Standardizing the assessment of emotional development in adults with intellectual and developmental disability.

OBJECTIVE: The Scale of Emotional Development-Short (SED-S) is an instrument to assess the level of emotional development (ED) in people with intellectual and developmental disability. Index cases are developed as a didactic tool to standardize the application of the scale. METHOD: In a stepwise process, a European working group from six countries developed five index cases, one for each level of ED. All cases were first scored by 20 raters using the SED-S and then rephrased to reduce inter-rater variations (SD > 0.5). RESULTS: All five index cases yielded overall ratings that matched the intended level of ED. Across the range of ED, Regulating Affect needed rephrasing most to ensure a distinct description within each level of ED. CONCLUSIONS: The tri-lingual, cross-cultural evolution of five index cases contributes to a standardized application of the SED-S and can serve as training material to improve the inter-rater reliability of the SED-S across different cultures and languages.

The Relationship Between Spiritual Life and Quality of Life in People with Intellectual Disability and/or Low-Functioning Autism Spectrum Disorders.

Spirituality seems to represent a relevant domain in the person-centred care planning and outcome assessment for persons with intellectual disability and low-functioning autism spectrum disorder. Despite this, the impact of spirituality on subjective well-being and quality of life (QoL) has been scarcely investigated. The aim of the present study was to map the international scientific literature in order to identify the reasons of such misconsideration and the key points for future research and practice implementation. The relationship between spirituality and QoL depends on a complexity of factors, ranging from QoL theoretical models to services' organisation. Personal attitude, family members, health and social-care personnel, training, faith and life communities, and even different religions seem to deserve an in-depth analysis.

Surgery in reference centers improves survival of sarcoma patients: a nationwide study.

BACKGROUND: NETSARC (netsarc.org) is a network of 26 sarcoma reference centers with specialized multidisciplinary tumor boards (MDTB) aiming to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and expert pathological review are mandatory for sarcoma patients nationwide. In the present work, the impact of surgery in a reference center on the survival of sarcoma patients investigated using this national NETSARC registry. PATIENTS AND METHODS: Patients' characteristics and follow-up are prospectively collected and data monitored. Descriptive, uni- and multivariate analysis of prognostic factors were conducted in the entire series (N = 35 784) and in the subgroup of incident patient population (N = 29 497). RESULTS: Among the 35 784 patients, 155 different histological subtypes were reported. 4310 (11.6%) patients were metastatic at diagnosis. Previous cancer, previous radiotherapy, neurofibromatosis type 1 (NF1), and Li-Fraumeni syndrome were reported in 12.5%, 3.6%, 0.7%, and 0.1% of patients respectively. Among the 29 497 incident patients, 25 851 (87.6%) patients had surgical removal of the sarcoma, including 9949 (33.7%) operated in a NETSARC center. Location, grade, age, size, depth, histotypes, gender, NF1, and surgery outside a NETSARC center all correlated to overall survival (OS), local relapse free survival (LRFS), and event-free survival (EFS) in the incident patient population. NF1 history was one of the strongest adverse prognostic factors for LRFS, EFS, and OS. Presentation to an MDTB was associated with an improved LRFS and EFS, but was an adverse prognostic factor for OS if surgery was not carried out in a reference center. In multivariate analysis, surgery in a NETSARC center was positively correlated with LRFS, EFS, and OS [P < 0.001 for all, with a hazard ratio of 0.681 (95% CI 0.618-0.749) for OS]. CONCLUSION: This nationwide registry of sarcoma patients shows that surgical treatment in a reference center reduces the risk of relapse and death.

[Complications with surgical treatment of basal cell carcinomas in individuals over 75 years of age: A retrospective study of prognostic factors in 158 cases]. / Complications du traitement chirurgical des carcinomes basocellulaires après 75 ans. Étude rétrospective des facteurs pronostiques sur 158 cas.

