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Lower limb muscle magnetic resonance imaging in Chinese patients with myotonic dystrophy type 1.

: Muscle magnetic resonance imaging (MRI) is a reliable noninvasion tool for detecting muscle abnormalities of myopathies. This study aimed to investigate the MRI features of lower limb muscles in Chinese patients with myotonic dystrophy type 1 (DM1) and to evaluate the correlation between clinical factors and muscle MRI.: We retrospectively reviewed the medical records and lower limb muscle MRI in 24 Chinese DM1 patients. Muscular Impairment Rating Scale (MIRS) was used to assess the clinical muscular impairment. Modified Mercuri's scale was used to assess the degree of fatty infiltration. Spearman rank correlation test was used to analyze the relationship between fatty degeneration score with age, age of onset, disease duration, MIRS grading and creatinine kinase (CK) level.: Fatty infiltration was found in 22 of 24 DM1 patients and 8 patients were asymmetrically affected. The medial gastrocnemius was the most affected muscle, followed by soleus and tibialis anterior muscles in lower legs. At thigh level, the anterior compartment was usually the most affected region with the rectus femoris relatively spared. 79.2% of DM1 patients had edema in lower limb muscles. The total mean score of fatty infiltration correlated with MIRS grading, age and disease duration but did not correlate with the age of onset or CK level.: Here, we found fatty infiltration present in most Chinese DM1 patients with a selective involvement pattern. There is a correlation between the total mean score of fatty infiltration and MIRS grading, age and disease duration.

1534 related Products with: Lower limb muscle magnetic resonance imaging in Chinese patients with myotonic dystrophy type 1.

Interferon-a Receptor Typ Rat monoclonal anti mouse Guinea Pig Anti-Haemophil Native Parainfluenza Viru Human interleukin 2(IL-2) Native Parainfluenza Viru Bovine prolactin-induced ELISA Insulin (S-type) ,M Multiple cancer (12 type) Multiple organ cancer and High density (208 cores), Native Parainfluenza Viru

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Comparison of diastolic function in children with transfusion dependent beta thalassemia major by tissue and conventional doppler imaging indices and its correlation with serum ferritin levels.

Nanjegowda CK, Kamath SP, Kamath P, Shah TD, Kulkarni V, Lashkari HP, Baliga BS. Comparison of diastolic function in children with transfusion dependent beta thalassemia major by tissue and conventional doppler imaging indices and its correlation with serum ferritin levels. Turk J Pediatr 2019; 61: 250-259. Regular blood transfusions for children with beta thalassemia major (β- TM) results in iron overload cardiomyopathy/cardiac failure. Mortality in these children is most often because of heart failure. We compared Tissue Doppler Imaging (TDI) and conventional pulse wave Doppler (PWD) indices in evaluating diastolic function in chronically transfused β-TM children and correlated the Doppler indices with mean serum ferritin levels. This was a prospective cross-sectional study conducted at tertiary teaching hospital. β-TM children aged 3 to 18 years were enrolled as per inclusion criteria. PWD parameters at the mitral inflow (E, A, E/A and DT) and TDI parameters at the medial mitral annulus (E'and E/E') were used for estimation of diastolic dysfunction. Of the 66 children with thalassemia, the mean age was 10.2±3.77yrs and 60.6% were boys. The E/E' ratio estimated diastolic dysfunction (34/66, 51.5%) greater than four times that assessed by E/A ratio indices (8/66, 12.1%) in the subjects. Association of serum ferritin levels with E/E' ratio by chi square test was significant statistically (P=0.027), however was not significant with E/A ratio. By Mann Whitney test, the median serum ferritin levels (ng/ml) were higher [4034.50, (IQR-2084-5340.25) in those with diastolic dysfunction (abnormal E/E'), when compared to those with normal E/E'[2037.50(1510.75- 3572.25)], with their difference being significant (p=0.011), however serum ferritin levels were not significant with E/A ratio and DT. E/E' parameter had a sensitivity and specificity of 76.5% and 53.1% respectively at a mean serum ferritin cutoff level of 2076 ng/mL by ROC analysis. In conclusion, TDI is a more reliable modality for diagnosing early diastolic dysfunction when compared to PWD. Threshold level of serum ferritin greater than 2076 ng/mL is associated with increased incidence of diastolic dysfunction.

