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| 1 | Peutz-Jeghers综合征发病机制及临床诊治研究进展显示文摘Peutz-Jeghers综合征(peutz-jeghers syndrome, PJS)是一种常染色体显性遗传疾病,目前已被证实的致病基因为STK11.该病的特征性表现为皮肤黏膜色素斑、消化道多发错构瘤性息肉及肿瘤易感性.基于目前研究报道, STK11胚系突变可能通过干扰正常的细胞凋亡、细胞分裂G1期阻滞、细胞极化及细胞间质水平的细胞增殖抑制等导致了PJS消化道息肉及恶性肿瘤的发生.治疗消化道息肉及息肉相关并发症是干预该病的主要手段.近年来,气囊辅助小肠镜治疗PJS小肠息肉经验不断积累、安全性得到了充分认可,使得大部分患者避免了外科开腹手术,这对改善了患者生活质量及远期预后有重要意义.另外,针对PJS随年龄增长消化道息肉生长特点及显著增加消化道及非消化道恶性肿瘤风险,我们按照年龄阶段进行有差别有针对的随访策略. | 李白容 孙涛 蒋宇亮 宁守斌 | 2019 | 世界华人消化杂志2019，27，9： | 10 |
| 2 | Endoscopicultrasonographyforsurveillanceofindividualsathighriskfor pancreaticcancer显示文摘Pancreatic cancer is a highly lethal disease with a ge-netic susceptibility and familial aggregation found in 3%-16% of patients. Early diagnosis remains the only hope for curative treatment and improvement of prog-nosis. This can be reached by the implementation of an intensive screening program, actually recommended for individuals at high-risk for pancreatic cancer de-velopment. The aim of this strategy is to identify pre-malignant precursors or asymptomatic pancreatic can-cer lesions, curable by surgery. Endoscopic ultrasound (EUS) with or without fine needle aspiration(FNA) seems to be the most promising technique for early de-tection of pancreatic cancer. It has been described as a highly sensitive and accurate tool, especially for small and cystic lesions. Pancreatic intraepithelial neoplasia, a precursor lesion which is highly represented in high-risk individuals, seems to have characteristics chronic pancreatitis-like changes well detected by EUS. Many screening protocols have demonstrated high diagnostic yields for pancreatic pre-malignant lesions, allowing prophylactic pancreatectomies. However, it shows a high interobserver variety even among experienced en-dosonographers and a low sensitivity in case of chronic pancreatitis. Some new techniques such as contrast-en-hanced harmonic EUS, computer-aided diagnostic tech-niques, confocal laser endomicroscopy miniprobe andthe detection of DNA abnormalities or protein markersby FNA, promise improvement of the diagnostic yield ofEUS. As the resolution of imaging improves and as ourknowledge of precursor lesions grows, we believe thatEUS could become the most suitable method to detectcurable pancreatic neoplasms in correctly identifiedasymptomatic at-risk patients. | Lami G Biagini MR Galli A | 2014 | World Journal of Gastrointestinal Endoscopy2014，6，7： | 8 |
