Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 56
Filtrar
1.
Huan Jing Ke Xue ; 45(5): 2952-2961, 2024 May 08.
Artigo em Chinês | MEDLINE | ID: mdl-38629556

RESUMO

To explore the pollution characteristics and source of soil heavy metal in a coal mine area near the Yellow River in Shandong, the geo-accumulation index method and improved Nemerow pollution index method were used to evaluate the pollution characteristics of soil heavy metal. The absolute principal component-multiple linear regression model (APCS-MLR) was used to quantitatively analyze the source of soil heavy metal, and the spatial distribution of Hg and Cd were analyzed using the Kriging spatial difference method in ArcGIS. The result accuracy of the APCS-MLR model was further verified. The results showed that:The measured contents of soil heavy metal Cu, Zn, Pb, Cr, Cd, Ni, As, and Hg all exceeded the normal site, among which, Hg and Cd exceeded the background values of soil elements in Shandong. The coefficient of variation (CV) of Hg was higher than 0.500, indicating significant spatial heterogeneity. Moreover, the correlation between Hg and other heavy metals was generally low, and the possibility of the same pollution source was small. The results of the geo-accumulation index and improved Nemerow pollution index showed that the overall soil heavy metal pollution was at a moderate level, among which the Hg pollution level was the highest, and its maximum value was at a slanted-heavy pollution level; Cu, Cd, and As in soil caused local pollution, which were at a slanted-light pollution level. Soil heavy metal pollution was closely related to mining activities, rehabilitation, and engineering construction in the coal mine area. The two major pollution sources of soil heavy metal in the research area were the compound source of the parent material and industrial and mining transportation sources (known source 1) and the compound source of atmospheric sedimentation and coal production (known source 2), the contribution rates of which were 76.705% and 16.171%, respectively. The results of the APCS-MLR model were shown to be reliable by analyzing the content distribution of Hg and Cd using the Kriging space difference mode. This research can provide scientific basis for the precise control and improvement of soil heavy metal pollution, ensuring the safety of food and agricultural products and improving the quality of the ecological environment in the coal mine area in the Shandong section of the Yellow River Basin.

2.
Front Endocrinol (Lausanne) ; 15: 1354511, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38590822

RESUMO

Background: Diabetic peripheral neuropathy (DPN) contributes to disability and imposes heavy burdens, while subclinical DPN is lack of attention so far. We aimed to investigate the relationship between vitamin D and distinct subtypes of subclinical DPN in type 2 diabetes (T2DM) patients. Methods: This cross-sectional study included 3629 T2DM inpatients who undertook nerve conduction study to detect subclinical DPN in Zhongshan Hospital between March 2012 and December 2019. Vitamin D deficiency was defined as serum 25-hydroxyvitamin D (25(OH)D) level < 50 nmol/L. Results: 1620 (44.6%) patients had subclinical DPN and they were further divided into subgroups: distal symmetric polyneuropathy (DSPN) (n=685), mononeuropathy (n=679) and radiculopathy (n=256). Compared with non-DPN, DPN group had significantly lower level of 25(OH)D (P < 0.05). In DPN subtypes, only DSPN patients had significantly lower levels of 25(OH)D (36.18 ± 19.47 vs. 41.03 ± 18.47 nmol/L, P < 0.001) and higher proportion of vitamin D deficiency (78.54% vs. 72.18%, P < 0.001) than non-DPN. Vitamin D deficiency was associated with the increased prevalence of subclinical DPN [odds ratio (OR) 1.276, 95% confidence interval (CI) 1.086-1.501, P = 0.003] and DSPN [OR 1. 646, 95% CI 1.31-2.078, P < 0.001], independent of sex, age, weight, blood pressure, glycosylated hemoglobin, T2DM duration, calcium, phosphorus, parathyroid hormone, lipids and renal function. The association between vitamin D deficiency and mononeuropathy or radiculopathy was not statistically significant. A negative linear association was observed between 25(OH)D and subclinical DSPN. Vitamin D deficiency maintained its significant association with subclinical DSPN in all age groups. Conclusions: Vitamin D deficiency was independently associated with subclinical DSPN, rather than other DPN subtypes.


