3,366 research outputs found

    Staking Our Claim to Water through Conservancy Subdistricts

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    This publication explains the establishment of conservancy subdistricts through the South Dakota Conservancy Act, and Federal and state laws and policies that govern the water resources of South Dakota

    Costs and Benefits of Developing the Wagner, Tower, and Greenwood Irrigation Units

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    Costs and benefits of developing the Wagner, Tower, and Greenwood irrigations units gives the specific costs to individual irrigators and the dollar value of benefits user are expected to receive

    Delivering Water to the Wagner, Tower, and Greenwood Irrigation Units

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    Guide to delivering water to the Water, Tower, and Greenwood irrigations units discusses proposed water resources developments, includes potential areas such as the proposed Geddes and Tyndall units in South Dakota

    Soils, Water, Drainage of the Wagner, Tower, and Greenwood Irrigation Units

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    Guide to soils, water, and drainage of the Wagner, Tower, and Greenwood irrigation units in South Dakota addresses general description of land classes to be irrigated, irrigation water, and surface drainage

    Outdoor Recreation in the Wagner Irrigation Unit

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    Guide to outdoor recreation in the Wagner Irrigation Unit explains the fish, wildlife, and other recreational aspects of the proposed Wagner Irrigation Unit in the general area between Lake Andes and Wagner, South Dakota

    Organizing Irrigation Districts in South Dakota

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    Guide to organizing irrigations districts in South Dakota outlines the major steps that must be taken to organize an irrigation district and answers questions frequently asked in relation to project irrigation as contrasted with privately developed irrigations systems

    Elevated cerebral spinal fluid biomarkers in children with mucopolysaccharidosis I-H.

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    Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results from mutations in the IDUA gene causing the accumulation of glycosaminoglycans (GAGs). Historically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegeneration with death in the first decade due to cardio-pulmonary complications. New data suggest that inflammation may play a role in MPS pathophysiology. To date there is almost no information on the pathophysiologic changes within the cerebral spinal fluid (CSF) of these patients. We evaluated the CSF of 25 consecutive patients with MPS-IH. While CSF glucose and total protein were within the normal range, we found a significantly mean elevated CSF opening pressure at 24 cm H2O (range 14-37 cm H2O). We observed a 3-fold elevation in CSF heparan sulfate and a 3-8 fold increase in MPS-IH specific non-reducing ends, I0S0 and I0S6. Cytokine analyses in CSF of children with MPS-IH showed significantly elevated inflammatory markers including: MCP-1 SDF-1a, IL-Ra, MIP-1b, IL-8, and VEGF in comparison to unaffected children. This is the largest report of CSF characteristics in children with MPS-IH. Identification of key biomarkers may provide further insight into the inflammatory-mediated mechanisms related to MPS diseases and perhaps lead to improved targeted therapies

    Chitotriosidase as a biomarker of cerebral adrenoleukodystrophy

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    <p>Abstract</p> <p>Background</p> <p>Adrenoleukodystrophy (ALD) is an X-linked peroxisomal disorder characterized by the abnormal beta-oxidation of very long chain fatty acids (VLCFA). In 35-40% of children with ALD, an acute inflammatory process occurs in the central nervous system (CNS) leading to demyelination that is rapidly progressive, debilitating and ultimately fatal. Allogeneic hematopoietic stem cell transplantation (HSCT) can halt disease progression in cerebral ALD (C-ALD) if performed early. In contrast, for advanced patients the risk of morbidity and mortality is increased with transplantation. To date there is no means of quantitating neuroinflammation in C-ALD, nor is there an accepted measure to determine prognosis for more advanced patients.</p> <p>Methods</p> <p>As cellular infiltration has been observed in C-ALD, including activation of monocytes and macrophages, we evaluated the activity of chitotriosidase in the plasma and spinal fluid of boys with active C-ALD. Due to genotypic variations in the chitotriosidase gene, these were also evaluated.</p> <p>Results</p> <p>We document elevations in chitotriosidase activity in the plasma of patients with C-ALD (n = 38; median activity 1,576 ng/mL/hr) vs. controls (n = 16, median 765 ng/mL/hr, p = 0.0004), and in the CSF of C-ALD patients (n = 38; median activity 4,330 ng/mL/hr) vs. controls (n = 16, median 0 ng/mL/hr, p < 0.0001). In addition, activity levels of plasma and CSF chitotriosidase prior to transplant correlated with progression as determined by the Moser/Raymond functional score 1 year following transplantation (p = 0.002 and < 0.0001, respectively).</p> <p>Conclusions</p> <p>These findings confirm elevation of chitotriosidase activity in patients with active C-ALD, and suggest that these levels predict prognosis of patients with C-ALD undergoing transplantation.</p
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