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.

Creutzfeldt-Jakob disease presenting as Korsakoff syndrome caused by E196A mutation in PRNP gene:A case report

BACKGROUND Prion diseases are a group of degenerative nerve diseases that are caused by infectious prion proteins or gene mutations.In humans,prion diseases result from mutations in the prion protein gene(PRNP).Only a limited number of cases involving a specific PRNP mutation at codon 196(E196A)have been reported.The coexistence of Korsakoff syndrome in patients with Creutzfeldt-Jakob disease(CJD)caused by E196A mutation has not been documented in the existing literature.CASE SUMMARY A 61-year-old Chinese man initially presented with Korsakoff syndrome,followed by rapid-onset dementia,visual hallucinations,akinetic mutism,myoclonus,and hyperthermia.The patient had no significant personal or familial medical history.Magnetic resonance imaging of the brain revealed extensive hyperintense signals in the cortex,while positron emission tomography/computed tomography showed a diffuse reduction in cerebral cortex metabolism.Routine biochemical and microorganism testing of the cerebrospinal fluid(CSF)yielded normal results.Tests for thyroid function,human immunodeficiency virus,syphilis,vitamin B1 and B12 levels,and autoimmune rheumatic disorders were normal.Blood and CSF tests for autoimmune encephalitis and autoantibody-associated paraneoplastic syndrome yielded negative results.A test for 14-3-3 protein in the CSF yielded negative results.Whole-genome sequencing revealed a diseasecausing mutation in PRNP.The patient succumbed to the illness 11 months after the initial symptom onset.CONCLUSION Korsakoff syndrome,typically associated with alcohol intoxication,also manifests in CJD patients.Individuals with CJD along with PRNP E196A mutation may present with Korsakoff syndrome.

Culture Self- Consciousness in Transmission of Chinese Philosophy from the Perspective of English Globalization -- Taking the Belt and Road Initiative for Example

The Belt and Road Initiative contains the aspiration of Chinese nation to pursue ideological freedom and desire of strengthening international exchanges and cooperation. This paper takes the Belt and Road Initiative for example,emphasizing the interpretation of the inheritance and development of Chinese culture so as to study the importance of Culture Self-Consciousness in Chinese Philosophy,in the context of English globalization. That is to say,this paper stresses the spirits and the stand of nation 's demonstration on pursuing peace,cooperation and sustainable development in the civilization continuity from traditional China to the contemporary China,with practical philosophic view.

Lateral resonances in initial stressed 1-3 piezocomposites

A theoretical analysis of the lateral resonances in 1-3 piezocomposites with poling initial stress is conducted using the Bloch wave theory. Based on the linear piezoelectricity theory, theoretical formulations that include initial stress for the propagation of acoustic plane waves are made. Numerical calculations are performed to study the effects of the initial stress on the lateral mode frequencies and the stop band. It is found that lateral mode frequencies increase with the piezoelectricity of the piezocomposites, but decrease with the poling initial stress. The influence of the initial shear stress on the lateral mode frequencies is minimal, and can thus be neglected.

17α-hydroxylase/17,20 carbon chain lyase deficiency caused by p.Tyr329fs homozygous mutation:Three case reports

p.Tyr329fs is a cytochrome P450c17 mutation among Chinese individuals.However,data on 17-α-hydroxylase deficiency caused by cytochrome P450c17 p.Tyr329fs homozygous mutation are lacking.This paper is a case report of three patients homozygous for p.Tyr329fs who were diagnosed with 17-α-hydroxylase deficiency between 2005 and 2019.CASE SUMMARY Case 1 presented with hypertension,hypokalemia,sexual infantilism and delayed bone age.The patient had a 46,XY karyotype,was homozygous for p.Tyr329fs and was recently treated with dexamethasone 0.375 mg qn.Case 2 presented with hypokalemia,sexual infantilism,osteoporosis and delayed bone age.The patient had a 46,XY karyotype,was homozygous for p.Tyr329fs and was treated with dexamethasone 0.75 mg qn at the last follow-up.Serum potassium and blood pressure could be maintained within normal range for cases 1 and 2.Case 3 presented with amenorrhea,sexual infantilism,osteopenia and delayed bone age.The patient had a 46,XX karyotype,was homozygous for p.Tyr329fs and was treated with dexamethasone 0.75 mg qn and progynova 1 mg qd.Outpatient follow-up revealed an adrenocorticotropic hormone(8 AM)of<5.00 pg/mL.CONCLUSION The homozygous p.Tyr329fs mutation usually manifests as a combined deficiency,and definitive diagnosis depends primarily on genetic testing.