Assuntos
Diabetes Mellitus Tipo 2 , Neuropatias Diabéticas , Mononeuropatias , Deficiência de Vitamina D , Humanos , Fatores de Risco , Neuropatias Diabéticas/epidemiologia , Neuropatias Diabéticas/etiologia , Estudos Transversais , Deficiência de Vitamina D/complicações , Deficiência de Vitamina D/epidemiologia , Mononeuropatias/complicações
3.
Orphanet J Rare Dis ; 19(1): 103, 2024 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-38454488

RESUMO

BACKGROUND: As the most common subtype of adult muscular dystrophy worldwide, large cohort reports on myotonic dystrophy type I (DM1) in China are still lacking. This study aims to analyze the genetic and clinical characteristics of Chinese Han DM1 patients. METHODS: Based on the multicenter collaborating effort of the Pan-Yangtze River Delta Alliance for Neuromuscular Disorders, patients with suspected clinical diagnoses of DM1 were genetically confirmed from January 2020 to April 2023. Peak CTG repeats in the DMPK gene were analyzed using triplet repeat-primed PCR (TP-PCR) and flanking PCR. Time-to-event analysis of onset age in females and males was performed. Additionally, detailed clinical features and longitudinal changes from the disease onset in 64 DM1 patients were retrospectively collected and analyzed. The Epworth Sleepiness Scale and Fatigue Severity Scale were used to quantify the severity of daytime sleepiness and fatigue. RESULTS: Among the 211 genetically confirmed DM1 patients, the mean age at diagnosis was 40.9 ± 12.2 (range: 12-74) with a male-to-female ratio of 124:87. The average size of CTG repeats was 511.3 (range: 92-1945). Among the DM1 patients with comprehensive clinical data (n = 64, mean age 41.0 ± 12.0), the age at onset was significantly earlier in males than in females (4.8 years earlier, p = 0.026). Muscle weakness (92.2%), myotonia (85.9%), and fatigue (73.4%) were the most prevalent clinical features. The predominant involved muscles at onset are hands (weakness or myotonia) (52.6%) and legs (walking disability) (42.1%). Of them, 70.3% of patients had daytime sleepiness, 14.1% had cataract surgery, 7.8% used wheelchairs, 4.7% required ventilatory support, and 1.6% required gastric tubes. Regarding the comorbidities, 4.7% of patients had tumors, 17.2% had diabetes, 23.4% had dyspnea, 28.1% had intermittent insomnia, 43.8% experienced dysphagia, and 25% exhibited cognitive impairment. Chinese patients exhibited smaller size of CTG repeats (468 ± 139) than those reported in Italy (613 ± 623), the US (629 ± 386), and Japan (625 [302, 1047]), and milder phenotypes with less multisystem involvement. CONCLUSION: The Chinese Han DM1 patients presented milder phenotypes compared to their Caucasian and Japanese counterparts. A male predominance and an early age of onset were identified in male Chinese Han DM1 patients.


Assuntos
Distúrbios do Sono por Sonolência Excessiva , Miotonia , Distrofia Miotônica , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Distúrbios do Sono por Sonolência Excessiva/diagnóstico , Fadiga , Distrofia Miotônica/genética , Distrofia Miotônica/diagnóstico , Estudos Retrospectivos , Criança , Adolescente , Adulto Jovem , Idoso , Estudos Multicêntricos como Assunto , Estudos de Coortes
4.
Int J Surg Case Rep ; 115: 109241, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38219512

RESUMO

INTRODUCTION: The perioperative efficacy and safety of efgartigimod in patients with thymoma associated myasthenia gravis have not been reported. CASE PRESENTATION: We described the case of a 47-year-old woman who presented thymoma associated myasthenia gravis. Primarily, the patient was treated with acetylcholinesterase inhibitors, immunosuppressive medications, and intravenous immunoglobulin. Unfortunately, the control of symptoms was unsatisfactory. The patient was treated with recommended dosage of efgartigimod (10 mg/kg administered as a 1 h intravenous infusion once weekly for 2 weeks) combined with immunosuppressive therapy. Consequently, improved outcomes and rapid clinical remission were observed. Then, modified subxiphoid thoracoscopic thymectomy was performed smoothly and the patient was discharged from hospital after recovery in short time. DISCUSSION: Administration of efgartigimod could control symptoms significantly and rapidly. Efgartigimod provides the opportunity of thymectomy in short time. Importantly, there was no any perioperative complication or any adverse event related to efgartigimod. CONCLUSION: The improved outcomes of the patient with thymoma associated myasthenia gravis highlight the importance of efgartigimod. Large-scale clinical trials are needed to validate the safety and efficacy of efgartigimod during the perioperative period of thymectomy.

