Hepatomegaly and jaundice as the presenting symptoms of systemic light-chain amyloidosis: A case report

BACKGROUND Light chain(AL)amyloidosis is a plasma cell dyscrasia characterized by the pathologic production and extracellular tissue deposition of fibrillar proteins derived from immunoglobulin AL fragments secreted by a clone of plasma cells,which leads to progressive dysfunction of the affected organs.The two most commonly affected organs are the heart and kidneys,and liver is rarely the dominant affected organ with only 3.9%of cases,making them prone to misdia-gnosis and missed diagnosis.CASE SUMMARY A 65-year-old woman was admitted with a 3-mo history of progressive jaundice and marked hepatomegaly.Initially,based on enhanced computed tomography scan and angiography,Budd-Chiari syndrome was considered and balloon dilatation of significant hepatic vein stenoses was performed.However,addi-tional diagnostic procedures,including liver biopsy and bone marrow-exami-nation,revealed immunoglobulin kapa AL amyloidosis with extensive liver involvement and hepatic vascular compression.The disease course was progre-ssive and fatal,and the patient eventually died 5 mo after initial presentation of symptoms.CONCLUSION AL amyloidosis with isolated liver involvement is very rare,and can be easily misdiagnosed as a vascular disease.

Periorbital purpura can be the only initial symptom of primary light chain amyloidosis:A case report

BACKGROUND Primary light chain amyloidosis is a rare and complex disease with complex clinical features and is highly susceptible to misdiagnosis and underdiagnosis in the early stages.CASE SUMMARY We report a case of a 47-year-old female patient whose only initial symptom was periorbital purpura,which was not taken seriously enough.As the disease progressed,pleural effusion gradually appeared,and after systematic diagnosis and treatment,she was diagnosed with“primary light chain amyloidosis”.She achieved rapid hematological remission after treatment with a daratumumab+bortezomib+cyclophosphamide+dexamethasone regimen.CONCLUSION Periorbital purpura can be the only initial symptom of primary light chain amyloidosis;we should pay attention to the cases where the initial clinical symptoms are only periorbital purpura.

Privacy-Preserving and Lightweight V2I and V2V Authentication Protocol Using Blockchain Technology

The confidentiality of pseudonymous authentication and secure data transmission is essential for the protection of information and mitigating risks posed by compromised vehicles.The Internet of Vehicles has meaningful applications,enabling connected and autonomous vehicles to interact with infrastructure,sensors,computing nodes,humans,and fellow vehicles.Vehicular hoc networks play an essential role in enhancing driving efficiency and safety by reducing traffic congestion while adhering to cryptographic security standards.This paper introduces a privacy-preserving Vehicle-to-Infrastructure authentication,utilizing encryption and the Moore curve.The proposed approach enables a vehicle to deduce the planned itinerary of Roadside Units(RSUs)before embarking on a journey.Crucially,the Certification Authority remains unaware of the specific route design,ensuring privacy.The method involves transforming all Roadside Units(RSUs)in a region into a vector,allowing for instant authentication of a vehicle’s route using RSU information.Real-world performance evaluations affirm the effectiveness of the proposed model.

Aligned Ion Conduction Pathway of Polyrotaxane‑Based Electrolyte with Dispersed Hydrophobic Chains for Solid‑State Lithium–Oxygen Batteries

A critical challenge hindering the practical application of lithium–oxygen batteries(LOBs)is the inevitable problems associated with liquid electrolytes,such as evaporation and safety problems.Our study addresses these problems by proposing a modified polyrotaxane(mPR)-based solid polymer electrolyte(SPE)design that simultaneously mitigates solvent-related problems and improves conductivity.mPR-SPE exhibits high ion conductivity(2.8×10^(−3)S cm^(−1)at 25℃)through aligned ion conduction pathways and provides electrode protection ability through hydrophobic chain dispersion.Integrating this mPR-SPE into solid-state LOBs resulted in stable potentials over 300 cycles.In situ Raman spectroscopy reveals the presence of an LiO_(2)intermediate alongside Li_(2)O_(2)during oxygen reactions.Ex situ X-ray diffraction confirm the ability of the SPE to hinder the permeation of oxygen and moisture,as demonstrated by the air permeability tests.The present study suggests that maintaining a low residual solvent while achieving high ionic conductivity is crucial for restricting the sub-reactions of solid-state LOBs.

