Diminished differentiation of rewards in individuals at clinical high-risk for psychosis.

Reward processing is impaired in people with schizophrenia, which may begin in the clinical high-risk (CHR) for psychosis period. The Monetary Incentive Delay (MID) task has been important in understanding the neural correlates of reward processing deficits in various psychiatric disorders. Previous research has found that CHR individuals have an imprecise mental representation of rewards, which leads to a diminished differentiation between rewards, though this has not been observed behaviorally. A total of 19 CHR individuals and 20 controls were given a novel variant of the MID task, designed to examine how modulating reward context may impact responses to reward cues, a process often referred to as "adaptive coding." Both groups appeared to update their behavior in response to the rewards available in this adaptive task. However, when compared to controls who showed a more graded decrease in response time to increasing reward contexts, CHR individuals appeared to have a sharp decrease in response time in the low reward context that is nearly stable across higher reward contexts. This is largely driven by the exponential component of the response time distribution, which is often interpreted to be more cognitively or effortfully influenced. Response times are related to negative symptoms, but not positive symptoms, disorganized symptoms, or estimated intelligence. Although an adaptive coding effect was not observed, these results provide novel insight into the reward processing mechanisms and volitional processes in the CHR population, as this was the first study to observe the diminished differentiation of rewards behaviorally.

[Implementation of biosecurity measures by hoof trimmers in Switzerland]. / Umsetzung von Biosicherheitsmassnahmen durch Klauenpfleger in der Schweiz.

INTRODUCTION: Biosecurity in livestock farming includes all measures preventing pathogen introduction onto a farm (external biosecurity) and pathogen transmission on the farm itself (internal biosecurity). An important risk factor for the dissemination of infectious diseases are specialised external persons working on numerous farms, such as professional hoof trimmers in Switzerland. In the present study, 49 hoof trimmers, participating in the Swiss claw health programme and working as professionals, were questioned regarding their biosecurity measures and observed by two veterinarians during hoof trimming in order to assess the implementation of biosecurity measures by hoof trimmers. Data were processed using a scoring system, in which points were allocated to the different working methods taking into account their assumed transmission potential for infectious diseases such as digital dermatitis (DD) and Salmonellosis. The working method, which complied with the ideal biosecurity measure, was always given a whole point, whereas less optimal working methods were only given an intermediate value or no point. The scoring system helped identify precisely the strengths and weaknesses of the hoof trimmers in terms of biosecurity. The level of implementation of biosecurity measures by hoof trimmers was overall quite low (53 %=average of the overall biosecurity scores of the 49 hoof trimmers). Hoof trimmers which attended specialised training courses tended to have a higher level of implementation of biosecurity measures. The answers given by the hoof trimmers and the observations made by the veterinarians were compared, whereby it was found that hoof trimmers generally evaluated themselves better in regard to biosecurity than veterinarians assessed them. In summary and based on the results of this study, the dissemination of pathogens, such as DD associated treponemes and salmonella is possible during hoof trimming performed by external persons working on numerous farms. Thus, future training and continuing education courses should place emphasis on biosecurity.

INTRODUCTION: Le concept de biosécurité englobe, en lien avec la production animale, toutes les mesures empêchant l'introduction de germes dans une exploitation (biosécurité externe) et la propagation de germes à l'intérieur de l'exploitation (biosécurité interne). Un facteur de risque important pour la propagation de maladies infectieuses est le personnel spécialisé externe travaillant sur plusieurs exploitations, dont font partie les pareurs d'onglons professionnels intervenant sur les exploitations bovines suisses. Dans la présente étude, afin de donner un aperçu de la situation actuelle concernant la mise en oeuvre de mesures de biosécurité par les pareurs d'onglons, 49 pareurs d'onglons participant au programme suisse de santé des onglons, ont été questionnés à ce sujet et observés lors du parage des onglons par des vétérinaires. Le traitement des données a été effectué à l'aide d'un système de notation, attribuant des points aux différentes pratiques de travail selon leur potentiel supposé de transmission des maladies infectieuses que sont la Dermatite digitale (DD) et la Salmonellose. La pratique de travail, qui correspondait à la mesure de biosécurité idéale, obtenait toujours un point entier, alors que les pratiques de travail moins optimales ne recevaient qu'une valeur intermédiaire ou aucun point. Le système de notation a permis de désigner précisément les forces et les faiblesses des pareurs d'onglons en terme de biosécurité. Le niveau de mise en œuvre de mesures de biosécurité par les pareurs d'onglons est de manière générale relativement faible (53 % = moyenne du score de biosécurité générale des 49 pareurs). Les pareurs d'onglons ayant suivi plus fréquemment des formations spécifiques présentaient tendanciellement un niveau de mise en oeuvre de mesures de biosécurité plus élevé. De plus, les réponses des pareurs d'onglons et les observations des vétérinaires ont été comparées. Il a été constaté, que les pareurs d'onglons s'évaluaient généralement meilleurs en matière de biosécurité que les vétérinaires ne les jugeaient. En résumé et en tenant compte des résultats de cette étude, la propagation de germes pathogènes par les pareurs d'onglons dans le cadre de leur activité professionnelle, tels que les tréponèmes associés à la DD et les salmonelles, est possible. Par conséquent, la biosécurité devrait être thématisée en priorité lors des formations et formations continues futures.

