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Recently, a novel group of CD34+ and S100+ spindle cell tumors with distinctive stromal and perivascular hyalinization showing recurrent gene fusions involving RAF1, BRAF, NTRK1/2/3, and RET has been identified. ALK rearrangements have been rarely reported in this group of tumors. We report a 24-year-old woman with a 1.5-cm pink mass of the scalp. The tumor was made of spindle cells organized in fascicles or haphazardly arranged in a patternless architecture, with areas of stromal and perivascular hyalinization. The tumor cells diffusely expressed CD34 and S100, without SOX-10 expression. The tumor showed diffuse immunopositivity for ALK. RNA sequencing using next-generation sequencing (NGS) detected an EML4-ALK fusion. This case extends the spectrum of this newly described group of CD34+/S100+ spindle cell tumors at the molecular-genetic level. Dermatopathologists should be aware of this recent entity, as it may fall in the differential diagnosis of many other spindle cell tumors with CD34 expression. NGS-based techniques should be performed when facing spindle cell tumors with similar morphology and immunophenotype. Identification of kinase fusions is essential for the precise classification and better knowledge of these tumors, and for targeted therapy in rare aggressive cases.

Small pelagic fishes are used for human consumption, fishmeal and fish oil. They constitute 25% of global fish catch and have been of considerable conservation concern because of their intermediate position in aquatic food webs, often being a dominant dietary component of marine predators. This paper provides an overview of trends in abundance and fishing pressure on small pelagic fish stocks from single-species scientific assessments that constitute 60% of global small pelagic catch. While most individual stocks have exhibited wide variability in abundance (typical of small pelagics compared with other fish taxa), across stocks there has been remarkable stability in average fishing pressure and biomass since 1970. On average, since 1970, the biomass of assessed small pelagic stocks is estimated to have been slightly above the biomass that would produce maximum sustainable yield, but estimation of this quantity for highly fluctuating stocks is quite uncertain. There were significant differences among assessed regions, with the Mediterranean and Black Sea of greatest concern for high and growing fishing pressure. The 40% of global small pelagic fish catch not covered by single-species quantitative stock assessments since 1970 comes largely from Asia, where catches have continued to increase. At regional levels, the average abundance of assessed small pelagic fish is largely unrelated to average fishing pressure, which we argue results both from the portfolio effect, where numerous stocks fluctuate with little correlation in abundance, and from the short life span of small pelagics coupled with recruitment largely independent of spawning abundance. publishedVersion

Many of the world's fisheries are unassessed, with little information about population status or risk of overfishing. Unassessed fisheries are particularly predominant in developing countries and in small-scale fisheries, where they are important for food security. Several catch-only methods based on time series of fishery catch and commonly available life-history traits have been developed to estimate stock status (defined as biomass relative to biomass at maximum sustainable yield: B/B-MSY). While their stock status performance has been extensively studied, performance of catch-only models as a management tool is unknown. We evaluated the extent to which a superensemble of three prominent catch-only models can provide a reliable basis for fisheries management and how performance compares across management strategies that control catch or fishing effort. We used a management strategy evaluation framework to determine whether a superensemble of catch-only models can reliably inform harvest control rules (HCRs). Across five simulated fish life histories and two harvest-dynamic types, catch-only models and HCR combinations reduced the risk of overfishing and increased the proportion of stocks above B-MSY compared to business as usual, though often resulted in poor yields. Precautionary HCRs based on fishing effort were robust and insensitive to error in catch-only models, while catch-based HCRs caused high probabilities of overfishing and more overfished populations. Catch-only methods tended to overestimate B/B-MSY for our simulated data sets. The catch-only superensemble combined with precautionary effort-based HCRs could be part of a stepping stone approach for managing some data-limited stocks while working towards more data-moderate assessment methods.

Background Communication among team members within hospitals is typically fragmented. Bedside interdisciplinary rounds (IDR) have the potential to improve communication and outcomes through enhanced structure and patient engagement. Objective To decrease length of stay (LOS) and complications through the transformation of daily IDR to a bedside model. Design Controlled trial. Setting 2 geographic areas of a medical unit using a clinical microsystem structure. Patients 2005 hospitalizations over a 12-month period. Interventions A bedside model (mobile interdisciplinary care rounds [MICRO]) was developed. MICRO featured a defined structure, scripting, patient engagement, and a patient safety checklist. Measurements The primary outcomes were clinical deterioration (composite of death, transfer to a higher level of care, or development of a hospital-acquired complication) and length of stay (LOS). Patient safety culture and perceptions of bedside interdisciplinary rounding were assessed pre- and postimplementation.. Results There was no difference in LOS (6.6 vs 7.0 days, P = 0.17, for the MICRO and control groups, respectively) or clinical deterioration (7.7% vs 9.3%, P = 0.46). LOS was reduced for patients transferred to the study unit (10.4 vs 14.0 days, P = 0.02, for the MICRO and control groups, respectively). Nurses and hospitalists gave significantly higher scores for patient safety climate and the efficiency of rounds after implementation of the MICRO model. Limitations The trial was performed at a single hospital. Conclusions Bedside IDR did not reduce overall LOS or clinical deterioration. Future studies should examine whether comprehensive transformation of medical units, including co-leadership, geographic cohorting of teams, and bedside interdisciplinary rounding, improves clinical outcomes compared to units without these features. Journal of Hospital Medicine 2017;12:137-142.

