A 13-year patient journey of infant giant clival chordoma: case report and literature review.

Chordomas are rare malignant bone tumours that develop from the ectopic remnants of the embryonic notochord. In contrast to adults, the majority in children under 16 present intra-cranially (63%). In 2006, we reported the youngest case of a large clival chordoma, a 15-week old baby, the second case to present without skull base involvement and the fourth case of chordoma in a patient with tuberous sclerosis (TS) Kombogiorgas (Childs Nerv Syst 22(10):1369-1374, 2006). In this report, we provide an update on this patient's journey through a range of therapeutic options and summarize an update of the literature, since 2006, for this patient group.

Is transportation a risk factor for African swine fever transmission in Australia: a review.

African swine fever (ASF) is a viral disease of the pigs that was first described in Africa during the early part of the twentieth century. The disease has periodically occurred outside of Africa, including an ongoing epidemic in Europe and Asia that started in 2007; the disease has never occurred in Australia or New Zealand. Once introduced into a country, spread can occur through direct and indirect routes of transmission. Infected feral pig populations have the potential to act as a long-term reservoir for the virus, making eradication difficult. Just before and throughout the period of clinical signs, ASF virus is shed in oronasal fluids, urine, faeces and blood. This results in contamination of the pig's environment, including flooring, equipment and vehicles. Transportation-related risk factors therefore are likely to play an important role in ASF spread, though evidence thus far has been largely anecdotal. In addition to the existing AUSVETPLAN ASF plan, efforts should be made to improve transportation biosecurity, from the time a pig leaves the farm to its destination. Collection of data that could quantify the capabilities and capacity of Australia to clean and disinfect livestock trucks would help to determine if private and/or public sector investment should be made in this area of biosecurity. No peer-reviewed research was identified that described a specific process for cleaning and disinfecting a livestock truck known to be contaminated with ASF virus, though literature suggests that transportation is an important route of transmission for moving the virus between farms and countries.

African swine fever: a New Zealand perspective on epidemiological risk factors for its occurrence.

This article reviews key epidemiological and clinical features of African swine fever (ASF). We identify particular aspects of New Zealand's pig populations (commercial, non-commercial, and wild) that may affect the risk of disease entry or spread. Review of published literature is supplemented by analysis of demographic and spatial aspects of the New Zealand commercial, non-commercial, and feral pig populations to provide context around risk factors for the disease that are most relevant to New Zealand. The current Eurasian outbreak of ASF, including recent spread into Oceania, has increased the risk of an incursion of the disease into New Zealand. Large volumes of fresh pork importation (including from countries affected by ASF), large non-commercial pig populations with substantial spatial overlap with the country's commercial industry, limited monitoring of compliance with waste food feeding regulations, and lack of mandatory premises identification for non-commercial pig holdings would likely contribute to the risk of spread of ASF in the event of an incursion. Awareness amongst veterinarians of these risk factors will contribute to national biosecurity and disease preparedness efforts in New Zealand.

Oral cavity colon adenocarcinoma metastases: case report with surgical approach and review of more than 30 years literature.

INTRODUCTION: Metastatic oral tumours are rare, contributing to 1% of all malignant oral cavity tumours. CASE REPORT: We report the case of a 59-year-old man with colon cancer at an advanced disease stage, with progression to the peritoneum and maxillary gingiva. Palliative surgery was indicated to improve the patient's quality of life. DISCUSSION: In a review of the literature, we compiled a list of 27 cases (including the present case) reflecting some 30 years of literature on oral cavity metastatic disease originating in colon cancer. CONCLUSION: Oral cavity metastasis should be taken into account in the differential diagnosis of a synchronous or metachronous oral cavity lesion. The therapeutic goal should include palliative alternatives when necessary. Intraoral reconstruction using local flaps may be a simple and reliable palliative resection option aimed at improving the patient's quality of life.

Probiotic effect of Bifidobacterium longum 51A and Weissella paramesenteroides WpK4 on gerbils infected with Giardia lamblia.

