Primary systemic treatment is a fundamental part of breast cancer therapy, and it is applied to non-surgical and locally advanced tumours as well as surgical tumours to increase the likelihood of conservative treatmen...Primary systemic treatment is a fundamental part of breast cancer therapy, and it is applied to non-surgical and locally advanced tumours as well as surgical tumours to increase the likelihood of conservative treatment. Its aim is to achieve the best possible survival with better cosmetic results and with the lowest number of treatment-related secondary effects. Before treatment is started, it is necessary to attain the best knowledge of the biological features and locoregional extension of the tumour. To do so, it is necessary to obtain a biopsy of the lesion with a wide bore needle,as well as good radiological knowledge of the disease.Therefore, currently, the use of a dynamic magnetic resonance imaging(MRI) of the breast should be included in all cases. In addition, before it is started,especially in those tumours in which conservative treatment is considered, one or several radiopaque markers should be put into place to make it possible to locate the area to be treated if there is a considerable or complete response. Systemic treatment is mainly based on combined chemotherapy with anthracyclins and taxanes, in addition to some biological agents with demonstrated efficiency for increasing the likelihood of complete disease response(trastuzumab in patients with Her-2/neu overexpression). However, there is room for neoadjuvant hormone treatment, in patients with hormone receptor overexpression, especially in those cases in which chemotherapy is contraindicated as well as in elderly patients with a relatively short life expectancy. The assessment of preoperative treatment should be based on adequate radiological tests, and nowad these should include MRI before taking decisions about adequate surgical treatment. The objective of primary treatment is to be able to increase survival and improve the chances of local treatment in the case of locally advanced treatment, achieving results that are at least equal to those of adjuvant treatment in the case of surgical tumours, but with greater chances of conservative surgery. Although the objective is survival, achieving complete pathological response seems to be a reasonable related objective, although these are more closely linked in some tumour subtypes.展开更多
BACKGROUND Aseptic loosening remains the leading cause of revision in primary total hip arthroplasty(pTHA).However,the literature demonstrates significant variability regarding the relative contributions of different ...BACKGROUND Aseptic loosening remains the leading cause of revision in primary total hip arthroplasty(pTHA).However,the literature demonstrates significant variability regarding the relative contributions of different factors.AIM To investigate the key determinants of aseptic loosening,we performed a systematic review and meta-analysis.METHODS A comprehensive search of PubMed,Web of Science,EMBASE,and the Cochrane Library was conducted,encompassing studies from database inception to January 1,2025.Meta-analyses were performed to evaluate factors associated with aseptic loosening following pTHA.Inclusion and exclusion criteria were systematically applied at each stage to ensure methodological transparency and reproducibility.Study quality was assessed using standardized categories.Pooled odds ratio(OR)with corresponding 95%confidence interval were calculated with random-or fixed-effects models to generate reliability estimates,and study heterogeneity was visualized using forest plots.Ten factors,categorized into patient-,surgeon-,and device-related domains,were reviewed and meta-analyzed.Funnel plot analysis demonstrated a relatively symmetrical distribution,suggesting minimal publication bias.RESULTS A meta-analysis of 20 studies(520789 participants)found a pooled prevalence of 1.96%.Significant risk factors for aseptic loosening after pTHA included elevated body mass index(OR=1.116,P<0.001),higher Charlson comorbidity index(OR=1.378,P<0.001),prosthesis-related factors(OR=1.497,P<0.001),and adverse lifestyles(OR=2.198,P=0.037).Protective factors were non-white race(OR=0.445,P<0.001)and favorable genetics(OR=0.723,P<0.001).Male sex increased risk(OR=1.232,P=0.016),while age and anatomy were not significant.Surgical expertise showed a slight protective effect(OR=1.048,P<0.001).A comprehensive understanding of the modifiable and non-modifiable factors contributing to aseptic loosening after pTHA requires consideration of patient-related factors,surgical expertise,and prosthesis characteristics.CONCLUSION The identification of these factors is critical for risk mitigation.High-risk patients should receive targeted counseling regarding individualized profiles.Further studies are warranted to establish clearer causal relationships and identify additional contributing factors.展开更多
BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right...BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right heart.Late diagnosis mimics common cardiac ailments,leading to poor prognosis.AIM To systematically review the efficacy of rituximab in treating PCL either alone or in various chemotherapeutic regimens.Secondary objectives include evaluating morphological subtypes,assessing treatment regimens,and analyzing outcomes focusing on remission and adverse events.METHODS Following PRISMA guidelines,a comprehensive literature search was conducted across multiple databases,including PubMed,Hinari,Web of Science,and Scopus.English-language studies reporting the use of rituximab in treating PCL in humans were included.Study selection involved initial screening of titles and abstracts followed by full-text examination and data extraction.RESULTS Thirty-three case reports involving 36 patients were included in this systematic review.Diffuse large B-cell lymphoma was the predominant morphological subtype observed.The rituximab,cyclophosphamide,doxorubicin,oncovin,and prednisolone regimen emerged as the most commonly employed treatment strategy,indicating widespread acceptance and efficacy in PCL management.Combination therapies,including surgical intervention,showed promise in achieving complete remission,while some studies reported mortality despite aggressive treatment approaches.CONCLUSION Rituximab,particularly in combination with chemotherapy regimens,represents a significant advancement in PCL management,offering hope for improved patient outcomes.However,challenges such as variable treatment responses and adverse events underscore the complexity of managing PCL.Further research is warranted to refine therapeutic strategies and enhance diagnostic approaches for this rare cardiac malignancy.展开更多
Casein kinase 1(CK1)is an important member of the serine/threonine protein kinase family,playing a crucial role in various cellular processes,including cell cycle regulation,signal transduction,DNA repair,and circadia...Casein kinase 1(CK1)is an important member of the serine/threonine protein kinase family,playing a crucial role in various cellular processes,including cell cycle regulation,signal transduction,DNA repair,and circadian rhythm control.CK1 is also essential in the nervous system,where it regulates neuronal growth,differentiation,and synaptic plasticity.Studies have shown that CK1δ phosphorylates neuron-specific proteins to regulate axonal growth and synaptogenesis.Primary cilia are non-motile microtubule structures present on the surface of most mammalian cells.Recent studies have revealed their multiple roles in cellular physiology and development,and dysfunction of cilia can impact the development and function of the nervous system.CK1 has an important role in the formation and function of primary cilia.By regulating various signaling pathways and the phosphorylation status of proteins,CK1 affects the generation,maintenance,and signaling transduction of cilia.In this review,the relationship between CK1,primary cilia,and the nervous system was explored,focusing on how CK1 influences cilia to regulate the structure and function of the nervous system.展开更多