[Prevalence of claw disorders in swiss cattle farms]. / Prävalenz von Klauenveränderungen in Schweizer Rindviehbetrieben.

INTRODUCTION: The project «Healthy claws - the foundation for the future¼ aims to establish a Swiss national claw health monitoring based on digital recordings by claw trimmers during claw trimming. To assess claw health on the participating farms, between-herd prevalence, within-herd prevalence and cow prevalence of all claw disorders based on the «ICAR Claw Health Atlas¼ were calculated during this study. Claw trimmers underwent an intensive training and examination in order to ensure data quality. To guarantee the representativity of the prevalences, only farm claw trimmings were considered, where ≥ 80 % of the cows in a herd were trimmed. The calculations were based on 7108 cows and 403 heifers from 238 farms, during the period from February 2020 to February 2021. At least one claw disorder was present in 99,2 % of the farms, with 49,6 % of the heifers and 77,7 % of the cows having at least one claw disorder. The high prevalence is seen as a result of all ICAR claw disorders being considered, whereas not all of them are painful and consequently not all of them cause lameness. The absence of lameness assessment limits the evaluation of existing herd problems. High between-herd and cow prevalences were observed for the following claw disorders: heel horn erosion (92,9 %/64,7 %), digital dermatitis (55,9 %/20,7 %), white line disease (81,5 %/17,7 %) and sole hemorrhage (66,4 %/11,6 %). Asymmetric claws, corkscrew claws, scissor claws, horn fissure, interdigital phlegmon, swelling of the coronet and/or bulb and toe necrosis had low prevalences. The proportion of cows treated with a hoof block (0,5 %) was comparatively small in regard of the cows suffering from ulcers (5,6 %) and white line abscesses (2,5 %). The median within-herd prevalence of digital dermatitis was 5,6 %, with a maximal within-herd prevalence of 87,5 %. Despite the contagious nature of digital dermatitis, no increase of between-herd and cow prevalence has been observed in the past ten years throughout Switzerland. Based on this data, the Swiss claw health situation can be monitored, compared over time and improved in the future.

INTRODUCTION: Le projet « Des onglons sains-de bon pied vers l'avenir ¼ vise à établir une surveillance nationale suisse de la santé des onglons basée sur des enregistrements numériques effectués par des ongleurs pendant le parage des onglons. Pour évaluer la santé des onglons dans les exploitations participantes, la prévalence entre les troupeaux, la prévalence au sein du troupeau et la prévalence chez les vaches de tous les troubles des onglons sur la base de l' « ICAR Claw Health Atlas ¼ ont été calculées au cours de cette étude. Les ongleurs ont subi une formation et un examen intensifs afin de garantir la qualité des données. Afin de garantir la représentativité des prévalences, seuls ont été considérés les parages d'exploitations où ≥ 80% des vaches du troupeau ont été parées. Les calculs ont été basés sur 7108 vaches et 403 génisses de 238 élevages, au cours de la période de février 2020 à février 2021. Au moins une lésion des onglons était présente dans 99,2 % des élevages, avec 49,6 % des génisses et 77,7 % des vaches présentant au moins une lésion. La prévalence élevée est considérée comme le résultat de toutes les lésions selon ICAR, alors que toutes ne sont pas douloureuses et par conséquent ne provoquent pas toute de boiterie. L'absence d'évaluation de la boiterie limite l'évaluation des problèmes de troupeau existants. Des prévalences élevées entre les troupeaux et les vaches ont été observées pour les lésions des onglons suivantes : érosion de la corne du talon (92,9%/64,7%), dermatite digitale (55,9%/20,7%), maladie de la ligne blanche (81,5 %/17,7%) et hémorragie de la sole (66,4%/11,6%). Les onglons asymétriques, les onglons en tire-bouchon, les onglons en ciseaux, la fissure de la corne, le phlegmon interdigital, le gonflement de la couronne et/ou la nécrose de la pince avaient de faibles prévalences. La proportion de vaches traitées avec un sabot (0,5 %) était comparativement faible par rapport aux vaches souffrant d'ulcères (5,6%) et d'abcès de la ligne blanche (2,5%). La prévalence médiane intra-troupeau de dermatite digitale était de 5,6%, avec une prévalence intra-troupeau maximale de 87,5%. Malgré le caractère contagieux de la dermatite digitale, aucune augmentation de la prévalence entre troupeaux et vaches n'a été observée au cours des dix dernières années dans toute la Suisse. Sur la base de ces données, la situation sanitaire des onglons en Suisse peut être surveillée, comparée dans le temps et améliorée à l'avenir.