| 3 | 结直肠非肿瘤性息肉的临床病理学分析显示文摘目的探讨结直肠非肿瘤性息肉的临床病理学特点,为临床及病理诊断提供帮助。方法收集哈尔滨医科大学附属第二医院2012年1月至2016年12月肠镜及手术切除病例共1 190例,分析结直肠非肿瘤性息肉患者的临床病理学特征。结果行肠镜及手术切除1 190例中,男性746例,女性444例,男女比例为1.7∶1.0;平均发病年龄为52岁;40岁以上者1 016例(85.4%)。1 190例患者共检出1 289枚息肉,其中炎症性息肉1 238枚(96.0%),错构瘤性息肉47枚(3.7%),其他类型的息肉性病变4枚(0.3%)。1 238枚炎症性息肉中,炎性息肉1 212枚(97.9%);炎症后息肉15枚(1.2%);炎性肌腺性息肉8枚(0.6%);黏膜脱垂相关性炎症性息肉3枚(0.2%),均为深在性囊性结肠炎。47枚错构瘤性息肉中,幼年性息肉39枚(83.0%),Peutz-Jeghers息肉8枚(17.0%)。其他类型的息肉性病变的4枚息肉均为结直肠子宫内膜异位症。1 289枚息肉中,751枚息肉发生于乙状结肠和直肠(58.3%)。结论结直肠非肿瘤性息肉多见于40岁以上的患者;好发于乙状结肠和直肠;病理类型以炎症性息肉最多见,而炎症性息肉中以炎性息肉最多见;错构瘤性息肉以幼年性息肉最多见。 | 姜磊 焦宇飞 | 2019 | 中华病理学杂志2019，48，2： | 8 |
| 4 | ConservativeapproachinPeutz-JeghersSyndrome:Single-balloon enteroscopyandsmallbowelpolypectomy显示文摘AIM: To assess the usefulness of the balloon assisted enteroscopy in preventing surgical intervention in pa-tients with Peutz-Jeghers syndrome (PJS) having a small bowel large polyps. METHODS: Seven consecutive asymptomatic pts(age 15-38 years) with PJS have been collected; six under-went polypectomy using single balloon enteroscopy(Olympus SIF Q180) with antegrade approach using push and pull technique. SBE system consists of the SIF-Q180 enteroscope, an overtube balloon control unit(OBCU Olympus Balloon Control Unit) and a dispos-able silicone splinting tube with balloon(ST-SB1). All procedures were performed under general anesthesia. Previously all pts received wireless capsule endos-copy(WCE). Prophylactic polypectomy was reservedmainly in pts who had polyps > 15 mm in diameter. The balloon is inflated and deflated by a balloon control unit with a safety pressure setting range from-6.0 kPa to +5.4 kPa. Informed consent has been obtained from pts or parents for each procedure.RESULTS: Six pts underwent polypectomy of small bowel polyps; in 5 pts a large polyp > 15 mm(range 20-50 mm in diameter) was resected; in 1 patient with WCE negative, SBE was performed for previous surgi-cal resection of gastrointestinal stromal tumors. In 2 pts endoscopic clips were placed due to a polypectomy. No surgical complication have been reported. SBE with