Fishery managers must often reconcile conflicting estimates of population status and trend. Superensemble models, commonly used in climate and weather forecasting, may provide an effective solution. This approach uses predictions from multiple models as covariates in an additional "superensemble" model fitted to known data. We evaluated the potential for ensemble averages and superensemble models (ensemble methods) to improve estimates of population status and trend for fisheries. We fit four widely applicable data-limited models that estimate stock biomass relative to equilibrium biomass at maximum sustainable yield (B/BMSY). We combined these estimates of recent fishery status and trends in B/BMSY with four ensemble methods: an ensemble average and three superensembles (a linear model, a random forest and a boosted regression tree). We trained our superensembles on 5,760 simulated stocks and tested them with cross-validation and against a global database of 249 stock assessments. Ensemble methods substantially improved estimates of population status and trend. Random forest and boosted regression trees performed the best at estimating population status: inaccuracy (median absolute proportional error) decreased from 0.42 -0.56 to 0.32 -0.33, rank-order correlation between predicted and true status improved from 0.02 - 0.32 to 0.44 - 0.48 and bias (median proportional error) declined from - 0.22 - 0.31 to - 0.12 - 0.03. We found similar improvements when predicting trend and when applying the simulation-trained superensembles to catch data for global fish stocks. Superensembles can optimally leverage multiple model predictions; however, they must be tested, formed from a diverse set of accurate models and built on a data set representative of the populations to which they are applied.

A 62-year-old human immunodeficiency virus-positive man was admitted for multiple cutaneous and subcutaneous nodules on his lower limbs, corresponding to an undifferentiated proliferation of spindle and pleomorphic cells, with irregular nuclei and numerous mitoses. The tumor cells were negative for a large panel of immunohistochemical markers, except CD10. MDM2 immunohistochemical staining was also negative, leading to the diagnosis of Federation Nationale des Centres de Lutte contre le Cancer grade III undifferentiated pleomorphic sarcoma (UPS). Array-comparative genomic hybridization showed a highly complex karyotype, with amplification of the 4q12 region, an area that contains only the platelet-derived growth factor receptor α (PDGFRa) gene. This amplification of PDFGRa, molecular hallmark of intimal sarcoma (IS), led to the diagnosis of skin IS metastasis. A positron emission tomography showed a hypermetabolic mass protruding in the preaortic area, consistent with the diagnosis of aortic IS. Our study shows that a rare differential diagnosis in peripheral UPS can be IS skin metastasis, and underlines the importance of molecular analyses in UPS.

Background Pilomatricomas are tumors that emulate the differentiation of matrix cells of the hair follicle, showing cortical differentiation, with sequential expression of K35 and K31 keratins. Beta-catenin gene is frequently mutated in pilomatricoma, leading to beta-catenin nuclear accumulation, and to downstream expression of LEF1. Skin matrical tumors other than pilomatricoma are very rare, and comprise purely matrical tumors and focally matrical tumors. We aimed at studying cortical differentiation, beta-catenin pathway and expression of the follicular stem-cell marker PHLDA1 in a series of matrical tumors other than pilomatricoma. Methods In 36 prospectively collected tumors, K31, K35, CK17, LEF1, HOXC13, beta-catenin and PHLDA1 expressions were evaluated. Five pilomatricomas were used as controls. Results In 18 purely matrical tumors (11 matrical carcinomas, 4 melanocytic matricomas, 3 matricomas) and 18 focally matrical tumors (11 basal cell carcinomas, 3 trichoepithelioma/trichoblastomas, 4 others), sequential K35, HOXC13 and K31 expressions were found, indicating cortical differentiation. Germinative matrix cells were always CK17–, and showed nuclear beta-catenin accumulation, with LEF1 and PHLDA1 expressions. Conclusions Nuclear beta-catenin and LEF1 expression was highly conserved in matrical tumors, and suggested a common tumorigenesis driven by Wnt pathway activation. PHLDA1 was consistently expressed in matrical tumors and in areas of matrical differentiation.