[Evaluation of a novel training course for hoof trimmers to participate in a Swiss national cattle claw health monitoring programme]. / Evaluation eines Ausbildungskonzeptes für Klauenpfleger zur Durchführung eines schweizweiten Klauengesundheitsmonitorings für Rinder.

INTRODUCTION: The main goal of the resources project «Healthy claws - the foundation for the future¼ is to establish a Swiss national claw health monitoring programme for cattle, similar to what has already been established in other countries (e. g. Finland, Sweden). So far in the course of the project, 30 claw trimmers have been trained to use a digital recording software. An appropriate training concept and information to the work environment of Swiss claw trimmers are necessary to ensure that the recorded claw health data is of good quality. The newly developed training programme for claw trimmers was evaluated using the 30 first trained claw trimmers of the project. The training consisted of group events and individual schooling sessions, during which the claw trimmers were trained to recognise, classify and digitally record foot and claw disorders according to the «ICAR claw health atlas¼. At the end of the training programme, demographic and work relevant data about the claw trimmers was collected and their abilities when using the recording software, as well as recognising and classifying foot and claw disorders using pictures or live animals, were evaluated. The demographic and work relevant data collected at the end of the training showed an ageing of the profession (43% of the participants were over 50 years old), a lack of full-time claw trimmers (23% of the claw trimmers worked 90% or 100%), a neglect of locomotion scoring during claw trimming (no locomotion scoring standing from 60% and in movement from 33% of the participants) and a broad use of the Swiss claw trimming method (90% of the claw trimmers indicate using this method). An average Cohens kappa value of 0,74, and thus an overall substantial agreement with the first author, respectively the ICAR Atlas, was achieved for the identification and classification of foot and claw disorders. It was also noted, that all the participants were capable of using the recording software in practice after their schooling. The calculation of Cohens kappa values helps to recognise claw trimmers which fall below the limiting value and therefore show an insufficient agreement. These claw trimmers can thus be excluded from the scientific evaluation or undergo further training. It was concluded that the described training concept is suitable to establish a national claw health monitoring programme.

INTRODUCTION: L'objectif principal du projet «Des onglons sains ­ de bon pied vers l'avenir¼ est la mise en place d'un programme national suisse de surveillance de la santé des onglons des bovins, similaire à ce qui a déjà été mis en place dans d'autres pays (par ex. Finlande, Suède). Jusqu'à présent, au cours du projet, 30 pareurs d'onglons ont été formés à l'utilisation d'un logiciel de documentation électronique. Un concept de formation approprié et des informations sur l'environnement de travail des pareurs suisses sont nécessaires pour garantir la bonne qualité des données enregistrées. Le programme de formation nouvellement mis au point a été évalué à l'aide des 30 premiers pareurs formés. La formation a consisté en des formations de groupe et des formations individuelles, au cours desquelles les pareurs ont été formés à reconnaître, classifier et enregistrer électroniquement les maladies des onglons selon l'«Atlas ICAR santé des onglons¼. À la fin de la formation, des données démographiques et professionnelles concernant les pareurs ont été collectées et leurs capacités à utiliser le logiciel d'enregistrement, à reconnaître et classifier les maladies des onglons à l'aide d'images ou sur des animaux vivants, ont été évaluées. Les données démographiques et professionnelles collectées à l'issue de la formation ont entre autre montré un vieillissement de la profession (43% des participants avaient plus de 50 ans), un manque d'ongleurs à temps plein (23% des ongleurs travaillent à 90% resp. 100%), une négligence de la notation de la motricité lors du parage des onglons (pas de notation de la motricité stationnaire chez de 60% et en mouvement chez33% des participants) et une large utilisation de la méthode suisse de parage des onglons (90% des ongleurs indiquent utiliser cette méthode). Une valeur moyenne du coefficient kappa de Cohens de 0,74, et donc une concordance globale avec le premier auteur, respectivement l'Atlas ICAR, a été obtenue pour l'identification et la classification des maladies des onglons. Il a également été constaté que tous les participants étaient capables après leur formation d'utiliser le logiciel d'enregistrement dans la pratique. Le calcul de valeurs kappa de Cohens permet de reconnaître les pareurs qui descendent en dessous d'une valeur limite et présentent donc une concordance insuffisante. Ces pareurs peuvent ainsi être exclus de l'évaluation scientifique ou suivre une formation complémentaire. On peut en conclure que le concept de formation décrit convient pour la mise en place d'un programme national de surveillance de la santé des onglons.