Successful multidisciplinary therapy for a patient with liver metastasis from ascending colon adenocarcinoma:A case report and review of literature

BACKGROUND Liver metastasis is the most common form of distant metastasis in colorectal cancer,and the only possible curative treatment for patients with colorectal liver metastases(CRLM)is hepatectomy.However,approximately 25%of patients with CRLM have indications for liver resection at the initial diagnosis.Strategies aimed at downstaging large or multifocal tumors to enable curative resection are appealing.CASE SUMMARY A 42-year-old man was diagnosed with ascending colon cancer and liver metastases.Due to the huge lesion size and compression of the right portal vein,the liver metastases were initially diagnosed as unresectable lesions.The patient was treated with preoperative transcatheter arterial chemoembolization(TACE)consisting of 5-fluorouracil/Leucovorin/oxaliplatin/Endostar®.After four courses,radical right-sided colectomy and ileum transverse colon anastomosis were performed.Postoperatively,the pathological analysis revealed moderately differentiated adenocarcinoma with necrosis and negative margins.Thereafter,S7/S8 partial hepatectomy was performed after two courses of neoadjuvant chemotherapy.Pathological examination of the resected specimen revealed a pathologically complete response(pCR).Intrahepatic recurrence was detected more than two months after the operation,and the patient was then treated with TACE consisting of irinotecan/Leucovorin/fluorouracil therapy plus Endostar®.Subsequently,the patient was treated with aγ-knife to enhance local control.Notably,a pCR was reached,and the patient's overall survival time was>9 years.CONCLUSION Multidisciplinary treatment can promote the conversion of initially unresectable colorectal liver metastasis and facilitate complete pathological remission of liver lesions.

Obama Team Overseeing ITA Transition Expected to Submit Initial Report

The team appointed by President-elect Obama to oversee the transition at the U.S. Commerce Department’s International Trade Administration (ITA) plans to put together its initial report on its ongoing on-site review of

State of Country Report——Foreign Trade 2009-2010

Facing economic crisis in the world 2009, international market demands suffered a serious recession, also China's foreign trade has encountered unprecedented difficulties. In front of this, Chinese Government takes series of adjustment timely to get over this rough time. So how is China's foreign trade in 2009, and where it will go in 2010, let's have a look at this…

基于QAR数据的波音747-400飞机巡航报文解析研究（英文）

为充分挖掘QAR数据中所包含的大量原始监控数据,检验ACARS报文的可靠性,更好地协助航空公司进行飞机和发动机的状态监控及故障诊断,对发动机稳态报文DFD的报文结构进行了解析研究,提出了一种从QAR数据中提取满足触发条件的DFD报文的研究思路和方法,以丰富发动机性能监控的信息量。通过对B747-400飞机实际ACARS报文数据和所提取的报文数据进行计算分析,验证了该方法的可靠性,具有良好的工程应用前景。

“一带一路”倡议十周年俄媒报道现状调查

2023年是“一带一路”倡议提出十周年,在此期间,俄罗斯主流媒体进行了较为广泛的报道。利用道琼斯全球新闻数据库,以2014年以来的媒体报道情况为依据进行定量分析发现,在俄媒近700篇相关报道中,客观中立的立场成为主流,外交领域成为主线,“一带一路”国际合作高峰论坛成为焦点,并发挥了积极的舆论牵引作用。俄罗斯突显出基于本国视角的针对性审视,密切关注由其推动的欧亚经济联盟同“一带一路”之间的相互影响。

“一带一路”倡议在非洲传播分析——以泛非通讯社法语版报道为例

以非洲泛非通讯社中有关“一带一路”的法语报道为文本,通过数据梳理揭示“一带一路”倡议在非洲的宣传现状和报道重点。研究表明:非洲国家对“一带一路”倡议整体持积极正面态度,但报道的体裁、主题和深度具有局限性,亲民度欠缺。在未来的宣传中,可进一步加强中非媒体间的互动,丰富报道题材,以促进中国和非洲国家间更好的合作互信。