INTRODUCTION: Basal cell carcinoma (BCC) is a pathology that evolves locally and it is common in elderly subjects whose frailty could restrict the indications for the reference treatment, which is based on surgery. The aim of this study was to investigate the morbidity and the mortality associated with surgical treatment of BCC in patients over 75 years of age, so as to identify possible prognostic factors for postoperative complications. MATERIALS AND METHODS: This was a retrospective study carried out with patients over 75 years of age who were treated surgically for one or several BCC between 2010 and 2015 in the reconstructive and cosmetic plastic surgery unit of the Saint-Louis Hospital in Paris (France). We collated the demographic characteristics, the characteristics of the treatment, as well as the rate of major postoperative complications. We performed a univariate and then a multivariate analysis of the various risk factors that were identified. RESULTS: A total of 158 patients were analyzed and they exhibited a rate of major complications of 12%. The statistical analysis identified five significant risk factors: being over 85 years of age (P=0.006), long-term use of anticoagulant treatment (P=0.02), the presence of at least one comorbidity (P=0.018), a conventional hospitalization (P=0.002), and the use of general anesthesia (P=1.2e-10). CONCLUSION: Five risk factors for major postoperative complications with the surgical treatment of BCC in patients over 75 years of age were identified. These factors may provide direction to medico-surgical teams in regard to the optimal treatment of BCC in elderly patients.

[Study of satisfaction concerning the navel after abdominal dermolipectomy with transposition: A report of 96 cases]. / Ombilicoplastie en aile de mouette : technique et satisfaction ; à propos de 96 cas.

GOAL OF THE STUDY: The umbilicus has a major role in the aesthetics of the anterior abdominal wall. Many publications deal with abdominal dermolipectomies but few focus on umbilicoplasty. However, these are essential in assessing the aesthetic result. Umbilicoplasty in "aile de mouette" used in our service is reliable and easily reproducible. In this article, we evaluate the satisfaction of patients with abdominal dermolipectomy with this technique of transposition. MATERIALS AND METHOD: In the plastic surgery department of the Saint-Louis Hospital in Paris, we carried out a retrospective study of patients undergoing abdominal dermolipectomy with transposition of the umbilicus, between 1 January 2012 and 31 December 2012. All patients were operated according to our technique of umbilicoplasty: disinsertion of the umbilicus in V, reinsertion of the umbilic in "aile de mouette", a degreasing periumbilical associated with a plication of the umbilical stem. The complications identified in patients medical records and satisfaction were assessed by a telephone questionnaire. RESULTS: Ninety-six patients were included. No patient presented umbilical necrosis. The overall result of umbilical transposition was considered good to excellent for 92.7% of patients. CONCLUSION: Umbilicoplasty in gull wing has many advantages: it is a simple, easily reproducible, reliable technique, the patients of which are for the most part very satisfied.

Our 35 years' experience on postburn heterotopic ossification: A three-step treatment.

Our retrospective study of burn patients presents a three-step treatment of heterotopic ossification: excision surgery, early rehabilitation, and analgesia. We included patients admitted to the department for treatment of postburn heterotopic ossification between January 1, 1979, and September 30, 2015. The mean age at the time of the burn was 43.3 years. Men accounted for the majority of burn patients who developed an osteoma (70.8%). The mean total skin area burned was 38.4%. No osteoma justifying surgery was found for any patient with a total burned skin area less than 19%. The burned zones were related to the osteoma development in 94.3% of cases. On average, the surgery took place 10.8 months after the burn. The osteotomy was accompanied by surgical treatment of a contracture in 37.1% of patients. Most of the osteomata were found at the elbows (30), followed by the shoulders (3), and finally the knees (2). Rehabilitation began on D0 after the surgery, except if a flap or a thin-skin graft was used. Regarding analgesia, opiates were prescribed systematically during the immediate postoperative period. Elbow range of motion on flexion improved by a mean of 84.1°. During the postoperative period, we found 2 recurrences of osteoma and 1 elbow hematoma in two separate patients. There were no postoperative infections or neurological sequelae. Our retrospective French study confirmed results found in the international literature. The three-step treatment - excision surgery, early rehabilitation, and antalgia - seems to be the best means of treating osteoma with satisfactory results. Surgery is indicated only in the case of functional impairment and not simply based on imaging.