In the current era of digitalization sweeping the education field,primary school English education is facing new challenges and opportunities of deep integration with artificial intelligence.This study focuses on prim...In the current era of digitalization sweeping the education field,primary school English education is facing new challenges and opportunities of deep integration with artificial intelligence.This study focuses on primary school English teachers and uses various methods such as questionnaire surveys,visits,and interviews to conduct an in-depth exploration of their artificial intelligence literacy.After data analysis,optimization strategies are proposed to further improve the artificial intelligence literacy of primary school English teachers and promote the development of educational soft power.展开更多
Nonhuman primates are increasingly being used as animal models in neuroscience research.However,efficient neuronal tracing techniques for labeling motor neurons and primary sensory afferents in the monkey spinal cord ...Nonhuman primates are increasingly being used as animal models in neuroscience research.However,efficient neuronal tracing techniques for labeling motor neurons and primary sensory afferents in the monkey spinal cord are lacking.Here,by injecting the cholera toxin B subunit into the sciatic nerve of a rhesus monkey,we successfully labeled the motor neurons and primary sensory afferents in the lumbar and sacralspinal cord.Labeled alpha motor neurons were located in lamina IX of the L6–S1 segments,which innervate both flexors and extensors.The labeled primary sensory afferents were mainly myelinated Aβfibers that terminated mostly in laminae I and II of the L4–L7 segments.Together with the labeled proprioceptive afferents,the primary sensory afferents formed excitatory synapses with multiple types of spinal neurons.In summary,our methods successfully traced neuronal connections in the monkey spinal cord and can be used in spinal cord studies when nonhuman primates are used.展开更多
AIM:To investigate the long-term outcomes in acute primary angle closure(APAC)patients treated with lens extraction(LE)surgery and to identify risk factors for glaucomatous optic neuropathy(GON).METHODS:In this longit...AIM:To investigate the long-term outcomes in acute primary angle closure(APAC)patients treated with lens extraction(LE)surgery and to identify risk factors for glaucomatous optic neuropathy(GON).METHODS:In this longitudinal observational study,detailed medical histories of APAC patients and comprehensive ophthalmic examinations at final followup were collected.Logistic regression analysis was performed to identify predictors of blindness.Univariate and multivariate linear regression analyses were conducted to determine risk factors associated with visual outcomes.RESULTS:This study included 39 affected eyes of 31 subjects(26 females)with an average age of 74.1±8.0y.At 6.7±4.2y after APAC attack,2(5.7%)eyes had bestcorrected visual acuity(VA)worse than 3/60.Advanced glaucomatous visual field loss was observed in 15(39.5%)affected eyes and 5(25.0%)fellow eyes.Nine affected eyes(23.7%)had GON,and 11(28.9%)were blind.Six(15.4%)affected eyes and 2(9.1%)fellow eyes had suspicious progression.A significantly higher blindness rate in factory workers compared to office workers.Logistic regression identified that worse VA at attack(OR 10.568,95%CI 1.288-86.695;P=0.028)and worse early postoperative VA(OR 13.214,95%CI 1.157-150.881;P=0.038)were risk factors for blindness.Multivariate regression showed that longer duration of elevated intraocular pressure(P=0.004)and worse early postoperative VA(P=0.009)were associated with worse visual outcomes.CONCLUSION:Despite LE surgery,some APAC patients experience continued visual function deterioration.Lifelong monitoring is necessary.Target pressure and progression rates should be re-evaluated during follow-up.展开更多
AIM:To study the relationships between amplitude of low-frequency fluctuations(ALFF)changes and clinical ophthalmic parameters in patients with primary open angle glaucoma(POAG)and analyze the diagnostic value of ALFF...AIM:To study the relationships between amplitude of low-frequency fluctuations(ALFF)changes and clinical ophthalmic parameters in patients with primary open angle glaucoma(POAG)and analyze the diagnostic value of ALFF.METHODS:Twenty-four POAG patients and 24 healthy controls(HCs)underwent resting-state functional magnetic resonance imaging(rs-fMRI).Nonparametric rank-sum tests were used to compare the ALFF values in the slow-4 and slow-5 bands,and Spearman or Pearson correlation analysis was used to assess the correlation between ALFF changes and clinical ophthalmic parameters in POAG patients.Receiver operating characteristic(ROC)curves were used to evaluate the diagnostic performance of the ALFF.RESULTS:There were 16 males in POAG patients(median age 48y)and 12 males in HCs(median age 39y).Compared with HCs,POAG patients presented increased or decreased ALFF values in different brain regions,and similar changes were observed in mild POAG patients.The ALFF values were correlated with retinal nerve fiber layer(RNFL)thickness,inner limiting membrane-retinal pigment epithelium thickness changes and the degree of visual field defects.Analysis of the diagnostic value of the ALFF via ROC curves revealed that the right medial frontal gyrus[area under the curve(AUC)=0.9063]and superior frontal gyrus(AUC=0.9097)had better diagnostic value than did the optic disc area(AUC=0.8019),visual field index(VFI%,AUC=0.8988)and macular parameters.CONCLUSION:POAG patients present altered cortical function that is significantly correlated with the optic nerve and retinal thickness and had good diagnostic value,which may reflect the underlying neuropathological mechanism of POAG.展开更多
Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore wh...Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.展开更多
Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract atten...Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion,as well unusual skin vegetations.Case presentation:A 61-year-old man was hospitalized due to repeated burning sensation on his back,multiple ecchymosis,and skin vegetations.Through a series of examinations(mainly including skin histopathology,bone marrow cytology,bone marrow flow cytometry,immunofixation electrophoresis),Primary systemic amyloidosis was diagnosed,but multiple myeloma could not be diagnosed.Subsequently,he received chemotherapy.In the half-year follow-up,there was no significant change in his symptoms and signs.Discussion:In this case,in addition to the typical skin damage of primary amyloidosis,the multiple skin vegetations in the buttocks,abdomen,and arms are particularly noteworthy.According to the histopathology and Immunohistochemistry of the skin vegetation,we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid,which leads to lymphatic dilatation,lymph leakage,and dermal edema.Conclusion:Primary systemic amyloidosis is a rare disease,which is often difficult to diagnose.We should be alert to those atypical skin features so as not to delay diagnosis.展开更多
Objective To investigate the difference in tumor conventional imaging findings and texture features on T2 weighted images between glioblastoma and primary central neural system(CNS) lymphoma. Methods The pre-operative...Objective To investigate the difference in tumor conventional imaging findings and texture features on T2 weighted images between glioblastoma and primary central neural system(CNS) lymphoma. Methods The pre-operative MRI data of 81 patients with glioblastoma and 28 patients with primary CNS lymphoma admitted to the Chinese PLA General Hospital and Hainan Hospital of Chinese PLA General Hospital were retrospectively collected. All patients underwent plain MR imaging and enhanced T1 weighted imaging to visualize imaging features of lesions. Texture analysis of T2 weighted imaging(T2 WI) was performed by use of GLCM texture plugin of ImageJ software, and the texture parameters including Angular Second Moment(ASM), Contrast, Correlation, Inverse Difference Moment(IDM), and Entropy were measured. Independent sample t-test and Mann-Whitney U test were performed for the between-group comparisons, regression model was established by Binary Logistic regression analysis, and receiver operating characteristic(ROC) curve was plotted to compare the diagnostic efficacy. Results The conventional imaging features including cystic and necrosis changes(P = 0.000), ‘Rosette' changes(P = 0.000) and ‘incision sign'(P = 0.000), except ‘flame-like edema'(P = 0.635), presented significantly statistical difference between glioblastoma and primary CNS lymphoma. The texture features, ASM, Contrast, Correlation, IDM and Entropy, showed significant differences between glioblastoma and primary CNS lympoma(P = 0.006,0.000, 0.002, 0.000, and 0.015 respectively). The area under the ROC curve was 0.671, 0.752, 0.695, 0.720 and 0.646 respectively, and the area under the ROC curve was 0.917 for the combined texture variables(Contrast, cystic and necrosis, ‘Rosette' changes, and ‘incision sign') in the model of Logistic regression. Binary Logistic regression analysis demonstrated that cystic and necrosis changes, ‘Rosette' changes and ‘incision sign' and texture Contrast could be considered as the specific texture variables for the differential diagnosis of glioblastoma and primary CNS lymphoma. Conclusion The texture features of T2 WI and conventional imaging findings may be used to distinguish glioblastoma from primary CNS lymphoma.展开更多