Murine hepatocellular carcinoma derived stem cells reveal epithelial-to-mesenchymal plasticity

AIM To establish a model to enrich and characterize stemlike cells from murine normal liver and hepatocellular carcinoma(HCC) cell lines and to further investigate stem-like cell association with epithelial-to-mesenchymal transition(EMT).METHODS In this study,we utilized a stem cell conditioned serumfree medium to enrich stem-like cells from mouse HCC and normal liver cell lines,Hepa 1-6 and AML12,respectively.We isolated the 3-dimensional spheres and assessed their stemness characteristics by evaluating theRNA levels of stemness genes and a cell surface stem cell marker by quantitative reverse transcriptase-PCR(q RTPCR).Next,we examined the relationship between stem cells and EMT using q RT-PCR.RESULTS Three-dimensional spheres were enriched by culturing murine HCC and normal hepatocyte cell lines in stem cell conditioned serum-free medium supplemented with epidermal growth factor,basic fibroblast growth factor and heparin sulfate.The 3-dimensional spheres had enhanced stemness markers such as Klf4 and Bmi1 and hepatic cancer stem cell(CSC) marker Cd44 compared to parental cells grown as adherent cultures.We report that epithelial markers E-cadherin and ZO-1 were downregulated,while mesenchymal markers Vimentin and Fibronectin were upregulated in 3-dimensional spheres.The 3-dimensional spheres also exhibited changes in expression of Snai,Zeb and Twist family of EMT transcription factors.CONCLUSION Our novel method successfully enriched stem-like cells which possessed an EMT phenotype.The isolation and characterization of murine hepatic CSCs could establish a precise target for the development of more effective therapies for HCC.

Unexpected metastasis of intraductal papillary neoplasm of the bile duct without an invasive component to the brain and lungs:A case report

BACKGROUND Despite an expanding number of studies on intraductal papillary neoplasm of the bile duct(IPNB),distant metastasis remains unexplained especially in cases of carcinoma in situ.In the present study,we report a rare and interesting case of IPNB without invasive components that later metastasized to lungs and brain.CASE SUMMARY A 69-year-old male was referred to our hospital due to suspected cholangiocarcinoma.Laboratory tests on admission reported a mild elevation of alkaline phosphatase,γ-glutamyl transpeptidase,and total bilirubin in serum.Endoscopic retrograde cholangiography revealed a filling defect in the common bile duct(CBD)extending to the left hepatic duct.Peroral cholangioscopy delineated a tumor in the CBD that had a papillary pattern.Multidetector computed tomography and magnetic resonance cholangiopancreatography detected partial blockage ot interlude in the CBD leading to cholestasis without evidence of metastasis.Therefore,a diagnosis of IPNB cT1N0M0 was established.Left hepatectomy with bile duct reconstruction was performed.Pathological examination confirmed an intraepithelial neoplasia pattern without an invasive component and an R0 resection achievement.The patient was monitored carefully by regular examinations.However,at 32 mo after the operation,a 26 mm tumor in the lungs and a 12 mm lesion in the brain were detected following a suspicious elevated CA 19-9 level.Video-assisted thoracoscopic surgery of left upper lobectomy and stereotactic radiotherapy are indicated.In addition to histopathological results,a genomic profiling analysis using whole exome sequencing subsequently confirmed lung metastasis originating from bile duct cancer.CONCLUSION This case highlights the important role of genomic profiling analysis using whole exome sequencing in identifying the origin of metastasis in patients with IPNB.

