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| 1 | Gene mutations and clinical phenotypes in Chinese children with Blau syndrome显示文摘The mutations of CARD15 gene and clinical features of Chinese patients with Blau syndrome were analyzed. We identified10 missense mutations, out of which five were new: R334 L, E383 D, R471 C, C495 R and D512 F. The rest of them, R334 W,R334Q, G481 D, M513 T and R587 C, have been reported previously. Among all the mutations, R334 W, R334 Q and C495 R had the highest frequency. Blau syndrome was found at early age after birth. It began with lepidic rash and symmetric polyarthritis and was phenotypically characterized by typical rash, arthritis, iridocyclitis and arteritis. Cardiac involvement was also found in Blau syndrome. In addition to nerve deafness, renal involvement, osteochondroma and central nervous system involvement were also found in our patients. Therefore, Chinese children with Blau syndrome have unique gene mutations and complicated clinical phenotypes. Pathologic examination and CARD15 mutation testing should be considered for diagnosis as early as possible for suspected patients. | Caifeng Li Junmei Zhang Shipeng Li Tongxin Han Weiying Kuang Yifang Zhou Jianghong Deng Xiaohua Tan | 2017 | Science China(Life Sciences)2017,60,7: | 13 |
| 2 | Gene mutations and clinical phenotypes in 15 Chinese children with cryopyrin-associated periodic syndrome(CAPS)显示文摘The aim of our study is to explore the features of clinical manifestations and genetic mutations in Chinese CAPS patients. Fifteen confirmed patients with CAPS were enrolled. The onset time ranges from 2 days after birth to 6 years and 1 month. Recurrent urticaria rash(93.3%) with fever(100%) were two dominant characteristics of these patients that were presented as either acute or chronic process. Systemic involvements were found in all patients except for one with only rash and fever. The top three symptoms were fever(100%), rash(93.3%) and myalgia(76%). Other clinical manifestations include arthritis(11 cases), lung involvement(seven cases), optical dysfunction(seven cases), nerve deafness(six cases), nervous system involvement(five cases), hepatomegaly, splenomegaly and lymphadenectasis(five cases). Also, four patients had heart involvement and one patient suffered kidney involvement. The laboratory inflammation index such as leukocyte counts, platelet counts, erythrocyte sedimentation rate(ESR), C-reactive protein(CRP), serum amyloid A(SAA) and fibrinogen(FIB) increased significantly at initial stage, but decreased after therapy. As for gene mutation detection, Twelve out of 15 patients were confirmed with mutation in NLRP3, including 11 mutant site: c1789 A>G, c.1703 T>A, c.913 G>A, c.1710 G>C, c.1057 G >T, c.2335 C>T, c.932 T>C,c.296 G>C, c.663 C>T, t.1702 T>A, 299 G>A. Mutation sites c.1703 T>A, c.2335 C>T, c.296 G>C, c.663 C>T, and 299 G>A were newly identified. The association between gene mutation and clinical manifestation shows that D305 N was highly associated with severe organs involvements, and therefore, the time of therapy and regimen were critical for the prognosis of disease. As the largest cohort study of Chinese CAPS patients, we confirmed that all patients presented typical clinical manifestations, identified five new mutation sites on NLRP3 and analyzed the correlation between the genotypes and phenotypes. We also raise concerns for one case with serious conditions that only had two nonsense mutations(c.663 C>T and t.1702 T>A) detected. | Caifeng Li Xiaohua Tan Junmei Zhang Shipeng Li Wenxiu Mo Tongxin Han Weiying Kuang Yifang Zhou Jianghong Deng | 2017 | Science China(Life Sciences)2017,60,12: | 9 |