5.
J Med Genet ; 2023 Nov 03.
Artigo em Inglês | MEDLINE | ID: mdl-37923380

RESUMO

BACKGROUND: Oculopharyngodistal myopathy (OPDM) is a rare adult-onset neuromuscular disease, associated with CGG repeat expansions in the 5' untranslated region of LRP12, GIPC1, NOTCH2NLC and RILPL1. However, the genetic cause of a proportion of pathoclinically confirmed cases remains unknown. METHODS: A total of 26 OPDM patients with unknown genetic cause(s) from 4 tertiary referral hospitals were included in this study. Clinical data and laboratory findings were collected. Muscle samples were observed by histological and immunofluorescent staining. Long-read sequencing was initially conducted in six patients with OPDM. Repeat-primed PCR was used to screen the CGG repeat expansions in LOC642361/NUTM2B-AS1 in all 26 patients. RESULTS: We identified CGG repeat expansion in the non-coding transcripts of LOC642361/NUTM2B-AS1 in another two unrelated Chinese cases with typical pathoclinical features of OPDM. The repeat expansion was more than 70 times in the patients but less than 40 times in the normal controls. Both patients showed no leucoencephalopathy but one showed mild cognitive impairment detected by Montreal Cognitive Assessment. Rimmed vacuoles and p62-positive intranuclear inclusions (INIs) were identified in muscle pathology, and colocalisation of CGG RNA foci with p62 was also found in the INIs of patient-derived fibroblasts. CONCLUSIONS: We identified another two unrelated cases with CGG repeat expansion in the long non-coding RNA of the LOC642361/NUTM2B-AS1 gene, presenting with a phenotype of OPDM. Our cases broadened the recognised phenotypic spectrum and pathogenesis in the disease associated with CGG repeat expansion in LOC642361/NUTM2B-AS1.

6.
Front Vet Sci ; 10: 1259760, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38026674

RESUMO

This study aimed to investigate the effects of the combination of selenium and Bacillus subtilis (Se-BS) on the quality and flavor of meat and slaughter performance of broilers. A total of 240 one-day-old Arbor Acres broilers were randomly allotted to four treatments of a basal diet supplemented with no selenium (control), sodium selenite (SS), BS, or Se-BS and raised for 42 days. Compared with the control group, Se-BS significantly increased the carcass weight, the half-eviscerated weight, the completely eviscerated weight, the carcass rate, and redness in broiler muscles; improved the antioxidant state by increasing glutathione peroxidase (GPx) and glutathione S-transferase activities, the total antioxidant capacity, and GPx-1 and thioredoxin reductase 1 messenger RNA (mRNA) levels; promoted biological activity by increasing the contents of glutamate, phenylalanine, lysine, and tyrosine; and increased Se and five types of nitrogenous volatile substances in muscles. On the other hand, Se-BS treatment decreased the shear force, drip loss, and the malondialdehyde, glutathione, and lead contents in muscles. Se-BS exerted a better effect on slaughter performance, the physicochemical quality of meat, the redox status, the amino acid contents, the trace element contents, and volatile substances compared with SS and BS. In conclusion, Se-BS had a positive effect on the quality and flavor of meat and slaughter performance of broilers, suggesting that Se-BS may be a beneficial feed additive.

7.
Toxics ; 11(11)2023 Oct 30.
Artigo em Inglês | MEDLINE | ID: mdl-37999540

RESUMO

It is crucial for effectively controlling potentially toxic element (PTE) pollution to understand the pollution situation, ecological risks, health risks, and migration patterns of PTEs. However, currently, no research has been conducted on the migration patterns of soil PTEs from coal mining subsidence areas to waterlogged areas under different restoration modes. In this study, a total of 15 sediment samples and 60 soil samples were collected from landscaped wetlands, aquaculture wetland, fish-photovoltaic complementary wetland, photovoltaic wetland, and waterlogged areas with untreated coal mining subsidence. The PTE pollution status, ecological risks, health risks, migration patterns, and the important factors influencing the migration were analyzed. The results indicated that the comprehensive pollution level of PTEs in waterlogged areas with coal mining subsidence can be reduced by developing them into landscaped wetlands, aquaculture wetlands, fish-photovoltaic complementary wetlands, and photovoltaic wetlands. Additionally, the closer to the waterlogged area, the higher the Cu content in the subsidence area soil is, reaching its peak in the waterlogged area. The Cd was influenced positively by SOC and pH. The research results were of great significance for formulating reclamation plans for waterlogged areas and controlling PTE pollution.

