Expert Center for cardiac amyloidosis: reality and perspectives

Aim. To evaluate the features of diagnosis of amyloid cardiomyopathy (ACMP), differential diagnosis of different types of amyloidosis and its clinical manifestations. Materials and methods. Were analyzed 150 cases of patients who consulted at the Expert Center for Amyloidosis with suspicion of the presence of ACMP. 63 patients were diagnosed with ACMP: 25 (39.7%) – women, 38 (60.3%) – men, with an average age of 64.1±1.5. 36 (57.1%) patients had AL-amyloidosis (immunoglobulin amyloid light-chain amyloidosis), 25 (39.7%) – ATTR-amyloidosis (transthyretin amyloidosis), 2 (3.2%) – AA-amyloidosis with heart failure (reactive systemic amyloidosis caused by hypersecretion of á-globulin). The analysis of clinical manifestations depending on the type of amyloidosis, data of laboratory and instrumental methods of diagnosis is carried out. Results. In most cases, 53 (84.1%) patients, amyloidosis manifested as signs of heart failure. Among cardiac manifestations, shortness of breath (95.2%), general weakness (93.7%), lower limb edema (76.2%) were the most common. To confirm the diagnosis, despite the high accuracy of the speckle-tracking echocardiography and magnetic resonance imaging of the heart with gadolinium, in rare cases a biopsy is required (e.g. there is a combination of clinical signs of several types of amyloidosis). Biopsy of the affected organ was performed in 31 (49.2%) patients. The strategy for further pathogenetic treatment depends on the determination of the type of amyloidosis. Free light chains of immunoglobulins were detected in 57.1% of cases, which allowed diagnosis of AL-amyloidosis. In 17 (38.6%) patients myocardial scintigraphy with 99mTc-pyrophosphate showed signs of ATTR-amyloidosis, which with a negative result of immunochemical studies allows non-invasive diagnosis of it. Conclusion. ACMP is a disease with an extremely adverse prognosis. Raising the awareness of specialists about ACMP is an important goal. With timely diagnosis, pathogenetic therapy can be started early, which will improve the quality of life and prognosis of patients with ACMP

Transcatheter transfemoral aortic valve replacement in a patient with acromegaly and severe left ventricular myocardial hypertrophy. Case report

Main causes of secondary hypertrophic cardiomyopathy include acromegalic cardiomyopathy. Heart damage in patients with the acromegaly is mediated both by the direct action of growth hormone and insulin-like growth factor-1, and increased deposition of collagen and lymphomononuclear cells in the myocardium, which leads to architectural changes, disturbances in fluid and electrolyte balance, severe left ventricular myocardial hypertrophy, diastolic and systolic left ventricular dysfunction and chronic heart failure. This article presents the world's first described observation demonstrating the possibility of successful transfemoral aortic valve repair to a comorbid patient with severe aortic stenosis according to the potential risks caused by the active form of the acromegaly

Effects of frozen soil on soil temperature, spring infiltration, and runoff: results from the PILPS 2(d) experiment at Valdai, Russia

Permission to place copies of these works on this server has been provided by the American Meteorological Society (AMS). The AMS does not guarantee that the copies provided here are accurate copies of the published work. © Copyright 2003 American Meteorological Society (AMS). Permission to use figures, tables, and brief excerpts from this work in scientific and educational works is hereby granted provided that the source is acknowledged. Any use of material in this work that is determined to be “fair use” under Section 107 of the U.S. Copyright Act or that satisfies the conditions specified in Section 108 of the U.S. Copyright Act (17 USC §108, as revised by P.L. 94-553) does not require the AMS’s permission. Republication, systematic reproduction, posting in electronic form on servers, or other uses of this material, except as exempted by the above statement, requires written permission or a license from the AMS. Additional details are provided in the AMS Copyright Policy, available on the AMS Web site located at (http://www.ametsoc.org/AMS) or from the AMS at 617-227-2425 or [email protected] Project for Intercomparison of Land-Surface Parameterization Schemes phase 2(d) experiment at Valdai, Russia, offers a unique opportunity to evaluate land surface schemes, especially snow and frozen soil parameterizations. Here, the ability of the 21 schemes that participated in the experiment to correctly simulate the thermal and hydrological properties of the soil on several different timescales was examined. Using observed vertical profiles of soil temperature and soil moisture, the impact of frozen soil schemes in the land surface models on the soil temperature and soil moisture simulations was evaluated. It was found that when soil-water freezing is explicitly included in a model, it improves the simulation of soil temperature and its variability at seasonal and interannual scales. Although change of thermal conductivity of the soil also affects soil temperature simulation, this effect is rather weak. The impact of frozen soil on soil moisture is inconclusive in this experiment due to the particular climate at Valdai, where the top 1 m of soil is very close to saturation during winter and the range for soil moisture changes at the time of snowmelt is very limited. The results also imply that inclusion of explicit snow processes in the models would contribute to substantially improved simulations. More sophisticated snow models based on snow physics tend to produce better snow simulations, especially of snow ablation. Hysteresis of snow-cover fraction as a function of snow depth is observed at the catchment but not in any of the models

Effects of Frozen Soil on Soil Temperature, Spring Infiltration, and Runoff: Results from the PILPS 2(d) Experiment at Valdai, Russia