人LIGHT和HSV-TK-EGFP基因共转染的MSCs抗肿瘤免疫的体内研究

目的研究与单纯疱疹病毒的糖蛋白D竞争结合单纯疱疹病毒进入介导物(herpes virus entry mediator,HVEM)的淋巴毒素类似物(homologous to lymphotoxins,exhibits inducible expression,and competes with HSV glycoprotein D for HVEM,a receptor expressed by T lymphocytes,LIGHT)基因和单纯疱疹病毒胸苷激酶(herpes simplex virus thymidine kinase,HSV-TK)基因共转染的骨髓间充质干细胞(mesenchymal stem cells,MSCs)在体内的抗肿瘤免疫功能。方法将pIRES2-LIGHT基因和HSV-TK-EGFP基因共转染小鼠骨髓间充质干细胞(MSCs/LT组),以转染空载体和转染HSV-TK-EGFP基因的骨髓间充质干细胞作对照。流式细胞仪检测LIGHT分子和HSV-TK-EGFP分子在稳定转染的骨髓间充质干细胞上的表达。体内迁移实验观察MSCs/LT在小鼠体内迁移情况。观察更昔洛韦注射前后MSCs/LT对荷瘤小鼠体内肿瘤的治疗作用。ELISA法检测小鼠肿瘤组织中IFN-γ,IL-2和IL-10的水平。结果流式细胞仪检测发现,MSCs/LT能稳定高表达LIGHT分子。MSCs/LT有特异地向肿瘤组织趋化的特性。MSCs/LT和MSCs/T有较好的抑制肿瘤生长的能力,但在更昔洛韦诱导后,MSCs/LT的抗肿瘤效应下降甚至消失。同时,MSCs/LT可促使T细胞进入肿瘤组织,并促进T细胞分泌IL-2、IFN-γ,抑制IL-10分泌(P<0.05)。结论共转染人LIGHT和HSV-TK-EGFP基因的骨髓间充质干细胞能稳定高表达LIGHT分子,能特异性地向荷瘤小鼠体内肿瘤组织趋化并抑制肿瘤的生长,这种体内抗肿瘤功能可能与促进T淋巴细胞IL-2、IFN-γ等细胞因子的分泌,改善局部免疫抑制环境有关。

Preliminary research on myosin light chain kinase in rabbit liver

AIM: To study preliminarily the properties of myosin light chain kinase (MLCK) in rabbit liver. METHODS: The expression of MLCK was detected by reverse transcription-polymerase chain reaction(RT-PCR); the MLCK was obtained from rabbit liver, and its activity was analyzed by gamma-(32)P incorporation technique to detect the phosphorylation of myosin light chain. RESULTS: MLCK was expressed in rabbit liver, and the activity of the enzyme was similar to rabbit smooth muscle MLCK, and calmodulin-dependent. When the concentration was 0.65 mg x L(-1), the activity was at the highest level. CONCLUSION: MLCK expressed in rabbit liver may catalyze the phosphorylation of myosin light chain, which may play important roles in the regulation of hepatic cell functions.

Analysis of the autophagy gene expression profile of pancreatic cancer based on autophagy-related protein microtubule-associated protein 1A/1B-light chain 3