| 3 | Synthesis,structure and physical properties of the one-dimensional chain complex of tetrathiafulvalene carboxylate显示文摘A new Co(Ⅱ) coordination polymer bearing TTF carboxylate group, [{Co2(trioTTF)2(H2O)6}·5H2O]n (1) (trioTTF=2-(5,6,8,9,11,12,14,15-octahydro-[1,3]dithiolo[4,5-h][1,4,13,7,10]trioxadithiacyclopentadecin-2-ylidene)-1,3-dithiole-4,5-dicarboxylate), has been prepared and characterized. In the structure of 1, shorter intermolecular S····S contacts (3.565 △) are found between the trioTTF groups from neighboring chains. The electric conductivity of 1 is poor due to the bulky crown-ether group, but it exhibits ferromagnetic interaction at low temperature. | CHEN Ya LI ChengHui WANG CaiFeng WU Di ZUO JingLin YOU XiaoZeng | 2009 | Science China Chemistry2009,52,10: | 4 |
| 4 | Diagnosis and clinical manifestations of subacute combined degeneration of the spinal cord: Analysis of 21 cases显示文摘BACKGROUND: Subacute combined degeneration of the spinal cord is caused by vitamin B12 deficiency and is a kind of degenerative disease owing the characteristics of nervous system diseases. In addition, different patients have variously clinical manifestations and various prognoses after vitamin B12 therapy. OBJECTIVE: To investigate and analyze diagnosis, clinical manifestations and prognosis of subacute combined degeneration of the spinal cord. DESIGN: Case analysis. SETTING: Department of Neurology, the Third Hospital of Peking University. PARTICIPANTS: A total of 21 subacute combined degeneration of the spinal cord patients including 14 males and 7 females aged from 33 to 82 years were selected from Department of Neurology, the Third Hospital of Peking University from January 1999 to December 2005. Duration from onset to final diagnosis lasted for 1.5–108 months. All patients had typically clinical manifestations; meanwhile, level of serum vitamin B12 was decreased and/or vitamin B12 therapy was effective. All patients provided the confirmed consent. METHODS: Clinical data of 21 subacute combined degeneration of the spinal cord patients were retrospectively analyzed, while general data and clinical characteristics were recorded at the same time. Levels of blood routine, serum vitamin B12 and homocysteine were measured at the phase of hospitalization. Normal value of serum vitamin B12 was 187–1 059 ng/L and normal value of serum homocysteine was 5– 15 μmol/L. All patients received neuroelectrophysiological examination and 15 patients received MRI examinations of spinal cord. After final diagnosis, patients were given vitamin B12 therapy. And follow-up was performed to investigate the prognosis. MAIN OUTCOME MEASURES: ① Levels of blood routine, serum vitamin B12 and homocysteine; ② results of neuroelectrophysiological examination; ③ results of MRI examination of spinal cord; ④ prognosis. RESULTS: Clinical data of 21 patients and follow-up data of 20 patients were involved in the final analysis and 1 patient was lost because of living in the other province. ① Clinical manifestations: All 21 patients had typically clinical manifestations. The original symptoms included numbness of lower and/or upper limbs (5 cases), unstable gait (3 cases), limb asthenia (4 cases), limb numbness combined with light asthenia (5 cases), limb numbness combined with unskillful activity (3 cases), and limb numbness combined with unstable gait (1 case). ② Experimental results: Eight subacute combined degeneration of the spinal cord patients accompanied with mild-severe anemia and mean corpuscular volume of 13 patients were increased. Among 13 subacute combined degeneration of the spinal cord patients not administrating vitamin B12 before hospitalization, the levels of serum vitamin B12 of 2 patients were not measured but those of other patients were decreased. After vitamin B12 therapy,the levels of serum vitamin B12 of 8 patients were normal or increased. In addition, the levels of serum homocysteine of 6 patients were not measured but those of 7 patients were increased. While, the levels of homocysteine of 5 following-up patients were normal. The levels of serum vitamin B12 of 8 patients who received with vitamin B12 therapy