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Non-alcoholic fatty liver disease (NAFLD) is the most frequent liver disease worldwide and can progress to non-alcoholic steatohepatitis (NASH), which is characterized by triglyceride accumulation, inflammation, and fibrosis. No pharmacological agents are currently approved to treat these conditions, but it is clear now that modulation of lipid synthesis and autophagy are key biological mechanisms that could help reduce or prevent these liver diseases. The folliculin (FLCN) protein has been recently identified as a central regulatory node governing whole body energy homeostasis, and we hypothesized that FLCN regulates highly metabolic tissues like the liver. We thus generated a liver specific Flcn knockout mouse model to study its role in liver disease progression. Using the methionine- and choline-deficient diet to mimic liver fibrosis, we demonstrate that loss of Flcn reduced triglyceride accumulation, fibrosis, and inflammation in mice. In this aggressive liver disease setting, loss of Flcn led to activation of transcription factors TFEB and TFE3 to promote autophagy, promoting the degradation of intracellular lipid stores, ultimately resulting in reduced hepatocellular damage and inflammation. Hence, the activity of FLCN could be a promising target for small molecule drugs to treat liver fibrosis by specifically activating autophagy. Collectively, these results show an unexpected role for Flcn in fatty liver disease progression and highlight new potential treatment strategies.

Synopsis The biological sciences are inherently interdisciplinary and important advances in biology cannot be made without collaboration. Despite the increasing emphasis on interdisciplinarity in higher education, science courses only rarely extend to content outside of the STEM discipline. Classes are typically taught by one faculty member in one discipline. To demonstrate relevance while addressing genuine community needs, faculty can use service-learning in their courses. Service-learning is an experiential learning strategy where students learn course content and additional relevant skills through completing service with a community partner. Community needs are frequently beyond the scope of a single course or discipline. In order to better meet community needs, an interdisciplinary collaboration provides a more comprehensive experience that highlights the application and interconnection of course content. This article presents a generalisable model for successful interdisciplinary collaborations. While the nature of course scheduling, academic department structure, and faculty workload can be barriers to collaboration between faculty, they are not insurmountable and accomplishable within this framework. The benefits to the students and the community far outweigh navigating these challenges. Using an interdisciplinary approach in teaching will not only enrich course content and expand student learning in multiple areas, but also increase collaboration within the academy while better meeting community needs.

Neuroblastoma is the most common extra-cranial solid tumor in children, representing approximately 8% of all malignant childhood tumors and 15% of pediatric cancer-related deaths. Recent sequencing and transcriptomics studies have demonstrated the RAS-MAPK pathway’s contribution to the development and progression of neuroblastoma. This review compiles up-to-date evidence of this pathway’s involvement in neuroblastoma. We discuss the RAS-MAPK pathway’s general functioning, the clinical implications of its deregulation in neuroblastoma, and current promising therapeutics targeting proteins involved in signaling. Supplementary Information The online version contains supplementary material available at 10.1186/s13046-021-01967-x.

Purpose: Adolescents and young adults (AYA) with cancer present a unique challenge to health care institutions. Their cancer diagnosis and treatment have a profound impact upon their health and well-being. Despite the various support services aimed at improving their quality of life, their needs and preferences are often underestimated or misjudged. Recent studies show that patients are empowered by the knowledge and support they receive online. Given the extensive use of social media among AYA, we aim to identify promises, challenges, and recommendations for integrating these platforms in AYA cancer care. Methodology: We systematically searched seven databases systematically: Scopus, PubMed, PsycInfo, Web of Science, CINAHL, SocINDEX, and Media. We placed no restriction on the type of methodology used in the studies. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses was used to frame the research. Results: Many studies argued that health care professionals need to integrate social media in their clinical practice to engage with patients' lifeworld. Social media were considered important allies in optimizing cancer care at all levels of support, ranging from information provision, treatment adherence, diet and exercise interventions, to professional, peer, and psychosocial self-care. Lack of research on the efficacy of social media in the context of psychosocial support was a commonly cited problem. A small number of publications paid attention to the inherent risks of promoting self-care online. Conclusion: Future studies should continue to pursue empirical research on the efficacy of online psychosocial care, while not neglecting the ethical challenges of social media research.

ZusammenfassungUntersuchungen zur olfaktorischen Wahrnehmung bei psychiatrischen und neurodegenerativen Erkrankungen zeigen vielfältige Veränderungen auf psychophysikalischer, neurophysiologischer und anatomischer Ebene. Diese Veränderungen stellen sich zum Teil erkrankungsspezifisch dar. So wird z. B. die dysfunktionale Geruchswahrnehmung bei affektiven Erkrankungen mit anatomisch-funktionellen Überlappungen von geruchs- und emotionsverarbeitenden Arealen in Verbindung gebracht. Auf Basis der aktuellen Datenlange zur veränderten Riechfähigkeit bei Major Depression, Schizophrenie, Alzheimer-Demenz und der Parkinson-Erkrankung wird hier die Erfassung der olfaktorischen Wahrnehmung als zusätzlicher diagnostischer Marker bei neuropsychiatrischen Erkrankungen diskutiert. Dabei besteht die Notwendigkeit einer objektiven Erfassung der Riechfunktion über validierte Testverfahren, verknüpft mit Kenntnissen in der Interpretation der erhaltenen Befunde. Ziel dieser Übersichtsarbeit ist es, die Veränderungen der olfaktorischen Wahrnehmung bei den genannten Erkrankungen darzustellen und ausgewählte, im deutschen Sprachraum verfügbare Verfahren vorzustellen und anwendungsbezogen einzuordnen. Eigene aktuelle Daten zur Validierung eines Geruchsdiskriminationstests (Düsseldorf Odour Discrimination Test) an einer klinischen Stichprobe werden vorgestellt.