Persistent Skyrmion Lattice of Noninteracting Electrons with Spin-Orbit Coupling.

A persistent spin helix (PSH) is a robust helical spin-density pattern arising in disordered 2D electron gases with Rashba α and Dresselhaus ß spin-orbit (SO) tuned couplings, i.e., α=±ß. Here, we investigate the emergence of a persistent Skyrmion lattice (PSL) resulting from the coherent superposition of PSHs along orthogonal directions-crossed PSHs-in wells with two occupied subbands ν=1, 2. For realistic GaAs wells, we show that the Rashba α_{ν} and Dresselhaus ß_{ν} couplings can be simultaneously tuned to equal strengths but opposite signs, e.g., α_{1}=ß_{1} and α_{2}=-ß_{2}. In this regime, and away from band anticrossings, our noninteracting electron gas sustains a topologically nontrivial Skyrmion-lattice spin-density excitation, which inherits the robustness against spin-independent disorder and interactions from its underlying crossed PSHs. We find that the spin relaxation rate due to the interband SO coupling is comparable to that of the cubic Dresselhaus term as a mechanism of the PSL decay. Near anticrossings, the interband-induced spin mixing leads to unusual spin textures along the energy contours beyond those of the Rahsba-Dresselhaus bands. Our PSL opens up the unique possibility of observing topological phenomena, e.g., topological and Skyrmion Hall effects, in ordinary GaAs wells with noninteracting electrons.

[T plasty for the treatment of skin forehead defects]. / La plastie en T pour la reconstruction des pertes de substances cutanées du front.

INTRODUCTION: The closure of the skin defect of the forehead area with local flap must prevent the distortions of the eyebrow and hairlines and must limit the visibility of the scars. The H plasty is the most common procedure to treat this kind of defect but the scars are extensive and numerous. The H cutaneous flap presents necrosis risks if the skin is thin and the defect important. The H musculocutaneous flap presents sensitive and motors lesions risks. The T cutaneous flap is a good choice because of its excellent cosmetic result and minimized risks. MATERIALS AND METHODS: Twenty-six patients have been treated by the same surgeon between 2006 and 2012 with T cutaneous flap in order to treat forehead and temples' skin defects (the surface goes from 1.5 to 15cm). RESULTS: Excisions were completed in the every 26 cases. We had excellent cosmetic result and no complications such as necrosis, infection, sensitive and motor troubles after the surgery. DISCUSSION: The T plasty is easy for treating skin forehead defects. The scars are 25% less extensive than with a H plasty. The T flap permits to treat important defects without distortion or risks. CONCLUSION: The T cutaneous flap can easily substitute the H plasty for the forehead and temples reconstruction with no risks but good cosmetics results.

[Major chest wall resection for the treatment of invasive breast carcinoma: A series of 33 patients]. / Pariétectomie élargie dans le traitement du cancer du sein invasif: à propos d'une série de 33 cas.

INTRODUCTION: Breast cancer can spread to the chest wall. It is an aggressive stage of poor prognosis. We have developed a technique of major chest wall resection extended beyond the breast area in order to reduce the recurrence. PATIENTS AND METHODS: This is a retrospective single-center series of 33 patients with breast cancer spread to the chest wall without metastasis (13 patients present with primary breast cancer and 20 patients present with recurrent breast cancer) treated by major chest wall resection between January 1993 and January 2013, by the same surgeon. Analysis of the results was made by another surgeon. RESULTS: Patients aged between 27-83years with an average of 55years. The removed parts measured 350cm(2) to 1200cm(2), and the average duration of complete healing was 7.9months. The mean follow-up time was 122months (6months-240months). The 1 year survival was 84.4%, at 2years 72.6%, at 3years 69.5% and at 5 years of 66.4%. CONCLUSION: The technique of major chest wall resection is simple and reproducible. Breast cancer with chest wall extension has a poor prognosis. At the end of the study, 20 of 33 patients who benefit from this technique are still alive.