before hospitalization were normal or increased. Among them,the levels of homocysteine were not measured in 4 patients, those of 3 patients were increased, and that of 1 patient was normal. ③ Results of neuroelectrophysiological examination: Among all patients, 95% (20/21) patients had abnormal sensory-evoked potential, 89% (8/9) patients had abnormal motor evoked potential, 67% (10/15) patients had abnormal nerve conduction, 13% (2/15) patients had neurogenic muscle injury showed by electromyography (EMG), 70% (7/10) patients had abnormal brain-stem auditory evoked potential, and 40% (4/10) patients had abnormal visual evokedpotential. ④ Results of MRI examination of spinal cord: MRI examination demonstrated that 40% (6/15) patients had spinal cord lesion, but spinal cord lesion disappeared in 2 patients during follow up. In addition, clinical manifestations of patients were improved after standard vitamin B12 therapy. CONCLUSION: Nervous system lesion caused by vitamin B12 deficiency is not only involved in spinal cord, also in peripheral nerve, optic nerve, auditory pathway, etc. Diagnosis of the lesion depends on clinical characteristics and level of serum vitamin B12. Especially, neuroelectrophysiological examination, measurement of homocysteine and MRI examination of spinal cord are beneficial for diagnosis and evaluation of therapeutic effects. | Yanhong Shou Caifeng Li Dongsheng Fan Yang Shen Jun Zhang Weizhong Xiao Shuqing Zhao Jinsheng Liu Wei Sui | 2007 | Neural Regeneration Research2007,2,2: | 3 |
| 5 | GOLM1 restricts colitis and colon tumorigenesis by ensuring Notch signaling equilibrium in intestinal homeostasis显示文摘Intestinal epithelium serves as the first barrier against the infections and injuries that mediate colonic inflammation.Colorectal cancer is often accompanied with chronic inflammation.Differed from its well-known oncogenic role in many malignancies,we present here that Golgi membrane protein 1(G0LM1,also referred to as GP73)suppresses colorectal tumorigenesis via maintenance of intestinal epithelial barrier.G0LM1 deficiency in mice conferred susceptibility to mucosal inflammation and colitis-induced epithelial damage,which consequently promoted colon cancer.Mechanistically,depletion of G0LM1 in intestinal epithelial cells(lECs)led to aberrant Notch activation that interfered with IEC differentiation,maturation,and lineage commitment in mice.Pharmacological inhibition of Notch pathway alleviated epithelial lesions and restrained pro-tumorigenic inflammation in G0LM1-deficient mice.Therefore,G0LM1 maintains IEC homeostasis and protects against colitis and colon tumorigenesis by modulating the equilibrium of Notch signaling pathway. | Yang Pu Ya Song Mengdi Zhang Caifeng Long Jie Li Yanan Wang Yinzhe Xu Fei Pan Na Zhao Xinyu Zhang Yanan Xu Jianxin Cui Hongying Wang Yan Li Yong Zhao Di Jin Hongbing Zhang | 2021 | Signal Transduction and Targeted Therapy2021,6,5: | 2 |
| 6 | Anti-inflammatory activity of ethyl acetate fraction of the seeds of Brucea Javanica显示文摘 | Jianhong Yang Shucai Li Caifeng Xie Haoyu Ye Huan Tang Lijuan Chen Aihua Peng | 2013 | Journal of Ethnopharmacology2013,,: | 1 |
| 7 | Evaluation of breast lesions by contrast enhanced ultrasound: Qualitative and quantitative analysis显示文摘 | Caifeng Wan Jing Du Hua Fang Fenghua Li Lin Wang | 2011 | European Journal of Radiology2011,,4: | 1 |
| 8 | Evaluation of breast lesions by contrast enhanced ultrasound: Qualitative and quantitative analysis显示文摘 | Caifeng Wan Jing Du Hua Fang Fenghua Li Lin Wang | 2011 | European Journal of Radiology2011,,4: | 1 |
| 9 | Facile synthesis of fluorescent quantum dot-polymer nanocomposites via frontal polymerization显示文摘 | Juan Fang Li Chen CaiFeng Wang | 2010 | Journal of Polymer Science: Part A: Polymer Chemistry2010,48,: | 1 |
