Liszaj śluzowaty z towarzyszącym zespołem Sjögrena

Lichen myxedematosus belongs to the group of disorders with the deposition of mucins in the skin. The disease may coexist with monoclonal gammapathy, e.g. myeloma multiplex or Waldenström macroglobulinemia. A case of a 77-year-old man with lichen myxedematosus and monoclonal gammapathy is presented. Treatment with cyclophosphamide followed by intravenous immunoglobulins was initiated, however, no significant improvement of the skin condition was observed. Currently, the patient is receiving talidomide. This case report indicates, that lichen myxedematosus still remains a challenge for physicians and causes both diagnostics and therapeutic dilemma.Liszaj śluzowaty należy do grupy schorzeń związanych z odkładaniem się złogów mucyn w skórze. Choroba ta może dodatkowo współistnieć z gammapatią monoklonalną, na przykład szpiczakiem mnogim lub makroglobulinemią Waldenströma. W artykule został omówiony przypadek 77-letniego mężczyzny z liszajem śluzowatym i monoklonalną gammapatią. W leczeniu zmian skórnych zastosowano u niego w pierwszej kolejności cyklofosfamid, a następnie ludzkie immunoglobuliny G. Próby te nie przyniosły jednak zadowalającej poprawy, stąd pacjent został obecnie poddany terapii talidomidem. Opisywany przypadek wskazuje, że liszaj śluzowaty wciąż pozostaje wyzwaniem dla lekarzy i stwarza problemy zarówno diagnostyczne (brak jasnych kryteriów klasyfikacyjnych), jak i terapeutyczne

Taxonomy and conservation of grassland earless dragons:New species and an assessment of the first possible extinction of a reptile on mainland Australia

Taxonomic research is of fundamental importance in conservation management of threatened species, providing an understanding of species diversity on which management plans are based. The grassland earless dragon lizards (Agamidae: <i>Tympanocryptis</i>) of south-eastern Australia have long been of conservation concern but there have been ongoing taxonomic uncertainties. We provide a comprehensive taxonomic review of this group, integrating multiple lines of evidence, including phylogeography (mtDNA), phylogenomics (SNPs), external morphology and micro x-ray CT scans. Based on these data we assign the lectotype of <i>T. lineata</i> to the Canberra region, restrict the distribution of <i>T. pinguicolla</i> to Victoria and name two new species: <i>T. osbornei sp. nov.</i> (Cooma) and <i>T. mccartneyi sp. nov.</i> (Bathurst). Our results have significant conservation implications. Of particular concern is <i>T. pinguicolla</i>, with the last confident sighting in 1969, raising the possibility of the first extinction of a reptile on mainland Australia. However, our results are equivocal as to whether <i>T. pinguicolla</i> is extant or extinct, emphasizing the immediate imperative for continued surveys to locate any remaining populations of <i>T. pinguicolla</i>. We also highlight the need for a full revision of conservation management plans for all the grassland earless dragons

Comparing encoding mechanisms in realistic virtual reality and conventional 2D laboratory settings: Event-related potentials in a repetition suppression paradigm

Although the human brain is adapted to function within three-dimensional environments, conventional laboratory research commonly investigates cognitive mechanisms in a reductionist approach using two-dimensional stimuli. However, findings regarding mnemonic processes indicate that realistic experiences in Virtual Reality (VR) are stored in richer and more intertwined engrams than those obtained from the conventional laboratory. Our study aimed to further investigate the generalizability of laboratory findings and to differentiate whether the processes underlying memory formation differ between VR and the conventional laboratory already in early encoding stages. Therefore, we investigated the Repetition Suppression (RS) effect as a correlate of the earliest instance of mnemonic processes under conventional laboratory conditions and in a realistic virtual environment. Analyses of event-related potentials (ERPs) indicate that the ERP deflections at several electrode clusters were lower in VR compared to the PC condition. These results indicate an optimized distribution of cognitive resources in realistic contexts. The typical RS effect was replicated under both conditions at most electrode clusters for a late time window. Additionally, a specific RS effect was found in VR at anterior electrodes for a later time window, indicating more extensive encoding processes in VR compared to the laboratory. Specifically, electrotomographic results (VARETA) indicate multimodal integration involving a broad cortical network and higher cognitive processes during the encoding of realistic objects. Our data suggest that object perception under realistic conditions, in contrast to the conventional laboratory, requires multisensory integration involving an interconnected functional system, facilitating the formation of intertwined memory traces in realistic environments

Information needs after stroke: What to include and how to structure it on a website. A qualitative study using focus groups and card sorting

Background: Use of the Internet to obtain health and other information is increasing. Previous studies have identified the specific information needs of people with stroke but not in relation to the Internet. People with aphasia (PwA) may face barriers in accessing the Internet: Navigating websites requires an ability to categorise information and this ability is often impaired in PwA. The website categorisation preferences of people with stroke and with aphasia have not yet been reported. Aims: This study aimed: (a) to determine what information people who have had a stroke would like to see on a website about living with stroke; (b) to determine the most effective means of structuring information on the website so that it is accessible to people with stroke; and c) to identify any differences between people with and without aphasia in terms of preferences for structuring information on the website. Methods & Procedures: Participants were recruited from a hospital's Stroke Database. Focus groups were used to elicit what information participants wanted on a website about living with stroke. The themes raised were depicted on 133 cards. To determine the most effective way of structuring information on the website, and whether there were any differences in preferences between PwA and PwoA, participants used a modified closed card-sorting technique to sort the cards under website categories. Outcomes & Results: A total of 48 people were invited, and 12 (25%) agreed to take part. We ran three focus groups: one with PwA (n = 5) and two with people without aphasia (PwoA) (n = 3, n = 4). Participants wanted more information about stroke causes and effects (particularly emotional issues), roles of local agencies, and returning to previous activities (driving, going out). All participants completed the card-sorting exercise. Few cards (6%) were categorised identically by everyone. Cards relating to local agencies and groups were not consistently categorised together. Cards relating to emotions were segregated. The categorisation preferences for PwA were more fragmented than those for PwoA: 60% of PwA agreed on the categorisation of 51% of the cards, whereas 60% of PwoA agreed on the categorisation of 76% of the cards. Conclusions: Information needs covered all stages of the stroke journey. The card sorting was accessible to everyone, and provided evidence of structuring preferences and of some of the categorisation difficulties faced by PwA. More research is needed on what an accessible website looks like for PwA