A new classification system and taxonomic synopsis for Malpighiaceae (Malpighiales, Rosids) based on molecular phylogenetics, morphology, palynology, and chemistry.

Malpighiaceae has undergone unprecedented changes in its traditional classification in the past two decades due to several phylogenetic studies shedding light on the non-monophyly of all subfamilies and most tribes and genera. Even though morphological characters were used to reconstruct the last molecular generic phylogeny of Malpighiaceae, a new classification system has never been proposed for this family. Based on a comprehensive review of the last twenty years of published studies for this family, we propose a new classification system and provide a taxonomic synopsis for Malpighiaceae based on molecular phylogenetics, morphology, palynology, and chemistry as a baseline for the systematics, conservation, and taxonomy of this family worldwide. Malpighiaceae currently comprises two subfamilies (Byrsonimoideae and Malpighioideae), 12 tribes ( Acmanthereae, Acridocarpeaetrib. nov., Barnebyeaetrib. nov., Bunchosieaetrib. nov., Byrsonimeae, Galphimieae, Gaudichaudieae, Hiptageae, Hiraeeae, Malpighieae, Mcvaughieaetrib. nov., and Ptilochaeteaetrib. nov.), 72 genera (incl. Mamedeagen. nov.), and 1,499 accepted species (715 of which are currently under some kind of extinction threat). We present identification keys for all subfamilies, tribes, and genera, a full morphological description for the proposed new genus, the re-circumscription of ten genera alongside the needed new combinations, the proposition of several new synonyms, the typification of several names, and notes on the taxonomy, distribution, conservation, and ecology up to the genus rank. Morphological plates are also provided to illustrate the immense diversity of morphological traits used in the new classification and synopsis.

Field and laboratory methods in human milk research.

Human milk is a complex and variable fluid of increasing interest to human biologists who study nutrition and health. The collection and analysis of human milk poses many practical and ethical challenges to field workers, who must balance both appropriate methodology with the needs of participating mothers and infants and logistical challenges to collection and analysis. In this review, we address various collection methods, volume measurements, and ethical considerations and make recommendations for field researchers. We also review frequently used methods for the analysis of fat, protein, sugars/lactose, and specific biomarkers in human milk. Finally, we address new technologies in human milk research, the MIRIS Human Milk Analyzer and dried milk spots, which will improve the ability of human biologists and anthropologists to study human milk in field settings.

Isometric myography is a feasible method to identify and quantify endothelial dysfunction in dogs with myxomatous mitral valve disease.

OBJECTIVE: To assess the feasibility of isometric myography in pet dogs with myxomatous mitral valve disease (MMVD) to determine its use in quantifying endothelial dysfunction. ANIMALS: 9 dogs euthanized for medical reasons. METHODS: Femoral, renal, and mesenteric arteries were collected postmortem and stored in physiological saline solution at 4 °C for myography. Mitral valves were scored for myxomatous degeneration (grades 1 to 4). Sections of arteries were mounted in wells, immersed in physiological saline solution perfused with 95% O2 and 5% CO2 at 37 °C, and stretched to an internal circumference (IC) that generated the maximal difference between active and passive wall tension (IC1). Normalization factors were calculated by dividing the IC1 by the IC at which the passive wall tension was 100 mm Hg (IC100). Vasoconstriction to phenylephrine and vasodilation to acetylcholine (endothelial dependent) and sodium nitroprusside (endothelial independent) were assessed by cumulative dose-response curves. RESULTS: Median MMVD grade was 3. Mean values of normalization factors were 1.00 ± 0.14 (renal, n = 15), 1.00 ± 0.10 (femoral, 8), and 1.05 ± 0.12 (mesenteric, 6). Responses to phenylephrine were similar between dogs (P = .14). Reduced responses to acetylcholine compared with sodium nitroprusside were identified in 15 arteries, suggesting endothelial dysfunction. CLINICAL RELEVANCE: Isometric myography of arteries from pet dogs is feasible and can identify loss of endothelial-dependent relaxation in dogs with MMVD postmortem. Its use in further research can lead to a better understanding of the pathophysiology mechanisms of this disease.

