|
|
|
题名
|
作者
|
年代
|
出处
|
被引量
|
| 1 | A novel long-term intravenous combined with local treatment with human amnion-derived mesenchymal stem cells for a multidisciplinary rescued uremic calciphylaxis patient and the underlying mechanism显示文摘Calciphylaxis is a rare disease characterized histologically by microvessel calcification and microthrombosis,with high mortality and no proven therapy.Here,we reported a severe uremic calciphylaxis patient with progressive skin ischemia,large areas of painful malodorous ulcers,and mummified legs.Because of the worsening symptoms and signs refractory to conventional therapies,treatment with human amnion-derived mesenchymal stem cells(hAMSCs)was approved.Preclinical release inspections of hAMSCs,efficacy,and safety assessment,including cytokine secretory ability,immunocompetence,tumorigenicity,and genetics analysis in vitro,were introduced.We further performed acute and long-term hAMSC toxicity evaluations in C57BL/6 mice and rats,abnormal immune response tests in C57BL/6 mice,and tumorigenicity tests in neonatal Balbc-nu nude mice.After the preclinical research,the patient was treated with hAMSCs by intravenous and local intramuscular injection and external supernatant application to the ulcers.When followed up to 15 months,the blood-based markers of bone and mineral metabolism improved,with skin soft tissue regeneration and a more favorable profile of peripheral blood mononuclear cells.Skin biopsy after 1-month treatment showed vascular regeneration with mature noncalcified vessels within the dermis,and 20 months later,the re-epithelialization restored the integrity of the damaged site.No infusion or local treatment-related adverse events occurred.Thus,this novel long-term intravenous combined with local treatment with hAMSCs warrants further investigation as a potential regenerative treatment for uremic calciphylaxis due to effects of inhibiting vascular calcification,stimulating angiogenesis and myogenesis,anti-inflammatory and immune modulation,multidifferentiation,re-epithelialization,and restoration of integrity. | Lianju Qin Jing Zhang Yujie Xiao Kang Liu Yugui Cui Fangyan Xu Wenkai Ren Yanggang Yuan Chunyan Jiang Song Ning Xiaoxue Ye Ming Zeng Hanyang Qian Anning Bian Fan Li Guang Yang Shaowen Tang Zhihong Zhang Juncheng Dai Jing Guo Qiang Wang Bin Sun Yifei Ge Chun Ouyang Xueqiang Xu Jing Wang Yaoyu Huang Hongqing Cui Jing Zhou Meilian Wang Zhonglan Su Yan Lu Di Wu Jingping Shi Wei Liu Li Dong Yinbing Pan Baiqiao Zhao Ying Cui Xueyan Gao Zhanhui Gao Xiang Ma Aiqin Chen Jie Wang Meng Cao Qian Cui Li Chen Feng Chen Youjia Yu Qiang Ji Zhiwei Zhang Mufeng Gu Xiaojun Zhuang Xiaolin Lv Hui Wang Yanyan Pan Ling Wang Xianrong Xu Jing Zhao Xiuqin Wang Cuiping Liu Ningxia Liang Changying Xing Jiayin Liu Ningning Wang | 2022 | Journal of Molecular Cell Biology2022，14，2： | 1 |
| 2 | Stability of Fe-As composites formed with As(Ⅴ)and aged ferrihydrite显示文摘During the aging process,ferrihydrite was transformed into mineral mixtures composed of different proportions of ferrihydrite,goethite,lepidocrocite and hematite.Such a transformation may affect the fixed ability of arsenic.In this study,the stability of Fe-As composites formed with As(Ⅴ)and the minerals aged for 0,1,4,10 and 30 days of ferrihydrite were systematically examined,and the effects of molar of ratios Fe/As were also clarified using kinetic methods combined with multiple spectroscopic techniques.The results indicated that As(Ⅴ)was rapidly adsorbed on minerals during the initial polymerization process,which delayed both the ferrihydrite conversion and the hematite formation.When the Fe/As molar ratio was 1.875 and 5.66,the As(Ⅴ)adsorbed by ferrihydrite began to release after 6 hr and 12 hr,respectively.The corresponding release amounts of As(Ⅴ)were 0.55 g/L and 0.07 g/L,and the adsorption rates were 92.43%and 97.50%at 60 days,respectively.However,the As(Ⅴ)adsorbed by the transformation products aged for 30 days of ferrihydrite began to release after adsorbed 30 days.The corresponding release amounts of As(Ⅴ)were 0.25 g/L and 0.03 g/L,and the adsorption rates were 84.23%and 92.18%after adsorbed 60 days,for the Fe/As=1.875 and 5.66,respectively.Overall,the combination of As(Ⅴ)with ferrihydrite and aged products transformed from a thermodynamically metastable phase to a dynamically stable state within a certain duration.Moreover,the aging process of ferrihydrite reduced the sorption ability of arsenate by iron(hydr)oxide but enhanced the stability of the Fe-As composites. | Zhonglan Yang Lingyu Bai Shiming Su Yanan Wang Cuixia Wu Xibai Zeng Benhua Sun | 2021 | Journal of Environmental Sciences2021，33，2： | 0 |
| 3 | A novel variant in the GJB6 gene in a large Chinese family with a unique phenotype of Clouston syndrome显示文摘Clouston syndrome(OMIM#129500),also known as hidrotic ectodermal dysplasia type 2,is a rare autosomal dominant skin disorder.To date,four mutations in the GJB6 gene,G11R,V37E,A88V,and D50N,have been confirmed to cause this condition.In previous studies,the focus has been mainly on gene sequencing,and there has been a lack of research on clinical manifestations and pathogenesis.To confirm the diagnosis of this pedigree at the molecular level and summarize and analyse the clinical phenotype of patients and to provide a basis for further study of the pathogenesis of the disease,we performed whole-exome and Sanger sequencing on a large Chinese Clouston syndrome pedigree.Detailed clinical examination included histopathology,hair microscopy,and scanning electron microscopy.We found a novel heterozygous missense variant(c.134G>C:p.G45A)for Clouston syndrome.We identified a new clinical phenotype involving all nail needling pain in all patients and found a special honeycomb hole structure in the patients’hair under scanning electron microscopy.Our data reveal that a novel variant(c.134G>C:p.G45A)plays a likely pathogenic role in this pedigree and highlight that genetic testing is necessary for the diagnosis of Clouston syndrome. | Hequn Huang Mengyun Chen Xia Liu Xixi Xiong Lanbo Zhou Zhonglan Su Yan Lu Bo Liang | 2023 | Frontiers of Medicine2023，17，2： | 0 |
您所在的机构暂未开通此部分的功能，请联系管理员