Background： Common bile duct （CBD） stones may occur in up to 3%–14.7% of all patients with cholecystectomy.Various approaches of laparoscopic CBD exploration plus primary duct closure （PDC） are the most commonly...Background： Common bile duct （CBD） stones may occur in up to 3%–14.7% of all patients with cholecystectomy.Various approaches of laparoscopic CBD exploration plus primary duct closure （PDC） are the most commonly used and the best methods to treat CBD stone. This systematic review was to comparethe effectiveness and safety of the various approaches of laparoscopic CBD exploration plus PDC forcholedocholithiasis展开更多
Net primary productivity （NPP） is the structure and function of the ecosystem. NPP can most important index that represents the be simulated by dynamic global vegetation models （DGVM）, which are designed to repres...Net primary productivity （NPP） is the structure and function of the ecosystem. NPP can most important index that represents the be simulated by dynamic global vegetation models （DGVM）, which are designed to represent vegetation dynamics relative to environ- mental change. This study simulated the NPP of China＇s ecosystems based on the DGVM Integrated Biosphere Simulator （IBIS） with data on climate, soil, and topography. The appli- cability of IBIS in the NPP simulation of China＇s terrestrial ecosystems was verified first. Comparison with other relevant studies indicates that the range and mean value of simula- tions are generally within the limits of observations; the overall pattern and total annual NPP are close to the simulations conducted with other models. The simulations are also close to the NPP estimations based on remote sensing. Validation proved that IBIS can be utilized in the large-scale simulation of NPP in China＇s natural ecosystem. We then simulated NPP with climate change data from 1961 to 2005, when warming was particularly striking. The following are the results of the simulation. （1） Total NPP varied from 3.61 GtC/yr to 4.24 GtC/yr in the past 45 years and exhibited minimal significant linear increase or decrease. （2） Regional differences in the increase or decrease in NPP were large but exhibited an insignificant overall linear trend. NPP declined in most parts of eastern and central China, especially in the Loess Plateau. （3） Similar to the fluctuation law of annual NPP, seasonal NPP also displayed an insignificant increase or decrease; the trend line was within the general level. （4） The re- gional differences in seasonal NPP changes were large. NPP declined in spring, summer, and autumn in the Loess Plateau but increased in most parts of the Tibetan Plateau.展开更多
The Three-River Headwater Region（TRHR）, known as the ＂Water Tower of China＂, is an important ecological shelter for national security interests and regional sustainable development activities for many downstream r...The Three-River Headwater Region（TRHR）, known as the ＂Water Tower of China＂, is an important ecological shelter for national security interests and regional sustainable development activities for many downstream regions in China and a number of Southeast Asian countries. The TRHR is a high-elevation, cold environment with a unique, but typical alpine vegetation system. Net primary productivity（NPP） is a key vegetation parameter and ecological indicator that can reflect both natural environmental changes and carbon budget levels. Given the unique geographical environment and strategic location of the TRHR, many scholars have estimated NPP of the TRHR by using different methods; however, these estimates vary greatly for a number of reasons. To date, there is no paper that has reviewed and assessed NPP estimation studies conducted in the TRHR. Therefore, in this paper, we（1） summarized the related methods and results of NPP estimation in the TRHR in a systematic review of previous research;（2） discussed the suitability of existing methods for estimating NPP in the TRHR and highlighted the most significant challenges; and（3） assessed the estimated NPP results. Finally, developmental directions of NPP estimation in the TRHR were prospected.展开更多
Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate...Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.展开更多
BACKGROUND: The life expectancy of a patient with primary hepatic carcinoma (PHC) is hard to predict, and it is related to many prognostic factors. The Chinese classification system including five parameters: tumor, v...BACKGROUND: The life expectancy of a patient with primary hepatic carcinoma (PHC) is hard to predict, and it is related to many prognostic factors. The Chinese classification system including five parameters: tumor, vascular thrombosis, lymph node metastasis, distant metastasis and Child-Pugh stage developed in 1999 was adopted by the 8th National Conference on liver Cancer of the Chinese Anti-Cancer Association in 2001. In this study, the discriminatory ability of the Chinese classification system -was compared with that of the TNM staging in patients for resection of PHC, in addition to the evaluation of prognostic value. METHODS: The data of 246 patients who had undergone resection of PHC from January 1986 to December 2000 (average age, 51 years; male/female ratio, 213/33) were retrospectively studied. Among the 246 patients, 227 were followed up for at least 3 years. RESULTS: The 1-, 3-, 5-, 7-, and 10-year tumor-free survival rates were 55% , 30% , 25% , 20% and 18% , respectively. The Chinese classification system was better than the TNM staging system in predicting survival rate of patients with PHC, as confirmed by survival curves shown by the Kaplain-Meier method. The mean survival time was 155, 70, 39, 16, and 4 months in patients with die Chinese classification stages Ⅰa, Ⅰb, Ⅱa,Ⅱb, and Ⅲ, respectively. The 1-, 3-, 5-, 7-, and 10-year tumor-free survival rates of the Chinese classification system and TNM staging were statistically significant and had a slightly positive relationship. The predictive capacity of the Chinese classification system was confirmed in any two subgroups of patients undergoing operation. COX proportional hazards regression analysis showed that the Chinese classification system was the only independent prognostic factor for survival. CONCLUSIONS: Taking both tumor extension and liver function into account, we consider that the Chinese classification system making up for the deficiency of UICC TNM staging is more precise in predicting the prognosis of patients with resection of PHC.展开更多
Primary hyperoxaluria type 1(PH1)is a rare but devastating autosomal recessive inherited disease caused by mutations in gene AGXT.Pathogenic mutations of AGXT were mostly reported in Caucasian but infrequently in Asia...Primary hyperoxaluria type 1(PH1)is a rare but devastating autosomal recessive inherited disease caused by mutations in gene AGXT.Pathogenic mutations of AGXT were mostly reported in Caucasian but infrequently in Asian,especially in Chinese.To update the genotypes of PH1 in the Chinese population,we collected and identified 7 Chinese probands with PH1 from 2013 to 2017 in our center,five of whom had delayed diagnosis and failed in kidney transplantation.Samples of peripheral blood DNA from the 7 patients and their family members were collected and sequencing analysis was performed to test the mutations of gene AGXT.Western blotting and enzyme activity analysis were conducted to evaluate the function of the mutations.Furthermore,a systematic review from 1998 to 2017 was performed to observe the genetic characteristics between Chinese and Caucasian. The results showed that a total of 12 mutations were identified in the 7 pedigrees.To the best of ourknowledge,2 novel variants of A GXT,p.Gly41 Trp and p.Leu33Met,were first reported.Bioinformatics and functional analysis showed that only 7 mutations led to a reduced expression of alanine-glyoxylate amino transferase (AGT)at a protein level.The systematic review revealed significant population heterogeneity in PH1.In conclusion,new genetic subtypes and genetic characteristics of PH1 are updated in the Chinese population. Furthermore,a genotype-phenotype correlation is found in PH1.展开更多