STUDY QUESTION What are the characteristics of patients with conceptions transplanted in childhood and adolescence? SUMMARY ANSWER Insemination and conception after hematopoietic stem cell transplantation (HCT) in childhood or adolescence was possible, even after myeloablative conditioning regimes, although some patients required reproductive medicine support. WHAT IS KNOWN ALREADY Preparative regimens of HCT are highly gonadotoxic, which leads to gonadal failure and pubertal development disorders. There are few population-based studies assessing the risk of future infertility in children after HCT. STUDY DESIGN, SIZE, DURATION We conducted a retrospective study to investigate natural or assisted conceptions and their outcomes in patients PARTICIPANTS/MATERIALS, SETTING, METHODS Detailed information concerning pregnancy occurrences and outcomes were obtained by a separate questionnaire. Quantitative variables were presented as medians with their interquartile range (IQR) or range, and categorical variables were presented as frequencies and percentages. MAIN RESULTS AND THE ROLE OF CHANCE In total, 62 988 pediatric patients received a first HCT in EBMT centers between 1995 and 2016. Pregnancy was reported in 406 patients in the database. The median age at transplantation was 15.7 (range: 0.7–18) years, and the median age at declared conception was 25.0 (range: 16.3–38.8) years. Details concerning the first pregnancy and pregnancy outcome were obtained from 99 patients (24%) from the returned questionnaires. The median age at delivery or pregnancy interruption of the females was 23.0 (IQR: 20.8–27) years, with a median time after transplant of 10.7 (IQR: 6.6–15.4) years. Compared with the mean age of healthy women at their first child’s birth (29 years old), the transplanted women delivered 5 years earlier (mean: 24.3 years). In terms of conception modality, 13/25 (52%) females conditioned with total body irradiation (TBI) and 50/52 (96%) of those conditioned without TBI conceived naturally. All seven male patients who had been conditioned with TBI achieved fatherhood but required assisted fertilization or used their cryopreserved sperm. In the females, 63/70 (90%) of all conceptions resulted in a live birth, 49/63 (84.5%) were at term and 43/46 (93%) had normal birthweight. Cesarean delivery was performed in 9/61 (15%) especially in women who had received a myeloablative regimen. LIMITATIONS, REASONS FOR CAUTION In the EBMT pediatric dataset, the age at last follow-up or death was WIDER IMPLICATIONS OF THE FINDINGS Reproductive health surveillance and fertility preservation counseling are important in younger transplanted patients. Our results showed that there is a window of opportunity to conceive naturally or with reproductive medicine support. STUDY FUNDING/COMPETING INTEREST(S) Funding was provided by the ‘Stiftung für krebskranke Kinder Regio Basiliensis’, Basel, Switzerland. All authors have no conflicts of interest to declare. TRIAL REGISTRATION NUMBER N/A.

Background Optoelectronic Plethysmography (OEP) is an effective method for evaluating thoracoabdominal kinematics. However, little is known about the viability and use the OEP in people with obesity. Objective Summarize information on the use and feasibility of OEP in the assessment of thoracoabdominal kinematics in people with obesity. Methods Studies were identified through PubMed, EMBASE, Science Direct, Web of Science and Scopus databases in the February 2021. Results Six studies met the inclusion criteria. The studies used the OEP to assess abdominal and thoracic mobility in obese people of different BMI, using the default setting. No study reported the impossibility of using OEP in people with obesity. Conclusion Has been shown that it is possible to use the OEP in people with obesity with a BMI of up to 50 kg/m2. More studies are needed to demonstrate the feasibility of the method in people with a BMI greater than this value.

Kurzfassung Viele Unternehmen erhoffen sich enorme Wachstumschancen durch Digitalisierung, Vernetzung und neue Technologien. Daher ist der Reifegrad der Digitalisierung eines Unternehmens von besonderer Bedeutung, um den Status des Unternehmens zu überprüfen. Im Rahmen der acatech-Studie wurde ein Index entwickelt, um den Reifegrad von produzierenden Unternehmen zu bestimmen. Diese Bewertung berücksichtigt jedoch den Bereich des Lieferkettenmanagements nicht ausreichend. Infolgedessen müssen diesbezüglich weitere Merkmale entwickelt werden, um den Reifegrad der Digitalisierung innerhalb einer Lieferkette zu bestimmen. Der Schwerpunkt liegt auf Datenverarbeitung, Kommunikation, Schnittstellenarbeit und Sicherheit, Netzwerkzusammenarbeit und Änderungsmanagement.

The role of catheter ablation as an adjunct and alternative to ICD implantation is not known in patients at risk for recurrent ventricular tachycardia (VT) and sudden cardiac death (SCD) across Asia. Patients with nonischemic etiologies of cardiomyopathy, which are highly prevalent in Asia, have not been previously enrolled in randomized trials of VT ablation. To evaluate whether preemptive catheter ablation in patients with monomorphic VT and an indication for ICD implantation results in improved clinical outcomes compared to ICD implantation with standard medical therapy alone. To examine the natural history of ablation outcomes in the absence of background ICD therapy in patients that refuse randomization. The PAUSE-SCD study (NCT02848781) is a prospective, multi-center, randomized controlled trial enrolling patients with structural heart disease (EF

BackgroundChildhood cancer and its treatment may lead to many acute and chronic health complications. Related impairment in quality of life, excess in deaths, and accumulated health care costs are relevant. There is a wide inter-individual variability in the type and severity of health complications. Genetic variations are suggested to contribute to individual susceptibility. So far, only few genetic variants have been used to risk-stratify treatment and follow-up care. This study platform aims to identify germline genetic variants associated with acute and late complications of childhood cancer.MethodsThe Genetic Risks for Childhood Cancer Complications Switzerland (GECCOS) study is a nationwide cohort study. It includes patients and survivors who were diagnosed with childhood cancers or Langerhans cell histiocytosis before age 21 years, were registered in the Swiss Childhood Cancer Registry (SCCR) since 1976 and have consented to the Pediatric Biobank for Research in Hematology and Oncology (BaHOP), Geneva, host of the Germline DNA Biobank Switzerland for Childhood Cancer and Blood Disorders (BISKIDS). BISKIDS is a national biobank for the collection of germline DNA in childhood cancer patients and survivors.GECCOS uses demographic and clinical data from the SCCR and the associated Swiss Childhood Cancer Survivor Study (SCCSS), which contains health-related data of survivors. Phenotypic data consist of objective measurements, health conditions diagnosed by physicians, second primary neoplasms, self-reported and health-related information from participants. Germline genetic samples and sequencing data have been collected in BISKIDS. We will perform gene panel sequencing, whole-exome sequencing, or whole-genome sequencing depending on the research questions. We will perform association analyses to identify genetic variants associated with specified health conditions. We will use clustering and machine-learning techniques and assess multiple health conditions in different models.DiscussionGECCOS will serve as an overarching platform to enable genotype-phenotype association analyses on complications associated with childhood cancer and its treatments. Knowledge of germline genetic variants associated with childhood cancer-associated health conditions will help to further individualize cancer treatment and follow-up care, potentially resulting in improved efficacy and reduced side effects, for personalized cancer care.Trial registrationClinicaltrials.gov: NCT04702321

Aim: Sperm cryopreservation (SCP) should be offered to every adolescent before gonadotoxic treatment, but experience in this age range is still relatively limited. The goal of this study is to assess how to optimize this procedure.Methods and Patients: One hundred thirty-three patients between 12 and 20 years old, who underwent SCP between 1980 and 2017, were included. Baseline data (age, indication for SCP, and semen parameters at freezing) and follow-up data (outcome of sperm straws and follow-up of sperm quality) were collected and analyzed.Results: SCP is feasible from the age of 12. Semen assessment parameters at this age were close to parameters of adults. However, we observed quantitative impairments in testicular tumors and qualitative impairments in leukemia and bone marrow failure. Four patients (3%) used their cryopreserved semen for medically assisted reproduction, 15 patients died (11.3%), 18 asked for destruction of their straws (13.5%), and nine samples were destroyed because of lack of news (6.8%). Very few patients underwent a sperm analysis after treatment.Conclusions: SCP is an efficient, still underused, procedure for adolescents and young adults. Cryopreserved sperm is rarely used and rarely destroyed, but studies with a longer follow-up are needed to better assess these observations. Follow-up with a specialist of reproductive medicine is valuable for better information of the patient.