AIMS: The objective of this study was to assess the probiotic potential of genuine strains of Bifidobacterium longum 51A and Weissella paramesenteroides WpK4, in experimental giardiasis. METHODS AND RESULTS: The bacteria were administered orally to gerbils (Meriones unguiculatus) 10 days before oral infection with trophozoites of Giardia lamblia. After 7 days of infection, the animals were euthanized and portions of the duodenum were processed for histopathologic, histochemical and morphometric assessment. The height of the intestinal crypts and crypt/villi ratio were higher in infected groups (P < 0·05) than in noninfected groups. The area of mucus production was higher (P < 0·05) in infected animals pretreated with B. longum 51A than in other groups. The parasitic load of the animals that received both bacteria decreased significantly (P < 0·05) compared to the ones of the control group. CONCLUSIONS: Our results suggest a probiotic function of B. longum 51A and W. paramesenteroides WpK4 and may result in their use as a prophylactic and therapeutic alternative for promoting human and animal health. SIGNIFICANCE AND IMPACT OF THE STUDY: Bifidobacterium longum 51A and W. paramesenteroides WpK4 may constitute prophylactic alternatives, reversing the emergence of side effects and resistance observed in the conventional treatment of giardiasis.

[Rheumatoid arthritis]. / Rheumatoide Arthritis.

Rheumatoid arthritis (RA) is a chronic and progressive systemic disease of the connective tissue, which is particularly manifested with destructive alterations to the joints. Inflammatory reactions in the synovium lead to the influx of peripheral inflammatory cells as well as the activation of local cells. Released growth factors, chemokines and especially cytokines play a key role in chronic inflammatory responses. In addition to the central lymphocytes, the T and B cells and their subpopulations, locally resident cells, such as neutrophils, macrophages and fibroblasts as well as cells of bone metabolism are activated by the inflammatory milieu and contribute to and drive inflammation and tissue damage. The destruction of cartilage and bone substance by local tissue cells, synovial fibroblasts and osteoclasts is characteristic for this disease. Untreated, the local inflammatory and destructive processes as well as systemic inflammatory factors lead to progressive and irreversible joint destruction. Cellular and immunological processes in RA are closely interwoven; therefore, besides the general inhibition of immunological processes, specific inhibition of central key molecules can reduce or completely stop the inflammatory destructive processes; however, a high heterogeneity can be observed among RA patients and disease progression. Therefore, an expansion of the therapeutic options is desirable as not all patients are able to equally benefit from the therapeutic treatment. It is important to characterize new molecular mechanisms, which could lead to the development of new therapeutic options. Some of the more recent insights are summarized in this overview.

Visfatin alters the cytokine and matrix-degrading enzyme profile during osteogenic and adipogenic MSC differentiation.

OBJECTIVES: Age-related bone loss is associated with bone marrow adiposity. Adipokines (e.g., visfatin, resistin, leptin) are adipocyte-derived factors with immunomodulatory properties and might influence differentiation of bone marrow-derived mesenchymal stem cells (MSC) in osteoarthritis (OA) and osteoporosis (OP). Thus, the presence of adipokines and MMPs in bone marrow and their effects on MSC differentiation were analyzed. METHODS: MSC and ribonucleic acid (RNA) were isolated from femoral heads after hip replacement surgery of OA or osteoporotic femoral neck fracture (FF) patients. Bone structural parameters were evaluated by microcomputed tomography (µCT). MSC were differentiated towards adipocytes or osteoblasts with/without adipokines. Gene expression (adipokines, bone marker genes, MMPs, TIMPs) and cytokine production was evaluated by realtime-polymerase chain reaction (realtime-PCR) and enzyme-linked immunosorbent assay (ELISA). Matrix mineralization was quantified using Alizarin red S staining. RESULTS: µCT showed an osteoporotic phenotype of FF compared to OA bone (reduced trabecular thickness and increased ratio of bone surface vs volume of solid bone). Visfatin and leptin were increased in FF vs OA. Visfatin induced the secretion of IL-6, IL-8, and MCP-1 during osteogenic and adipogenic differentiation. In contrast to resistin and leptin, visfatin increased MMP2 and MMP13 during adipogenesis. In osteogenically differentiated cells, MMPs and TIMPs were reduced by visfatin. Visfatin significantly increased matrix mineralization during osteogenesis, whereas collagen type I expression was reduced. CONCLUSION: Visfatin-mediated increase of matrix mineralization and reduced collagen type I expression could contribute to bone fragility. Visfatin is involved in impaired bone remodeling at the adipose tissue/bone interface through induction of proinflammatory factors and dysregulated MMP/TIMP balance during MSC differentiation.