Giant simple hepatic cyst with multiple elevated serum tumormarkers: A case report

BACKGROUND Simple hepatic cysts are relatively common in adults, and mostly appear asasymptomatic incidental radiologic findings. Occasionally, a large cyst will causesymptoms. Elevations in the serum biomarkers protein induced by vitamin Kabsence (PIVKA)-II, cancer antigen (CA) 12-5, and CA19-9 are often associatedwith malignant tumors in the liver or bile ducts. This is the first report to describea case of hepatic cyst with elevated levels of PIVKA-II and CA12-5.CASE SUMMARY An 84-year-old Chinese woman was admitted with gradual abdominal distension.Her symptoms started 1 year ago, and she had poor appetite and a weight loss of5 kg within the past 2 wk. She denied any symptoms associated with abdominalpain, fever and chills, nausea and vomiting, etc. The abdomen was enlarged, morein the right upper quadrant, without tenderness. Laboratory examination showedsignificantly increased serum levels of PIVKA-II, CA12-5, and CA19-9. Acomputed tomography scan revealed multiple round cysts in the liver with clearboundaries. The largest cyst was 20.1 cm × 12.2 cm × 19.6 cm in size, located in theright lobe of the liver with mild dilatation of the intrahepatic bile duct, but therewas no contrast enhancement. Percutaneous drainage on the largest hepatic cystand polycinnamol sclerosing agent injection into the cyst cavity were performed.After treatment, the patient’s symptoms relieved and the elevated serum tumormakers reduced to the normal levels dramatically.CONCLUSION The present report identifies an unusual case of a giant hepatic cyst with markedelevation of serum tumor marker levels of PIVKA-II, CA12-5, and CA19-9. Aftertreatment, these three serum markers dramatically decreased to normal levels.The mechanisms for the elevation of these tumor markers may be as follows: (1) Agiant hepatic cyst compresses the liver, causing injury to the hepatocytes, whichmay lead to secretion of a large amount of PIVKA-II;and (2) Some tumorassociatedantigens, such as carcinoembryonic antigen, CA19-9, CA12-5, andCA15-3, are expressed on inflammatory cells.

Reporting on Land as Natural Capital by Gold Mining Houses in South Africa

Motivated by the increasing popularity and advocating for integrated reporting by companies on their operations,the aim of this paper is to determine the disclosure levels on land by mining houses in South Africa in their integrated reports.Developing countries,such as South Africa should ensure sustainability of their natural resources in their endeavour of growing their economies.The mining sector in South Africa is a huge contributor to the economy,but also has a significant impact on land conservation.The complexity of mining activities and impacts thereof make it challenging for organisations to disclose information on land.This paper is descriptive by design;it contains a literature review,followed by a content analysis of the mining houses integrated reports by using a checklist.The checklist was based on recommendations by the Global Reporting Initiative(GRI)guidelines as to what the content of the integrated report on land should be.Fourteen mining houses operating in South Africa and registered on the Johannesburg Stock Exchange(JSE)were identified and analysed for this study.The findings of the paper display low levels of overall disclosure on land by the South African mining companies.However,there were high levels of disclosure for EN11,EN12,and MM1 sector-specific supplement in the integrated reports.Low levels of disclosure by the mining companies were evident in MM2 the sector-specific recommendations as well as the physical and monetary information related to MM2.

Searching for initial stage of massive star formation around the HⅡ region G18.2–0.3

Sometimes, early star formation can be found in cold and dense molecular clouds, such as an infrared dark cloud. Considering that star formation often occurs in clusters, H II regions may be triggering a new generation of star formation, so we can search for the initial stage of massive star formation around H II regions. Based on the above, this work introduces one method to search for the initial stage of massive star formation around H II regions. Towards one section of the H II region G18.2-0.3, multiwavelength observations are carried out to investigate its physical properties. Through analysis, we find three potential initial stages of massive star formation, suggesting that it is feasible to use in searching for the initial stage of massive star formation around H II regions.

Evaluation of Sustainability Reporting Practices in Turkey

The purpose of the present study is to determine the reflection of sustainability reporting （SR）, which gained momentum considerably during the recent years, on the organizations that are active in Turkey. Turkish businesses that carried out SR during January 1, 2005 and June 1, 2015 were included in the study. Altogether, 186 sustainability reports containing 6,240 pages of a total of 80 businesses were examined. Data obtained from these reports were classified into various categories. According to the results, it was determined that 84% of the reporting businesses reported according to Global Reporting Initiative （GRI） principles and preferred G3 framework （40%）; and the maximum number of reporting was published in 2014. The present study is the first research article including all businesses making SR in Turkey with all details.