resection of small bowel large polyps in PJS pts was useful to avoid gastrointestinal bleeding and emergency laparotomy due to intestinal intussuscep-tions. No gastrointestinal tumors were found in sub-sequent enteroscopic surveillance in all seven pts. In order surveillance, all pts received WCE, upper en-doscopy, ileocolonoscopy every 2 years. No pts had extraintestinal malignant lesions. SBE was performed when WCE was positive for significant polyps(> 15 mm).CONCLUSION: The effective of prophylactic polyp-ectomy of small bowel large polyps(> 15 mm) could be the first line treatment for conservative approach in management of PJS patients. | Torroni F Romeo E Rea F De Angelis P Foschia F Faraci S Federici di Abriola G Contini AC Caldaro T Dall’Oglio L | 2014 | World Journal of Gastrointestinal Endoscopy2014，6，7： | 7 |
| 5 | Peutz-Jeghers综合征的诊治进展和预防性治疗显示文摘Peutz-Jeghers综合征(Peutz-Jeghers syndrome,PJS)以皮肤黏膜色素斑、胃肠道错构瘤息肉和遗传性为临床特征.PJS胃肠道息肉可产生梗阻、出血、套叠、恶变等严重并发症,目前其临床治疗以手术和内镜治疗为主,其中双气囊电子小肠镜对于PJS胃肠道息肉的诊断和治疗具有重要的临床意义.随着转化医学的进步,分子靶向治疗为PJS胃肠道息肉带来预防性治疗的新途径,其代表就是环氧合酶-2的选择性抑制剂.而以'济生乌梅丸'为代表的中药也为PJS息肉的预防性治疗提供另一个选择.本文在总结近年来国内外学者在PJS研究中所取得的共识与进展的基础上,结合自身经验;提出了PJS胃肠道息肉的中西医结合综合治疗设想.以期提高临床医生对PJS胃肠道息肉的诊治能力,从而使PJS患者能得到最大的临床获益. | 魏学明 顾国利 徐丽梅 毛高平 王石林 | 2011 | 世界华人消化杂志2011，19，30： | 7 |
| 6 | 儿童结肠息肉的CT影像学表现显示文摘目的:探讨儿童结肠息肉的特征性CT表现。方法:通过对2010年7月-2015年11月间病理诊断为结肠幼年性息肉的病例进行回顾性分析。结果:搜集的122例患儿中,发生在升结肠22例,横结肠18例,降结肠13例,乙状结肠28例,直肠41例,〈5mm的32例,5~10mm的53例,〉10mm的37例,结肠息肉可以表现为在充气肠管轮廓内的结节样软组织密度影。息肉CT平扫表现为等密度结节,局部与邻近肠管关系密切,增强后呈辐轮状强化表现,辐轮CT值约77~92HU之间,辐轮中心强化小血管影CT值约98~165HU之间,并延续至肠壁血管,辐轮间稍低密度影未见明显强化,CT值约23~27HU之间。在搜集的122例患儿中有105例患儿的结肠息肉表现为这种特征性的辐轮状强化,占到总例数的86%。结论:儿童结肠息肉具有特征性的辐轮状CT增强表现,可以作为与其他肠道病变进行鉴别的重要依据。 | 辛涛 于啸 | 2016 | 放射学实践2016，31，10： | 6 |
| 7 | 56例Peutz-Jeghers综合征临床分析显示文摘目的探讨Peutz-Jeghers综合征(PJS)的临床特点。方法回顾性分析1998年1月至2011年6月该院收治PJS综合征患者56例的临床资料。结果 56例患者中有PJS家族史患者共21例。56例患者可见胃、十二指肠息肉及结肠、直肠息肉。56例患者共行病理检查113例次,错构瘤66例次、腺瘤24例次、炎性息肉11例次、幼年性息肉5例次、增生性息肉3例次、息肉癌变2例次、错构瘤息肉合并腺瘤性息肉1例次、幼年性息肉合并炎性息肉1例次。结论 PJS是临床上较为少见但易于诊断、容易复发的疾病,一旦明确诊断,主张早期治疗。 | 刘海燕 兰春慧 孙文静 沈小春 李平 | 2013 | 重庆医学2013，42，8： | 6 |