Rapamycin-based graft-versus-host disease prophylaxis increases the immunosuppressivity of myeloid-derived suppressor cells without affecting T cells and anti-tumor cytotoxicity.

The immunosuppressant rapamycin (RAPA) inhibits mammalian target of rapamycin (mTOR) functions and is applied after allogeneic bone marrow transplantation (BMT) to attenuate the development of graft-versus-host disease (GVHD), although the cellular targets of RAPA treatment are not well defined. Allogeneic T cells are the main drivers of GVHD, while immunoregulatory myeloid-derived suppressor cells (MDSCs) were recently identified as potent disease inhibitors. In this study, we analyzed whether RAPA prevents the deleterious effects of allogeneic T cells or supports the immunosuppressive functions of MDSCs in a BMT model with major histocompatibility complex (MHC) classes I and II disparities. RAPA treatment efficiently attenuated clinical and histological GVHD and strongly decreased disease-induced mortality. Although splenocyte numbers increased during RAPA treatment, the ratio of effector T cells to MDSCs was unaltered. However, RAPA treatment induced massive changes in the genomic landscape of MDSCs preferentially up-regulating genes responsible for uptake or signal transduction of lipopeptides and lipoproteins. Most importantly, MDSCs from RAPA-treated mice exhibited increased immunosuppressive potential, which was primarily inducible nitric oxide synthase (iNOS)-dependent. Surprisingly, RAPA treatment had no impact on the genomic landscape of T cells, which was reflected by unchanged expression of activation and exhaustion markers and cytokine profiles in T cells from RAPA-treated and untreated mice. Similarly, T cell cytotoxicity and the graft-versus-tumor effect were maintained as co-transplanted tumor cells were efficiently eradicated, indicating that the immunosuppressant RAPA might be an attractive approach to strengthen the immunosuppressive function of MDSCs without affecting T cell immunity.

Abnormal cognitive effort allocation and its association with amotivation in first-episode psychosis.

BACKGROUND: Abnormal effort-based decision-making represents a potential mechanism underlying motivational deficits (amotivation) in psychotic disorders. Previous research identified effort allocation impairment in chronic schizophrenia and focused mostly on physical effort modality. No study has investigated cognitive effort allocation in first-episode psychosis (FEP). METHOD: Cognitive effort allocation was examined in 40 FEP patients and 44 demographically-matched healthy controls, using Cognitive Effort-Discounting (COGED) paradigm which quantified participants' willingness to expend cognitive effort in terms of explicit, continuous discounting of monetary rewards based on parametrically-varied cognitive demands (levels N of N-back task). Relationship between reward-discounting and amotivation was investigated. Group differences in reward-magnitude and effort-cost sensitivity, and differential associations of these sensitivity indices with amotivation were explored. RESULTS: Patients displayed significantly greater reward-discounting than controls. In particular, such discounting was most pronounced in patients with high levels of amotivation even when N-back performance and reward base amount were taken into consideration. Moreover, patients exhibited reduced reward-benefit sensitivity and effort-cost sensitivity relative to controls, and that decreased sensitivity to reward-benefit but not effort-cost was correlated with diminished motivation. Reward-discounting and sensitivity indices were generally unrelated to other symptom dimensions, antipsychotic dose and cognitive deficits. CONCLUSION: This study provides the first evidence of cognitive effort-based decision-making impairment in FEP, and indicates that decreased effort expenditure is associated with amotivation. Our findings further suggest that abnormal effort allocation and amotivation might primarily be related to blunted reward valuation. Prospective research is required to clarify the utility of effort-based measures in predicting amotivation and functional outcome in FEP.

Apparent Diffusion Coefficient Levels and Neurodevelopmental Outcome in Fetuses with Brain MR Imaging White Matter Hyperintense Signal.