BACKGROUND Pancreatic cancer is a highly invasive malignant tumor. Expression levels of the autophagy-related protein microtubule-associated protein 1 A/1 B-light chain 3(LC3) and perineural invasion(PNI) are closely related to its occurrence and development. Our previous results showed that the high expression of LC3 was positively correlated with PNI in the patients with pancreatic cancer. In this study, we further searched for differential genes involved in autophagy of pancreatic cancer by gene expression profiling and analyzed their biological functions in pancreatic cancer, which provides a theoretical basis for elucidating the pathophysiological mechanism of autophagy in pancreatic cancer and PNI.AIM To identify differentially expressed genes involved in pancreatic cancer autophagy and explore the pathogenesis at the molecular level.METHODS Two sets of gene expression profiles of pancreatic cancer/normal tissue(GSE16515 and GSE15471) were collected from the Gene Expression Omnibus.Significance analysis of microarrays algorithm was used to screen differentially expressed genes related to pancreatic cancer. Gene Ontology(GO) analysis and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway analysis were used to analyze the functional enrichment of the differentially expressed genes. Protein interaction data containing only differentially expressed genes was downloaded from String database and screened. Module mining was carried out by Cytoscape software and ClusterOne plug-in. The interaction relationship between the modules was analyzed and the pivot nodes between the functional modules were determined according to the information of the functional modules and the data of reliable protein interaction network.RESULTS Based on the above two data sets of pancreatic tissue total gene expression, 6098 and 12928 differentially expressed genes were obtained by analysis of genes with higher phenotypic correlation. After extracting the intersection of the two differential gene sets, 4870 genes were determined. GO analysis showed that 14 significant functional items including negative regulation of protein ubiquitination were closely related to autophagy. A total of 986 differentially expressed genes were enriched in these functional items. After eliminating the autophagy related genes of human cancer cells which had been defined, 347 differentially expressed genes were obtained. KEGG pathway analysis showed that the pathways hsa04144 and hsa04020 were related to autophagy. In addition,65 clustering modules were screened after the protein interaction network was constructed based on String database, and module 32 contains the LC3 gene,which interacts with multiple autophagy-related genes. Moreover, ubiquitin C acts as a pivot node in functional modules to connect multiple modules related to pancreatic cancer and autophagy.CONCLUSION Three hundred and forty-seven genes associated with autophagy in human pancreatic cancer were concentrated, and a key gene ubiquitin C which is closely related to the occurrence of PNI was determined, suggesting that LC3 may influence the PNI and prognosis of pancreatic cancer through ubiquitin C.

雷公藤多苷片对IgA肾病大鼠LIGHT-HVEM/LTβR通路的影响

目的 基于炎症相关通路探讨雷公藤多苷片对IgA肾病大鼠肾脏的作用机制。方法 雄性SPF级SD大鼠,随机分为空白组、造模组。造模组采用联合“牛血清白蛋白+四氯化碳+脂多糖”建立IgA肾病大鼠模型。造模成功的大鼠随机分为模型组、泼尼松组、雷公藤多苷片组,于第13周开始治疗组灌胃给药,给药4周后留取大鼠24 h尿液、血液、肾组织并检测尿红细胞数、24 h-UTP、BUN、Scr;ELISA检测血清IL-1β、TNF-α水平;HE染色观察各组大鼠肾组织病理学变化;Western blot及RT-PCR检测大鼠肾组织LIGHT、HVEM、LTβR蛋白及其mRNA的表达。结果 雷公藤多苷片明显降低IgA肾病大鼠尿红细胞数、24 h-UTP、BUN、Scr水平,改善肾组织病理,降低血清炎症因子IL-1β、TNF-α水平,降低肾组织中LIGHT、HVEM、LTβR蛋白及其mRNA表达水平。结论 雷公藤多苷片可能通过下调LIGHT-HVEM/LTβR信号通路,抑制免疫反应,减少炎症因子释放,从而减轻炎症反应,降低尿红细胞及尿蛋白,改善肾脏病理损伤,保护肾功能。

A blockchain and IoT-based lightweight framework for enabling information transparency in supply chain finance