Updates in the care of youths with intellectual disability and autism spectrum disorder.

PURPOSE OF REVIEW: Since the turn of the new millennium, care for people with intellectual disability/intellectual developmental disorders (IDD) and/or autism spectrum disorder (ASD) has drawn increasing attention because of the continuous prevalence increase of these two conditions, the high health vulnerability, especially mental vulnerability, and the crucial role of care provision in the setting of continued deinstitutionalization and the shift to community care. The present review addresses the main issues related to mental healthcare in youth with IDD and/or ASD with a specific focus on adolescence and transition to adulthood. RECENT FINDINGS: Despite the existence of specific recommendations, persons with IDD/ASD lack access to quality mental healthcare within communities, receive inadequate diagnosis and intervention, including medication overuse as well as ineffective residential, educational, employment, financial, and social support services. The lack of a unified care system that provides services across all domains and life ages may be the cause of users' perceptions and experiences of inefficiencies. SUMMARY: The scientific community seems to converge on that care should be provided in accordance with principles of interdisciplinarity, life-span view, precision, personalization, and participation. Quality of life appears to be an ideal conceptual reference for care planning and outcome evaluation.

Appropriateness of psychopharmacological therapies to psychiatric diagnoses in persons with autism spectrum disorder with or without intellectual disabilities: a cross-sectional analytic study.

BACKGROUND: Observational studies highlighted high rates of psychotropic medication in persons with autistic spectrum disorder (ASD) with or without intellectual disability, which seems to be associated with the management of problem behaviors more than co-occurrent psychiatric disorders. The purpose of the study is to investigate psychopharmacology use and diagnoses of co-occurrent psychiatric disorder (PD) in persons with ASD attending a public mental health service in Emilia Romagna, Italy. METHODS: The present study is a multicenter, cross-sectional study. RESULTS: 275 persons out of 486 (56.5%) resulted to receive at least one psychotropic drug, compared to 74 persons (15.2%) that were diagnosed with a PD. 63.6% were on poly-pharmacotherapy (2-10 compounds), with 37.8% receiving 3 or more medications. Antipsychotics were the most frequently prescribed class of psychotropic drugs (89%), followed by antiepileptics/mood stabilizers/lithium (42.1%) and anxiolytics (BDZ) (38.5%). Most common psychiatric disorders were psychotic disorders (29.7%), followed by anxiety disorders (17.5%), bipolar disorders (12.2%), and depressive disorders (9.4%). CONCLUSIONS: Our findings support earlier research showing that many individuals with ASD receive pharmacotherapy without being diagnosed with a co-occurring psychiatric disorder, indicating that the main reasons for prescription and the type of compound frequently have little to no link with specific psychopathology.

Radiation from a D-dimensional collision of shock waves: a remarkably simple fit formula.

Recently we estimated the energy radiated in the head-on collision of two equal D-dimensional Aichelburg-Sexl shock waves, for even D, by solving perturbatively, to first order, the Einstein equations in the future of the collision. Here, we report on the solution for the odd D case. After finding the wave forms, we extract the estimated radiated energy for D=5, 7, 9, and 11 and unveil a remarkably simple pattern, given the complexity of the framework: (for all D) the estimated fraction of radiated energy matches the analytic expression 1/2-1/D, within the numerical error (less than 0.1%). Both this fit and the apparent horizon bound converge to 1/2 as D→∞.

Charon's size and an upper limit on its atmosphere from a stellar occultation.

Pluto and its satellite, Charon (discovered in 1978; ref. 1), appear to form a double planet, rather than a hierarchical planet/satellite couple. Charon is about half Pluto's size and about one-eighth its mass. The precise radii of Pluto and Charon have remained uncertain, leading to large uncertainties on their densities. Although stellar occultations by Charon are in principle a powerful way of measuring its size, they are rare, as the satellite subtends less than 0.3 microradians (0.06 arcsec) on the sky. One occultation (in 1980) yielded a lower limit of 600 km for the satellite's radius, which was later refined to 601.5 km (ref. 4). Here we report observations from a multi-station stellar occultation by Charon, which we use to derive a radius, R(C) = 603.6 +/- 1.4 km (1sigma), and a density of rho = 1.71 +/- 0.08 g cm(-3). This occultation also provides upper limits of 110 and 15 (3sigma) nanobar for an atmosphere around Charon, assuming respectively a pure nitrogen or pure methane atmosphere.

