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.

Magnetic resonance imaging focused on the ferritin heavy chain 1 reporter gene detects neuronal differentiation in stem cells

The neuronal differentiation of mesenchymal stem cells offers a new strategy for the treatment of neurological disorders.Thus,there is a need to identify a noninvasive and sensitive in vivo imaging approach for real-time monitoring of transplanted stem cells.Our previous study confirmed that magnetic resonance imaging,with a focus on the ferritin heavy chain 1 reporter gene,could track the proliferation and differentiation of bone marrow mesenchymal stem cells that had been transduced with lentivirus carrying the ferritin heavy chain 1 reporter gene.However,we could not determine whether or when bone marrow mesenchymal stem cells had undergone neuronal differentiation based on changes in the magnetic resonance imaging signal.To solve this problem,we identified a neuron-specific enolase that can be differentially expressed before and after neuronal differentiation in stem cells.In this study,we successfully constructed a lentivirus carrying the neuron-specific enolase promoter and expressing the ferritin heavy chain 1 reporter gene;we used this lentivirus to transduce bone marrow mesenchymal stem cells.Cellular and animal studies showed that the neuron-specific enolase promoter effectively drove the expression of ferritin heavy chain 1 after neuronal differentiation of bone marrow mesenchymal stem cells;this led to intracellular accumulation of iron and corresponding changes in the magnetic resonance imaging signal.In summary,we established an innovative magnetic resonance imaging approach focused on the induction of reporter gene expression by a neuron-specific promoter.This imaging method can be used to noninvasively and sensitively detect neuronal differentiation in stem cells,which may be useful in stem cell-based therapies.

Effect of temperature on heavy ion-induced single event transient on 16-nm FinFET inverter chains

The variations of single event transient(SET)pulse width of high-LET heavy ion irradiation in 16-nm-thick bulk silicon fin field-effect transistor(Fin FET)inverter chains with different driven strengths are measured at different temperatures.Three-dimensional(3D)technology computer-aided design simulations are carried out to study the SET pulse width and saturation current varying with temperature.Experimental and simulation results indicate that the increase in temperature will enhance the parasitic bipolar effect of bulk Fin FET technology,resulting in the increase of SET pulse width.On the other hand,the increase of inverter driven strength will change the layout topology,which has a complex influence on the SET temperature effects of Fin FET inverter chains.The experimental and simulation results show that the device with the strongest driven strength has the least dependence on temperature.

基于LightGBM算法和出行链理论的电动汽车充电负荷多时间尺度预测模型

为提高电动汽车充电负荷预测的准确性,设计了一种基于轻量级梯度提升机(LightGBM)算法和出行链理论的电动汽车充电负荷多时间尺度预测模型。利用出行链描述用户出行过程,采用蒙特卡洛法抽取时空数据,计算不同区域出行和停留时间的概率密度函数,采用牛顿法划分多时间尺度充电概率,明确驾驶时空分布与充电状况,并运用模糊数学定理与LightGBM分类充电负荷数据,构建了多季节多时段预测模型。采用LightGBM高效并行计算模式,明确充电负荷变化规律,实现了多时间尺度预测。试验结果表明:所建立的模型在不同季节和电动汽车数量条件下,预测误差低于100 kW,预测空报率低于3%,可准确展现充电负荷的变化规律。

基于强降水时序特征的TSVGG-Light暴雨灾情指数预测模型构建及风险灾情一致性分布验证

文章针对强降水灾情预测问题,提出了一种基于强降水时序特征的TSVGG-Light暴雨灾情指数预测模型。该模型利用气象观测数据中的降水时序特征作为输入,对数据进行特征提取和建模,预测出未来一段时间内的强降水灾情指数。为了验证模型的有效性,文中还进行了风险灾情一致性分布验证。实验结果表明,TSVGG-Light模型在强降水灾情预测上具有较高的准确性和稳定性,并且模型预测结果与实际灾情分布具有较好的一致性。TSVGG-Light模型可以成为预测和评估强降水灾情的有效工具。

