Evaluating Effectiveness of Floodplain Sites along the Lamoille Valley Rail Trail: A Blueprint for Future Rail-River Projects

Floodplains perform many functions of value to society, including conveyance and storage of floodwaters for reduced downstream impacts, sediment and nutrient deposition to support soil formation, and maintenance of pulsed overbank flows to support diverse habitats. When constructed along Vermont’s river valleys in the mid-to-late 1800s, railroads often isolated large areas of natural floodplain, leading to decreased flood and sediment storage, and increased downstream flood stages, sediment and nutrient delivery. Where rail lines have been federally-banked and converted to recreational trails, floodplain reconnection could be achieved by modifying the rail embankment through lowering or installing cross culverts or bridges. With the Lamoille Valley Rail Trail (LVRT) in the Lamoille and Missisquoi River basins as a focal study area, this research has generated tools and planning frameworks for transportation and river managers to identify and prioritize candidate reconnection sites, and to holistically evaluate the benefits of these projects alongside potential impacts to adjacent infrastructure or land uses. Effectiveness of completed and proposed floodplain reconnection sites along the LVRT was evaluated at various spatial scales using a suite of tools. At the watershed and reach scales, a screening protocol was developed, leveraging stream geomorphic assessment data to prioritize potential floodplain reconnection sites for further vetting through field inspection. Ten out of twelve floodplain reconnection sites completed along the LVRT in 2006-2008 were predicted as a priority in a retrospective application of this screening protocol. Low-complexity (Height Above Nearest Drainage) hydraulic modeling results confirmed that most completed projects provided significant increases in the floodplain capacity for floods of 2- to 500-year recurrence intervals. Event-scale monitoring conducted at selected sites has confirmed accumulation of fine sediment and phosphorus. A conservative estimate of a half-ton of phosphorus deposited during one storm on 57 acres highlights the water quality benefits of restoring floodplains. Reconnection alternatives were evaluated in more detail using two-dimensional hydraulic modeling (2D HEC-RAS) at a demonstration reach of the Black Creek near East Fairfield spanning two completed reconnection sites and one proposed site on the LVRT. Modeled reconnection alternatives resulted in modest changes in flooding parameters due to an unexpected, existing degree of cross connection between floodplains of the Black Creek and Elm Brook tributary. Nevertheless, this research project has created a framework for more holistic analysis of floodplain reconnection opportunities at similar sites across Vermont and beyond. The hydraulic modeling products and scenarios developed for this project are being adapted to support analysis and modeling of fine sediment and phosphorus attenuation as the Vermont Agency of Transportation continues to collaborate with the Vermont Agency of Natural Resources and other stakeholders to develop a phosphorus-crediting framework for floodplain reconnection projects

Management of CLN1 Disease : International Clinical Consensus

Background: CLN1 disease (neuronal ceroid lipofuscinosis type 1) is a rare, genetic, neurodegenerative lysosomal storage disorder caused by palmitoyl-protein thioesterase 1 (PPT1) enzyme deficiency. Clinical features include developmental delay, psychomotor regression, seizures, ataxia, movement disorders, visual impairment, and early death. In general, the later the age at symptom onset, the more protracted & nbsp;Pediatric Neurology 120 (2021) 38e51 the disease course. We sought to evaluate current evidence and to develop expert practice consensus to support clinicians who have not previously encountered patients with this rare disease. Methods: We searched the literature for guidelines and evidence to support clinical practice recommendations. We surveyed CLN1 disease experts and caregivers regarding their experiences and recommendations, and a meeting of experts was conducted to ascertain points of consensus and clinical practice differences. Results: We found a limited evidence base for treatment and no clinical management guidelines specific to CLN1 disease. Fifteen CLN1 disease experts and 39 caregivers responded to the surveys, and 14 experts met to develop consensus-based recommendations. The resulting management recommendations are uniquely informed by family perspectives, due to the inclusion of caregiver and advocate perspectives. A family-centered approach is supported, and individualized, multidisciplinary care is emphasized in the recommendations. Ascertainment of the specific CLN1 disease phenotype (infantile-, late infantile-, juvenile-, or adult-onset) is of key importance in informing the anticipated clinical course, prognosis, and care needs. Goals and strategies should be periodically reevaluated and adapted to patients' current needs, with a primary aim of optimizing patient and family quality of life. (c) 2021 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).Peer reviewe

Management Strategies for Cln2 Disease

CLN2 disease (neuronal ceroid lipofuscinosis type 2) is a rare, autosomal recessive, pediatric-onset, rapidly progressive neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 (TPP1) enzyme deficiency, and is characterized by language delay, seizures, rapid cognitive and motor decline, blindness, and early death. No management guidelines exist and there is a paucity of published disease-specific evidence to inform clinical practice, which currently draws upon experience from the field of childhood neurodisability. Twenty-four disease experts were surveyed on CLN2 disease management and a subset met to discuss current practice. Management goals and strategies are consistent among experts globally and are guided by the principles of pediatric palliative care. Goals and interventions evolve as the disease progresses, with a shift in focus from maintenance of function early in the disease to maintenance of quality of life. A multidisciplinary approach is critical for optimal patient care. This work represents an initial step toward the development of consensus-based management guidelines for CLN2 disease.WoSScopu