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Grouting materials with good thermal insulation and reinforcement properties are the key factors in solving the temperature control problems of high geothermal tunnels using curtain grouting, as the existing grouting materials are unable to take into account the working performance and thermal insulation properties of high-temperature environments. In view of the above problems, this paper configures a high geothermal tunnel red-mud-based grouting material (RMGS) using red mud, carries out tests on the working performance (viscosity, setting time, and compressive strength) and thermal insulation performance (thermal conductivity and specific heat capacity) of the grouting materials at different temperatures (20, 40, 60, and 80 °C), and analyses the variation rules and micro-mechanisms of the various properties at different temperatures. The results show that the increase in temperature will accelerate the viscosity development and condensation of the grouting material and will also lead to the acceleration of the attenuation of the thermal conductivity of the three types of grouting material and the reduction in specific heat capacity. In addition, the appropriate temperature can improve the compressive strength of the material. The increase in temperature will accelerate the hydration reaction speed of the grouting material and will also lead to the development of the internal pore space of the material, which affects the macroscopic properties of the material and is the reason for the effect of the temperature on the performance of the grouting material. In terms of application, the cement slurry is suitable for grouting in a static water environment, the cement–water glass bi-liquid slurry is suitable for grouting in a dynamic water environment, and the RMGS is suitable for grouting in a high-ground-temperature environment.

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common muscular dystrophy. FSHD type 1 (FSHD1) is caused by multicopy contraction of D4Z4 repeats on chromosome 4q35. Human induced pluripotent stem cell (hiPSC) lines serve as important research models for various types of diseases in vitro. Here, we reprogrammed human peripheral blood mononuclear cells (PMBCs) into hiPSCs with episomal plasmid from two FSHD1 patients. These hiPSC lines maintained normal karyotype and exhibited typical morphology. Both of them could express pluripotency markers and differentiate into three layers. The hiPSC lines could be used for screening potential therapeutic targets and mechanism research.

Summary: Background: Facioscapulohumeral muscular dystrophy type 1 (FSHD1) is a rare disease, which is often underdiagnosed due to its heterogeneous presentations and complex molecular genetic basis, leading to a lack of population-based epidemiology data, especially of prevalence and disease progression. Methods: Fujian Neuromedical Centre (FNMC) is a diagnosis centre for clinical-genetic FSHD in China, and the only one employing pulsed-field gel electrophoresis (PFGE)-based Southern blotting for all FSHD1 genetic tests. Three sources distributed across all six spatial zones in China, were used to obtain information regarding FSHD1 events, namely, FNMC, Genetic and Myopathy Group (branches of the Neurology Society of the Chinese Medical Association), and “FSHD-China” (an organization supported by FSHD patients). During 2001-2020, all genetically-confirmed FSHD1 from China were registered in FNMC. Follow-up was conducted in the 20-year period to obtain data on disease progression, which was mainly described in terms of independent ambulation loss. Findings: Of the 1,744 FSHD1 genetic tests (total test number 1,802) included in the analysis, 997 (57.2%) patients from 620 families were diagnosed with FSHD1. The estimated prevalence of genetically-confirmed FSHD1 in China is 0.75 per million (95% confidence interval [CI], 0.70-0.79) during 2001-2020, with 0.78 (95% CI, 0.72-0.85) in males and 0.71 (95% CI, 0.65-0.78) in females. The estimated prevalence increased from 0.22 (95% CI, 0.19-0.26) per million in 2001-2015 to 0.53 (95% CI, 0.49-0.57) per million in 2016-2020 (p < 0.001). The prevalence in Fujian province was 7.10 per million, 4.66 per million, and 2.44 per million, during 2001-2020, 2001-2015, and 2016-2020, respectively. Among the 861 symptomatic plus asymptomatic patients of the total 997 patients, the median onset age at first-ever muscle weakness was 16 years of age (range 1-81); the median number of contracted D4Z4 repeats was 5 units (range 1-9); the median 4qA-allele-specific methylation level was 41% (range 14%-69%). Of the 977 symptomatic patients followed-up during 2001-2020, 117 patients (12.0%) lost independent ambulation. The expected duration from onset of first-ever muscle weakness to onset of independent ambulation loss was 40 years. The group with loss of independent ambulation had a smaller number of contracted D4Z4 repeats (p < 0.001) and had an earlier onset age of first-ever muscle weakness (p < 0.001) compared to the group without loss of independent ambulation. Interpretation: Our research captures the largest genetically-confirmed FSHD1 population worldwide, to calculate its prevalence of 0.75 per million in China from 2001 to 2020. Approximately 12.0% of symptomatic plus asymptomatic patients of FSHD1 will lose independent ambulation in 40 years from onset of first-ever muscle weakness. Funding: This work has been supported by the grants (U2005201, 81870902, N.W.) and (81974193, 81671237, Z.Q.W.) from the National Natural Science Foundation of China; Joint Funds for the Innovation of Science and Technology of Fujian Province (2018Y9082) (N.W.), and the Key Clinical Specialty Discipline Construction Program of Fujian (N.W.).