8.
Opt Express ; 31(21): 34477-34492, 2023 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-37859203

RESUMO

Off-axis optical systems have several important advantages over on-axis ones. However, high polarization aberrations, which play important roles in many applications, become critical disadvantages of off-axis systems. Thanks to the seven free design parameters, three-mirror reflective systems have a good potential to achieve low polarization. A general method to design low polarization off-axis three-mirror reflective optical systems is proposed in this paper. Based on genetic algorithms, several off-axis three-mirror systems with both low polarization aberrations and good wave aberrations are designed. The method proposed in this paper is versatile and can be used to design other types of optical systems that demand low polarization aberrations.

9.
BMC Genomics ; 24(1): 600, 2023 Oct 09.
Artigo em Inglês | MEDLINE | ID: mdl-37814208

RESUMO

BACKGROUND: MicroRNAs (miRNAs) play an important regulatory role in mammalian reproduction. Currently, most studies are primarily concentrated on ovarian miRNAs, ignoring the influence of endometrial miRNAs on the fecundity of female sheep. To uncover potential regulators of sheep fecundity, RNA-seq was used to comparatively analyze miRNA expression profiles of endometrium between high prolificacy sheep (HP, litter size = 3) and low prolificacy sheep (LP, litter size = 1) with FecB genotype. RESULTS: Firstly, genomic features of miRNAs from endometrium were analyzed. Furthermore, 58 differentially expressed (DE) miRNAs were found in the endometrium of Hu sheep with different litter size. A co-expression network of DE miRNAs and target genes has been constructed, and hub genes related litter size are included, such as DE miRNA unconservative_NC_019472.2_1229533 and unconservative_NC_019481.2_1637827 target to estrogen receptor α (ESR1) and unconservative_NC_019481.2_1637827 targets to transcription factor 7 (TCF7). Moreover, functional annotation analysis showed that the target genes (NRCAM and NEGR1) of the DE miRNAs were significantly enriched in cell adhesion molecules (CAMs) signaling pathway, which was related to uterine receptivity. CONCLUSION: Taken together, this study provides a new valuable resource for understanding the molecular mechanisms underlying Hu sheep prolificacy.


Assuntos
MicroRNAs , Ovinos/genética , Feminino , Animais , MicroRNAs/genética , MicroRNAs/metabolismo , Perfilação da Expressão Gênica , RNA Mensageiro/genética , Fertilidade/genética , Endométrio/metabolismo , Mamíferos/genética
10.
Environ Res ; 237(Pt 2): 116936, 2023 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-37648185

RESUMO

Subsidence waterbodies play an important role in carbon cycle in coal mining area. However, little effort has been made to explore the carbon dioxide (CO2) release characteristics and influencing factors in subsidence waterbodies, especially under different restoration modes. Here, we measured CO2 release fluxes (F(CO2)) across Anguo wetland (AW), louts pond (LP), fishpond (FP), fishery-floating photovoltaic wetland (FFPV), floating photovoltaic wetland (FPV) in coal mining subsidence area, with unrestored subsidence waterbodies (SW) and unaffected normal Dasha river (DR) as the control area. We sampled each waterbody and tested which physical, chemical, and biological characteristics of water and sediment related to variability in CO2. The results indicated that F(CO2) exhibited the following patterns: FFPV > FPV > FP > SW > DR > LP > AW. Trophic lake index (TLI) and microbial biomass carbon content (MBC) in sediment had a positive impact on F(CO2). The dominant archaea Euryarchaeota and Thaumarchaeota, and dominant bacteria Proteobacteria promoted F(CO2). This study can help more accurately quantify CO2 emissions and guide CO2 future emission reduction and subsidence waterbodies estoration.