Supply Chain Finance(SCF)refers to the financial service in which banks rely on core enterprises to manage the capital flow and logistics of upstream and downstream enterprises.SCF adopts a self-testing and closed-loop credit model to control funds and risks.The key factor in a successful SCF service is the deployment of SCF businessoriented information systems that allow businesses to form partnerships efficiently and expedite cash flows throughout the supply chain.Blockchain Technology(BCT),featuring decentralization,tamper-proofing,traceability,which is usually paired with the Internet of Things(IoT)in real-world contexts,has been widely adopted in the field of finance and is perfectly positioned to facilitate innovative collaborations among participants in supply chain networks.In this paper,we propose a BCT and IoT-based information management framework(named BC4Regu),which works as the regulatory to improve the information transparency in the business process of SCF.With BC4Regu,the operation cost of the whole supply chain can be significantly reduced through the coordination and integration of capital flow,information flow,logistics and trade flow in the supply chain.The contributions in this paper include:(1)proposing a novel information management framework which leverages Blockchain and IoT to solve the problem of information asymmetry in the trade of SCF;(2)proposing the technical design of BC4Regu,including the Blockchain infrastructure,distributed ledger-based integrated data flow service,and reshaped SCF process;and(3)applying BC4Regu to a group of scenarios and conducting theoretical analysis by introducing the principal-agent model to validate the BC4Regu.

Submucosal hematoma is a highly suggestive finding for amyloid light-chain amyloidosis:Two case reports

The clinical and endoscopic features of amyloid lightchain(AL) amyloidosis are diverse and mimic various other diseases.Endoscopically,few reports on submucosal hematomas of the gastrointestinal(GI) tract are available in the literature.Here,we report two cases of AL amyloidosis presenting as submucosal hematomas in the absence of clinical disease elsewhere in the body.The 2 cases were referred to our hospital because of hematochezia.The endoscopic findings in both cases were similar in submucosal hematoma formation.However,the clinical courses differed.In the first case,there was no evidence of systemic amyloidosis and the disease was conservatively managed.In the second case,the disease progressed to systemic amyloidosis and the patient died within a short time.We conclude that the endoscopic detection of a submucosal hematoma in the setting of GI bleeding should raise suspicion of AL amyloidosis.Referral to a hematologist should be done immediately for treatment while the involvement is limited to the GI tract.

IMMUNOLOGICAL DIAGNOSIS OF LIGHT CHAIN DISEASE ANALYSIS OF 11 CASES FOUND IN FUJIAN PROVINCE

The present paper reports 11 cases of light chain disease (LCD) sequently found in several citles over Fujian province, Immunological classification of this group of LCD ww as follows: six of me cases belonged to type λ, four of them were type κ, and another one was a double LCD. We found that LCD was common in Fujlan only next to multiple myeloma (MM) of IgG class and accounted for 20% of the total 55 MM cases found in recent yean.It to well known that In matt patients of LCD M protein or Bence Jones proteinemia (BJPemia) to not detectable by conventional electro-phoresis. Our studies show that by making serum protein along with urinary BJP electrophoresis on the same one gel plate the sltuation can be greatly Improved It not only favour* the recognition of smail and faint band or bands of free light chain in serum, but also provides a repid and sensitive way, i. e. , immunofixation, to directly detect urinary light chain on the gel plate Immediately after electrophresis has been run.

基于改进GWO-LightGBM的磨煤机故障预警方法研究

为提高燃煤电厂磨煤机运维效率、降低运维成本,对磨煤机故障预警进行了研究。创新性地提出一种基于改进灰狼优化(GWO)算法的轻量级梯度提升机(LightGBM)故障预警方法。通过建立LightGBM轴承温度预测模型获取磨煤机轴承温度阈值,并引入改进GWO算法优化模型超参数,以提高算法效率和性能。试验结果表明,改进GWO-LightGBM算法相比支持向量机(SVM)等传统算法具有更高的精度和更优的泛化能力。通过实际故障案例证明,该方法能够提前2 h对磨煤机进行早期故障预警。该方法对燃煤电厂磨煤机安全运维具有指导意义。

SMALL ANGLE LIGHT SCATTERING MEASUREMENT OF THE ISOTROPIC TO NEMATIC TRANSITION OF A SIDE CHAIN NEMATIC POLYMETHACRYLATE

The kinetics of I-->N transition of a side chain nematic polymethacrylate has been studied by small angle depolarized light scattering intensity measurements using a charge coupled device linear image sensor. The polymer shows the transition temperatures K52N79I in degreesC, The H-v scattering intensity T(q,t) during the transition I (at 80.2degreesC)-->N (at 75.8degreesC) shows that T(q) is independent of q for all t, and during the initial stage (in 6 s) T(t) increases exponentially with t. In the later stage of the transition T(t) approaches a saturation value in 2 min. This experimental result indicates that the I-->N transition of a liquid crystalline polymer is a spinodal type of phase transition mediated by orientation fluctuation.