AIM： To analyze the characteristics of multiple primary malignancies （MPMs） of digestive system; including incidence, types of tumor combinations, time intervals between development of multiple tumors, clinical cou...AIM： To analyze the characteristics of multiple primary malignancies （MPMs） of digestive system; including incidence, types of tumor combinations, time intervals between development of multiple tumors, clinical course,and prognostic factors affecting survival and mortality.METHODS： Data from a total of 129 patients treated from January 1991 to December 2000 for pathologically proved MPMs, including at least one originating from the digestive system, were reviewed retrospectively.RESULTS： Among 129 patients, 120 （93.02%） had two primary cancers and 9 （6.98%） had three primary cancers. The major sites of MPMs of the digestive system were large intestine, stomach, and liver. Associated nondigestive cancers included 40 cases of gynecological cancers, of which 31 were carcinoma of cervix and 10 cases of genitourinary cancers, of which 5 were bladder cancers. Other cancers originated from the lung, breast,nasopharynx, larynx, thyroid, brain, muscle, and skin.Reproductive tract cancers, especially cervical, ovarian,bladder, and prostate cancers were the most commonlyassociated non-G! cancers, followed by cancer of the lung and breasts. Forty-three cases were synchronous, while the rest （86 cases） were metachronous cancers. Staging of MPMs and treatment regimes correlated with the prognosis between survival and non-survival groups.CONCLUSION： As advances in cancer therapy bring about a progressively larger percentage of long-term survivors, the proportion of patients with subsequent primary lesions will increase. Early diagnosis of these lesions, based on an awareness of the possibility of second and third cancers, and multidiscipiinary treatment strategies will substantially increase the survival of these patients.展开更多
This research classified vegetation types and evaluated net primary productivity （NPP） of southern China＇s grasslands based on the improved comprehensive and sequential classification system （CSCS）, and proposed ...This research classified vegetation types and evaluated net primary productivity （NPP） of southern China＇s grasslands based on the improved comprehensive and sequential classification system （CSCS）, and proposed 5 thermal grades and 6 humidity grades. Four classes of grasslands vegetation were recognized by improved CSCS, namely, tundra grassland class, typical grassland class, mixed grassland class and alpine grassland class. At the type level, 14 types of vegetations （9 grasslands and 5 forests） were classified. The NPP had a trend to decrease from east to west and south to north, and the annual mean NPP was estimated to be 656.3 g C m-2 yr-1. The NPP value of alpine grassland class was relatively high, generally more than 1200 g C m2 yr-1. The NPP value of mixed grassland class was in a range from 1 000 to 1200 g C m-2 yr-1. Tundra grassland class was located in southeastern Tibet with high elevation, and its NPP value was the lowest （〈600 g C m＇2yrl）. The typical grassland class distributed in most of the area, and its NPP value was generally from 600 to 1000 g C m-2 yr-1. The total NPP value in the study area was 68.46 Tg C. The NPP value of typical grassland class was the highest （48.44 Tg C）, and mixed grassland class was the second （16.54 Tg C）, followed by alpine grassland class （3.22 Tg C）, with tundra grassland class being the lowest （0.25 Tg C）. For all the grasslands types, the total NPP of forest meadow was the highest （34.81 Tg C）, followed by sparse forest brush （16.54 Tg C）, and montane meadow was the lowest （0.01 Tg C）.展开更多
AIM: To evaluate the diagnostic value of B-scan ultrasound and explore the cytological characteristics of patients with vitreoretinal lymphoma(VRL) and primary central nervous system lymphoma(PCNSL).METHODS: The clini...AIM: To evaluate the diagnostic value of B-scan ultrasound and explore the cytological characteristics of patients with vitreoretinal lymphoma(VRL) and primary central nervous system lymphoma(PCNSL).METHODS: The clinical data and pathologic specimens from patients with VRL diagnosed at the North Huashan Hospital from 2016 to 2017 were retrospectively reviewed. The patients were diagnosed by slit lamp ophthalmoscopy, B-scan ultrasound, cytology of the vitreous, which was obtained by vitrectomy, and cytokine measurements of interleukin(IL)-10 and IL-6.RESULTS: Twenty-six eyes(19.4%) out of 134 eyes of 67 patients(47 men and 20 women) with PCNSL were diagnosed with VRL by B-scan ultrasound, and 14 eyes(10.4%) were diagnosed by slit lamp ophthalmoscopy. Twenty-four eyes(17.9%) of 17 patients were confirmed as having VRL with cytology. No difference in the association between intracranial lesion location and ocular involvement was found. VRL patients had higher levels of vitreous IL-10 and IL-10/IL-6 when compared with macular hole cases, but the difference was not statistically significant.CONCLUSION: A total of 25.4% of the PCNSL patients had VRL, B-scan ultrasound examination had characteristic features and is recommended over slit lamp ophthalmoscopy for the screening diagnosis of PCNSL with intraocular involvement. Moreover, the cytological and immunohistochemical analyses performed after 25-gauge diagnostic vitrectomy were accurate diagnostic techniques.展开更多
文摘Primary systemic treatment is a fundamental part of breast cancer therapy, and it is applied to non-surgical and locally advanced tumours as well as surgical tumours to increase the likelihood of conservative treatment. Its aim is to achieve the best possible survival with better cosmetic results and with the lowest number of treatment-related secondary effects. Before treatment is started, it is necessary to attain the best knowledge of the biological features and locoregional extension of the tumour. To do so, it is necessary to obtain a biopsy of the lesion with a wide bore needle,as well as good radiological knowledge of the disease.Therefore, currently, the use of a dynamic magnetic resonance imaging(MRI) of the breast should be included in all cases. In addition, before it is started,especially in those tumours in which conservative treatment is considered, one or several radiopaque markers should be put into place to make it possible to locate the area to be treated if there is a considerable or complete response. Systemic treatment is mainly based on combined chemotherapy with anthracyclins and taxanes, in addition to some biological agents with demonstrated efficiency for increasing the likelihood of complete disease response(trastuzumab in patients with Her-2/neu overexpression). However, there is room for neoadjuvant hormone treatment, in patients with hormone receptor overexpression, especially in those cases in which chemotherapy is contraindicated as well as in elderly patients with a relatively short life expectancy. The assessment of preoperative treatment should be based on adequate radiological tests, and nowad these should include MRI before taking decisions about adequate surgical treatment. The objective of primary treatment is to be able to increase survival and improve the chances of local treatment in the case of locally advanced treatment, achieving results that are at least equal to those of adjuvant treatment in the case of surgical tumours, but with greater chances of conservative surgery. Although the objective is survival, achieving complete pathological response seems to be a reasonable related objective, although these are more closely linked in some tumour subtypes.