The Project for Intercomparison of Land-Surface Parameterization Schemes phase 2(d) experiment at Valdai, Russia, offers a unique opportunity to evaluate land surface schemes, especially snow and frozen soil parameterizations. Here, the ability of the 21 schemes that participated in the experiment to correctly simulate the thermal and hydrological properties of the soil on several different timescales was examined. Using observed vertical profiles of soil temperature and soil moisture, the impact of frozen soil schemes in the land surface models on the soil temperature and soil moisture simulations was evaluated. It was found that when soil-water freezing is explicitly included in a model, it improves the simulation of soil temperature and its variability at seasonal and interannual scales. Although change of thermal conductivity of the soil also affects soil temperature simulation, this effect is rather weak. The impact of frozen soil on soil moisture is inconclusive in this experiment due to the particular climate at Valdai, where the top 1 m of soil is very close to saturation during winter and the range for soil moisture changes at the time of snowmelt is very limited. The results also imply that inclusion of explicit snow processes in the models would contribute to substantially improved simulations. More sophisticated snow models based on snow physics tend to produce better snow simulations, especially of snow ablation. Hysteresis of snow-cover fraction as a function of snow depth is observed at the catchment but not in any of the models

The representation of snow in land surface schemes: results from PILPS 2(d)

Permission to place copies of these works on this server has been provided by the American Meteorological Society (AMS). The AMS does not guarantee that the copies provided here are accurate copies of the published work. © Copyright 2001 American Meteorological Society (AMS). Permission to use figures, tables, and brief excerpts from this work in scientific and educational works is hereby granted provided that the source is acknowledged. Any use of material in this work that is determined to be “fair use” under Section 107 of the U.S. Copyright Act or that satisfies the conditions specified in Section 108 of the U.S. Copyright Act (17 USC §108, as revised by P.L. 94-553) does not require the AMS’s permission. Republication, systematic reproduction, posting in electronic form on servers, or other uses of this material, except as exempted by the above statement, requires written permission or a license from the AMS. Additional details are provided in the AMS Copyright Policy, available on the AMS Web site located at (http://www.ametsoc.org/AMS) or from the AMS at 617-227-2425 or [email protected] land surface schemes (LSSs) performed simulations forced by 18 yr of observed meteorological data from a grassland catchment at Valdai, Russia, as part of the Project for the Intercomparison of Land-Surface Parameterization Schemes (PILPS) Phase 2(d). In this paper the authors examine the simulation of snow. In comparison with observations, the models are able to capture the broad features of the snow regime on both an intra- and interannual basis. However, weaknesses in the simulations exist, and early season ablation events are a significant source of model scatter. Over the 18-yr simulation, systematic differences between the models’ snow simulations are evident and reveal specific aspects of snow model parameterization and design as being responsible. Vapor exchange at the snow surface varies widely among the models, ranging from a large net loss to a small net source for the snow season. Snow albedo, fractional snow cover, and their interplay have a large effect on energy available for ablation, with differences among models most evident at low snow depths. The incorporation of the snowpack within an LSS structure affects the method by which snow accesses, as well as utilizes, available energy for ablation. The sensitivity of some models to longwave radiation, the dominant winter radiative flux, is partly due to a stability-induced feedback and the differing abilities of models to exchange turbulent energy with the atmosphere. Results presented in this paper suggest where weaknesses in macroscale snow modeling lie and where both theoretical and observational work should be focused to address these weaknesses

Application of a technique for scenario prediction of climate change impact on the water balance components of northern river basins

The scenario forecasting technique for assessing changes of water balance components of the northern river basins due to possible climate change was developed. Three IPCC global emission scenarios corresponding to different possible scenarios for economic, technological, political and demographic development of the human civilization in the 21st century were chosen for generating climate change projections by an ensemble of 16 General Circulation Models with a high spatial resolution. The projections representing increments of monthly values of meteorological characteristics were used for creating 3-hour meteorological time series up to 2063 for the Northern Dvina River basin, which belongs to the pan-Arctic basin and locates at the north of the European part of Russia. The obtained time series were applied as forcing data to drive the land surface model SWAP to simulate possible changes in the water balance components due to different scenarios of climate change for the Northern Dvina River basi

Validation of the energy budget of an alpine snowpack simulated by several snow models (SnowMIP project)

Many snow models have been developed for various applications such as hydrology, global atmospheric circulation models and avalanche forecasting. The degree of complexity of these models is highly variable, ranging from simple index methods to multi-layer models that simulate snow-cover stratigraphy and texture. In the framework of the SnowModel Intercomparison Project (SnowMIP), 23 modelswere compared using observedmeteorological parameters fromtwo mountainous alpine sites.The analysis here focuses on validation of snow energy-budget simulations. Albedo and snow surface temperature observations allow identification of the more realistic simulations and quantification of errors for two components of the energy budget: the net short- and longwave radiation. In particular, the different albedo parameterizations are evaluated for different snowpack states (in winter and spring). Analysis of results during the melting period allows an investigation of the different ways of partitioning the energy fluxes and reveals the complex feedbacks which occur when simulating the snow energy budget. Particular attention is paid to the impact of model complexity on the energy-budget components. The model complexity has a major role for the net longwave radiation calculation, whereas the albedo parameterization is the most significant factor explaining the accuracy of the net shortwave radiation simulation