BACKGROUND AND PURPOSE: One of the perplexing findings of fetal brain MR imaging is white matter T2 hyperintense signal. The aims of our study were initially to determine the main etiologies associated with white matter T2 hyperintense signal, then to examine whether the different etiologies have different ADC values, and, last, to assess the association of white matter T2 hyperintense signal with developmental outcome. MATERIALS AND METHODS: This was a prospective cohort study of 44 MR imaging scans of fetal brains obtained for suspected brain pathologies at a tertiary medical center during 2011-2015. Clinical data were collected from electronic medical charts. ADC values were measured and averaged in the frontal, parietal, occipital, and temporal lobes. Neurodevelopmental assessments were performed with the Vineland Adaptive Behavior Scales II. RESULTS: Half of the cases of MRI hyperintense T2 signal of the fetal brain were associated with congenital cytomegalovirus infection. The other half were mainly idiopathic. Thus, the study group was divided to subgroups positive and negative for cytomegalovirus. Both groups had hyperintense signal in the temporal lobe. The group positive for cytomegalovirus had involvement of the parietal lobe. Only this group had increased ADC values in the temporal and parietal lobes. There was no association between the neurodevelopment outcome and the etiologies or ADC values. CONCLUSIONS: T2 hyperintense signal in fetal brain MRI associated with positive cytomegalovirus infection has increased ADC values in the temporal and parietal lobes, suggestive of brain edema in these areas. However, the association between this finding and neurodevelopment outcome requires further evaluation.

[Standardization and modeling of surgical processes]. / Standardisierung und Modellierung von Operationsprozessen.

BACKGROUND: Due to the technological developments around the operating room, surgery in the twenty-first century is undergoing a paradigm shift. OBJECTIVE: Which technologies have already been integrated into the surgical routine? How can a favorable cost-benefit balance be achieved by the implementation of new software-based assistance systems? METHODS: This article presents the state of the art technology as exemplified by a semi-automated operation system for otorhinolaryngology surgery. The main focus is on systems for implementation of digital handbooks and navigational functions in situ. RESULTS: On the basis of continuous development in digital imaging, decisions may by facilitated by individual patient models thus allowing procedures to be optimized. The ongoing digitization and linking of all relevant information enable a high level of standardization in terms of operating procedures. This may be used by assistance systems as a basis for complete documentation and high process reliability. CONCLUSION: Automation of processes in the operating room results in an increase in quality, precision and standardization so that the effectiveness and efficiency of treatment can be improved; however, care must be taken that detrimental consequences, such as loss of skills and placing too much faith in technology must be avoided by adapted training concepts.

[Evaluation of a Micro-Manipulator for Middle Ear Surgery: A Preclinical Trial]. / Evaluation eines Mikromanipulators für die Mittelohrchirurgie: Eine präklinische Studie.

BACKGROUND: Microsurgical preparation is limited by geometric and mechanical constraints. In preparation for clinical use, this study investigates performance, ease of handling and precision of a novel manipulator concept for microsurgery. MATERIAL AND METHODS: A group of 15 ENT experienced doctors, as well as a group of 17 medical students with low/non surgical experience participated in the study. Each of the subjects carried out 4 trials of simulated surgeries on a phantom with built-in force sensors. The task was to apply a defined force between 1.5 and 2 N using a Fisch micro perforator, 16 cm length, 0.4 mm (Storz) targeting holes with a diameter of 0.5 mm. For comparison, the Fisch micro perforator was moved manually or with the manipulator. RESULTS: Assessing the total number of errors proved a significantly lower error number (p<0.0001) and an improvement of the accuracy of 76% with the manipulator. The time requirement for the procedure with the micro manipulator is on average 2-3 times higher than with manual control (p<0.0001). But it is notable that this time requirement significantly decreases with training (p<0.0001). CONCLUSION: The study shows a significant reduction in the number of errors by using a new manipulator concept compared to the non-augmented human hand in an experimental setup. We observed a significant learning effect when subjects applied the micro manipulator, resulting in reduction of the time requirement while maintaining a constant number of errors.

Outcomes of mismatched and unrelated donor hematopoietic stem cell transplantation in Fanconi anemia conditioned with chemotherapy only.