Heteropterys rosmarinifolia, a new species of Malpighiaceae with verticillate leaves from savannas grasslands of central Brazil.

A new species of Heteropterys belonging to the Parabanisteria informal group is described for the savannas grasslands of the Serra do Tombador Natural Reserve, municipality of Cavalcante, State of Goiás, Brazil. Heteropterys rosmarinifolia is easily distinguished from the remaining Brazilian species of the Parabanisteria informal group due to its stems unbranched, leaves associated with the inflorescence verticillate, blades linear to very narrowly oblong, strongly conduplicate, ascending to upright, and sparsely sericeous to glabrous at age. We present a complete morphological description for the new species, photographic plates, comments on its distribution, ecology, and taxonomy, besides an identification key to the species of Heteropterys from the Parabanisteria group from the State of Goiás, Brazil.

Stereotypies in the Autism Spectrum Disorder: Can We Rely on an Ethological Model?

BACKGROUND: Stereotypic behaviour can be defined as a clear behavioural pattern where a specific function or target cannot be identified, although it delays on time. Nonetheless, repetitive and stereotypical behaviours play a key role in both animal and human behaviour. Similar behaviours are observed across species, in typical human developmental phases, and in some neuropsychiatric conditions, such as Autism Spectrum Disorder (ASD) and Intellectual Disability. This evidence led to the spread of animal models of repetitive behaviours to better understand the neurobiological mechanisms underlying these dysfunctional behaviours and to gain better insight into their role and origin within ASD and other disorders. This, in turn, could lead to new treatments of those disorders in humans. METHOD: This paper maps the literature on repetitive behaviours in animal models of ASD, in order to improve understanding of stereotypies in persons with ASD in terms of characterization, pathophysiology, genomic and anatomical factors. RESULTS: Literature mapping confirmed that phylogenic approach and animal models may help to improve understanding and differentiation of stereotypies in ASD. Some repetitive behaviours appear to be interconnected and mediated by common genomic and anatomical factors across species, mainly by alterations of basal ganglia circuitry. A new distinction between stereotypies and autotypies should be considered. CONCLUSIONS: Phylogenic approach and studies on animal models may support clinical issues related to stereotypies in persons with ASD and provide new insights in classification, pathogenesis, and management.

Perspectives of the Biomedical Engineering Program at UASLP after ten years - analysis and criticism.

The Biomedical Engineering (BME) bachelor pro-gram of the Faculty of Sciences in Universidad Autónoma de San Luis Potosí (UASLP) was created in June of 2010, with the aim of training professionals with an integral perspective in the engineering field by considering a multidisciplinary approach to develop and apply technology in the areas of medicine and biology. After 10 years, our BME program has achieved national recognition. Despite of being an emerging program, this achievement has been obtained by the consolidation of our academic staff, the outstanding participation of our students in national and international academic events, and the historical graduation results. In our comprehensive evaluation, we report an overall terminal efficiency (completion rate) of 67% and a graduation rate of 47.2%, where these values are above the average for an engineering program in our institution. Additionally, the BME program provides students with solid skills and background to carry out research activities, which has resulted in a considerable number of alumni pursuing graduate studies or have already completed one. Our results show that 90% of our former students are working after graduation, but only 44% work in the field of biomedical engineering, since the regional labor market starts to saturate given the fact that, at present, students from six generations have completed our BME bachelor program. In this way, few graduates visualize the wide spectrum of job options where a biomedical engineer can impact, by their distinctive comprehensive and multidisciplinary training. Therefore, it is necessary to propose new curricular design strategies to provide our students with an academic training that allows them to enter a globalized world, where there is an even greater spectrum of engineering possibilities related to the fields of medicine and biology, in line with current trends.