| 8 | Molecular approach to genetic and epigenetic pathogenesis of early-onset colorectal cancer显示文摘Colorectal cancer(CRC) is the third most frequent cancer type and the incidence of this disease is increasing gradually per year in individuals younger than 50 years old. The current knowledge is that early-onset CRC(EOCRC) cases are heterogeneous population that includes both hereditary and sporadic forms of the CRC. Although EOCRC cases have some distinguishing clinical and pathological features than elder age CRC, the molecular mechanism underlying the EOCRC is poorly clarified. Given the significance of CRC in the world of medicine, the present review will focus on the recent knowledge in the molecular basis of genetic and epigenetic mechanism of the hereditary forms of EOCRC, which includes Lynch syndrome, Familial CRC type X, Familial adenomatous polyposis, Mut YH-associated polyposis, Juvenile polyposis syndrome, Peutz-Jeghers Syndrome and sporadic forms of EOCRC. Recent findings about molecular genetics and epigenetic basis of EOCRC gave rise to new alternative therapy protocols. Although exact diagnosis of these cases still remains complicated, the present review paves way for better predictions and contributes to more accurate diagnostic and therapeutic strategies into clinical approach. | gulcin tezcan berrin tunca secil ak gulsah cecener unal egeli | 2016 | World Journal of Gastrointestinal Oncology2016，8，1： | 5 |
| 9 | Update on imaging of Peutz-Jeghers syndrome显示文摘Peutz-Jeghers syndrome(PJS) is a rare, autosomal dominant disease linked to a mutation of the STK 11 gene and is characterized by the development of benign hamartomatous polyps in the gastrointestinal tract in association with a hyperpigmentation on the lips and oral mucosa. Patients affected by PJS have an increased risk of developing gastrointestinal and extra-digestive cancer. Malignancy most commonly occurs in the smallbowel. Extra-intestinal malignancies are mostly breast cancer and gynecological tumors or, to a lesser extent, pancreatic cancer. These polyps are also at risk of acute gastrointestinal bleeding, intussusception and bowel obstruction. Recent guidelines recommend regular smallbowel surveillance to reduce these risks associated with PJS. Small-bowel surveillance allows for the detection of large polyps and the further referral of selected PJS patients for endoscopic enteroscopy or surgery. Video capsule endoscopy, double