| 10 | Correlations between the North China Craton and the Indian Shield:Constraints from regional metallogeny显示文摘The correlation between the North China Craton(NCC) and the Indian Shield(IND) has been a hot topic in recent years,On the basis of ore deposit databases,the NCC and IND have shown broad similarity in metallogenesis from the middle Archaean to the Mesoproterozoic,The two blocks both have three major metallogenic systems:(1) the Archaean BIF metallogenic system;(2) the Paleoproterozoic Cu-Pb-Zn metallogenic system;and(3) the Mesoproterozoic Fe-Pb-Zn system,In the north margin of the NCC and the west margin of the IND,the Archaean BIF-Au-Cu-Pb-Zn deposits had the same petrogenesis and host rocks,the Paleoproterozoic Cu-Pb-Zn deposits were controlled by active belts,and the Mesoproterozoic Fe-Pb-Zn deposits were mainly related to multi-stage rifting,Matching regional mineralization patterns and geological features has established the continental assembly referred to as 'NCWI',an acronym for the north margin of the NCC(NC) and the west margin of the IND(WI) during the middle Archaean to the Mesoproterozoic,In this assembly,the available geological and metallogenic data from the Eastern Block and active belts of NC fit those from the Dharwar craton and the Aravalli-DelhiVindhyan belt of WI,respectively,Moreover,the depositional model and environment of Paleoproterozoic metasedimentary manganese deposits in NCWI implied that the assembly may be located at low latitudes,where the conditions were favorable for dissolving ice and precipitating manganese deposits, | Caifeng Li Dongyue Chen Jianping Chen Xizhen Chen Xingchen Yang M.A.Aboelnour | 2015 | Geoscience Frontiers2015,6,6: | 1 |
| 11 | A novel agonist of PPAR-γ based on barbituric acid alleviates the development of non-alcoholic fatty liver disease by regulating adipocytokine expression and preventing insulin resistance显示文摘 | Hao Zheng Shilin Li Liang Ma Ling Cheng Chongyang Deng Zhizhi Chen Caifeng Xie Mingli Xiang Wei Jiang Lijuan Chen | 2011 | European Journal of Pharmacology2011,,2: | 1 |
| 12 | Responses and sensitivity of N, P and mobile carbohydrates of dominant species to increased water, N and P availability in semi- arid grasslands in northern China显示文摘Aims We aimed to improve the understanding of the carbon and nutri-ent physiological responses and adaptation of semi-arid grassland plants to environmental changes.Methods We investigated plant leaf non-structural carbohydrate(NSC=solu-ble sugars+starch),nitrogen(N)and phosphorus(P)levels in an Inner Mongolian semi-arid grassland community treated with water,N and P additions for 8 years.Two dominant grasses(Agropyron cris-tatum(L.)Gaertn.,Stipa krylovii Roshev.)and two forbs(Artemisia frigida Willd.,Potentilla bifurca L.)were analyzed.Important Findings Water addition decreased plant leaf N and P concentrations,whereas N and P addition increased them,indicating that the semi-arid grassland studied suffers from a shortage of N and P sup-ply.Both N and P addition decreased the levels of soluble sugars,starch and thus also NSC in plant leaves,which may be attributed to(i)increased carbohydrate consumption associated with a higher growth rate,and(ii)a dilution effect of greater plant size under N and P addition.Water addition tended to increase the leaf NSC levels both in the grasses(+9.2%)and forbs(+0.6%only),which may be a result of increased photosynthesis of plants with increased water availability.Under conditions of ambient and increased water supply in the present study,N addition resulted in an N/P ratio of>16 in the grasses but a significantly lower N/P ratio of<11 in the forb species.This finding implies that growth of the two grass spe-cies will be limited mainly by P availability but the forbs will still be mainly limited by N supply if N deposition,alone or in combination with summer precipitation,continues to increase as predicted in Inner Mongolia. | Xue Wang Zhuwen Xu Caifeng Yan Wentao Luo Ruzhen Wang Xingguo Han Yong Jiang Mai-He Li | 2017 | Journal of Plant Ecology2017,10,3: | 1 |
