Clinical manifestations and acid alpha-glucosidase mutation characterisation of a cohort of patients with late-onset Pompe disease in eastern China.

Background: Pompe disease is a rare, progressive, and life-threatening autosomal recessive disorder. In its late-onset form, the disease is primarily characterised by mild progressive proximal limb and respiratory muscle weakness. Mutations in the acid alpha-glucosidase () gene cause lysosomal enzyme GAA to be significantly reduced or missing altogether, for which supplementation can be given through enzyme replacement therapy.

Influence of the tilt error motion of the rotation axis on the test of the equivalence principle with a rotating torsion pendulum.

Improving the precision of current tests of the equivalence principle with a rotating torsion pendulum requires a more complete analysis of systematic effects. Here, we discuss in detail one of the important systematic effects, the influence from the tilt error motion of the rotation axis of a rotary stage, namely, wandering of the instantaneous rotation axis around its average direction. Its influence on the rotating torsion pendulum is modeled phenomenologically, and the parameters in the model are calibrated.

Loss accounting of environmental pollution within Pearl River Delta region, South China.

Economic development puts pressure on environment through air, water and land degradation, which in turn brings real costs to the economy. Actual economy growth should therefore consider the environmental degradation cost resulted from economic activities. Pearl River Delta (PRD) region as a typical delta area with rapid development in China, is with great significance to understand the loses resulted from environmental pollution.

Time-resolved quantification of the dynamic extracellular space in the brain: study of cortical spreading depression.

Extracellular diffusion in the brain is customarily characterized by two parameters, the extracellular space (ECS) volume fraction α and the diffusion tortuosity λ. How these two parameters are temporarily modified and correlated in a physiological/pathological event remains unclear to date. Using tetramethylammonium (TMA) as an ECS ion tracer in a newly updated iontophoretic sinusoidal method, we studied in this work the dynamic α() and λ() in rat somatosensory cortex during spreading depression (SD).

Time-resolved quantification of the dynamic extracellular space in the brain during short-lived event: methodology and simulations.

Two macroscopic parameters describe the interstitial diffusion of substances in the extracellular space (ECS) of the brain, the ECS volume fraction α and the diffusion tortuosity λ. Past methods based on sampling the extracellular concentration of a membrane-impermeable ion tracer, such as tetramethylammonium (TMA), can characterize either the dynamic α() alone or the constant α and λ in resting state but never the dynamic α() and λ() simultaneously in short-lived brain events. In this work, we propose to use a sinusoidal method of TMA to provide time-resolved quantification of α() and λ() in acute brain events.

Test of the Equivalence Principle with Chiral Masses Using a Rotating Torsion Pendulum.

Here we present a new test of the equivalence principle designed to search for the possible violation of gravitational parity using test bodies with different chiralities. The test bodies are a pair of left- and right-handed quartz crystals, whose gravitational acceleration difference is measured by a rotating torsion pendulum. The result shows that the acceleration difference towards Earth Δa_{left-right}=[-1.

Magnetic effect in the test of the weak equivalence principle using a rotating torsion pendulum.

The high precision test of the weak equivalence principle (WEP) using a rotating torsion pendulum requires thorough analysis of systematic effects. Here we investigate one of the main systematic effects, the coupling of the ambient magnetic field to the pendulum. It is shown that the dominant term, the interaction between the average magnetic field and the magnetic dipole of the pendulum, is decreased by a factor of 1.

Molecular Analysis-Based Genetic Characterization of a Cohort of Patients with Duchenne and Becker Muscular Dystrophy in Eastern China.

Background: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are common X-linked recessive neuromuscular disorders caused by mutations in dystrophin gene. Multiplex polymerase chain reaction (multiplex PCR) and multiplex ligation-dependent probe amplification (MLPA) are the most common methods for detecting dystrophin gene mutations. This study aimed to contrast the two methods and discern the genetic characterization of patients with DMD/BMD in Eastern China.

[Intestinal absorption and metabolism of Puerariae Lobatae Radix decoction by in situ closed-loop method].

Biopharmaceutics classification system of Chinese materia medica (CMMBCS) emphasizes characteristic of the multi-component environment based on the drug solubility and permeability. In this study, the in situ closed-loop method combined with LC-MS technique was utilized to study the intestinal absorption and metabolism of Puerariae Lobatae Radix decoction (PLRD), providing selection basis for intestinal permeability components in CMMBCS. A total of 36 components were identified from PLRD.

Effects of Astragalus Polysaccharide on Mechanical Characterization of Liver Sinusoidal Endothelial Cells by Atomic Force Microscopy at Nanoscale.

Objective: To study the effects of Astragalus polysaccharide (APS), the primary effective component of the Chinese herb medicine Astragalus membranaceus (frequently used for its anti-hepatic fibrosis effects), on nanoscale mechanical properties of liver sinusoidal endothelial cells (SECs).

Methods: Using endothelial cell medium as the control, 5 experimental groups were established utilizing different concentrations of APS, i.e.

The Effect of the Treatment with Heated Humidified High-Flow Nasal Cannula on Neonatal Respiratory Distress Syndrome in China: A Single-Center Experience.

. Noninvasive respiratory support is considered the optimal method of providing assistance to preterm babies with breathing problems, including nasal continuous positive airway pressure (NCPAP) and humidified high flow nasal cannula (HHHFNC). The evidence of the efficacy and safety of HHHFNC used as the primary respiratory support for respiratory distress syndrome (RDS) is insufficient in low- and middle-income countries.

TP53-induced glycolysis and apoptosis regulator protects from spontaneous apoptosis and predicts poor prognosis in chronic lymphocytic leukemia.