11.
Inorg Chem ; 62(33): 13328-13337, 2023 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-37556609

RESUMO

It is a challenging task to utilize efficient electrocatalytic metal hydroxide-based materials for the oxygen evolution reaction (OER) in order to produce clean hydrogen energy through water splitting, primarily due to the restricted availability of active sites and the undesirably high adsorption energies of oxygenated species. To address these challenges simultaneously, we intentionally engineer a hollow star-shaped Ag/CoMo-LDH heterostructure as a highly efficient electrocatalytic system. This design incorporates a considerable number of heterointerfaces between evenly dispersed Ag nanoparticles and CoMo-LDH nanosheets. The heterojunction materials have been prepared using self-assembly, in situ transformation, and spontaneous redox processes. The nanosheet-integrated hollow architecture can prevent active entities from agglomeration and facilitate mass transportation, enabling the constant exposure of active sites. Specifically, the powerful electronic interaction within the heterojunction can successfully regulate the Co3+/Co2+ ratio and the d-band center, resulting in rational optimization of the adsorption and desorption of the intermediates on the site. Benefiting from its well-defined multifunctional structures, the Ag0.4/CoMo-LDH with optimal Ag loading exhibits impressive OER activity, the overpotential being 290 mV to reach a 10 mA cm-2 current density. The present study sheds some new insights into the electron structure modulation of hollow heterostructures toward rationally designing electrocatalytic materials for the OER.

12.
Brain Sci ; 13(7)2023 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-37508981

RESUMO

BACKGROUND: Various neurologic complications of hyperthyroidism are reported, and most of these complications are reversible with the amelioration of thyrotoxicosis. We report a previously undescribed concurrence of hyperthyroid-associated exercise-induced myalgia and stiffness, pyramidal tract dysfunction, and myoclonic movements that make an initial clinical diagnosis difficult. CASE PRESENTATION: A 17-year-old male was hospitalized in the department of neurology, presenting with a 4-year history of severe exercise-induced myalgia and stiffness, weakness of lower limbs, and myoclonic movements. Laboratory investigations unexpectedly revealed hyperthyroidism. MRI of the brain and spine, electrophysiology, and whole exome sequencing were also performed. Antithyroid therapy led to marked improvement of neurologic symptoms, accompanied by a significant improvement of the time-dependent decline in compound muscle action potentials (CMAP) amplitudes after exercise and normalization of the prolonged QTc interval. Genetic analysis identified a rare variant in SCN5A. CONCLUSION: This case report provides important insights into the relationship between hyperthyroidism and neurologic/cardiac complications, particularly in those with a genetic predisposition. SCN5A mutation possibly plays a role in the complex neurological syndrome associated with hyperthyroidism. Further studies are warranted to better understand the underlying mechanisms and potential therapeutic options for these complex conditions.

13.
Opt Express ; 31(9): 13981-13997, 2023 Apr 24.
Artigo em Inglês | MEDLINE | ID: mdl-37157272

RESUMO

A segmented primary mirror (PM) is an efficient solution to the problems of a monolithic PM manufacture, testing, transportation, and launch. However, the problem of the radius of curvature (ROC) matching among PM segments will arise, which if not solved will seriously degrade the final imaging quality of the system. Accurately detecting ROC mismatch among PM segments from the wavefront map is of crucial importance for efficiently correcting this kind of manufacturing error, while currently there are few related studies. Based on the inherent relation between the PM segment's ROC error and corresponding sub-aperture defocus aberration, this paper proposes that the ROC mismatch can be accurately estimated from the sub-aperture defocus aberration. Secondary mirror (SM) lateral misalignments will influence the accuracy of estimating ROC mismatch. A strategy is also proposed to reduce the influence of SM lateral misalignments. Detailed simulations are performed to demonstrate the effectiveness of the proposed method for detecting ROC mismatch among PM segments. This paper paves a road for detecting ROC mismatch using image-based wavefront sensing methods.