Acute Graft versus Host Disease (aGvHD) grades 2–4 occurs in 15–60% of pediatric patients undergoing allogeneic haematopoietic stem-cell transplantation (allo-HSCT). The collateral damage to normal tissue by conditioning regimens administered prior to allo-HSCT serve as an initial trigger for aGvHD. DNA-repair mechanisms may play an important role in mitigating this initial damage, and so the variants in corresponding DNA-repair protein-coding genes via affecting their quantity and/or function. We explored 51 variants within 17 DNA-repair genes for their association with aGvHD grades 2–4 in 60 pediatric patients. The cumulative incidence of aGvHD 2–4 was 12% (n = 7) in the exploratory cohort. MGMT rs10764881 (G>A) and EXO rs9350 (c.2270C>T) variants were associated with aGvHD 2–4 [Odds ratios = 14.8 (0 events out of 40 in rs10764881 GG group) and 11.5 (95% CI: 2.3–191.8), respectively, multiple testing corrected p ≤ 0.001]. Upon evaluation in an extended cohort (n = 182) with an incidence of aGvHD 2–4 of 22% (n = 40), only MGMT rs10764881 (G>A) remained significant (adjusted HR = 2.05 [95% CI: 1.06–3.94]; p = 0.03) in the presence of other clinical risk factors. Higher MGMT expression was seen in GG carriers for rs10764881 and was associated with higher IC50 of Busulfan in lymphoblastoid cells. MGMT rs10764881 carrier status could predict aGvHD occurrence in pediatric patients undergoing allo-HSCT.

Ovarian failure is a major long-term adverse event following gonadotoxic treatment of malignant diseases. Ovarian tissue cryopreservation can be offered in some conditions to preserve fertility. We report the case of a 13-year-old female with a diagnosis of acute myeloid leukemia, who presented with hypergonadotropic hypogonadism after unilateral ovariectomy for fertility preservation and before highly gonadotoxic treatment. Even though damage seemed only partial, this case suggests that the remaining contralateral ovarian function may be compromised after ovarian tissue cryopreservation, leading per se to a hypergonadotropic hypogonadism. Although indication of ovarian cryopreservation is not called into question in situations of highly gonadotoxic therapy, this procedure should only be performed after evaluation by a specialized multidisciplinary team and provided a solid indication.

Non-alcoholic steatohepatitis (NASH) represents a major economic burden and is characterized by triglyceride accumulation, inflammation, and fibrosis. No pharmacological agents are currently approved to treat this condition. Emerging data suggests an important role of autophagy in this condition, which serves to degrade intracellular lipid stores, reduce hepatocellular damage, and dampen inflammation. Autophagy is primarily regulated by the transcription factors TFEB and TFE3, which are negatively regulated by mTORC1. Given that FLCN is an mTORC1 activator via its GAP activity towards RagC/D, we generated a liver specific Flcn knockout mouse model to study its role in NASH progression. We demonstrate that loss of FLCN results in reduced triglyceride accumulation, fibrosis, and inflammation in mice exposed to a NASH-inducing diet. Hence, the GAP activity of FLCN could a promising target for small molecule drugs to treat NASH progression by specifically activating autophagy and lysosomal biogenesis while leaving mRNA translation machinery unperturbed. Collectively, these results show an unexpected role for FLCN in NASH progression and highlight new possibilities for treatment strategies through its role in hepatocyte homeostasis.

With conventional production, storage and delivery strategies, it is often not possible to meet customersn’ expectations of continuous availability of individualized components in the context of spare parts logistics. Due to the possibility of producing spare parts according to current demand and close to the place of actual demand, additive manufacturing (AM) has the potential to solve this challenge. Cost-intensive toolmaking, long transport distances and high warehouse stock can thus be prevented. In order to make optimum use of the above-mentioned advantages of AM, new general conditions for spare parts logistics must be created. One possibility is to provide IT support through the development of a software tool. The aim of the software tool is to ensure easy access to AM for all participants of the supply chain. As part of the research project 3Dsupply (Project ID: 02K16C162), a software solution is developed, that consists of two different modules: AM feasibility assessment and AM producer selection. During the AM feasibility assessment, the economic and technological benefit of AM based on the spare part data is estimated. The subsequent selection of appropriate 3D print service providers is conducted based on a matching of customer requirements and producer characteristics. Possible users of the software tool are not only the spare parts demanders and the producers of additive parts itself, but also the logistics service provider, which, in particular in the scenario of a full supply service provider, can act as an intermediary between spare parts demanders and producers.

Introduction Pediatric neuroendocrine tumors (NETs) of the appendix are mostly detected incidentally after appendectomy for acute appendicitis. NET management is a matter of controversy. In this national, multicenter study, we aimed to establish guidelines based on our results and the literature. Materials and Methods Medical records of children (0–16 years) with NET of the appendix, treated in Switzerland (1991–2012), were reviewed. Results Forty cases (28 girls) were analyzed. Median age at diagnosis was 12.7 years (interquartile range [IQR]: 4.0). Tumor size was 0.1–24 mm (median: 0.6, IQR: 0.6). Four patients (10%) underwent additional surgery because of either tumor size > 15 mm (1/4), extension to the mesoappendix (1/4), or incomplete resection (2/4). Three patients with a tumor of ≥ 20 mm had no additional surgery. No patient had lymph node metastases. All patients were in complete remission at the last follow-up (median: 3.0 years, IQR: 10.9). Conclusion We conclude from this study and from an extensive review of the literature that two criteria may point to the need for additional surgery, i.e., the possibility of regional lymph node involvement: tumor size > 20 mm and incomplete surgical resection margins. Childhood NET of the appendix has an excellent prognosis.

Due to the increasing spread of Information and Communication Technologies (ICT) suitable for shop floors, the production environment can more easily be digitally connected to the various decision making levels of a production system. This connectivity as well as an increasing availability of high-resolution feedback data, can be used for decision support for all levels of the company and supply chain. To enable data driven decision support, different data sources were structured and linked. The data was combined in task-specific digital shadows, selecting clustering and aggregation rules to gain information. Visual interfaces for task-specific decision support systems (DSS) were developed and evaluated positively by domain experts. The complexity of decision making on different levels was successfully reduced as an effect of the processed amounts of data. These interfaces support decision making, but can additionally be improved if DSS are extended with smart agents as proposed in the Internet of Production.

The Dutch paint manufacturer Talens & Co (now Royal Talens) started the production of the brand Rembrandt Olieverven in 1904 in Apeldoorn and much of the company’s historical archive still remains.