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Determination of picomolar levels of methylmercury complexes with low molecular mass thiols by liquid chromatography tandem mass spectrometry and online preconcentration.

Methylmercury (MeHg) is one of the most potent neurotoxins. It is produced in nature through the methylation of inorganic divalent mercury (Hg) by phylogenetically diverse anaerobic microbes. The mechanistic understanding of the processes that govern the extent of bacterial export of MeHg, its bioaccumulation, and bio-toxicity depends on accurate quantification of its species, especially its complexation with low molecular mass thiols; organometallic complexes that are difficult to detect and measure in natural conditions. Here, we report the development of a novel analytical method based on liquid chromatography tandem mass spectrometry (LC-MS/MS) to determine 13 MeHg complexes with important thiol compounds which have been observed in the environment and in biological systems. By using online preconcentration via solid phase extraction (SPE), the method offers picomolar (12-530 pM) detection limits, the lowest reported so far for the determination of MeHg compounds. Among three different SPE materials, a weak cation exchange phase showed the best efficiency at a low pH of 2.5. We further report the presence of MeHg-cysteine, MeHg-cysteamine, MeHg-penicillamine, MeHg-cysteinylglycine, and MeHg-glutamylcysteine as the predominant MeHg-thiol complexes in the extracellular milieu of an important Hg methylating bacterium, Geobacter sulfurreducens PCA, exposed to 100 nM of Hg.

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Takotsubo cardiomyopathy caused by infusion reaction to trastuzumab.

Takotsubo cardiomyopathy (TCM) is also known as stress-induced cardiomyopathy. The occurrence of TCM due to infusion reaction is extremely rare. A 65-year-old man began receiving trastuzumab monotherapy for gastric cancer. However, he developed an infusion reaction after administration. Electrocardiography revealed negative T waves, ST segment elevation, and apical akinesis and hypokinesis of the left ventricle with apical ballooning in the systole and diastole. Furthermore, troponin I and creatinine kinase (CK) levels and CK-myocardial band were elevated. Based on these findings, he was diagnosed with TCM. This is the first report of TCM due to an infusion reaction.

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Natural Compound Library Screening Identifies New Molecules for the Treatment of Cardiac Fibrosis and Diastolic Dysfunction.

Myocardial fibrosis is a hallmark of cardiac remodeling and functionally involved in heart failure (HF) development, a leading cause of deaths worldwide. Clinically there is no therapeutic strategy available that specifically attenuates maladaptive responses of cardiac fibroblasts, the effector cells of fibrosis in the heart. Therefore, we aimed at the development of novel anti-fibrotic therapeutics based on natural-derived substance library screens for the treatment of cardiac fibrosis. Anti-fibrotic drug candidates were identified by functional screening of 480 chemically diverse natural compounds in primary human cardiac fibroblasts (HCFs), subsequent validation and mechanistic and studies. Hits were analyzed for dose-dependent inhibition of proliferation of HCFs, for modulation of apoptosis and extracellular matrix expression. findings were confirmed , using an angiotensin II (Ang II)-mediated murine model of cardiac fibrosis in both preventive and therapeutic settings, as well as in the Dahl salt sensitive rat model. To investigate the mechanism underlying the anti-fibrotic potential of the lead compounds, treatment-dependent changes in the noncoding RNAome in primary HCFs were analyzed by RNA-deep sequencing. High-throughput natural compound library screening identified 15 substances with antiproliferative effects in HCFs. Using multiple in vitro fibrosis assays and stringent selection algorithms we identified the steroid bufalin (from Chinese toad venom) and the alkaloid lycorine (from species) to be effective anti-fibrotic molecules both and leading to improvement in diastolic function in two hypertension-dependent rodent models of cardiac fibrosis. Administration at effective doses did not change plasma damage markers nor the morphology of kidney and liver, providing first toxicological safety data. By next-generation sequencing we identified the conserved microRNA (miR) miR-671-5p and downstream the antifibrotic selenoprotein P1 (SEPP1) as common effectors of the anti-fibrotic compounds. We identified the molecules bufalin and lycorine as drug candidates for therapeutic applications in cardiac fibrosis and diastolic dysfunction.

