Prenatal diagnosis of herlitz junctional epidermolysis bullosa by amniocentesis

Herlitz junctional epidermolysis bullosa (HJEB) is a severe blistering disorder which usually results in death during infancy. We have previously shown that the anchoring filament protein laminin-5 (kalinin/nicein), which mediates keratinocyte attachment and dermal–epidermal cohesion, is abnormally expressed in individuals with HJEB. Laminin-5 was detected by Western blot analysis in amniotic fluid from 44 consecutive normal secondtrimester control pregnancies, but was undetectable in second-trimester amniotic fluid from four pregnancies with fetuses affected by HJEB. In one case of severe non-Herlitz JEB, laminin-5 was detected in both amniotic fluid and skin. In human amniotic fluid, the laminin-5 a3 subunit was processed to a major 165 kD species and a minor 145 kD species and the β2 subunit was partially processed to 105 kD. Although laminin-5 was covalently associated with laminin-6 (K-laminin) in amniotic membrane, no covalent interaction was detected in amniotic fluid. Laminin-5 from amniotic fluid strongly supported keratinocyte attachment. These results suggest that Western blot analysis of second-trimester amniotic fluid is useful in determining the prenatal diagnosis of HJEB and that laminin-5 may serve a physiologically important function in amniotic fluid.     

THE ARMA MODELS APPLIED TO THE FLOW OF KARSTIC SPRINGS1

ABSTRACT: Autoregressive moving average (ABMA) models have been applied to study the flow series of the karstic springs of La Villa, Fuente Mayor (Spain), and Aliou (France). The theoretical meaning of the parameters involved in the model upon applying it to a simplified scheme of the emptying of a karstic aquifer is first analyzed. The types of transformations necessary to apply these models to the flow series that lack normality and have strong periodic components are also indicated, as are the advantages of this type of model and the physical significance of the parameters obtained, with respect to the standpoint of hydraulics, ranging from rather homogeneous aquifers (La Villa) to extremely karstic (Aliou), including aquifers with intermediate characteristics (Fuente Mayor).     

A reflection on four impactful Ambio papers: The biotic perspective: This article belongs to Ambio’s 50th Anniversary Collection. Theme: Climate change impacts

Climate change represents one of the most pressing societal and scientific challenges of our time. While much of the current research on climate change focuses on future prediction, some of the strongest signals of warming can already be seen in Arctic and alpine areas, where temperatures are rising faster than the global average, and in the oceans, where the combination of rising temperatures and acidification due to increased CO₂ concentrations has had catastrophic consequences for sensitive marine organisms inhabiting coral reefs. The scientific papers highlighted as part of this anniversary issue represent some of the most impactful advances in our understanding of the consequences of anthropogenic climate change. Here, we reflect on the legacy of these papers from the biotic perspective.     

Aboriginal world views and colonisation: implications for coastal sustainability†

In this paper, we show how the Aboriginal people in the south-west of Australia (the Nyungar) developed systems of knowledge, of caring for country and of family relations that enabled them to survive for tens of thousands of years and continue to have importance today. The impacts of British colonisation on cultural continuity and knowledge in the south-west have been significant and include loss of land, break-up of families and massacre. These practices led to a loss of knowledge of language and culture in some cases. However, Nyungar culture is alive and dynamic, constantly being reclaimed, re-energised and rebuilt through the interaction of contemporary and traditional research praxis. Focusing on Derbal Nara (Cockburn Sound) on the coast in the southern metropolitan area of Perth, we provide case examples of the action-research-learning methodologies used by Whadjuk Nyungar Traditional Owners. We also provide examples of stories about Derbal Nara that are still alive and being recounted up to the present day, including those that account for the recent ice age and the end of the ice age 8000 years BC when sea levels rose, drowning land in the area of Derbal Nara. Finally, we argue that Whadjuk Nyungar experiences and world views based on relationality and reflexivity are central to sustainable coastal management and that in some respects there has already been a convergence of Indigenous and Western coastal management. We present a set of principles that support the development of this “third space” for coastal sustainability.     

