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Peroral traction-assisted natural orifice trans-anal flexible endoscopic rectosigmoidectomy followed by intracorporeal colorectal anastomosis in a live porcine model 被引量:1
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作者 Hong Shi Su-Yu Chen +8 位作者 Zhao-Fei Xie Rui Huang Jia-Li Jiang Juan Lin Fang-Fen Dong Jia-Xiang Xu zhi-lifang Jun-Jie Bai Ben Luo 《World Journal of Gastrointestinal Endoscopy》 CAS 2020年第11期451-458,共8页
BACKGROUND Compared to traditional open surgery,laparoscopic surgery has become a standard approach for colorectal cancer due to its great superiorities including less postoperative pain,a shorter hospital stay,and be... BACKGROUND Compared to traditional open surgery,laparoscopic surgery has become a standard approach for colorectal cancer due to its great superiorities including less postoperative pain,a shorter hospital stay,and better quality of life.In 2007,Whiteford et al reported the first natural orifice trans-anal endoscopic surgery(NOTES)sigmoidectomy using transanal endoscopic microsurgery.To date,all cases of NOTES colorectal resection have included a hybrid laparoscopic approach with the use of established rigid platforms.AIM To introduce a novel technique of peroral external traction-assisted transanal NOTES rectosigmoidectomy followed by intracorporeal colorectal end-to-end anastomosis by using only currently available and flexible endoscopic instrumentation in a live porcine model.METHODS Three female pigs weighing 25-30 kg underwent NOTES rectosigmoid resection.After preoperative work-up and bowel preparation,general anesthesia combined with endotracheal intubation was achieved.One dual-channel therapeutic endoscope was used.Carbon dioxide insufflation was performed during the operation.The procedure of trans-anal NOTES rectosigmoidectomy included the following eight steps:(1)The rectosigmoid colon was tattooed with India ink by submucosal injection;(2)Creation of gastrostomy by directed submucosal tunneling;(3)Peroral external traction using endoloop ligation;(4)Creation of rectostomy on the anterior rectal wall by directed 3 cm submucosal tunneling;(5)Peroral external traction-assisted dissection of the left side of the colon;(6)Transanal rectosigmoid specimen transection,where an anvil was inserted into the proximal segment after purse-string suturing;(7)Intracorporeal colorectal end-toend anastomosis using a circular stapler by a single stapling technique;and(8)Closure of gastrostomy using endoscopic clips.All animals were euthanized immediately after the procedure,abdominal exploration was performed,and the air-under-water leak test was carried out.RESULTS The procedure was completed in all three animals,wit 展开更多
关键词 TRANSANAL Natural orifice trans-anal endoscopic surgery Rectosigmoidectomy Intracorporeal anastomosis External traction
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5例法布里病患者临床表现、基因变异及酶替代疗法效果分析 被引量:2
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作者 陈文清 李恒 +4 位作者 支丽芳 田炯 江艳 叶慧娟 陈江华 《中华肾脏病杂志》 CAS CSCD 北大核心 2022年第8期678-684,共7页
目的分析酶替代疗法治疗法布里病(Fabry disease)的有效性和安全性。方法回顾性收集和分析2020年7月至2021年5月期间浙江大学医学院附属第一医院收治的5例法布里病患者的临床资料、基因检测及家系调查结果、酶替代治疗效果和不良反应。... 目的分析酶替代疗法治疗法布里病(Fabry disease)的有效性和安全性。方法回顾性收集和分析2020年7月至2021年5月期间浙江大学医学院附属第一医院收治的5例法布里病患者的临床资料、基因检测及家系调查结果、酶替代治疗效果和不良反应。5例患者阿加糖酶β用量为1 mg·kg^(-1)·次^(-1)静脉泵注,每2周用药1次。结果5例患者均为男性,中位年龄37岁(29~51岁),均通过结合临床表现、家族史、肾病理检查、α⁃半乳糖苷酶A(α⁃Gal A)活性检测及基因检测结果明确诊断。临床表现多样,所有患者均有尿检异常及心电图和心脏B超异常,肾功能不全4例,幼年时肢端疼痛3例(持续至成年1例),皮肤血管角化瘤3例,少汗4例,腹泻4例,角膜涡状混浊4例,高频听力异常2例。基因测序结果显示,2例为GLA基因错义变异[c.272T>C(p.I91T),c.868A>G(p.Met290Val)],1例为移码突变[c.348del p.(Ile117Phefs*4)],2例为无义突变[c.1024C>T(p.Arg342*),c.838C>T(p.Gln280*)],其中移码突变患者表现为经典型法布里病,该变异在已知数据库中未见收录。5例患者输注阿加糖酶β过程中均未发生严重不良反应。酶替代治疗2~10个月后,5例患者血浆三己糖酰基鞘脂醇(Lyso⁃GL⁃3)水平较治疗前显著下降(P<0.05),4例患者长期腹泻症状在用药后明显改善。结论法布里病临床表现和基因变异多样,短期阿加糖酶β治疗未见严重不良反应,治疗后患者血浆Lyso⁃GL⁃3水平显著下降。 展开更多
关键词 法布里病 基因变异 酶替代治疗 临床表现 不良反应
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