14.
Biol Trace Elem Res ; 201(12): 5756-5763, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-36862247

RESUMO

This study aimed to investigate the effects of selenium (Se)-enriched Bacillus subtilis (Se-BS) on growth performance, antioxidant capacity, immune status, and gut health in broilers. A total of 240 one-day-old Arbor Acres broilers were randomly allotted to four groups and fed with basal diet (control group), 0.30 mg/kg Se (SS group), 3 × 109 CFU/g B. subtilis (BS group), and 0.30 mg/kg Se + 3 × 109 CFU/g B. subtilis (Se-BS group) for 42 days. The results showed that Se-BS supplementation increased body weight (BW), average daily gain, the activities of superoxide dismutase (SOD), glutathione peroxidase (GPx), catalase (CAT), and peroxidase (POD), total antioxidant capacity (T-AOC), and the contents of interleukin (IL)-2, IL-4, and immunoglobulin (Ig) G in plasma, the index and wall thickness of the duodenum, the villus height and crypt depth of the jejunum, and GPx-1 and thioredoxin reductase 1 mRNA levels in liver and intestine and decreased feed conversion ratio (FCR) and plasma malondialdehyde (MDA) content compared with the control group on day 42 (P < 0.05). Compared with groups SS and BS, Se-BS supplementation increased BW, the activities of GPx, CAT, and POD, and the contents of IL-2, IL-4, and IgG in plasma, the index and wall thickness of the duodenum, the crypt depth and secretory IgA content of the jejunum, and GPx-1 mRNA levels in liver and intestine and decreased FCR and plasma MDA content on day 42 (P < 0.05). In conclusion, Se-BS supplementation effectively improved the growth performance antioxidant capacity, immune status, and gut health of broilers.


Assuntos
Antioxidantes , Selênio , Animais , Selênio/farmacologia , Galinhas , Bacillus subtilis , Suplementos Nutricionais , Interleucina-4 , Dieta/veterinária , Glutationa Peroxidase , RNA Mensageiro/genética , Ração Animal/análise
15.
Materials (Basel) ; 16(3)2023 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-36770318

RESUMO

As a direct reflection of the interaction between the stirring tool and the base metal in the friction stir welding process, the force signal is an important means to characterize welding quality. In this paper, the variation mechanism of three-dimensional force and its relation with welding quality were explored. The acquired signals were subject to interference from high-frequency noise, so mean filtering and variational mode decomposition were applied to obtain the real signals. The denoised signals were analyzed and the results showed that the traverse force was ahead of the lateral force by a ratio of π /4, while the phase difference between the axial force and the other two forces changed with the process parameters. Through application of the least square method and polynomial fitting, the empirical formulas of three-dimensional force were obtained, and these were applicable regardless of tunnel defects. The minimum value of the lateral force increased several times more than that of traverse force when the welding speed increased from 80 mm/min to 240 mm/min. When the pole radiuses of most data points had a value greater than 4, tunnel defects were highly likely to generate. In order to predict welding quality more accurately, a prediction model based on long short-term memory was constructed. The model recognized the various modes of good welds and tunnel defects with 100% accuracy. The identification ability for large and small defects was relatively poor, and the average accuracy of classifying the three categories of welding quality was 84.67%.

16.
Front Neurol ; 13: 965207, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36237614

RESUMO

Introduction: The lack of knowledge regarding the differences between Chinese and other ethnicities in the early manifestation of late-onset Pompe disease (LOPD) prohibits the development of an effective screening strategy. We conducted a multicenter screening study to determine LOPD prevalence in high-risk populations and define the early manifestation of LOPD in China. Methods: Between August 2020 and April 2021, the participants were prospectively identified through medical examination at 20 centers from inpatient departments and outpatient neuromuscular clinics in China. The inclusion criteria were as follows: (1) age ≥ 1 year and (2) either one of the following conditions: (a) persistent hyperCKemia, (b) muscle weakness of the axial and/or limb-girdle muscles, or (c) unexplained restrictive respiratory insufficiency (RI). Enzymatic activity of acid α-glucosidase (GAA) was measured in a dried blood spot (DBS) using a tandem mass spectrometry (MS/MS) assay. Next-generation sequencing (NGS) was used to evaluate all samples with decreased GAA activity, searching for GAA mutations and pseudodeficiency alleles. Results: Among the 492 cases, 26 positive samples (5.3%) were detected in the DBS test. Molecular studies confirmed a diagnosis of LOPD in eight cases (1.6%). Using MS/MS assay, GAA activities in individuals with pseudodeficiency could be distinguished from those in patients with LOPD. The median interval from the onset of symptoms to diagnosis was 5 years. All patients also showed RI, with a mean forced vital capacity (FVC) of 48%, in addition to axial/proximal muscle weakness. The creatine kinase (CK) level ranged from normal to no more than 5-fold the upper normal limit (UNL). LOPD with isolated hyperCKemia was not identified. Conclusion: Less frequent hyperCKemia and predominant RI depict a different early portrait of adult Chinese patients with LOPD. A modified high-risk screening strategy should be proposed for the early diagnosis of Chinese patients with LOPD.