磁性Fe_3O_4纳米颗粒对大鼠脏器组织中Caveolin-1和Clathrin Heavy Chain蛋白表达的影响

目的:探讨磁性Fe_3O_4纳米颗粒对大鼠主要脏器组织中Caveolin-1及Clathrin Heavy Chain蛋白表达的影响,阐明其作用机制。方法:将24只Wistar大鼠按体质量随机分成对照组和低、中、高剂量磁性Fe_3O_4纳米颗粒组,尾静脉注射不同剂量磁性Fe_3O_4纳米颗粒24h后取脏器组织,Western blotting法检测大鼠主要脏器组织中Caveolin-1及Clathrin Heavy Chain蛋白的表达水平,荧光实时定量PCR法检测大鼠主要脏器组织中Caveolin-1及Clathrin Heavy Chain mRNA的表达水平。结果:与对照组比较,中和高剂量组大鼠肝脏和脾脏组织中Clathrin Heavy Chain蛋白和mRNA表达水平明显升高(P<0.05)。高剂量组大鼠肾脏组织中Clathrin Heavy Chain mRNA的表达水平与其他3组比较明显升高(P<0.05)。Caveolin-1蛋白表达水平在各剂量组之间比较差异无统计学意义(P>0.05);与对照组比较,低、中和高剂量组大鼠肝脏、肺脏和脾脏组织中Caveolin-1mRNA表达水平明显升高(P<0.05);各组肾脏组织中Caveolin-1 mRNA表达水平差异无统计学意义(P>0.05)。结论:磁性Fe_3O_4纳米颗粒能够诱导大鼠肝脏、肺脏、脾脏中Clathrin Heavy Chain蛋白表达增强,通过Clathrin Heavy Chain蛋白的内吞作用是磁性Fe_3O_4纳米颗粒进入大鼠肝脏、肺脏和脾脏细胞的途径之一。

Biotransfer of heavy metals along a soil-plant-insect-chicken food chain:Field study

The accumulation and transfer of Pb,Zn,Cu,and Cd along a soil-plant-insect-chicken food chain at contaminated sites were investigated.The study site nearing the Pb/Zn mine had been contaminated by heavy metals severely.Cadmium and Pb concentrations steadily declined with increasing trophic level（p 〈 0.01）,but concentrations of Zn and Cu slightly increased from plant to insect larva（p 〉 0.05）.The concentrations of heavy metals were the highest in chicken muscle,with lower values in liver and blood.The bioaccumulation of Pb was observed in chicken livers.The eliminations of Pb,Zn,Cu,and Cd via insect and chicken feces avoid metal bioaccumulation in insect and chicken body.These results suggest that the accumulation of heavy metals in specific animal organ of tissues could not be neglected,although transfer of metals to chicken from plant and insect was limited.

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.

Correction Method of Light Hydrocarbons Losing and Heavy Hydrocarbon Handling for Residual Hydrocarbon (S_1) from Shale

In China, hot researches on shale oil were raised by the important breakthrough of shale oil in America. Obviously, the first important issue is the actual shale oil resource potential of China, and the selection of the key appraisement parameter is vital to the shale oil resource amount. Among the appraisement parameters, the oil content parameter（S1） is the key one, but the evaluation result is generally lower because of light hydrocarbon losing and heavy hydrocarbon handling. And the more important thing is that the light hydrocarbon with small molecular weight is more recoverable, and therefore its amount is important to the total shale oil yields. Based on pyrolysis experiments and the kinetic model of hydrocarbon generation, correction factors and a model of light hydrocarbon losing and heavy hydrocarbon handling were established. The results show that the correction factor of heavy hydrocarbon handling is 3.2, and that of light hydrocarbon losing is controlled by kerogen type, maturity and hydrocarbon generation environment（closed or open）.

Clonal immunoglobulin heavy chain and T-cell receptor γ gene rearrangements in primary gastric lymphoma

AIM:To study the diagnostic value of immunoglobulin heavy chain(IgH)and T-cell receptorγ (TCR-γ)gene monoclonal rearrangements in primary gastric lymphoma(PGL).METHODS:A total of 48 patients with suspected PGL at our hospital were prospectively enrolled in this study from January 2009 to December 2011.The patients were divided into three groups(a PGL group,a gastric linitis plastica group,and a benign gastric ulcer group)based on the pathological results(gastric mucosal specimens obtained by endoscopy or surgery)and follow-up.Endoscopic ultrasonography(EUS)and EUSguided biopsy were performed in all the patients.The tissue specimens were used for histopathological examination and for IgH and TCR-γ gene rearrangement polymerase chain reaction analyses.RESULTS:EUS and EUS-guided biopsy were successfully performed in all 48 patients.In the PGL group(n=21),monoclonal IgH gene rearrangements were detected in 14(66.7%)patients.A positive result for each set of primers was found in 12(57.1%),8(38.1%),and 4(19.0%)cases using FR1/JH,FR2/JH,and FR3/JH primers,respectively.Overall,12(75%)patients with mucosal-associated lymphoid tissue lymphoma(n=16)and 2(40%)patients with diffuse large B-cell lymphoma(n=5)were positive for monoclonal IgH gene rearrangements.No patients in the gastric linitis plastica group(n=17)and only one(10%)patient in the benign gastric ulcer group(n=10)were positive for a monoclonal IgH gene rearrangement.No TCRgene monoclonal rearrangements were detected.The sensitivity of monoclonal IgH gene rearrangements was 66.7%for a PGL diagnosis,and the specificity was96.4%.In the PGL group,8(100%)patients with stage IIE PGL(n=8)and 6(46.1%)patients with stage IE PGL(n=13)were positive for monoclonal IgH gene rearrangements.CONCLUSION:IgH gene rearrangements may be associated with PGL staging and may be useful for the diagnosis of PGL and for differentiating between PGL and gastric linitis plastica.