Deep-buried tunnel at high-ground temperature is prone to the sudden surge of water when crossing the water-rich formation, resulting in casualties and economic losses. Slurry injection is a common and effective method of governance for addressing this issue. However, there has been little research into pulping at high-ground temperature, with most of the existing research focusing on room temperature conditions. We studied two kinds of slurry commonly used in grouting, cement slurry and cement-sodium silicate slurry. Indoor testing was used in this paper to clarify the effect of temperature on slurry viscosity, and numerical simulation was used to study slurry diffusion in flat cracks at different temperatures. The results showed that in a hydrostatic environment, the spread rate of slurry was independent of temperature and type of slurry. For the same kind of slurry, the higher the temperature, the greater the maximum slurry pressure. The higher the temperature in a flow environment, the faster the slurry diffusion. As the temperature rised, the grouting pressure required for crack sealing decreased at first then rises. In a flow environment, the sealing effect of cement-sodium silicate slurry was superior to that of cement slurry.

When mountain tunnel passes through completely weathered granite strata, water and mud inrush is easy to occur, causing casualties and economic losses. Grouting is a common and effective treatment method for water and mud inrush disaster. The current existing researches focus on theoretical analysis, numerical simulation, and laboratory test, and the researches based on the field of engineering application are few. Based on the fully weathered granite tunnel of Junchang tunnel in Guangxi, this paper studies the grouting treatment of water and mud disaster in the fully weathered granite tunnel. This paper first introduces the geological and hydrological conditions of Junchang tunnel in order to understand the causes of water inrush and mud inrush. Then, the treatment method of full-section curtain grouting is introduced. During the grouting, the combination of exploration and injection is adopted. According to different hydrological conditions, different kinds of grouting materials are adopted. After grouting is completed, Transient Electromagnetic Methods, water inflow analysis, borehole investigation, and P-Q-T method are used to evaluate the grouting effect. According to the reaction of the detection results, the weak part of grouting can be supplemented to improve the grouting quality. The results show that the curtain grouting is effective for the treatment of water-mud inrush disaster in the fully weathered granite tunnel. This study provides a reference for the treatment of water and mud inrush in other similar tunnels.

Purpose To analyse the clinical spectrum, genetic features, specific D4Z4 hypomethylation status and genotype-phenotype correlations for somatic mosaicism in facioscapulohumeral dystrophy (FSHD). Methods This was a prospective, hospital-based, case- control, observational study of 35 participants with FSHD with somatic mosaicism recruited over 10 years, with 17 penetrant patients and 18 non-penetrant mutation carriers. This study also included a univariate comparison of 17 paired mosaic and non-mosaic patients with FSHD. Results Mosaic participants with FSHD varied in age of diagnosis (median 45; range 15-65 years), muscle strength (FSHD clinical score median 0; range 0-10 points), clinical severity (age-corrected clinical severity score (ACSS) median 0; range 0-467 points), D4Z4 repeats (median 3; range 2-5 units), mosaic proportion (median 55%; range 27%-72%) and D4Z4 methylation extent (median 49.82%; range 27.17%-64.51%). The genotypic severity scale and D4Z4 methylation extent were significantly associated with ACSS (p1=0.003; p2=0.002). Among the matched pairs, the 17 mosaic patients had shorter D4Z4 repeats, lower FSHD clinical scores and lower ACSS than non-mosaic patients. Additionally, 34 of 35 (97%) participants carried two mosaic arrays, while a single patient had three mosaic arrays (3%). Two cases also carried four-type non-mosaic arrays on chromosome 10 (translocation configuration). Conclusions Broadly, this large mosaic FSHD cohort exhibited significant clinical heterogeneity and relatively slight disease severity. Both genotypic severity scale and D4Z4 hypomethylation status served as modifiers of clinical phenotypes. Consistent with previous reports, mitotic interchromosomal/intrachromosomal gene conversion without crossover was here identified as a major genetic mechanism underlying mosaic FSHD. [ABSTRACT FROM AUTHOR]