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Characterization of Effectiveness in Concerted Inhibition and Stimulation by Pterostilbene (Trans-3,5-dimethoxy-4'-hydroxystilbene), a Stilbenoid.

Pterostilbene (PTER), a natural dimethylated analog of resveratrol, has been demonstrated to produce anti-neoplastic or neuroprotective actions. However, how and whether this compound can entail any perturbations on ionic currents in electrically excitable cells remains unknown. In whole-cell current recordings, addition of PTER decreased the amplitude of macroscopic during long-lasting hyperpolarization in GH cells in a concentration-dependent manner, with an effective IC value of 0.84 μM. Its presence also shifted the activation curve of along the voltage axis to a more hyperpolarized potential, by 11 mV. PTER at a concentration greater than 10 μM could also suppress l-type Ca and transient outward K currents in GH cells. With the addition of PTER, amplitude was increased, with an EC value of 2.23 μM. This increase in amplitude was attenuated by further addition of verruculogen, but not by tolbutamide or TRAM-39. Neither atropine nor nicotine, in the continued presence of PTER, modified the PTER-stimulated . PTER (10 μM) slightly suppressed the amplitude of l-type Ca current and transient outward K current. The presence of PTER (3 μM) was also effective at increasing the open-state probability of large-conductance Ca-activated K (BK) channels identified in hippocampal mHippoE-14 neurons; however, its inability to alter single-channel conductance was detected. Our study highlights evidence to show that PTER has the propensity to perturb ionic currents (e.g., and ), thereby influencing the functional activities of neurons, and neuroendocrine or endocrine cells.

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Porcine Collagen-Bone Composite Induced Osteoblast Differentiation and Bone Regeneration In Vitro and In Vivo.

Due to autogenous bone limitations, some substitute bone grafts were developed. Collagenated porcine graft (CPG) is able to regenerate new bone, although the number of studies is insufficient, highlighting the need for future studies to better understand the biomaterial. In order to understand better CPG's possible dental guided bone regeneration indications, the aim of this work was to determine CPG's biological capacity to induce osteoblast differentiation in vitro and guided bone regeneration in vivo, whilst being compared with commercial hydroxyapatite and beta tricalcium phosphate (HA/β-TCP) and porcine graft alone. Cell cytotoxicity (WST-1), alkaline phosphatase activity (ALP), and real-time polymerase chain reaction (qPCR) were assessed in vitro. Critical size defects of New Zealand white rabbits were used for the in vivo part, with critical size defect closures and histological analyses. WST-1 and ALP indicated that CPG directly stimulated a greater proliferation and confluency of cells with osteoblastic differentiation in vitro. Gene sequencing indicated stable bone formation markers, decreased resorption makers, and bone remodeling coupling factors, making the transition from osteoclast to osteoblast expression at the end of seven days. CPG resulted in the highest new bone regeneration by osteoconduction in critical size defects of rabbit calvaria at eight weeks. Nonetheless, all biomaterials achieved nearly complete calvaria defect closure. CPG was found to be osteoconductive, like porcine graft and HA/β-TCP, but with higher new bone formation in critical size defects of rabbit calvaria at eight weeks. CPG can be used for different dental guided bone regeneration procedures; however, further studies are necessary.

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Foliage application of selenium and silicon nanoparticles alleviates Cd and Pb toxicity in rice (Oryza sativa L.).