Effects of Light and Heavy Rare Earths on Anti-Degradation of Nodular Cast Iron

The effects of rare earths with different contents on anti-degradation of Y base heavy rare earths-magnesium and cerium base light rare earths-magnesium nodular cast iron were studied. Curves of the relationship between the spheroidization grade of liguid iron treated by different nodulizers and holding time were obtained. The results show that the more the remains of the rare earth, the longer the anti-degradation time, the liquid iron was treated either by Y-Mg-Si or by Ce-Mg-Si nodulizer. When the rare earth remains are above certain quantity, the spheroidization grade starts to decline. When liquid iron treated by either Y-Mg-Si or Ce-Mg-Si nodulizer with proper remains of rare earths about 0.04% 0.06%（mass fraction）, the anti-degradation is the best, and the remains of Mg being about 0.04% -0.07% （mass fraction）. Rare earths-Mg nodulizer with moderate RE remains is better than that of Mg-Si nodulizer in anti-degradation property. However, Y-Mg-Si nodulizer with moderate remins of Re is better than Ce-Mg-Si nodulizer. Sometimes Ce-Mg- Si nodulizer can be used for the production of heavy section nodular cast iron.

Induction of Cardiomyocyte Apoptosis by Anti-Cardiac Myosin Heavy Chain Antibodies in Patients with Acute Myocardial Infarction

Autoimmune is involved in the pathogenesis of ventricular remodeling in acute myocardial infarction (AMI).In the present study, we investigated the effect of anti-cardiac myosin heavy chain antibodies (AMHCA) from patients with AMI on rat cardiomyocyte apoptosis.Cardiomyocyte apoptosis was observed and measured by DNA end labeling and Annexin-Ⅴ/PI double-staining assay.The expression of apoptosis related p53 and Bcl-2 protein and the second messenger calcium were detected respectively by Western blotting, patch clamp and confocal calcium imaging.The results showed that AMHCA was able to induce cardiomyocyte apoptosis in a dose dependent manner.Apoptosis-accelerating nucleoprotein p53 was up-regulated, while apoptosis-inhibiting cytoplasmic protein Bcl-2 was down-regulated.In parallel, cytoplasmic calcium concentration was elevated.There was no effect on L-type calcium currents.It is concluded that AMHCA in patients with AMI as a novel triggering factor can induce cardiomyocyte apoptosis, which contributes to ventricular remodeling.

Molecular cloning and mRNA expression analysis of myosin heavy chain(MyHC)from fast skeletal muscle of grass carp,Ctenopharyngodon idella

The myosin heavy chain(MyHC)is one of the major structural and contracting proteins of muscle.We have isolated the cDNA clone encoding MyHC of the grass carp,Ctenopharyngodon idella. The sequence comprises 5 934 bp,including a 5 814 bp open reading frame encoding an amino acid sequence of 1 937 residues.The deduced amino acid sequence showed 69%homology to rabbit fast skeletal MyHC and 73%–76%homology to the MyHCs from the mandarin fish,walleye pollack,white croaker,chum salmon,and carp.The putative sequences of subfragment-1 and the light meromyosin region showed 61.4%–80%homology to the corresponding regions of other fish MyHCs.The tissue-specific and developmental stage-specific expressions of the MyHC gene were analyzed by quantitative real-time PCR.The MyHC gene showed the highest expression in the muscles compared with the kidney,spleen and intestine.Developmentally,there was a gradual increase in MyHC mRNA expression from the neural formation stage to the tail bud stage.The highest expression was detected in hatching larva.Our work on the MyHC gene from the grass carp has provided useful information for fish molecular biology and fish genomics.

