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6. Growth hormone treatment in pre-pubertal short Chinese children with chronic kidney disease prior to transplantation

Author

Tang, Xiaoshan, Chen, Qiuxia, Chen, Jing, Fang, Xiaoyan, Zhang, Aihua, Zhao, Fei, Huang, Wenyan, Wang, Ping, Sun, Liwen, Xiao, Huijie, Xu, Ke, Liu, Xiaorong, Chen, Zhi, Chen, Chaoying, Tu, Juan, Wu, Yubin, Wang, Xiuli, Mao, Jianhua, Lu, Zhihong, Wang, Jingjing, Nie, Xiaojing, Yu, Zihua, Huang, Jun, Liu, Cuihua, Cao, Guanghai, Li, Yufeng, Zhu, Yaju, Zhang, Jianjiang, Wang, Miao, Wang, Mo, Yang, Haiping, Shen, Qian, and Xu, Hong

Published
2023
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15. Clinical Characteristics and Treatment of Blau Syndrome in Chinese Children-a National Multicenter Study

Author

ZHANG Junmei, ZHAO Xiaozhen, TANG Xuemei, ZHAO Yi'nan, LI Li, GAO Fengqiao, SHI Xinwei, JIN Yanliang, ZHANG Yu, CAO Lanfang, YIN Wei, XIAO Jihong, KUANG Weiying, DENG Jianghong, WANG Jiang, TAN Xiaohua, LI Chao, LI Shipeng, XUE Haiyan, LIU Cuihua, LIU Xiaohui, ZHAO Dongmei, CHEN Yuqing, ZHENG Wenjie, and LI Caifeng

Subjects
blau syndrome, demographic characteristics, clinical manifestations, treatment, Medicine
Abstract

Objective To study the demographic and clinical characteristics, correlation of genotype and phenotype and treatment of Blau syndrome to facilitate early diagnosis and timely treatment of Blau syndrome. Methods Seventy-two patients with Blau syndrome from 11 centers from May 2006 to April 2022 were retrospectively analyzed, and their general information, clinical data, laboratory examination and treatment medication were collected. Results The distribution of patients with Blau syndrome was uniform in geographical north and south of China, and there was no obvious gender bias. The mean age of onset was (14.30±12.81) months, and the age of diagnosis was (55.18±36.22) months. 35% of patients with Blau syndrome happened before 1 year old, and all patients developed before 5 years old. 87.50% (63/72) had granulomatous arthritis, 65.28% (47/72) had rash, 36.11% (26/72) had ocular involvement, 27.78% (20/72) had fever, and 15.28% (11/72) had pulmonary involvement. Arthritic manifestations of Blau syndrome were most at risk, followed by rash, ocular involvement, and fever.The first 25 months of the disease, the risk of developing a rash was the greatest. The risk of developing arthritis was the greatest between 25 months and 84 months. The main mutations were p.R334Q and p.R334W, and patients with p.R334Q mutation had relatively high incidence of fever (35.71%[5/14] vs. 14.29%[1/7], P=0.43) and ocular involvement (42.86%[6/14]vs. 28.57%[2/7], P=0.51). There was a relatively high incidence of rash (85.71%[6/7] vs. 64.29%[9/14], P=0.59) in patients with the p.R334W mutation. Forty-five patients(62.50%)were treated with a combina-tion of glucocorticoid and methotrexate. Twenty-two patients were treated with tumor necrosis factor antagonist in addition to glucocorticoid and methotrexate. Conclusions The risk of different clinical manifestations of Blau syndrome from high to low was arthritis, followed by rash, ocular involvement and fever. The main treatment was glucocorticoid combined with methotrexate, to which biological agents could be added.

Published
2022
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17. Oral Coenzyme Q10 supplementation leads to better preservation of kidney function in steroid-resistant nephrotic syndrome due to primary Coenzyme Q10 deficiency