Objectives: Circulating chronic lymphocytic leukemia (CLL) cells appear not to be overly utilizing aerobic glycolysis. However, recurrent contact with CLL cells in a stromal microenvironment leads to increased aerobic glycolysis and the cells' overall glycolytic capacity, which promotes cell survival and proliferation. TP53-induced glycolysis and apoptosis regulator (TIGAR) has been directly implicated in cellular metabolism in the control of glycolysis.

[Effect of Long-term Oil Contamination on the Microbial Molecular Ecological Networks in Saline-alkali soils].

Long-term oil contamination alters the structures of soil microbial communities, as well as decreases the community diversity, posing a great threat to the ecosystem. To determine the possible influence of long-term oil contamination on microbial interactions in saline-alkali soils, microbial functional molecular ecological networks were studied in both contaminated and uncontaminated soils from Shengli oil field by constructing random networks based on null model. The results demonstrated that the overall networks were significantly different between contaminated and uncontaminated soils (<0.

G measurements with time-of-swing method at HUST.

We review the G measurements with time-of-swing method at HUST. Two independent experiments have been completed and an improved experiment is in progress. The first G value was determined as 6.

[A correlation research on Chinese medical syndromes of chronic heart failure and various complications].

Objective: To study the correlation between various complications of chronic heart failure (CHF) patients and Chinese medical syndromes, thus indicating distribution laws of Chinese medical syndromes in various complications of CHF patients.

Methods: Chinese medical syndrome typing was performed in 630 CHF patients by cross-sectional study of the demographic data, history of present diseases, related information on Chinese medical four diagnostic methods, and the distribution of complications. Logistic regression analysis was used to determine the correlation of various complications of CHF patients and Chinese medical syndromes.

[The FTIR spectral characteristics and comparison study of Astragalus menbranceus soil].

To study the genuine soil of Astragalus menbranceus grows, FTIR spectrometry was used, which is accurate, simple and efficient and has high resolution. The genuine soils include six areas in Hunyuan of Shanxi province, three areas in Yingxian of Shanxi province, Fansi of Shanxi province, and Guyang of Inner Mongolia. Different growth years of two to five for each area were also studied.

[Diagnosis and treatment rule of traditional Chinese medicine for syndrome factors of chronic congestive heart failure: a study based on Shannon entropy method].

Objective: The primary aim of this research is to systematically sort out and analyze available clinical documents for chronic congestive heart failure in traditional Chinese medicine (TCM) and integrated traditional Chinese and Western medicine, and to explore the diagnosis and treatment rule of TCM syndrome factors with data mining method.

Methods: Based on the China National Knowledge Internet (CNKI) and Chinese Biomedical Literature Database (CBM), using "chronic heart failure" or "chronic congestive heart failure" or "chronic cardiac insufficiency" and "traditional Chinese medicine" or "syndrome" or "integrated traditional Chinese and Western medicine" as keywords for literature selection, we used Shannon entropy nonlinear complex system method for the feature extraction after data preprocessing.

Results: The statistics showed that yang deficiency, qi deficiency, blood stasis, water retention, yin deficiency, and turbid phlegm were principal TCM syndrome factors; herbs of qi-tonifying, blood-activating, water-draining, phlegm-dispelling, exterior-releasing, interior-warming were main drugs for clinical application.

[Edaphic FTIR finger print and the area choice of Chinese herb culture].

Unlabelled: In order to search for the difference of the soil of Chinese herb growth, and provide useful information for the area choice of Chinese herb culture, 53 soil samples were detected by FTIR. The results showed that all the samples have 8 peaks at 3,621, 3,425, 1,642, 1,435, 1,029, 878, 760 and 690 cm(-1), respectively, but the relative intensity is different. In addition, the peaks at 1,796, 2,982 and 2,874 cm(-1) show difference too.

[Investigative strategy for research on biological basis of traditional Chinese medicine syndrome: feature selection-based data mining methods].

This paper is devoted to discussing two research patterns of biological basis of traditional Chinese medicine syndrome and presenting a research strategy for data mining methods. It points out that data mining methods which are based on feature selection are better fit for investigating biological basis of traditional Chinese medicine syndrome. Based on such a discussion, the concept of "characteristic pattern" is proposed to bridge the gap between "golden index" and biological basis of traditional Chinese medicine syndrome.

[Study on Chinese herb Astragalus membranceus by FTIR fingerprint].

Unlabelled: The aim of the study is to find new approach to identifying different growth years and different producing areas of astragalus membranceus simply and rapidly. The FTIR spectrometry, which is accurate, simple and efficient and has high resolution, was used to obtain the sample's FTIR spectrometry. After simple separation and extraction, the drying powder of the sample was detected.

[Gel electrophoresis analysis on plasma differential protein in patients with unstable angina of blood-stasis pattern].

Objective: To seek the plasma differential proteins in patients with unstable angina of blood-stasis pattern (UA-BSS) for exploring the proteomic specialty in them by way of two-dimensional difference gel electrophoresis (DIGE) detection on plasma of patients and healthy persons.

Methods: Using DIGE and tandem mass spectrometry, comparative proteomic study was conducted on the plasma of 12 UA patients of qi-deficiency and blood-stasis pattern (UA-QBS), 12 UA patients of phlegm-stasis cross-blocking pattern (UA-PSS) and 12 healthy volunteers.

Results: Preliminary results showed that Haptoglobin beta chain, DBP, HBB, HBA, Transthyretin, ApoA- I, ApoA-IV were significantly differentially expressed in both patterns, while Haptoglobin alpha1 chain, alpha-1-acid glycoprotein, ApoC-III, ApoA-II, ApoC-II, ApoJ, and Haptoglobin alpha 2 chain were only seen differentially expressed in the UA-PSS patients, alpha1-antitrypsin, Fibrinogen gamma chain, and Fibrin beta were only seen differentially expressed in UA-QBS patients.