Aims. To assess the performance of the non-contact infrared thermometer compared with mercury-in-glass thermometer in children; to assess the diagnostic accuracy of non-contact infrared thermometer for detecting children with fever; to compare the discomfort caused by the two procedures in children aged > one month. Background. Non-contact infrared thermometer is a quick and non-invasive method to measure body temperature, not requiring sterilisation or disposables. It is a candidate for temperature recording in children. Design. Prospective multicenter study. Methods. Body temperature readings were taken from every child consecutively admitted to the Pediatric Emergency Departments or Pediatric Clinics participating in the study. Two bilateral axillary temperature measurements using the mercuryin-glass thermometers and three mid-forehead temperature measurements using the non-contact infrared thermometer were performed. Results. Two hundred and fifty-one children were enrolled in the study. Mean body temperature obtained by mercury-in-glass thermometer and non-contact infrared thermometer was 37AE18 (SD 0AE96) � C and 37AE30 (SD 0AE92) � C, respectively (p =0 AE153). Non-contact infrared thermometer clinical repeatability was 0AE108 (SD 0AE095) � C, similar to that of the mercury-in-glass thermometer (0AE11 SD 01 � C;p=0AE517). Bias was 0AE0150 (SD0AE09) � C. The proportion of outliers >1 � C was 4/251 children (1AE59%). A significant correlation between temperature values obtained with the two procedures was observed (r 2 =0 AE84; p 38AE0 � C, for a non-contact infrared thermometer temperature = 37AE98 � C the sensitivity was 88AE7% and the specificity 89AE9%. Mean distress score (on a 5-point scale) was significantly lower using the non-contact infrared thermometer than using the mercury-in-glass thermometer (1AE92 SD 0AE56 and 2AE40 SD0AE93, respectively; p one month in hospital or ambulatory.

The purpose of this study was to perform a careful neurophysiological examination to identify subclinical signs of botulinum toxin spread distant to the injection site following intragastric injection for obesity treatment. Single-fiber electromyography of extensor digitorum communis and repetitive stimulation of abductor digiti minimi were performed before and 8 days after multiple intragastric injections of botulinum toxin A (Botox, 200 U per patient) or placebo. The study was performed in a randomized double-blind fashion. No patient in either group displayed results indicative of neuromuscular dysfunction either before or after the treatment. No significant change in muscle jitter was observed when comparing baseline with the after-treatment evaluation in either group, and no significant differences between groups were observed. After intragastric botulinum toxin injection no subclinical sign of distant spread was observed.

The application of ecosystem-based management (EBM) in marine environments has been widely supported by scientists, managers, and policy makers, yet implementation of this approach is difficult for various scientific, political, and social reasons. A key, but often overlooked, challenge is how to account for multiple and varied human activities and ecosystem services and incorporate ecosystem-level thinking into EBM planning. We developed methods to systematically identify the natural and human components of a specific ecosystem and to qualitatively evaluate the strength of their interactions. Using the Gulf of Maine marine ecosystem as a case study, we show how these methods may be applied, in order to identify and prioritize the most important components to be included in an EBM plan – particularly the human activities that are the strongest drivers of ecosystem change and the ecosystem services most threatened by cumulative and indirect effects of these activities.

Aim To assess whether children with Noonan syndrome on long-term growth hormone (GH) therapy improve their final height to near mid-parental height. Methods Twenty-five prepubertal children (13 girls) with Noonan syndrome (NS) were studied. A single clinician made the diagnosis based on clinical criteria. GH treatment started at an age ranging from 3.1 to 13.8 y and was continued for at least 2 y. Improvement or "gain" in final height (FH) was defined as either the difference between adult height SD scores (SDS) and pre-treatment height SDS (the childhood component of the Swedish reference) or height SDS compared to the Noonan reference. Results Ten children received a GH dose of 33 microg/kg/d (mean age at start 7.7+/-2.1 y, mean age at stop 17.6+/-1.7 y) and 15 received a dose of 66 microg/kg/d (mean age at start 8.6+/-3.3 y, mean age at stop 18.4+/-2.1 y). Eighteen out of 25 patients reached FH. A substantial improvement in FH of 1.7 SDS, equivalent to 10.4 cm compared to pre-treatment height, was observed. No significant difference was seen between the two GH doses. Females gained a mean height of 9.8 cm and males 1-13 cm (FH 174.5+/-7.8 cm vs mean adult height of 162.5+/-5.4 cm for males with NS) at final height. Moreover, 60% reached a mid-parental height of+/-1 SD. Conclusion GH treatment improves final height in patients with Noonan syndrome, with a mean gain of 1.7 SDS. The prepubertal height gain is maintained to final height and the children achieve a height close to their mid-parental height.

The Fas death receptor is expressed by activated lymphocytes and is involved in switching-off the immune response. Its inherited defects cause auto-immune lymphoproliferative syndrome. Impaired Fas function may also play a role in other auto-immune diseases, such as multiple sclerosis and type 1 diabetes mellitus. The aim of this work was to evaluate Fas function in T cells from patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We evaluated Fas-induced apoptosis in T-cell lines from 27 patients with CIDP, 12 patients with acute inflammatory demyelinating polyneuropathy (AIDP), and 110 controls. CIDP patients displayed lower Fas function than both AIDP patients and controls, whereas no statistically significant difference was found between AIDP patients and controls. Moreover, Fas function was lower in CIDP patients with progressive course than in those with relapsing-remitting course and lower in CIDP patients with axonal damage than in those with pure demyelination. These data suggest that defective Fas function favours CIDP development and aggressive evolution.