基于CEEMDAN-VMD-TCN-lightGBM模型的水质预测研究

针对目前水质预测模型中因为数据本身的复杂性、在信号处理过程中存在的噪声干扰以及分解深度不够导致单一分解难以全面捕捉信号非线性特征的问题,提出了一种基于二次分解的水质预测模型。该模型采用完全自适应噪声集合经验模态分解(CEEMDAN)对原始数据进行分解,再利用变分模态分解(VMD)对熵值最高的模态分量进行二次分解,最终将处理后的时间序列输入到TCN-lightGBM多特征预测模型中。同时,采用麻雀算法(SSA)对预测模型进行优化。以山东省玉符河水质为例,本模型的均方根误差(RMSE)是0.1053,平均绝对误差(MAE)是0.0815,决定系数(R2)是0.9471,与GRU、LSTM、LightGBM、TCN等当下较为流行的模型的预测指标进行比较。结果显示,在R2上本模型提升了53.04%、70.41%、66.07%、65.20%等,在RMSE上减少了62.76%、65.50%、64.93%、64.80%等,在MAE上降低了62.76%、66.24%、63.80%、65.24%等。由此可知,基于CEEMDAN-VMD-TCN-lightGBM的模型具有更好的预测性能、泛化能力和捕捉信号非线性特征的能力。

Persistent diarrhea with petechial rash-unusual pattern of light chain amyloidosis deposition on skin and gastrointestinal biopsies:A case report

BACKGROUND Amyloidosis is a rare disease characterized by extracellular deposition of misfolded protein aggregated into insoluble fibrils.Gastrointestinal involvement in systemic amyloidosis is common,but is often subclinical or presents as vague and nonspecific symptoms.It is rare for gastrointestinal symptoms to be the main presenting symptom in patients with systemic amyloidosis,causing it to be undiagnosed until late-stage disease.CASE SUMMARY A 53 year-old man with diarrhea,hematochezia,and weight loss presented to a community hospital.Colonoscopy with biopsy at that time was suspicious for Crohn disease.Due to worsening symptoms including nausea,vomiting,and a new petechial rash,an abdominal fat pad biopsy was done.The biopsy showed papillary and adnexal dermal amyloid deposition,in a pattern usually seen with cutaneous amyloidosis.However,Cytokeratin 5/6 was negative,excluding cutaneous amyloidosis.The patterns of nodular amyloidosis,subcutaneous amyloid deposits and perivascular amyloid were not seen.Periodic Acid-Schiff stain was negative for lipoid proteinosis,Congo red was positive for apple green birefringence on polarization and amyloid typing confirmed amyloid light chain amyloidosis.Repeat endoscopic biopsies of the gastrointestinal tract showed amyloid deposition from the esophagus to the rectum,in a pattern usually seen in serum amyloid A in the setting of chronic inflammatory diseases,including severe inflammatory bowel disease.Bone marrow biopsy showed kappa-restricted plasma cell neoplasm.CONCLUSION Described is an unusual presentation of primary systemic amyloidosis,highlighting the risk of misdiagnosis with subsequent significant organ dysfunction and high mortality.

Pomolidomide for relapsed/refractory light chain amyloidosis after resistance to both bortezomib and daratumumab:A case report

BACKGROUND Immunoglobulin light chain(AL)amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue,with cardiac involvement being very frequent(61%).Early diagnosis is of high importance because early initiation of treatment in AL amyloidosis may improve outcomes.Despite the administration of immunotherapeutic agents,in particular bortezomib and daratumumab,which have improved the outcomes of AL amyloidosis,antiplasma cell therapy remains suboptimal for some patients.CASE SUMMARY We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac AL amyloidosis by an endomyocardial biopsy.He experienced a short-term hematological remission with no organ response after being administered a bortezomib-daratumumab containing regimen.The treatment was switched to pomolidomide due to pulmonary involvement and progressive pleural effusion,in which flow cytometry analysis showed abnormal plasma cells.After two cycles of this regimen,the pleural effusion was controlled effectively with no recurrence.CONCLUSION This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.