基金Supported by the National Natural Science Foundation of China,No.82402789Beijing Jishuitan Hospital Youcai Plan,No.KYYC202402+2 种基金Beijing Jishuitan Research Funding,No.HL202402and Beijing Natural Science Foundation,No.L232062No.L222063.
文摘BACKGROUND Aseptic loosening remains the leading cause of revision in primary total hip arthroplasty(pTHA).However,the literature demonstrates significant variability regarding the relative contributions of different factors.AIM To investigate the key determinants of aseptic loosening,we performed a systematic review and meta-analysis.METHODS A comprehensive search of PubMed,Web of Science,EMBASE,and the Cochrane Library was conducted,encompassing studies from database inception to January 1,2025.Meta-analyses were performed to evaluate factors associated with aseptic loosening following pTHA.Inclusion and exclusion criteria were systematically applied at each stage to ensure methodological transparency and reproducibility.Study quality was assessed using standardized categories.Pooled odds ratio(OR)with corresponding 95%confidence interval were calculated with random-or fixed-effects models to generate reliability estimates,and study heterogeneity was visualized using forest plots.Ten factors,categorized into patient-,surgeon-,and device-related domains,were reviewed and meta-analyzed.Funnel plot analysis demonstrated a relatively symmetrical distribution,suggesting minimal publication bias.RESULTS A meta-analysis of 20 studies(520789 participants)found a pooled prevalence of 1.96%.Significant risk factors for aseptic loosening after pTHA included elevated body mass index(OR=1.116,P<0.001),higher Charlson comorbidity index(OR=1.378,P<0.001),prosthesis-related factors(OR=1.497,P<0.001),and adverse lifestyles(OR=2.198,P=0.037).Protective factors were non-white race(OR=0.445,P<0.001)and favorable genetics(OR=0.723,P<0.001).Male sex increased risk(OR=1.232,P=0.016),while age and anatomy were not significant.Surgical expertise showed a slight protective effect(OR=1.048,P<0.001).A comprehensive understanding of the modifiable and non-modifiable factors contributing to aseptic loosening after pTHA requires consideration of patient-related factors,surgical expertise,and prosthesis characteristics.CONCLUSION The identification of these factors is critical for risk mitigation.High-risk patients should receive targeted counseling regarding individualized profiles.Further studies are warranted to establish clearer causal relationships and identify additional contributing factors.
文摘BACKGROUND Primary cardiac lymphoma(PCL)is a rare subset of cardiac tumors,often diagnosed late due to nonspecific symptoms.It predominantly affects immunocompromised individuals,primarily in the pericardium and right heart.Late diagnosis mimics common cardiac ailments,leading to poor prognosis.AIM To systematically review the efficacy of rituximab in treating PCL either alone or in various chemotherapeutic regimens.Secondary objectives include evaluating morphological subtypes,assessing treatment regimens,and analyzing outcomes focusing on remission and adverse events.METHODS Following PRISMA guidelines,a comprehensive literature search was conducted across multiple databases,including PubMed,Hinari,Web of Science,and Scopus.English-language studies reporting the use of rituximab in treating PCL in humans were included.Study selection involved initial screening of titles and abstracts followed by full-text examination and data extraction.RESULTS Thirty-three case reports involving 36 patients were included in this systematic review.Diffuse large B-cell lymphoma was the predominant morphological subtype observed.The rituximab,cyclophosphamide,doxorubicin,oncovin,and prednisolone regimen emerged as the most commonly employed treatment strategy,indicating widespread acceptance and efficacy in PCL management.Combination therapies,including surgical intervention,showed promise in achieving complete remission,while some studies reported mortality despite aggressive treatment approaches.CONCLUSION Rituximab,particularly in combination with chemotherapy regimens,represents a significant advancement in PCL management,offering hope for improved patient outcomes.However,challenges such as variable treatment responses and adverse events underscore the complexity of managing PCL.Further research is warranted to refine therapeutic strategies and enhance diagnostic approaches for this rare cardiac malignancy.
文摘Casein kinase 1(CK1)is an important member of the serine/threonine protein kinase family,playing a crucial role in various cellular processes,including cell cycle regulation,signal transduction,DNA repair,and circadian rhythm control.CK1 is also essential in the nervous system,where it regulates neuronal growth,differentiation,and synaptic plasticity.Studies have shown that CK1δ phosphorylates neuron-specific proteins to regulate axonal growth and synaptogenesis.Primary cilia are non-motile microtubule structures present on the surface of most mammalian cells.Recent studies have revealed their multiple roles in cellular physiology and development,and dysfunction of cilia can impact the development and function of the nervous system.CK1 has an important role in the formation and function of primary cilia.By regulating various signaling pathways and the phosphorylation status of proteins,CK1 affects the generation,maintenance,and signaling transduction of cilia.In this review,the relationship between CK1,primary cilia,and the nervous system was explored,focusing on how CK1 influences cilia to regulate the structure and function of the nervous system.
文摘In the current era of digitalization sweeping the education field,primary school English education is facing new challenges and opportunities of deep integration with artificial intelligence.This study focuses on primary school English teachers and uses various methods such as questionnaire surveys,visits,and interviews to conduct an in-depth exploration of their artificial intelligence literacy.After data analysis,optimization strategies are proposed to further improve the artificial intelligence literacy of primary school English teachers and promote the development of educational soft power.
基金supported by a grant from Ministry of Science and Technology China,No.2022ZD0204704(to WW)the National Natural Science Foundation of China,No.82301572(to XZ)the China Postdoctoral Science Foundation,No.2023M731202(to XZ)。
文摘Nonhuman primates are increasingly being used as animal models in neuroscience research.However,efficient neuronal tracing techniques for labeling motor neurons and primary sensory afferents in the monkey spinal cord are lacking.Here,by injecting the cholera toxin B subunit into the sciatic nerve of a rhesus monkey,we successfully labeled the motor neurons and primary sensory afferents in the lumbar and sacralspinal cord.Labeled alpha motor neurons were located in lamina IX of the L6–S1 segments,which innervate both flexors and extensors.The labeled primary sensory afferents were mainly myelinated Aβfibers that terminated mostly in laminae I and II of the L4–L7 segments.Together with the labeled proprioceptive afferents,the primary sensory afferents formed excitatory synapses with multiple types of spinal neurons.In summary,our methods successfully traced neuronal connections in the monkey spinal cord and can be used in spinal cord studies when nonhuman primates are used.
