Global dataset of soil organic carbon in tidal marshes.

Tidal marshes store large amounts of organic carbon in their soils. Field data quantifying soil organic carbon (SOC) stocks provide an important resource for researchers, natural resource managers, and policy-makers working towards the protection, restoration, and valuation of these ecosystems. We collated a global dataset of tidal marsh soil organic carbon (MarSOC) from 99 studies that includes location, soil depth, site name, dry bulk density, SOC, and/or soil organic matter (SOM).

Sexual dysfunction in Wilson disease: Prevalence and influencing factors.

Wilson disease is a rare neurogenetic disorder that receives significant attention due to its manifestations, such as jaundice, cirrhosis, tremor, dystonia, and others. However, the impact of Wilson disease on sexual function has been overlooked. In this study, we aimed to investigate current status of sexual dysfunction in Wilson disease.

Case report: A Chinese patient with spinocerebellar ataxia finally confirmed as Gerstmann-Sträussler-Scheinker syndrome with P102L mutation.

Gerstmann-Sträussler-Scheinker syndrome (GSS) is a rare genetic prion disease caused by a mutation in the prion protein () gene. It is typically characterized by progressive cerebellar ataxia and slowly progressive dementia. We present a case study of the GSS from China in which a 45-year-old male with a progressive gait and balance disorder developed cerebellar ataxia onset but was misdiagnosed as spinocerebellar ataxia (SCA) for 2 years.

Identification of Lncrna-Mrna Networks in Hepg2 Cells upon Knockout and Copper Accumulation.

Background: Hepatolenticular degeneration (HLD) is an inherited disorder caused by the mutation in the adenosine triphosphatase copper transporting β gene (). W aimed to explore the genetic changes in HLD using bioinformatics analysis.

Methods: The study was conducted in Nepal, in 2019.

Age and Serum Creatinine Can Differentiate Wilson Disease Patients with Pseudonormal Ceruloplasmin.

Methods: We retrospectively screened individuals with serum Cp ≥ 140 mg/L from 1032 WD patients who were hospitalised for the first time. Logistic regression analyses were performed in a case-control study between the WD cohort and another liver disease cohort to explore the independent risk factors for WD diagnosis and establish a regression model to identify them. The follow-up medical records of the WD cohort were subjected to mixed-effects model analysis in a longitudinal study to discover factors associated with Cp normalisation.

Exploring the likely relationship between soil carbon change and environmental controls using nonrevisited temporal data sets: Mapping soil carbon dynamics across China.

The prediction of soil organic carbon (SOC) changes in response to environmental change is often limited by a scarcity of revisited temporal data, which constrains scientific understanding and realistic predictions of soil carbon change. The present study improved the potential of nonrevisited temporal data in the prediction of SOC stocks (SOCS) variations. We proposed a method to develop predictions of SOCS change using two independent temporal data sets (pertaining to the 1980s and 2010s) in China based on the digital soil mapping technique.

Association of and Gene Polymorphisms and their DNA Methylation Status with Steroid-Induced Osteonecrosis of the Femoral Head in the Chinese Population.

Osteonecrosis of the femoral head (ONFH) is a severe pathological state with multiple etiologies. Steroid hormone metabolism-related genes play an important role in ONFH. The aim of this study was to investigate the relationships between polymorphisms of the drug-metabolizing enzyme gene, cytochrome P450 (), and the drug transporter gene, ATP-binding cassette subfamily B member 1 (), as well as their DNA methylation status with the pathogenesis of steroid-induced ONFH.

Management Perspective of Wilson's Disease: Early Diagnosis and Individualized Therapy.

Wilson's disease (WD) is an inherited disease caused by mutations in ATP7B and is characterized by the pathological accumulation of copper in the liver and brain. Common clinical manifestations of WD include a wide range of liver disease and neurological symptoms. In some patients, psychiatric symptoms may be the only manifestation at the time of diagnosis.

The study of Wilson disease in pregnancy management.

Introduction: Pregnancy management in women with Wilson disease (WD) remains an important clinical problem. This research was conducted to investigate how to avoid worsening of WD symptoms during pregnancy and increase pregnancy success in women with WD by identifying the best pregnancy management approaches in these patients.

Patients And Methods: The clinical data of 117 pregnancies among 75 women with WD were retrospectively analyzed.

Mechanisms of soil organic carbon storage response to Spartina alterniflora invasion and climate change.

Coastal wetlands have been identified as vital global carbon (C) sinks; however, soil C sequestration in these ecosystems is susceptible to impacts of non-native species invasion and climate change worldwide. Although their potential impacts on soil organic C (SOC) storage have been reported in previous literature, the well-established mechanisms that control SOC storage response, especially in relation to soil depths, is still limited. To fill this knowledge gap, we developed a structural equation model (SEM) to identify mechanisms that account for SOC changes in topsoil (0-0.

Using time-series Sentinel-1 data for soil prediction on invaded coastal wetlands.

Coastal soils are particularly sensitive to nonnative species invasion. In this context, spatially explicit soil information is essential for improving the knowledge of the role of soil in changing environments, supporting coastal sustainable management. Synthetic-aperture radar (SAR) data provides an attractive opportunity to monitor soil because the acquisition of images is independent of weather and daylight.

Inflammatory cytokines expression in Wilson's disease.

Background: Wilson's disease (WD) is an autosomal recessive inherited disorder of copper (Cu) metabolism. Inflammation is a self-defensive reaction aimed at eliminating or neutralizing injurious stimuli, and restoring tissue integrity. Copper deposition may lead to inflammation in the organs and tissues of WD patients.

Long-term outcomes of a patient with late-onset multiple acyl-CoA dehydrogenase deficiency caused by novel mutations in ETFDH: A case report.