Secondary light chain amyloidosis with Waldenstr?m’s macroglobulinemia and intermodal marginal zone lymphoma:A case report

BACKGROUND The co-existence of Waldenstr?m’s macroglobulinemia(WM) with internodal marginal zone lymphoma(INMZL) is rare and often associated with poor prognosis.CASE SUMMARY We present a Chinese female patient who developed secondary light chain amyloidosis due to WM and INMZL and provides opinions on its systemic treatment.A 65-year-old woman was diagnosed with WM 6 years ago and received Bruton tyrosine kinase inhibitor monotherapy for two years.Her INMZL was confirmed due to left cervical lymphadenopathy.The patient presented with oedema in both lower limbs one year ago,and was diagnosed with secondary light chain amyloidosis.Treatment with the BC regimen(rituximab 375 mg/m~2 monthly for 6-8 courses,and bendamustine 90 mg/m~2 per day × 2,monthly for six courses) was initiated,but not tolerated due to toxic side effects.Bortezomibbased therapy was given for two months,including bortezomib,dexamethasone,and zanubrutinb.Oedema in both lower limbs was relieved and treatment efficacy was evaluated as partial remission.CONCLUSION A detailed clinical evaluation and active identification of the aetiology are recommended to avoid missed diagnosis and misdiagnosis.

Public health importance of light intensity physical activity

1.Light intensity physical activity(PA)benefits health PA guidelines worldwide recommend that adults accumulate 150 min of moderate intensity or 75 min of vigorous intensity PA(moderate-to-vigorous PA(MVPA))weekly to achieve health benefits.1-3 Absent from these guidelines are recommendations for light intensity PA(LPA,e.g.,walking at a leisurely pace of 3 km/h or less,equivalent to 1.5-2.9 metabolic equivalents).

Effects of thinning on the understory light environment of different stands and the photosynthetic performance and growth of the reforestation species Phoebe bournei

Light levels determine regeneration in stands and a key concern is how to regulate the light environment of different stand types to the requirements of the understory.In this study,we selected three stands typical in south China(a Cryptomeria japonica plantation,a Quercus acutissima plantation,and a mixed stand of both)and three thinning intensities to determine the best understory light environ-ment for 3-year-old Phoebe bournei seedlings.The canopy structure,understory light environment,and photosynthe-sis and growth indicators were assessed following thin-ning.Thinning improved canopy structure and understory light availability of each stand;species composition was the reason for differences in the understory light environ-ment.Under the same thinning intensity,the mixed stand had the greatest light radiation and most balanced spectral composition.P.bournei photosynthesis and growth were closely related to the light environment;all three stands required heavy thinning to create an effective and sustained understory light environment.In a suitable understory light environment,the efficiency of light interception,absorption,and use by seedlings was enhanced,resulting in a higher carbon assimilation the main limiting factor was stomatal conductance.As a shade-avoidance signal,red/far-red radia-tion is a critical factor driving changes in photosynthesis and growth of P.bournei seedlings,and a reduction increased light absorption and use capacity and height:diameter ratios.The growth advantage transformed from diameter to height,enabling seedlings to access more light.Our findings suggest that the regeneration of shade-tolerant species such as P.bournei could be enhanced if a targeted approach to thinning based on stand type was adopted.

Power-Law Behavior in Signal Scattering Process in Vertical Granular Chain with Light Impurities

We investigate the scattering process of impulse in vertical granular chain with light impurities. When the perturbation is weak, the quantities describing the reflection rate exhibit power-law behavior with the impurity depth. The exponent is nearly independent of vi. When the perturbation is very strong, the vertical chain shows similar behavior to that of the horizontal chain, so the exponent is nearly zero. Our numerical investigation begins from the weak perturbation region and extends to the nonlinear region and found a peak of the exponent. The difficulty in extending the numerical investigation to a stronger perturbation region is analyzed.PACS numbers: 45.70.-n, 43.25.+y, 46.40.