Direct discharge of untreated industrial waste water in water bodies and then irrigation from these sources has increased trace metals contamination in paddy fields of southern China. Among trace metals, cadmium (Cd) and lead (Pb) are classified as most harmful contaminants in farmland to many organisms including plants, animals and humans. Rice is a staple food which is consumed by half population of the world; due to longer growth period it can easily absorb and accumulate the trace metals from soil. The objective of study was to check the efficacy of Se and Si NPs (nanoparticles) alone or in combination on metals accumulation and Se-fortified rice (Oryzasativa L.) production as their efficiency remained untested. Alone as well as combined application of Se- and Si-NPs (5, 10 and 20 mg L) was achieved along with CK. All the treatments significantly reduced the Cd and Pb contents in brown rice, except CK, Se3, Si1 and Se1Si3. Combined application of Se and Si (Se3Si2) was more effective in reducing the Cd and Pb contents by 62 and 52%, respectively. In addition, foliar application of both NPs improved the rice growth and quality by increasing the grain yield, rice biomass, and Se contents in brown rice. Highest concentration of Se (1.35 mg kg) in brown rice was observed with combined application of Se- and Si-Nps (Se3Si2). Selenium speciation revealed the presence of organic species (74%) in brown rice. The combinations of different doses of Se- and Si-Nps are the main determining factor for total concentration of metals in grains. These results demonstrate that foliage supplementation of Se and Si-Nps alleviate the Cd and Pb toxicity by reducing the metals' concentration in brown rice. Additionally foliage supplementation improved the nutritional quality by reducing the phytic acid contents in rice grains.

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Preliminary evaluation of a novel nine-biomarker profile for the prediction of autism spectrum disorder.

Autism spectrum disorder (ASD) is a complex group of heterogeneous neurodevelopmental disorders the prevalence of which has been in the rise in the past decade. In an attempt to better target the basic causes of ASD for diagnosis and treatment, efforts to identify reliable biomarkers related to the body's metabolism are increasing. Despite an increase in identifying biomarkers in ASD, there are none so far with enough evidence to be used in routine clinical examination, unless medical illness is suspected. Promising biomarkers include those of mitochondrial dysfunction, oxidative stress, energy metabolism, and apoptosis.

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Molecular characterization of variant infectious bronchitis virus in China, 2019: Implications for control programs.

Infectious bronchitis virus (IBV), an ongoing emergence enveloped virus with a single-stranded positive-sense RNA genome, belongs to the Gammacoronavirus genus in the Coronaviridae family. IBV-associated tracheitis, nephritis, salpingitis, proventriculitis and egg drop have caused devastating economic losses to poultry industry worldwide. Since the end of 2018, a remarkably increasing number of commercial broilers and layers, vaccinated or not, were infected with IBV in China. Here, we described two IB outbreaks with severe respiratory system or kidney injury in IBV-vaccinated commercial poultry farms in central China. Other possible causative viral pathogens, including Avian influenza virus (AIV), Newcastle disease virus (NDV) and Kedah fatal kidney syndrome virus (KFKSV), were excluded by reverse transcription-polymerase chain reaction (RT-PCR), and three virulent IBV strains, HeN-1/China/2019, HeN-2/China/2019 and HeN-101/China/2019, were identified. Although the gross pathologic appearance of these two IB outbreaks were different, the newly identified IBV strains were all closely related to the ck/China/I0529/17 strain and grouped into GI-19 genotype clade based on the sequencing and phylogenetic analysis of the complete S1 genes. Moreover, there are still some evolutionary distance between the newly identified IBV strains, HeN-101/China/2019 in particular, and other GI-19 strains, suggesting that Chinese IBV strains constantly emerge and evolve toward different directions. In conclusion, this study provided an insight of the recently emerging IBV outbreaks in IBV-vaccinated commercial poultry farms and identified the genetic characteristics of three virulent GI-19 IBV strains, which shows the need to carry out proper preventive measures and control strategies.

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