This chapter covers the use of phase change materials in textile coatings and laminates. The chapter reviews the thermal effects obtained by phase change materials and methods to measure these thermal effects. Based on these thermal effects, specific applications of textile coatings and laminates treated with phase change materials in protective garments, active wear, and architectural roofing systems are discussed.

Current supply chain structures in the spare parts logistics are changing profoundly due to the influence of digitalization and additive manufacturing (AM). In particular the Logistics Service Provider (LSP) is influenced by the change, as the physical transport of goods could become redundant due to the digital transmission of production data. This leads to a reduction of the LSP’s share in the value chain. Conceptualizing a new role for the LSP for additively manufactured spare parts is necessary. Therefore, five different scenarios are identified in which the LSP serves as a transport carrier, digital distributor, an AM decision maker, a selector of the manufacturer and as an AM service provider.

In the past few decades, researchers have been examining natural microbial communities with a variety of culture-independent methods. These methods have also been applied to the study of microbial communities impacted by anthropogenic activity. Microbial communities are of interest because of their innate metabolic activities that can be exploited for a variety of beneficial purposes, such as the remediation of harmful pollutants. This manuscript reviews three widely used culture-independent methods as applied to the field of bioremediation (sequencing of rDNA clone libraries, terminal restriction fragment length polymorphism, and microarrays). These techniques are economically feasible and appropriate for undergraduate researchers. Each technique is briefly explained before case studies of their successful applications in bioremediation are reviewed. Drawbacks of each method are also presented. The advancement of microbial community analysis techniques in the future is discussed.

Optimal and efficient machining of high-temperature alloys is one of the main goals set by the manufacturing technology today, taking into account the unique properties of these materials, the required performance and the process parameters. One of the most important parameters investigated and presented in this paper is the optimal cooling and lubrication strategy. The application of various hybrid cooling strategies such as high-pressure cooling, cryogenic cooling and aerosol dry lubrication offers the potential to extend profitability and energy efficiency as well as high performance and improved surface integrity. The paper introduces fundamental functions, cooling systems and relationships that characterize the efficient utilization on standard machine tools. Based on fundamental investigations conducted using high-temperature alloys with poor machinability such as Titanium alloy Ti6Al4 V and Inconel 718, relevant influencing parameters are derived and optimization options are presented for the cooling strategies.

Background and objectives. Financial aid for the blind which is awarded based only on medical certificates and results of examinations has in the past resulted in too many false diagnoses;therefore, Bavaria seeks to pay financial aid to the blind only on the basis of a specific ophthalmological assessment according to the standards of the German pension medical ordinance (VersMedV, Versorgungsmedizinische Verordnung). Because these ophthalmological assessments initially contribute to a higher financial burden on the state, longer processing times and inconvenience to the patient, investigations should be undertaken to determine if ophthalmological findings, reports and medical certificates can be a suitable basis for an expert assessment and in how many cases blindness which had been certified by the original examination could be confirmed by a specific ophthalmological assessment. Methods. A total of 925 applications for financial assistance to the blind within the catchment area of the Bavarian Center for Family and Social Services (ZBFS, Zentrum Bayern Familie und Soziales) regional center in Upper Bavaria between 2003 and 2008, all of which had been subjected to an assessment by the same practitioner acting as external expert, were statistically analyzed. Results. Of the 357 applicants who had been classified as blind according to the medical certificate and findings, 283 (79 %) were confirmed as being blind after the assessment and in 73 (21 %) blindness could not be confirmed. Of the 262 applicants who were classified as not being blind during the first examination, the diagnosis was confirmed in 192 (73 %) while 70 cases (27 %) were classified as blind. Of the 304 applicants for whom an assessment was not possible by the medical certification, 165 were ultimately classified as blind and 139 as not blind. Out of 32 applicants who were explicitly classified as being blind in the medical certificate, 13 were confirmed as being blind while the remaining 18 were classified in the subsequent assessment as not blind. Conclusion. Apart from unambiguous cases, the awarding of financial aid to the blind should only be based on an ophthalmological assessment which follows the VersMedV guidelines. Only this approach can result in an equal treatment of all applicants before the law and the awarding of financial aid to the blind to assist those truly in need. In addition, fiscal budget results revealed that the falsely awarded financial aid to the blind reached well beyond millions of Euros.

This paper introduces a new textile-based hybrid PV/T system utilizing a spacer fabric treated with phase change material (PCM). The PCM absorbs excess heat from the PV modules, which keeps the modules in their optimal temperature range so that their power output is enhanced. The thermal energy stored in the PCM is then used for hot water generation. Cold water, which is pumped through thin capillaries integrated into the spacer fabric, absorbs the heat stored in the PCM and is heated up. The warm water can be used immediately or stored in an intermediate storage tank. Comparing the proposed textile-based hybrid PV/T system to conventional hybrid PV/T systems, the proposed system is less expensive and provides a higher overall efficiency. The textile-based collector is lightweight, thin and flexible.

BACKGROUND Smoking harms health, particularly that of childhood cancer survivors, who face risk of pulmonary and cardiovascular diseases because of chemotherapy and radiotherapy to the chest. This nationwide study assessed smoking habits and reasons for smoking in adolescent survivors and healthy peers. METHODS As part of the Swiss Childhood Cancer Survivor Study, we sent a questionnaire to all Swiss resident survivors, who were aged 16-19 years. We compared smoking status and reasons for smoking between 511 survivors, 141 of their siblings, and 1,727 adolescents in a representative population-based study, the Tobacco Monitoring Switzerland (TMS). RESULTS Current smoking was less prevalent in survivors (17%) and their siblings (17%) compared with TMS (32%). Survivors and TMS adolescents gave similar reasons for smoking. Stress control, smoking being a habit, and good taste were the reasons for smoking cited most often in both groups. Peer smoking was more important in survivors (49%) than in TMS (34%, P = 0.004). Most important reasons for not smoking in both groups were smoking being unhealthy and not wanting to be addicted. CONCLUSIONS In Switzerland, survivors smoke as often as their siblings but less than the general population. Peer smoking was a more important reason for smoking in survivors than in the general population, suggesting that reducing smoking in peers could result in a reduction of smoking in survivors. Overall, reasons for smoking were very similar, thus interventions to reduce smoking in survivors could be the same as those used in the general population.

Industrie 4.0 and the consequent necessity of digitalization has also implications to the field of procurement, resulting in the so-called term of Procurement 4.0. Digitalization can be a valuable tool to increase the efficiency of the procurement organization and to exploit new opportunities of growth. A mandatory requirement to perform the digital transformation is an increased transparency along the procurement process chain. This paper aims to conceptualize a digital shadow for the procurement process in manufacturing industry as a basis for advanced data analytics procedures. The term digital shadow stands for a sufficiently accurate, digital image of a company’s processes, information and data. This image is needed to create a real-time evaluable basis of all relevant data in order to finally derive recommendations for action. The formation of the Digital Shadow is thus a central field of action for Industrie 4.0 and forms the basis for all further activities.