Author

Baiko, Sergey, Serna Higuita, Lina Maria, Schaefer, Franz, Trautmann, Agnes, Tabatabaeifar, Mansoureh, Gheissari, Alaleh, Hooman, Nakysa, Benetti, Elisa, Emma, Francesco, Nigmatullina, Nazym, Lipska-Ziętkiewicz, Beata S., Bałasz-Chmielewska, Irena, Tkaczyk, Marcin, Stańczyk, Małgorzata, Borzecka, Halina, Tsygin, Alexey N., Prikhodina, Larisa, Bogdanovic, Radovan, Anarat, Ali, Ozaltin, Fatih, Mir, Sevgi, Fomina, Svitlana, Klopstock, Thomas, Prokisch, Holger, Kornblum, Cornelia, Xu, Hong, Shen, Qian, Rao, Jia, Liu, Cui-Hua, Sun, Shu-Zhen, Deng, Fang, Dong, Yang, Wang, Xiao-Wen, Luan, Jiang-Wei, Drovandi, Stefania, Gulhan, Bora, Boyer, Olivia, Riedhammer, Korbinian M., Heemann, Uwe, Hoefele, Julia, Stenton, Sarah L., Ng, Kar-Hui, Aurelle, Manon, Schreuder, Michiel F., Jankowski, Maciej, Mao, Jianhua, Feng, Chunyue, Liu, Cuihua, Sun, Shuzhen, Wang, Xiaowen, Clavé, Stéphanie, Fila, Marc, Durkan, Anne M., Levart, Tanja Kersnik, Dursun, Ismail, Esfandiar, Nasrin, Haas, Dorothea, Bjerre, Anna, Benz, Marcus R., Talebi, Saeed, and Ariceta, Gema

Published
2022
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20. Synergetic control of multi‐level interface structure of basalt fibers by plasma and chemical functionalization to enhance the mechanical, thermal and interfacial performances of PBZ composites.

Author

Zhao, Wei, Zhang, Aiping, Li, Xinkang, Lin, Hai Lan, Ni, Keyang, Bian, Jun, Jing, Siyi, Yang, Ke Cheng, Yang, Shangke, Liu, Cuihua, and Chen, Daiqiang

Abstract

The composites of polybenzoxazine (PBZ) containing functionalized plasma treatment basalt fibers (fP‐BFs) were fabricated through in‐situ polymerization‐mixing technique. fP‐BFs is an effective reinforcement material that can improve the mechanical, thermal, and interfacial properties of composites. Surface activation of fP‐BFs can be enhanced through plasma modification, which improves the interaction between fP‐BFs and 3‐aminopropyltriethoxysilane. A systematic study on fP‐BFs/PBZ composite showed that a processing power of 300 W, a processing time of 5 min, and a fiber content of 9% can achieve good comprehensive mechanical properties. Thermogravimetric analysis results showed that fP‐BFs‐7/PBZ has the highest thermal stability, and its carbon residue rate, decomposition temperature and thermal decomposition activation energy are 36.80%, 452.2 °C and 133.56 kJ/mol, respectively. The dynamic mechanical analysis experiment showed that the addition of fP‐BFs improved the Tg of the composites. Among them, fP‐BFs‐5/PBZ showed the highest Tg, reaching 233.2 °C. Highlights: Plasma treatment produced multi‐level structure of basalt fibers (fP‐BFs).Polybenzoxazine (PBZ)/fP‐BFs composites showed excellent performance.Interfacial bonding mechanism between fP‐BFs and PBZ was elucidated.Interfacial interactions between BFs and PBZ contributed to property enhancement.This work provided a green fabrication strategy of PBZ composites. [ABSTRACT FROM AUTHOR]

Published
2024
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23. Genetic Architecture of Childhood Kidney and Urological Diseases in China

Author

Fang, Ye, Shi, Hua, Xiang, Tianchao, Liu, Jiaojiao, Liu, Jialu, Tang, Xiaoshan, Fang, Xiaoyan, Chen, Jing, Zhai, Yihui, Shen, Qian, Li, Guomin, Sun, Li, Bi, Yunli, Wang, Xiang, Qian, Yanyan, Wu, Bingbing, Wang, Huijun, Zhou, Wenhao, Ma, Duan, Mao, Jianhua, Jiang, Xiaoyun, Sun, Shuzhen, Shen, Ying, Liu, Xiaorong, Zhang, Aihua, Wang, Xiaowen, Huang, Wenyan, Li, Qiu, Wang, Mo, Gao, Xiaojie, Wu, Yubin, Deng, Fang, Zhang, Ruifeng, Liu, Cuihua, Yu, Li, Zhuang, Jieqiu, Sun, Qing, Dang, Xiqiang, Bai, Haitao, Zhu, Ying, Lu, Siguang, Zhang, Bili, Shao, Xiaoshan, Liu, Xuemei, Han, Mei, Zhao, Lijun, Liu, Yuling, Gao, Jian, Bao, Ying, Zhang, Dongfeng, Ma, Qingshan, Zhao, Liping, Xia, Zhengkun, Lu, Biao, Wang, Yulong, Zhao, Mengzhun, Zhang, Jianjiang, Jian, Shan, He, Guohua, Zhang, Huifeng, Zhao, Bo, LI, Xiaohua, Wang, Feiyan, Li, Yufeng, Zhu, Hongtao, Luo, Xinhui, Li, Jinghai, Rao, Jia, and Xu, Hong

Published
2021
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27. Chrysomycins, Anti-Tuberculosis C-Glycoside Polyketides from Streptomyces sp. MS751.

