324例肠神经元发育异常患儿病理特点与术后并发症关系分析

目的 探讨肠神经元发育异常的病理特点及其与术后并发症之间的关系。方法 总结324例肠神经元发育异常患儿的术后病理检查的特点,并对照相应的治疗效果和术后并发症,进行统计分析。结果 324例患儿中,先天性巨结肠（Hirschsprung＇s disease,HD）210例,肠神经元发育不良（intestinal neuronal dysplasia,IND）38例,HD伴IND45例,神经节细胞减少症8例,HD伴神经细胞节减少22例,神经节细胞未成熟症1例。不同病理类型在扩张段神经元正常的比例分别为HD88,1％,HD伴IND24,4％,IND18,4％,神经节细胞减少症4／8,HD伴神经细胞节减少27．7％,神经节细胞未成熟症0／1。全组有46例患儿术后出现反复小肠结肠炎（EC）。HD、HD伴IND、IND患儿的术后反复EC发生率分别为6,7％、35,6％、28．9％;切缘正常组与切缘IND组术后反复EC发生率分别为8,7％、38．2％;经肛门手术和经腹手术术后EC的发生率分别为18．0％和8．3％。术后仍有反复腹胀、便秘或严重的EC行再次手术9例,其中4例为HD伴IND,1例为IND,3例HD,1例HD伴神经细胞节减少。结论 肠神经元发育异常的神经元分布与大体病理改变有不平衡性,巨结肠同源病较HD神经元分布更不典型;单纯HD的治疗效果较好,术后小肠结肠炎发生率低;切缘仍有IND病变以及经肛门手术是术后反复EC的危险因素;术中冰冻切片对判断切缘神经元有重要意义;IND的切除范围仍有不确定性。     

B型肠神经元发育异常症的诊断与手术治疗

目的：了解B型肠神经元发育异常症的诊断与手术治疗效果。方法：对45例该病患儿进行回顾性分析及远期随访。术前所有患儿均行钡灌肠X线检查，23例行下消化道动力学检测，17例行直肠粘膜活检加S100蛋白免疫组织化学染色。所有患儿均行病变肠段切除、结肠直肠吻合术。行多处全层活检手术标本。结果：本组中16例为单纯性B型肠神经元发育异常症，28例合并先天性巨结肠，1例合并肠神经节细胞减少。16例单纯性行钡灌肠X线检查时仅有4例可见确切的狭窄段，9例单纯性中有6例未出现直肠肛管抑制反射，17例活检中只有7例可获得提示性诊断。全层活检可准确诊断该病。术后3例发生小肠结肠炎患儿经保守治疗痊愈，1例发生闸门症候群而再次手术，其他患儿均能自解、自控大便。结论：全层活检是诊断该病的可靠方法。病变肠段切除、结肠直肠吻合术治疗本病可获得满意的疗效。     

胃肠道炎性纤维性息肉的临床与病理分析

目的 探讨胃肠道炎性纤维性息肉（IFP）的诊断、病理学特点及治疗原则。方法 对1984年～1999年经治的12例IFP的临床和病理资料进行分析总结。结果 12例均表现为胃肠道粘膜下息肉样肿块,以纤维血管增生,呈特征性排列和嗜酸性粒细胞浸润为其组织学特征,免疫组化结果显示病变组织表达纤维和肌纤维母细胞标记。本组均经手术切除或内镜下摘除。11例获随访,其中2例出现复发,均予再次手术切除;其余病例均恢复满意。结论 IFP是胃肠道少见的良性病变,可能是对感染、异物或损伤等的过度反应;病理上有特征性组织学改变,应注意与胃肠道肿瘤或其它息肉样病变的鉴别;手术切除或内镜下摘除为其主要治疗方法。     