| 13 | Pulmonary embolism after transcatheter arterial chemoembolization for hepatocellular carcinoma:a retrospective analysis on 10 years' experience显示文摘Objective:To study the clinical characteristics and treatment of pulmonary embolism(PE)after transcatheter arterial chemoembolization(TACE)for hepatocellular carcinoma(HCC).Methods:The clinical records of 13 512 patients diagnosed with HCC and received TACE from January 2000 to December 2009 were reviewed.Among these patients,5 031 were allocated into group A who had one or more disorders like diabetes,hypertension,coronary heart disease,obesity or varicose vein of lower limb,while the other 8 481 patients who did not have such disorders were in group B.Results:A total of 39 185 TACE procedures were performed for the 13 512 patients.Five(0.01%)patients in group A developed PE after TACE,of whom two recovered 4 and 5 d later with early anticoagulant therapy while the hypertension,coronary heart disease,obesity or varicose vein of lower limb are possibly more likely to develop PE other 3 died of respiratory failure within 5 h.The mortality of PE was 60%(3/5).Conclusion:HCC patients with diabetes, after TACE than those without such disorders.Patients who have such disorders should be more carefully observed after TACE and early treatment with heparin should be applied once PE develops. | Li Jing YanYiqun Huang Liang Yan Jianjun Zhou Feiguo Zhang Xianghua Liu Caifeng | 2011 | Journal of Medical Colleges of PLA(China)2011,26,3: | 1 |
| 14 | Component analysis and utilization of whole-plant corn silage effluent显示文摘 | YANG Fuyou LI Caifeng DU Renrang | 2011 | Journal of Shanxi Agricultural Science2011,,2: | 1 |
| 15 | Reactivation of hepatitis C virus replication in a patient undergoing hepatectomy for hepatocellular carcinoma显示文摘1.Background Hepatocellular carcinoma (HCC) is one of the most common neoplasms encountered,and its incidence is increasing worldwide because of the increasing prevalence of hepatitis B virus (HBV) and hepatitis C virus (HCV) infection [1,2].Patients with HBV or HCV-related HCC suffer not only from malignancy but also chronic virus infection.It follows that treatments for HCC could also affect the activity of virus replication,a factor that has important implication for hepatitis activity.In previous studies [3,4],high incidences (28% and 21% respectively) of HBV reactivation were observed in HBV-related HCC patients undergoing liver resections.So far,there have been very few reports concerning reactivation of HCV replication in HCV-related HCC patients undergoing liver resection.Here we present the case of a patient with HCV-related HCC underwent liver resection and experienced postoperative liver failure duo to HCV reactivation. | Liu Caifeng Li Jing Huang Liang Yan Yiqun | 2012 | Journal of Medical Colleges of PLA(China)2012,27,5: | 1 |
| 16 | Characteristics of pediatric inpatients with primary and secondary hypertension显示文摘Importance:Extensive population-based studies have explored the prevalence of primary hypertension(HTN)in children and adolescents.However,there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.Objective:To investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.Methods:The study cohort comprised pediatric inpatients(<18 years of age)discharged with a diagnosis of HTN from Beijing Children's Hospital during 2015-2020.Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses,family history of HTN,and findings on physical examination,as documented in their medical records.The Mann-Whitney U test,χ^(2) and Fisher's exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.Results:Data of 1470 inpatients with HTN from 18 clinical departments were included in the analysis.Among them,458(31.2%)had primary HTN,and 1012(68.8%)had secondary HTN.Compared with patients had primary HTN,children with secondary HTN were younger and had lower body mass indexes and longer lengths of stay.Moreover,children with primary HTN had mostly been managed by the Endocrinology and Cardiology Departments,75.8%of them having obesity-related comorbidities.In contrast,most patients with secondary HTN had been managed by the Nephrology Department,renal diseases being the leading cause of their HTN(46.3%).Interpretation:Secondary HTN is more common than primary HTN in pediatric clinical settings,renal diseases being the leading cause of secondary HTN. | Yijun Chen Peiyu Ye Xiaorong Liu Chunxiu Gong Caifeng Li Yue Yuan Huyong Zheng Xin Xu Hongbo Dong Qin Kong Yinkun Yan Jie Mi | 2021 | Pediatric Investigation2021,5,1: | 0 |
| 17 | Treatment of STING-associated vasculopathy with onset in infancy显示文摘To the Editor:Gain-of-function mutations in TMEM173,which encodes the protein stimulator of interferon(IFN)genes(STING),can reportedly cause an autoinflammatory syndrome termed STING-associated vasculopathy with onset in infancy(SAVI).1-3 SAVI is characterized by cutaneous necrotic lesions,growth failure,systemic inflammation,and interstitial lung disease.1,4 Therapeutic management is challenging and difficult:steroids are only partially effective,and patients respond poorly to immunosuppressants. | Xiaozhen Zhao Junmei Zhang Caifeng Li | 2023 | Rheumatology & Autoimmunity2023,3,2: | 0 |
| 18 | The Effects of Turbulence Intensity and Tip Speed Ratio on the Coherent Structure of Horizontal-Axis Wind Turbine Wake:A Wind Tunnel Experiment显示文摘The evolution laws of the large-eddy coherent structure of the wind turbine wake have been evaluated via wind tunnel experiments under uniform and turbulent inflow conditions.The spatial correlation coefficients,the turbulence integral scales and power spectrum are obtained at different tip speed ratios(TSRs)based on the time-resolved particle image velocity(TR-PIV)technique.The results indicate that the large-eddy coherent structures are more likely to dissipate with an increase in turbulence intensity and TSR.Furthermore,the spatial correlation of the longitudinal pulsation velocity is greater than its axial counterpart,resulting into a wake turbulence dominated by the longitudinal pulsation.With an increase of turbulence intensity,the integral scale of the axial turbulence increases,meanwhile,its longitudinal counterpart decreases.Owing to an increase in TSR,the integral scale of axial turbulence decreases,whereas,that of the longitudinal turbulence increases.By analyzing the wake power spectrum,it is found that the turbulent pulsation kinetic energy of the wake structure is mainly concentrated in the low-frequency vortex region.The dissipation rate of turbulent kinetic energy increases with an increase of turbulence intensity and the turbulence is transported and dissipated on a smaller scale vortex,thus promoting the recovery of wake. | Yuxia Han Jianwen Wang Xin Li Xueqing Dong Caifeng Wen | 2022 | Energy Engineering2022,119,6: | 0 |
| 19 | Genomic analysis of schistosomiasis-associated colorectal cancer reveals a unique mutational landscape and therapeutic implications显示文摘The schistosomiasis-associated colorectal cancer (SA-CRC) is thought to be caused by the chronic inflammation as a result of schistosomal ova deposition in the submucosal layer of the intestine (Fig. S1). It is reported that SA-CRC differs from sporadic colorectal cancer (S-CRC) in terms of epidemiological features, histological patterns, and clinical outcomes,1 which is also supported by our institutional data (Table S1 and Fig. S2). Here we investigate the genomic alternations and probable molecular determinants of pathogenesis in Chinese SA-CRC. | Dong Yu Anqi Wang Jing Zhang Xinxing Li Caifeng Jiang Haiyang Zhou | 2023 | Genes & Diseases2023,10,3: | 0 |
| 20 | On the Toughness and Fractional Perfect Matching of Graphs显示文摘 | Jirumutu Zhenping Li Caifeng Du | 2006 | Journal of Systems Science and Information2006,4,3: | 0 |