No influence of smoking status on the performance of urine markers for the detection of bladder cancer.

PURPOSE: The performance of urinary markers for detecting bladder cancer (BC) is influenced by various factors. The aim of the present study was to evaluate the influence of smoking habits on the performance of four commonly used urine markers. METHODS: Urine samples of 723 patients with suspected BC were analysed using urine cytology, fluorescence in situ hybridization (FISH), immunocytology (uCyt+ test), and quantitative nuclear matrix protein 22 (NMP22) immunoassay. The smoking habits of all patients were recorded and a cystoscopy performed within 2 weeks after urinary marker testing. Rates of false negative and false positive results were compared between non-smokers, former smokers, and current smokers by contingency analyses. RESULTS: We included 723 patients in this study, 431 (59.6%) of which were non-smokers, 215 former smokers (29.7%), and 77 (10.7%) current smokers. 148 patients (20.5%) had a tumour at the time of urinary marker testing. Respective rates of false positive test results among non-smokers, former smokers, and current smokers were: 16.3, 19.1, and 11.5% (p = 0.81) for urine cytology; 36.8, 42.0, and 32.7% for the uCyt+ test (p = 0.88); 18.0, 19.1, and 13.5% for FISH (p = 0.66); and 69.5, 71.6, and 71.2% for NMP22 (p = 0.67). Respective rates of false negatives among non-smokers, former smokers, and current smokers were: 31.4, 15.1, and 28.0% for cytology (p = 0.34); 21.4, 22.6, and 16.0% for uCyt+ test (p = 0.67); 24.3, 13.2, and 28.0% for FISH (p = 0.88); and 10.0, 18.9, and 8.0% for NMP22 (p = 0.80). CONCLUSIONS: Our results strongly suggest that smoking habits do not affect performance characteristics of urinary markers in the diagnostics of BC.

Ultrastructure aspects of Brycon gouldingi (Teleostei, Characidae) related to swimming ability and feeding during larval development.

The larval ultrastructure of Brycon gouldingi related to swimming and feeding from hatching to total yolk absorption is described from scanning electron micrographs. Newly hatched larvae (time zero) had no mouth opening, undefined optic vesicles, an olfactory plate visible as a shallow depression, rudimentary gill arches, neural groove, embryonic fin and a primary neuromast in the dorsal region of the head. At the time of yolk absorption, 55 h post hatching, the larvae presented an optic vesicle comprising an optic cup and crystalline lens; a mouth with tongue, tapered teeth and taste buds; a ciliated olfactory cavity; branched gill arches; filled neural groove signalling central nervous system development; caudal, pectoral, dorsal and anal fins; and neuromasts distributed throughout the head and body. These characters are related to prey capture and swimming ability, key aspects of survival during the larval stage. The results of this study provide important information for exploitation and aquaculture of B. gouldingi.

Selection of a candidate probiotic strain of Pediococcus pentosaceus from the faecal microbiota of horses by in vitro testing and health claims in a mouse model of Salmonella infection.

AIMS: The aim of this study was to verify the suitable use of candidate 'probiotics' selected by in vitro tests and the importance of in vivo assays to nominate micro-organisms as probiotics and alternative prophylactic treatments for Salmonella Typhimurium infection. METHODS AND RESULTS: Thirty-three lactic acid bacteria (LAB) isolated from foal's faeces were assessed based on the main desirable functional in vitro criteria. Based on these results, Pediococcus pentosaceus strain 40 was chosen to evaluate its putative probiotic features in a mouse model of Salmonella infection. Daily intragastric doses of Ped. pentosaceus 40 for 10 days before and 10 days after Salmonella challenge (106 CFU of Salm. Typhimurium per mouse) led to a significant aggravation in mouse health by increasing weight loss, worsening clinical symptoms and anticipating the time and the number of deaths by Salmonella. Pediococcus pentosaceus modulated cell-mediated immune responses by up-regulation of the gene expression of the proinflammatory cytokines IFN-γ and TNF-α in the small intestine. CONCLUSION: The usual criteria were used for in vitro screening of a large number of LAB for desirable probiotic functional properties. However, the best candidate probiotic strain identified, Ped. pentosaceus #40, aggravated the experimental disease in mice. SIGNIFICANCE AND IMPACT OF THE STUDY: These findings emphasize the need for prophylactic or therapeutic effectiveness to be demonstrated in in vivo models to make precise health claims.