The effects and the toxicity increases caused by bicarbonate,chloride, and other water components during the UV/TiO<Subscript>2</Subscript> degradation of oxazaphosphorine drugs

The influences of HCO₃ ^?, Cl^?, and other components on the UV/TiO₂ degradation of the antineoplastic agents ifosfamide (IFO) and cyclophosphamide (CP) were studied in this work. The results indicated that the presence of HCO₃ ^?, Cl^?, NO₃ ^?, and SO₄ ^2? in water bodies resulted in lower degradation efficiencies. The half-lives of IFO and CP were 1.2 and 1.1 min and increased 2.3–7.3 and 3.2–6.3 times, respectively, in the presence of the four anions (initial compound concentration = 100 μg/L, TiO₂ loading =100 mg/L, anion concentration = 1000 mg/L, and pH = 8). Although the presence of HCO₃ ^? in the UV/TiO₂/HCO₃ ^? system resulted in a lower degradation rate and less byproduct formation for IFO and CP, two newly identified byproducts, P11 (M.W. = 197) and P12 (M.W. = 101), were formed and detected, suggesting that additional pathways occurred during the reaction of ?CO₃ ^? in the system. The results also showed that ?CO₃ ^? likely induces a preferred ketonization pathway. Besides the inorganic anions HCO₃ ^?, Cl^?, NO₃ ^?, and SO₄ ^2?, the existence of dissolved organic matter in the water has a significant effect and inhibits CP degradation. Toxicity tests showed that higher toxicity occurred in the presence of HCO₃ ^? or Cl^? during UV/TiO₂ treatment and within 6 h of reaction time, implying that the effects of these two anions should not be ignored when photocatalytic treatment is applied to treat real wastewater.     

Decomposition of perfluorocarboxylic acids (PFCAs) by heterogeneous photocatalysis in acidic aqueous medium

Decomposition of perfluorocarboxylic acids (PFCAs) is of prime importance since they are recognized as persistent organic pollutants and are widespread in the environment. PFCAs with longer carbon chain length are particularly of interest because of their noted recalcitrance, toxicity, and bioaccumulation. Here in this study, we demonstrate efficient decomposition of three important PFCAs such as perfluorooctanoic acid (PFOA), perfluorononanoic acid (PFNA) and perfluorodecanoic acid (PFDA) by heterogeneous photocatalysis with TiO₂ as a photocatalyst in acidic aqueous solutions. The PFCAs were decomposed into shorter carbon chain length PFCAs and fluoride ions. Photoholes of excited TiO₂ generated upon UV-irradiation are found to be the oxidation sites for PFCAs. Therefore, creation and sustenance of these photoholes in the acidic aqueous medium has enhanced the decomposition of PFCAs. Heterogeneous photocatalytic treatment achieved more than 99% decomposition and 38% complete mineralization of PFOA in 7 h. The decomposition of other PFCAs was as high as 99% with a defluorination efficiency of 38% for PFDA and 54% for PFNA. The presence of perchloric acid was found to enhance the decomposition by facilitating the ionization of PFCAs. The oxygen present in the medium served both as an oxidant and an electron acceptor. The mechanistic details of PFCA decomposition and their corresponding mineralization are elaborated.     

A novel high rotation bubble reactor for the treatment of a model pollutant in ozone/goethite/H2O2 and UV/goethite coupled processes

Environmental Science and Pollution Research - This work proposes a novel approach for the coupling of ozonation and Fenton processes using a new prototype of a high rotation bubble reactor (HRBR),...     

Use of Preservative Agents and Antibiotics for Increased Poliovirus Survival on Positively Charged Filters

Environmental surveillance of poliovirus (PV) and other non-enveloped viruses can help identify silent circulation and is necessary to certify eradication. The bag-mediated filtration system is an efficient method to filter large volumes of environmental waters at field sites for monitoring the presence of viruses. As filters may require long transit times to off-site laboratories for processing, viral inactivation or overgrowth of bacteria and fungi can interfere with virus detection and quantification (Miki and Jacquet in Aquatic Microb Ecol 51(2):195–208, 2008). To evaluate virus survival over time on ViroCap^? filters, the filters were seeded with PV type 1 (PV1) and/or MS2 and then dosed with preservatives or antibiotics prior to storage and elution. These filters were stored at various temperatures and time periods, and then eluted for PV1 and MS2 recovery quantification. Filters dosed with the preservative combination of 2% sodium benzoate and 0.2% calcium propionate had increased virus survival over time when stored at 25 °C, compared to samples stored at 25 °C with no preservatives. While elution within 24 h of filtration is recommended, if storage or shipping is required then this preservative mixture can help preserve sample integrity. Addition of an antibiotic cocktail containing cephapirin, gentamicin, and Proclin^? 300 increased recovery after storage at 4 and 25 °C, when compared to storage with no antibiotics. The antibiotic cocktail can aid sample preservation if access to appropriate antibiotics storage is available and sample cold chain is unreliable. This study demonstrated that the use of preservatives or antibiotics is a simple, cost-effective method to improve virus detection from ViroCap cartridge filters over time.