Rationale: Late-onset multiple acyl-coenzyme A dehydrogenase deficiency (MADD) mainly affects the neck extensor muscle group, which has been confirmed by novel mutations in electron-transferring-flavoprotein dehydrogenase (ETFDH). So far, a few cases have been reported with long-term follow-up. Here we report a case of late-onset MADD where the patient was followed up for 8 years during which time he underwent 2 muscle biopsies and 2 pathological examinations and his symptoms were significantly alleviated after appropriate treatments.

Sex Differences in Clinical Characteristics and Brain MRI Change in Patients With Wilson's Disease in a Chinese Population.

Wilson's disease (WD) is an inborn copper metabolism disease. Sex differences in clinical features of WD patients have been reported; however, the effect of sex on brain MRI is still unclear, especially for Chinese WD patients. Therefore, we aimed to examine sex differences in clinical correlates and brain MRI changes in WD patients in a Chinese Han population.

Association study of gut flora in Wilson's disease through high-throughput sequencing.

In this study, we analyzed the difference of intestinal flora polymorphisms between Wilson's disease (WD) patients and healthy people by high-throughput sequencing technology, and explored the correlation between WD and intestinal flora polymorphism.A total of 22 cases of WD patients and 22 healthy persons as control were recruited. The total DNA was extracted from the fecal specimens of all the subjects, V4 high variable region of 16S rRNA gene was amplified and sequenced by high-throughput sequencing.

Pedogenic knowledge-aided modelling of soil inorganic carbon stocks in an alpine environment.

Accurate estimation of soil carbon is essential for accounting carbon cycling on the background of global environment change. However, previous studies made little contribution to the patterns and stocks of soil inorganic carbon (SIC) in large scales. In this study, we defined the structure of the soil depth function to fit vertical distribution of SIC based on pedogenic knowledge across various landscapes.

Serum pentraxin 3 is elevated in patients with neurological Wilson's disease.

Background: Wilson's disease (WD) is an autosomal recessive inherited disorder of copper (Cu) metabolism, resulting in pathological accumulation of Cu in many organs and tissues, predominantly in the liver and brain. Cu deposition may lead to neuroinflammation in the brain of WD patients. Pentraxin 3 (PTX3) may play an important role in innate immunity and in WD.

[Analysis of a cerebrotendinous xanthomatosis case with mental retardation as the initial symptom].

Objective: To analyze a case of cerebrotendinous xanthomatosis (CTX) with mental retardation as the initial neurological symptom.

Methods: Medical imaging, histopathological assay and genetic testing were carried out to analyze the patient.

Results: Neurological manifestations of the 27-year-old male patient were initiated by mental retardation and subsequently memory lapses, ataxia, spastic paraplegia and fuzzy language.

Precise estimation of soil organic carbon stocks in the northeast Tibetan Plateau.

There is a need for accurate estimate of soil organic carbon (SOC) stocks for understanding the role of alpine soils in the global carbon cycle. We tested a method for mapping digitally the continuous distribution of the SOC stock in three dimensions in the northeast of the Tibetan Plateau. The approach integrated the spatial distribution of the mattic epipedon which is a special surface horizon widespread and rich in organic matter in Tibetan grasslands.

Current Drug Managements of Wilson's Disease: From West to East.

Wilson's disease (WD), also called hepatolenticular degeneration, is an autosomal recessive inheritance disorder of copper metabolism characterized by the multiple mutations in the ATP-ase 7B gene of chromosome 13q. About half of the WD patients have neurological or psychiatric symptoms. As WD is a kind of medicable or nearly curable neurodegenerative disease in the field of medicine, early consideration/examination and without delay/ life-long treatment usually lead to better prognoses.

Predictive Mapping of Topsoil Organic Carbon in an Alpine Environment Aided by Landsat TM.

The objective of this study was to examine the reflectance of Landsat TM imagery for mapping soil organic Carbon (SOC) content in an Alpine environment. The studied area (ca. 3*104 km2) is the upper reaches of the Heihe River at the northeast edge of the Tibetan plateau, China.

Renal impairment in different phenotypes of Wilson disease.

Wilson's disease (WD) is a rare autosomal recessive genetic disease resulting in the chronic deposition of copper in both liver and brain. This can lead to hepatic, neurologic, and psychiatric manifestations. Renal impairment can occur in any period of WD, but the mechanism is not yet known.

Elevated serum brain natriuretic peptide and matrix metalloproteinases 2 and 9 in Wilson's disease.

Wilson's disease (WD) is a disease of copper metabolism characterized by excessive copper deposition in the body. It is reported abnormal copper metabolism has been associated with cardiovascular disease. BNP and MMP2/9 were biomarkers of congestive heart failure (CHF).

Susceptibility-Weighted Imaging Manifestations in the Brain of Wilson's Disease Patients.

Purpose: It is well known that patients with Wilson's disease (WD) suffer copper metabolism disorder. However, recent studies point to an additional iron metabolism disorder in WD patients. The purpose of our study was to examine susceptibility-weighted imaging (SWI) manifestations of WD in the brains of WD patients.

Wilson disease in the South chinese han population.

Purpose: to prospectively investigate the incidence and prevalence of Wilson disease (Wd) in Chinese han population in anhui province, to analyze the genetic mutations in individuals with Wd, and to provide basic epidemiological data regarding Wd in this Chinese han population.

Methods: between november 2008 and June 2010, individuals aged from 7 to 75 years were screened for the cornea K-f ring in both eyes using slit lamp examination and random sampling methods based on age stratification and cluster level 1. the participants were from anhui province’s hanshan County, Jinzhai County, and lixin County.