17.
Front Vet Sci ; 9: 993039, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36176699

RESUMO

The research evaluated the effects of Aflatoxin B1 on growth performance, antioxidant status, immune response, and pro-inflammatory cytokine mRNA expression in ISA chicks. In total, 240 7-day-old ISA chicks were randomly assigned to four treatment groups. The control group comprised chicks fed a basal diet. The aflatoxin (AFB1)-treatment groups (T1, T2, and T3) comprised chicks fed the basal diet supplemented with AFB1 at concentrations of 5, 8, and 10 µg/kg, respectively. The growth performance, antioxidant status, immune responses, and pro-inflammatory cytokine mRNA expression in all groups were measured. In the T1 treatment group (receiving the lowest AFB1 dose), a reduction in the Newcastle disease virus antibody (NDV-Ab) titer, and increases in interleukin 2 (IL-2), IL-6, and interferon γ (IFN-γ) mRNA levels were observed on days 21 and 42 (P < 0.05). Treatment with the higher AFB1 doses (groups T2 and T3) reduced the chicks' growth performance on days 21 and 42, measured as reductions in body weight (BW) and average daily gain (ADG) compared with the control group. In the T2 and T3 groups, the total antioxidant capacity (T-AOC), glutathione peroxidase (GPX) and superoxide dismutase (SOD) activities, serum immunoglobulin A (IgA) and IgG levels, and IL-2, IL-6, and IFN-γ levels were also lower than in the control group. On days 21 and 42, these two groups also showed increased malondialdehyde (MDA) content, higher feed to gain ratio (F/G), and higher IL-2, IL-6, and IFN-γ mRNA levels than the control group (P < 0.05). The T2 and T3 groups also showed reduced T-AOC, NDV-Ab titer, IL-2 content, and GPx-1 mRNA levels on days 21 and 42 (P < 0.05), increased IL-6 and IFN-γ mRNA levels on day 21, and increased F/G and MDA content on day 42 (P < 0.05) compared with group (T1). Increased MDA content and IL-6 mRNA levels in the liver and ileum were observed in group T3 compared with group T2 on day 21, and lower IgM and IL-6 levels were observed on days 21 and 42 (P < 0.05). In conclusion, our data showed that AFB1 exposure resulted in dose-dependent oxidative and inflammatory damage, immunosuppression, and a decline in the growth performance of chicks.

18.
J Dairy Sci ; 105(7): 6030-6040, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35637003

RESUMO

When ketosis occurs, supraphysiological levels of free fatty acids (FFA) can cause oxidative injury to the mammary gland and autophagy can regulate the cellular oxidative status. The aim of this study was to investigate the autophagy status of mammary tissue and its associations with oxidative stress in healthy and clinically ketotic dairy cows. Mammary tissue and blood samples were collected from healthy cows [n = 15, ß-hydroxybutyrate (BHB) <0.6 mM] and clinically ketotic cows (n = 15, BHB >3.0 mM) at 3 to 15 (average = 7) days in milk. For in vitro study, bovine mammary epithelial cells (BMEC) isolated from healthy cows were treated with 0, 0.3, 0.6, or 1.2 mM FFA for 24 h. Furthermore, BMEC were pretreated with 100 nM rapamycin, an autophagy activator, for 4 h or 50 mM 3-methyladenine (3-MA), an autophagy inhibitor, for 1 h, followed by treatment with or without FFA (1.2 mM) for another 24 h. Oxidation indicators and autophagy-related protein abundance were measured. Compared with healthy cows, serum concentrations of FFA, BHB, and malondialdehyde were greater in clinically ketotic cows, but milk production (kg/d), milk protein (kg/d), activities of superoxide dismutase, catalase, and glutathione peroxidase were lower. Abundances of mRNA and protein of autophagy-related gene 5 (ATG5) and 7 (ATG7) were lower, but sequestosome-1 (SQSTM1, also called p62) greater in mammary tissue of clinically ketotic cows. The mRNA abundance of microtubule-associated protein 1 light chain 3 (MAP1LC3, also called LC3) and protein abundance of LC3-II were lower in mammary tissue of clinically ketotic cows. In vitro, exogenous FFA increased the content of malondialdehyde and reactive oxygen species, but decreased the activities of superoxide dismutase, catalase, and plasma glutathione peroxidase. Compared with the 0 mM FFA group, abundance of ATG5, ATG7, LC3-II was greater, but p62 was lower in the 0.6 mM FFA-treated cells. Similarly, abundance of ATG5, ATG7, and LC3-II was lower, but p62 greater in the 1.2 mM FFA-treated cells relative to 0 mM FFA group. Culture with rapamycin alleviated oxidative stress induced by 1.2 mM FFA, whereas 3-MA aggravated it. Overall, results indicated that a low concentration (0.6 mM) of FFA can induce oxidative stress and activate autophagy in BMEC. At higher concentrations of FFA (1.2 mM), autophagy is impaired and oxidative stress is aggravated. Autophagy is a mechanism for BMEC to counteract FFA-induced stress. As such, it could serve as a potential target for further development of novel strategies against oxidative stress.