Objective: Childhood cancer survivors are at high risk for late effects. Regular attendance to long-term follow-up care is recommended and helps monitoring survivors' health. Using the Theory of Planned Behavior (TPB) we aimed to 1) investigate the predictors of the intention to attend follow-up care, and 2) examine the associations between perceived control and behavioral intention with actual follow-up care attendance in Swiss childhood cancer survivors. Methods: We conducted a questionnaire survey in Swiss childhood cancer survivors (diagnosed with cancer aged

Isocitrate Dehydrogenase-1 (IDH1) is a driver gene in several cancers including brain tumors such as low-grade and high-grade gliomas. Mutations of SMARCB1 were described in atypical teratoid rhabdoid tumors and to date have not been associated with the pathogenesis of medulloblastoma. We report concurrent IDH1 and SMARCB1 mutations in a medulloblastoma patient. We searched the catalog of somatic mutations in cancer (COSMIC) database and other mutation databases and -to our knowledge- this is the first reported case of medulloblastoma harboring both mutations together. Our patient is a 13-year-old male presenting with headache and vomiting at diagnosis. MRI revealed left cerebellar expansive lesion with no evidence of metastasis. A histopathological diagnosis of desmoplastic/nodular medulloblastoma was made after complete resection of the tumor. Immunophenotypic characterization and methylation profiling suggested a medulloblastoma with SHH activation. Next generation sequencing of a panel of 400 genes revealed heterozygous somatic IDH1(p.R132C), SMARCB1(p.R201Q), and CDH11(p.L625T) mutations. The patient was treated according to the HIT-SIOP PNET 4 protocol. He is in complete remission more than 2 years after diagnosis. In conclusion, increasing use of high throughput sequencing will certainly increase the frequency with which rare mutations or mutation combinations are identified. The exact frequency of this mutation combination and whether it has any particular therapeutic implications or prognostic relevance requires further investigation.

In humans, stress can be contagiously transmitted via chemosignals on a subconscious level. This study investigates how pregnancy affects neural responses to anxiety chemosignals. Using cotton pads, 28 men donated axillary sweat immediately before an academic examination (anxiety sweat) and during ergometer training (control). Via a constant-flow olfactometer, samples were presented (oddball paradigm) to 12 non-pregnant (NP) women, 14 women in their first (T1), and 18 in their third (T3) trimester of pregnancy. Chemosensory event-related potentials and current source densities (CSD) were analysed (60 electrode setup). Compared to NP-women, pregnant women display diminished evaluative processing of the sweat samples (targets; P3-1/ P3-2 amplitudes) and delayed evaluative processing of the anxiety sweat (targets; P3-2 latency). T3-women show attenuated early processing (targets; N1 amplitude) compared to NP-women, and reduced evaluative processing compared to T1-women (standards; P3-2 amplitude). CSDs (P3-1/ P3-2 latency ranges) reveal that T1- and T3-women show an atypical activation distribution to anxiety sweat. Most participants were unable to detect the sweat samples (anxiety sweat: 79.5%, sport sweat 88.6%). The results demonstrate that the processing of anxiety chemosignals progressively vanishes during pregnancy. This effect is likely to occur without any cognitive control.

BACKGROUND Little is known about follow-up care attendance of adolescent survivors of childhood cancer, and which factors foster or hinder attendance. Attending follow-up care is especially important for adolescent survivors to allow for a successful transition into adult care. We aimed to (i) describe the proportion of adolescent survivors attending follow-up care; (ii) describe adolescents' health beliefs; and (iii) identify the association of health beliefs, demographic, and medical factors with follow-up care attendance. PROCEDURE Of 696 contacted adolescent survivors diagnosed with cancer at ≤16 years of age, ≥5 years after diagnosis, and aged 16-21 years at study, 465 (66.8%) completed the Swiss Childhood Cancer Survivor Study questionnaire. We assessed follow-up care attendance and health beliefs, and extracted demographic and medical information from the Swiss Childhood Cancer Registry. Cross-sectional data were analyzed using descriptive statistics and logistic regression models. RESULTS Overall, 56% of survivors reported attending follow-up care. Most survivors (80%) rated their susceptibility for late effects as low and believed that follow-up care may detect and prevent late effects (92%). Few (13%) believed that follow-up care is not necessary. Two health beliefs were associated with follow-up care attendance (perceived benefits: odds ratio [OR]: 1.56; 95% confidence interval [CI]: 1.07-2.27; perceived barriers: OR: 0.70; 95%CI: 0.50-1.00). CONCLUSIONS We show that health beliefs are associated with actual follow-up care attendance of adolescent survivors of childhood cancer. A successful model of health promotion in adolescent survivors should, therefore, highlight the benefits and address the barriers to keep adolescent survivors in follow-up care. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.

Impacts of human civilization on ecosystems threaten global biodiversity. In a changing environment, traditional in situ approaches to biodiversity monitoring have made significant steps forward to quantify and evaluate BD at many scales but still, these methods are limited to comparatively small areas. Earth observation (EO) techniques may provide a solution to overcome this shortcoming by measuring entities of interest at different spatial and temporal scales. This paper provides a comprehensive overview of the role of EO to detect, describe, explain, predict and assess biodiversity. Here, we focus on three main aspects related to biodiversity − taxonomic diversity, functional diversity and structural diversity, which integrate different levels of organization − molecular, genetic, individual, species, populations, communities, biomes, ecosystems and landscapes. In particular, we discuss the recording of taxonomic elements of biodiversity through the identification of animal and plant species. We highlight the importance of the spectral traits (ST) and spectral trait variations (STV) concept for EO-based biodiversity research. Furthermore we provide examples of spectral traits/spectral trait variations used in EO applications for quantifying taxonomic diversity, functional diversity and structural diversity. We discuss the use of EO to monitor biodiversity and habitat quality using different remote-sensing techniques. Finally, we suggest specifically important steps for a better integration of EO in biodiversity research. EO methods represent an affordable, repeatable and comparable method for measuring, describing, explaining and modelling taxonomic, functional and structural diversity. Upcoming sensor developments will provide opportunities to quantify spectral traits, currently not detectable with EO, and will surely help to describe biodiversity in more detail. Therefore, new concepts are needed to tightly integrate EO sensor networks with the identification of biodiversity. This will mean taking completely new directions in the future to link complex, large data, different approaches and models.