[Inflammation and bone : Osteoimmunological aspects]. / Entzündung und Knochen : Osteoimmunologische Aspekte.

Microscopic fractures (so-called microcracks) or traumatic macrofractures require bone, as the basic scaffold of the human body, to have a high regenerative capability. In order to be able to provide this regenerative capability, bone is in a constant process of remodeling. This finely tuned homeostasis of bone formation and degradation can become disrupted, which leads to osteoporosis or other bone disorders. It has been shown that the immune system is substantially involved in the regulation of bone homeostasis and that chronic inflammation in particular can disturb this balance; therefore, this article reviews the osteoimmunological aspects contributing to osteoporosis and other diseases associated with bone degradation.

Ultra-thin resin embedding method for scanning electron microscopy of individual cells on high and low aspect ratio 3D nanostructures.

The preparation of biological cells for either scanning or transmission electron microscopy requires a complex process of fixation, dehydration and drying. Critical point drying is commonly used for samples investigated with a scanning electron beam, whereas resin-infiltration is typically used for transmission electron microscopy. Critical point drying may cause cracks at the cellular surface and a sponge-like morphology of nondistinguishable intracellular compartments. Resin-infiltrated biological samples result in a solid block of resin, which can be further processed by mechanical sectioning, however that does not allow a top view examination of small cell-cell and cell-surface contacts. Here, we propose a method for removing resin excess on biological samples before effective polymerization. In this way the cells result to be embedded in an ultra-thin layer of epoxy resin. This novel method highlights in contrast to standard methods the imaging of individual cells not only on nanostructured planar surfaces but also on topologically challenging substrates with high aspect ratio three-dimensional features by scanning electron microscopy.

Weissella paramesenteroides WpK4 reduces gene expression of intestinal cytokines, and hepatic and splenic injuries in a murine model of typhoid fever.

Diarrhoea in piglets by Salmonella and other pathogens can be a serious health problem. Non-drug treatments such as probiotic microorganisms have various effects on the gastrointestinal microbiota dysbiosis and host immune system modulation. The aim of this study was to demonstrate the suitable use of Weissella paramesenteroides WpK4 strain isolated from healthy piglets as an alternative prophylactic or therapeutic treatment against Salmonella Typhimurium. Out of 37 lactic acid bacteria isolates, 24 strains belonging to the Weissella and Lactobacillus genera were analysed in vitro for desirable probiotic characteristics. The W. paramesenteroides WpK4 strain fulfilled all in vitro tests: resistance to acidic pH and bile salts, hydrophobic cell surface, antagonism against bacterial pathogens, H2O2 production and exopolysaccharide secretion, and non-transferable resistance to antibiotics. Mice fed with WpK4 showed no signs of bacterial translocation to the liver or spleen and decreased Salmonella translocation to these organs. Significantly, WpK4 intake attenuated the weight loss, fostered the preservation of intestinal architecture and integrity, and promoted survival in mice following infection with Salmonella Typhimurium. In addition, WpK4 modulated immune cellular response by inhibiting the production of pro-inflammatory cytokines and inducing anti-inflammatory mediators. These findings validate the probiotic properties of W. paramesenteroides WpK4 strain, and its eventual use in piglets.

Bronchial compression following pulmonary artery stenting in single ventricle lesions: how to prevent, and how to decompress.