Mechanical Strength of 17 134 Model Proteins and Cysteine Slipknots

A new theoretical survey of proteins' resistance to constant speed stretching is performed for a set of 17 134 proteins as described by a structure-based model. The proteins selected have no gaps in their structure determination and consist of no more than 250 amino acids. Our previous studies have dealt with 7510 proteins of no more than 150 amino acids. The proteins are ranked according to the strength of the resistance. Most of the predicted top-strength proteins have not yet been studied experimentally. Architectures and folds which are likely to yield large forces are identified. New types of potent force clamps are discovered. They involve disulphide bridges and, in particular, cysteine slipknots. An effective energy parameter of the model is estimated by comparing the theoretical data on characteristic forces to the corresponding experimental values combined with an extrapolation of the theoretical data to the experimental pulling speeds. These studies provide guidance for future experiments on single molecule manipulation and should lead to selection of proteins for applications. A new class of proteins, involving cystein slipknots, is identified as one that is expected to lead to the strongest force clamps known. This class is characterized through molecular dynamics simulations.Comment: 40 pages, 13 PostScript figure

International comparison of cosmetic outcomes of breast conserving surgery and radiation therapy for women with ductal carcinoma in situ of the breast

Purpose: To assess the cosmetic impact of breast conserving surgery (BCS), whole breast irradiation (WBI) fractionation and tumour bed boost (TBB) use in a phase III trial for women with ductal carcinoma in situ (DCIS) of the breast. Materials and methods: Baseline and 3-year cosmesis were assessed using the European Organization for Research and Treatment of Cancer (EORTC) Cosmetic Rating System and digital images in a randomised trial of non-low risk DCIS treated with postoperative WBI +/- TBB. Baseline cosmesis was assessed for four geographic clusters of treating centres. Cosmetic failure was a global score of fair or poor. Cosmetic deterioration was a score change from excellent or good at baseline to fair or poor at three years. Odds ratios for cosmetic deterioration by WBI dose-fractionation and TBB use were calculated for both scoring systems. Results: 1608 women were enrolled from 11 countries between 2007 and 2014. 85-90% had excellent or good baseline cosmesis independent of geography or assessment method. TBB (16 Gy in 8 fractions) was associated with a >2-fold risk of cosmetic deterioration (p

Characteristics of Stem Cells Derived from the Degenerated Human Intervertebral Disc Cartilage Endplate

Mesenchymal stem cells (MSCs) derived from adult tissues are an important candidate for cell-based therapies and regenerative medicine due to their multipotential differentiation capability. MSCs have been identified in many adult tissues but have not reported in the human intervertebral disc cartilage endplate (CEP). The initial purpose of this study was to determine whether MSCs exist in the degenerated human CEP. Next, the morphology, proliferation capacity, cell cycle, cell surface epitope profile and differentiation capacity of these CEP-derived stem cells (CESCs) were compared with bone-marrow MSCs (BM-MSCs). Lastly, whether CESCs are a suitable candidate for BM-MSCs was evaluated. Isolated cells from degenerated human CEP were seeded in an agarose suspension culture system to screen the proliferative cell clusters. Cell clusters were chosen and expanded in vitro and were compared with BM-MSCs derived from the same patient. The morphology, proliferation rate, cell cycle, immunophenotype and stem cell gene expression of the CESCs were similar to BM-MSCs. In addition, the CESCs could be induced into osteoblasts, adipocytes, chondrocytes, and are superior to BM-MSCs in terms of osteogenesis and chondrogenesis. This study is first to demonstrate the presence of stem cells in the human degenerated CEP. These results may improve our understanding of intervertebral disc (IVD) pathophysiology and the degeneration process, and could provide cell candidates for cell-based regenerative medicine and tissue engineering

Human subcortical brain asymmetries in 15,847 people worldwide reveal effects of age and sex

The two hemispheres of the human brain differ functionally and structurally. Despite over a century of research, the extent to which brain asymmetry is influenced by sex, handedness, age, and genetic factors is still controversial. Here we present the largest ever analysis of subcortical brain asymmetries, in a harmonized multi-site study using meta-analysis methods. Volumetric asymmetry of seven subcortical structures was assessed in 15,847 MRI scans from 52 datasets worldwide. There were sex differences in the asymmetry of the globus pallidus and putamen. Heritability estimates, derived from 1170 subjects belonging to 71 extended pedigrees, revealed that additive genetic factors influenced the asymmetry of these two structures and that of the hippocampus and thalamus. Handedness had no detectable effect on subcortical asymmetries, even in this unprecedented sample size, but the asymmetry of the putamen varied with age. Genetic drivers of asymmetry in the hippocampus, thalamus and basal ganglia may affect variability in human cognition, including susceptibility to psychiatric disorders