balloon pushed enteroscopy,multidetector computed tomography and magnetic resonance enteroclysis or enterography, all of which are relatively new techniques, have an important role in the management of patients suffering from PJS. This review illustrates the pathological, clinical and imaging features of small-bowel abnormalities as well as the role and performance of the most recent imaging modalities for the detection and follow-up of PJS patients. | Catherine Tomas Philippe Soyer Anthony Dohan Xavier Dray Mourad Boudiaf Christine Hoeffel | 2014 | World Journal of Gastroenterology2014，20，31： | 5 |
| 10 | CT在黑斑息肉综合征中的应用价值(附5例报告)显示文摘目的:探讨黑斑息肉综合征(PJS)的CT表现及其临床诊断价值。方法:2014年2月至2015年3月本院经临床及病理证实的PJS患者5例,回顾性分析PJS的CT影像特点。结果:5例中3例因腹痛、黑便并多次呕吐就诊,CT表现为胃肠道多发息肉合并肠套叠(空肠-空肠);1例病史提示肠套叠患者,CT表现为套叠头部单发息肉并肠套叠;1例因腹部不适就诊,CT表现为胃多发息肉。术后病理结果类型多样,1例增生息肉,1例管状腺瘤息肉,3例错构瘤息肉。结论:PJS患者的主要CT表现为胃肠道多发息肉、肠套叠;CT检查主要用于明确息肉的大小及数量以及肠套叠的部位,或评估患者发生肠套叠的风险。 | 葛亮 刘洋 高剑波 郭华 | 2016 | 放射学实践2016，31，1： | 5 |
| 11 | 1例黑斑息肉综合征合并空肠套叠梗阻病例报道显示文摘患者女性,16岁,因“反复下腹痛1年,复发伴加重10 d”就诊。体格检查发现:患者口唇有散在点状黑斑,直径1~4 mm,界限清,不融合,压之不褪色;左上腹压痛。进一步完善实验室检查和影像学检查,以“1.肠套叠;2.肠道多发息肉;3.黑斑息肉综合征可能性大”收治入院。入院后行剖腹探查+十二指肠、空肠息肉切除术,术后明确诊断为黑斑息肉综合征。黑斑息肉综合征是一种临床罕见病,具有皮肤黏膜色素沉着、胃肠道多发性息肉及家族遗传倾向三大典型特征。介绍该病例的临床资料可提高对黑斑息肉综合征的再认识,为后续同类患者的诊断和治疗提供参考,减少紧急手术和短肠综合征的发生。 | 罗明旺 张兆祺 卫媛 闫文帝 严东旺 | 2021 | 上海交通大学学报(医学版)2021，41，8： | 4 |
| 12 | 多层螺旋 CT 在 Peutz-Jeghers 综合征的诊断价值显示文摘Peutz-Jeghers 综合征(Peutz-Jeghers syndrome,PJS)又称黑斑息肉病、色素沉着-息肉综合征等,是一种常染色体显性遗传性疾病,以胃肠道多发息肉、皮肤及黏膜黑斑为特征。PJS易并发肠梗阻、肠套叠等,病灶癌变发生率高。笔者收集本院2005-05-2013-09 PJS 患者11例,回顾性分析多层螺旋 CT 的表现特征,以提高对本病的认识和诊断正确率。 | 余捷 吴恩福 陈玲 蔡庆虎 林达 | 2014 | 实用放射学杂志2014，30，8： | 4 |
| 13 | 经典Wnt通路关键成员在Peutz-Jeghers综合征息肉组织中的表达显示文摘目的:探讨经典Wnt信号通路的关键成员-上皮性钙黏附素(E-Cadherin,E-Cad),β-链接素(β-catenin,β-Cat),基质金属蛋白酶-7(matrix metalloproteinase-7,MMP-7),C-Myc和细胞周期素D1(CyclinD1)在黑斑息肉病(Peutz-Jeghers syndrome,PJS)息肉组织中的表达及意义.方法:应用免疫组织化学SP法检测40例PJS息肉组织、20例大肠癌组织及20例正常大肠道黏膜组织中E-Cad、β-Cat、MMP-7、C-Myc、CyclinD1蛋白的表达情况.并结合其临床病理材料进行回顾性分析.结果:E-Cad、β-Cat、MMP-7、C-Myc、CyclinD1蛋白在正常黏膜组、PJS息肉组和大肠癌组中的表达具有显著差异.PJS息肉组中E-Cad和膜表达β-Cat随着患者年龄增长、息肉增大而表达减弱;而核表达β-Cat、CyclinD1、C-Myc、MMP-7则随着息肉增大而表达增强.E-Cad和膜表达β-Cat呈明显正相关,而与核表达β-Cat、CyclinD1、C-Myc、MMP-7均呈明显的负相关.而膜表达β-Cat与核表达β-Cat、CyclinD1、C-Myc、MMP-7呈明显的负相关;核表达β-Cat与CyclinD1、C-Myc、MMP-7呈明显的正相关;CyclinD1与C-Myc、MMP-7呈明显的正相关;C-Myc与MMP-7呈明显的正相关.结论:经典Wnt信号通路的异常活化,以及由Wnt所介导的上皮间质转化(epithelialmesenchymal transition,EMT)可能在PJS息肉的发生发展过程中扮演着重要角色.这可能与LKB1/mTOR信号通路的整合有关. | 陆相吉 顾国利 魏学明 任力 宁守斌 李德昌 | 2013 | 世界华人消化杂志2013，21，8： | 4 |