OBJECTIVES: To assess airway compression during pulmonary artery (PA) intervention in single ventricle (SV) palliation. BACKGROUND: SV lesions with a prominent neo-aortic root are considered a high risk for branch PA and/or bronchial stenosis. PA stenting is well established, but may result in ipsilateral bronchial compression. METHODS: Single-centre retrospective analysis of 19 palliated SV patients with branch PA stenosis and close proximity to the ipsilateral main bronchus who underwent cardiac catheterisation at a median age and weight of 8.5 years (0.5-25) and 16.5 kg (6-82) between 12/2011 and 05/2015. RESULTS: Two of the 19 patients suffered an almost-closed left-main bronchus (LMB) following PA stenting. Fortunately, LMB decompression succeeded in both those patients by re-shaping the PA stents by compressing the chest while splinting the LMB with an inflated balloon. To prevent the other 17 patients from suffering this serious complication, we adopted a thorough preparation strategy: 13 patients underwent safe simultaneous bronchoscopy and cardiac catheterisation; in the remaining 4 patients CT-angiography enabled accurate risk evaluation prior to re-catheterisation. CONCLUSIONS: In SV lesions accompanied by branch PA stenosis, thorough preparation via cross-sectional imaging is mandatory, including simultaneous bronchoscopy and cardiac catheterisation in selected cases, to rule out any airway compression before considering endovascular stent implantation. If a PA stent's compression has already caused severe bronchial obstruction, our balloon-splinted decompression technique should be considered.

Morphological and morphometric aspects of early life stages of piabanha Brycon gouldingi (Characidae).

Adult specimens of piabanha Brycon gouldingi were collected from Rio das Mortes (Mato Grosso, Brazil), adapted to captivity and induced to spawn at Buriti Fisheries (Nova Mutum, MT, Brazil). The early developmental stages of B. gouldingi were then characterized. Samples were collected at pre-determined times from oocyte extrusion to total yolk absorption. Oocyte diameter, total larval length (LT ) and yolk-sac volume were measured. The mean ± s.d. duration of embryo developmental of B. gouldingi was 13·90 ± 0·06 h at 26·40 ± 1·13° C. The mean ± s.d. oocyte diameter was 1·13 ± 0·06 mm with 54% of oocytes ranging from 1·11 to 1·20 mm. Seven stages characterized the early developmental phase of this species: zygote, cleavage, morula, blastula, gastrula, histogenesis-organogenesis and hatching, with unique features related to each stage. At hatching, the larvae measured 3·40 ± 0·07 mm, presented an elongated shape with yolk-sac volume of 0·46 ± 0·08 µl, non-pigmented eyes and exhibited swimming ability. When the yolk was completely absorbed at 55 h post-hatch, mean ± larval LT was 6·68 ± 0·65 mm, the eyes were highly pigmented and the teeth were visible. These are the first reported findings on the initial developmental stages of B. gouldingi and could be used to improve captive breeding management and conservation practices.

[Fibroblasts as pathogenic cells in rheumatic inflammation]. / Fibroblasten als pathogene Zellen in der rheumatischen Entzündung.

Rheumatoid arthritis (RA) is an autoimmune disease characterized by synovitis, synovial hyperplasia and progressive degeneration of affected joints. These processes are mediated by cells of the immune system as well as by synovial fibroblasts (RASF) originating from the lining layer of the synovium. In this scenario RASFs display an activated phenotype: they show an altered expression of adhesion molecules which allows attachment to articular cartilage and by synthesis of proteases they mediate progressive cartilage and bone destruction. Furthermore, they produce various cytokines and chemokines, which are essential for promoting the inflammatory response. In recent years it has become evident that RASFs not only passively respond to the proinflammatory milieu in the joints of RA patients but also actively contribute by the overproduction of several cytokines and chemokines. These proinflammatory cytokines trigger the transformation of RASFs into an aggressive and invasive phenotype. Additionally, the primarily altered genuine RASFs are actively involved in the recruitment and activation of immune cells. Taken together, they are key players in the development of the well-known chronic, destructive inflammatory response in joints affected by RA.

Role of synovial fibroblasts in rheumatoid arthritis.