Fanconi anemia (FA) is a genomic instability syndrome associated with bone marrow failure, myelodysplastic syndrome (MDS), and/or acute myeloid leukemia (AML) requiring hematopoietic stem cell transplantation (HSCT) to restore normal hematopoiesis. Although low-intensity fludarabine-based preparative regimens without radiation confer excellent outcomes in FA HSCTs with HLA-matched sibling donors, outcomes for FA patients with alternative donors are less encouraging, albeit improving. We present our experience with 17 FA patients who completed mismatched related or unrelated donor HSCT using a non-radiation fludarabine-based preparative regimen at Charité University Medicine Berlin. All patients engrafted; however, one patient had unstable chimerism in the setting of multi-viral infections that necessitated a stem cell boost to revert to full donor chimerism. Forty-seven percent of patients developed grade I acute graft-verus-host disease (aGVHD). No grade II-IV aGVHD or chronic graft-versus-host disease of any severity occurred. At a median follow-up of 30 months, 88 % of patients are alive with normal hematopoiesis. Two patients died of infections 4 months post-transplantation. These results demonstrate that short-term outcomes for FA patients with mismatched and unrelated donor HSCTs can be excellent using chemotherapy only conditioning. Viral reactivation, however, was a major treatment-related complication.

Adjuvant chemotherapy for elderly patients with stage I non-small-cell lung cancer ≥4 cm in size: an SEER-Medicare analysis.

BACKGROUND: The role of adjuvant chemotherapy for non-small-cell lung cancer (NSCLC) stage I patients with tumors size ≥4 cm is not well established in the elderly. PATIENTS AND METHODS: We identified 3289 patients with stage I NSCLC (T2N0M0 and tumor size ≥4 cm) who underwent lobectomy from the Surveillance, Epidemiology and End Results (SEER)-Medicare linked database diagnosed from 1992 to 2009. Overall survival and rates of serious adverse events (defined as those requiring admission to hospital) were compared between patients treated with resection alone, platinum-based adjuvant chemotherapy, or postoperative radiation (PORT) with or without adjuvant chemotherapy. Propensity scores for receiving each treatment were calculated and survival analyses were conducted using inverse probability weights based on the propensity score. RESULTS: Overall, 84% patients were treated with resection alone, 9% received platinum-based adjuvant chemotherapy, and 7% underwent PORT with or without adjuvant chemotherapy. Adjusted analysis showed that adjuvant chemotherapy [hazard ratio (HR), 0.82; 95% confidence interval (CI) 0.68-0.98] was associated with improved survival compared with resection alone. Conversely, the use of PORT with or without adjuvant chemotherapy (HR 1.91; 95% CI 1.64-2.23) was associated with worse outcomes. Patients receiving adjuvant chemotherapy had more serious adverse events compared with those treated with resection alone, with neutropenia (odds ratio, 21.2; 95% CI 5.8-76.6) being most significant. No significant difference was observed in rates of fever, cytopenias, nausea, and renal dysfunction. CONCLUSIONS: Platinum-based adjuvant chemotherapy is associated with reduced mortality and increased serious adverse events in elderly patients with stage I NSCLC and tumor size ≥4 cm.

Dynamics of fertility impairment and recovery after allogeneic haematopoietic stem cell transplantation in childhood and adolescence: results from a longitudinal study.

PURPOSE: Fertility impairment and recovery after haematopoietic stem cell transplantation (HSCT) have been reported in both sexes, but little is known about how they develop over time. Our aim was to describe the dynamics of fertility impairment and recovery after HSCT. METHODS: We retrieved treatment and fertility data for up to 12 years of 361 paediatric patients with malignant and non-malignant diseases from seven European centres. The patients had been treated with allogeneic HSCT between 2000 and 2005. RESULTS: Development of fertility impairment was observed in males (123/217, 56%) after a median time of 2.6 years (range 0.1-11.4) and in females (82/144, 57%) after 2.3 years (range 0.1-12.0) after HSCT. Different busulfan dosages had only a slight impact on the onset of fertility impairment (busulfan ≥ 16 mg/kg with a median time to fertility impairment of 2.9 vs. 3.9 years after busulfan <14 mg/kg). Recovery from fertility impairment was observed in 17 participants after a median time of 4.1 years (range 1-10.6) in females (10/144, 7%) and 2.0 years (range 1-6.3) in males (7/217, 3 %) after fertility impairment first appeared. CONCLUSIONS: In the light of the dynamics of fertility impairment and recovery in the HSCT patients reviewed, these patients should be counselled comprehensively regarding fertility preservation measures.

[Therapy of inherited diseases of platelet function. Interdisciplinary S2K guideline of the Permanent Paediatric Committee of the Society of Thrombosis and Haemostasis Research (GTH e. V.)]. / Therapie hereditärer Thrombozytopathien. Interdisziplinäre S2K-Leitlinie der Ständigen Kommission Pädiatrie der Gesellschaft für Thrombose- und Hämostaseforschung e. V.

