28 research outputs found
Неинвазивные методы изучения коллекторских свойств ископаемых углей
Розглянуті фізичні неінвазивні методи дослідження, які дозволяють отримати уявлення про такі властивості вугільної речовини як повна пористість, питома поверхня, розподіл пір по розмірах та ін., що дають уявлення про колекторні властивості вугільних пластів. Отримані характеристики структури порового простору, що визначають транспортні властивості флюїдів.Physical noninvasion methods of studies are considered, which allow characterizing such
properties of coal as total porosity, specific surface, size distribution of pores and etc.,
demonstrating the reservoir features of coal beds. The description of pore space structure,
determining transport properties of fluids, is presented
2022 Update for the Differences Between Thermodynamic Temperature and ITS-90 Below 335 K
In 2011, a working group of the Consultative Committee for Thermometry published their best estimates of the differences between the thermodynamic temperature T and its approximation (T-90), the temperature according to the International Temperature Scale of 1990, ITS-90. These consensus estimates, in combination with measurements made in accordance with ITS-90, are an important alternative to primary thermometry for those requiring accurate measurements of thermodynamic temperature. Since 2011, there has been a change in the definition of the kelvin and significant improvements in primary thermometry. This paper updates the (T - T-90) estimates by combining and analyzing the data used for the 2011 estimates and data from more recent primary thermometry. The results of the analysis are presented as a 12th-order polynomial representing the updated consensus values for the differences and a sixth-order polynomial for their uncertainty estimates. (C) 2022 Author(s)
Improved upper limb function in non-ambulant children with SMA type 2 and 3 during nusinersen treatment: a prospective 3-years SMArtCARE registry study
Background
The development and approval of disease modifying treatments have dramatically changed disease progression in patients with spinal muscular atrophy (SMA). Nusinersen was approved in Europe in 2017 for the treatment of SMA patients irrespective of age and disease severity. Most data on therapeutic efficacy are available for the infantile-onset SMA. For patients with SMA type 2 and type 3, there is still a lack of sufficient evidence and long-term experience for nusinersen treatment. Here, we report data from the SMArtCARE registry of non-ambulant children with SMA type 2 and typen 3 under nusinersen treatment with a follow-up period of up to 38 months.
Methods
SMArtCARE is a disease-specific registry with data on patients with SMA irrespective of age, treatment regime or disease severity. Data are collected during routine patient visits as real-world outcome data. This analysis included all non-ambulant patients with SMA type 2 or 3 below 18 years of age before initiation of treatment. Primary outcomes were changes in motor function evaluated with the Hammersmith Functional Motor Scale Expanded (HFMSE) and the Revised Upper Limb Module (RULM).
Results
Data from 256 non-ambulant, pediatric patients with SMA were included in the data analysis. Improvements in motor function were more prominent in upper limb: 32.4% of patients experienced clinically meaningful improvements in RULM and 24.6% in HFMSE. 8.6% of patients gained a new motor milestone, whereas no motor milestones were lost. Only 4.3% of patients showed a clinically meaningful worsening in HFMSE and 1.2% in RULM score.
Conclusion
Our results demonstrate clinically meaningful improvements or stabilization of disease progression in non-ambulant, pediatric patients with SMA under nusinersen treatment. Changes were most evident in upper limb function and were observed continuously over the follow-up period. Our data confirm clinical trial data, while providing longer follow-up, an increased number of treated patients, and a wider range of age and disease severity
Refractive-index gas thermometry
International audienceThe principles and techniques of primary refractive-index gas thermometry (RIGT) are reviewed. Absolute primary RIGT using microwave measurements of helium-filled quasispherical resonators has been implemented at the temperatures of the triple points of neon, oxygen, argon and water, with relative standard uncertainties ranging from 9.1 × 10 −6 to 3.5 × 10 −5. Researchers are now also using argon-filled cylindrical microwave resonators for RIGT near ambient temperature, with relative standard uncertainties between 3.8 × 10 −5 and 4.6 × 10 −5 , and conducting relative RIGT measurements on isobars at low temperatures. RIGT at optical frequencies is progressing, and has been used to perform a Boltzmann constant measurement at room temperature with a relative standard uncertainty of 1.2 × 10 −5. Uncertainty budgets from implementations of absolute primary microwave RIGT, relative primary microwave RIGT and absolute primary optical RIGT are provided
Refractive-index gas thermometry
The principles and techniques of primary refractive-index gas thermometry (RIGT) are
reviewed. Absolute primary RIGT using microwave measurements of helium-filled quasi-
spherical resonators has been implemented at the temperatures of the triple points of neon,
oxygen, argon and water, with relative standard uncertainties ranging from 9.1 × 10 −6 to
3.5 × 10 −5 . Researchers are now also using argon-filled cylindrical microwave resonators
for RIGT near ambient temperature, with relative standard uncertainties between 3.8 × 10 −5
and 4.6 × 10 −5 , and conducting relative RIGT measurements on isobars at low temperatures.
RIGT at optical frequencies is progressing, and has been used to perform a Boltzmann
constant measurement at room temperature with a relative standard uncertainty of 1.2 × 10 −5 .
Uncertainty budgets from implementations of absolute primary microwave RIGT, relative
primary microwave RIGT and absolute primary optical RIGT are provided