Background: Smoking is a risky health behavior, in particular for vulnerable populations such as childhood cancer survivors (CCS), who have elevated risks for chronic pulmonary and cardiac disease because of chemotherapy and chest irradiation. This nationwide study assessed smoking habits and reasons for smoking in CCS compared to healthy controls. Methods: As part of the Swiss Childhood Cancer Survivor Study, we sent a questionnaire to all CCS living in Switzerland, aged 16-20 years, who had survived ≥5 years, and had been diagnosed 1976-2005. We compared smoking status and reasons for smoking of CCS with their siblings, and with participants in the Tobacco Monitoring Switzerland (TMS, 2001-2010), a representative survey of the adolescent population. Results: We included 464 CCS, 141 siblings, and 3455 participants of TMS. Smoking status was similar in CCS and their siblings: current smoker: 18% vs. 16%, p=0.765; ex-smoker: 3% vs. 2%, p=0.765. Compared to TMS CCS were less likely to be current (18% vs. 24%, p Conclusion: CCS have similar smoking habits as their siblings, but both smoke less than the general population. Better knowledge of their reasons for smoking might help to tailor smoking cessation and prevention programs for CCS during follow-up care.

Entropy noise affects thermoacoustic stability in lean pre-mixed gas turbine combustion chambers. It is defined as acoustic noise that is emitted at the first turbine stage due to the acceleration of entropy waves that are advected from the reaction zone in the combustor to the turbine inlet. These non-isentropic temperature waves are caused by equivalence ratio fluctuations which are inherently present in a technically premixed combustion system. To experimentally study the generation and transport of entropy waves, an estimation of the spatial distribution of the entropy spots is highly valuable as it allows the accurate determination of the cross-section averaged entropy, which is the relevant quantity for the formation mechanism of entropy noise at the turbine stage. In this work, a time-of-flight based temperature measurement method is applied to a circular combustion test rig equipped with a premixed swirl-stabilized combustor. Downstream of the burner, an electric spark discharge is employed to generate a narrow acoustic pulse which is detected with a circumferentially arranged microphone array. The measured time of flight of the acoustic signal corresponds to the line-integrated inverse of the speed of sound between the acoustic source and each microphone. By modulating a share of the injected gaseous fuel, equivalence ratio fluctuations are generated upstream of the reaction zone and consequently entropy spots are advected through the axial measurement plane. The spark discharge is triggered at distinct phase angles of the entropy oscillation, thus allowing a time resolved-analysis of the thermo-acoustic phenomenon. Estimating the spatial temperature distribution from the measured line integrated inverse speed of sounds requires tomographic reconstruction. A Tikhonov regularized Onion Peeling is employed to deduce radial temperature profiles. To increase the number of independent data, the spark location is radially traversed, which enhances the resolution of the reconstructed temperature field. A phantom study is conducted, which allows the assessment of the capabilities of the reconstruction algorithm. By means of the reconstructed radial entropy field, spatially resolved entropy waves are measured and their amplitudes and phases are extracted. The characteristics of the entropy waves measured in this way correspond well to former studies.

Social communication refers to a basic human need, and findings accumulate that show humans communicate numerous kinds of social information via chemosignals. Briefly, it will be introduced which chemicals are conveyed through body fluids and which sensory systems are considered to process social chemosignals. Then, it will be shown that pheromones in humans have not yet been discovered. Studies on putative pheromones in humans often are performed disregarding the biological underpinnings of chemical communication and seem randomly to investigate volatile substances without any theoretical background. However, evidence will be provided that human chemosensory communication has been well demonstrated, using natural body fluids (e. g., sweat) as the source of chemosignals. Humans can decode information about the immunogenetic profile and the level of sexual hormones from volatiles released from the sweat of other individuals. These chemical signals are considered to affect mate choice. However, the signal extraction also depends on the sexual orientation of the perceiver. Furthermore, the recognition of kin and mother-infant communication comprise the release and decoding of chemosignals. Both phenomena are important prerequisites for the formation of social bonding and harm protection. Finally, the communication of stress and anxiety in humans will be presented as an example of a chemical transmission of emotional states. At the end of the chapter it will be questioned whether chemosensory communication is a skill, protective for certain mental disorders.

Holy Motors, France and Germany, 2012, Pierre Grise Productions, directed and written by Leos Carax, starring Denis Lavant, Edith Scob, Eva Mendes, Kylie Minogue, and Michel Piccoli.

Introduction: In the last decade transplant activity increased constantly and consequently the number of long-term survivors. Most applied conditioning regimes are known to impair normal puberty development and can lead to infertility. Pregnancies in women who had undergone a HSCT as well as in partners of transplanted men have been documented. However, the true incidence of pregnancies and its related complications in patients transplanted during their childhood remains unknown. The Pediatric WP of the EBMT conducted a study aiming to analyze pregnancy rates and to evaluate pregnancy outcomes in survivors of childhood HSCT. Material and Methods: In this study, we first evaluate the available data on the EBMT registry. To collect additional data a survey was conducted between July 2017- June 2018. 203 centers with pediatric activity reported to the EBMT patients were asked to fill-out the survey with 36 items related to treatment plan, pregnancies and pregnancies outcome. Adoptions were not considered in the study. Results: Data obtained from the EBMT database "Promise" reported that 62'988 pediatric patients were transplanted in the EBMT centers from 1995 to end of 2016. In 484 (0.76%) patients a conception after HSCT has been reported. 341/484 patients (70%) had an allograft, 134 of them (40%) were males. 143/484 had an autologous, 53 of them (37%) were male. The median age of patients was 15.02 years (range 0.6 - 17.9 years), was similar for male and females regardless of whether they were auto or allogeneic transplanted. 283 patients (58%) had conditioning regimes containing alkylating agents, irradiation or both. Data obtained from the conducted survey provide precise information of 27 (13 %) EBMT centers from 16 countries about 114 pregnancies of 99 patients and their partners. Pregnancies were in 30/99 (30%) from partners of post HSCT male patients and 60/99 (70%) were of post HSCT females. Underlying diseases for HSCT were reported in 82 of all 99 patients. 47/82 (57%) had a malignant disease and35/82 (43%) had a non-malignant disease. 29/99 (29%) had a reduced conditioning regime, 70/99 (71%) a myeloablative conditioning regime. 25/82 (30%) received TBI as part of the conditioning regimes. Regarding conception in patients post TBI, 12/23 (52%) became naturally pregnant (2/12 had 2 Gy only), 9/23(39%) required assisted fertilization and 2 used cryopreserved sperms. In patients conditioned without TBI, 54/59 (92%) had a natural conception, 4/59 (7%) required assisted fertilization and 1 used cryopreserved sperms. In 90/99 (90%) patients pregnancy resulted in a livebirth. 13 of the 90 livebirths (14%) were preterm, with a higher proportion than in normal the population (5.5-11%) and 6 of the 90 livebirths (6%) presented a low birthweight which is comparable to the normal population (6.5%). Conclusion: This study confirms a low pregnancy rate in survivors of childhood HSCT. Natural conception was possible when patients are conditioned without TBI. After TBI, pregnancy assisted fertilization techniques may increase pregnancy rate. The number of live births and complications indicate the need to advise survivors of childhood HSCT about the potential risks and outcome of pregnancy and advise them appropiately. Disclosures Bader: Cellgene: Consultancy; Neovii: Research Funding; Medac: Patents & Royalties, Research Funding; Riemser: Research Funding; Novartis: Consultancy, Speakers Bureau.

INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a recently described histologic pattern of diffuse pulmonary disease. In children, all cases reported to date have been fatal. In this study, we describe the first nonfatal AFOP in a child and review the literature. DESCRIPTION: A 10-year-old boy developed very severe aplastic anemia (VSAA) after being admitted to our hospital with a fulminant hepatic failure of unknown origin. A chest computed tomography scan revealed multiple lung nodules and a biopsy of a pulmonary lesion showed all the signs of AFOP. Infectious workup remained negative. We started immunosuppressive therapy with antithymocyte globulin and cyclosporine to treat VSAA. Subsequent chest computed tomography scans showed a considerable diminution of the lung lesions but the VSAA did not improve until we performed hematopoietic stem cell transplantation 5 months later. CONCLUSIONS: Aplastic anemia is associated with a variety of autoimmune syndromes. The sequence of events in our patient suggests that the hepatic failure, AFOP, and the VSAA may all have been part of an autoimmune syndrome. AFOP could be the result of immune dysregulation in this pediatric case with favorable outcome after immunosuppressive therapy and hematopoietic stem cell transplantation.

This paper presents an electronic ballast for driving high intensity discharge (HID) lamps with low frequency square waveform from a dc supply. From the dc source (e.g., PFC rail), a flyback arrangement is inserted into a switching structure whose output provides low frequency voltage yet avoiding instability issues. Proposal's main advantage is the use of one single choke of simple assembly and only two active switches for current control and inversion. Converter design for DCM is presented in this work, besides an equivalent circuit from a control point of view. Small signal ac model is obtained and a feedback control loop is designed, based upon frequency response stability and performance analysis. HID dynamics is inserted into converter model, with feedback directly implemented. A prototype is presented and described in detail, consisting of a complete power and sensing circuit fully referenced to the switches common ground proposal. For design conformity, experimental and simulation results from Matlab and PSIM are presented, as well as discussions about the converter feasibility.

Theories of neuroaesthetics assume, that looking at traces of actions used in creating artwork (e.g. brush marks) is associated with a simulation of these actions in the observer’s sensorimotor-cortex. The aim of the current study is to dissociate the activation of the sensorimotor-cortex by the observation of action traces from associated visual processes. Twenty-eight participants observed handmade graphics (acrylic paint on paper) of different complexity (line, triangle, shape of a house) and computer-generated counterparts. Central mu-activity, as an index of sensorimotor-cortex activity, and occipital alpha-activity, as an index of visual cortex activity were recorded in the 8–13 Hz EEG-band. In line with the hypothesis, mu-activity at electrode C4 is sensitive for the complexity of handmade (p = 0.001), but not computer-generated graphics (p > 0.500). In contrast, occipital alpha-activity is sensitive for the complexity of both handmade and computer-generated graphics (p

Background Dapoxetine hydrochloride is a selective serotonin reuptake inhibitor and the first drug approved for the on-demand treatment of premature ejaculation (PE). Its safety was established in a thorough clinical development program. Objective To characterize the safety profile of dapoxetine in PE treatment and to report the incidence, severity, and type of adverse events. Design, setting, and participants We conducted a 12-wk, open-label, observational study with a 4-wk, postobservational contact. A total of 10 028 patients were enrolled, with 6712 patients (67.6%) treated with dapoxetine 30–60mg (group A)and 3316 (32.4%) treated with alternative care/nondapoxetine (group B). Interventions Treatment with dapoxetine or alternative care/nondapoxetine. Outcome measurements and statistical analysis Treatment-emergent adverse events (TEAEs) and concomitant therapy use during the 12-wk observational and the postobservational period were reported. Results and limitations The mean age for all patients was 40.5 yr. In group A, 93.0% of the patients were initially prescribed dapoxetine 30mg. Treatment options for group B patients included clomipramine, paroxetine, fluoxetine, sertraline, topical drugs, condoms, and behavioral counseling. Both treatment regimens were well tolerated. TEAEs were reported by 12.0% and 8.9% of group A and group B, respectively, with the highest incidence observed in patients aged >65 yr for group A (21.4%) and 30–39 yr (9.8%) for group B. The most commonly reported TEAEs were nausea, headache, and vertigo, with a higher incidence in group A (3.1%, 2.6%, and 1.0%, respectively) than in group B (oral drugs: 2.3%, 1.3%, and 0.9%, respectively). There were no cases of syncope in group A and one case in group B. A major limitation is that this was a nonrandomized, open-label, short-term study lacking efficacy data. Conclusions The results of this postmarketing observational study demonstrated that dapoxetine for treatment of PE has a good safety profile and low prevalence of TEAEs. Syncope and major cardiovascular adverse events were not reported. The high level of adherence by healthcare providers to the contraindications, special warnings, and precautions for dapoxetine minimizes the risk for its use in routine clinical practice. The current risk minimization measures for its identified and potential risks are effective.

libRadtran is a widely used software package for radiative transfer calculations. It allows one to compute (polarized) radiances, irradiance, and actinic fluxes in the solar and thermal spectral regions. libRadtran has been used for various applications, including remote sensing of clouds, aerosols and trace gases in the Earth's atmosphere, climate studies, e.g., for the calculation of radiative forcing due to different atmospheric components, for UV forecasting, the calculation of photolysis frequencies, and for remote sensing of other planets in our solar system. The package has been described in Mayer and Kylling (2005). Since then several new features have been included, for example polarization, Raman scattering, a new molecular gas absorption parameterization, and several new parameterizations of cloud and aerosol optical properties. Furthermore, a graphical user interface is now available, which greatly simplifies the usage of the model, especially for new users. This paper gives an overview of libRadtran version 2.0.1 with a focus on new features. Applications including these new features are provided as examples of use. A complete description of libRadtran and all its input options is given in the user manual included in the libRadtran software package, which is freely available at http://www.libradtran.org.

Influenced by the high dynamic of the markets the optimization of supply chains gains more importance. However, analyzing different procurement strategies and the influence of various production parameters is difficult to achieve in industrial practice. Therefore, simulations of supply chains are used in order to improve the production process. The objective of this research is to evaluate different procurement strategies in a four-stage supply chain. Besides, this research aims to identify main influencing factors on the supply chain’s performance. The performance of the supply chain is measured by means of back orders (backlog). A scenario analysis of different customer demands and a Design of Experiments analysis enhance the significance of the simulation results.