Inherited disorders of platelet function are a heterogeneous group. For optimal prevention and management of bleeding, classification and diagnosis of the underlying defect are highly recommended. An interdisciplinary guideline for a diagnostic approach has been published (AWMF # 086-003 S2K; Hämostaseologie 2014; 34: 201-212). Underlying platelet disorder, platelet count, age and clinical situation modify treatment. Exclusive transfusion of platelet concentrates may be inappropriate as potentially adverse effects can outweigh its benefit. A stepwise and individually adjusted approach for restitution and maintenance of haemostasis is recommended. Administration of antifibrinolytics is generally endorsed, but is of particular use in Quebec disease. Restricted to older children, desmopressin is favourable in storage pool disease and unclassified platelet disorders. Although licensed only for patients with Glanzmann thrombasthenia and alloantibodies, in clinical practice rFVIIa is widely used in inherited platelet disorders with severe bleeding tendency. This guideline aims at presenting the best available advice for the management of patients with inherited platelet function disorders.

[Diagnosis of inherited diseases of platelet function. Interdisciplinary S2K guideline of the Permanent Paediatric Committee of the Society of Thrombosis and Haemostasis Research (GTH e. V.)]. / Diagnose angeborener Störungen der Thrombozytenfunktion. Interdisziplinäre S2K-Leitlinie der Ständigen Kommission Pädiatrie der Gesellschaft für Thrombose- und Hämostaseforschung e. V.

Congenital disorders of platelet function are a heterogeneous group of disorders that are often not detected until bleeding occurs. In clinical settings only a few methods have proven to be useful for identification and classification of inherited platelet disorders. For a rational diagnostic approach, a stepwise algorithm is recommended. Patient history and clinical investigation are mandatory. Von Willebrand disease and other coagulation disorders should always be ruled out prior to specific platelet testing. Platelet count, size, volume (MPV) and morphology may guide further investigations. The PFA-100® CT is suited for screening for severe platelet defects. Platelet aggregometry allows assessment of multiple aspects of platelet function. Flow cytometry enables diagnosis of thrombasthenia Glanzmann, Bernard-Soulier syndrome and storage pool defects. Molecular genetics may confirm a putative diagnosis or pave the way for identifying new defects. We present an unabridged version of the interdisciplinary guideline.

[Effect of a multifunctional instrument (HF scissors) in parotis surgery]. / Effekte eines multifunktionalen Instruments (HF-Schere) in der Parotischirurgie.

PROBLEM: Lateral parotidectomy is a demanding surgical procedure and requires a large number of instruments with a high frequency (HF) of alternating. Many functions, such as preparing, spreading, coagulating and cutting could be combined by using scissors with an integrated function of bipolar coagulation. This study has targeted an investigation of technical application, influence on surgery time, frequency of HF application and change of instruments. MATERIAL AND METHODS: In the period between 01 April 2011 and 30 September 2012 (18 months) 35 procedures of lateral parotidectomy in 35 patients were investigated. In all cases lateral parotidectomy was carried out with a similar technique (modified extracapsular preparation). Workflow data were used from a control group in the period between 01 January 2009 and 31 December 2010 (24 months). The following parameters from both groups were documented and evaluated: incision-suture times (subdivided into nine sections of the procedure), change of instruments, period of application for HF function and early facial nerve function (6 h and 6 days after surgery according to House-Brackmann). Additionally, in the HF+ group a questionnaire that facilitated subjective evaluation of instruments was analyzed. RESULTS: It was possible to perform the surgery in both groups with neither technical nor surgical intraoperative complications. Incision-suture times showed an average reduction of 31.6 min (34.8 %) in the HF+ group. There was a reduction in the change of instruments compared to a conventional group (CONV) by up to 62.7 %. With the deployment of HF scissors there was a clear increase in the use of HF surgery by more than 100 % when comparing incision-suture times. Evaluation of both groups according to House-Brackmann showed a similar postoperative facial nerve function. The surgeons involved were of the opinion that in all 35 surgeries with HF scissors the intervention was easier and more comfortable. DISCUSSION: The use of HF scissors is appropriate for parotid gland surgery and can be used in all steps of the procedure. Incision-suture time can be reduced and the ergonomics of the intervention can be improved. Up to now no negative effects have been found in the quality of the results.

Impact of navigated-control assistance on performance, workload and situation awareness of experienced surgeons performing a simulated mastoidectomy.