Rheumatoid arthritis (RA) is the most common autoimmune articular disorder. It is characterized by chronic inflammation and progressive joint destruction. As research traditionally focused on immune cells and cytokines, the role of stromal cells was addressed only to a limited extent. However, cell-cell interactions within the rheumatoid synovium alter the phenotype of synovial fibroblasts (SFs), which are nowadays considered as active and aggressive drivers in the destructive process of RA. SFs actively attach to and invade articular cartilage, thereby expressing increased amounts of adhesion molecules and proinflammatory and matrix-degrading mediators. Furthermore, RASFs stimulate synovial vascularization through the release of proangiogenic factors. As a result, angiogenesis supports the influx of immune cells into affected joints, thereby perpetuating inflammatory processes, and facilitates access of RASFs to the bloodstream, thus boosting dissemination of RA. Despite intensive research, early pathophysiological processes still remain largely unknown. In this respect, a prearthritic phase of RA is discussed. Early and intensive therapy is considered to be very effective and beneficial for long-term outcome. However, although innovative therapy and improved treatment strategies are applied to achieve clinical remission, failure of or only partial response to therapy remains common. Given that none of the currently approved therapies target RASFs, intensive research into new strategies is warranted. In this review, novel findings leading to the altered fibroblast phenotype in RA are discussed in terms of progressive inflammation and destruction. Potential novel therapeutic concepts are also addressed.

Fresh Pork and Porcine Reproductive and Respiratory Syndrome Virus: Factors Related to the Risk of Disease Transmission.

Porcine reproductive and respiratory syndrome virus (PRRS) is a highly infectious virus. Experimentally, the disease can be induced in naïve pigs by the oral, intranasal and intramuscular routes. Depending on the virulence of the strain of the virus and the age of the pig, peak viremia can occur within 7 days of infection, and live virus can be isolated from blood or lymph nodes for several months post-infection. Young pigs tend to develop higher titres of viremia than older pigs infected by the same route and dose with the same strain of virus. Porcine reproductive and respiratory syndrome virus survives in pork harvested from infected pigs for extended periods at temperatures of -20 or -70°C. In experimentally infected pigs, survival of PRRS virus in muscle held at 4°C has been demonstrated for at least 7 days, and infectivity of the virus in these samples was confirmed by bioassay. The optimal pH range for the survival of PRRS virus is thought to be 6.0 to 7.5. The elevated pH of non-meat tissues (generally one pH unit higher) is likely to favour extended survival of PRRS virus in pig carcasses from which all superficial and deep lymph nodes have not been removed. It is likely that exsanguinated carcasses held at 4°C retain sufficient blood or lymph tissue to contain infective doses of PRRS virus. Porcine reproductive and respiratory syndrome virus is rapidly inactivated by heat, providing a predictable method to ensure that pork tissues are free of viable virus and feeding of cooked swill or garbage should not constitute a risk to pigs. While the probability of viable PRRS virus being present in a pig carcass may be low, the risk is not zero. The importation of raw pork into countries where PRRS is not endemic represents a hazard with potentially severe economic consequences.

A pilot study on the effectiveness of a rose hip shell powder in patients suffering from chronic musculoskeletal pain.

We carried out a 3-month preliminary investigation on the effectiveness of a rose hip shell powder and its mechanism of action. Of 52 patients suffering from acute exacerbations of low back pain (n = 39) or knee pain (n = 13), 29 had participated earlier in the pilot study with the pseudofruit powder Litozin(®) . After assessing the baseline values, patients were offered up to 20 g of a rose hip shell powder per day. Patients were encouraged to adjust the daily dose upwards or downwards according to their symptoms for the period of 3 months. The examination for possible effectiveness was by intention-to-treat analysis with last observation carried forward. There was no difference in any generic or disease-specific outcome variables between the patients consuming the rose hip shell powder and those consuming the pseudofruit powder Litozin(®) in the previous surveillance study. A human protein array system and fractions from the rose powders were used to study their effect on cytokine expression in vitro. The data indicate that lipophilic rose hip fractions from the shell and the pseudofruit inhibit cytokine expression and that the shell powder may be the better starting material for a future rose hip extract prepared with a lipophilic solvent.