文摘AIM:To investigate the long-term outcomes in acute primary angle closure(APAC)patients treated with lens extraction(LE)surgery and to identify risk factors for glaucomatous optic neuropathy(GON).METHODS:In this longitudinal observational study,detailed medical histories of APAC patients and comprehensive ophthalmic examinations at final followup were collected.Logistic regression analysis was performed to identify predictors of blindness.Univariate and multivariate linear regression analyses were conducted to determine risk factors associated with visual outcomes.RESULTS:This study included 39 affected eyes of 31 subjects(26 females)with an average age of 74.1±8.0y.At 6.7±4.2y after APAC attack,2(5.7%)eyes had bestcorrected visual acuity(VA)worse than 3/60.Advanced glaucomatous visual field loss was observed in 15(39.5%)affected eyes and 5(25.0%)fellow eyes.Nine affected eyes(23.7%)had GON,and 11(28.9%)were blind.Six(15.4%)affected eyes and 2(9.1%)fellow eyes had suspicious progression.A significantly higher blindness rate in factory workers compared to office workers.Logistic regression identified that worse VA at attack(OR 10.568,95%CI 1.288-86.695;P=0.028)and worse early postoperative VA(OR 13.214,95%CI 1.157-150.881;P=0.038)were risk factors for blindness.Multivariate regression showed that longer duration of elevated intraocular pressure(P=0.004)and worse early postoperative VA(P=0.009)were associated with worse visual outcomes.CONCLUSION:Despite LE surgery,some APAC patients experience continued visual function deterioration.Lifelong monitoring is necessary.Target pressure and progression rates should be re-evaluated during follow-up.
基金Supported by National Natural Science Foundation of China(No.82260203).
文摘AIM:To study the relationships between amplitude of low-frequency fluctuations(ALFF)changes and clinical ophthalmic parameters in patients with primary open angle glaucoma(POAG)and analyze the diagnostic value of ALFF.METHODS:Twenty-four POAG patients and 24 healthy controls(HCs)underwent resting-state functional magnetic resonance imaging(rs-fMRI).Nonparametric rank-sum tests were used to compare the ALFF values in the slow-4 and slow-5 bands,and Spearman or Pearson correlation analysis was used to assess the correlation between ALFF changes and clinical ophthalmic parameters in POAG patients.Receiver operating characteristic(ROC)curves were used to evaluate the diagnostic performance of the ALFF.RESULTS:There were 16 males in POAG patients(median age 48y)and 12 males in HCs(median age 39y).Compared with HCs,POAG patients presented increased or decreased ALFF values in different brain regions,and similar changes were observed in mild POAG patients.The ALFF values were correlated with retinal nerve fiber layer(RNFL)thickness,inner limiting membrane-retinal pigment epithelium thickness changes and the degree of visual field defects.Analysis of the diagnostic value of the ALFF via ROC curves revealed that the right medial frontal gyrus[area under the curve(AUC)=0.9063]and superior frontal gyrus(AUC=0.9097)had better diagnostic value than did the optic disc area(AUC=0.8019),visual field index(VFI%,AUC=0.8988)and macular parameters.CONCLUSION:POAG patients present altered cortical function that is significantly correlated with the optic nerve and retinal thickness and had good diagnostic value,which may reflect the underlying neuropathological mechanism of POAG.
基金This work was funded by the National Natural Science Foundation of China （No.81370612） and the Youth Fund Project of the National Natural Science Foundation of China （No.81102211）.
文摘Background Primary systemic light chain amyloidosis （AL） is a rare plasma cell disease,our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients,and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry （FCM） could be used as an important indicator of AL diagnosis.Methods Fresh bone marrow samples were collected from 51 AL,21 multiple myeloma （MM）,and 5 Waldenstr（o）m＇s macroglobulinemia （WM） patients.The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45,CD38,CD138,CD117,CD56,and CD19.Results In AL,light chain restriction could be identified in 31 cases （60.9%）,in which the λ light chain restriction was found in 24 cases （77.4%）.In MM,κ light chain restriction was found in 13 cases （61.9%）,and λ light chain restriction in eight cases.CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM,but was positive to strongly positive in WM.In the bone marrow plasma cells of the 51 AL,78.4% were CD56＋,68.6% were CD117＋,and 88.2% were CD19-.While in the 21 MM cases,66.7% were CD56＋,38.1% were CD117＋,and 90.4% were CD19-.The plasmacytoid lymphocytes in the five WM patients were CD19＋ and CD56-,CD117-.Conclusion Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.
文摘Introduction:Primary systemic amyloidosis is characterized by clonal plasma cell disorder,and its signs and symptoms are various and complex,damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion,as well unusual skin vegetations.Case presentation:A 61-year-old man was hospitalized due to repeated burning sensation on his back,multiple ecchymosis,and skin vegetations.Through a series of examinations(mainly including skin histopathology,bone marrow cytology,bone marrow flow cytometry,immunofixation electrophoresis),Primary systemic amyloidosis was diagnosed,but multiple myeloma could not be diagnosed.Subsequently,he received chemotherapy.In the half-year follow-up,there was no significant change in his symptoms and signs.Discussion:In this case,in addition to the typical skin damage of primary amyloidosis,the multiple skin vegetations in the buttocks,abdomen,and arms are particularly noteworthy.According to the histopathology and Immunohistochemistry of the skin vegetation,we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid,which leads to lymphatic dilatation,lymph leakage,and dermal edema.Conclusion:Primary systemic amyloidosis is a rare disease,which is often difficult to diagnose.We should be alert to those atypical skin features so as not to delay diagnosis.
