% (ASGE) [p<0.001], and whether unassisted CIR was specified (ESGE: 94%, ASGE: 96%, P<0.001). The proportion of trainees failing to achieve CIR ≥90% varied significantly across the different definitions, from 4.9% in the crude ASGE definition, to 18.6% in the unassisted ESGE definition (p<0.001). Conclusions CIR calculation methods vary between guidelines and research studies, which impact on trainee performance measures. With CIR used as an example, this study highlights the need for standardized definitions and calculations of KPIs in endoscopy.No Full Tex

Adolescent Self-Organization and Adult Smoking and Drinking over Fifty Years of Follow-Up:The British 1946 Birth Cohort

Variations in markers of adolescent self-organization predict a range of economic and health-related outcomes in general population studies. Using a population-based birth cohort study we investigated associations between adolescent self-organization and two common factors over adulthood influencing health, smoking and alcohol consumption. The MRC National Survey of Health and Development (the British 1946 birth cohort) was used to test associations between a dimensional measure of adolescent self-organization derived from teacher ratings, and summary longitudinal measures of smoking and alcohol consumption over the ensuing five decades. Multinomial regression models were adjusted for sex, adolescent emotional and conduct problems, occupational social class of origin, childhood cognition, educational attainment and adult occupational social class. With all covariates adjusted, higher adolescent self-organization was associated with fewer smoking pack years, although not with quitting; there was no association with alcohol consumption across adulthood (none or heavy compared with light to moderate). Adolescent self-organization appears to be protective against smoking, but not against heavy alcohol consumption. Interpretation of this differential effect should be embedded in an understanding of the social and sociodemographic context in which these health behaviours occur over time

Mortality from pandemic A/H1N1 2009 influenza in England: public health surveillance study

Objective To establish mortality from pandemic A/H1N1 2009 influenza up to 8 November 2009