原位肛门重建的实验研究及临床应用

对４８只家猫采用４种不同术式行原位肛门重建，即Ａ、Ｂ、Ｃ三种切除肛门内外括约肌，其中Ａ组游离股薄肌包裹，Ｂ组结肠套叠，Ｃ组结肠套叠加股薄肌包裹，Ｄ组结肠套叠保留肛门外括约肌。术前术后１、３、６个月测量重建肛管及直肠内压，并观察猫的排便情况，结果第Ｃ、Ｄ组有意义的高于ＡＢ两组，将ＣＤ组术式应用临床２５例，术后控便功能优良者占８４％。     

胰胆管合流异常与胆道疾病的关系

我院自1980-1994年间经影像学检查证实胰胍管合流异常28例．其中合并胆总管囊状扩张15例，柱状扩张7例，胆囊癌变4例，胆总管癌变1例。本文就胰胆管合流异常的慨念，分型及与胆道疾患发病之间的关系，病理和病因方面进行了讨论，井对胰胆管台流异常并发胆道疾患时的特殊处理原则进行探讨。     

215例性发育异常疾病的分类比较研究

目的了解性发育异常疾病谱的变化,检验葛氏性发育异常疾病分类法的实用性和有效性。方法统计北京协和医院1996至2006年间性发育异常215例,根据葛氏性发育异常疾病分类法进行分类,并与1994年性发育异常疾病谱和性腺肿瘤发生率进行比较分析。结果215例性发育异常均能按葛氏性发育异常疾病分类法归人性染色体、性腺与性激素异常三大类;11年期间发生率较高的是性激素异常类性发育异常疾病。结论葛氏性发育异常疾病分类法是一种实用、简单、开放的分类法,并将随着对性发育异常疾病的认识而不断充实、发展。近年来性发育异常疾病的疾病谱和性腺肿瘤发生率也发生了一定的改变。     

肛门直肠测压技术及其临床应用

肛门直肠运动功能障碍性疾病与肛门直肠动力及感觉功能密切相关,进行肛门直肠压力及感觉检测已成为研究肛门直肠运动功能障碍性疾病生理和病理机制的重要手段之一。本文对肛门直肠测压技术的适应症及临床意义做一综述。     

儿童特发性便秘的诊断(86例临床分析)

目的：探讨儿童特发性便秘的诊断。方法：对2000年6月至2002年10月经结肠造影和肛门直肠测压诊断的86例特发性便秘的临床资料进行分析。结果：男44例，女42例，婴儿期9例，幼儿期66例，学龄期11例。结肠造影检查86例，其中未见异常75例，直肠扩张5例，乙状结肠冗长3例，直肠扩张合并乙状结肠冗长3例；肛门直肠测压44例，其中未见异常16例，排便弛缓反射异常12例，直肠肛门抑制反射(RAIR)异常22例，感觉功能障碍10例。获得随访68例，其中痊愈47例(69．1％)，好转11例(16．2％)，无效10例(14．7％)。结论：结肠造影结合肛门直肠测压检查可提高儿童特发性便秘的诊断。     

肛管直肠动力学改变与肛门疾病关系的研究

目的探讨肛管直肠动力学改变与肛门疾病(痔、裂)的关系。方法分别记录正常人、有肛交史者、肛门疾病患者肛管舒张压、肛管静息压、直肠静息压、肛管最大收缩压、直肠感知阈值、直肠最大耐受量,并进行比较。结果三者差异均有统计学意义(P<0.05)。结论肛管直肠动力学改变与肛门疾病关系密切。     

高分辨肛门直肠测压的最新研究进展

肛门直肠测压是评价肛门张力、直肠顺应性、肛门直肠感觉以及证实直肠肛门抑制反射完整性的客观检测方法,通过获得的有关肛管直肠功能的信息,了解、量化和评估肛管、直肠控便功能,并为研究排便功能障碍、大便失禁以及肛管直肠外伤等疾病提供病理生理学依据。近年来,     