TA-TMA is a pathology that occurs after allogenic HSC transplantation with an incidence of 4-13%, and represents one of the most severe vascular damage related with this therapy. We report here the case of a nine-yr-old girl suffering from a severe refractory aplastic anemia who received an unrelated, 9/10 HLA-matched HSC. Soon after transplantation, the patient developed a graft-versus-host disease (GvHD), a TA-TMA, and renal insufficiency. These pathologies remained refractory to the various treatments undertaken and required several hospitalizations in the intensive care unit. On day 106 post-HSC transfusion, after several episodes of intensive care, the patient was infused with mismatched, third-party MSCs. Schizocyte levels rapidly decreased after MSC infusion, and two wk later, most biological parameters returned to normal. Erythrocyte and thrombocyte transfusions were discontinued, and the patient remained stable for 10 wk. Thereafter, TA-TMA symptoms, viral reactivation, pleural and cardiac effusions reappeared and lead to the death of the patient. Our observations suggest that allogenic MSC infusion may decrease the symptoms of TA-TMA, but further investigation is required to determine how and when MSC should be infused to develop a long-lasting protective effect.

Nach Abschluss der Akutversorgung bei schweren neurologischen Erkrankungen werden Patienten in Deutschland in der sog. Phase B fruhzeitig rehabilitiert. Uber den Langzeitverlauf dieser Patienten existieren bisher keine Studien. Patienten der Phase B aus 9 Zentren wurden im Jahr 2002 prospektiv erfasst und nach 5 und 6 Jahren nachuntersucht. Als Assessmentinstrumente dienten der Barthel-Index, die Rankin-Skala und der EQ-5D. Mittels multivariater logistischer Regressionen wurden Faktoren fur das Risiko fur schlechtes bzw. Chancen fur gutes Outcome ermittelt. Insgesamt 1280 Patienten wurden in die Studie eingeschlossen. Ein hohes Alter (Hazard-Quotient [HQ] 1,05; 95%-KI: 1,04–1,06) erhohte und eine hohe Punktzahl in der Komaremissionsskala (HQ 0,93; 95%-KI: 0,92–0,96) und im Barthel-Index (HQ 0,97; 95%-KI: 0,97–0,98) bei Entlassung verringerten das Sterberisiko nach 5 Jahren. Die Faktoren Schluckstorung (OR 3,1; 95%-KI: 1,7–5,5) und Uberwachungspflicht am Ende der Rehabilitation (OR 3,2; 95%-KI: 1,2–8,6) erhohten das Risiko fur ein schlechtes Ergebnis in der Rankin-Skala 2–4 und die Faktoren Verstandigungsstorung (OR 5,0; 95%-KI: 2,0–12,8) und perkutane endoskopische Gastrostomie (OR 19,7; 95%-KI: 2,7–144,4) bei Entlassung erhohten das Risiko fur eine reduzierte gesundheitsbezogene Lebensqualitat (definiert als EQ-5D-VAS

Die Laterodysgnathie ist eine Sonderform der skelettalen Dysgnathie, die sich durch eine Asymmetrie des Gesichtsschadels auszeichnet. Bei der chirurgischen Korrektur kommen die Verfahren der Umstellungsosteotomien zur Anwendung. Zusatzliche Masnahmen zur Verbesserung der auseren Kontur und der Okklusion sind moglich. Je nach Auspragung der Dysgnathie werden die Eingriffe in abgestufter Form durchgefuhrt. Insbesondere bei Defiziten im Weichgewebe konnen Restasymmetrien verbleiben. Vier klinische Falle werden hier vorgestellt.

Socioeconomic status (SES) discrepancies exist for child and adult cancer morbidity and are a major public health concern. In this Swiss population-based matched case-control study on the etiology of childhood leukemia, we selected the cases from the Swiss Childhood Cancer Registry diagnosed since 1991 and the controls randomly from census. We assigned eight controls per case from the 1990 and 2000 census and matched them by the year of birth and gender. SES information for both cases and controls was obtained from census records by probabilistic record linkage. We investigated the association of SES with childhood leukemia in Switzerland, and explored whether it varied with different definitions of socioeconomic status (parental education, living condition, area-based SES), time period, and age. In conditional logistic regression analyses of 565 leukemia cases and 4433 controls, we found no consistent evidence for an association between SES and childhood leukemia. The odds ratio comparing the highest with the lowest SES category ranged from 0.95 (95% CI: 0.71-1.26; P trend = 0.73) for paternal education to 1.37 (1.00-1.89; P trend = 0.064) for maternal education. No effect modification was found for time period and age at diagnosis. Based on this population-based study, which avoided participation and reporting bias, we assume the potential association of socioeconomic status and childhood leukemia if existing to be small. This study did not find evidence that socioeconomic status, of Switzerland or comparable countries, is a relevant risk factor or strong confounder in etiological investigations on childhood leukemia.

Delphine Arni, Fabienne Gumy-Pause, Marc Ansari, Johanna A. Kremer Hovinga, and Val erie A. McLin Division of Pediatric Surgery, Pediatric Onco-hematology unit, Geneva University Hospitals, Geneva, Switzerland; Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, Switzerland; and Swiss Center of Liver Disease, Geneva University Hospitals, Geneva, Switzerland

Objective: To study the effects of augmented exercise therapy time for arm rehabilitation as either Bobath therapy or the impairment-oriented training (Arm BASIS training) in stroke patients with arm severe paresis. Design: Single blind, multicentre randomized control trial. Setting: Three inpatient neurorehabilitation centres. Subjects: Sixty-two anterior circulation ischaemic stroke patients. Interventions: Random assignment to three group: (A) no augmented exercise therapy time, (B) augmented exercise therapy time as Bobath therapy and (C) augmented exercise therapy time as Arm BASIS training. Main measures: Main outcome measure: Fugl-Meyer arm motor score. Secondary measure: Action Research Arm Test (ARA). Ancillary measures: Fugl-Meyer arm sensation and joint motion/pain scores and the Ashworth Scale (elbow flexors). Results: An overall effect of augmented exercise therapy time on Fugl-Meyer scores after four weeks was not corroborated (mean and 95% confidence interval (CI) of change scores: no augmented exercise therapy time ( n = 20) 8.8, 5.2–12.3; augmented exercise therapy time ( n = 40) 9.9, 6.8-13.9; p = 0.2657). The group who received the augmented exercise therapy time as Arm BASIS training ( n = 20) had, however, higher gains than the group receiving the augmented exercise therapy time as Bobath therapy ( n = 20) (mean and 95% CI of change scores: Bobath 7.2, 2.6-11.8; BASIS 12.6, 8.4 - 16.8; p = 0.0432). Passive joint motion/pain deteriorated less in the group who received BASIS training (mean and 95% CI of change scores: Bobath -3.2, -5.2 to -1.1; BASIS 0.1, -1.8-2.0; p = 0.0090). ARA, Fugl-Meyer arm sensation, and Ashworth Scale scores were not differentially affected. Conclusions: The augmented exercise therapy time as Arm BASIS training enhanced selective motor control. Type of training was more relevant for recovery of motor control than therapeutic time spent.