Assuntos
Cetose , Ácido 3-Hidroxibutírico , Animais , Autofagia/genética , Catalase/metabolismo , Bovinos , Ácidos Graxos não Esterificados , Feminino , Glutationa Peroxidase/metabolismo , Cetose/veterinária , Malondialdeído , Estresse Oxidativo , RNA Mensageiro/metabolismo , Sirolimo , Superóxido Dismutase/metabolismo
19.
Chemosphere ; 301: 134422, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35358560

RESUMO

Biochar inevitably goes through long-term aging under biotic and abiotic processes in the environment, which results in various changes in its physicochemical properties. However, the traditional characterization methods based on particle separation cannot effectively monitor biochar in complex matrixes. Molecular markers, especially benzene polycarboxylic acids (BPCAs), can be used to directly identify the source and properties of biochar. In this study, biochars were prepared using corn straw (CS) and pinewood (PW) and were oxidized with HNO3/H2SO4 to simulate the aging processes. Molecular markers of lignin-derived phenols showed that PW has more vanillyl unit and thus more stable than CS. The overall BPCAs content and the relative content of mellitic acid (B6CA) both increased with pyrolysis temperature, indicating increased aromatic condensation/aromaticity. The pristine CS biochar has a higher BPCAs content compared to PW biochar. HNO3/H2SO4 treatment greatly decreased the lignin components and more vanillyl and cinnamyl units were removed from CS biochar than PW biochar. In addition, BPCAs contents decreased by 41-60 mg/g for CS biochar, while increased by 86-133 mg/g for PW biochar after HNO3/H2SO4 oxidation. This is owing to the release of the condensed aromatic structures in CS biochars, but the concentration of the condensed aromatic structures in PW biochars after oxidation. These results showed that PW biochars are more stable than CS biochars. The application of the molecular markers can help understanding the dynamic change of biochar in the environment.


Assuntos
Carvão Vegetal , Lignina , Benzeno/química , Ácidos Carboxílicos , Carvão Vegetal/química , Fenóis , Pirólise
20.
Materials (Basel) ; 15(3)2022 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-35160641

RESUMO

Friction stir lap welding (FSLW) is expected to join the hybrid structure of aluminum alloy and steel. In this study, the Al-Mg-Si aluminum alloy and 301L stainless steel were diffusion bonded by FSLDW with the addition of 0.1 mm thick pure Zn interlayer, when the tool pin did not penetrate the upper aluminum sheet. The characteristics of lap interface and mechanical properties of the joint were analyzed. Under the addition of Zn interlayer, the diffusion layer structure at lap interface changed from continuous to uneven and segmented. The components of the diffusion layer were more complex, including Fe-Al intermetallic compounds (IMCs), Fe-Zn IMCs and Al-Zn eutectic. The largely changed composition and thickness of uneven and segmented diffusion layer at the lap interface played a significant role in the joint strength. The tensile shear load of Zn-added joint was 6.26 kN, increasing by 41.3% than that of Zn-not-added joint. These two joints exhibited interfacial shear fracture, while the Zn interlayer enhanced the strength of diffusion bonding by extending the propagation path of cracks.

SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...