| 14 | 小肠镜联合部分肠段切除治疗P-J综合征显示文摘[目的]探讨单气囊小肠镜联合肠段切除治疗P-J综合征的优越性及临床疗效。[方法]回顾分析了接受单气囊小肠镜联合肠段切除治疗的7例复杂P-J综合征患者的临床资料。[结果]所有患者通过小肠镜摘除大部分息肉后,因为肠套叠、肠黏连、息肉过大或密集、息肉可疑恶变原因行开腹肠段切除术。术后患者均恢复良好,顺利出院。其中1例患者病理证实恶变。[结论]单气囊小肠镜联合肠段切除能显著改善P-J综合征患者治疗的疗效。 | 陈勇 骆晓业 潘锋 肖国虎 郑锋 林仙明 | 2014 | 肿瘤学杂志2014，20，7： | 4 |
| 15 | 黑斑息肉综合征及其并发症2例报告并文献复习显示文摘目的 探讨黑斑息肉综合征及其常见并发症的诊断及治疗. 方法 回顾性分析本院收治的两例P-J综合征患儿的临床资料.结果 病例1术后病理检查结果提示结肠息肉部分腺上皮细胞增生,符合P-J息肉改变,诊断为P-J综合征、肠套叠.病例2术后病理检查结果提示幼年性息肉并绒毛管状腺瘤,部分区域腺上皮伴轻度不典型增生.两例均好转出院. 结论 P-J综合征常并发肠梗阻,应予尽早以手术治疗. | 颜禄斌 李斌德 李来元 李刚 沈阳 谢良恩 | 2013 | 临床小儿外科杂志2013，12，5： | 4 |
| 16 | 小肠CT联合单气囊小肠镜对黑斑息肉综合征的诊治价值显示文摘目的探讨小肠CT联合单气囊小肠镜诊治黑斑息肉综合征(PJS)胃肠道多发息肉的应用价值。方法对临床拟诊或诊断为PJS的5例患者行小肠cT检查,观察小肠息肉数目、大小、部位、相关肠道急性并发症等。根据小肠CT检查结果,采用单气囊小肠镜对小肠息肉进行相应镜下治疗,分析小肠息肉数目、大小、完成时间和并发症等。结果5例患者经小肠CT检查,发现大小不等小肠息肉,其中直径〉1.0cm的息肉共计68枚,最大的直径约4cm。共完成9例次小肠镜下治疗,平均完成时间120min,发现直径〉1.0cm的小肠息肉65枚,与小肠CT符合率为95.59%;共摘除息肉52枚,最大直径4cm,术后未发生并发症。结论小肠CT联合单气囊小肠镜诊治PJS胃肠道多发息肉安全、有效。 | 蒋彩凤 姚明荣 施斌 | 2017 | 中华消化内镜杂志2017，34，11： | 4 |
| 17 | 黑斑息肉综合征一例基因分析及临床诊治显示文摘目的:进一步掌握黑斑息肉综合征(PJS)的临床诊治重点。方法:回顾性分析一例PJS患者55 a的病史资料和6 a随访资料。采用PCR和测序法对患者进行STK11基因筛查。结果:该患者有典型的口唇黏膜色素沉着,反复多发消化道息肉并出现肠套叠1次,经基因检测提示携带STK11基因c.910C→T(p.R304W)杂合突变。经积极诊治,至今患者病情稳定,状况良好。结论:内镜定期监测消化道息肉发生和积极干预治疗可改善该病的预后;对PJS家庭开展基因筛查和妊娠相关的遗传咨询有重要意义。 | 杨治宇 吴庆华 信艳萍 张毅 史惠蓉 | 2018 | 郑州大学学报(医学版)2018，53，2： | 3 |
| 18 | Pentz-Jegher综合征1例显示文摘患者女,27岁,因"反复上腹痛十余年,加重2个月"入院。患者6岁时发现口唇、鼻周出现点片状黑褐色斑,并逐渐增多,累及额面部、双侧手指、脚掌跖等,以口唇及鼻周明显。15岁时曾因肠套叠行手术治疗(术中具体情况不详)。 | 张瑜 王磊 郭继中 | 2010 | 实用医学杂志2010，26，20： | 3 |
| 19 | 多镜联合治疗P-J综合征并肠套叠1例显示文摘P-J综合征(Peutz-Jeghers syndrome)是一种临床上少见的家族遗传性、常染色体显性遗传病,又称色素沉着息肉综合征。我们回顾分析1例P-J综合征并肠套叠患者的临床资料及实施结肠镜、腹腔镜、胃镜联合,多学科合作微创诊疗经过,并复习相关文献,以期为P-J综合征的临床诊治提供参考。 | 刘勇峰 李亚军 孟步军 | 2021 | 温州医科大学学报2021，51，7： | 3 |
| 20 | 大肠非肿瘤性息肉的分类及病理学特征显示文摘大肠息肉(colorectal polyps)是指所有向大肠腔内突出的赘生物的总称,包括肿瘤性息肉(neoplastic colorectal polyps)和非肿瘤性息肉(non-neoplastic colorectal polyps)两大类。大肠非肿瘤性息肉主要分为炎症性息肉、错构瘤性息肉及其它类型的息肉性病变三大类[1-2]。本文着重对大肠非肿瘤性息肉的分类及病理学特征予以阐述,为病理诊断提供帮助。 | 姜磊 焦宇飞 | 2015 | 中华结直肠疾病电子杂志2015，4，4： | 3 |
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