Inter- and intraindividual reproducibility of anorectal manometry

Background: This study investigates the inter- and intraindividual variability of normal values and, thus, the reproducibility of anorectal manometry. Materials and Methods: Following a standardized protocol, three anorectal manometries were performed 4 h apart on 2 days of investigation, with an interval of 4 weeks, in ten healthy volunteers. Measured parameters in all 60 manometries were: sphincter length (SL), resting pressure (RP), maximum squeeze pressure (MSP), relaxation of the internal anal sphincter (RIAS), and rectal compliance (RC). Interindividual variability was expressed as standard deviation from calculated mean values and intraindividual variability was tested with Wilcoxon's test for tied samples and Spearman's rank correlation test. Results: A large interindividual variability was found for all measured parameters, except for SL, reflecting the extensive absolute range of measured values. Median intraindividual variability among the six individual measurements and between both measurement days revealed that MSP, RIAS and RC are parameters which were not reproducible in this volunteer study. A significant correlation between the results of the repetitive measurements and, thus, a good reproducibility was only found for the parameters SL and RP. Conclusions: Anorectal manometry has only limited diagnostic value; although rather exact quantifications of individual para-meters can be achieved, the impact of these measurements should be regarded rather critically, since only SL and RP appeared to be reproducible parameters. Received: 25 May 1998 / Accepted: 13 July 1998     

原发性小肠淋巴瘤临床特点及生存分析

目的对原发性小肠淋巴瘤的临床及病理、治疗方法及特点、预后进行分析.方法：回顾性分析天津市肿瘤医院及天津市南开医院1993年1月-2009年1月经病理证实的48例原发性小肠淋巴瘤患者的临床资料,并比较1993年1月-2001年1月（A组）和2001年2月-2009年1月（B组）两个时期的临床特点、生存分析.结果：A组患者15例,发生于十二指肠4例（27%）,空肠2例（13%）,回肠9例（60%）;临床表现腹痛9例（60%）,腹胀6例（40%）,腹部包块5例（33%）,;15例均行手术治疗;3年生存率46% ,IE期2例（13%）.B组患者33例,发生于十二指肠5例（15%）,空肠8例（24%）,回肠18例（55%）,回盲部2例（6%）;临床表现为腹痛21例（63%）,腹部包块10例（30%）,消化道出血8例（24%）;33例均行手术治疗,29例辅助化疗,11例CD20（＋）患者接受了美罗华治疗;3年生存率为72%,IE期11例（33%）.结论：原发性小肠淋巴瘤首发症状多为腹痛, 诊断率上升,最好发的部位是空肠, 早期肿瘤数量增多,最常见的组织学类型是弥漫大细胞非霍奇金淋巴瘤,采用手术为主的综合治疗,其预后与病理类型和临床分期有关.B组联合靶向治疗后,3年生存率明显增高.     

Comparison of clinical outcomes and anorectal manometry in patients with congenital anorectal malformations treated with posterior sagittal anorectoplasty and laparoscopically assisted anorectal pull through

Purpose

The objective of this study is to analyze the clinical outcomes and anorectal manometry (AM) in infants with congenital high anorectal malformations treated with posterior sagittal anorectoplasty (PSARP) and laparoscopically assisted anorectal pull through (LAARP).

Materials and Methods

From August 2005 to December 2008, 23 patients with congenital high anorectal malformations were randomly distributed into PSARP and LAARP groups. All of them underwent LAARP (11 cases) or PSARP (12 cases) at 2 or 3 months old. Clinical outcomes and results of anorectal manometry were compared between patients at the age of 17.4 ± 4.9 and 19.3 ± 6.2 months (P = .4270), respectively.

Results

Kelly's clinical score for patients in LAARP and PSARP groups was 3.91 ± 1.14 and 3.83 ± 1.40 (P = .8827), respectively. Anal canal resting pressure and high-pressure zone length were 29.4 ± 7.2 vs 23.4 ± 6.5 mm Hg (P = .0479) and 14.9 ± 3.0 vs 13.9 ± 3.1 mm (P = .4414), respectively. Rectal anal inhibitory reflex was observed in 81.8% (9/11) and 83.3% (10/12) patients (P = 1.0000), respectively. The mean length of stay during the second hospitalization was 10.6 ± 0.9 and 14.3 ± 1.4 days (P < .0001), respectively.

