How I use allogeneic HSCT for adults with inborn errors of immunity

Inborn Errors of Immunity (IEI) are rare inherited disorders arising from monogenic germline mutations in genes that regulate the immune system. The majority of IEI are Primary Immunodeficiencies characterised by severe infection often associated with autoimmunity, autoinflammation and/or malignancy. Allogeneic hematopoietic stem cell transplant (HSCT) has been the corrective treatment of choice for many IEI presenting with severe disease in early childhood and experience has made this a successful and comparatively safe treatment in affected children. Early HSCT outcomes in adults were poor, resulting in extremely limited use worldwide. This is changing due to a combination of improved IEI diagnosis to inform patient selection, better understanding of the natural history of specific IEI and improvements in transplant practice. Recently published HSCT outcomes for adults with IEI have been comparable with pediatric data, making HSCT an important option for correction of clinically severe IEI in adulthood. Here we discuss our practice for patient selection, timing of HSCT, donor selection and conditioning, peri- and post HSCT management and our approach to long term follow up. We stress the importance of multidisciplinary involvement in the complex decision-making process that we believe is required for successful outcomes in this rapidly emerging area

Adaptability among science teachers in schools: A multi-nation examination of its role in school outcomes

Adaptability helps teachers to navigate change, novelty, and uncertainty at work. We sought to extend understanding of adaptability by considering it at the school-level in 2189 high schools across eight nations. We investigated whether two job demands (disruptive student behavior, student diversity) and a job resource (teacher collaboration) are associated with school-average teacher adaptability. We also examined the association that school-average teacher adaptability has with teacher self-efficacy and, in turn, student self-efficacy. Findings showed, for example, that greater school-average teacher adaptability was positively associated with teacher self-efficacy and, in turn, student self-efficacy. Findings were similar across the eight nations

High-Definition Optical Coherence Tomography for the in vivo Detection of Demodex Mites

Background: Demodex mites are involved in different skin diseases and are commonly detected by skin scrape tests or superficial biopsies. A new high-definition optical coherence tomography (HD-OCT) with high lateral and axial resolution in a horizontal (en-face) and vertical (slice) imaging mode might offer the possibility of noninvasive and fast in vivo examination of demodex mites. Methods: Twenty patients with demodex-related skin diseases and 20 age- and gender-matched healthy controls were examined by HD-OCT. Mites per follicle and follicles per field of view were counted and compared to skin scrape tests. Results: HD-OCT images depicted mites in the en-face mode as bright round dots in groups of 3-5 mites per hair follicle. In the patients with demodex-related disease, a mean number of 3.4 mites per follicle were detected with a mean number of 2.9 infested follicles per area of view compared to a mean of 0.6 mites in 0.4 infested follicles in the controls. The skin scrape tests were negative in 21% of the patients. Conclusion: The innovative HD-OCT enables fast and noninvasive in vivo recognition of demodex mites and might become a useful tool in the diagnosis and treatment monitoring of demodex-related skin diseases. Copyright (C) 2012 S. Karger AG, Base

X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis

The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation

Exploring the quality of social information disclosed in non-financial reports of Croatian companies

By enacting the provisions of Directive 2014/95/EU and the Croatian Accounting Act on disclosing non-financial and diversity information, companies of public interest registering 500 and more employees are required to disclose non-financial information. The purpose of this research is to assess the quality of disclosed social information in non- financial/sustainability reports of Croatian companies. The assessment of the social information was grounded on the framework defined by globally accepted sustainability reporting standards by assessing the quality of social subcategories of human rights, labour practice, community/society and product, measured by attributes of relevance, clarity, verifiability, comparability and clarity. With the overall quality score of 13.16 (out of possible 36), the results prove that Croatian companies do disclose certain social information, but the reliability of this information for benchmarking and competitiveness assessment is questionable, as a consensus on the minimum of information to be disclosed as a fundamental requirement for benchmarking has not yet been reached

Histopathology of Growth Anomaly Affecting the Coral, Montipora capitata: Implications on Biological Functions and Population Viability