Relationship between light and heavy fractions of organic matter for several agricultural soils in China

Although numerous studies about the nature and turnover of soil organic matter（SOM） in light and heavy fractions（ LFOM and HFQM, respectively） have been made, little information is available in relation to the relationship between LFQM and HFOM, and no attempts have been made to quantify a general relationship between LFQM and HFQM for agricultural soils under field condition. Qur hypothesis is there may be an inherent relationship between LFQM and HFQM for agricultural soils under certain unaltered management practices for a long period, to this end, we therefore studied typically soils taken from different parts in China by using a simple density fractionation procedure. The results indicated that LFQM was positively correlated with LFOM/HFOM ratio for three typical soils. This information will be of particular use not only in deepening our understanding of the dynamics of SQM fractions but also in evaluating the potential of agricultural soils to sequestrate C under different management practices in a long term.

Effect of aerobic exercise on the contractile function of gastrocnemius myosin heavy chain

Objective To study the effect of 4-6 weeks’ treadmill training of male SD rats on the contractile function of their gastrocnemius myosin heavy chain (MHC). Methods Forty male SD rats were randomly divided into control group and training group. The treadmill training of the training group rats was incessantly performed for 4-6 weeks at an intensity of about 75% VO2max (18.5-24 m/min,gradient of 0°,each training session lasting 50 minutes,twice a day). The content of gastrocnemius MHC mRNA was tested by reverse transcription polymerase chain reaction (RT-PCR),and the changes of muscle fibre and its cross-section area (CSA) were measured using immunohistochemistry. Electric stimulation tests were used to determine the maximal tension of isometric contraction of the post-training gastrocnemius. Results ① After continuous treadmill training for 4-6 weeks,we found that the content of the total MHC,MHC Ⅰ,MHC Ⅱx,MHC Ⅱa mRNAs was 105%,105%,109% and 108% of that in the resting control group,respectively,and the MHC Ⅱb mRNA content did not change significantly. The percentage of MHC Ⅰ mRNA in the total MHC mRNA increased while that of MHC Ⅱ mRNA decreased after aerobic training. ② The slow type of fibre type Ⅰ was the main part of the MHC after training and the CSA of the muscle fibres increased simultaneously. ③ The maximal tension of isometric contraction by pulse stimulation of square wave in the training group increased significantly compared with that in the control group (P<0.01). Conclusion The findings indicate that aerobic exercise may promote an increase in the contractile function of MHC.

Agaro-Oligosaccharides Prevent Myostatin Hyperexpression and Myosin Heavy Chain Protein Degradation in C2C12 Myotubes Induced by Tumor Necrosis Factor-<i>α</i>

Myostatin is a major factor involved in the regulation of skeletal muscle protein mass. High myostatin levels have been associated with an increase in myotube shrinkage. Enhanced myostatin expression is caused by pro-catabolic reactions involving compounds such as tumor necrosis factor (TNF)-α. The present study investigated the effects of agaro-oligosaccharides (AOSs) on hypercatabolism of myotubes exposed to TNF-α. C2C12 myotubes exposed to TNF-α in the presence or absence of AOSs. Myotube exposure to TNF-α resulted in a reduction in the amount of myosin heavy chain (MyHC) protein and a decrease in myotube diameter, which was associated with increased myostatin mRNA expression. AOSs prevented TNF-α-induced MyHC protein loss and restored normal myostatin mRNA levels, with agarobiose and agarotetraose effectively suppressing the hyperexpression of the mRNA. In addition, expression levels of the known myostatin inhibitors, latent transforming growth factor beta binding protein 3 (Ltbp3) and growth and differentiation factor-associated serum protein 1 (Gasp1) mRNAs, decreased more in TNF-α-induced myotubes than in the TNF-α-free control, possibly resulting in myostatin upregulation. However, AOSs restored nearly normal expression levels of Ltbp3 and Gasp1 mRNA, potentially suppressing myostatin expression. These findings suggest that AOSs could prevent myotube shrinkage induced by TNF-α.

Isolation and Characterization of Recombinant Variable Domain of Heavy Chain Anti-idiotypic Antibodies Specific to Aflatoxin B_1

Some unique subclasses of Camelidae antibodies are devoid of the light chain, and the antigen binding site is comprised exclusively of the variable domain of the heavy chain （VHH）. The recombinant VHHs have a high potential as alternative reagents for the next generation of immunoassay. In particular, they might be very useful for molecular mimicry. The present study demonstrated an alpaca immunized with the F（ab＇）z fragment of anti-aflatoxin B1 mAb and developed an important anti-idiotypic （anti-ld） responses. Antigen-specific elution method was used for panning private anti-ld VHHs from the constructed alpaca VHH library. The selected VHHs were expressed, renatured, purified, and then identified by a competitive enzyme-linked immunosorbent assay （ELISA）. Our findings indicated that the VHH would be an alternative tool for haptens mimicry studies.

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.