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| 1 | Solvothermal synthesis of V2O5/graphene nanocomposites for high performance lithium ion batteries 显示文摘 | Chen Da Yi Ran Chen Shuru | 2014 | Materials Science and Engineering B2014，185，： | 1 |
| 2 | Diagnosis and treatment of icteric hepatitis caused by erythropoietic protoporphyria:A case report显示文摘Erythropoietic protoporphyria(EPP)is a rare inherited disease caused by partial deficiency activity of the enzyme ferrochelatase(FECH),resulting in excessive accumulation of protoporphyrin IX in erythrocyte and tissues.Here,we report a patient with photosensitive dermatitis and acute icteric hepatitis caused by EPP,whose clinical and biochemical results successfully improved following 2-month treatment with glucose load,ursodeoxycholic acid capsules,and cholestyramine powder.This case provides a reference for a combination therapy strategy for patients with liver and skin injury caused by EPP. | Hanqing Huang Leiqin Cai Xinhua Li Shuru Chen | 2022 | Liver Research2022，6，2： | 0 |
| 3 | A novel nomogram based on routine clinical indicators for screening for Wilson's disease显示文摘Background and aims:There is currently no single model for predicting Wilson's disease(WD).We aimed to create a nomogram using daily clinical parameters to improve the accuracy of WD diagnosis in patients with abnormal liver function.Methods:Between July 2016 and December 2020,we identified 90 WD patients with abnormal liver function who had homozygous or compound heterozygous mutations in the ATP7B gene.The control group included 128 patients with similar liver function but no WD during the same time period.To create a nomogram,we screened potential predictive variables using the least absolute shrinkage and selection operator model and multivariate logistic regression.Results:We developed a nomogram for screening for WD based on six predictive factors:serum copper,direct bilirubin,uric acid,cholinesterase,prealbumin,and reticulocyte percentage.In the training cohort,the area under curve(AUC)of the nomogram reached 0.967(95%confidence interval(CI)0.946e0.988),while the area under the precision-recall curve was 0.961.Based on the optimal cutpoint of 213.55,our nomogram performed well,with a sensitivity of 96%and a specificity of 87%.In the validation cohort,the AUC of the nomogram was as high as 0.991(95%CI 0.970e1.000).Conclusions:We developed a nomogram that can predict the risk of WD prior to the detection of serum ceruloplasmin or urinary copper,greatly increasing screening efficiency for patients with abnormal liver function. | Jiahui Pang Shuru Chen Weiqiang Gan Guofang Tang Yusheng Jie Zhanyi Li Yutian Chong Youming Chen Jiao Gong Xinhua Li Yongyu Mei | 2023 | Liver Research2023，7，1： | 0 |
| 4 | Clinical and Genetic Characteristics of Alagille Syndrome in Adults显示文摘Background and Aims:Alagille syndrome(AGS)is an autosomal dominant multisystem disorder caused by mutations in the JAG1 and NOTCH2 genes.AGS has been rarely reported in adult patients,mainly because its characteristics in adults are subtle.The study aimed to improve the understanding of adult AGS by a descriptive case series.Methods:Eight adults diagnosed with AGS at our hospital between June 2016 and June 2019 were included in the study.Clinical data,biochemical results,imaging results,liver histopathology,and genetic testing were analyzed.Results:Three female and five male patients with a median age of 24.5 years at the time of diagnosis were included in the analysis.The clinical manifestations were adult-onset(62.5%,5/8),cholestasis(50%,4/8),butterfly vertebrae(62.5%,5/8),systolic murmurs(12.5%,1/8),typical facies(12.5%,1/8),posterior embryotoxon,and renal abnormalities(0/8).Genetic sequencing showed that all patients had mutations,with four occurring in the JAG1 gene and four in the NOTCH2 gene.Six were substitution mutations,one was a deletion mutation,and one was a splicing mutation.Five had been previously reported;but the others,one JAG1 mutation and two NOTCH2 mutations were unique and are reported here for the first time.Conclusions:The clinical manifestations highlighted by the current diagnostic criteria for most adults with AGS are atypical.Those who do not meet the criteria but are highly suspicious of having AGS need further evaluation,especially genetic testing. | Jianguo Li Haicong Wu Shuru Chen Jiahui Pang Heping Wang Xinhua Li Weiqiang Gan | 2023 | Journal of Clinical and Translational Hepatology2023，11，1： | 0 |
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