Growth anomalies (GAs) affect the coral, Montipora capitata, at Wai'ōpae, southeast Hawai'i Island. Our histopathological analysis of this disease revealed that the GA tissue undergoes changes which compromise anatomical machinery for biological functions such as defense, feeding, digestion, and reproduction. GA tissue exhibited significant reductions in density of ova (66.1–93.7%), symbiotic dinoflagellates (38.8–67.5%), mesenterial filaments (11.2–29.0%), and nematocytes (28.8–46.0%). Hyperplasia of the basal body wall but no abnormal levels of necrosis and algal or fungal invasion was found in GA tissue. Skeletal density along the basal body wall was significantly reduced in GAs compared to healthy or unaffected sections. The reductions in density of the above histological features in GA tissue were collated with disease severity data to quantify the impact of this disease at the colony and population level. Resulting calculations showed this disease reduces the fecundity of M. capitata colonies at Wai'ōpae by 0.7–49.6%, depending on GA severity, and the overall population fecundity by 2.41±0.29%. In sum, GA in this M. capitata population reduces the coral's critical biological functions and increases susceptibility to erosion, clearly defining itself as a disease and an ecological threat

Analysis of the population structure of a gorgonian forest (Placogorgia sp.) using a photogrammetric 3D modeling approach at Le Danois Bank, Cantabrian Sea

The presence of gorgonian forests and deep-sea sponge aggregations in the Le Danois Bank promoted its declaration as the “El Cachucho” Marine Protected Area (MPA) by the Spanish Ministry of Environment, and its inclusion in the Natura 2000 network. Both habitats are considered vulnerable, so follow-up surveys are being performed to monitor their conservation in compliance with the EU Habitats Directive. The use of a non-invasive methodology, which does not cause damage or alterations on benthic communities, is particularly necessary in vulnerable ecosystem studies and MPA monitoring. This study analyzed the assemblage structure of a Placogorgia sp. population using a 3D photogrammetry-based method. The study was carried out through the analysis of the video transects obtained at the Le Danois Bank, using the Politolana underwater towed vehicle during the July 2017 ECOMARG survey. Recent developments in specific software of photogrammetric image analysis allowed extracting valuable information from these video transects. Using the Pix4D Mapper Pro software, 3D point clouds were obtained, and the size and morphometry of yellow fan-shaped gorgonian population structure could be evaluated. Due to gorgonian's high structural complexity, the use of length (i.e. height) as the morphometric descriptor of the real size of the colonies is not appropriate. Instead of length, the fan surface area covered by each gorgonian colony was selected as a suitable parameter of size. The direct measurement of this parameter was possible through a complete 3D reconstruction of the gorgonian forest. A total of 426 colonies of Placogorgia sp. were digitalized to obtain surface measurements and fan spread orientation calculations in 3D models. The results show that gorgonian populations were mostly composed of a high proportion of small colonies (0–0.10 m2). The population structure distribution shows a high proportion (~27%) of recruits (0.5 m2). In 78% of the gorgonian colonies, facing angles were grouped inside the first quadrant (0°-90°), in accordance with the main current direction in this zone. Colony distribution and fan orientation inside the gorgonian forest can be used as data sources to improve monitoring and management programs of these unique habitats in MPAs

Synthesis and Characterization of Tin Disulfide (SnS2) Nanowires

The ordered tin disulfide (SnS2) nanowire arrays were first fabricated by sulfurizing the Sn nanowires, which are embedded in the nanochannels of anodic aluminum oxide (AAO) template. SnS2nanowire arrays are highly ordered and highly dense. X-ray diffraction (XRD) and corresponding selected area electron diffraction (SAED) patterns demonstrate the SnS2nanowire is hexagonal polycrystalline. The study of UV/Visible/NIR absorption shows the SnS2nanowire is a wide-band semiconductor with three band gap energies (3.3, 4.4, and 5.8 eV)

Phase I trial of CYT997, a novel cytotoxic and vascular-disrupting agent

BACKGROUND: CYT997 is a novel microtubule inhibitor and vascular-disrupting agent with marked preclinical anti-tumour activity. METHODS: This phase I dose-escalation study assessed the safety, tolerability, pharmacokinetics and pharmacodynamics of CYT997 administered by continuous intravenous infusion over 24 h every 3 weeks to patients with advanced solid tumours