Author

Yu, Jiaming, Guo, Hui, Zhang, Jing, Hu, Jiansen, He, Hongtao, Chen, Caixia, Yang, Na, Yang, Fan, Lin, Zexu, Dai, Huanqin, Ouyang, Liming, Liu, Cuihua, Lei, Xiaoguang, Zhang, Lixin, Zhu, Guoliang, and Song, Fuhang

Abstract

A new dimeric C-glycoside polyketide chrysomycin F (1), along with four new monomeric compounds, chrysomycins G (2), H (3), I (4), J (5), as well as three known analogues, chrysomycins A (6), B (7), and C (8), were isolated and characterised from a strain of Streptomyces sp. obtained from a sediment sample collected from the South China Sea. Their structures were determined by detailed spectroscopic analysis. Chrysomycin F contains two diastereomers, whose structures were further elucidated by a biomimetic [2 + 2] photodimerisation of chrysomycin A. Chrysomycins B and C showed potent anti-tuberculosis activity against both wild-type Mycobacterium tuberculosis and a number of clinically isolated MDR M. tuberculosis strains. [ABSTRACT FROM AUTHOR]

Published
2024
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40. Gut microbiota and intestinal FXR mediate the clinical benefits of metformin

Author

Sun, Lulu, Xie, Cen, Wang, Guang, Wu, Yue, Wu, Qing, Wang, Xuemei, Liu, Jia, Deng, Yangyang, Xia, Jialin, Chen, Bo, Zhang, Songyang, Yun, Chuyu, Lian, Guan, Zhang, Xiujuan, Zhang, Heng, Bisson, William H., Shi, Jingmin, Gao, Xiaoxia, Ge, Pupu, Liu, Cuihua, Krausz, Kristopher W., Nichols, Robert G., Cai, Jingwei, Rimal, Bipin, Patterson, Andrew D., Wang, Xian, Gonzalez, Frank J., and Jiang, Changtao

Published
2018
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44. The impact of DNA double‐strand break repair pathways throughout the carbon ion spread‐out Bragg peak beam.

Author

Buglewicz, Dylan J., Buglewicz, Jessica K. F., Hirakawa, Hirokazu, Kato, Takamitsu A., Liu, Cuihua, Fang, YaQun, Kusumoto, Tamon, Fujimori, Akira, and Sai, Sei

Abstract

Following carbon ion beam irradiation in mammalian cells, such as used in carbon ion radiotherapy (CIRT), it has been suggested that the balance between whether nonhomologous end joining (NHEJ) or homologous recombination (HR) is utilized depends on the DNA double‐strand break (DSB) complexity. Here, we quantified DSB distribution and identified the importance of each DSB repair pathway at increasing depths within the carbon ion spread‐out Bragg peak (SOBP) beam range. Chinese hamster ovary (CHO) cell lines were irradiated in a single biological system capable of incorporating the full carbon ion SOBP beam range. Cytotoxicity and DSB distribution/repair kinetics were examined at increasing beam depths using cell survival as an endpoint and γ‐H2AX as a surrogate marker for DSBs. We observed that proximal SOBP had the highest number of total foci/cell and lowest survival, while distal SOBP had the most dense tracks. Both NHEJ‐ and HR‐deficient CHO cells portrayed an increase in radiosensitivity throughout the full carbon beam range, although NHEJ‐deficient cells were the most radiosensitive cell line from beam entrance up to proximal SOBP and demonstrated a dose‐dependent decrease in ability to repair DSBs. In contrast, HR‐deficient cells had the greatest ratio of survival fraction at entrance depth to the lowest survival fraction within the SOBP and demonstrated a linear energy transfer (LET)‐dependent decrease in ability to repair DSBs. Collectively, our results provide insight into treatment planning and potential targets to inhibit, as HR was a more beneficial pathway to inhibit than NHEJ to enhance the cell killing effect of CIRT in targeted tumor cells within the SOBP while maintaining limited unwanted damage to surrounding healthy cells. [ABSTRACT FROM AUTHOR]

Published
2023
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48. Recombinant cell-detecting RaDR-GFP in mice reveals an association between genomic instability and radiation-induced-thymic lymphoma

Author

Akira, Fujimori, Hirokazu, Hirakawa, Liu, Cuihua, Taishin, Akiyama, P. Engelward, Bevin, A. Nickoloff, Jac, Masao, Suzuki, Bing, Wang, Mitsuru, Nenoi, and Sei, Sai