文摘Objective To investigate the difference in tumor conventional imaging findings and texture features on T2 weighted images between glioblastoma and primary central neural system(CNS) lymphoma. Methods The pre-operative MRI data of 81 patients with glioblastoma and 28 patients with primary CNS lymphoma admitted to the Chinese PLA General Hospital and Hainan Hospital of Chinese PLA General Hospital were retrospectively collected. All patients underwent plain MR imaging and enhanced T1 weighted imaging to visualize imaging features of lesions. Texture analysis of T2 weighted imaging(T2 WI) was performed by use of GLCM texture plugin of ImageJ software, and the texture parameters including Angular Second Moment(ASM), Contrast, Correlation, Inverse Difference Moment(IDM), and Entropy were measured. Independent sample t-test and Mann-Whitney U test were performed for the between-group comparisons, regression model was established by Binary Logistic regression analysis, and receiver operating characteristic(ROC) curve was plotted to compare the diagnostic efficacy. Results The conventional imaging features including cystic and necrosis changes(P = 0.000), ‘Rosette' changes(P = 0.000) and ‘incision sign'(P = 0.000), except ‘flame-like edema'(P = 0.635), presented significantly statistical difference between glioblastoma and primary CNS lymphoma. The texture features, ASM, Contrast, Correlation, IDM and Entropy, showed significant differences between glioblastoma and primary CNS lympoma(P = 0.006,0.000, 0.002, 0.000, and 0.015 respectively). The area under the ROC curve was 0.671, 0.752, 0.695, 0.720 and 0.646 respectively, and the area under the ROC curve was 0.917 for the combined texture variables(Contrast, cystic and necrosis, ‘Rosette' changes, and ‘incision sign') in the model of Logistic regression. Binary Logistic regression analysis demonstrated that cystic and necrosis changes, ‘Rosette' changes and ‘incision sign' and texture Contrast could be considered as the specific texture variables for the differential diagnosis of glioblastoma and primary CNS lymphoma. Conclusion The texture features of T2 WI and conventional imaging findings may be used to distinguish glioblastoma from primary CNS lymphoma.
基金supported by a grant from the Research Program for Health Care of Lanzhou Science and Technology Bureau(No.2013-3-37)
文摘Background： Common bile duct （CBD） stones may occur in up to 3%–14.7% of all patients with cholecystectomy.Various approaches of laparoscopic CBD exploration plus primary duct closure （PDC） are the most commonly used and the best methods to treat CBD stone. This systematic review was to comparethe effectiveness and safety of the various approaches of laparoscopic CBD exploration plus PDC forcholedocholithiasis
基金"Strategic Priority Research Program of China"of the Chinese Academy of Sciences,No.XDA05090307National Key Technology R&D Program of the 12th Five-Year Plan,No.2012BAC19B10Open Project of Shanghai Key Lab for Urban Ecological Processes and Eco-Restoration,No.SHUES2012A04
文摘Net primary productivity （NPP） is the structure and function of the ecosystem. NPP can most important index that represents the be simulated by dynamic global vegetation models （DGVM）, which are designed to represent vegetation dynamics relative to environ- mental change. This study simulated the NPP of China＇s ecosystems based on the DGVM Integrated Biosphere Simulator （IBIS） with data on climate, soil, and topography. The appli- cability of IBIS in the NPP simulation of China＇s terrestrial ecosystems was verified first. Comparison with other relevant studies indicates that the range and mean value of simula- tions are generally within the limits of observations; the overall pattern and total annual NPP are close to the simulations conducted with other models. The simulations are also close to the NPP estimations based on remote sensing. Validation proved that IBIS can be utilized in the large-scale simulation of NPP in China＇s natural ecosystem. We then simulated NPP with climate change data from 1961 to 2005, when warming was particularly striking. The following are the results of the simulation. （1） Total NPP varied from 3.61 GtC/yr to 4.24 GtC/yr in the past 45 years and exhibited minimal significant linear increase or decrease. （2） Regional differences in the increase or decrease in NPP were large but exhibited an insignificant overall linear trend. NPP declined in most parts of eastern and central China, especially in the Loess Plateau. （3） Similar to the fluctuation law of annual NPP, seasonal NPP also displayed an insignificant increase or decrease; the trend line was within the general level. （4） The re- gional differences in seasonal NPP changes were large. NPP declined in spring, summer, and autumn in the Loess Plateau but increased in most parts of the Tibetan Plateau.
基金National Key Research and Development Program of China,No.2016YFC0500205National Basic Research Program of China(973 Program),No.2015CB954103,No.2015CB954101
文摘The Three-River Headwater Region（TRHR）, known as the ＂Water Tower of China＂, is an important ecological shelter for national security interests and regional sustainable development activities for many downstream regions in China and a number of Southeast Asian countries. The TRHR is a high-elevation, cold environment with a unique, but typical alpine vegetation system. Net primary productivity（NPP） is a key vegetation parameter and ecological indicator that can reflect both natural environmental changes and carbon budget levels. Given the unique geographical environment and strategic location of the TRHR, many scholars have estimated NPP of the TRHR by using different methods; however, these estimates vary greatly for a number of reasons. To date, there is no paper that has reviewed and assessed NPP estimation studies conducted in the TRHR. Therefore, in this paper, we（1） summarized the related methods and results of NPP estimation in the TRHR in a systematic review of previous research;（2） discussed the suitability of existing methods for estimating NPP in the TRHR and highlighted the most significant challenges; and（3） assessed the estimated NPP results. Finally, developmental directions of NPP estimation in the TRHR were prospected.
基金Supported by Grants-in-Aid for Clinical Research from National Hospital Organization,Grants-in-Aid for Scientific Research from the Ministry of Health,Labour and Welfare of Japan
文摘Chronic hepatobiliary inflammatory diseases are not widely acknowledged as underlying disorders of systemic AA amyloidosis,except epidemic schistosomiasis.Among them,primary sclerosing cholangitis (PSC) might initiate amyloid A protein deposition in diverse tissues,giving rise to systemic amyloidosis,due to a progressive and unresolved inflammatory process,and its possible association with inflammatory bowel diseases.Nevertheless,only one such case has been reported in the literature to date.We report a 69-year-old Japanese woman with cirrhosis who was diagnosed with PSC complicated with systemic AA amyloidosis,without any evidence of other inflammatory disorders.As a result of cholestasis in conjunction with biliary strictures and increased serum IgG4,the presence of IgG4 + plasma cells was examined systemically,resulting in unexpected documentation of Congo-red-positive amyloid deposits,but not IgG4 + plasma cells,in the liver,stomach and salivary glands.Elevated serum IgG4 is the hallmark of IgG4-related disease,including IgG4-associated cholangitis,but it has also been demonstrated in certain patients with PSC.Amyloid A deposits in multiple organs associated with an indolent clinical course that progresses over many years might have a diagnostic value in discriminating PSC from IgG4-associated cholangitis.
文摘BACKGROUND: The life expectancy of a patient with primary hepatic carcinoma (PHC) is hard to predict, and it is related to many prognostic factors. The Chinese classification system including five parameters: tumor, vascular thrombosis, lymph node metastasis, distant metastasis and Child-Pugh stage developed in 1999 was adopted by the 8th National Conference on liver Cancer of the Chinese Anti-Cancer Association in 2001. In this study, the discriminatory ability of the Chinese classification system -was compared with that of the TNM staging in patients for resection of PHC, in addition to the evaluation of prognostic value. METHODS: The data of 246 patients who had undergone resection of PHC from January 1986 to December 2000 (average age, 51 years; male/female ratio, 213/33) were retrospectively studied. Among the 246 patients, 227 were followed up for at least 3 years. RESULTS: The 1-, 3-, 5-, 7-, and 10-year tumor-free survival rates were 55% , 30% , 25% , 20% and 18% , respectively. The Chinese classification system was better than the TNM staging system in predicting survival rate of patients with PHC, as confirmed by survival curves shown by the Kaplain-Meier method. The mean survival time was 155, 70, 39, 16, and 4 months in patients with die Chinese classification stages Ⅰa, Ⅰb, Ⅱa,Ⅱb, and Ⅲ, respectively. The 1-, 3-, 5-, 7-, and 10-year tumor-free survival rates of the Chinese classification system and TNM staging were statistically significant and had a slightly positive relationship. The predictive capacity of the Chinese classification system was confirmed in any two subgroups of patients undergoing operation. COX proportional hazards regression analysis showed that the Chinese classification system was the only independent prognostic factor for survival. CONCLUSIONS: Taking both tumor extension and liver function into account, we consider that the Chinese classification system making up for the deficiency of UICC TNM staging is more precise in predicting the prognosis of patients with resection of PHC.