BACKGROUND: Navigated control (NC) is an advanced image-guided navigation system that provides an additional control function to enhance patient safety. It automatically stops the surgical instrument if it comes close to critical anatomical structures that need to be protected during surgery. The purpose of this study was to explore the human performance consequences of computer-based navigated control assistance. METHODS: Seven experienced surgeons conducted a simulated mastoidectomy manually and with support of the NC system. The impact on surgical performance, workload and situation awareness was analysed. RESULTS: NC support led to a better quality of surgical outcome and a lower level of physiological effort during surgery. Cost effects were reflected in reduced time efficiency and an increased subjectively experienced workload. CONCLUSION: The results demonstrate the potential of NC support in terms of lower workload and enhanced patient safety. Cost effects might be reduced by remodelling the control function.

[Instrument navigation in ensonasal sinus surgery]. / Instrumentennavigation in der endonasalen Stirnhöhlenchirurgie: Gibt es eine Operation unter "reiner Navigationsführung"?

BACKGROUND: It is to be expected that, microsurgical operations in ENT must be partially performed without direct vision, e. g. without the possibility of controlling the preparation progress. This study provides a clinical example of how instrument navigation may be used in a safe and reasonable manner to the benefit of treatment quality. It is assumed that a median frontal sinus drainage procedure may be performed by means of a tracked sinus drill using solely instrument navigation. MATERIAL AND METHODS: The sinus drill was registered using an optoelectric navigation system. An optical registration device was attached to the drill. In the period from 1 December 2011 to 30 April 2012 (5 months) 24 patients underwent a median frontal sinus drainage. 12 of the surgeries were performed under the condition operation navigation. The other 12 surgeries were performed using solely instrument navigation. RESULTS: When applying mode Instrument Navigation (IN), a significantly reduced incision-to-suture time was registered within both groups. Within the group Draf IIB, it was reduced to 68.5% (from 67.1 min to 46.0 min). Resection efficiency, i. e., the ratio between the width of the frontal sinus ostium and the required total surgery time, widely differed to the benefit of group IN. Within both groups, the questionnaire revealed a high level of confidence in Surgical Management and Guidance Systems (SMGS) functionalities (100%). CONCLUSION: The present study was designed to show for the first time a median frontal sinus drainage under the condition solely instrument navigation.

[Interdisciplinary teleconsultation: first practical experiences with 100 patients]. / Interdisziplinäre Telekonsultation: Erste praktische Erfahrungen mit 100 Patienten.

UNLABELLED: PROBLEM DEFINITION: Increasing specialization can be observed in the various medical fields and as a consequence there is little professional exchange between ear nose and throat (ENT) specialists and general practitioners. At the same time there has been significant technological development in telemedicine over the last 5 years; however, this potential is not being sufficiently exploited. The objective of this project is to implement a practicable solution for teleconsulation between ENT specialists and general practitioners. MATERIALS AND METHODS: The connection is based on a secure broadband internet connection, the corresponding server structure and a video endoscopic system. In the period from 01 January 2011 to 30 June 2012 (18 months) a total of 102 patients were included in the study in which 4 general practitioners and 5 ENT specialists participated. The protocol comprised the following parameters: indications and typical questions, number of patients presenting to the ENT specialist due to the available teleconsultation service (CBO), number of patients referred to the ENT specialist after teleconsultation (CIO), assessment of the quality of the additional expert opinion (LOQ GP), assessment of the accuracy of the specialist physician tele-diagnosis (TDS-FA) and assessment of the impact of the expert physician diagnosis on the general medical treatment (COS). RESULTS: Teleconsultation was most frequently used for diagnostics on tonsils (37% of the presented cases), the external auditory canal (32%) and the inner nose (15%). Of the patients analyzed were 53.9% presented to the ENT specialist only because the teleconsultation service provided an effortless opportunity and after teleconsultation 40.1% of the patients were referred to the ENT specialist. General practitioners assessed the benefit from the specialist opinion with 64 points. Diagnostic certainty of the specialist opinion, i.e. the validity scale of the diagnosis made, was on average 2.0. In approximately 35.3% of the cases (36 out of 102 patients) participating general practitioners documented a considerable impact of teleconsultation on the diagnosis and/or therapy. CONCLUSIONS: Teleconsultation between general practitioners and ENT specialists can provide an advantage in treatment quality and patient comfort. According to the experience gained there is a very low risk of diminishing the professional competency of ENT medicine and the involvement of the expert group in the early project stage allows a greater leeway in project implementation. This could also have an impact on future medical performance specifications. According to the ENT experts involved in the project further applications of teleconsultation are very conceivable.