Abstract

In this study, we aimed to investigate how homologous recombinant (HR)-related genomic instability is involved in ionizing radiation (IR)-induced thymic lymphoma in mice. We divided five-week-old Rosa26 Direct Repeat- GFP (RaDR-GFP) transgenic mice into non-IR control and IR groups and exposed the mice in the IR group to a 7.2 Gy dose of γ-rays, delivered in 1.8 Gy fractions, once a week for four weeks. We then estimated mouse survival and recorded their body, thymus, and spleen weights. The frequency of HR events in the chromosomes of the thymus, bone marrow, and spleen cells and the phenotype of thymic lymphoma cells were analyzed using fluorescence- activated cell sorting (FACS). We found that most mice in the IR group developed thymic lymphoma, their survival rate decreasing to 20% after 180 days of IR exposure, whereas no mice died in the non-IR control group until day 400. The thymus and spleen weighed significantly more in the IR-4-month group than that in the non-IR group; however, we observed no significant differences between the body weights of the control and IR mice. FACS analysis indicated that the frequency of HR events significantly increased at two and four months after the last IR dose in the bone marrow and thymus cells, but not in the spleen cells of RaDR-GFP transgenic mice, suggesting that recombi- nant cells accumulated in the thymus upon IR exposure. This suggests that IR induces genome instability, revealed as increased HR, that drives the development of thymic lymphoma. Additionally, phenotypic analysis of lymphoma cells showed an increase in the CD4-/CD8+ (CD8SP) cell population and a decrease in the CD4+/CD8- (CD4SP) cell population in the IR-4-month group compared to that in the non-IR group, indicating that IR induces an aberrant cell phenotype characteristic of lymphoma. In conclusion, we observed a significant increase in HR events and abnormal phenotype in thymic lymphoma cells at two and four months after IR exposure in both the thymus and bone marrow tissues, suggesting that genomic instability is involved in the early stages of thymic lymphomagenesis. Our study indicates that HR-visualizing RaDR-GFP transgenic mice can help explore the links between the molecular mecha- nisms of genome instability and IR-induced tumorigenesis.

Published
2022

49. Hypoxia and Proton microbeams: Role of Gap Junction Intercellular Communication in Inducing Bystander Responses on Human Lung Cancer Cells and Normal Cells

Author

Autsavapromporn, Narongchai, Kobayashi, Alisa, Liu, Cuihua, Jaikang, Churdsak, Ahbrizal Tengku Ahmad, Tengku, Oikawa, Masakazu, Konishi, Teruaki, Alisa, Kobayashi, Masakazu, Oikawa, and Teruaki, Konishi

Abstract

Hypoxia plays an important role in the development of malignant solid tumor and is associated with therapeutic resistance and poor outcome treatment. This condition may be due to the ability of hypoxic cells to influence bystander response through gap junction intercellular communication (GJIC) that may reduce the effectiveness of radiation therapy (RT) and increase normal tissue toxicity. The present study is aimed to investigate the role of GJIC and hypoxia-induced bystander response in lung cancer (A549) and normal lung fibroblast (WI-38) under normoxia and hypoxia, when exposed to high LET (linear energy transfer) proton microbeams. The results of our study showed that for the first time there was a difference hypoxia-induced bystander response be-tween cancer and normal cells and GJIC plays a key role in the observed effects.

Published
2021

50. Growth hormone treatment in pre-pubertal short Chinese children with chronic kidney disease prior to transplantation

Author

Tang, Xiaoshan, primary, Chen, Qiuxia, additional, Chen, Jing, additional, Fang, Xiaoyan, additional, Zhang, Aihua, additional, Zhao, Fei, additional, Huang, Wenyan, additional, Wang, Ping, additional, Sun, Liwen, additional, Xiao, Huijie, additional, Xu, Ke, additional, Liu, Xiaorong, additional, Chen, Zhi, additional, Chen, Chaoying, additional, Tu, Juan, additional, Wu, Yubin, additional, Wang, Xiuli, additional, Mao, Jianhua, additional, Lu, Zhihong, additional, Wang, Jingjing, additional, Nie, Xiaojing, additional, Yu, Zihua, additional, Huang, Jun, additional, Liu, Cuihua, additional, Cao, Guanghai, additional, Li, Yufeng, additional, Zhu, Yaju, additional, Zhang, Jianjiang, additional, Wang, Miao, additional, Wang, Mo, additional, Yang, Haiping, additional, Shen, Qian, additional, and Xu, Hong, additional

Published
2022
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