Conclusions

Although no significant difference can be noted in clinical scoring between both groups, the results of anorectal manometry indicate that LAARP can significantly improve anal canal resting pressure and reduce the length of stay.     

老年性腰椎间盘突出症的病理特点及手术治疗(附49例分析)

对49例老年性腰椎间盘突出症的病理特点进行研究。自1989年～1996年共手术治疗老年性腰椎间盘突出症49例,占同期腰椎间盘突出症手术的5％。行小切口开窗髓核摘除36例,扩大开窗髓核摘除10例,半椎板切除髓核摘除3例。随访1年～8.5年,平均4年,优良率47.2％。作者分析了手术疗效差的三个原因,提出了符合老年性腰椎间盘突出症的三种手术方式及适应证。     

功能性便秘患儿肛门内括约肌检测的临床意义

目的探讨功能性便秘患儿肛门内括约肌结构及功能的改变以及内括约肌厚度与临床症状严重程度的关系。方法对中国医科大学附属盛京医院2008年6—12月间收治的35例功能性便秘患儿（便秘组,其中合并失禁者17例）进行肛门直肠测压和肛管内B超检测,并与同期住院治疗的23例经检查排除消化道和内分泌疾病患儿（对照组）进行对照．采用儿童便秘临床症状评分标准（65分法）评估症状的严重程度。结果便秘组与对照组相比．直肠感知阈值明显增加[（42．4±19．5）ml比（29．1±15．6）ml,P〈0．05）;引起肛门内括约肌松弛反射的最低充气量明显增高[（55．6±31．6）ml比（30．5±13．8）ml,P〈0．05）;肛门内括约肌明显增厚[（3．8±1．7）mm比（2．5±1．0）mm,P〈0．05）;但两组肛管静息压[（170．8±62．3）mmHg比（161．3±51．1）mmHg]的差异无统计学意义（P〉0．05）。便秘组患儿临床症状评分为（9．3±4．3）分：肛门内括约肌厚度与临床症状评分呈正相关（r=0．407,P=0．015）;与患儿年龄、性别及病程则无相关性（均P〉0．05）。结论功能性便秘患儿肛门内括约肌结构与功能均会发生改变。肛门内括约肌厚度在一定程度上可以反映便秘症状严重程度。     

OOC患者血清胆囊收缩素与直肠肛管压力的相关性研究

目的：研究直肠肛菅压力及血清胆囊收缩素（CCK）的改变在功能性出口梗阻型便秘（OOC）患者发病中的作用。方法：采用四通道环状固态压力传感器测压导管测定50例OCC患者直肠肛管压力;采用酶联免疫吸附测定（ELISA）检测其中20例血清CCK水平,并与20例正常人做对照。结果：与正常对9最组相比,OCC患者的肛管静息压、最大缩榨压明显降低,引起直肠初始感觉、初始便意感觉和最大感觉阈值的容积均明显升高（P值均〈O．01）。OCC组血清CCK水平显著低于正常对照组,与最大感觉阈值呈负相关。结论：OCC患者存在直肠肛管压力异常、直肠感觉功能下降及血清CCK的异常。直肠容量的感知能力与血清CCK含量呈负相关。     

A clinical and manometric correlation for assessment of postoperative continence in imperforate anus

Functional results after surgical correction of anorectal malformations were assessed on a clinical basis following Kelly score and by manometric study. Forty-six patients, aged 2--17, were personally interviewed and 25 of these 46 had manometric studies to evaluate postoperative continence. The manometric study was also performed on 35 normal children as a control group. Continent patients characteristically had marked high pressure zones as did the normal subjects. On the other hand, in the patients with fair or poor results, the anorectal pressure profile had no marked high pressure zone in the anal canal. The presence of normal anal pressure at rest as well as adequate ano-rectal pressure difference was found to correlate well with continence. In patients with perineoplasty, the ano-rectal reflex correlated well with continence but not in patients treated by abdominoperineal rectoplasty.