基金This work was supported by the grants from the Special Project of Ministry of Health (No.201302009)and the National Natural Science Foundation of China (No. 81700300).
文摘Primary hyperoxaluria type 1(PH1)is a rare but devastating autosomal recessive inherited disease caused by mutations in gene AGXT.Pathogenic mutations of AGXT were mostly reported in Caucasian but infrequently in Asian,especially in Chinese.To update the genotypes of PH1 in the Chinese population,we collected and identified 7 Chinese probands with PH1 from 2013 to 2017 in our center,five of whom had delayed diagnosis and failed in kidney transplantation.Samples of peripheral blood DNA from the 7 patients and their family members were collected and sequencing analysis was performed to test the mutations of gene AGXT.Western blotting and enzyme activity analysis were conducted to evaluate the function of the mutations.Furthermore,a systematic review from 1998 to 2017 was performed to observe the genetic characteristics between Chinese and Caucasian. The results showed that a total of 12 mutations were identified in the 7 pedigrees.To the best of ourknowledge,2 novel variants of A GXT,p.Gly41 Trp and p.Leu33Met,were first reported.Bioinformatics and functional analysis showed that only 7 mutations led to a reduced expression of alanine-glyoxylate amino transferase (AGT)at a protein level.The systematic review revealed significant population heterogeneity in PH1.In conclusion,new genetic subtypes and genetic characteristics of PH1 are updated in the Chinese population. Furthermore,a genotype-phenotype correlation is found in PH1.
文摘AIM： To analyze the characteristics of multiple primary malignancies （MPMs） of digestive system; including incidence, types of tumor combinations, time intervals between development of multiple tumors, clinical course,and prognostic factors affecting survival and mortality.METHODS： Data from a total of 129 patients treated from January 1991 to December 2000 for pathologically proved MPMs, including at least one originating from the digestive system, were reviewed retrospectively.RESULTS： Among 129 patients, 120 （93.02%） had two primary cancers and 9 （6.98%） had three primary cancers. The major sites of MPMs of the digestive system were large intestine, stomach, and liver. Associated nondigestive cancers included 40 cases of gynecological cancers, of which 31 were carcinoma of cervix and 10 cases of genitourinary cancers, of which 5 were bladder cancers. Other cancers originated from the lung, breast,nasopharynx, larynx, thyroid, brain, muscle, and skin.Reproductive tract cancers, especially cervical, ovarian,bladder, and prostate cancers were the most commonlyassociated non-G! cancers, followed by cancer of the lung and breasts. Forty-three cases were synchronous, while the rest （86 cases） were metachronous cancers. Staging of MPMs and treatment regimes correlated with the prognosis between survival and non-survival groups.CONCLUSION： As advances in cancer therapy bring about a progressively larger percentage of long-term survivors, the proportion of patients with subsequent primary lesions will increase. Early diagnosis of these lesions, based on an awareness of the possibility of second and third cancers, and multidiscipiinary treatment strategies will substantially increase the survival of these patients.
基金the National Basic Research Program of China(2010CB950702)the National High-Technology Reaearch and Development Program of China(2007AA10Z231)the Asia-Pacific Network for Global Change Research Project(ARCP201106CMY-Li)
文摘This research classified vegetation types and evaluated net primary productivity （NPP） of southern China＇s grasslands based on the improved comprehensive and sequential classification system （CSCS）, and proposed 5 thermal grades and 6 humidity grades. Four classes of grasslands vegetation were recognized by improved CSCS, namely, tundra grassland class, typical grassland class, mixed grassland class and alpine grassland class. At the type level, 14 types of vegetations （9 grasslands and 5 forests） were classified. The NPP had a trend to decrease from east to west and south to north, and the annual mean NPP was estimated to be 656.3 g C m-2 yr-1. The NPP value of alpine grassland class was relatively high, generally more than 1200 g C m2 yr-1. The NPP value of mixed grassland class was in a range from 1 000 to 1200 g C m-2 yr-1. Tundra grassland class was located in southeastern Tibet with high elevation, and its NPP value was the lowest （〈600 g C m＇2yrl）. The typical grassland class distributed in most of the area, and its NPP value was generally from 600 to 1000 g C m-2 yr-1. The total NPP value in the study area was 68.46 Tg C. The NPP value of typical grassland class was the highest （48.44 Tg C）, and mixed grassland class was the second （16.54 Tg C）, followed by alpine grassland class （3.22 Tg C）, with tundra grassland class being the lowest （0.25 Tg C）. For all the grasslands types, the total NPP of forest meadow was the highest （34.81 Tg C）, followed by sparse forest brush （16.54 Tg C）, and montane meadow was the lowest （0.01 Tg C）.
基金Supported by the National Natural Science Foundation of China(No.81700123)the Shanghai Hospital Development Centre(No.16CR2043B)
文摘AIM: To evaluate the diagnostic value of B-scan ultrasound and explore the cytological characteristics of patients with vitreoretinal lymphoma(VRL) and primary central nervous system lymphoma(PCNSL).METHODS: The clinical data and pathologic specimens from patients with VRL diagnosed at the North Huashan Hospital from 2016 to 2017 were retrospectively reviewed. The patients were diagnosed by slit lamp ophthalmoscopy, B-scan ultrasound, cytology of the vitreous, which was obtained by vitrectomy, and cytokine measurements of interleukin(IL)-10 and IL-6.RESULTS: Twenty-six eyes(19.4%) out of 134 eyes of 67 patients(47 men and 20 women) with PCNSL were diagnosed with VRL by B-scan ultrasound, and 14 eyes(10.4%) were diagnosed by slit lamp ophthalmoscopy. Twenty-four eyes(17.9%) of 17 patients were confirmed as having VRL with cytology. No difference in the association between intracranial lesion location and ocular involvement was found. VRL patients had higher levels of vitreous IL-10 and IL-10/IL-6 when compared with macular hole cases, but the difference was not statistically significant.CONCLUSION: A total of 25.4% of the PCNSL patients had VRL, B-scan ultrasound examination had characteristic features and is recommended over slit lamp ophthalmoscopy for the screening diagnosis of PCNSL with intraocular involvement. Moreover, the cytological and immunohistochemical analyses performed after 25-gauge diagnostic vitrectomy were accurate diagnostic techniques.
